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1. Toyama K, Oka H, Obata K, Ogawa H: Primary systemic amyloidosis with bloody pericardial effusion. Intern Med; 2009;48(10):821-6
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  • [Title] Primary systemic amyloidosis with bloody pericardial effusion.
  • Pathological autopsy revealed primary systemic amyloidosis.
  • Pathologically it was possible that the local inflammation (epicarditis) due to the deposition of amyloid in the epicardium and perivascular tissue caused the bloody effusion.
  • There are no reports of primary systemic amyloidosis with hemorrhagic pericardial effusion.
  • [MeSH-major] Amyloidosis / complications. Pericardial Effusion / etiology
  • [MeSH-minor] Aged. Fatal Outcome. Female. Heart Diseases / pathology. Hemorrhage / diagnosis. Hemorrhage / etiology. Humans. Pericardium / pathology. Tachycardia, Ventricular / etiology

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  • (PMID = 19443978.001).
  • [ISSN] 1349-7235
  • [Journal-full-title] Internal medicine (Tokyo, Japan)
  • [ISO-abbreviation] Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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2. Matsuda M, Gono T, Shimojima Y, Yoshida T, Katoh N, Hoshii Y, Yamada T, Ikeda S: AL amyloidosis manifesting as systemic lymphadenopathy. Amyloid; 2008 Jun;15(2):117-24
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  • [Title] AL amyloidosis manifesting as systemic lymphadenopathy.
  • We report three patients with AL amyloidosis manifesting as systemic lymphadenopathy, mainly in the cervical and supraclavicular regions.
  • Histopathology of lymph nodes showed massive deposition of AL amyloid with no abnormal findings suggestive of lymphoproliferative disorders.
  • Two of the patients were considered to be classifiable as primary systemic AL amyloidosis based on the presence of M-protein in serum and abnormal plasma cells or lymphoplasmacytoid cells in the bone marrow probably producing the precursor immunoglobulin, although no visceral organs were affected.
  • The size of the involved lymph nodes in these two patients increased gradually, and one was treated with rituximab and VAD (vincristine, doxorubicin and dexamethasone) followed by high-dose melphalan with autologous peripheral blood stem cell transplantation (auto-PBSCT).
  • The prognosis of AL amyloidosis manifesting as lymphadenopathy is usually good as long as there are no hematological malignancies or rapid increases in the size of lymph nodes, but in cases of the systemic type, intensive chemotherapy, such as high-dose melphalan with auto-PBSCT, should be actively considered in order to avoid possible involvement of visceral organs.
  • [MeSH-major] Amyloidosis / diagnosis. Lymphatic Diseases / diagnosis
  • [MeSH-minor] Aged. Amyloid / metabolism. Antineoplastic Agents, Alkylating / therapeutic use. Diagnosis, Differential. Female. Humans. Lymph Nodes / metabolism. Lymph Nodes / pathology. Male. Melphalan / therapeutic use. Middle Aged. Peripheral Blood Stem Cell Transplantation. Transplantation, Autologous

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  • (PMID = 18484338.001).
  • [ISSN] 1744-2818
  • [Journal-full-title] Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
  • [ISO-abbreviation] Amyloid
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid; 0 / Antineoplastic Agents, Alkylating; Q41OR9510P / Melphalan
  • [Number-of-references] 22
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3. Chiti F, Dobson CM: Protein misfolding, functional amyloid, and human disease. Annu Rev Biochem; 2006;75:333-66
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  • [Title] Protein misfolding, functional amyloid, and human disease.
  • Such transitions can give rise to pathological conditions ranging from neurodegenerative disorders to systemic amyloidoses.
  • In this review, we identify the diseases known to be associated with formation of fibrillar aggregates and the specific peptides and proteins involved in each case.
  • We review recent advances toward the elucidation of the structures of amyloid fibrils and the mechanisms of their formation at a molecular level.
  • Finally, we discuss the relative importance of the common main-chain and side-chain interactions in determining the propensities of proteins to aggregate and describe some of the evidence that the oligomeric fibril precursors are the primary origins of pathological behavior.
  • [MeSH-major] Amyloid. Amyloidosis. Neurodegenerative Diseases. Protein Conformation. Protein Folding

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  • (PMID = 16756495.001).
  • [ISSN] 0066-4154
  • [Journal-full-title] Annual review of biochemistry
  • [ISO-abbreviation] Annu. Rev. Biochem.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid
  • [Number-of-references] 179
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4. Borovkov NN, Amineva NV, Kovaleva GV, Sal'tseva MT, Paĭkova NN, Kuznetsov SS: [Primary generalized amyloidosis with restrictive cardiomyopathy and severe chronic cardiac insufficiency]. Klin Med (Mosk); 2009;87(12):60-2
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  • [Title] [Primary generalized amyloidosis with restrictive cardiomyopathy and severe chronic cardiac insufficiency].
  • The paper reports a case of restrictive cardiomyopathy due to cardiac amyloidosis.
  • Diagnosis of this condition encounters difficulty created by the absence of pathognomonic symptoms of the disease.
  • Major manifestations of amyloid cardiomyopathy are refractive chronic cardiac insufficiency, absence of cardiomyalgia, marked deterioration of diastolic filling of both ventricles, systemic hypotension, and disturbed heart rhythms.
  • [MeSH-major] Amyloidosis / complications. Cardiomyopathy, Restrictive / complications. Heart Failure / etiology


5. Peers C, Pearson HA, Boyle JP: Hypoxia and Alzheimer's disease. Essays Biochem; 2007;43:153-64
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  • [Title] Hypoxia and Alzheimer's disease.
  • Numerous cardiorespiratory disorders result in persistent systemic hypoxia, or at worst (as a consequence of stroke) deprive the brain of oxygen completely for a period of time.
  • Patients suffering from such conditions are much more susceptible to the development of dementias such as AD (Alzheimer's disease).
  • Until recently, the cellular and molecular basis for the predisposition to AD by systemic hypoxia has been completely unknown.
  • Furthermore, prolonged hypoxia can induce formation of Abetas (amyloid beta peptides), the primary neurotoxic elements of AD, which accumulate over years to form the extracellular plaques that are the hallmark feature of the disease.
  • [MeSH-major] Alzheimer Disease / pathology. Hypoxia

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  • (PMID = 17705799.001).
  • [ISSN] 0071-1365
  • [Journal-full-title] Essays in biochemistry
  • [ISO-abbreviation] Essays Biochem.
  • [Language] eng
  • [Grant] United Kingdom / Medical Research Council / / G0600936; United Kingdom / Wellcome Trust / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Calcium Channels; 0 / Reactive Oxygen Species; S88TT14065 / Oxygen; SY7Q814VUP / Calcium
  • [Number-of-references] 33
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6. Villa F, Dionigi G, Tanda ML, Rovera F, Boni L: Amyloid goiter. Int J Surg; 2008;6 Suppl 1:S16-8
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  • [Title] Amyloid goiter.
  • BACKGROUND AND AIM: Amyloidosis refers to a variety of conditions in which amyloid proteins are abnormally deposited in organs and/or tissues.
  • Amyloidosis is a rare occurrence in thyroid gland.
  • METHODS: A systematic review of the published data on amyloid goiter was carried out by searching Medline and other online databases (such as Scopus and Endnote) for the period from 1951 to March 2008.
  • Six articles have been considered for our review because they regard amyloid goiter as a manifestation of both primary and secondary amyloidosis (a total of 30 cases have been analyzed).
  • Exclusion criterion was the presence of primary thyroid cancer.
  • RESULTS: The preoperative diagnosis of amyloid goiter should be considered in patients with known systemic amyloidosis or with a long-standing predisposing disease who present a rapidly growing thyroid volume in association with a euthyroid state.
  • Fine-needle aspiration biopsy can be performed to exclude primary malignant lesions of thyroid gland and immunohistochemical studies can identify and characterize the amyloid deposits.
  • CONCLUSION: Amyloid goiter has to be suspected in all patients with a progressive, rapidly growing, bilateral thyroid enlargement and a concomitant history of chronic inflammatory processes.
  • Moreover, this should be suspected in patients who are known to have disease predisposing to amyloid deposition.
  • [MeSH-major] Amyloidosis / complications. Goiter / etiology. Thyroid Gland / pathology
  • [MeSH-minor] Biopsy, Fine-Needle. Diagnosis, Differential. Humans. Tomography, X-Ray Computed

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  • (PMID = 19168408.001).
  • [ISSN] 1743-9159
  • [Journal-full-title] International journal of surgery (London, England)
  • [ISO-abbreviation] Int J Surg
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 8
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7. Kim UR, Khazaei H, Stewart WB, Shah AD: Spectrum of orbital disease in South India: an aravind study of 6328 consecutive patients. Ophthal Plast Reconstr Surg; 2010 Sep-Oct;26(5):315-22
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  • [Title] Spectrum of orbital disease in South India: an aravind study of 6328 consecutive patients.
  • AIM: To review the incidence of orbital diseases in South India and to compare with other case series published.
  • METHODS: Retrospective review of 6328 consecutive patients with orbital disease evaluated at Aravind Eye Hospital between January 1997 and December 2008.
  • The main outcome measure was incidence of orbital disease in South Indian population, as determined by clinical and histopathologic criteria.
  • RESULTS: Of the 6328 patients, 2161 (34.1%) had inflammatory orbital disease, 1965 (31.0%) had systemic conditions involving the orbit, 1277 (20.1%) had neoplasm, 600 (9.4%) had congenital lesions, 308 (4.8%) had trauma, and 17 (0.2%) had vascular disease.
  • Of the 2161 patients presenting with inflammatory disease, 1473 (68.1%) had idiopathic orbital inflammation, 270 (12.5%) had infection, 126 (5.8%) had dacryoadenitis, and 292 (13.5%) had other etiologies.
  • Among the 1965 patients presenting with systemic disease involving the orbit, 1938 (98.6%) were diagnosed with thyroid orbitopathy, 22 (1.1%) with amyloidosis, and 5 (0.2%) with sarcoidosis.
  • CONCLUSIONS: The most common causes of orbital disease in South India are inflammatory (34.1%) and systemic conditions (31.0%).
  • [MeSH-major] Orbital Diseases / epidemiology

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  • (PMID = 20592641.001).
  • [ISSN] 1537-2677
  • [Journal-full-title] Ophthalmic plastic and reconstructive surgery
  • [ISO-abbreviation] Ophthal Plast Reconstr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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8. Sainz-Esteban A, Saornil-Alvarez MA, Méndez-Díaz MC, Blanco-Mateos G: [Primary localized conjunctival amyloidosis: two case reports]. Arch Soc Esp Oftalmol; 2005 Jan;80(1):49-52
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  • [Title] [Primary localized conjunctival amyloidosis: two case reports].
  • [Transliterated title] Amiloidosis primaria conjuntival: análisis de dos casos.
  • The conjunctival biopsy revealed the presence of amyloid within the conjunctiva itself.
  • CONCLUSIONS: Primary localized conjunctival amyloidosis is a rare disease.
  • Diagnosis consists of biopsy in order to detect amyloid material in the conjunctival tissue together with a systemic evaluation in order to rule out the presence of primary systemic amyloidosis.
  • [MeSH-major] Amyloidosis / diagnosis. Conjunctival Diseases / diagnosis

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  • (PMID = 15692895.001).
  • [ISSN] 0365-6691
  • [Journal-full-title] Archivos de la Sociedad Española de Oftalmología
  • [ISO-abbreviation] Arch Soc Esp Oftalmol
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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9. Kyle RA, Rajkumar SV: Monoclonal gammopathies of undetermined significance. Best Pract Res Clin Haematol; 2005;18(4):689-707
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  • The monoclonal gammopathies include multiple myeloma (MM), monoclonal gammopathy of undetermined significance (MGUS), primary systemic amyloidosis (AL), and Waldenström's macroglobulinemia (WM).
  • MGUS is characterized by the presence of a serum monoclonal protein value <3 g/dL, fewer than 10% plasma cells in the bone marrow, no or a small amount of monoclonal protein in the urine, and absence of lytic bone lesions, anemia, hypercalcemia, or renal insufficiency related to the plasma-cell proliferative process.
  • During long-term follow-up of 241 patients with MGUS seen at Mayo Clinic from 1956 to 1970, MM, WM, AL, or a related disorder developed in 64.
  • The risk of progression to a malignant plasma-cell disorder was 1% per year.
  • [MeSH-minor] Cell Transformation, Neoplastic. Diagnosis, Differential. Disease Progression. Humans

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  • (PMID = 16026745.001).
  • [ISSN] 1521-6926
  • [Journal-full-title] Best practice & research. Clinical haematology
  • [ISO-abbreviation] Best Pract Res Clin Haematol
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 62242
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] England
  • [Number-of-references] 80
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10. Sharma PP, Payvar S, Litovsky SH: Histomorphometric analysis of intramyocardial vessels in primary and senile amyloidosis: epicardium versus endocardium. Cardiovasc Pathol; 2008 Mar-Apr;17(2):65-71
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  • [Title] Histomorphometric analysis of intramyocardial vessels in primary and senile amyloidosis: epicardium versus endocardium.
  • BACKGROUND: The literature is unclear as to the frequency and degree of vascular involvement among the various types of cardiac amyloidosis, specifically the senile type.
  • METHODS: We analyzed the intramyocardial vascular involvement, both qualitatively and quantitatively, in 18 patients with autopsy-proven cardiac amyloidosis [11 with primary amyloidosis (AL) and 7 with senile systemic amyloidosis (SSA)].
  • RESULTS: AL patients (10/11) showed focally transmural vascular involvement by amyloid with thickening of the wall and impingement on the lumens.
  • In SSA patients (6/7), the amyloid deposition was concentrated largely in the adventitia and external media.
  • Histomorphometric analysis revealed that all patients with AL, SSA, and heart failure without amyloidosis had greater luminal areas than normal controls.
  • In addition, the median of the ratio of subendocardial to subepicardial vascular luminal areas was 0.59 in AL patients, 1.17 in SSA patients, 0.94 in heart failure without amyloidosis patients, and 0.89 in normal controls (P=.034).
  • All patients with heart failure, with and without amyloidosis, displayed greater luminal areas than normal controls.
  • This may be due to transmural vascular involvement by amyloid in these patients, which concomitantly decreases the transmural ventricular perfusion pressure, leading to compensatory expansion of the subepicardial vessels.
  • [MeSH-major] Amyloidosis / pathology. Cardiomyopathies / pathology. Coronary Vessels / pathology. Endocardium / pathology. Myocardium / pathology. Pericardium / pathology
  • [MeSH-minor] Adult. Aged. Amyloid / isolation & purification. Amyloid / metabolism. Dilatation, Pathologic / pathology. Female. Heart. Humans. Male. Middle Aged. Organ Size

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  • (PMID = 18329550.001).
  • [ISSN] 1054-8807
  • [Journal-full-title] Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology
  • [ISO-abbreviation] Cardiovasc. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid
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11. Zubkov AY, Rabinstein AA, Dispenzieri A, Wijdicks EF: Primary systemic amyloidosis with ischemic stroke as a presenting complication. Neurology; 2007 Sep 11;69(11):1136-41
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  • [Title] Primary systemic amyloidosis with ischemic stroke as a presenting complication.
  • BACKGROUND: Amyloidosis is an uncommon disorder that ultimately leads to fatal multiorgan failure.
  • The purpose of this study was to review the pathophysiologic relationship between primary systemic amyloidosis and ischemic stroke, and to determine how often stroke is the first defining manifestation.
  • METHODS: Retrospective study of 49 patients with confirmed primary amyloidosis and ischemic stroke.
  • All included patients had biopsy proven amyloidosis.
  • Ischemic strokes occurred in 13 patients (32.5%) as the initial presentation of amyloidosis.
  • Patients with initial stroke presentation had the worst outcome, with average survival of 6.9 months after established diagnosis with amyloidosis; strokes developed 9.6 months before diagnosis with primary amyloidosis.
  • CONCLUSIONS: Ischemic stroke is an underappreciated complication of primary amyloidosis.
  • In the absence of obvious clinical and cardiogenic manifestations, primary amyloidosis should be considered when echocardiography demonstrates thickening of the valves, restrictive pattern, and increased echogenicity.
  • Ischemic strokes as an initial presentation of primary amyloidosis carries a worse prognosis.
  • [MeSH-major] Amyloidosis / complications. Amyloidosis / physiopathology. Brain Ischemia / etiology. Brain Ischemia / physiopathology. Stroke / etiology. Stroke / physiopathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Atrial Fibrillation / complications. Atrial Fibrillation / physiopathology. Atrial Fibrillation / ultrasonography. Echocardiography. Female. Heart Diseases / complications. Heart Diseases / physiopathology. Heart Diseases / ultrasonography. Humans. Intracranial Embolism and Thrombosis / etiology. Intracranial Embolism and Thrombosis / physiopathology. Male. Middle Aged. Retrospective Studies. Survival Rate


12. Gilliam AC, Mullen RH, Oviedo G, Bhatnagar R, Smith MK, Patton DF, Rodriguez-Soto J, Mostow E: Isolated benign primary cutaneous plasmacytosis in children: two illustrative cases. Arch Dermatol; 2009 Mar;145(3):299-302
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  • [Title] Isolated benign primary cutaneous plasmacytosis in children: two illustrative cases.
  • They appear in the skin in malignant conditions, autoimmune diseases, infection, and idiopathic and poorly understood disorders such as primary nodular amyloidosis.
  • There was no history of previous trauma, malignant conditions, autoimmune disease, or infection in either child.
  • CONCLUSION: Although incipient or occult systemic disease cannot be definitively ruled out, the course of these 2 individuals suggests that isolated primary cutaneous plasmacytosis in children is a benign chronic process with no adverse sequelae.

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  • (PMID = 19289761.001).
  • [ISSN] 1538-3652
  • [Journal-full-title] Archives of dermatology
  • [ISO-abbreviation] Arch Dermatol
  • [Language] ENG
  • [Grant] United States / NIAMS NIH HHS / AR / AR039750-14; United States / NIAMS NIH HHS / AR / P30-AR 39750; United States / NIAMS NIH HHS / AR / P30 AR039750-14; United States / NIAMS NIH HHS / AR / P30 AR039750; United States / NIAMS NIH HHS / AR / AR039750-15; United States / NIAMS NIH HHS / AR / P30 AR039750-13; United States / NIAMS NIH HHS / AR / R01 AR049284; United States / NIAMS NIH HHS / AR / R01 AR 049284; United States / NIAMS NIH HHS / AR / P30 AR039750-15; United States / NIAMS NIH HHS / AR / AR039750-13
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS223897; NLM/ PMC3130592
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13. Sanchez-Ramos J, Song S, Sava V, Catlow B, Lin X, Mori T, Cao C, Arendash GW: Granulocyte colony stimulating factor decreases brain amyloid burden and reverses cognitive impairment in Alzheimer's mice. Neuroscience; 2009 Sep 29;163(1):55-72
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  • [Title] Granulocyte colony stimulating factor decreases brain amyloid burden and reverses cognitive impairment in Alzheimer's mice.
  • The overall objective of the study was to determine if G-CSF administration in a mouse model of Alzheimer's disease (AD) (Tg APP/PS1) would impact hippocampal-dependent learning by modifying the underlying disease pathology.
  • A course of s.c. administration of G-CSF for a period of less than three weeks significantly improved cognitive performance, decreased beta-amyloid deposition in hippocampus and entorhinal cortex and augmented total microglial activity.
  • Additionally, G-CSF reduced systemic inflammation indicated by suppression of the production or activity of major pro-inflammatory cytokines in plasma.
  • The primary objective of the present study was to determine whether a short course of G-CSF administration would have an impact on the pathological hallmark of AD, the age-dependent accumulation of A beta deposits, in a transgenic mouse model of AD (APP+ PS1; Tg).
  • To explain the G-CSF triggered amyloid reduction and associated reversal of cognitive impairment, several mechanisms of action were explored. (1) G-CSF was hypothesized to increase activation of resident microglia and to increase mobilization of marrow-derived microglia.
  • [MeSH-major] Alzheimer Disease / drug therapy. Cognition Disorders / drug therapy. Granulocyte-Macrophage Colony-Stimulating Factor / therapeutic use. Hippocampus / drug effects. Neurogenesis / drug effects. Plaque, Amyloid / drug effects
  • [MeSH-minor] Animals. Calbindin 2. Cell Movement / drug effects. Cell Movement / immunology. Cytokines / drug effects. Cytokines / metabolism. Dentate Gyrus / drug effects. Dentate Gyrus / metabolism. Disease Models, Animal. Encephalitis / drug therapy. Encephalitis / metabolism. Encephalitis / physiopathology. Entorhinal Cortex / drug effects. Entorhinal Cortex / metabolism. Entorhinal Cortex / physiopathology. Green Fluorescent Proteins / metabolism. Humans. Mice. Mice, Transgenic. Microglia / drug effects. Microglia / physiology. Neuroprotective Agents / pharmacology. Neuroprotective Agents / therapeutic use. S100 Calcium Binding Protein G / drug effects. S100 Calcium Binding Protein G / metabolism. Synaptophysin / drug effects. Synaptophysin / metabolism

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  • (PMID = 19500657.001).
  • [ISSN] 1873-7544
  • [Journal-full-title] Neuroscience
  • [ISO-abbreviation] Neuroscience
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CALB2 protein, human; 0 / Calb2 protein, mouse; 0 / Calbindin 2; 0 / Cytokines; 0 / Neuroprotective Agents; 0 / S100 Calcium Binding Protein G; 0 / Synaptophysin; 147336-22-9 / Green Fluorescent Proteins; 83869-56-1 / Granulocyte-Macrophage Colony-Stimulating Factor
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14. Zhao XY, Jia JD, Wang BE, Ou XJ, Qian LX, Zhang FK, Wang Y, Cui Y: [Clinical features of 30 cases of amyloidosis]. Zhonghua Gan Zang Bing Za Zhi; 2005 Jan;13(1):42-4
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  • [Title] [Clinical features of 30 cases of amyloidosis].
  • OBJECTIVE: Clinical features of 30 cases of amyloidosis, a rare disease in China, were analyzed in order to improve the recognition of the disease here.
  • METHODS: 30 cases of biopsy-proven amyloidosis, admitted to Beijing Friendship Hospital from July 1980 to December 2003 were retrospectively reviewed.
  • RESULTS: 12 of the 30 cases were systemic amyloidosis.
  • Among them 9 were primary amyloidosis, 1 secondary amyloidosis and 2 familial amyloid polyneuropathy.
  • The other 18 cases were localized amyloidosis.
  • In the 12 primary amyloidosis patients, kidney (75.00%), liver (58.33%), peripheral nervous system (58.33%) and heart (50.00%) were most commonly involved.
  • Localized amyloidosis involved only one organ, such as skin, alimentary tract and nasopharynx without evidences of a systemic disease.
  • Excision of the localized amyloid deposits was performed in 13 cases.
  • CONCLUSION: Systemic amyloidosis usually involves multiple organs and systems, leading to highly variable clinical manifestations.
  • An increase in the vigilance of the awareness of this disease among clinicians will improve the possibilities for its diagnosis.
  • [MeSH-major] Amyloidosis / diagnosis

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  • (PMID = 15670491.001).
  • [ISSN] 1007-3418
  • [Journal-full-title] Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
  • [ISO-abbreviation] Zhonghua Gan Zang Bing Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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15. Carvalho LO, Calabrese Kda S, da Costa SC, Mendes VG, da Silva AP, Barros AC, Melo Sde A, Abreu-Silva AL: Leishmania (Leishmania) amazonensis: experimental cutaneous leishmaniasis associated with systemic amyloidosis in mice. Exp Parasitol; 2008 Sep;120(1):123-5
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  • [Title] Leishmania (Leishmania) amazonensis: experimental cutaneous leishmaniasis associated with systemic amyloidosis in mice.
  • Histopathologically, the primary lesion showed an extensive liquefactive necrosis and inflammatory infiltrate, mainly consisting of macrophages filled with amastigotes, and rare lymphocytes.
  • The inflammatory reaction in liver, spleen and kidney showed amyloid deposits.
  • Additionally, C57BL/6 had accentuated amyloidosis in both ovarian cortical and medullar region and inflammatory infiltrates in the pancreas and adrenal gland.
  • [MeSH-major] Amyloidosis / complications. Leishmania mexicana. Leishmaniasis, Cutaneous / complications

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  • (PMID = 18601926.001).
  • [ISSN] 1090-2449
  • [Journal-full-title] Experimental parasitology
  • [ISO-abbreviation] Exp. Parasitol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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16. Baumgratz JF, Vila JH, Guilhen CJ, Fonseca Ld, Leite WF, D'Andretta C, Tângari Junior A, Silva JP: Heart transplantation in primary amyloidosis. Rev Bras Cir Cardiovasc; 2009 Jul-Sep;24(3):409-12
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  • [Title] Heart transplantation in primary amyloidosis.
  • Cardiac amyloidosis is a disease with a gloom life expectancy after the beginning of the symptomatic phase, usually with sudden death as the final event.
  • The aim is to report the evolution with a survival of seven years after heart transplantation and in very fair condition of a patient with amyloidosis.
  • One year after the heart transplantation, there was indication of renal transplantation also from the aggression from the disease.
  • This patient compares' favorable with three other patients also from our service, who died early after de diagnosis.
  • Even considering the multi systemic nature of amyloidosis, we can accept that in peculiar patients justified the heart transplantation, taking in the consideration the very bad prognosis of the disease.
  • [MeSH-major] Amyloidosis / surgery. Cardiomyopathies / surgery. Heart Failure / surgery. Heart Transplantation
  • [MeSH-minor] Disease Progression. Female. Humans. Kidney Diseases / surgery. Kidney Transplantation. Middle Aged


17. Bataille Y, Bovy C, Lancellotti P, Melchior V, Delbecque K, Beguin Y, Krzesinski JM: Primary amyloidosis (AL) as a cause of nephrotic syndrome. Acta Clin Belg; 2005 Mar-Apr;60(2):94-7
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  • [Title] Primary amyloidosis (AL) as a cause of nephrotic syndrome.
  • AL amyloidosis is a rare systemic disease resulting from tissue accumulation of amyloid fibrils derived from monoclonal immunoglobulin light chains.
  • By illustrating the case of a patient whose AL amyloidosis was detected after presenting a nephrotic syndrome, the characteristics of the disease are reviewed as well as diagnostic criteria and current available therapeutics.
  • [MeSH-major] Amyloidosis / complications. Amyloidosis / diagnosis. Nephrotic Syndrome / etiology
  • [MeSH-minor] Biopsy, Needle. Disease Progression. Fatal Outcome. Humans. Immunohistochemistry. Kidney Function Tests. Male. Middle Aged. Multiple Organ Failure. Severity of Illness Index. Ultrasonography, Doppler

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  • (PMID = 16082995.001).
  • [ISSN] 1784-3286
  • [Journal-full-title] Acta clinica Belgica
  • [ISO-abbreviation] Acta Clin Belg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Belgium
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18. Moreau P, Jaccard A, Benboubker L, Royer B, Leleu X, Bridoux F, Salles G, Leblond V, Roussel M, Alakl M, Hermine O, Planche L, Harousseau JL, Fermand JP: Lenalidomide in combination with melphalan and dexamethasone in patients with newly diagnosed AL amyloidosis: a multicenter phase 1/2 dose-escalation study. Blood; 2010 Dec 2;116(23):4777-82
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  • [Title] Lenalidomide in combination with melphalan and dexamethasone in patients with newly diagnosed AL amyloidosis: a multicenter phase 1/2 dose-escalation study.
  • New treatment options are required for primary systemic amyloid light chain (AL) amyloidosis.
  • This phase 1/2 dose-escalation study aimed to determine the maximum tolerated dose (MTD) of lenalidomide in combination with melphalan and dexamethasone (M-dex), and assess the efficacy and tolerability of this therapy for patients with de novo AL amyloidosis.
  • No dose limiting toxicity (DLT) was observed in cohorts 1, 2, and 3.
  • A complete hematologic response was achieved in 42% at the dose of 15 mg of lenalidomide per day.
  • Lenalidomide 15 mg/d + M-dex is a new effective combination therapy in patients with newly diagnosed AL amyloidosis.
  • [MeSH-major] Amyloidosis / drug therapy. Anti-Inflammatory Agents / administration & dosage. Dexamethasone / administration & dosage. Thalidomide / analogs & derivatives
  • [MeSH-minor] Adult. Aged. Disease-Free Survival. Female. Humans. Kaplan-Meier Estimate. Male. Maximum Tolerated Dose. Melphalan / administration & dosage. Melphalan / adverse effects. Middle Aged


19. Fietta AM, Morosini M, Passadore I, Cascina A, Draghi P, Dore R, Rossi S, Pozzi E, Meloni F: Systemic inflammatory response and downmodulation of peripheral CD25+Foxp3+ T-regulatory cells in patients undergoing radiofrequency thermal ablation for lung cancer. Hum Immunol; 2009 Jul;70(7):477-86
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  • [Title] Systemic inflammatory response and downmodulation of peripheral CD25+Foxp3+ T-regulatory cells in patients undergoing radiofrequency thermal ablation for lung cancer.
  • We assessed whether RFTA could affect multiple systemic inflammatory and immunological parameters, including CD25+Foxp+ cells, in patients with primary or metastatic lung tumors.
  • Three days after RFTA, a moderate and temporary systemic inflammatory response developed, as demonstrated by the increase in peripheral neutrophils and monocytes and in plasma levels of proinflammatory chemokines (MIP-1alpha, MIP-1beta, eotaxin, and interleukin[IL]-8) and acute phase reactants (complement C3 and C4, serum amyloid, alpha1 antichymotrypsin, and C-reactive protein).
  • The use of RFTA in lung cancer patients has an immunomodulatory activity: it induces a self-limiting systemic inflammation early and later a reduction of circulating CD25+Foxp3+ Tregs.

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  • (PMID = 19332094.001).
  • [ISSN] 1879-1166
  • [Journal-full-title] Human immunology
  • [ISO-abbreviation] Hum. Immunol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Acute-Phase Proteins; 0 / Blood Proteins; 0 / Cytokines; 0 / FOXP3 protein, human; 0 / Forkhead Transcription Factors; 0 / Inflammation Mediators; 0 / Interleukin-2 Receptor alpha Subunit
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20. Demina AB, Radenska-Lopovok SG, Folomeeva OM, Erdes Sh: [The causes of death of patients with rheumatic diseases in Moscow]. Klin Med (Mosk); 2005;83(1):36-43
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  • [Title] [The causes of death of patients with rheumatic diseases in Moscow].
  • The study was held in order to analyze the main causes of death in cases of rheumatic diseases (RD) in Moscow.
  • There were 99 cases (60%) of rheumatic heart disease (RHD), 4 cases (2.4%) of rheumatic fever (RF) relapse, 28 cases (17%) of rheumatoid arthritis (RA), 8 cases (4.8%) of systemic lupus erythematosus (SLE), 3 cases (1.8%) of scleroderma systematica (SS), 2 cases (1.2%) of ankylosing spondylitis (AS), 2 cases (1.2%) of systemic vasculitis (SPV), 11 cases (7.3%) of osteoarthrosis, 3 cases (1.8%) of gout, 1 case (0.6%) of polymyositis.
  • Secondary amyloidosis resulting in chronic renal failure and uremia occurred in 5 out of 28 cases of RA, HD--in 3, ACC--in 7, TE--in 1, infectious complications--in 5, other complications--in 7 cases.
  • The main cause of death in RD was cardiovascular disorders.
  • [MeSH-major] Rheumatic Diseases / mortality
  • [MeSH-minor] Adult. Age Factors. Aged. Aged, 80 and over. Arthritis, Rheumatoid / mortality. Arthritis, Rheumatoid / pathology. Autopsy. Cause of Death. Female. Gout / mortality. Gout / pathology. Humans. Lupus Erythematosus, Systemic / mortality. Lupus Erythematosus, Systemic / pathology. Male. Middle Aged. Moscow. Osteoarthritis / mortality. Osteoarthritis / pathology. Polymyositis / mortality. Polymyositis / pathology. Rheumatic Fever / mortality. Rheumatic Fever / pathology. Rheumatic Heart Disease / mortality. Rheumatic Heart Disease / pathology. Scleroderma, Systemic / mortality. Scleroderma, Systemic / pathology. Sex Factors. Spondylitis, Ankylosing / mortality. Spondylitis, Ankylosing / pathology. Vasculitis / mortality. Vasculitis / pathology

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  • (PMID = 15759489.001).
  • [ISSN] 0023-2149
  • [Journal-full-title] Klinicheskaia meditsina
  • [ISO-abbreviation] Klin Med (Mosk)
  • [Language] rus
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Russia
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21. Klein R, Klein BE, Knudtson MD, Wong TY, Shankar A, Tsai MY: Systemic markers of inflammation, endothelial dysfunction, and age-related maculopathy. Am J Ophthalmol; 2005 Jul;140(1):35-44
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  • [Title] Systemic markers of inflammation, endothelial dysfunction, and age-related maculopathy.
  • PURPOSE: To examine the association of systemic markers of inflammatory disease and endothelial dysfunction with age-related maculopathy (ARM). DESIGN:.
  • Included in the prospective analyses as a random sample of 321 persons were those who participated in a 5-year and/or 10-year follow-up. main outcome measures: Prevalent and incident ARM.
  • RESULTS: Serum C-reactive protein, amyloid A, interleukin-6, tumor necrosis factor-alpha, intracellular adhesion molecule, E-selectin, folate, and Chlamydia pneumoniae IgG antibody were not associated with either prevalent or incident ARM.

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  • (PMID = 15939388.001).
  • [ISSN] 0002-9394
  • [Journal-full-title] American journal of ophthalmology
  • [ISO-abbreviation] Am. J. Ophthalmol.
  • [Language] eng
  • [Grant] United States / NEI NIH HHS / EY / EY06594
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers
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22. Schreml S, Schroeder J, Eder F, Szeimies RM, Landthaler M, Babilas P: [Hereditary and non-hereditary cutaneous amyloidoses]. Pathologe; 2009 May;30(3):197-204
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  • [Title] [Hereditary and non-hereditary cutaneous amyloidoses].
  • [Transliterated title] Hereditäre und nichthereditäre kutane Amyloidosen.
  • Amyloid and amyloidosis describes a heterogeneous group of diseases which are characterized by the pathological extracellular deposition of autologous proteins.
  • Basically, amyloidoses can be divided into systemic or organ-limited (e.g. cutaneous) forms and can be acquired or hereditary in nature.
  • The subclassification discriminates between primary amyloidosis (in the absence of an obvious predisposing disease) and secondary amyloidosis (if caused by a certain underlying disease).
  • The subclassification of amyloidoses is based on the main protein constituent and therefore on the chemical composition of the amyloid fibrils.
  • However, the exact etiopathogenesis of amyloid formation remains unclear.
  • In addition to the clinical presentation, histology, electron microscopy and biochemical-immunological differentiation are also decisive for a proper diagnosis.
  • In cutaneous amyloidosis the deposition of amyloid either occurs along reticulin fibers and the basal membrane (perireticulary amyloidoses) or along collagen fibers (pericollagenous amyloidosis).
  • The purpose of this article is to provide an up-to-date overview on the different kinds of cutaneous amyloidoses.
  • [MeSH-major] Amyloidosis / pathology. Amyloidosis, Familial / pathology. Skin Diseases, Genetic / pathology
  • [MeSH-minor] Amyloid / analysis. Amyloid / ultrastructure. Basement Membrane / pathology. Diagnosis, Differential. Humans. Microscopy, Electron. Skin / pathology

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  • (PMID = 19319536.001).
  • [ISSN] 1432-1963
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Amyloid
  • [Number-of-references] 36
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23. Kastritis E, Roussou M, Michael M, Gavriatopoulou M, Michalis E, Migkou M, Delimpasi S, Kyrtsonis MC, Gogos D, Liapis K, Charitaki E, Repousis P, Terpos E, Dimopoulos MA, Greek Myeloma Study Group: High levels of serum angiogenic growth factors in patients with AL amyloidosis: comparisons with normal individuals and multiple myeloma patients. Br J Haematol; 2010 Sep;150(5):587-91
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  • [Title] High levels of serum angiogenic growth factors in patients with AL amyloidosis: comparisons with normal individuals and multiple myeloma patients.
  • Serum levels of five angiogenic cytokines were evaluated in 82 patients with primary systemic amyloidosis (AL).
  • The increased angiogenic cytokine levels observed in AL seem to represent either a toxic effect of amyloid fibrils or light chains, or a compensatory response to organ dysfunction.
  • [MeSH-major] Amyloidosis / blood. Angiogenic Proteins / blood. Multiple Myeloma / blood

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  • (PMID = 20618328.001).
  • [ISSN] 1365-2141
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Angiogenic Proteins; 0 / Angiopoietin-1; 0 / Biomarkers; 0 / Neoplasm Proteins; 0 / VEGFA protein, human; 0 / Vascular Endothelial Growth Factor A; 103107-01-3 / Fibroblast Growth Factor 2; EC 3.1.27.- / angiogenin; EC 3.1.27.5 / Ribonuclease, Pancreatic
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24. Silverstein SR: Primary, systemic amyloidosis and the dermatologist: where classic skin lesions may provide the clue for early diagnosis. Dermatol Online J; 2005;11(1):5
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  • [Title] Primary, systemic amyloidosis and the dermatologist: where classic skin lesions may provide the clue for early diagnosis.
  • Primary amyloidosis may present with skin lesions as a primary or sole expression of underlying plasma-cell dyscrasia.
  • Classic skin lesions of primary, systemic amyloidosis are listed, and features suggestive of the diagnosis are discussed.
  • Where this condition is considered in the dermatologist's differential, the investigations described may lead to an early diagnosis.
  • [MeSH-major] Amyloidosis / diagnosis. Skin Diseases / diagnosis
  • [MeSH-minor] Aged. Biopsy. Diagnosis, Differential. Early Diagnosis. Humans. Middle Aged

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  • (PMID = 15748546.001).
  • [ISSN] 1087-2108
  • [Journal-full-title] Dermatology online journal
  • [ISO-abbreviation] Dermatol. Online J.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 21
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25. Brill AK, Woelke K, Schädlich R, Weinz C, Laier-Groeneveld G: Tracheobronchial amyloidosis--bronchoscopic diagnosis and therapy of an uncommon disease: a case report. J Physiol Pharmacol; 2007 Nov;58 Suppl 5(Pt 1):51-5
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  • [Title] Tracheobronchial amyloidosis--bronchoscopic diagnosis and therapy of an uncommon disease: a case report.
  • The diagnosis of amyloidosis was made by bronchoscopic tissue biopsies, during which severe bleeding occurred.
  • Argon-plasma-laser treatment stopped the bleeding and resulted in a successful recanalization of the left main bronchus.
  • Further diagnostic procedures did not show any signs of systemic or malignant disease.
  • This led us to the diagnosis of a rare form of isolated tracheobronchial amyloidosis.
  • [MeSH-major] Amyloidosis. Bronchial Diseases. Bronchoscopy. Laser Coagulation. Tracheal Diseases


26. Jabbour SA, Davidovici BB, Wolf R: Rare syndromes. Clin Dermatol; 2006 Jul-Aug;24(4):299-316
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  • Dermatologists may also encounter patients presenting with skin lesions that reflect an underlying endocrine disorder not commonly seen in daily practice.
  • Multiple endocrine neoplasia type 2A is characterized by medullary thyroid cancer, pheochromocytoma, and primary parathyroid hyperplasia.
  • In some patients with multiple endocrine neoplasia type 2A, cutaneous lichen amyloidosis may also be present.
  • Carney complex may be viewed as a form of multiple endocrine neoplasia because affected patients often have tumors of two or more endocrine glands, including primary pigmented nodular adrenocortical disease (some with Cushing's syndrome), pituitary adenoma, testicular neoplasms, thyroid adenoma or carcinoma, and ovarian cysts.
  • Mast cell diseases include all disorders of mast cell proliferation.
  • These diseases can be limited to the skin, referred to as "cutaneous mastocytosis," or involve extracutaneous tissues, called "systemic mastocytosis."
  • Systemic involvement may be gastronintestinal, hematologic, neurologic, and skeletal.
  • [MeSH-major] Endocrine System Diseases / genetics. Endocrine System Diseases / pathology. Mastocytosis, Cutaneous / pathology. Multiple Endocrine Neoplasia / genetics. Multiple Endocrine Neoplasia / pathology

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  • (PMID = 16828412.001).
  • [ISSN] 0738-081X
  • [Journal-full-title] Clinics in dermatology
  • [ISO-abbreviation] Clin. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 155
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27. Esteve V, Almirall J, Ponz E, García N, Ribera L, Larrosa M, Andreu X, García M: [Renal involvement in amyloidosis. Clinical outcomes, evolution and survival]. Nefrologia; 2006;26(2):212-7
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  • [Title] [Renal involvement in amyloidosis. Clinical outcomes, evolution and survival].
  • [Transliterated title] Afectación renal en la amiloidosis. Características clínicas, evolución y supervivencia.
  • BACKGROUND: Systemic amyloidosis is a disease resulting from extracellular deposition of fibrillar protein in various organs.
  • Main systemic amyloidosis are: primary (AL) and Secondary (AA).
  • In the last decade there has been a change in the aetiology of AA amyloidosis.
  • OBJECTIVES: To analyse the incidence of AL and AA amyloidosis in our current population as well as the aetiology of AA amyloidosis.
  • PATIENTS AND METHODS: We performed a descriptive analysis of all cases of amyloidosis diagnosed from 1992 to 2004 in our hospital.
  • Diagnosis was assessed on histological criteria: positivity Congo Red stain.
  • Types: 55 AA (72%), 21 AL (28%) systemic amyloidosis.
  • AA aetiology was: 66% rheumatic disorders, 28% infectious disease, 6% others.
  • Renal involvement was present in 75% at diagnosis (69% Creatinine clearance < 60 ml/min, 37% urinary protein > 3 g/24 hours).
  • 21 cases (28%) progressed to renal disease stage V in the 8.1 +/- 9.8 months follow up period, and 14 cases started dialysis treatment (10 HD, 4 CAPD).
  • Mean global survival at diagnosis was 55% and 40% at 12 and 24 months (AL 58% and 19%; AA 55% and 44%).
  • CONCLUSIONS: Although amyloidosis has a low incidence in our population, the kidney is usually involved.
  • Rheumatological disorders are the principal aetiology of AA amyloidosis.
  • [MeSH-major] Amyloidosis / complications. Kidney Diseases / etiology

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  • (PMID = 16808259.001).
  • [ISSN] 0211-6995
  • [Journal-full-title] Nefrología : publicación oficial de la Sociedad Española Nefrologia
  • [ISO-abbreviation] Nefrologia
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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28. Kikuchi N, Sakai E, Nishibu A, Ohtsuka M, Yamamoto T: Primary localized cutaneous amyloidosis in patients with scleroderma. Acta Derm Venereol; 2010 May;90(3):326-7
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  • [Title] Primary localized cutaneous amyloidosis in patients with scleroderma.
  • [MeSH-major] Amyloidosis / etiology. Scleroderma, Systemic / complications. Skin / pathology. Skin Diseases / etiology


29. Moles MP, Brousseau M, Rachieru AP, Godon A, Schmidt A, Furber A, Rousselet MC, Hunault-Berger M: [Tumor like presentation of primitive amyloidosis: amyloidoma]. Rev Med Interne; 2007 May;28(5):339-42
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  • [Title] [Tumor like presentation of primitive amyloidosis: amyloidoma].
  • [Transliterated title] Forme pseudotumorale d'une amylose primitive: l'amyloïdome.
  • INTRODUCTION: AL-amyloidosis is a rare disease due to monoclonal immunoglobulin deposits, secondary to lymphoproliferative disorder or primitive.
  • The deposits of amyloidosis have usually a systemic repartition.
  • We report a tumor like presentation of amyloidosis, so-called amyloidoma.
  • The biopsy of the abdominal mass showed AL-amyloidosis with kappa light chains.
  • Since no secondary etiology could be found, the final diagnosis of primary AL-amyloidosis in a tumour like presentation, or amyloidoma, was performed.
  • The prognosis and the treatment of this unusual disease are discussed.
  • CONCLUSION: Amyloidoma is a rare presentation of amyloidosis which should be evocated in front of a soft tissue mass with no clear etiology.

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  • (PMID = 17360073.001).
  • [ISSN] 0248-8663
  • [Journal-full-title] La Revue de medecine interne
  • [ISO-abbreviation] Rev Med Interne
  • [Language] FRE
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Immunoglobulin kappa-Chains
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30. Du J, Murphy RM: Characterization of the interaction of β-amyloid with transthyretin monomers and tetramers. Biochemistry; 2010 Sep 28;49(38):8276-89
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  • [Title] Characterization of the interaction of β-amyloid with transthyretin monomers and tetramers.
  • β-Amyloid (Aβ) is the main protein component of the amyloid plaques associated with Alzheimer's disease.
  • Wild-type (wt) TTR amyloid deposits are linked to senile systemic amyloidosis, a common disease of aging, while several TTR mutants are linked to familial amyloid polyneuropathy.

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  • (PMID = 20795734.001).
  • [ISSN] 1520-4995
  • [Journal-full-title] Biochemistry
  • [ISO-abbreviation] Biochemistry
  • [Language] ENG
  • [Grant] United States / NIA NIH HHS / AG / R01 AG033493; United States / NIA NIH HHS / AG / R01AG033493
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid beta-Peptides; 0 / Macromolecular Substances; 0 / Prealbumin; 0 / Recombinant Proteins
  • [Other-IDs] NLM/ NIHMS233605; NLM/ PMC2943652
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31. Haridas A, Basu S, King A, Pollock J: Primary isolated amyloidoma of the lumbar spine causing neurological compromise: case report and literature review. Neurosurgery; 2005 Jul;57(1):E196; discussion E196
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  • [Title] Primary isolated amyloidoma of the lumbar spine causing neurological compromise: case report and literature review.
  • It is the fourth published report of primary lumbar amyloidoma causing neurological compromise.
  • There was no evidence of systemic amyloidosis or development of multiple myeloma.
  • Diagnosis is made at histological examination of a Congo red-stained section under polarized light.
  • Complete resection of the localized epidural amyloid mass is associated with a good prognosis.
  • [MeSH-major] Amyloidosis / complications. Nerve Compression Syndromes / etiology. Nerve Compression Syndromes / surgery. Spinal Diseases / complications

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  • (PMID = 15987561.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 59
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32. Dvir E, Elman A, Simmons D, Shapiro I, Duvdevani R, Dahan A, Hoffman A, Friedman JE: DP-155, a lecithin derivative of indomethacin, is a novel nonsteroidal antiinflammatory drug for analgesia and Alzheimer's disease therapy. CNS Drug Rev; 2007;13(2):260-77
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  • [Title] DP-155, a lecithin derivative of indomethacin, is a novel nonsteroidal antiinflammatory drug for analgesia and Alzheimer's disease therapy.
  • Indomethacin is the principal metabolite of DP-155 in rat serum and, after DP-155 oral administration, the half-life of the metabolite was 22 and 93 h in serum and brain, respectively, compared to 10 and 24 h following indomethacin administration.
  • DP-155 did not produce gastric toxicity at the highest acute dose tested (0.28 mmol/kg), while indomethacin caused gastric ulcers at a dose 33-fold lower.
  • Moreover, DP-155 and indomethacin were equally efficacious in reducing levels of amyloid ss (Ass)42 in transgenic Alzheimer's disease mouse (Tg2576) brains as well as reducing Ass42 intracellular uptake, neurodegeneration, and inflammation in an in vitro AD model.
  • The relatively high brain levels of indomethacin after DP-155 administration explain the equal efficacy of DP-155 despite its low systemic blood concentrations.
  • [MeSH-major] Alzheimer Disease / drug therapy. Analgesics / pharmacology. Anti-Inflammatory Agents, Non-Steroidal / pharmacology. Indomethacin / analogs & derivatives. Phosphatidylcholines / pharmacology

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  • (PMID = 17627676.001).
  • [ISSN] 1080-563X
  • [Journal-full-title] CNS drug reviews
  • [ISO-abbreviation] CNS Drug Rev
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Analgesics; 0 / Anti-Inflammatory Agents, Non-Steroidal; 0 / DP 155; 0 / Drug Combinations; 0 / Phosphatidylcholines; EC 1.14.99.1 / Cyclooxygenase 2; EC 3.1.1.- / Phospholipases A; EC 3.1.1.4 / Group II Phospholipases A2; EC 3.1.1.4 / Phospholipases A2; XXE1CET956 / Indomethacin
  • [Number-of-references] 63
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33. Scalvini T, Martini PR, Gambera A, Tardanico R, Biasi L, Scolari F, Gregorini G, Agabiti Rosei E: Spermatogenic and steroidogenic impairment of the testicle characterizes the hereditary leucine-75-proline apolipoprotein a-I amyloidosis. J Clin Endocrinol Metab; 2008 May;93(5):1850-3
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  • [Title] Spermatogenic and steroidogenic impairment of the testicle characterizes the hereditary leucine-75-proline apolipoprotein a-I amyloidosis.
  • CONTEXT: The leucine-75-proline variant of apolipoprotein A-I leads to a new hereditary systemic amyloidosis involving mostly the liver and kidney.
  • OBJECTIVE: The objective of the study was to examine the effects of this amyloidosis on testicular structure and function.
  • DESIGN: This was an observational study in which patients with testicular amyloidosis were characterized.
  • PATIENTS OR OTHER PARTICIPANTS: Over a 13-yr period, 25 patients were found to be affected by leucine-75-proline apolipoprotein A-I testicular amyloidosis.
  • MAIN OUTCOME MEASURE: Hormone and lipidic profiles, semen analysis, echographic volume of testicles, testicular histology, and genetic analysis were carried out.
  • Biopsies showed the germinal epithelium replaced by amyloid.
  • CONCLUSIONS: This amyloidosis may determine infertility, macroorchidism, and hypogonadism.
  • [MeSH-major] Amyloidosis, Familial / pathology. Apolipoprotein A-I / genetics. Spermatogenesis. Steroids / biosynthesis. Testis / pathology

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  • (PMID = 18285420.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Apolipoprotein A-I; 0 / Lipids; 0 / Steroids; 9DLQ4CIU6V / Proline; GMW67QNF9C / Leucine
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34. Young SM, Bologa CM, Fara D, Bryant BK, Strouse JJ, Arterburn JB, Ye RD, Oprea TI, Prossnitz ER, Sklar LA, Edwards BS: Duplex high-throughput flow cytometry screen identifies two novel formylpeptide receptor family probes. Cytometry A; 2009 Mar;75(3):253-63
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  • Of recent, clinical interest have been two related human G-protein coupled receptors: formylpeptide receptor (FPR), linked to antibacterial inflammation and malignant glioma cell metastasis; and FPR like-1 (FPRL1), linked to chronic inflammation in systemic amyloidosis, Alzheimer's disease, and prion diseases.
  • In primary single concentration HTS of 24,304 NIH Small Molecule Repository compounds, 253 resulted in inhibition >30% (181 for FPR, 72 for FPRL1) of which 40 had selective binding inhibition constants (K(i)) < or = 4 microM (34 for FPR and 6 for FPRL1).

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  • [Copyright] 2008 International Society for Advancement of Cytometry.
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  • (PMID = 18785269.001).
  • [ISSN] 1552-4930
  • [Journal-full-title] Cytometry. Part A : the journal of the International Society for Analytical Cytology
  • [ISO-abbreviation] Cytometry A
  • [Language] ENG
  • [Grant] United States / NIMH NIH HHS / MH / U54 MH074425; United States / NIMH NIH HHS / MH / R03 MH076381-01; United States / NIMH NIH HHS / MH / MH076381-01; United States / NIMH NIH HHS / MH / R03 MH076381; United States / NIMH NIH HHS / MH / U54 MH074425-01
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chemotactic Factors; 0 / FPR2 protein, human; 0 / Fluorescent Dyes; 0 / Ligands; 0 / Molecular Probes; 0 / Receptors, Formyl Peptide; 0 / Receptors, Lipoxin
  • [Other-IDs] NLM/ NIHMS76367; NLM/ PMC2645486
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35. Sjöwall C, Wetterö J: Pathogenic implications for autoantibodies against C-reactive protein and other acute phase proteins. Clin Chim Acta; 2007 Mar;378(1-2):13-23
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  • Systemic lupus erythematosus (SLE) is a systemic rheumatic disease characterized clinically by multiorgan involvement and serologically by the occurrence of antinuclear antibodies.
  • Another feature of SLE is that serum levels of C-reactive protein (CRP) often remain low despite high disease activity and despite high levels of other acute phase proteins and interleukin-6, i.e. the main CRP inducing cytokine.
  • This paper thus highlights the biological and clinical aspects of native and monomeric CRP and anti-CRP, as well as autoantibodies against mannose-binding lectin, serum amyloid A and serum amyloid P component.
  • [MeSH-minor] Apoptosis. Complement C1q / immunology. Complement System Proteins / physiology. Humans. Lupus Erythematosus, Systemic / immunology. Mannose-Binding Lectin / immunology. Serum Amyloid P-Component / immunology

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  • (PMID = 17239838.001).
  • [ISSN] 0009-8981
  • [Journal-full-title] Clinica chimica acta; international journal of clinical chemistry
  • [ISO-abbreviation] Clin. Chim. Acta
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Acute-Phase Proteins; 0 / Autoantibodies; 0 / Mannose-Binding Lectin; 0 / Serum Amyloid P-Component; 80295-33-6 / Complement C1q; 9007-36-7 / Complement System Proteins; 9007-41-4 / C-Reactive Protein
  • [Number-of-references] 163
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36. Teoh SC, Liew GC, Yap WM: Incidental hypoglobus: primary amyloidosis of the superior rectus. Singapore Med J; 2006 Jan;47(1):65-7
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  • [Title] Incidental hypoglobus: primary amyloidosis of the superior rectus.
  • Diagnosis of primary amyloidosis of superior rectus was made on incisional biopsy and negative systemic work-up.
  • This is an unusual manifestation and site for amyloidosis and should be a differential of any extraocular muscle mass.
  • [MeSH-major] Amyloidosis / diagnosis. Eye Diseases / diagnosis. Oculomotor Muscles / pathology
  • [MeSH-minor] Aged. Biopsy. Diagnosis, Differential. Humans. Male

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  • (PMID = 16397724.001).
  • [ISSN] 0037-5675
  • [Journal-full-title] Singapore medical journal
  • [ISO-abbreviation] Singapore Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Singapore
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37. Shimojima Y, Matsuda M, Ishii W, Koyama J, Yamamoto K, Shimodaira S, Sakashita K, Koike K, Ikeda S: High-dose melphalan followed by autologous stem cell support in primary systemic AL amyloidosis with multiple organ involvement. Intern Med; 2005 May;44(5):484-9
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  • [Title] High-dose melphalan followed by autologous stem cell support in primary systemic AL amyloidosis with multiple organ involvement.
  • A patient with primary systemic AL amyloidosis achieved partial hematological response after 2 courses of VAD (vincristine, doxorubicin and dexamethasone) and subsequent high-dose melphalan followed by autologous peripheral blood stem cell transplantation despite involvement of multiple organs, including the heart.
  • When amyloidosis-related dysfunction is seen in multiple organs, this intensive chemotherapy might be a possible therapeutic option, although several modifications in the regimen and careful management are necessary.
  • [MeSH-major] Amyloidosis / therapy. Antineoplastic Agents, Alkylating / therapeutic use. Melphalan / therapeutic use. Peripheral Blood Stem Cell Transplantation
  • [MeSH-minor] Amyloid / metabolism. Biopsy, Needle. Bone Marrow / pathology. Echocardiography. Follow-Up Studies. Humans. Immunoglobulin Light Chains / blood. Male. Middle Aged. Myocardium / metabolism. Myocardium / pathology. Natriuretic Peptides / blood. Submandibular Gland / metabolism. Submandibular Gland / pathology. Time Factors. Tongue / metabolism. Tongue / pathology. Transplantation, Autologous

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  • (PMID = 15942100.001).
  • [ISSN] 0918-2918
  • [Journal-full-title] Internal medicine (Tokyo, Japan)
  • [ISO-abbreviation] Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Amyloid; 0 / Antineoplastic Agents, Alkylating; 0 / Immunoglobulin Light Chains; 0 / Natriuretic Peptides; Q41OR9510P / Melphalan
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38. Ooms V, Decupere M, Lerut E, Vanrenterghem Y, Kuypers DR: Secondary renal amyloidosis due to long-standing tubulointerstitial nephritis in a patient with Sjögren syndrome. Am J Kidney Dis; 2005 Nov;46(5):e75-80
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  • [Title] Secondary renal amyloidosis due to long-standing tubulointerstitial nephritis in a patient with Sjögren syndrome.
  • A 53-year-old patient with long-standing primary Sjögren syndrome presented with acute renal failure and nephrotic syndrome caused by secondary (AA) renal amyloidosis.
  • Ten years before, he had been admitted because of exacerbation of the systemic disease.
  • To our knowledge, this is the first report of a patient with primary Sjögren syndrome and secondary (AA) amyloidosis with amyloid deposition in the kidneys causing nephrotic syndrome.
  • [MeSH-major] Amyloidosis / etiology. Nephritis, Interstitial / complications. Sjogren's Syndrome / complications
  • [MeSH-minor] Acute Kidney Injury / etiology. Aortic Valve / pathology. Aortic Valve Insufficiency / etiology. Aortic Valve Insufficiency / pathology. Dyspnea / etiology. Fibrosis. Humans. Kidney Diseases / etiology. Male. Middle Aged. Nephrotic Syndrome / etiology. Pseudolymphoma / etiology. Serum Amyloid A Protein / analysis


39. Palma CL, Grünholz D, Osorio G: [Clinical features of patients with the pathological diagnosis of amyloidosis]. Rev Med Chil; 2005 Jun;133(6):655-61
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  • [Title] [Clinical features of patients with the pathological diagnosis of amyloidosis].
  • [Transliterated title] Amiloidosis, Comunicación de 11 casos y revisión de la literatura.
  • BACKGROUND: Amyloidosis is characterized by the extracellular deposit of an insoluble fibrillar protein that leads to tissue atrophy and necrosis.
  • AIM: To report the clinical features of cases of amyloidosis diagnosed in a public hospital in Santiago, Chile, from 2000 to 2004.
  • In all cases reported as "amyloidosis", the clinical features of such patients were obtained from their medical records.
  • RESULTS: The medical records of 11 patients with amyloidosis were obtained (aged 35 to 71 year old, seven females).
  • Seven had a systemic and four a localized disease.
  • Six patients had primary amyloidosis and in one, it was secondary to a disseminated tuberculosis.
  • Five patients with the generalized disease consulted for anarsarca, three for weight loss and 2 for chronic diarrhea.
  • Patients with localized disease consulted for tonsil enlargement, dysphonia and skin lesions.
  • Five patients with generalized disease had renal involvement and five had cardiac involvement.
  • CONCLUSIONS: The most common presentation of systemic amyloidosis is anasarca and renal involvement is common.
  • [MeSH-major] Amyloidosis / pathology. Kidney Diseases / pathology. Liver Diseases / pathology. Skin Diseases / pathology


40. Ruban JM, Baggio E: [Cutaneous abnormalities of the eyelid and systemic diseases]. J Fr Ophtalmol; 2005 Oct;28(8):881-8
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  • [Title] [Cutaneous abnormalities of the eyelid and systemic diseases].
  • Systemic diseases are unknown diffuse inflammatory disorders.
  • They include systemic and metabolic diseases and connective tissue diseases.
  • Among them, lupus erythematosus, scleroderma, polymyositis and dermatomyositis, Sjögren syndrome, Wegener's granulomatosis, sarcoidosis, Vogt-Koyanagi-Harada's syndrome, and amyloidosis are the diseases encountered most frequently that can manifest cutaneous abnormalities of the eyelids.
  • The main eyelid disorders involved in these diseases are described in this paper.
  • [MeSH-major] Connective Tissue Diseases / complications. Eyelid Diseases / etiology. Inflammation / complications. Vascular Diseases / complications


41. Samanez C, Domingo A, Cibeira MT, Miquel R, Soler M, Bladé J: Development of rapidly progressive liver light chain deposition under VAD chemotherapy in multiple myeloma. Eur J Haematol; 2006 Jan;76(1):83-5
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  • Light chain deposition disease (LCDD) is a multisystemic disorder seen in the setting of plasma cell dyscrasias.
  • The histological characteristic of this disorder is the deposition of a homogeneous, granular, slightly eosinophilic and non-Congophilic material that shows immunostaining for monoclonal light chains (kappa or gamma), while in primary amyloidosis (AL) the proteinaceous substance is fibrillar and Congo red positive.
  • Patients with this disease may also have heart, liver or other organ involvement, mimicking the picture of primary systemic amyloidosis.
  • [MeSH-major] Amyloid / metabolism. Amyloidosis / pathology. Immunoglobulin kappa-Chains / metabolism. Liver / pathology. Multiple Myeloma / pathology. Renal Insufficiency / pathology

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  • (PMID = 16343276.001).
  • [ISSN] 0902-4441
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Amyloid; 0 / Anti-Inflammatory Agents; 0 / Antineoplastic Agents; 0 / Immunoglobulin kappa-Chains; 5J49Q6B70F / Vincristine; 7S5I7G3JQL / Dexamethasone; 80168379AG / Doxorubicin
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42. Clos AL, Lasagna-Reeves CA, Wagner R, Kelly B, Jackson GR, Kayed R: Therapeutic removal of amyloid deposits in cutaneous amyloidosis by localised intra-lesional injections of anti-amyloid antibodies. Exp Dermatol; 2010 Oct;19(10):904-11
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  • [Title] Therapeutic removal of amyloid deposits in cutaneous amyloidosis by localised intra-lesional injections of anti-amyloid antibodies.
  • In the skin, amyloidosis can be found with or without systemic disease.
  • Primary cutaneous amyloidosis defines those amyloidoses restricted to the skin without involvement of other systems.
  • Here, we used conformation-specific antibodies to characterise both fibrillar and oligomeric amyloid aggregates in the skin from patients with cutaneous amyloidosis.
  • Localised cutaneous amyloidosis with different morphology was reproduced in mice by intra-dermal (i.d.) and subdermal administration of amyloid-enhancing factor.
  • Moreover, we demonstrated that conformational antibodies were effective in clearing amyloid deposits caused by localised intra-lesional injections without the necessity of an immune response.
  • Given the accessibility and amyloid localization in this disease, direct i.d. injections of conformational antibodies could be a convenient and direct method for treatment.
  • [MeSH-major] Amyloid / immunology. Amyloidosis. Antibodies / pharmacology. Immunotherapy / methods. Skin Diseases

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  • [Copyright] © 2010 John Wiley & Sons A/S.
  • (PMID = 20626464.001).
  • [ISSN] 1600-0625
  • [Journal-full-title] Experimental dermatology
  • [ISO-abbreviation] Exp. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Amyloid; 0 / Antibodies
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43. Nakayama M, Kashiwagi M, Katafuchi R, Hori K, Hayashi S, Fujimi S: Resolution of primary amyloidosis by melphalan and prednisolone: a case report. Clin Nephrol; 2005 Mar;63(3):215-20
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  • [Title] Resolution of primary amyloidosis by melphalan and prednisolone: a case report.
  • We here report a case of a 50-year-old man who showed histologically evident resolution of primary amyloidosis by melphalan and prednisolone.
  • A renal biopsy revealed global deposition of amyloid in all glomeruli, interstitium and blood vessels.
  • Liver biopsy specimens showed extensive deposition of amyloid along sinusoid walls.
  • Based on these findings, systemic AL- (amyloid light chain) amyloidosis was diagnosed, and the treatment with combinations of melphalan and prednisolone was started from October 1998 at intervals of 4-6 weeks.
  • A second renal biopsy was performed on November 20, 2001, which showed markedly decreased amyloid deposition and a proliferation of mesangial cells and increase in matrix in various degrees.
  • We report a case of a patient with primary amyloidosis who was successfully treated by melphalan and prednisolone, resulting in marked resolution of renal amyloidosis.
  • [MeSH-major] Alkylating Agents / administration & dosage. Amyloidosis / drug therapy. Glucocorticoids / administration & dosage. Kidney Diseases / drug therapy. Melphalan / administration & dosage. Prednisolone / administration & dosage

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  • (PMID = 15786823.001).
  • [ISSN] 0301-0430
  • [Journal-full-title] Clinical nephrology
  • [ISO-abbreviation] Clin. Nephrol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Alkylating Agents; 0 / Glucocorticoids; 9PHQ9Y1OLM / Prednisolone; Q41OR9510P / Melphalan
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44. Li J, Hu J, Shao B, Zhou W, Cui Y, Dong C, Ezoulin JM, Zhu X, Ding W, Heymans F, Chen H: Protection of PMS777, a new AChE inhibitor with PAF antagonism, against amyloid-beta-induced neuronal apoptosis and neuroinflammation. Cell Mol Neurobiol; 2009 Jun;29(4):589-95
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  • [Title] Protection of PMS777, a new AChE inhibitor with PAF antagonism, against amyloid-beta-induced neuronal apoptosis and neuroinflammation.
  • Amyloid-beta (Abeta) plays a central role in the neuroinflammation and cholinergic neuronal apoptosis in Alzheimer's disease, and thus has been considered as a main determinant of this disease.
  • In vivo experimental study demonstrated that PMS777 could attenuate Abeta-induced microglial and astrocytic activation in the rat hippocampus after systemic administration.
  • These results suggest that PMS777 potently protects against Abeta-induced neuronal apoptosis and neuroinflammation, and warrants further investigations in connection with its potential value in the treatment of Alzheimer's disease.
  • [MeSH-major] Amyloid beta-Peptides / pharmacology. Apoptosis / drug effects. Cholinesterase Inhibitors / pharmacology. Furans / pharmacology. Inflammation / metabolism. Neurons / drug effects. Platelet Activating Factor / antagonists & inhibitors

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  • (PMID = 19194797.001).
  • [ISSN] 1573-6830
  • [Journal-full-title] Cellular and molecular neurobiology
  • [ISO-abbreviation] Cell. Mol. Neurobiol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid beta-Peptides; 0 / Cholinesterase Inhibitors; 0 / Furans; 0 / PMS 777; 0 / Platelet Activating Factor; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / bcl-2-Associated X Protein; EC 3.4.22.- / Caspase 3
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45. Ozdemir BH, Uyar P, Ozdemir FN: Diagnosing amyloid goitre with thyroid aspiration biopsy. Cytopathology; 2006 Oct;17(5):262-6
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  • [Title] Diagnosing amyloid goitre with thyroid aspiration biopsy.
  • OBJECTIVE: The aim of the study was to evaluate the value of fine needle aspiration biopsy of the thyroid as a tool for diagnosing amyloid goitre and assess how amyloidosis affects thyroid tissue and thyroid function.
  • METHODS: Clinical and laboratory evaluation of 50 patients with secondary systemic amyloidosis was done, and goitre was found in 38 of them.
  • RESULTS: Of the 38 cases of amyloid goitre, 10 showed euthyroid sick syndrome, two showed primary hyperthyroidism, two showed hypothyroidism and one showed subacute thyroiditis.
  • Thirty-five of the 38 patients (92%) showed amyloidosis after thyroid aspiration.
  • One of these patients had papillary carcinoma in addition to amyloid goitre.
  • Microscopic evaluation revealed that the thyroid parenchyma in all patients was largely replaced with amyloid and adipose tissue.
  • CONCLUSION: Fine needle aspiration of the thyroid is a valuable and sensitive method for diagnosing amyloid goitre, especially because it is a safe and easily performed procedure.
  • Further, amyloid goitre has no significant influence on thyroid function even when it causes extensive parenchyma replacement.
  • [MeSH-major] Amyloidosis / diagnosis. Goiter / diagnosis

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  • (PMID = 16961655.001).
  • [ISSN] 0956-5507
  • [Journal-full-title] Cytopathology : official journal of the British Society for Clinical Cytology
  • [ISO-abbreviation] Cytopathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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46. Dispenzieri A, Kyle RA: Multiple myeloma: clinical features and indications for therapy. Best Pract Res Clin Haematol; 2005;18(4):553-68
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  • Multiple myeloma is a malignant plasma-cell proliferative disease with an expected 15,270 new cases and 11,070 deaths in the USA in 2004 alone.
  • The diagnosis is based on the presence of bone pain, anemia, and plasma-cell infiltrate in the bone marrow or within bone lesions.
  • Other related disorders include primary systemic amyloidosis, POEMS syndrome, and acquired Fanconi syndrome.
  • [MeSH-major] Multiple Myeloma / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Humans. Monoclonal Gammopathy of Undetermined Significance / pathology

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  • (PMID = 16026737.001).
  • [ISSN] 1521-6926
  • [Journal-full-title] Best practice & research. Clinical haematology
  • [ISO-abbreviation] Best Pract Res Clin Haematol
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA62242
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] England
  • [Number-of-references] 64
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47. Zielińska M, Koniarek W, Kaczmarek K, Maciejewski M, Wagrowska-Danilewicz M, Goch JH: [Primary cardiac amyloidosis -- condition which can be diagnosed by a cardiologist]. Kardiol Pol; 2006 May;64(5):517-21
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  • [Title] [Primary cardiac amyloidosis -- condition which can be diagnosed by a cardiologist].
  • [Transliterated title] Amyloidoza pierwotna AL (postać sercowa) -- rozpoznanie, które moze być postawione przez kardiologów.
  • Primary amyloidosis is a systemic disorder caused by the clonal production and tissue deposition of immunoglobulin light chain proteins.
  • The disease symptoms are typical of multisystem failure.
  • Only the patients with earliest diagnosis made and advanced treatment (chemotherapy, autologous stem-cell transplantation, heart transplantation) introduced have the chance of the lengthening of life.
  • The authors present a case of 52-year-old man with a primary amyloidosis, who suffered from severe, not responding to treatment, congestive heart failure.
  • Because of lack of the other organ involvement symptoms, the correct diagnosis was made very late.
  • The combination of specific ECG, echocardiographic findings and positive extracardiac tissue biopsy may be sufficient to reach correct diagnosis.
  • [MeSH-major] Amyloidosis / diagnosis. Cardiomyopathies / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Echocardiography. Electrocardiography. Fatal Outcome. Heart Failure / etiology. Heart Ventricles / ultrasonography. Humans. Male. Middle Aged

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  • (PMID = 16752338.001).
  • [ISSN] 0022-9032
  • [Journal-full-title] Kardiologia polska
  • [ISO-abbreviation] Kardiol Pol
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Poland
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48. Economou EV, Malamitsi-Puchner AV, Pitsavos CP, Kouskouni EE, Magaziotou-Elefsinioti I, Creatsas G: Low-grade systemic inflammation profile, unrelated to homocysteinemia, in obese children. Mediators Inflamm; 2005 Dec 14;2005(6):337-42
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  • [Title] Low-grade systemic inflammation profile, unrelated to homocysteinemia, in obese children.
  • To investigate in prepubertal obese children (POC) the profile of chronic low-grade systemic inflammation (CLGSI) and its relation to homocysteinemia, 72 POC were evaluated for serum C-reactive protein (CRP) and amyloid A (SAA) levels, both markers of CLGSI, and plasma levels of total homocysteine (tHcy), an independent risk factor for adult atherosclerosis, in comparison to 42 prepubertal lean children (PLC).
  • The main observations in POC were higher CRP levels compared to PLC, positive association of SAA levels to CRP levels, no association of CRP or SAA levels to tHcy levels.
  • [MeSH-minor] Adult. Biomarkers / blood. Body Mass Index. C-Reactive Protein / metabolism. Child. Female. Homocysteine / blood. Humans. Male. Risk Factors. Serum Amyloid A Protein / metabolism

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  • (PMID = 16489253.001).
  • [ISSN] 0962-9351
  • [Journal-full-title] Mediators of inflammation
  • [ISO-abbreviation] Mediators Inflamm.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers; 0 / Serum Amyloid A Protein; 0LVT1QZ0BA / Homocysteine; 9007-41-4 / C-Reactive Protein
  • [Other-IDs] NLM/ PMC1533896
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49. Tanskanen M, Paetau A, Salonen O, Salmi T, Lamminen A, Lindsberg P, Somer H, Kiuru-Enari S: Severe ataxia with neuropathy in hereditary gelsolin amyloidosis. Amyloid; 2009 Jun 25;:1-7
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  • [Title] Severe ataxia with neuropathy in hereditary gelsolin amyloidosis.
  • Hereditary gelsolin amyloidosis (AGel amyloidosis) is a systemic disorder caused by a G654A or G654T gelsolin mutation, reported from Europe, North America, and Japan.
  • Principal clinical signs are corneal lattice dystrophy, cutis laxa and cranial neuropathy, often deleterious at advanced age.
  • Neuropathological examination revealed marked gelsolin amyloid deposition at vascular and connective tissue sites along the entire length of the peripheral nerves extending to the spinal nerve roots, associated with severe degeneration of nerve fibers and posterior columns.
  • Our report shows that advanced AGel amyloidosis due to degeneration of central and distal sensory nerve projections results in deleterious ataxia with fatal outcome.

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  • [RetractionIn] Amyloid. 2009 Dec;16(4):246 [19842787.001]
  • (PMID = 19557557.001).
  • [ISSN] 1744-2818
  • [Journal-full-title] Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
  • [ISO-abbreviation] Amyloid
  • [Language] eng
  • [Publication-type] Journal Article; Retracted Publication
  • [Publication-country] England
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50. Karacostas D, Soumpourou M, Mavromatis I, Karkavelas G, Poulios I, Milonas I: Isolated myopathy as the initial manifestation of primary systemic amyloidosis. J Neurol; 2005 Jul;252(7):853-4
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  • [Title] Isolated myopathy as the initial manifestation of primary systemic amyloidosis.
  • [MeSH-major] Amyloidosis / complications. Muscle, Skeletal / pathology. Muscular Diseases / etiology
  • [MeSH-minor] Aged. Amyloid / metabolism. Humans. Male

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  • (PMID = 15742103.001).
  • [ISSN] 0340-5354
  • [Journal-full-title] Journal of neurology
  • [ISO-abbreviation] J. Neurol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Amyloid
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51. García-Escudero López A, Arruza Echevarría A, Leunda Saizar J, Infante Riaño R, Padilla Nieva J, Ortiz Barredo E: [Secondary amyloidosis of the bladder and massive hematuria]. Actas Urol Esp; 2010 Jan;34(1):111-5
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  • [Title] [Secondary amyloidosis of the bladder and massive hematuria].
  • [Transliterated title] Amiloidosis vesical secundaria y hematuria masiva.
  • OBJECTIVE: To report four additional cases of secondary amyloidosis of the bladder, an extremely rare condition, as shown by the cases reported in the literature.
  • RESULTS: Secondary amyloidosis of the bladder is of the AA type, which is more common in females and mainly secondary to rheumatoid arthritis, but also to ankylosing spondylitis and long-standing chronic inflammatory conditions.
  • Hematuria is the main and virtually only symptom.
  • A pathological and immunohistochemical study confirmed diagnosis.
  • CONCLUSIONS: Despite its rarity, as shown by the few cases reported, secondary amyloidosis of the bladder should be considered in patients already diagnosed with systemic amyloidosis and/or the conditions reported who require simple urethral catheterization.
  • [MeSH-major] Amyloidosis / etiology. Arthritis, Rheumatoid / complications. Hematuria / etiology. Urinary Bladder Diseases / etiology


52. Yoshita M, Ishida C, Yanase D, Yamada M: Immunoglobulin light-chain (AL) amyloidosis with myasthenic symptoms and echocardiographic features of dilated cardiomyopathy. Intern Med; 2006;45(3):159-62
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  • [Title] Immunoglobulin light-chain (AL) amyloidosis with myasthenic symptoms and echocardiographic features of dilated cardiomyopathy.
  • Myasthenic symptoms and the echocardiographic findings of dilated cardiomyopathy are very rare in primary AL amyloidosis.
  • His electrocardiogram showed ischemic heart disease and echocardiography indicated dilated cardiomyopathy.
  • Muscle biopsy revealed amyloidosis with deposits of lambda light chain-derived amyloid within the vessel wall.
  • This patient indicates that myasthenic symptoms and dilated cardiomyopathy would be a unique syndrome associated with systemic AL amyloidosis involving mainly the small vessels, i.e., AL amyloid angiopathy, in the skeletal muscles and myocardium vessels.
  • [MeSH-major] Amyloidosis / complications. Cardiomyopathy, Dilated / complications. Echocardiography. Immunoglobulin Light Chains / analysis. Myasthenia Gravis / complications


53. Katoh N, Tazawa K, Ishii W, Matsuda M, Ikeda S: Systemic AL amyloidosis mimicking rheumatoid arthritis. Intern Med; 2008;47(12):1133-8
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  • [Title] Systemic AL amyloidosis mimicking rheumatoid arthritis.
  • We report a patient with myeloma-associated systemic AL amyloidosis who showed chronic polyarthralgia as the main symptom.
  • He was diagnosed as having systemic AL amyloidosis based on deposition of kappa-light chain-immunoreactive amyloid in biopsied tissue and Bence Jones protein in serum and urine.
  • Magnetic resonance imaging and bone scintigraphy suggested this disease as the cause of the polyarthralgia.
  • Systemic AL amyloidosis may be important in the differential diagnosis of chronic polyarthralgia.
  • [MeSH-major] Amyloidosis / diagnosis. Arthralgia / etiology. Arthritis, Rheumatoid / diagnosis. Multiple Myeloma / complications
  • [MeSH-minor] Bence Jones Protein / urine. Bone Marrow Examination. Chronic Disease. Diagnosis, Differential. Humans. Immunoglobulin kappa-Chains / analysis. Male. Middle Aged


54. Tam M, Seldin DC, Forbes BM, Connors LH, Skinner M, Oran B, Quillen K, Sanchorawala V: Spontaneous rupture of the liver in a patient with systemic AL amyloidosis undergoing treatment with high-dose melphalan and autologous stem cell transplantation: a case report with literature review. Amyloid; 2009;16(2):103-7
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  • [Title] Spontaneous rupture of the liver in a patient with systemic AL amyloidosis undergoing treatment with high-dose melphalan and autologous stem cell transplantation: a case report with literature review.
  • A 55-year-old woman with primary Immunoglobulin light chain (AL) systemic amyloidosis died due to spontaneous rupture of her liver following treatment with high-dose melphalan and autologous stem cell transplant (HDM/SCT).
  • Amyloid fibrils were extracted from the autopsied liver sample (05-135L) and the biochemical nature of the fibrils was analyzed using electrophoretic and immunohistochemical techniques.
  • While the liver is often involved in AL amyloidosis, this is the first documented case of a spontaneous hepatic rupture in a patient during treatment with HDM/SCT.
  • A literature review of spontaneous liver rupture in patients with amyloidosis is presented.

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  • (PMID = 20536404.001).
  • [ISSN] 1744-2818
  • [Journal-full-title] Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
  • [ISO-abbreviation] Amyloid
  • [Language] ENG
  • [Grant] United States / NHLBI NIH HHS / HL / P01 HL68705; United States / NHLBI NIH HHS / HL / HL068705-010003; United States / NHLBI NIH HHS / HL / HL068705-01; United States / NHLBI NIH HHS / HL / P01 HL068705-040003; United States / NHLBI NIH HHS / HL / P01 HL068705-030003; United States / NHLBI NIH HHS / HL / P01 HL068705-01; United States / NHLBI NIH HHS / HL / P01 HL068705-050003; United States / NHLBI NIH HHS / HL / P01 HL068705-010003; United States / NHLBI NIH HHS / HL / HL068705-020003; United States / NHLBI NIH HHS / HL / P01 HL068705; United States / NHLBI NIH HHS / HL / P01 HL068705-020003; United States / NHLBI NIH HHS / HL / HL068705-040003; United States / NHLBI NIH HHS / HL / HL068705-050003; United States / NHLBI NIH HHS / HL / HL068705-030003; United States / NHLBI NIH HHS / HL / HL068705-02; United States / NHLBI NIH HHS / HL / P01 HL068705-02
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Amyloid; Q41OR9510P / Melphalan
  • [Other-IDs] NLM/ NIHMS220595; NLM/ PMC2911629
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55. Prat C, Moreno A, Viñas M, Jucglà A: Nail dystrophy in primary systemic amyloidosis. J Eur Acad Dermatol Venereol; 2008 Jan;22(1):107-9
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  • [Title] Nail dystrophy in primary systemic amyloidosis.
  • [MeSH-major] Amyloidosis / complications. Amyloidosis / diagnosis. Nail Diseases / diagnosis. Nail Diseases / etiology
  • [MeSH-minor] Aged. Atrophy. Early Diagnosis. Humans. Male. Nails / pathology

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  • (PMID = 18181984.001).
  • [ISSN] 1468-3083
  • [Journal-full-title] Journal of the European Academy of Dermatology and Venereology : JEADV
  • [ISO-abbreviation] J Eur Acad Dermatol Venereol
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Netherlands
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56. Zelichowski G, Lubas A, Wańkowicz Z: [Advances in diagnosis and treatment of AL amyloidosis]. Pol Merkur Lekarski; 2008 Apr;24(142):340-5
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  • [Title] [Advances in diagnosis and treatment of AL amyloidosis].
  • AL amyloidosis is a systemic disease characterized by extracellular amyloid deposition in tissues, causing progressive dysfunction of affected organs.
  • The main clinical syndroms include nephrotic-range proteinuria with or without renal dysfunction, congestive heart failure, hepatomegaly and peripheral or autonomic neuropathy.
  • Recent therapeutic strategies involve intermediate-dose chemotherapy or high-dose melphalan supported by peripheral blood stem cell transplantation.
  • This paper reviews the pathogenesis, diagnosis and therapy of the AL amyloidosis, focusing on clinico-morphological symptoms of renal involvement, monitoring of treatment response and supportive therapy.
  • [MeSH-major] Amyloidosis / diagnosis. Amyloidosis / therapy. Kidney Diseases / diagnosis. Kidney Diseases / therapy
  • [MeSH-minor] Amyloid / metabolism. Antineoplastic Agents / therapeutic use. Humans. Immunotherapy. Nephrotic Syndrome / etiology

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  • (PMID = 18634370.001).
  • [ISSN] 1426-9686
  • [Journal-full-title] Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego
  • [ISO-abbreviation] Pol. Merkur. Lekarski
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Amyloid; 0 / Antineoplastic Agents
  • [Number-of-references] 48
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57. Xu L, Cai BQ, Zhong X, Zhu YJ: Respiratory manifestations in amyloidosis. Chin Med J (Engl); 2005 Dec 20;118(24):2027-33
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  • [Title] Respiratory manifestations in amyloidosis.
  • BACKGROUND: Amyloidosis is a collection of diseases in which different proteins are deposited.
  • Amyloid deposits occur in systemic and organ-limited forms.
  • In both systemic and localized forms of the disease, lung can be involved.
  • The aim of this study was to explore the different respiratory manifestations of amyloidosis.
  • METHODS: Chest radiology, clinical presentations, bronchoscopic/laryngoscopic findings and lung function data of 59 patients with amyloidosis involving respiratory tract collected during January 1986 to March 2005, were analysed.
  • RESULTS: Of the 16 cases with localized respiratory tract amyloidosis, 8 had the lesions in the trachea and the bronchi, 2 in the larynx and the trachea, 5 in the larynx and/or the pharynx, and 1 in the lung parenchyma.
  • Of 43 systemic amyloidosis with respiratory tract involvement, 3 had the lesions in bronchi, 13 in lung parenchyma, 33 in pleura, 8 in mediastina, 1 in nose and 1 in pharynx.
  • Chest X-rays were normal in most cases of tracheobronchial amyloidosis.
  • Localized lung parenchymal amyloidosis presented as multiple nodules.
  • Multiple nodular opacities, patch shadows and reticular opacities were the main radiological findings in systemic amyloidosis with lung parenchymal involvement.
  • In pleural amyloidosis, pleural effusions and pleural thickening were detected.
  • Mediastinal and/or hilar adenopathy were also a form of lung involvement in systemic amyloidosis.
  • The major bronchoscopic findings of tracheobronchial amyloidosis were narrowing of airway lumen, while nodular, 'tumour like' or 'bubble like' masses, with missing or vague cartilaginous rings, were detected in about half of the patients.
  • CONCLUSIONS: Localized respiratory tract amyloidosis mostly affects the trachea and the bronchi.
  • Systemic amyloidosis often involves lung parenchyma and the pleura.
  • Open lung biopsy or pleural biopsy should be performed for the diagnosis.
  • [MeSH-major] Amyloidosis / complications. Respiratory Tract Diseases / etiology

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  • (PMID = 16438898.001).
  • [ISSN] 0366-6999
  • [Journal-full-title] Chinese medical journal
  • [ISO-abbreviation] Chin. Med. J.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] China
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58. Pentenero M, Davico Bonino L, Tomasini C, Conrotto D, Gandolfo S: Localized oral amyloidosis of the palate. Amyloid; 2006 Mar;13(1):42-6
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  • [Title] Localized oral amyloidosis of the palate.
  • BACKGROUND: Amyloidosis is a rare disease with multifactorial pathogenesis.
  • Localized amyloidosis affecting the head and neck region is an uncommon and benign process, which has almost no clinical consequences.
  • The most reported characteristic features of localized oral amyloidosis appear as multiple soft nodules of the tongue, lip and cheek.
  • METHODS: We report the case of a 68-year-old woman suffering from a primary localized amyloidosis presenting as a purple patch on the palate.
  • CONCLUSIONS: The presence of systemic amyloidosis or underlying plasma cell dyscrasia have to be ruled out in patients presenting with a diagnosis of amyloidosis of the oral mucosa.
  • If a primary localized amyloidosis is proven, the surgical therapy may be useful to eliminate a functional impairment.
  • [MeSH-major] Amyloidosis / diagnosis. Mouth Diseases / diagnosis. Palate / pathology

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  • (PMID = 16690500.001).
  • [ISSN] 1350-6129
  • [Journal-full-title] Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
  • [ISO-abbreviation] Amyloid
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antifungal Agents
  • [Number-of-references] 29
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59. Fariña Pérez LA, Ortiz Rey JA: [Secondary bladder amylodosis with severe recurrent hematuria: transurethral Mikuliz procedure as hemostatic option]. Arch Esp Urol; 2005 Sep;58(7):665-8
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  • [Transliterated title] Hematuria grave recidivante por amiloidosis vesical secundaria. Mikulicz transuretral como opción hemostática.
  • OBJECTIVES: Primary localized amyloidosis of the urinary bladder generally has a benign course.
  • On the contrary, secondary amyloidosis, a consequence of systemic amyloidosis, may have massive bleeding and produce complications such as bladder rupture or lifethreatening hemodynamic problems requiring desperate hemostatic procedures such as hypogastric artery embolization or ligature, or cystectomy.
  • METHODS: 58 year old female with very aggressive rheumatoid arthritis and secondary renal amyloidosis under chronic hemodialysis presenting with severe hematuria after hip replacement.
  • [MeSH-major] Amyloidosis / complications. Hematuria / etiology. Hemostatic Techniques. Urinary Bladder Diseases / complications

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  • (PMID = 16294789.001).
  • [ISSN] 0004-0614
  • [Journal-full-title] Archivos españoles de urología
  • [ISO-abbreviation] Arch. Esp. Urol.
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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60. Jhingan A, Lee JS, Kumarasinghe SP: Lichen amyloidosis in an unusual location. Singapore Med J; 2007 Jun;48(6):e165-7
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  • [Title] Lichen amyloidosis in an unusual location.
  • We report lichen amyloidosis occurring on the upper lip and nasolabial folds of a 61-year-old woman from Singapore.
  • She had a past history of systemic lupus erythematosus, which was in remission for three years.
  • Skin biopsy from one of the lesions showed amyloid deposits in the dermis which were Congo red stain positive.
  • Immunohistochemical staining of the amyloid deposits stained positive for cytokeratins (CK) 5 and 6, and negative for CK 14.
  • Further investigations, including multiple myeloma screen and rectal biopsy, ruled out systemic amyloidosis.
  • There was no other evidence of cutaneous amyloidosis on her limbs or trunk.
  • This case highlights the commonly-seen form of primary localised cutaneous amyloidosis in an unusual location.
  • [MeSH-major] Amyloidosis / pathology. Face / pathology. Lichen Sclerosus et Atrophicus / pathology


61. Silva D, Sargento L, Varela MG, Brito D, Lopes MG: Behind heart failure syndrome: remember AL amyloidosis. Two case-reports. Rev Port Cardiol; 2010 Nov;29(11):1751-9
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  • [Title] Behind heart failure syndrome: remember AL amyloidosis. Two case-reports.
  • Amyloidosis is a systemic disease that is a consequence of extracellular deposition of insoluble fibrils composed of subunits of low molecular weight (5-25 kD) derived from a variety of plasma proteins.
  • Identification of the amyloidogenic protein determines the type of amyloidosis.
  • In primary systemic amyloidosis (classically called AL amyloidosis), the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia.
  • Cardiac manifestations are the most common clinical presentation of this type of amyloidosis, occurring in 50% of patients.
  • The authors describe two cases in which hospitalization was due to decompensated heart failure, which were similar in their etiology (multiple myeloma/amyloid cardiomyopathy) and evolution (sudden death).
  • [MeSH-major] Amyloidosis / complications. Heart Failure / etiology

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  • (PMID = 21309362.001).
  • [ISSN] 0870-2551
  • [Journal-full-title] Revista portuguesa de cardiologia : orgão oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
  • [ISO-abbreviation] Rev Port Cardiol
  • [Language] eng; por
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Portugal
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62. Porrata LF, Gertz MA, Litzow MR, Lacy MQ, Dispenzieri A, Inwards DJ, Ansell SM, Micallef IN, Gastineau DA, Elliott M, Hogan WJ, Hayman SR, Tefferi A, Markovic SN: Early lymphocyte recovery predicts superior survival after autologous hematopoietic stem cell transplantation for patients with primary systemic amyloidosis. Clin Cancer Res; 2005 Feb 1;11(3):1210-8
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  • [Title] Early lymphocyte recovery predicts superior survival after autologous hematopoietic stem cell transplantation for patients with primary systemic amyloidosis.
  • The relationship of ALC-15 with clinical outcomes in primary systemic amyloidosis is unknown.
  • EXPERIMENTAL DESIGN: We evaluated 145 consecutive patients with primary systemic amyloidosis who underwent ASCT at the Mayo Clinic from 1996 to 2003.
  • CONCLUSIONS: ALC-15 > or = 500 cells/microL is associated with significantly improved clinical outcomes following ASCT in patients with primary systemic amyloidosis.
  • [MeSH-major] Amyloidosis / therapy. Hematopoietic Stem Cell Transplantation. Lymphocytes / cytology

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  • (PMID = 15709191.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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63. Yamazaki Y, Ochi K, Nakata Y, Dohi E, Eguchi K, Yamaguchi S, Matsushige T, Ueda T, Amatya VJ, Takeshima Y, Nakamura T, Ohtsuki T, Kohriyama T, Matsumoto M: Trigeminal neuropathy from perineural spread of an amyloidoma detected by blink reflex and thin-slice magnetic resonance imaging. Muscle Nerve; 2010 Jun;41(6):875-8
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  • There was no evidence of systemic amyloidosis or underlying inflammatory or neoplastic disorders.
  • A rare trigeminal neuropathy resulted from the perineural spread of a primary amyloidoma that was difficult to detect by conventional magnetic resonance imaging.
  • [MeSH-major] Amyloidosis / diagnosis. Blinking / physiology. Trigeminal Ganglion / pathology. Trigeminal Nerve Diseases / etiology. Trigeminal Nerve Diseases / pathology
  • [MeSH-minor] Biopsy. Female. Humans. Magnetic Resonance Imaging. Meckel Diverticulum / diagnosis. Meckel Diverticulum / pathology. Middle Aged

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  • (PMID = 20513106.001).
  • [ISSN] 1097-4598
  • [Journal-full-title] Muscle & nerve
  • [ISO-abbreviation] Muscle Nerve
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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64. Modesto KM, Dispenzieri A, Gertz M, Cauduro SA, Khandheria BK, Seward JB, Kyle R, Wood CM, Bailey KR, Tajik AJ, Miller FA, Pellikka PA, Abraham TP: Vascular abnormalities in primary amyloidosis. Eur Heart J; 2007 Apr;28(8):1019-24
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  • [Title] Vascular abnormalities in primary amyloidosis.
  • AIMS: Primary amyloidosis (AL) is a systemic disease; however, there is limited information regarding the presence and character of vascular abnormalities.
  • [MeSH-major] Amyloidosis / pathology. Vascular Diseases / pathology

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  • (PMID = 17430997.001).
  • [ISSN] 0195-668X
  • [Journal-full-title] European heart journal
  • [ISO-abbreviation] Eur. Heart J.
  • [Language] eng
  • [Grant] United States / NHLBI NIH HHS / HL / HL076513-02
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] England
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65. Bergesio F, Ciciani AM, Santostefano M, Brugnano R, Manganaro M, Palladini G, Di Palma AM, Gallo M, Tosi PL, Salvadori M, Immunopathology Group, Italian Society of Nephrology: Renal involvement in systemic amyloidosis--an Italian retrospective study on epidemiological and clinical data at diagnosis. Nephrol Dial Transplant; 2007 Jun;22(6):1608-18
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  • [Title] Renal involvement in systemic amyloidosis--an Italian retrospective study on epidemiological and clinical data at diagnosis.
  • BACKGROUND: Few data are available on epidemiology and clinical picture of renal involvement in different forms of systemic amyloidosis.
  • METHODS: Patients with biopsy-proven systemic amyloidosis diagnosed in Italy between January 1995 and December 2000 were selected from 49 Nephrology and Internal Medicine Units provided they showed signs characteristic of renal involvement.
  • Clinical and laboratory information were collected by using a specific data form for diagnosis integrated by a questionnaire on diagnostic tools.
  • Collected data were matched both with the Italian Registry of Renal Biopsies (IRRB) and the Registry of the Italian Society of Amyloidosis (SIA) in order to approximate the incidence of the disease.
  • RESULTS: Of all patients, 373 were finally selected throughout Italy with an estimated mean incidence of renal amyloidosis of 2.1 per million population (p.m.p.) per year.
  • Of those, 237 were affected from AL (primary) amyloidosis, 104 from AA (secondary) amyloidosis and 6 from AF (heredofamilial) forms.
  • In 26 cases the type of amyloidosis remained undetermined.
  • Rheumatoid arthritis (RA) was the commonest underlying disease in AA.
  • The main clinical features of renal involvement were represented by nephrotic syndrome and renal failure observed in 59 and 54% of cases, respectively.
  • Patients with AA showed a worse renal function at presentation than patients with AL, possibly due to a late diagnosis and/or referral to nephrology units.
  • Diagnosis was obtained by renal biopsy in 315 cases, by abdominal fat tissue (AFT) aspiration/biopsy in 156 patients and by other organ biopsies in 47 patients.
  • CONCLUSIONS: Our results point to an increased incidence of renal amyloidosis observed in Italy over the period 1996-2000 with AL as the prevalent type.
  • Characterization of amyloid deposits still remains the major diagnostic challenge of the disease.
  • The institution of networks dedicated to rare diseases is strongly recommended in order to effectively afford this challenge.
  • [MeSH-major] Amyloidosis / diagnosis. Amyloidosis / epidemiology. Kidney Diseases / diagnosis. Kidney Diseases / epidemiology

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  • (PMID = 17395661.001).
  • [ISSN] 0931-0509
  • [Journal-full-title] Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • [ISO-abbreviation] Nephrol. Dial. Transplant.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] England
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66. Sarkar C, Chand Sharma M, Nayak A, Mercy Ralte A, Gupta V, Singh S, Behari M: Primary AL (kappa-light chain) amyloidosis manifesting as peripheral neuropathy in a young male without increase in serum and urine immunoglobulin load: a diagnostic challenge. Clin Neuropathol; 2005 May-Jun;24(3):118-25
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  • [Title] Primary AL (kappa-light chain) amyloidosis manifesting as peripheral neuropathy in a young male without increase in serum and urine immunoglobulin load: a diagnostic challenge.
  • Primary systemic or AL amyloidosis is a multisystem disorder characterized by diffuse extracellular infiltration of a fibrillar protein of monoclonal light chain origin (AL).
  • This disease has the widest spectrum of organ involvement, most commonly affecting the kidneys, heart and liver.
  • Involvement of peripheral nervous system is not infrequent and may be the presenting feature of the disease process.
  • Thus, recognition of peripheral neuropathy and affecting the kidney as an early symptom of AL amyloidosis may widen the scope for therapeutic intervention.
  • We describe here a rare case of primary amyloidosis (AL) kappa-light chain presenting with clinical features of peripheral neuropathy and affecting the kidney and heart at an early age of 18 years, hitherto unreported in literature.
  • Diagnosis was confirmed using immuno-electron microscopy on sural nerve biopsy.
  • [MeSH-major] Amyloidosis / complications. Immunoglobulin kappa-Chains / immunology. Peripheral Nervous System Diseases / immunology
  • [MeSH-minor] Adolescent. Age Factors. Amyloid / metabolism. Axons / pathology. Biopsy. Diagnosis, Differential. Humans. Leg / pathology. Leg / physiopathology. Male. Muscle, Skeletal / innervation. Muscle, Skeletal / pathology. Muscle, Skeletal / physiopathology. Muscular Atrophy / immunology. Muscular Atrophy / pathology. Muscular Atrophy / physiopathology. Paresis / immunology. Paresis / pathology. Paresis / physiopathology. Plasma Cells / immunology. Plasma Cells / metabolism. Plasma Cells / pathology. Sensation Disorders / immunology. Sensation Disorders / pathology. Sensation Disorders / physiopathology. Sural Nerve / pathology. Sural Nerve / physiopathology

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  • (PMID = 15943163.001).
  • [ISSN] 0722-5091
  • [Journal-full-title] Clinical neuropathology
  • [ISO-abbreviation] Clin. Neuropathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Amyloid; 0 / Immunoglobulin kappa-Chains
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67. Gan EY, Tey HL: Primary cutaneous nodular amyloidosis initially presenting with eczema. Singapore Med J; 2010 Sep;51(9):e158-60
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  • [Title] Primary cutaneous nodular amyloidosis initially presenting with eczema.
  • We report an unusual case of a 76-year-old woman with primary cutaneous amyloidosis who initially presented with features of asteatotic eczema that was unresponsive to topical corticosteroid treatment.
  • Histological examination revealed amyloid deposits involving the superficial and deep dermis.
  • These lesions later gradually evolved into erythematous nodules, and a second biopsy performed 29 months after the initial presentation again revealed diffuse collections of amyloid throughout the dermis.
  • Further investigations did not reveal evidence of systemic involvement, thus indicating a diagnosis of primary cutaneous nodular amyloidosis.
  • [MeSH-major] Amyloidosis / diagnosis. Eczema / diagnosis. Skin Diseases / diagnosis
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Aged. Amyloid. Biopsy. Collagen / chemistry. Congo Red / pharmacology. Dermatology / methods. Female. Humans. Skin


68. Bogov B, Lubomirova M, Kiperova B: Biopsy of subcutaneus fatty tissue for diagnosis of systemic amyloidosis. Hippokratia; 2008;12(4):236-9
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  • [Title] Biopsy of subcutaneus fatty tissue for diagnosis of systemic amyloidosis.
  • BACKGROUND: The diagnosis of systemic amyloidosis is determined through histological material from biopsy of different parenchymal organs, which have high diagnostic and informative value, but hide a high risk of bleeding because of the accumulation of amyloid in the vessels' wall.
  • The main methods are kidney, liver, gastro-intestinal tract biopsy and aspiration of subcutaneous fatty tissue.
  • The first group consisted of patients with kidney biopsy proved amyloidosis compared to biopsy findings from other parenchymal organs.
  • The second group consisted of patients suspected having amyloidosis who underwent biopsies from various tissues or organs except kidney biopsy because there was contraindication.
  • RESULTS: One hundred fifteen biopsies of subcutaneous fatty tissue (SFT) were performed for the diagnosis of systemic amyloidosis.
  • In order to compare the data from the BSFT to the other known and practiced till the moment methods BSFT was performed in 54 patients with proved amyloidosis by KB.
  • In 51/54 the positive result for amyloid was confirmed.
  • In 14/19 the amyloidosis was typed as AA (74.2%) and 5/19 non-AA, probably AL (25.8%).
  • To reveal the meaning of so called screening-biopsy of subcutaneous fatty tissue for excluding accompanying amyloidosis in patients with significant proteinuria and/or uremia, dysglobulinemia, laboratory constellations for nephritic syndrome in immune nephropathies and chronic infections (Chronic Obstructive Lung Disease, purulent infections) with contraindications for kidney biopsy 61 screening BSFT were performed, accumulation of amyloid was defined in 37.
  • CONCLUSION: The purposeful searching and proving of amyloid in subcutaneous fatty tissue of the abdominal wall is a new, highly sensitive method.
  • The receiving of richer material from SFT in the method "biopsy" in stead of "aspiration", makes it more reliable for proving amyloid in the case that it exists.
  • The method is enough informative for proving not only amyloidosis AL, but also for amyloidosis AA, in treating with KMnO4.
  • The biopsy of SFT in combination with biopsies from other mucosa can prove the accumulation of amyloid in contraindications for performing KB.

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  • (PMID = 19158968.001).
  • [ISSN] 1790-8019
  • [Journal-full-title] Hippokratia
  • [ISO-abbreviation] Hippokratia
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Other-IDs] NLM/ PMC2580046
  • [Keywords] NOTNLM ; aspiration of subcutaneous fatty tissue / biopsy of subcutaneous fatty tissue / histological diagnosis / systemic amyloidosis
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69. Fernández de Larrea C, Cibeira MT, Rovira M, Rosiñol L, Esteve J, Bladé J: Spontaneous rupture of the spleen as immediate complication in autologous transplantation for primary systemic amyloidosis. Eur J Haematol; 2008 Feb;80(2):182-4
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  • [Title] Spontaneous rupture of the spleen as immediate complication in autologous transplantation for primary systemic amyloidosis.
  • Although splenic rupture is a recognized complication of primary amyloidosis, very few cases have been reported in the context of stem cell transplantation.
  • A patient with systemic primary amyloidosis with renal and cardiac involvement and factor X deficiency, who developed a splenic rupture 24 h after the peripheral blood stem cells infusion during autologous transplantation, is described.
  • [MeSH-major] Amyloidosis / complications. Amyloidosis / therapy. Hematopoietic Stem Cell Transplantation / adverse effects. Hematopoietic Stem Cell Transplantation / methods. Rupture, Spontaneous / diagnosis. Spleen / pathology. Splenic Rupture / diagnosis. Transplantation, Autologous / adverse effects. Transplantation, Autologous / methods

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  • (PMID = 18081703.001).
  • [ISSN] 1600-0609
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Denmark
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70. Oğuz H, Safak MA, Demirci M, Arslan N: Familial primary localized laryngeal amyloidosis in two sisters. Kulak Burun Bogaz Ihtis Derg; 2007;17(5):283-6
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  • [Title] Familial primary localized laryngeal amyloidosis in two sisters.
  • Laryngeal amyloidosis is rare, accounting for less than 1% of all benign laryngeal tumors.
  • Although familial primary localized amyloidosis has been reported in other parts of the body, no familial cases have been reported in the larynx.
  • Primary localized laryngeal amyloidosis was detected in two sisters whose ages were 35 years and 38 years, respectively.
  • The results of endolaryngeal biopsies performed in both patients were reported as amyloidosis.
  • Further evaluations were negative for systemic amyloidosis.
  • The patients were monitored for more than two years without any other coexisting disease.
  • [MeSH-major] Amyloidosis, Familial / diagnosis. Laryngeal Diseases / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Genetic Predisposition to Disease. Humans. Pedigree. Siblings

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  • (PMID = 18187988.001).
  • [ISSN] 1300-7475
  • [Journal-full-title] Kulak burun boğaz ihtisas dergisi : KBB = Journal of ear, nose, and throat
  • [ISO-abbreviation] Kulak Burun Bogaz Ihtis Derg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Turkey
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71. Dispenzieri A, Lacy MQ, Katzmann JA, Rajkumar SV, Abraham RS, Hayman SR, Kumar SK, Clark R, Kyle RA, Litzow MR, Inwards DJ, Ansell SM, Micallef IM, Porrata LF, Elliott MA, Johnston PB, Greipp PR, Witzig TE, Zeldenrust SR, Russell SJ, Gastineau D, Gertz MA: Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation. Blood; 2006 Apr 15;107(8):3378-83
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  • [Title] Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation.
  • The immunoglobulin free light chain (FLC) is the precursor protein of amyloid in primary systemic amyloidosis (AL).
  • Historically, the ability to monitor the amyloid protein precursor protein has been crude.
  • Baseline FLC correlated with serum cardiac troponin levels, and higher FLC levels were associated with more organs involved by amyloid, suggesting that high FLC levels may be associated with more advanced disease.

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  • (PMID = 16397135.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA62 242
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid; 0 / Biomarkers; 0 / Immunoglobulin Light Chains
  • [Other-IDs] NLM/ PMC1895763
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72. Shukla S, Cohen A, Josephberg RG: Nonfamilial vitreous amyloidosis diagnosed by portable sutureless vitrectomy. Retin Cases Brief Rep; 2008;2(4):264-5
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  • [Title] Nonfamilial vitreous amyloidosis diagnosed by portable sutureless vitrectomy.
  • PURPOSE: To describe a case of primary nonfamilial vitreous amyloidosis and a novel technique for expediting vitreous biopsy.
  • A systemic medical workup including family history was noncontributory.
  • Given a high clinical suspicion of vitreous amyloidosis, the decision was made to obtain a vitreous biopsy for ultrastructural study.
  • Pathologic study of the vitreous specimen confirmed the diagnosis of amyloidosis.
  • CONCLUSION: Vitreous amyloid deposition may occur with neither systemic involvement nor family history.
  • Sutureless pars plana vitrectomy may facilitate diagnosis while saving time and expense for both the physician and the patient.

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  • (PMID = 25390585.001).
  • [ISSN] 1935-1089
  • [Journal-full-title] Retinal cases & brief reports
  • [ISO-abbreviation] Retin Cases Brief Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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73. Leung N, Griffin MD, Dispenzieri A, Haugen EN, Gloor JM, Schwab TR, Textor SC, Lacy MQ, Litzow MR, Cosio FG, Larson TS, Gertz MA, Stegall MD: Living donor kidney and autologous stem cell transplantation for primary systemic amyloidosis (AL) with predominant renal involvement. Am J Transplant; 2005 Jul;5(7):1660-70
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  • [Title] Living donor kidney and autologous stem cell transplantation for primary systemic amyloidosis (AL) with predominant renal involvement.
  • Primary systemic amyloidosis (AL) is characterized by multiorgan deposition of monoclonal immunoglobulin light chain.
  • Autologous stem cell transplantation (SCT) for AL achieves superior response rates compared to chemotherapy alone but patients with end-stage renal disease (ESRD) may be excluded from consideration.
  • One patient, who has thus far elected not to undergo SCT, has proteinuria and histologic evidence of recurrent renal amyloidosis.
  • [MeSH-major] Amyloidosis / surgery. Kidney Diseases / surgery. Kidney Transplantation. Living Donors. Stem Cell Transplantation


74. Seccia V, Dallan I, Cervetti G, Lenzi R, Marchetti M, Casani AP, Muscatello L: A rare case of primary systemic amyloidosis of the neck with massive cervical lymph node involvement: a case report and review of the literature. Leuk Res; 2010 Apr;34(4):e100-3
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  • [Title] A rare case of primary systemic amyloidosis of the neck with massive cervical lymph node involvement: a case report and review of the literature.
  • Amyloidosis is a term applied to a diverse group of disorders that share the deposition of amyloid protein in various extracellular tissues.
  • Systemic amyloidosis may involve almost any organ system in the body including regions in the head and neck; however, neck lymph node involvement is rare, with only five previous cases reported.
  • We present the case of a primary systemic AL amyloidosis with hepatic, cervical, retroperitoneal, axillary and inguinal lymphnode localizations, unresponsive to medical therapy and treated with a surgical approach followed by autologous bone marrow transplantation.
  • [MeSH-major] Amyloidosis / complications. Lymphatic Diseases / etiology. Neck. Otorhinolaryngologic Diseases / complications

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  • [Copyright] Copyright (c) 2009 Elsevier Ltd. All rights reserved.
  • (PMID = 19931179.001).
  • [ISSN] 1873-5835
  • [Journal-full-title] Leukemia research
  • [ISO-abbreviation] Leuk. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Letter; Review
  • [Publication-country] England
  • [Number-of-references] 16
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75. Hoffman HM, Simon A: Recurrent febrile syndromes: what a rheumatologist needs to know. Nat Rev Rheumatol; 2009 May;5(5):249-56
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  • These syndromes are all characterized by recurrent episodes of fever and systemic inflammation; however, some syndromes have unique historical and physical features that can help with making a diagnosis.
  • The primary associated morbidity is systemic amyloidosis, usually with renal involvement.
  • [MeSH-major] Fever / diagnosis. Periodicity. Rheumatology / methods
  • [MeSH-minor] Amyloidosis. Anti-Inflammatory Agents, Non-Steroidal / therapeutic use. Colchicine / therapeutic use. Familial Mediterranean Fever / diagnosis. Familial Mediterranean Fever / drug therapy. Familial Mediterranean Fever / physiopathology. Fatigue / diagnosis. Fatigue / drug therapy. Fatigue / physiopathology. Glucocorticoids / therapeutic use. Humans. Inflammation / diagnosis. Inflammation / drug therapy. Inflammation / physiopathology. Kidney Diseases. Professional Practice. Syndrome

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  • (PMID = 19412191.001).
  • [ISSN] 1759-4804
  • [Journal-full-title] Nature reviews. Rheumatology
  • [ISO-abbreviation] Nat Rev Rheumatol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents, Non-Steroidal; 0 / Glucocorticoids; SML2Y3J35T / Colchicine
  • [Number-of-references] 56
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76. Pest EP, McQuaker G, Hunter JA, Moffat D, Stanley AJ: Primary hyperparathyroidsm, amyloid and multiple myeloma: an unusual association. Scott Med J; 2005 Feb;50(1):32-4
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  • [Title] Primary hyperparathyroidsm, amyloid and multiple myeloma: an unusual association.
  • We report the case of a 76-year-old woman with a diagnosis of Primary Hyperparathyroidsm and Systemic Amyloidosis, in whom subsequent investigations revealed the presence of Multiple Myeloma.
  • [MeSH-major] Amyloidosis / complications. Hyperparathyroidism / complications. Multiple Myeloma / complications

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  • (PMID = 15792389.001).
  • [ISSN] 0036-9330
  • [Journal-full-title] Scottish medical journal
  • [ISO-abbreviation] Scott Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Scotland
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77. Zaky ZS, Liepnieks JJ, Rex DK, Cummings OW, Benson MD: Lambda II immunoglobulin light chain protein in primary localized rectal amyloidosis. Amyloid; 2007 Dec;14(4):299-304
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  • [Title] Lambda II immunoglobulin light chain protein in primary localized rectal amyloidosis.
  • Rectal involvement is usually part of a systemic amyloidosis, whereas, localized rectal amyloidosis is a rare entity.
  • Amyloid fibrils were isolated from rectal biopsy tissue and characterized by sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) which showed bands at 17 kDa, 21 kDa and 28 kDa, a broad doublet band at 7-8 kDa and weaker bands at 15 kDa and 24 kDa.
  • Edman sequence analysis of the isolated protein and its tryptic peptides showed that the amyloid protein was derived from an immunoglobulin lambdaII-light chain.
  • To our knowledge, this is the first reported case to isolate and chemically characterize amyloid fibrils from a localized rectal amyloidoma.
  • The development of specific therapies for patients with amyloid-associated disorders emphasizes the need to characterize the biochemical nature of the amyloid fibril protein.
  • [MeSH-major] Amyloid / metabolism. Amyloidosis / metabolism. Immunoglobulin lambda-Chains / metabolism. Rectum / metabolism

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  • (PMID = 17968691.001).
  • [ISSN] 1350-6129
  • [Journal-full-title] Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
  • [ISO-abbreviation] Amyloid
  • [Language] eng
  • [Grant] United States / NIA NIH HHS / AG / AG10133; United States / NIDDK NIH HHS / DK / DK42111; United States / NCRR NIH HHS / RR / RR-00750
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid; 0 / Immunoglobulin lambda-Chains
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78. Huang CY, Shun CT, Huang KH, Chen J, Pu YS: Primary amyloidosis of the urinary bladder. J Formos Med Assoc; 2006 Feb;105(2):164-7
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  • [Title] Primary amyloidosis of the urinary bladder.
  • Amyloidosis is a systemic disease that usually occurs in the gastrointestinal tract or in muscular or adipose tissue.
  • Primary amyloidosis of the urinary bladder is a rare disease that can mimic bladder cancer on cystoscopic examination as well as in its clinical presentation of painless gross hematuria.
  • Hence, the diagnosis of amyloidosis of the urinary bladder was confirmed.
  • Screening for amyloidosis was negative in other organ systems and the patient has remained disease-free up to the last follow-up 4 years after the second transurethral resection.
  • Amyloidosis should be considered in the differential diagnosis of patients with recurrent hematuria who have symptoms characteristic of bladder cancer but negative pathologic study for malignancy.
  • Correct diagnosis relies on clinical alertness and the use of a special staining technique during pathologic examination.

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  • (PMID = 16477338.001).
  • [ISSN] 0929-6646
  • [Journal-full-title] Journal of the Formosan Medical Association = Taiwan yi zhi
  • [ISO-abbreviation] J. Formos. Med. Assoc.
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Singapore
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79. Iqbal S, Reehana S, Lawrence D: Unique type of isolated cardiac valvular amyloidosis. J Cardiothorac Surg; 2006;1:38
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  • [Title] Unique type of isolated cardiac valvular amyloidosis.
  • BACKGROUND: Amyloid deposition in heart is a common occurrence in systemic amyloidosis.
  • But localised valvular amyloid deposits are very uncommon.
  • It was only in 1922 that the cases of valvular amyloidosis were reported.
  • Then in 1980, Goffin et al reported another type of valvular amyloidosis, which he called the dystrophic valvular amyloidosis.
  • We report a case of aortic valve amyloidosis which is different from the yet described valvular amyloidosis.
  • Intraoperatively, a lesion was found attached to the inferior surface of his bicuspid aortic valve.Histopathology examination of the valve revealed that the lesion contained amyloid deposits, identified as AL amyloidosis.
  • The serum amyloid A protein (SAP) scan was normal and showed no evidence of systemic amyloidosis.
  • The ECG and echocardiogram were not consistent with cardiac amyloidosis.
  • CONCLUSION: Two major types of cardiac amyloidosis have been described in literature: primary-myelomatous type (occurs with systemic amyolidosis), and senile type(s).
  • Recently, a localised cardiac dystrophic valvular amyloidosis has been described.
  • In all previously reported cases, there was a strong association of localised valvular amyloidosis with calcific deposits.Ours is a unique case which differs from the previously reported cases of localised valvular amyloidosis.
  • This may well be a yet unknown type of isolated valvular amyloidosis.
  • [MeSH-major] Amyloidosis. Aortic Valve. Heart Valve Diseases

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  • (PMID = 17062163.001).
  • [ISSN] 1749-8090
  • [Journal-full-title] Journal of cardiothoracic surgery
  • [ISO-abbreviation] J Cardiothorac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1634846
  • [General-notes] NLM/ Original DateCompleted: 20070808
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80. Kawaji T, Ando Y, Hara R, Tanihara H: Novel therapy for transthyretin-related ocular amyloidosis: a pilot study of retinal laser photocoagulation. Ophthalmology; 2010 Mar;117(3):552-5
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  • [Title] Novel therapy for transthyretin-related ocular amyloidosis: a pilot study of retinal laser photocoagulation.
  • OBJECTIVE: The occurrence of ocular complications associated with transthyretin-related familial amyloidotic polyneuropathy increases with time, even after liver transplantation, which leads to a halt in the progression of systemic neurologic complications.
  • METHODS: We used panretinal laser photocoagulation, which damages the retinal pigment epithelium, the main location for synthesis of amyloidogenic transthyretin in ocular tissues, to treat 1 eye of each patient.
  • MAIN OUTCOME MEASURES: Fundus photography, visual acuity, and intraocular pressure.
  • RESULTS: Panretinal laser photocoagulation clearly prevented progression of amyloid deposition in the vitreous and on the retinal surface in both cases during 3 years of follow-up.
  • [MeSH-major] Amyloid Neuropathies, Familial / surgery. Amyloidosis / surgery. Laser Coagulation. Prealbumin / metabolism. Retinal Diseases / surgery. Vitreous Body / surgery

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  • [Copyright] Copyright 2010 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.
  • (PMID = 20045573.001).
  • [ISSN] 1549-4713
  • [Journal-full-title] Ophthalmology
  • [ISO-abbreviation] Ophthalmology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Prealbumin
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81. Arendt BK, Ramirez-Alvarado M, Sikkink LA, Keats JJ, Ahmann GJ, Dispenzieri A, Fonseca R, Ketterling RP, Knudson RA, Mulvihill EM, Tschumper RC, Wu X, Zeldenrust SR, Jelinek DF: Biologic and genetic characterization of the novel amyloidogenic lambda light chain-secreting human cell lines, ALMC-1 and ALMC-2. Blood; 2008 Sep 1;112(5):1931-41
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  • Primary systemic amyloidosis (AL) is a rare monoclonal plasma cell (PC) disorder characterized by the deposition of misfolded immunoglobulin (Ig) light chains (LC) in vital organs throughout the body.
  • Using a comprehensive genetic approach, we established the genetic relationship between the cell lines and the primary patient cells, and we were also able to identify new genetic changes accompanying tumor progression that may explain the natural history of this patient's disease.
  • Importantly, we demonstrate that free lambda LC secreted by both cell lines contained a beta structure and formed amyloid fibrils.
  • Despite absolute Ig LC variable gene sequence identity, the proteins show differences in amyloid formation kinetics that are abolished by the presence of Na(2)SO(4).
  • The formation of amyloid fibrils from these naturally secreting human LC cell lines is unprecedented.
  • Moreover, these cell lines will provide an invaluable tool to better understand AL, from the combined perspectives of amyloidogenic protein structure and amyloid formation, genetics, and cell biology.

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  • (PMID = 18567838.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA062242; United States / NIGMS NIH HHS / GM / R01 GM071514; United States / NCI NIH HHS / CA / CA062242; United States / NIGMS NIH HHS / GM / GM071514
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid; 0 / Cytokines; 0 / DNA Primers; 0 / Immunoglobulin lambda-Chains
  • [Other-IDs] NLM/ PMC2518895
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82. Hoyer RJ, Leung N, Witzig TE, Lacy MQ: Treatment of diuretic refractory pleural effusions with bevacizumab in four patients with primary systemic amyloidosis. Am J Hematol; 2007 May;82(5):409-13
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  • [Title] Treatment of diuretic refractory pleural effusions with bevacizumab in four patients with primary systemic amyloidosis.
  • Refractory pleural effusions present a challenging management problem and are associated with a poor prognosis in patients with primary systemic amyloidosis (AL).
  • [MeSH-major] Amyloidosis / complications. Antibodies, Monoclonal / therapeutic use. Pleural Effusion / drug therapy

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17326106.001).
  • [ISSN] 0361-8609
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Diuretics; 0 / Serum Albumin; 0 / Vascular Endothelial Growth Factor A; 27O7W4T232 / Spironolactone; 2S9ZZM9Q9V / Bevacizumab; 4Z8R6ORS6L / Thalidomide; 7LXU5N7ZO5 / Furosemide; 7S5I7G3JQL / Dexamethasone; 9G64RSX1XD / Captopril; 9PHQ9Y1OLM / Prednisolone; Q41OR9510P / Melphalan; TZ7V40X7VX / Metolazone
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83. Leung N, Slezak JM, Bergstralh EJ, Dispenzieri A, Lacy MQ, Wolf RC, Gertz MA: Acute renal insufficiency after high-dose melphalan in patients with primary systemic amyloidosis during stem cell transplantation. Am J Kidney Dis; 2005 Jan;45(1):102-11
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  • [Title] Acute renal insufficiency after high-dose melphalan in patients with primary systemic amyloidosis during stem cell transplantation.
  • BACKGROUND: Patients with primary systemic amyloidosis (AL) have a poor prognosis.
  • High-dose intravenous melphalan followed by peripheral blood stem cell transplant (PBSCT) appears to be the most promising therapy, but treatment mortality can be high.
  • Acute renal insufficiency (ARI) after high-dose melphalan was defined by a minimum increase of 0.5 mg/dL (44 micromol/L) in the serum creatinine level that is greater than 50% of baseline immediately after conditioning.
  • RESULTS: Of the 80 patients studied, ARI developed in 18.8% of the patients after high-dose melphalan.
  • Patients who had ARI after high-dose melphalan underwent dialysis more often (P = 0.007), and had a worse 1-year survival (P = 0.03).
  • [MeSH-major] Acute Kidney Injury / chemically induced. Amyloidosis / complications. Amyloidosis / drug therapy. Amyloidosis / therapy. Melphalan / administration & dosage. Melphalan / adverse effects. Stem Cell Transplantation / adverse effects

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  • (PMID = 15696449.001).
  • [ISSN] 1523-6838
  • [Journal-full-title] American journal of kidney diseases : the official journal of the National Kidney Foundation
  • [ISO-abbreviation] Am. J. Kidney Dis.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] Q41OR9510P / Melphalan
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84. Benson MD, Kincaid JC: The molecular biology and clinical features of amyloid neuropathy. Muscle Nerve; 2007 Oct;36(4):411-23
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  • [Title] The molecular biology and clinical features of amyloid neuropathy.
  • Neuropathy is often a major manifestation of systemic amyloidosis.
  • It is most frequently seen in patients with hereditary transthyretin (TTR) amyloidosis, but is also present in 20% of patients with systemic immunoglobulin light chain (primary) amyloidosis.
  • Familial amyloid polyneuropathy (FAP) is the most common form of inherited amyloidotic polyneuropathy, with clinical and electrophysiologic findings similar to neuropathies with differing etiologies (e.g., diabetes mellitus).
  • Hereditary amyloidosis is an adult-onset autosomal-dominant disease with varying degrees of penetrance.
  • It is caused by specific gene mutations, but demonstration that a patient has one such mutation does not confirm the diagnosis of amyloidosis.
  • Diagnosis requires tissue biopsy with demonstration of amyloid deposits either by special histochemical stains or electron microscopy.
  • Transthyretin amyloidosis is treated by liver transplantation, which eliminates the mutated transthyretin from the blood, but for some patients continued amyloid deposition can occur from wild-type (normal) transthyretin.
  • Presently, a study is ongoing to determine whether amyloid deposition can be inhibited by small organic molecules that are hypothesized to affect the fibril-forming ability of transthyretin.
  • [MeSH-major] Amyloid Neuropathies / genetics. Amyloid Neuropathies / pathology. Molecular Biology


85. Jesudason EP, Masilamoni JG, Kirubagaran R, Davis GD, Jayakumar R: The protective role of DL-alpha-lipoic acid in biogenic amines catabolism triggered by Abeta amyloid vaccination in mice. Brain Res Bull; 2005 Apr 30;65(4):361-7
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  • [Title] The protective role of DL-alpha-lipoic acid in biogenic amines catabolism triggered by Abeta amyloid vaccination in mice.
  • The major pathological consequence of Alzheimer disease (AD) is accumulation of beta-amyloid (Abeta) peptide fibrillar plaque in the brain and subsequent inflammatory reaction associated with the surrounding cells due to the presence of these aggregates.
  • For the first time, levels of principal neurotransmitters and their major metabolites in hippocampus and neocortex regions of brain are quantified to find out the level of inflammation.
  • We have shown a significant (p<0.05) reduction of 5-hydroxytryptamine (5-HT), dopamine (DA) and norepinephrine (NE) in the systemic inflammation induced (SI), vaccinated (VA) and inflammation induced vaccinated (IV) mice.
  • Interestingly, antioxidant LA treated mice with systemic inflammation (IL), vaccinated (VL) and inflammation induced vaccinated (IVL) mice exhibited enhanced level of 5-HT, DA and NE and the concentration of 5-HIAA and HVA gradually returned to normal.
  • [MeSH-major] Amyloid beta-Peptides / immunology. Antioxidants / therapeutic use. Biogenic Amines / metabolism. Inflammation / prevention & control. Peptide Fragments / immunology. Thioctic Acid / therapeutic use

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  • (PMID = 15811602.001).
  • [ISSN] 0361-9230
  • [Journal-full-title] Brain research bulletin
  • [ISO-abbreviation] Brain Res. Bull.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid beta-Peptides; 0 / Antioxidants; 0 / Biogenic Amines; 0 / Peptide Fragments; 0 / amyloid beta-protein (25-35); 73Y7P0K73Y / Thioctic Acid; 95IT3W8JZE / Silver Nitrate
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86. Sanchorawala V, Blanchard E, Seldin DC, O'Hara C, Skinner M, Wright DG: AL amyloidosis associated with B-cell lymphoproliferative disorders: frequency and treatment outcomes. Am J Hematol; 2006 Sep;81(9):692-5
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  • [Title] AL amyloidosis associated with B-cell lymphoproliferative disorders: frequency and treatment outcomes.
  • AL amyloidosis, a systemic disorder characterized by widespread deposition of amyloid fibrils derived from monoclonal Ig light chains in organs and soft tissues, is typically caused by an underlying plasma cell dyscrasia.
  • However, this disease can also be associated rarely with a B-cell lymphoproliferative disorder.
  • Although amyloid-related organ involvement in these patients was typical of AL amyloidosis, the patients in this series were on average older and more likely to be female than patients with disease associated with a plasma cell dyscrasia.
  • Treatment decisions were based primarily on the dominent hematopathologic features of the associated lymphoproliferative disorder.
  • However, high-dose melphalan and stem cell transplantation was the primary therapy in 5 patients, and each of these patients had prolonged survival, ranging from 36 to 102 months.
  • [MeSH-major] Amyloidosis. B-Lymphocytes / pathology. Bone Marrow Cells / pathology. Paraproteinemias
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Agents, Alkylating / therapeutic use. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Male. Melphalan / administration & dosage. Melphalan / therapeutic use. Middle Aged. Peripheral Blood Stem Cell Transplantation. Radiotherapy. Treatment Outcome

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  • [Copyright] (c) 2006 Wiley-Liss, Inc.
  • (PMID = 16795060.001).
  • [ISSN] 0361-8609
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Grant] United States / NHLBI NIH HHS / HL / HL68705
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; Q41OR9510P / Melphalan
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87. Jeannin G, Marson H, Merle P, Janicot H, Greil A, Caillaud D: [An unusual cause of chylothorax: primary amyloidosis]. Rev Mal Respir; 2008 May;25(5):601-4
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  • [Title] [An unusual cause of chylothorax: primary amyloidosis].
  • [Transliterated title] Une cause exceptionnelle de chylothorax: l'amylose primitive.
  • BACKGROUND: Amyloidosis is a large family of diseases defined by the presence of extra cellular protein deposits which can remain localised but are generally diffuse.
  • Echocardiography revealed a restrictive cardiomyopathy and suggested the diagnosis of a systemic disease.
  • Negative peripheral biopsies led us to perform an endomyocardial biopsy, which confirmed the diagnosis of amyloidosis AL.
  • CONCLUSION: We report an original case of primary amyloidosis presenting as a chylothorax and confirmed by an endomyocardial biopsy.
  • We highlight the multi factorial character of pleural effusions associated with amyloidosis.
  • This explains the delay in treatment and the disease's critical nature (median survival 2 months).
  • The prognostic value of proBNP is also emphasised.
  • [MeSH-major] Amyloidosis / complications. Chylothorax / etiology

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  • (PMID = 18535528.001).
  • [ISSN] 0761-8425
  • [Journal-full-title] Revue des maladies respiratoires
  • [ISO-abbreviation] Rev Mal Respir
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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88. Shimojima Y, Matsuda M, Gono T, Ishii W, Fushimi T, Hoshii Y, Yamada T, Ikeda S: Correlation between serum levels of free light chain and phenotype of plasma cells in bone marrow in primary AL amyloidosis. Amyloid; 2005 Mar;12(1):33-40
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  • [Title] Correlation between serum levels of free light chain and phenotype of plasma cells in bone marrow in primary AL amyloidosis.
  • To investigate whether there is a correlation between subtypes of plasma cells in the bone marrow and the production of M-protein, flow cytometry and serum free light chain (FLC) analyses were carried out in 17 patients with primary systemic AL amyloidosis (mean age, 59.9+/-8.8 years) and controls with M-protein (MGUS controls, n=6) and without it (negative controls, n=9).
  • The patients showed a significantly higher value in the serum predominant FLC:serum creatinine ratio (43.8+/-63.2) and CD38++ CD19- CD56+ subpopulation (monoclonal plasma cells) (2.57+/-5.35%) than either the negative (p<0.0005 and p<0.001, respectively) or MGUS controls (p<0.05).
  • In primary AL amyloidosis M-protein is probably produced by increased monoclonal plasma cells in the bone marrow, particularly by the intermediate subpopulation with a phenotype of MPC-1+ CD45- CD49e-.
  • [MeSH-major] Amyloidosis / blood. Antigens, CD / analysis. Bone Marrow Cells / pathology. Immunoglobulin Light Chains / blood. Plasma Cells / pathology

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  • (PMID = 16076609.001).
  • [ISSN] 1350-6129
  • [Journal-full-title] Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
  • [ISO-abbreviation] Amyloid
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Immunoglobulin Light Chains; 0 / Myeloma Proteins; 0 / multiple myeloma M-proteins
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89. Chciałowski A, Carewicz R, Zielińska-Krawczyk M: [Respiratory tract amyloidosis]. Pol Merkur Lekarski; 2006 Jan;20(115):109-11
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  • [Title] [Respiratory tract amyloidosis].
  • [Transliterated title] Skrobiawica układu oddechowego.
  • Amyloidosis is a group of biochemical disturbances, leading to extracellular deposition of misfolded protein fibril's.
  • It can be of primary, secondary or hereditary (familial) origin.
  • The disorder is known from 150 years, and as already 23 fibril precursor proteins have been identified.
  • Its symptoms can be systemic, localized; some forms don't produce any clinical manifestation.
  • In this article amyloidosis pathogenesis, classification, epidemiology, prognosis and clinical characteristics are described, mainly with reference to the respiratory system.
  • [MeSH-major] Amyloidosis / pathology. Amyloidosis / physiopathology. Respiratory Tract Diseases / pathology. Respiratory Tract Diseases / physiopathology

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  • (PMID = 16617749.001).
  • [ISSN] 1426-9686
  • [Journal-full-title] Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego
  • [ISO-abbreviation] Pol. Merkur. Lekarski
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Poland
  • [Number-of-references] 14
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90. Martin y Porras M, Vivario M, Delfosse V, Materne P, Warling X, Hoffer E: [Cardiac amyloidosis]. Rev Med Liege; 2009 Sep;64(9):434-9
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  • [Title] [Cardiac amyloidosis].
  • [Transliterated title] Le cas clinique du mois. Amyloïdose cardiaque de type AL: a propos d'un cas.
  • We present a case of a 54-year-old female presenting with renal failure and, two years later, heart failure, both due to primary systemic amyloidosis.
  • [MeSH-major] Amyloidosis / complications. Cardiomyopathy, Restrictive / etiology. Heart Failure / etiology

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  • (PMID = 19947312.001).
  • [ISSN] 0370-629X
  • [Journal-full-title] Revue médicale de Liège
  • [ISO-abbreviation] Rev Med Liege
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Belgium
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91. Chow LQ, Bahlis N, Russell J, Chaudhry A, Morris D, Brown C, Stewart DA: Autologous transplantation for primary systemic AL amyloidosis is feasible outside a major amyloidosis referral centre: the Calgary BMT Program experience. Bone Marrow Transplant; 2005 Oct;36(7):591-6
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  • [Title] Autologous transplantation for primary systemic AL amyloidosis is feasible outside a major amyloidosis referral centre: the Calgary BMT Program experience.
  • Recent reports from large amyloidosis referral centers suggest that primary systemic AL amyloidosis patients treated with high-dose melphalan (HDM) and autologous stem cell transplantation (ASCT) survive longer than historical controls treated with less intensive chemotherapy, despite high transplant-related mortality (TRM) rates of >10%.
  • A retrospective review was conducted to determine if the outcome of ASCT for AL amyloidosis at our institution was similar to that reported at major amyloidosis referral centers.
  • Over a 7 year period, we treated a total of 15 AL amyloidosis patients with ASCT, including four with poor prognosis cardiac or multisystem involvement.
  • In conclusion, ASCT for primary AL amyloidosis can safely be performed at experienced transplant centers that are not associated with major amyloidosis referral centers, and is feasible for patients who have multisystem involvement, particularly for motivated patients with good performance status.
  • [MeSH-major] Amyloidosis / therapy. Bone Marrow Transplantation / methods. Transplantation, Autologous / methods
  • [MeSH-minor] Adult. Aged. Amyloid / chemistry. Antigens, CD34 / biosynthesis. Antineoplastic Agents / pharmacology. Biopsy. Disease-Free Survival. Female. Hematopoietic Stem Cell Mobilization. Humans. Male. Middle Aged. Prognosis. Remission Induction. Retrospective Studies. Risk. Time Factors. Treatment Outcome


92. Prabhakaran VC, Babu K, Mahadevan A, Murthy SR: Amyloidosis of lacrimal gland. Indian J Ophthalmol; 2009 Nov-Dec;57(6):461-3
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  • [Title] Amyloidosis of lacrimal gland.
  • Primary localized amyloidosis of lacrimal gland is a rare occurrence.
  • This report describes a female patient with isolated amyloidosis of the lacrimal gland.
  • A lacrimal gland biopsy revealed amyloidosis.
  • No systemic involvement was detected on further investigation.
  • To our knowledge, this is the first report of lacrimal gland amyloidosis from India and our report also highlights the importance of lacrimal gland biopsy in diagnosing lacrimal gland masses.
  • [MeSH-major] Amyloidosis / diagnosis. Lacrimal Apparatus Diseases / diagnosis
  • [MeSH-minor] Biopsy. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Middle Aged. Tomography, X-Ray Computed

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  • [Cites] Ophthalmology. 2006 Sep;113(9):1657-64 [16828514.001]
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  • (PMID = 19861750.001).
  • [ISSN] 1998-3689
  • [Journal-full-title] Indian journal of ophthalmology
  • [ISO-abbreviation] Indian J Ophthalmol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC2812767
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93. Bhat A, Naguwa SM, Cheema GS, Gershwin ME: Colchicine revisited. Ann N Y Acad Sci; 2009 Sep;1173:766-73
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  • The drug has good oral bioavailability and some enterohepatic recirculation, requiring dose adjustments for kidney disease and avoidance in liver disease.
  • Federal Drug Administration for the treatment and prophylaxis of gout flares but has also been tried with varying success in the treatment of familial Mediterranean fever, primary biliary cirrhosis, psoriasis, Behçet's disease, aphthous stomatitis, linear IgA dermatosis, relapsing polychondritis, Sweet's syndrome, scleroderma, amyloidosis, leukocytoclastic vasculitis, epidermolysis bullosa, and dermatomyositis.
  • [MeSH-minor] Amyloidosis / drug therapy. Behcet Syndrome. Biological Availability. Colchicum / chemistry. Dermatomyositis / drug therapy. Epidermolysis Bullosa / drug therapy. Familial Mediterranean Fever / drug therapy. Humans. Intestinal Absorption. Liver Cirrhosis, Biliary / drug therapy. Molecular Structure. Polychondritis, Relapsing / drug therapy. Psoriasis / drug therapy. Scleroderma, Systemic / drug therapy. Stomatitis, Aphthous / drug therapy. Sweet Syndrome / drug therapy. Vasculitis, Leukocytoclastic, Cutaneous / drug therapy

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  • (PMID = 19758227.001).
  • [ISSN] 1749-6632
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Gout Suppressants; SML2Y3J35T / Colchicine
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94. Pietruszewska W, Kurnatowska I, Murlewska A, Zapała M, Wagrowska-Danilewicz M: [Localized amyloidosis of upper respiratory tract]. Otolaryngol Pol; 2007;61(5):730-5
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  • [Title] [Localized amyloidosis of upper respiratory tract].
  • [Transliterated title] Skrobiawica zlokalizowana w obrebie górnych dróg oddechowych.
  • Amyloidosis results from the deposition of amyloid proteins in organ and tissues.
  • It can be localized or systemic.
  • Amyloidosis may be classified as either primary or secondary.
  • Primary is idiopathic, whereas the secondary form is associated with chronic inflammatory or infectious process.
  • Amyloidosis is also related to myeloma multiplex (plasmacytoma) or located as tumor like deposits in isolated organ without systemic involvement.
  • Localized amyloidosis in the head and neck is rare and can have various manifestations.
  • A case of surgically treated localized amyloidosis of the pharynx is presented.
  • Current options on the structure of amyloid, making diagnosis, treatment and prognosis in patients with amyloidosis are presented.
  • [MeSH-major] Amyloidosis / pathology. Pharyngeal Diseases / pathology

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  • (PMID = 18552008.001).
  • [ISSN] 0030-6657
  • [Journal-full-title] Otolaryngologia polska = The Polish otolaryngology
  • [ISO-abbreviation] Otolaryngol Pol
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Poland
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95. Kimura S, Iwatsuka R, Aoki T, Odawara J, Asada N, Yamakura M, Takeuchi M, Matsue K: [Primary amyloidosis associated with IgD-lambda M-proteinemia]. Rinsho Ketsueki; 2007 Dec;48(12):1555-8
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  • [Title] [Primary amyloidosis associated with IgD-lambda M-proteinemia].
  • We describe here a case of primary AL amyloidosis associated with IgD monoclonal gammopathy of undetermined significance.
  • Systemic bone survey disclosed no lytic bone lesions.
  • Because the patient had macroglossia and multiple ecchymosis in the face and neck, primary amyloidosis was suspected.
  • Skin biopsy revealed extensive deposition of amyloid which was positively stained by Congo red dye.
  • A diagnosis of primary AL amyloidosis associated with IgD monoclonal gammopathy was made.
  • To our knowledge, this is the first report of primary AL amyloidosis associated with IgD monoclonal gammopathy with undetermined significance.
  • [MeSH-major] Amyloidosis / complications. Immunoglobulin D / blood. Immunoglobulin lambda-Chains / blood. Immunoglobulin mu-Chains / blood. Paraproteinemias / etiology

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  • (PMID = 18203516.001).
  • [ISSN] 0485-1439
  • [Journal-full-title] [Rinshō ketsueki] The Japanese journal of clinical hematology
  • [ISO-abbreviation] Rinsho Ketsueki
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Immunoglobulin D; 0 / Immunoglobulin lambda-Chains; 0 / Immunoglobulin mu-Chains
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96. Vázquez de la Iglesia F, Sánchez Ferrándis N, Rey Martínez J, Ruba San Miguel D, Rama López J, Fernández González S: [Amyloidosis in the ORL field]. Acta Otorrinolaringol Esp; 2006 Mar;57(3):145-8
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  • [Title] [Amyloidosis in the ORL field].
  • [Transliterated title] La amiloidosis en el area otorrinolaringológica.
  • OBJECTIVES: To review the clinical and pathological characteristics of upper aerodigestive tract amyloidosis with particular attention to laryngeal amyloidosis.
  • Amyloidosis of the upper aerodigestive tract is relatively rare.
  • The larynx is the most common site of involvement in head and neck isolated amyloidosis and the supraglottic region represents the major site of involvement.
  • MATERIAL AND METHODS: Retrospective review of 6 patients diagnosed with upper aerodigestive tract amyloidosis.
  • Hoarseness and airway compromise were the main presenting symptoms.
  • RESULTS: Laryngeal CO2 laser microsurgery was performed and then we refered the patients to the Medical Deparment seeking for systemic involvement and ENT Clinic follow up.
  • CONCLUSIONS: In our experience, laryngeal CO2 laser microsurgery is a succesfull way to treat isolated laryngeal amyloidosis with clinical improvement and low recurrence rates.
  • [MeSH-major] Amyloidosis. Otorhinolaryngologic Diseases

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  • (PMID = 16615568.001).
  • [ISSN] 0001-6519
  • [Journal-full-title] Acta otorrinolaringológica española
  • [ISO-abbreviation] Acta Otorrinolaringol Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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97. Lupi O, Peryassu MA: An emerging concept of prion infections as a form of transmissible cerebral amyloidosis. Prion; 2007 Oct-Dec;1(4):223-7
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  • [Title] An emerging concept of prion infections as a form of transmissible cerebral amyloidosis.
  • Besides the primary structure that is simply the sequence of amino acids that comprise a protein, the secondary structure represents the first step of folding defining its general conformation.
  • The amyloid diseases comprise Alzheimer's and Parkinson's diseases, type II diabetes mellitus and systemic amyloidosis.
  • Amyloid fibers are insoluble, resistant to proteolysis and show an extremely high content of beta-sheet, in a very similar structure to the one observed among prion rods, associated to the transmissible spongiform encephalopathies.
  • All these diseases are "infectious" in the sense that misfolded beta-sheeted conformers formed in a nucleation process in which preformed metastable oligomer acts as a seed to convert a normal isoform into an abnormal protein with a misfolded conformation.
  • Only prion infections have a proven infectivity in a microbiological sense; some recent observations, however, detected the transmissibility of systemic amyloidosis by a prion-like mechanism among mice.
  • Prions diseases and amyloidosis present many similar aspects of the so-called conformational diseases; according to this interpretation the prion infections could be considered as a form of transmissible cerebral amyloidosis.
  • [MeSH-major] Alzheimer Disease / metabolism. Amyloidosis / metabolism. Cerebellar Diseases / metabolism. Diabetes Mellitus, Type 2 / metabolism. Parkinson Disease / metabolism. Prions / metabolism


98. Wittich CM, Neben-Wittich MA, Mueller PS, Gertz MA, Edwards WD: Deposition of amyloid proteins in the epicardial coronary arteries of 58 patients with primary systemic amyloidosis. Cardiovasc Pathol; 2007 Mar-Apr;16(2):75-8
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  • [Title] Deposition of amyloid proteins in the epicardial coronary arteries of 58 patients with primary systemic amyloidosis.
  • INTRODUCTION: We sought to determine the distribution and the effect of amyloid on epicardial coronary arteries in patients with primary cardiac amyloidosis.
  • METHODS: We reviewed pathologic specimens taken after autopsy or cardiac transplantation from 58 patients with primary cardiac amyloidosis.
  • Multiple sections of epicardial coronary arteries (left anterior descending artery, left circumflex artery, and right coronary artery) were examined to determine the degree of amyloid deposition in the intima, media, adventitia, and vasa vasorum (vasa vasorum are nutrient arteries for the coronary arteries themselves).
  • RESULTS: In 56 of 58 patients (97%), amyloid was present in epicardial coronary arteries.
  • Amyloid was identified in all artery layers (intima, media, and adventitia), and more patients had amyloid in the adventitia.
  • However, amyloid did not cause intraluminal obstruction of epicardial coronary arteries in any patient.
  • The vasa vasorum had considerable deposits and, in many patients, were obstructed by amyloid.
  • Patients with obstruction of the vasa vasorum were significantly more likely to have obstructive intramural coronary amyloidosis than patients without vasa vasorum obstruction (P=.002).
  • CONCLUSIONS: The epicardial coronary arteries of patients with primary cardiac amyloidosis had extensive amyloid deposition.
  • Obstruction of the vasa vasorum was associated with obstructive intramural coronary amyloidosis.
  • [MeSH-major] Amyloid / metabolism. Amyloidosis / metabolism. Coronary Vessels / metabolism. Pericardium / metabolism

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  • (PMID = 17317539.001).
  • [ISSN] 1054-8807
  • [Journal-full-title] Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology
  • [ISO-abbreviation] Cardiovasc. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid
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99. Westermark P, Lundmark K, Westermark GT: Fibrils from designed non-amyloid-related synthetic peptides induce AA-amyloidosis during inflammation in an animal model. PLoS One; 2009;4(6):e6041
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  • [Title] Fibrils from designed non-amyloid-related synthetic peptides induce AA-amyloidosis during inflammation in an animal model.
  • BACKGROUND: Mouse AA-amyloidosis is a transmissible disease by a prion-like mechanism where amyloid fibrils act by seeding.
  • Synthetic peptides with no amyloid relationship can assemble into amyloid-like fibrils and these may have seeding capacity for amyloid proteins.
  • PRINCIPAL FINDINGS: Several synthetic peptides, designed for nanotechnology, have been examined for their ability to produce fibrils with Congo red affinity and concomitant green birefringence, affinity for thioflavin S and to accelerate AA-amyloidosis in mice.
  • It is shown that some amphiphilic fibril-forming peptides not only produced Congo red birefringence and showed affinity for thioflavin S, but they also shortened the lag phase for systemic AA-amyloidosis in mice when they were given intravenously at the time of inflammatory induction with silver nitride.
  • Peptides, not forming amyloid-like fibrils, did not have such properties.
  • [MeSH-major] Amyloid / chemistry. Amyloidosis / diagnosis. Amyloidosis / pathology
  • [MeSH-minor] Animals. Coloring Agents / pharmacology. Congo Red / pharmacology. Disease Models, Animal. Female. Humans. Inflammation. Mice. Peptides / chemistry. Spleen / metabolism. Thiazoles / chemistry. Time Factors

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  • (PMID = 19582162.001).
  • [ISSN] 1932-6203
  • [Journal-full-title] PloS one
  • [ISO-abbreviation] PLoS ONE
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid; 0 / Coloring Agents; 0 / Peptides; 0 / Thiazoles; 2390-54-7 / thioflavin T; 3U05FHG59S / Congo Red
  • [Other-IDs] NLM/ PMC2702095
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100. Arikan S, Sen I, Bahceci M, Tuzcu A, Ayli M: An interesting case of pachydermoperiostosis with idiopathic myelofibrosis associated with monosomy 22. Int J Dermatol; 2009 Aug;48(8):882-5
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  • Nail abnormalities: clues to systemic disease.
  • Amyloid deposition was not determined in rectal biopsy.
  • [MeSH-major] Chromosomes, Human, Pair 22. Monosomy. Osteoarthropathy, Primary Hypertrophic / genetics. Osteoarthropathy, Primary Hypertrophic / pathology. Primary Myelofibrosis / genetics. Primary Myelofibrosis / pathology

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  • (PMID = 19659869.001).
  • [ISSN] 1365-4632
  • [Journal-full-title] International journal of dermatology
  • [ISO-abbreviation] Int. J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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