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1. Lecuit M, Abachin E, Martin A, Poyart C, Pochart P, Suarez F, Bengoufa D, Feuillard J, Lavergne A, Gordon JI, Berche P, Guillevin L, Lortholary O: Immunoproliferative small intestinal disease associated with Campylobacter jejuni. N Engl J Med; 2004 Jan 15;350(3):239-48
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Immunoproliferative small intestinal disease associated with Campylobacter jejuni.
  • BACKGROUND: Immunoproliferative small intestinal disease (also known as alpha chain disease) is a form of lymphoma that arises in small intestinal mucosa-associated lymphoid tissue (MALT) and is associated with the expression of a monotypic truncated immunoglobulin alpha heavy chain without an associated light chain.
  • Early-stage disease responds to antibiotics, suggesting a bacterial origin.
  • METHODS: We performed polymerase chain reaction (PCR), DNA sequencing, fluorescence in situ hybridization, and immunohistochemical studies on intestinal-biopsy specimens from a series of patients with immunoproliferative small intestinal disease.
  • RESULTS: Analysis of frozen intestinal tissue obtained from an index patient with immunoproliferative small intestinal disease who had a dramatic response to antibiotics revealed the presence of Campylobacter jejuni.
  • A follow-up retrospective analysis of archival intestinal-biopsy specimens disclosed campylobacter species in four of six additional patients with immunoproliferative small intestinal disease.
  • CONCLUSIONS: These results indicate that campylobacter and immunoproliferative small intestinal disease are associated and that C. jejuni should be added to the growing list of human pathogens responsible for immunoproliferative states.
  • [MeSH-major] Campylobacter jejuni / isolation & purification. Immunoproliferative Small Intestinal Disease / microbiology
  • [MeSH-minor] Anti-Bacterial Agents. Anti-Ulcer Agents / therapeutic use. Campylobacter Infections. DNA, Bacterial / analysis. Drug Therapy, Combination / therapeutic use. Female. Genes, Immunoglobulin. Humans. Immunoglobulin A / blood. Immunoglobulin Fragments / analysis. Immunoglobulin Fragments / genetics. Immunohistochemistry. In Situ Hybridization, Fluorescence. Intestine, Small / immunology. Intestine, Small / microbiology. Intestine, Small / pathology. Middle Aged. Omeprazole / therapeutic use. Polymerase Chain Reaction. Sequence Analysis, DNA

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  • [Copyright] Copyright 2004 Massachusetts Medical Society
  • [CommentIn] Rev Gastroenterol Disord. 2005 Summer;5(3):181-2 [16156008.001]
  • [CommentIn] N Engl J Med. 2004 Apr 15;350(16):1685-6; author reply 1685-6 [15084705.001]
  • [CommentIn] N Engl J Med. 2004 Jan 15;350(3):213-5 [14724298.001]
  • (PMID = 14724303.001).
  • [ISSN] 1533-4406
  • [Journal-full-title] The New England journal of medicine
  • [ISO-abbreviation] N. Engl. J. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 0 / Anti-Ulcer Agents; 0 / DNA, Bacterial; 0 / Immunoglobulin A; 0 / Immunoglobulin Fragments; KG60484QX9 / Omeprazole
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2. Hohaus S, Teofili L, Balducci M, Manfrida S, Pompucci A, D'Alo' F, Massini G, Larocca LM, Marra R, Storti S: Combined Modality Treatment Including Methotrexate-Based Chemotherapy For Primary CENTRAL Nervous System Lymphoma: A Single Institution Experience. Mediterr J Hematol Infect Dis; 2009;1(2):e2009020

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Combined Modality Treatment Including Methotrexate-Based Chemotherapy For Primary CENTRAL Nervous System Lymphoma: A Single Institution Experience.
  • Chemotherapy including high-dose methotrexate (HD-MTX), with or without radiotherapy, is standard treatment for primary central nervous system lymphoma (PCNSL).
  • It remains controversial whether addition of other drugs will add to therapeutic efficacy.
  • We report here on 41 patients with PCNSL treated using a combined treatment modality, including HD-MTX (3.5 g/m(2) for 2 cycles) prior to whole brain radiotherapy (WBRT).
  • In 22 patients, the chemotherapy was intensified by adding high-dose cytosine arabinoside (HD-AraC) (2g/m(2) for 4 doses for 2 cycles).
  • Complete remission at the end of the combined treatment was obtained in 23 of 34 assessable patients (67%), and the predicted 5-year overall and disease-free survival rates were 24% and 46%, respectively, without differences between treatment groups.
  • Our study indicates that addition of HD-AraC may not improve clinical outcome in PCNSL, while it increases toxicity.
  • More targeted and less toxic therapies are warranted.

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  • (PMID = 21416006.001).
  • [ISSN] 2035-3006
  • [Journal-full-title] Mediterranean journal of hematology and infectious diseases
  • [ISO-abbreviation] Mediterr J Hematol Infect Dis
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC3033175
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3. Hara T, Tsurumi H, Kato T, Imao Y, Kojima Y, Kojima K, Kitagawa J, Katsumura N, Araki H, Takami T, Moriwaki H: Immunoproliferative small intestinal disease with protein loss complicated with duodenal T cell lymphoma during progression. Intern Med; 2008;47(4):299-303
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  • [Title] Immunoproliferative small intestinal disease with protein loss complicated with duodenal T cell lymphoma during progression.
  • A 52-year-old man was admitted to our hospital in October 2001 with abdominal pain.
  • Abdominal X-ray indicated a diagnosis of ileus.
  • Histopathological and immunological examination resulted in a diagnosis of immunoproliferative small intestinal disease (IPSID).
  • He was treated with THP-COP therapy (pirarubicin, cyclophosphamide, vincristine, and prednisolone), which resulted in complete remission.
  • He was diagnosed with relapsed IPSID and salvage chemotherapy was started.
  • Follow-up endoscopy confirmed that the therapy was effective, but uncovered another duodenal mucosal nodularity.
  • Immunohistochemical staining revealed T-cell lymphoma.
  • Chemotherapy was discontinued and the patient died in December 2004.
  • [MeSH-major] Duodenal Neoplasms / etiology. Immunoproliferative Small Intestinal Disease / complications. Lymphoma, T-Cell / etiology
  • [MeSH-minor] Disease Progression. Fatal Outcome. Humans. Male. Middle Aged. Proteins / metabolism

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  • (PMID = 18277034.001).
  • [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
  • [Chemical-registry-number] 0 / Proteins
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4. Stavrou E, Lazarus HM: Thrombotic microangiopathy in haematopoietic cell transplantation: an update. Mediterr J Hematol Infect Dis; 2010;2(3):e2010033

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Allogeneic hematopoietic cell transplantation (HCT) represents a vital procedure for patients with various hematologic conditions.
  • Pathophysiologic mechanisms associated with TA-TMA, include loss of endothelial cell integrity induced by intensive conditioning regimens, immunosuppressive therapy, irradiation, infections and graft-versus-host (GVHD) disease.
  • The reported incidence of TA-TMA ranges from 0.5% to 75%, reflecting the difficulty of accurate diagnosis in these patients.
  • Two different groups have proposed consensus definitions for TA-TMA, yet they fail to distinguish the primary syndrome from secondary causes such as infections or medication exposure.
  • Despite treatment, mortality rate in TA-TMA ranges between 60% to 90%.
  • The treatment strategies for TA-TMA remain challenging.
  • Daclizumab, a humanized monoclonal anti-CD25 antibody, has shown promising results in the treatment of TA-TMA.

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  • (PMID = 21776339.001).
  • [ISSN] 2035-3006
  • [Journal-full-title] Mediterranean journal of hematology and infectious diseases
  • [ISO-abbreviation] Mediterr J Hematol Infect Dis
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC3134219
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5. Ghoshal UC, Chetri K, Banerjee PK, Choudhuri G, Pal BB, Dabadghao S, Dhar K, Naik S, Naik SR: Is immunoproliferative small intestinal disease uncommon in India? Trop Gastroenterol; 2001 Jan-Mar;22(1):14-7
Hazardous Substances Data Bank. TETRACYCLINE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Is immunoproliferative small intestinal disease uncommon in India?
  • Till date only three series of immunoproliferative small intestinal disease (IPSID) describing 22 patients have been reported from India.
  • Seven patients with IPSID in two tertiary referral centers in India are included in the study.
  • Diagnosis was based on typical clinical features [diarrhoea (7/7), weight loss (7/7), clubbing (6/7), fever (3/7), abdominal pain and lump (3/7)], biochemical evidence of malabsorption and duodenal biopsy findings.
  • Atypical features included gastric involvement (1/7), colonic involvement (1/7) and appearance of pigmented nails following anti-cancer chemotherapy (1/7) which disappeared six months after omitting doxorubin from chemotherapy regimen.
  • In the latter patient S. stercoralis became disseminated after anti-malignant chemotherapy.
  • This raises doubt on efficacy of tetracycline alone in treatment of IPSID.
  • One other patient was misdiagnosed and treated as intestinal tuberculosis.
  • Early diagnosis and administration of chemotherapy may improve survival in this disease.
  • [MeSH-major] Immunoproliferative Small Intestinal Disease / epidemiology
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cyclophosphamide / therapeutic use. Doxorubicin / therapeutic use. Humans. India / epidemiology. Middle Aged. Prednisolone / therapeutic use. Prognosis. Tetracycline / therapeutic use. Vincristine / therapeutic use

  • Hazardous Substances Data Bank. DOXORUBICIN .
  • Hazardous Substances Data Bank. CYCLOPHOSPHAMIDE .
  • Hazardous Substances Data Bank. PREDNISOLONE .
  • Hazardous Substances Data Bank. VINCRISTINE .
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  • (PMID = 11398237.001).
  • [ISSN] 0250-636X
  • [Journal-full-title] Tropical gastroenterology : official journal of the Digestive Diseases Foundation
  • [ISO-abbreviation] Trop Gastroenterol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; 9PHQ9Y1OLM / Prednisolone; F8VB5M810T / Tetracycline; VAP-cyclo protocol
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6. Pagano L, Caira M, Valentini CG, Fianchi L: Clinical aspects and therapy of sporadic burkitt lymphoma. Mediterr J Hematol Infect Dis; 2009;1(2):e2009030

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical aspects and therapy of sporadic burkitt lymphoma.
  • Burkitt's lymphoma is a highly aggressive mature B-cell neoplasm consisting of endemic, sporadic, and immunodeficiency-associated variants, sharing many morphologic and immunophenotypic features.
  • Brief-duration, high-intensity chemotherapy regimens including aggressive central nervous system prophylaxis have had remarkable success in the treatment of this disease in the sporadic form, with very high complete remission rate and overall survival in adults.
  • Although Burkitt's lymphoma is extremely chemosensitive, biologically targeted therapies should be developed, because current treatment options are suboptimal for patients with poor prognostic features or with relapsed disease.

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  • (PMID = 21416007.001).
  • [ISSN] 2035-3006
  • [Journal-full-title] Mediterranean journal of hematology and infectious diseases
  • [ISO-abbreviation] Mediterr J Hematol Infect Dis
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC3033171
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7. Landolsi A, Chabchoub I, Limem S, Gharbi O, Chaafai R, Hochlef M, Fatma LB, Trimech M, Krifa A, Ajmi S, Mokni M, Hadj Hmida MB, Ahmed SB: [Primary digestive tract lymphoma in central region of Tunisia: anatomoclinical study and therapeutic results about 153 cases]. Bull Cancer; 2010 Apr;97(4):435-43

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Primary digestive tract lymphoma in central region of Tunisia: anatomoclinical study and therapeutic results about 153 cases].
  • [Transliterated title] Les lymphomes primitifs du tube digestif (LPTD) dans le centre tunisien: étude anatomoclinique et résultats thérapeutiques à propos de 153 cas.
  • Primary gastro-intestinal lymphoma (PGIL) is the most common type of extra-nodal non Hodgkin's lymphoma.
  • Their clinical and histological presentations are heterogeneous depending on the site of the lesion.
  • There is no consensus regarding the role of surgery and chemotherapy in the therapeutic approach.
  • In our country epidemiology of the disease is unknown with IPSID being the most frequent type.
  • We report anatomo-clinical features and prognostic factors of PGIL and compare intestinal to gastric forms in our region.
  • Tumor sites were gastric (67%), intestinal (26%) and gastrointestinal (7%).
  • Abdominal pain (87%) followed by vomiting and diarrhoea (37 and 15%) were the most common symptoms.
  • Performance status (PS) < 2 was seen in 80% of patients, high grade lymphoma in 70.5% of cases and B phenotype was noted in 85%.
  • MALT lymphoma accounts for 50% of cases, and IPSID for only 5% of PGIL.
  • About 47.5% of cases were stage IE, 138 patients had chemotherapy with an objective response rate of 77%.
  • Only 46% of patients had surgery (14 for surgical complication, 6 for residual tumor after chemotherapy and 22 to have histological diagnosis).
  • In high grade lymphoma patients favorable prognostic factors for OS included young age < or = 60 years, PS < 2, normal serum LDH, hemoglobin > 12 g/dL, B phenotype, localised stage (IE-IIE1), anthracycline-based chemotherapy regimen, achieving complete or partial response to induction chemotherapy and no relapse.
  • In low-grade lymphoma patients, none of these factors had a significant correlation with OS: age < or = 60 years, PS < 2, stage (IE-IIE1), response to induction chemotherapy, relapse.
  • Compared to gastric lymphomas, intestinal cases occurred at a younger age, frequently with diarrhoea, weight loss, and occlusion.
  • We conclude that stomach is the main site of PGIL in our region, intestinal lymphoma is less frequent and IPSID has become rare.
  • Recent progress in chemotherapy has allowed good therapeutic results with a conservative approach.
  • Surgery may be performed in case of emergency or for residual lesions after medical treatment.
  • [MeSH-major] Gastrointestinal Neoplasms. Lymphoma, Non-Hodgkin
  • [MeSH-minor] Abdominal Pain / etiology. Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Diarrhea / etiology. Female. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Tunisia. Vomiting / etiology. Young Adult

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  • (PMID = 20395189.001).
  • [ISSN] 1769-6917
  • [Journal-full-title] Bulletin du cancer
  • [ISO-abbreviation] Bull Cancer
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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8. Bibas M, Antinori A: EBV and HIV-Related Lymphoma. Mediterr J Hematol Infect Dis; 2009;1(2):e2009032

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] EBV and HIV-Related Lymphoma.
  • HIV-associated lymphoproliferative disorders represent a heterogeneous group of diseases, arising in the presence of HIV-associated immunodeficiency.
  • The overall prevalence of HIV-associated lymphoma is significantly higher compared to that of the general population and it continues to be relevant even after the wide availability of highly active antiretroviral therapy (HAART) (1).
  • It has been largely implicated in the development of B-cell lymphoproliferative disorders as Burkitt lymphoma (BL), Hodgkin disease (HD), systemic non Hodgkin lymphoma (NHL), primary central nervous system lymphoma (PCNSL), nasopharyngeal carcinoma (NC).
  • Virus-associated lymphomas are becoming of significant concern for the mortality of long-lived HIV immunocompromised patients, and therefore, research of advanced strategies for AIDS-related lymphomas is an important field in cancer chemotherapy.
  • Detailed understanding of the EBV lifecycle and related cancers at the molecular level is required for novel strategies of molecular-targeted cancer chemotherapy The linkage of HIV-related lymphoma with EBV infection of the tumor clone has several pathogenetic, prognostic and possibly therapeutic implications which are reviewed herein.

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  • (PMID = 21416008.001).
  • [ISSN] 2035-3006
  • [Journal-full-title] Mediterranean journal of hematology and infectious diseases
  • [ISO-abbreviation] Mediterr J Hematol Infect Dis
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC3033170
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9. Dutta U, Udawat H, Noor MT, Sidhu GS, Kochhar R, Vaiphei K, Singh K: Regression of immunoproliferative small intestinal disease after eradication of Helicobacter pylori. J Gastrointest Cancer; 2010 Sep;41(3):212-5
Hazardous Substances Data Bank. Clarithromycin .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Regression of immunoproliferative small intestinal disease after eradication of Helicobacter pylori.
  • A 20-year-old male presented with low-grade fever, abdominal pain, anorexia, and weight loss of 4-month duration.
  • Contrast-enhanced computed tomography of the abdomen revealed extensive proximal small-bowel thickening with mesenteric lymphadenopathy.
  • Upper gastrointestinal endoscopy and enteroscopy revealed thickening of folds with multiple small superficial ulceration involving antrum, duodenum, and jejunum.
  • The duodenal and jejunal biopsy was suggestive of immunoproliferative small intestinal disease, stage 0 (Salem) or stage A (Galian).
  • He underwent H. pylori eradication following which he had significant clinical improvement; repeat evaluation at 6 months showed dramatic improvement in his clinical, radiological, and histological parameters.
  • [MeSH-major] Anti-Bacterial Agents / therapeutic use. Helicobacter Infections / complications. Immunoproliferative Small Intestinal Disease / drug therapy. Immunoproliferative Small Intestinal Disease / microbiology
  • [MeSH-minor] 2-Pyridinylmethylsulfinylbenzimidazoles / therapeutic use. Amoxicillin / therapeutic use. Clarithromycin / therapeutic use. Doxycycline / therapeutic use. Drug Therapy, Combination. Helicobacter pylori. Humans. Lansoprazole. Male. Omeprazole / therapeutic use. Organometallic Compounds / therapeutic use. Tinidazole / therapeutic use. Young Adult

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  • (PMID = 20300878.001).
  • [ISSN] 1941-6636
  • [Journal-full-title] Journal of gastrointestinal cancer
  • [ISO-abbreviation] J Gastrointest Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 2-Pyridinylmethylsulfinylbenzimidazoles; 0 / Anti-Bacterial Agents; 0 / Organometallic Compounds; 033KF7V46H / Tinidazole; 0K5C5T2QPG / Lansoprazole; 804826J2HU / Amoxicillin; H1250JIK0A / Clarithromycin; HS813P8QPX / bismuth tripotassium dicitrate; KG60484QX9 / Omeprazole; N12000U13O / Doxycycline
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10. Pai RK, Snider WK, Starkey CR, Viswanatha D, Foucar MK, Wilson CS: Nonsecretory variant of immunoproliferative small intestinal disease: a case report with pathologic, immunophenotypic, and molecular findings. Arch Pathol Lab Med; 2005 Nov;129(11):1487-90
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  • [Title] Nonsecretory variant of immunoproliferative small intestinal disease: a case report with pathologic, immunophenotypic, and molecular findings.
  • We report a case of the nonsecretory variant of immunoproliferative small intestinal disease involving the distal small bowel and the mesenteric and retroperitoneal lymph nodes in a 19-year-old woman from Mexico.
  • This variant extranodal marginal zone B-cell lymphoma appeared similar in the different sites of involvement, with more interspersed large cells and greater plasmacytic differentiation present in intestinal specimens.
  • Characteristic lymphoepithelial lesions and follicular colonization were seen in intestinal and lymph node sections, respectively.
  • The neoplastic B cells were cytoplasmic immunoglobulin (Ig) A heavy-chain restricted and lacked surface and cytoplasmic light-chain expression by flow cytometric analysis.
  • Molecular studies showed absence of immunoglobulin heavy-chain (IgH) gene rearrangement, with a nonfunctional clonotypic rearrangement of the kappa light-chain gene.
  • This case highlights the role for kappa light-chain gene evaluation in immunoproliferative small intestinal disease, because IgH gene rearrangement analysis is often negative.
  • [MeSH-major] Immunoproliferative Small Intestinal Disease / pathology. Lymph Nodes / pathology. Lymphoma, B-Cell, Marginal Zone / pathology
  • [MeSH-minor] 2-Pyridinylmethylsulfinylbenzimidazoles. Adult. Amoxicillin / therapeutic use. Anti-Bacterial Agents / therapeutic use. Benzimidazoles / therapeutic use. Drug Therapy, Combination. Female. Gene Rearrangement, B-Lymphocyte, Light Chain / genetics. Humans. Immunophenotyping. Intestine, Small / pathology. Mesentery. Metronidazole / therapeutic use. Omeprazole / analogs & derivatives. Omeprazole / therapeutic use. Retroperitoneal Space. Sulfoxides / therapeutic use

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  • (PMID = 16253033.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 2-Pyridinylmethylsulfinylbenzimidazoles; 0 / Anti-Bacterial Agents; 0 / Benzimidazoles; 0 / Sulfoxides; 140QMO216E / Metronidazole; 804826J2HU / Amoxicillin; D8TST4O562 / pantoprazole; KG60484QX9 / Omeprazole
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11. Ramaiah SK, Seguin MA, Carwile HF, Raskin RE: Biclonal gammopathy associated with immunoglobulin A in a dog with multiple myeloma. Vet Clin Pathol; 2002;31(2):83-9
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  • Multiple myeloma was diagnosed based on bone marrow plasmacytosis, multiple lytic bone lesions, and hyperglobulinemia with a clonal gammopathy on serum protein electrophoresis.
  • Temporary responses to 2 different chemotherapy protocols (melphalan and prednisone, and cyclophosphamide and prednisone) were seen, with remission of clinical signs and a decrease in the biclonal gammopathy but no resolution of the splenic mass.
  • Eventual return of clinical signs led to euthanasia at 175 days postdiagnosis.
  • An immunoglobulin-A (IgA) gammopathy was demonstrated by immunoelectrophoresis; biclonality was ascertained by immunofixation electrophoresis.
  • The clonal components consisted of intact Ig with heavy chain of the alpha class and light chain of an undetermined class.
  • To our knowledge, this is the first report of undimerized biclonal gammopathy in a dog caused by a single heavy chain class involving IgA.

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  • (PMID = 12040490.001).
  • [ISSN] 0275-6382
  • [Journal-full-title] Veterinary clinical pathology
  • [ISO-abbreviation] Vet Clin Pathol
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin A; 0 / Immunoglobulin Heavy Chains
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12. Economidou I, Manousos ON, Triantafillidis JK, Vaslamatzis MM, Zafiropoulou R, Papadakis T: Immunoproliferative small intestinal disease in Greece: presentation of 13 cases including two from Albania. Eur J Gastroenterol Hepatol; 2006 Sep;18(9):1029-38
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  • [Title] Immunoproliferative small intestinal disease in Greece: presentation of 13 cases including two from Albania.
  • OBJECTIVES: Immunoproliferative small intestinal disease (IPSID) represents a spectrum of clinicopathological entities including alpha-chain disease and other types of lymphoplasmacytic proliferations of the lamina propria of the small intestine, presenting with severe malabsorption.
  • IPSID has been described mainly in the Mediterranean, Middle East, and African countries.
  • METHODS: Current immunological and immunohistochemical methods for the detection of alpha heavy chains and the presence of clonality have been used to study 13 cases of IPSID diagnosed in Greece, two of whom were Albanian residents.
  • RESULTS: The patients were categorized in three subgroups of IPSID: alpha-chain disease (n=8), non-alpha chain disease with other monoclonal immunoglobulins (n=3), and polyclonal 'non-malignant' IPSID (n=2).
  • In several patients the disease had unusual features, and this in some cases delayed the diagnosis.
  • Patients with stage C disease had a short survival, whereas two patients with stage A alpha-chain disease responded to treatment with cyclophosphamide, vincristine and prednisolone, and cyclophosphamide, doxorubicine, vincristine and prednisolone, respectively, have a disease-free long survival of 35 and 12 years, and appear to be cured.
  • [MeSH-major] Immunoproliferative Small Intestinal Disease / diagnosis
  • [MeSH-minor] Adult. Albania. Antibiotics, Antineoplastic / therapeutic use. Antineoplastic Agents / therapeutic use. Cyclophosphamide / therapeutic use. Doxorubicin / therapeutic use. Drug Therapy, Combination. Female. Greece. Humans. Male. Middle Aged. Prednisolone / therapeutic use. Treatment Outcome. Vincristine / therapeutic use

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  • (PMID = 16894320.001).
  • [ISSN] 0954-691X
  • [Journal-full-title] European journal of gastroenterology & hepatology
  • [ISO-abbreviation] Eur J Gastroenterol Hepatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antineoplastic Agents; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; 9PHQ9Y1OLM / Prednisolone
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13. Dokmanovic L, Urosevic J, Janic D, Jovanovic N, Petrucev B, Tosic N, Pavlovic S: Analysis of thiopurine S-methyltransferase polymorphism in the population of Serbia and Montenegro and mercaptopurine therapy tolerance in childhood acute lymphoblastic leukemia. Ther Drug Monit; 2006 Dec;28(6):800-6
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  • [Title] Analysis of thiopurine S-methyltransferase polymorphism in the population of Serbia and Montenegro and mercaptopurine therapy tolerance in childhood acute lymphoblastic leukemia.
  • Thiopurine S-methyltransferase (TPMT) is an enzyme that converts thiopurine drugs into inactive metabolites.
  • The aim of this study was to determine the frequency and type of TPMT polymorphisms in the population of Serbia and Montenegro and to assess its relevance in the management of childhood acute lymphoblastic leukemia (ALL).
  • Blood samples from 100 healthy adults and 100 children with ALL were analyzed for common mutations in the TPMT gene using polymerase chain reaction-based assays.
  • The general pattern of TPMT-variant allele distribution as well as their frequencies in the population of Serbia and Montenegro, is similar to those determined for other Slavic and Mediterranean populations.
  • The ability to tolerate 6-mercaptopurine (6-MP) -based maintenance therapy was used as a surrogate marker of hematologic toxicity.
  • In the study of 50 patients with childhood ALL treated according to the BFM-like protocol, it was found that even TPMT-heterozygous patients are at greater risk of thiopurine drug-related leukopenia (mean duration of period when children missed therapy as a result of leukopenia for TPMT-heterozygous patients was 11.3 weeks vs 3.4 weeks for wild-type genotype patients, P < 0.01).
  • The therapy protocol was modified considering their TPMT genotype.
  • Administering reduced 6-MP dosages in the initial phase of maintenance allowed TPMT-heterozygous patients to later receive full protocol doses of both 6-MP and nonthiopurine therapy without omitting therapy resulting from myelotoxicity.
  • These results justify performing TPMT genotyping before initiating thiopurine therapy in all children with ALL to minimize consequent toxicity.
  • [MeSH-major] 6-Mercaptopurine / therapeutic use. Methyltransferases / genetics. Polymorphism, Genetic. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy. Precursor Cell Lymphoblastic Leukemia-Lymphoma / enzymology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics
  • [MeSH-minor] Child. Drug Tolerance. Humans. Male. Retrospective Studies. Yugoslavia

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  • (PMID = 17164697.001).
  • [ISSN] 0163-4356
  • [Journal-full-title] Therapeutic drug monitoring
  • [ISO-abbreviation] Ther Drug Monit
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] E7WED276I5 / 6-Mercaptopurine; EC 2.1.1.- / Methyltransferases; EC 2.1.1.67 / thiopurine methyltransferase
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14. Ben Ammar A, Cheikh I, Jouini M, Belkahla N, Fadhel SF, Hager O, Maamouri N, Chaabouni H, Ben Safta Z, Haouet S: [Alpha heavy chain disease. A Tunisian case]. Tunis Med; 2006 Sep;84(9):581-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Alpha heavy chain disease. A Tunisian case].
  • [Transliterated title] Maladie des chaines lourdes alpha. A propos d'un cas tunisien.
  • Alpha heavy chain disease is a rare affection in the West and reported mainly in developing countries with the improvement of hygienic conditions, the disease become rare in Tunisia, the last case was reported in 1991.
  • The aim of the study is to report a new Tunisian case and to describe clinical, endoscopical and histological characteristics of the disease.
  • The diagnosis of alpha heavy chain disease was confirmed by histological examination of the resected intestine after surgery for intestinal obstruction.
  • The patient received chemotherapy.
  • [MeSH-major] Immunoproliferative Small Intestinal Disease / diagnosis
  • [MeSH-minor] Adult. Humans. Intestinal Obstruction / etiology. Intestinal Obstruction / surgery. Male

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  • (PMID = 17263208.001).
  • [ISSN] 0041-4131
  • [Journal-full-title] La Tunisie médicale
  • [ISO-abbreviation] Tunis Med
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Tunisia
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15. Ramadeen A, Laurent G, dos Santos CC, Hu X, Connelly KA, Holub BJ, Mangat I, Dorian P: n-3 Polyunsaturated fatty acids alter expression of fibrotic and hypertrophic genes in a dog model of atrial cardiomyopathy. Heart Rhythm; 2010 Apr;7(4):520-8
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  • OBJECTIVE: The purpose of this study was to use a genome-wide approach to identify gene expression profiles involved in a new model of AF vulnerability and to determine whether they were altered by PUFA therapy.
  • Atrial tissue was sampled at the end of the protocol.
  • Results were confirmed with quantitative real-time polymerase chain reaction (RT-PCR) and histology on all 36 dogs.
  • RESULTS: Microarray or quantitative RT-PCR results showed that SAVP-No PUFAs dogs had significantly increased mRNA levels of protein kinase B (Akt), epidermal growth factor (EGF), JAM3, myosin heavy chain alpha (MHCalpha), and CD99 and significantly decreased levels of Smad6 compared with CTRL dogs.
  • [MeSH-major] Atrial Fibrillation / prevention & control. Atrial Function / drug effects. Cardiomyopathies / genetics. Cardiovascular Agents / pharmacology. Fatty Acids, Omega-3 / pharmacology. Heart Atria / drug effects
  • [MeSH-minor] Animals. Disease Models, Animal. Dogs. Fibrosis / genetics. Gene Expression / drug effects. Hypertrophy / genetics. Stress, Mechanical


16. Witzig TE, Wahner-Roedler DL: Heavy chain disease. Curr Treat Options Oncol; 2002 Jun;3(3):247-54
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  • [Title] Heavy chain disease.
  • The heavy chain diseases (HCDs) are rare B-cell malignancies that are distinguished by the production of a monoclonal immunoglobulin heavy chain (HC) without an associated light chain by the malignant B-cells.
  • There are three types of HCD defined by the class of immunoglobulin (Ig) HC produced: IgA (alpha-HCD), IgG (gamma-HCD), and IgM (mu-HCD).
  • Alpha-HCD is the most common and occurs most commonly as intestinal malabsorption in a young adult from a country bordering the Mediterranean Sea.
  • Treatment consists of antibiotics and improved nutrition and hygiene.
  • If there is no response to antibiotics or if aggressive non-Hodgkin's lymphoma (NHL) is diagnosed, the patient should be treated with chemotherapy.
  • Gamma- and mu-HCD are rare and essentially are found in patients with a B-cell NHL that produces an abnormal Ig heavy chain.
  • These patients occasionally may be diagnosed with a monoclonal gammopathy of undetermined significance (MGUS).
  • Patients with MGUS with NHL should be administered chemotherapy.
  • Screening the serum and urine of patients with lymphoplasmacytoid NHL would likely identify more patients with gamma- or mu-HCD.
  • [MeSH-major] Heavy Chain Disease / therapy
  • [MeSH-minor] Adult. Aged. Gene Rearrangement, B-Lymphocyte, Heavy Chain / genetics. Humans. Immunoglobulin Heavy Chains / genetics. Incidence. Middle Aged. Radiotherapy

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  • (PMID = 12057070.001).
  • [ISSN] 1527-2729
  • [Journal-full-title] Current treatment options in oncology
  • [ISO-abbreviation] Curr Treat Options Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin Heavy Chains
  • [Number-of-references] 36
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17. Siddiqi T, Joyce RM: A case of HIV-negative primary effusion lymphoma treated with bortezomib, pegylated liposomal doxorubicin, and rituximab. Clin Lymphoma Myeloma; 2008 Oct;8(5):300-4
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  • [Title] A case of HIV-negative primary effusion lymphoma treated with bortezomib, pegylated liposomal doxorubicin, and rituximab.
  • Primary effusion lymphoma (PEL) is a rare B-cell non-Hodgkin lymphoma that usually occurs in HIV-positive patients and has a very poor prognosis with limited treatment options.
  • Prospective studies are lacking to define the standard of care for this disease, and various case series report median survival at 6 months with anthracycline-based chemotherapy.
  • Reports of antiviral agents, immune modulators, and some targeted therapies are present in the literature with variable results.
  • Herein, we report a case of an elderly HIV-negative man of Mediterranean origin who was diagnosed with primary effusion lymphoma and responded dramatically to 6 cycles of a combination of bortezomib, pegylated liposomal doxorubicin, and rituximab.
  • He has since been maintained on rituximab and remains in complete remission 2 years after diagnosis.
  • In this report, we discuss the rationale for using these agents in this patient and advocate the further study of bortezomib-based therapy in PEL.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Lymphoma, Primary Effusion / diagnosis
  • [MeSH-minor] Aged. Antibodies, Monoclonal / administration & dosage. Antibodies, Monoclonal, Murine-Derived. Boronic Acids / administration & dosage. Bortezomib. Doxorubicin / administration & dosage. HIV. Herpesviridae Infections / physiopathology. Herpesvirus 8, Human. Humans. Immunohistochemistry. Liposomes. Male. Positron-Emission Tomography. Pyrazines / administration & dosage. Rituximab


18. Motta G, Conticello C, Amato G, Moschetti G, Colarossi C, Cosentino S, Ippolito M, Giustolisi R, Di Raimondo F: Pleuric presentation of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue: a case report and a review of the literature. Int J Hematol; 2010 Sep;92(2):369-73
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  • [Title] Pleuric presentation of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue: a case report and a review of the literature.
  • A primary pleural marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) is a very rare eventuality.
  • Here, we report a rare case of MALT lymphoma arising in the pleura and update the literature on this topic.
  • Video-assisted thoracoscopic exploration and a talc pleurodesis were performed and microscopic and immunohistochemical findings showed that the tumor was a pleural MALT lymphoma.
  • To the best of our knowledge, only seven cases of primary pleural MALT lymphoma have been documented until recently, mostly from Japan with a mean age for all patients of 60.5 years.
  • The pathogenesis of MALT lymphomas remains unclear, although a possible chronic antigenic stimulation by microbial pathogens and/or autoantigens has been hypothesized.
  • Surgical resection was performed in most cases, and some patients received postoperative chemotherapy or immunotherapy.
  • The clinicopathologic characteristics and management of this extremely rare disease are also discussed.
  • [MeSH-major] Lymphoma, B-Cell, Marginal Zone / diagnosis. Pleural Effusion / diagnosis
  • [MeSH-minor] Aged. Antibodies, Monoclonal, Murine-Derived / therapeutic use. Antineoplastic Agents. Female. Humans. Pleura / pathology. Remission Induction. Rituximab

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  • (PMID = 20725816.001).
  • [ISSN] 1865-3774
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antineoplastic Agents; 4F4X42SYQ6 / Rituximab
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19. Fakhar M, Asgari Q, Motazedian MH, Monabati A: Mediterranean visceral leishmaniasis associated with acute lymphoblastic leukemia (ALL). Parasitol Res; 2008 Jul;103(2):473-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mediterranean visceral leishmaniasis associated with acute lymphoblastic leukemia (ALL).
  • We report a case of Mediterranean visceral leishmaniasis (MVL) associated with acute lymphoblastic leukemia (ALL) from the South of Iran.
  • Moreover, by specific polymerase chain reaction (PCR) on peripheral blood, a 145 bp band corresponding to kDNA from the genus Leishmania was detected and the species was identified as Leishmania infantum using nested PCR.
  • [MeSH-major] Leishmania infantum / isolation & purification. Leishmaniasis, Visceral. Precursor Cell Lymphoblastic Leukemia-Lymphoma / complications
  • [MeSH-minor] Amphotericin B / therapeutic use. Animals. Antiprotozoal Agents / therapeutic use. Child. Drug Therapy, Combination. Female. Humans. Interferon-gamma / therapeutic use. Iran. Polymerase Chain Reaction / methods. Treatment Outcome

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  • (PMID = 18463894.001).
  • [ISSN] 0932-0113
  • [Journal-full-title] Parasitology research
  • [ISO-abbreviation] Parasitol. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antiprotozoal Agents; 7XU7A7DROE / Amphotericin B; 82115-62-6 / Interferon-gamma
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20. Tazi I, Rachid M, Quessar A, Harif M, Benchekroun S: [Acute pancreatitis secondary to L-asparaginase (a case report)]. East Mediterr Health J; 2009 Mar-Apr;15(2):475-9
MedlinePlus Health Information. consumer health - Pancreatitis.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-minor] Acute Disease. Adolescent. Amylases / blood. Anti-Bacterial Agents / therapeutic use. Drainage. Fatal Outcome. Humans. L-Lactate Dehydrogenase / blood. Male. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy. Severity of Illness Index. Tomography, X-Ray Computed

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  • (PMID = 19554997.001).
  • [ISSN] 1020-3397
  • [Journal-full-title] Eastern Mediterranean health journal = La revue de santé de la Méditerranée orientale = al-Majallah al-ṣiḥḥīyah li-sharq al-mutawassiṭ
  • [ISO-abbreviation] East. Mediterr. Health J.
  • [Language] fre
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Egypt
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 0 / Antineoplastic Agents; EC 1.1.1.27 / L-Lactate Dehydrogenase; EC 3.2.1.- / Amylases; EC 3.5.1.1 / Asparaginase
  • [Number-of-references] 20
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21. Celik AF, Pamuk GE, Pamuk ON, Uzunismail H, Oktay E, Doğusoy G: Should we suppress the antigenic stimulus in IPSID for lifelong? Am J Gastroenterol; 2000 Nov;95(11):3318-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Should we suppress the antigenic stimulus in IPSID for lifelong?
  • [MeSH-major] Anti-Bacterial Agents / therapeutic use. Immunoproliferative Small Intestinal Disease / drug therapy. Immunoproliferative Small Intestinal Disease / immunology. Tetracycline / therapeutic use
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cyclophosphamide / administration & dosage. Doxorubicin / administration & dosage. Female. Humans. Prednisone / administration & dosage. Vincristine / administration & dosage

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  • (PMID = 11095373.001).
  • [ISSN] 0002-9270
  • [Journal-full-title] The American journal of gastroenterology
  • [ISO-abbreviation] Am. J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; F8VB5M810T / Tetracycline; VB0R961HZT / Prednisone; CHOP protocol
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22. Xu SQ, Fang F, Zhou H: [Immunoproliferative small intestinal disease: a case report and literature review]. Zhongguo Dang Dai Er Ke Za Zhi; 2007 Aug;9(4):389-91

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Immunoproliferative small intestinal disease: a case report and literature review].
  • [MeSH-major] Immunoproliferative Small Intestinal Disease / drug therapy

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  • (PMID = 17706052.001).
  • [ISSN] 1008-8830
  • [Journal-full-title] Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics
  • [ISO-abbreviation] Zhongguo Dang Dai Er Ke Za Zhi
  • [Language] chi
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Immunoglobulin A
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