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1. Azevedo ZM, Silva DR, Dutra MV, Elsas MI, Barbosa-Silva MC, Fonseca VM: [Association between phase angle, PRISM I and sepsis severity]. Rev Bras Ter Intensiva; 2007 Sep;19(3):297-303
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND AND OBJECTIVES: Phase angle (PA) is the difference between voltage and current and can be used as an indicator of body cell mass.
  • The purpose of this study was to know the relation between phase angle and the Pediatric Risk of Mortality I (PRISM I) score, associating this score with the severity of sepsis.
  • METHODS: A transversal study was performed at the Pediatric Intensive Care Unit (PICU) in Instituto Fernandes Figueira.
  • CONCLUSIONS: Pediatric critical patients show low phase angle values, which might have prognostic implication.


2. Meyer PN, Clark JI, Flanigan RC, Picken MM: Xp11.2 translocation renal cell carcinoma with very aggressive course in five adults. Am J Clin Pathol; 2007 Jul;128(1):70-9
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  • [Title] Xp11.2 translocation renal cell carcinoma with very aggressive course in five adults.
  • Renal cell carcinomas associated with Xp11.2 translocations ( TFE3 gene fusions) are rare tumors predominantly reported in children.
  • Unlike pediatric patients, the adult patients followed a rapidly terminal course, with a mean survival of 18 months after diagnosis (range, 10-24 months).
  • [MeSH-major] Carcinoma, Renal Cell / genetics. Chromosomes, Human, X. Kidney Neoplasms / genetics. Translocation, Genetic

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  • (PMID = 17580272.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Neoplasm Proteins; 0 / TFE3 protein, human; 0 / TFEB protein, human
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3. Hornick JL, Fletcher CD: Cellular neurothekeoma: detailed characterization in a series of 133 cases. Am J Surg Pathol; 2007 Mar;31(3):329-40
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  • Cellular neurothekeomas have a predilection for the upper limbs and head and neck of pediatric and young adult females and rarely recur following incomplete excision.
  • There is no good evidence that these lesions show nerve sheath differentiation and the nomenclature will likely change when the tumor cell lineage is better defined.

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  • (PMID = 17325474.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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4. Ferguson CC, Roosevelt G, Bajaj L: Practice patterns of pediatric emergency medicine physicians caring for young febrile infants. Clin Pediatr (Phila); 2010 Apr;49(4):350-4
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  • [Title] Practice patterns of pediatric emergency medicine physicians caring for young febrile infants.
  • The objectives of the study were to describe the practice patterns of pediatric ED physicians caring for these infants and to determine whether the evaluation and management of these infants differed based on their age at presentation.
  • As compared with the younger age group, infants in the older age group had fewer complete blood cell counts (relative risk, RR = 3.57; 95% confidence interval [CI], 2.15-5.95), fewer blood cultures (RR = 3.38; 95% CI, 1.99-5.74), fewer urine cultures (RR = 3.83; 95% CI, 1.81-8.13), and fewer cerebrospinal fluid cultures (RR = 2.56; 95% CI, 1.94-3.40).
  • [MeSH-minor] Age Distribution. Bacteriological Techniques / methods. Bacteriological Techniques / utilization. Blood Cell Count / utilization. Emergency Medical Services / statistics & numerical data. Female. Guideline Adherence / statistics & numerical data. Humans. Infant. Infant, Newborn. Male. Retrospective Studies. Risk. Spinal Puncture / utilization

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  • (PMID = 19564450.001).
  • [ISSN] 1938-2707
  • [Journal-full-title] Clinical pediatrics
  • [ISO-abbreviation] Clin Pediatr (Phila)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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5. Cooper TM: Role of nelarabine in the treatment of T-cell acute lymphoblastic leukemia and T-cell lymphoblastic lymphoma. Ther Clin Risk Manag; 2007 Dec;3(6):1135-41
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  • [Title] Role of nelarabine in the treatment of T-cell acute lymphoblastic leukemia and T-cell lymphoblastic lymphoma.
  • T-cell malignancies have distinct biochemical, immunologic, and clinical features which set them apart from non-T-cell malignancies.
  • In the past, T-cell leukemia portended a worse prognosis than leukemia of B-cell origin.
  • Cure rates have improved with intensification of therapy and advanced understanding of the molecular genetics of T-cell malignancies.
  • Further advances in the treatment of T-cell leukemia will require the development of novel agents that can target specific malignancies without a significant increase in toxicity.
  • Clinical and pharmacokinetic investigations have established that nelarabine is active as a single agent which has led to exploration of an expanded role in the treatment of T-cell hematologic malignances.

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  • (PMID = 18516261.001).
  • [ISSN] 1176-6336
  • [Journal-full-title] Therapeutics and clinical risk management
  • [ISO-abbreviation] Ther Clin Risk Manag
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] New Zealand
  • [Other-IDs] NLM/ PMC2387290
  • [Keywords] NOTNLM ; 9-β-D-arabinofuranosylguanine / T-cell acute lymphoblastic leukemia / nelarabine
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6. Hassan R, White LR, Stefanoff CG, de Oliveira DE, Felisbino FE, Klumb CE, Bacchi CE, Seuánez HN, Zalcberg IR: Epstein-Barr virus (EBV) detection and typing by PCR: a contribution to diagnostic screening of EBV-positive Burkitt's lymphoma. Diagn Pathol; 2006 Aug 07;1:17
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  • We performed a systematic comparison between RISH and PCR for EBV detection, in a group of childhood B-cell Non-Hodgkin lymphomas (NHL), aiming to validate PCR as a first, rapid method for the diagnosis of EBV-associated B-cell NHL.
  • METHODS: EBV infection was investigated in formalin fixed paraffin-embedded tumor samples of 41 children with B-cell NHL, including 35 Burkitt's lymphoma (BL), from Rio de Janeiro, Brazil, by in situ hybridization of EBV-encoded small RNA (EBER-RISH) and PCR assays based on EBNA2 amplification.

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  • (PMID = 16893464.001).
  • [ISSN] 1746-1596
  • [Journal-full-title] Diagnostic pathology
  • [ISO-abbreviation] Diagn Pathol
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA082274
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1559641
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7. Kodo K, Nishizawa T, Furutani M, Arai S, Yamamura E, Joo K, Takahashi T, Matsuoka R, Yamagishi H: GATA6 mutations cause human cardiac outflow tract defects by disrupting semaphorin-plexin signaling. Proc Natl Acad Sci U S A; 2009 Aug 18;106(33):13933-8
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  • [MeSH-major] GATA6 Transcription Factor / genetics. Heart Defects, Congenital / genetics. Nerve Tissue Proteins / genetics. Receptors, Cell Surface / genetics. Semaphorins / metabolism


8. Tamburro RF, Barfield RC, Shaffer ML, Rajasekaran S, Woodard P, Morrison RR, Howard SC, Fiser RT, Schmidt JE, Sillos EM: Changes in outcomes (1996-2004) for pediatric oncology and hematopoietic stem cell transplant patients requiring invasive mechanical ventilation. Pediatr Crit Care Med; 2008 May;9(3):270-7
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  • [Title] Changes in outcomes (1996-2004) for pediatric oncology and hematopoietic stem cell transplant patients requiring invasive mechanical ventilation.
  • OBJECTIVE: To assess the following hypotheses regarding mechanically ventilated pediatric oncology patients, including those receiving hematopoietic stem cell transplant (HSCT) and those not receiving HSCT:.
  • SETTING: Free-standing, tertiary care, pediatric hematology oncology hospital.
  • Forty-five percent of HSCT admissions (92 of 206) vs. 75% of non-HSCT oncology admissions (146 of 195) were extubated and discharged from the pediatric intensive care unit (p < .0001).
  • Among admissions with an abnormal chest radiograph and a PaO2/FiO2 ratio <200, pediatric intensive care unit survival increased for each successive time period, with 45% of HSCT and 83% of non-HSCT admissions surviving during 2002-2004.
  • In multivariate analysis of all study patients, Pediatric Risk of Mortality scores on the day of intubation, allogeneic HSCT, cardiovascular failure, hepatic failure, neurologic failure, a previous course of mechanical ventilation within 6 months, and the time period intubated were associated with mortality.
  • Allogeneic transplant, higher Pediatric Risk of Mortality scores, need for repeated mechanical ventilation, and concomitant organ system dysfunction are risk factors for death.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation. Neoplasms / surgery. Respiration, Artificial

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  • [CommentIn] Pediatr Crit Care Med. 2008 May;9(3):334-5 [18446095.001]
  • (PMID = 18446105.001).
  • [ISSN] 1529-7535
  • [Journal-full-title] Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
  • [ISO-abbreviation] Pediatr Crit Care Med
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 21765
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 42
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9. Harlev D, Zaidman I, Sarig G, Ben Arush MW, Brenner B, Elhasid R: Prophylactic therapy with enoxaparin in children with acute lymphoblastic leukemia and inherited thrombophilia during L-asparaginase treatment. Thromb Res; 2010 Aug;126(2):93-7
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  • [Title] Prophylactic therapy with enoxaparin in children with acute lymphoblastic leukemia and inherited thrombophilia during L-asparaginase treatment.
  • INTRODUCTION: Thrombotic events (TE) are well documented in patients with acute lymphoblastic leukemia (ALL).
  • [MeSH-major] Anticoagulants / therapeutic use. Asparaginase / therapeutic use. Enoxaparin / therapeutic use. Genetic Predisposition to Disease. Precursor Cell Lymphoblastic Leukemia-Lymphoma / complications. Thrombophilia / genetics. Thrombophilia / prevention & control

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  • [Copyright] (c) 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 20546854.001).
  • [ISSN] 1879-2472
  • [Journal-full-title] Thrombosis research
  • [ISO-abbreviation] Thromb. Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anticoagulants; 0 / Enoxaparin; EC 3.5.1.1 / Asparaginase
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10. Kaspers GJ, Wijnands JJ, Hartmann R, Huismans L, Loonen AH, Stackelberg A, Henze G, Pieters R, Hählen K, Van Wering ER, Veerman AJ: Immunophenotypic cell lineage and in vitro cellular drug resistance in childhood relapsed acute lymphoblastic leukaemia. Eur J Cancer; 2005 Jun;41(9):1300-3
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  • [Title] Immunophenotypic cell lineage and in vitro cellular drug resistance in childhood relapsed acute lymphoblastic leukaemia.
  • At relapse, T-cell acute lymphoblastic leukaemia (ALL) has a worse patient outcome than B-cell precursor (BCP-) ALL.
  • To investigate this further, we compared in vitro cellular drug resistance profiles of T-cell and BCP-ALL samples obtained at relapse.
  • We investigated 237 paediatric relapsed ALL cases, including 151 samples taken at first relapse, of which 30 were T-cell ALL.
  • Similar results were found for first relapsed ALL samples and for the total group: T-cell ALL samples were more resistant to 4-HOO-ifosfamide (1.4-fold, P = 0.019) and cisplatin (3.7-fold, P = 0.005).
  • These results do not explain the relatively poor prognosis of T-cell ALL at relapse, but do suggest that the more intensive use of thiopurines in relapsed T-cell ALL may be beneficial.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Burkitt Lymphoma / drug therapy. Drug Resistance, Neoplasm / immunology. Leukemia-Lymphoma, Adult T-Cell / drug therapy. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy
  • [MeSH-minor] Cell Line, Tumor / immunology. Child. Drug Screening Assays, Antitumor. Humans. Immunophenotyping. Lethal Dose 50. Prognosis. Recurrence

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  • (PMID = 15869873.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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11. Storm DS, Boland MG, Gortmaker SL, He Y, Skurnick J, Howland L, Oleske JM, Pediatric AIDS Clinical Trials Group Protocol 219 Study Team: Protease inhibitor combination therapy, severity of illness, and quality of life among children with perinatally acquired HIV-1 infection. Pediatrics; 2005 Feb;115(2):e173-82
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  • METHODS: Cross-sectional data for 940 children, 5 to 18 years of age, who were enrolled in Pediatric AIDS Clinical Trials Group Late Outcomes Protocol 219 were used to examine domains of caregiver-reported QOL, as assessed with the General Health Assessment for Children, during 1999.
  • QOL differences between treatment groups were estimated with linear and logistic regressions that controlled for sociodemographic characteristics (age, gender, race/ethnicity, maternal/caregiver education, and respondent) and severity-of-illness indicators related to receipt of PI therapy (AIDS status, log(10) CD4+ cell counts, and height-for-age z scores).
  • When treatment groups were compared, children receiving PI therapy (72%) were older, had lower CD4+ cell percentages, and had lower height and weight z scores than did those receiving non-PI therapies.
  • Higher log(10) CD4+ cell counts, higher height-for-age z scores, and absence of AIDS at study entry were independently associated with fewer social/school limitations and better HIV symptom scores.
  • Health perceptions and physical functioning scores were associated with log(10) CD4+ cell counts and height z scores, respectively.
  • Findings suggest that the effects of PI combination therapies to slow or to prevent disease progression and to increase CD4+ cell counts and height growth have the potential to improve QOL among children with HIV infection.


12. Marvulo NL, Bonatto RC, Carpi MF, Ricchetti SM, Moraes MA, Fioretto JR: [Red blood cell transfusion in children admitted in a pediatric intensive care unit]. Rev Bras Ter Intensiva; 2006 Dec;18(4):390-5
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  • [Title] [Red blood cell transfusion in children admitted in a pediatric intensive care unit].
  • BACKGROUND AND OBJECTIVES: Indications of red blood cell transfusion in critically ill children are not very well determined.
  • Hence, a protocol to better prescribe red blood cell transfusion at the PICU was adopted.

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  • (PMID = 25310554.001).
  • [ISSN] 0103-507X
  • [Journal-full-title] Revista Brasileira de terapia intensiva
  • [ISO-abbreviation] Rev Bras Ter Intensiva
  • [Language] por
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Brazil
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13. Das P, Puri T, Suri V, Sharma MC, Sharma BS, Sarkar C: Medulloblastomas: a correlative study of MIB-1 proliferation index along with expression of c-Myc, ERBB2, and anti-apoptotic proteins along with histological typing and clinical outcome. Childs Nerv Syst; 2009 Jul;25(7):825-35
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Medulloblastoma (MB) is the most common pediatric brain tumor.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / physiopathology. Cell Proliferation. Medulloblastoma / diagnosis. Medulloblastoma / physiopathology

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  • (PMID = 19444455.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / BCL2L1 protein, human; 0 / Ki-67 Antigen; 0 / MYC protein, human; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / Proto-Oncogene Proteins c-myc; 0 / bcl-X Protein; EC 2.7.10.1 / ERBB2 protein, human; EC 2.7.10.1 / Receptor, ErbB-2
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14. Kobayashi H, Tanaka Y, Asagiri K, Asakawa T, Tanikawa K, Kage M, Yagi M: The antioxidant effect of green tea catechin ameliorates experimental liver injury. Phytomedicine; 2010 Mar;17(3-4):197-202
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  • Therefore, the administration of green tea catechin might have suppressed the oxidative stress, controlled the stellate cell activation and consequently reduced the fibrosis.
  • CONCLUSION: Green tea catechin may reduce hepatic fibrosis by suppressing oxidative stress and controlling the transcription factor expression involved in stellate cell activation.

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  • [Copyright] Copyright 2010 Elsevier GmbH. All rights reserved.
  • [ErratumIn] Phytomedicine. 2010 Sep;17(11):910
  • (PMID = 20092986.001).
  • [ISSN] 1618-095X
  • [Journal-full-title] Phytomedicine : international journal of phytotherapy and phytopharmacology
  • [ISO-abbreviation] Phytomedicine
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Actins; 0 / Aldehydes; 0 / Antioxidants; 0 / Plant Extracts; 0 / RNA, Messenger; 0 / Tissue Inhibitor of Metalloproteinase-1; 0 / Transcription Factor AP-1; 0 / Transforming Growth Factor beta1; 29343-52-0 / 4-hydroxy-2-nonenal; 8R1V1STN48 / Catechin; EC 2.6.1.1 / Aspartate Aminotransferases; EC 2.6.1.2 / Alanine Transaminase; G9481N71RO / Deoxyguanosine
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15. Gavhed D, Akefeldt SO, Osterlundh G, Laurencikas E, Hjorth L, Blennow K, Rosengren L, Henter JI: Biomarkers in the cerebrospinal fluid and neurodegeneration in Langerhans cell histiocytosis. Pediatr Blood Cancer; 2009 Dec 15;53(7):1264-70
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  • [Title] Biomarkers in the cerebrospinal fluid and neurodegeneration in Langerhans cell histiocytosis.
  • BACKGROUND: Progressive neurodegeneration may result in potentially severe cognitive and motor dysfunctions as a complication of Langerhans cell histiocytosis (LCH), a suggested IL-17A-associated inflammatory condition.
  • One hundred ten children with newly diagnosed acute lymphoblastic leukemia (ALL) served as controls.
  • [MeSH-major] Cerebrospinal Fluid Proteins / analysis. Glial Fibrillary Acidic Protein / cerebrospinal fluid. Histiocytosis, Langerhans-Cell / cerebrospinal fluid. Nerve Degeneration. Neurofilament Proteins / cerebrospinal fluid. tau Proteins / cerebrospinal fluid
  • [MeSH-minor] Adolescent. Biomarkers. Brain / pathology. Brain / radiography. Child. Child, Preschool. Cognition Disorders / cerebrospinal fluid. Cognition Disorders / etiology. Disease Progression. Female. Follow-Up Studies. Humans. Infant. Male. Pituitary Diseases / cerebrospinal fluid. Pituitary Diseases / etiology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / cerebrospinal fluid. Spinal Puncture / adverse effects. Young Adult

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  • (PMID = 19688833.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers; 0 / Cerebrospinal Fluid Proteins; 0 / Glial Fibrillary Acidic Protein; 0 / Neurofilament Proteins; 0 / neurofilament protein L; 0 / tau Proteins
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16. Al-Khabori M, Minden MD, Yee KW, Gupta V, Schimmer AD, Schuh AC, Xu W, Brandwein JM: Improved survival using an intensive, pediatric-based chemotherapy regimen in adults with T-cell acute lymphoblastic leukemia. Leuk Lymphoma; 2010 Jan;51(1):61-5
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  • [Title] Improved survival using an intensive, pediatric-based chemotherapy regimen in adults with T-cell acute lymphoblastic leukemia.
  • All patients with newly diagnosed T-cell acute lymphoblastic leukemia (T-ALL) and treated over a 17-year period at a single institution were retrospectively analyzed.
  • From 2000 to 2007, a pediatric-based protocol, DFCI (Dana Farber Cancer Institute), was used as the standard regimen for all patients (n = 32).
  • The results provide evidence supporting the superior efficacy of asparaginase-intensive pediatric-based regimens for adults with T-ALL.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Leukemia-Lymphoma, Adult T-Cell / drug therapy. Leukemia-Lymphoma, Adult T-Cell / mortality


17. Koga Y, Matsuzaki A, Suminoe A, Hattori H, Kanemitsu S, Hara T: Differential mRNA expression of glucocorticoid receptor alpha and beta is associated with glucocorticoid sensitivity of acute lymphoblastic leukemia in children. Pediatr Blood Cancer; 2005 Aug;45(2):121-7
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  • [Title] Differential mRNA expression of glucocorticoid receptor alpha and beta is associated with glucocorticoid sensitivity of acute lymphoblastic leukemia in children.
  • BACKGROUND: Sensitivity of leukemic blasts to glucocorticoid is one of the important prognostic factors for pediatric acute lymphoblastic leukemia (ALL).
  • We examined an association of the expression pattern of GR isoforms in leukemic blasts with their sensitivity to glucocorticoid in childhood ALL.
  • PROCEDURES: The relative mRNA expression of GRalpha, GRbeta, GRgamma, and GR-P was determined in leukemic blasts of 23 childhood ALL at initial presentation and of 14 ALL cell lines by quantitative RT-PCR.
  • RESULTS: The relative expression of GRalpha mRNA was significantly higher in blasts of B-precursor ALL than those of others (13.6 vs. 2.24, P = 0.015), while those of GRalpha, GRbeta, and GRgamma showed no difference.
  • GRbeta/GRalpha ratios were significantly lower in B-precursor ALL than others (0.80 vs. 4.64, P = 0.035).
  • The proportions of apoptotic cells after PSL exposure were inversely correlated with the GRbeta/GRalpha ratios in ALL cell lines (r = -0.612, P = 0.020).
  • PSL administration induced apoptosis efficiently in leukemic blasts with low GRbeta/GRalpha ratios compared with those of high ratios (cell lines: 4.93% vs. 1.90%, P = 0.013, primary leukemia: 11.7% vs. 3.6%, P = 0.037).
  • [MeSH-major] Antineoplastic Agents, Hormonal / pharmacology. Drug Resistance, Neoplasm / genetics. Glucocorticoids / pharmacology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics. Receptors, Glucocorticoid / genetics

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  • (PMID = 15704223.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Glucocorticoids; 0 / Protein Isoforms; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 0 / Receptors, Glucocorticoid
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18. Gutierrez A, Sanda T, Grebliunaite R, Carracedo A, Salmena L, Ahn Y, Dahlberg S, Neuberg D, Moreau LA, Winter SS, Larson R, Zhang J, Protopopov A, Chin L, Pandolfi PP, Silverman LB, Hunger SP, Sallan SE, Look AT: High frequency of PTEN, PI3K, and AKT abnormalities in T-cell acute lymphoblastic leukemia. Blood; 2009 Jul 16;114(3):647-50
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  • [Title] High frequency of PTEN, PI3K, and AKT abnormalities in T-cell acute lymphoblastic leukemia.
  • To more comprehensively assess the pathogenic contribution of the PTEN-PI3K-AKT pathway to T-cell acute lymphoblastic leukemia (T-ALL), we examined diagnostic DNA samples from children with T-ALL using array comparative genomic hybridization and sequence analysis.
  • [MeSH-major] Mutation. PTEN Phosphohydrolase / genetics. Phosphatidylinositol 3-Kinases / genetics. Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / genetics. Proto-Oncogene Proteins c-akt / genetics

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  • (PMID = 19458356.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / K08 CA133103-04; United States / NCI NIH HHS / CA / K08 CA133103-01; United States / NCI NIH HHS / CA / U24 CA114766; United States / NCI NIH HHS / CA / K08 CA133103; United States / NCI NIH HHS / CA / K08 CA133103-03; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / K08 CA133103-02; United States / NCI NIH HHS / CA / P01 CA068484
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.1.- / Phosphatidylinositol 3-Kinases; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt; EC 3.1.3.67 / PTEN Phosphohydrolase; EC 3.1.3.67 / PTEN protein, human
  • [Other-IDs] NLM/ PMC2713461
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19. Dvorak CC, Sanders RP, Dahl GV, Donaldson SS, Razzouk BI: Reinduction of relapsed acute promyelocytic leukemia with ATRA and low dose antimetabolite-based chemotherapy. Pediatr Blood Cancer; 2007 May;48(5):582-5
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  • [Title] Reinduction of relapsed acute promyelocytic leukemia with ATRA and low dose antimetabolite-based chemotherapy.
  • While the disease-free survival of acute promyelocytic leukemia (APML) now approaches 75%, some children continue to experience relapses, and questions remain as to the optimal management of these patients.
  • We describe two young children who experienced combined relapses in the bone marrow and extramedullary locations following hematopoietic stem cell transplantation (HSCT).
  • [MeSH-major] Antimetabolites, Antineoplastic / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Leukemia, Promyelocytic, Acute / drug therapy. Tretinoin / administration & dosage

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  • (PMID = 16123994.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA 21765
  • [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 / Antimetabolites, Antineoplastic; 5688UTC01R / Tretinoin; E7WED276I5 / 6-Mercaptopurine; YL5FZ2Y5U1 / Methotrexate
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20. Dorak MT, Mackay RK, Relton CL, Worwood M, Parker L, Hall AG: Hereditary hemochromatosis gene (HFE) variants are associated with birth weight and childhood leukemia risk. Pediatr Blood Cancer; 2009 Dec 15;53(7):1242-8
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  • [Title] Hereditary hemochromatosis gene (HFE) variants are associated with birth weight and childhood leukemia risk.
  • BACKGROUND: Our original studies reported an association between the iron-metabolism gene HFE and risk of childhood acute lymphoblastic leukemia (ALL), and a birth weight association in ALL.
  • Through its effect on cell proliferation, iron is involved in both fetal development and cancer.
  • We hypothesize that HFE links higher infant birth weight with leukemia risk and that maternal HFE genotype modifies this association.
  • PROCEDURE: Nine hundred ninety-five infants and their mothers from the North Cumbria Community Genetics Project, and 163 incident childhood ALL cases from the Newcastle Haematology Biobank were genotyped for HFE, HAMP, TFRC variants and 21 genomic control loci.
  • [MeSH-major] Antigens, CD / genetics. Antimicrobial Cationic Peptides / genetics. Birth Weight / genetics. Hemochromatosis / genetics. Histocompatibility Antigens Class I / genetics. Infant, Low Birth Weight. Membrane Proteins / genetics. Polymorphism, Single Nucleotide. Precursor Cell Lymphoblastic Leukemia-Lymphoma / epidemiology. Pregnancy Complications / genetics. Receptors, Transferrin / genetics


21. Bate J, Patel SR, Chisholm J, Heath PT, Supportive Care Group of the Children's Cancer and Leukaemia Group (CCLG): Immunisation practices of paediatric oncology and shared care oncology consultants: a United Kingdom survey. Pediatr Blood Cancer; 2010 Jul 1;54(7):941-6
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  • BACKGROUND: In March 2002, the Royal College of Paediatrics and Child Health (RCPCH) introduced guidelines for re-immunisation of children after completion of standard-dose chemotherapy and after haematopoietic stem cell transplant (HSCT).
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Child. Great Britain. Hematopoietic Stem Cell Transplantation. Humans. Neoplasms / therapy. Practice Guidelines as Topic

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  • [Copyright] Copyright 2010 Wiley-Liss, Inc.
  • (PMID = 20162684.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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22. Pandey GN, Dwivedi Y, Rizavi HS, Ren X, Zhang H, Pavuluri MN: Brain-derived neurotrophic factor gene and protein expression in pediatric and adult depressed subjects. Prog Neuropsychopharmacol Biol Psychiatry; 2010 May 30;34(4):645-51
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  • [Title] Brain-derived neurotrophic factor gene and protein expression in pediatric and adult depressed subjects.
  • BACKGROUND: Brain-derived neurotrophic factor (BDNF) is a member of a neurotrophin family and is involved in many physiological functions, including cell proliferation, migration, and differentiation, and neuron survival in the human nervous system.
  • METHODS: We examined the gene expression of BDNF in the lymphocytes and protein expression in the platelets of adult and pediatric depressed patients during a drug-free period.
  • RESULTS: We observed that the gene expression of BDNF was significantly decreased in the lymphocytes of adult and pediatric depressed patients compared with normal control subjects.
  • Similarly, the protein expression of BDNF was significantly decreased in the platelets of adult and pediatric depressed patients compared with normal control subjects.

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • [CommentIn] Prog Neuropsychopharmacol Biol Psychiatry. 2010 Aug 16;34(6):1160; author reply 1161 [20438792.001]
  • (PMID = 20227453.001).
  • [ISSN] 1878-4216
  • [Journal-full-title] Progress in neuro-psychopharmacology & biological psychiatry
  • [ISO-abbreviation] Prog. Neuropsychopharmacol. Biol. Psychiatry
  • [Language] eng
  • [Grant] United States / NIMH NIH HHS / MH / R01 MH077254
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Brain-Derived Neurotrophic Factor; 0 / RNA, Messenger
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23. Niedziela M: Pathogenesis, diagnosis and management of thyroid nodules in children. Endocr Relat Cancer; 2006 Jun;13(2):427-53
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  • The majority of thyroid carcinomas derive from the follicular cell (papillary, follicular, insular and undifferentiated (or anaplastic) thyroid carcinoma), whereas medullary thyroid carcinoma derives from calcitonin-producing cells.

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  • (PMID = 16728572.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Number-of-references] 246
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24. Rungoe C, Malchau EL, Larsen LN, Schroeder H: Infections during induction therapy for children with acute lymphoblastic leukemia. the role of sulfamethoxazole-trimethoprim (SMX-TMP) prophylaxis. Pediatr Blood Cancer; 2010 Aug;55(2):304-8
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  • [Title] Infections during induction therapy for children with acute lymphoblastic leukemia. the role of sulfamethoxazole-trimethoprim (SMX-TMP) prophylaxis.
  • BACKGROUND: Bacteremias are frequent during induction therapy for acute lymphoblastic leukemia (ALL) in children.
  • [MeSH-major] Antibiotic Prophylaxis / methods. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Opportunistic Infections / prevention & control. Precursor Cell Lymphoblastic Leukemia-Lymphoma / complications. Trimethoprim, Sulfamethoxazole Drug Combination / therapeutic use

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  • [Copyright] (c) 2010 Wiley-Liss, Inc.
  • (PMID = 20583218.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Infective Agents; 8064-90-2 / Trimethoprim, Sulfamethoxazole Drug Combination
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25. Karrman K, Andersson A, Björgvinsdóttir H, Strömbeck B, Lassen C, Olofsson T, Nguyen-Khac F, Berger R, Bernard O, Fioretos T, Johansson B: Deregulation of cyclin D2 by juxtaposition with T-cell receptor alpha/delta locus in t(12;14)(p13;q11)-positive childhood T-cell acute lymphoblastic leukemia. Eur J Haematol; 2006 Jul;77(1):27-34
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  • [Title] Deregulation of cyclin D2 by juxtaposition with T-cell receptor alpha/delta locus in t(12;14)(p13;q11)-positive childhood T-cell acute lymphoblastic leukemia.
  • OBJECTIVES: The t(12;14)(p13;q11)--a recurrent translocation in childhood T-cell acute lymphoblastic leukemia (T-ALL)--has very recently been molecularly characterized in one case, which displayed overexpression of the cyclin D2 gene (CCND2).
  • PATIENTS AND METHODS: We have characterized two pediatric t(12;14)-positive T-ALLs using fluorescence in situ hybridization (FISH), cDNA microarray, and real-time polymerase chain reaction (PCR).
  • RESULTS: FISH revealed breakpoints (BPs) in the T-cell receptor alpha/delta locus (14q11) and in the vicinity of the CCND2 gene at 12p13.
  • Furthermore, it is the first example of a T-cell neoplasm with a targeted deregulation of a member of a cyclin-encoding gene family.
  • [MeSH-major] Cyclins / genetics. Gene Expression Regulation, Neoplastic. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics. Receptors, Antigen, T-Cell, alpha-beta / genetics. Receptors, Antigen, T-Cell, gamma-delta / genetics. Translocation, Genetic

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  • (PMID = 16548914.001).
  • [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 / CCND2 protein, human; 0 / Cyclin D2; 0 / Cyclins; 0 / Receptors, Antigen, T-Cell, alpha-beta; 0 / Receptors, Antigen, T-Cell, gamma-delta
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26. Gassas A, Doyle JJ, Weitzman S, Freedman MH, Hitzler JK, Sharathkumar A, Dror Y: A basic classification and a comprehensive examination of pediatric myeloproliferative syndromes. J Pediatr Hematol Oncol; 2005 Apr;27(4):192-6
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  • [Title] A basic classification and a comprehensive examination of pediatric myeloproliferative syndromes.
  • Myeloproliferative syndromes (MPSs) are clonal stem cell disorders resulting in excessive proliferation of one or more cell lineages.
  • Since MPSs in children occur much less commonly than adults, one can argue that the biology and the categories of the various pediatric MPSs seem to be different from adults.
  • Furthermore, confusion exists between pediatric MPS and other overlapping conditions, such as myelodysplastic syndrome.
  • The authors' objectives were to develop a classification system with a list of disorders relevant to children and to characterize pediatric cases of MPS that were devised according to this classification.
  • Based on the predominant proliferating cell lineage, the authors established a classification system for childhood MPS.
  • Primary MPS was classified into granulocytic proliferation--chronic myelogenous leukemia (CML); monocytic--juvenile myelomonocytic leukemia (JMML); megakaryocytic--essential thrombocythemia (ET), familial thrombocytosis, transient myeloproliferative disorder of Down syndrome (TMD); erythrocytic--polycythemia vera, familial erythrocytosis; fibroblastic--idiopathic myelofibrosis (IMF); eosinophilic--idiopathic hypereosinophilic syndrome (IHES); and mast cells--mastocytosis.
  • Significant proportions of cases of childhood MPS (60%) were unique to the pediatric population and not seen in adults.
  • In contrast to adults, MPS in children is more frequently treated with hematopoietic stem cell transplantation (HSCT), the only available curative option for most of these diseases.
  • [MeSH-minor] Adolescent. Bone Marrow / pathology. Cell Lineage. Cell Proliferation. Child. Child, Preschool. Cytogenetic Analysis. Female. Hematopoietic Stem Cell Transplantation. Humans. Infant. Male. Prognosis. Survival Rate

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  • [CommentIn] J Pediatr Hematol Oncol. 2006 Oct;28(10):700-1 [17023836.001]
  • (PMID = 15838389.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Meta-Analysis
  • [Publication-country] United States
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27. Carey ME, Hockenberry MJ, Moore IM, Hutter JJ, Krull KR, Pasvogel A, Kaemingk KL: Brief report: effect of intravenous methotrexate dose and infusion rate on neuropsychological function one year after diagnosis of acute lymphoblastic leukemia. J Pediatr Psychol; 2007 Mar;32(2):189-93
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  • [Title] Brief report: effect of intravenous methotrexate dose and infusion rate on neuropsychological function one year after diagnosis of acute lymphoblastic leukemia.
  • OBJECTIVE: To compare the effects of two intravenous (IV) methotrexate (MTX) infusion protocols on cognitive function in children newly diagnosed with acute lymphoblastic leukemia (ALL).
  • [MeSH-major] Antimetabolites, Antineoplastic / adverse effects. Memory Disorders / chemically induced. Methotrexate / adverse effects. Neuropsychological Tests. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy. Verbal Learning / drug effects

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  • (PMID = 16675716.001).
  • [ISSN] 0146-8693
  • [Journal-full-title] Journal of pediatric psychology
  • [ISO-abbreviation] J Pediatr Psychol
  • [Language] eng
  • [Grant] United States / NICHD NIH HHS / HD / HD37816; United States / NINR NIH HHS / NR / NR04905
  • [Publication-type] Comparative Study; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; YL5FZ2Y5U1 / Methotrexate
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28. Yanofsky RA, Seshia SS, Dawson AJ, Stobart K, Greenberg CR, Booth FA, Prasad C, Del Bigio MR, Wrogemann JJ, Fike F, Gatti RA: Ataxia-telangiectasia: atypical presentation and toxicity of cancer treatment. Can J Neurol Sci; 2009 Jul;36(4):462-7
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  • BACKGROUND: The onset of progressive cerebellar ataxia in early childhood is considered a key feature of ataxia-telangiectasia (A-T), accompanied by ocular apraxia, telangiectasias, immunodeficiency, cancer susceptibility and hypersensitivity to ionizing radiation.
  • METHODS: We describe the clinical features and course of three Mennonite children who were diagnosed with A-T following the completion of therapy for lymphoid malignancies.
  • All three children were "cured" of their lymphoid malignancies, but experienced severe adverse effects from the treatments administered.
  • The diagnosis of A-T should be considered in all children with neuromotor dysfunction or peripheral neuropathy, particularly those who develop lymphoid malignancies.
  • [MeSH-major] Antineoplastic Agents / adverse effects. Ataxia Telangiectasia / chemically induced. Ataxia Telangiectasia / diagnosis. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy
  • [MeSH-minor] Adolescent. Ataxia Telangiectasia Mutated Proteins. Cell Cycle Proteins / metabolism. Child. Child, Preschool. DNA-Binding Proteins / metabolism. Humans. Magnetic Resonance Imaging. Male. Nerve Tissue Proteins / metabolism. Protein-Serine-Threonine Kinases / metabolism. Retrospective Studies. Tumor Suppressor Proteins / metabolism. alpha-Fetoproteins / metabolism

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  • (PMID = 19650357.001).
  • [ISSN] 0317-1671
  • [Journal-full-title] The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
  • [ISO-abbreviation] Can J Neurol Sci
  • [Language] eng
  • [Grant] United States / NIAID NIH HHS / AI / AI 067769; United States / NINDS NIH HHS / NS / NS 052528
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Cell Cycle Proteins; 0 / DNA-Binding Proteins; 0 / Nerve Tissue Proteins; 0 / Tumor Suppressor Proteins; 0 / alpha-Fetoproteins; EC 2.7.11.1 / ATM protein, human; EC 2.7.11.1 / Ataxia Telangiectasia Mutated Proteins; EC 2.7.11.1 / Protein-Serine-Threonine Kinases
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29. Carroll WL, Bhojwani D, Min DJ, Moskowitz N, Raetz EA: Childhood acute lymphoblastic leukemia in the age of genomics. Pediatr Blood Cancer; 2006 May 1;46(5):570-8
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  • [Title] Childhood acute lymphoblastic leukemia in the age of genomics.
  • DNA "chip" or microarray technology holds great promise for the development of more refined, biologically-based classification systems for childhood ALL, as well as the identification of new targets for novel therapy.
  • [MeSH-major] Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics

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  • (PMID = 16365862.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / U01CA114762
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Neoplasm Proteins
  • [Number-of-references] 44
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30. Janzen LA, Spiegler BJ: Neurodevelopmental sequelae of pediatric acute lymphoblastic leukemia and its treatment. Dev Disabil Res Rev; 2008;14(3):185-95
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  • [Title] Neurodevelopmental sequelae of pediatric acute lymphoblastic leukemia and its treatment.
  • This review will describe the neurocognitive outcomes associated with pediatric acute lymphoblastic leukemia (ALL) and its treatment.
  • Preventing or ameliorating treatment-related neuropsychological sequelae represents the next major challenge in pediatric ALL.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Brain Damage, Chronic / chemically induced. Cognition Disorders / chemically induced. Developmental Disabilities / chemically induced. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy. Survivors / psychology

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18924154.001).
  • [ISSN] 1940-5529
  • [Journal-full-title] Developmental disabilities research reviews
  • [ISO-abbreviation] Dev Disabil Res Rev
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 120
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31. Thompson GH, Florentino-Pineda I, Poe-Kochert C, Armstrong DG, Son-Hing JP: The role of Amicar in same-day anterior and posterior spinal fusion for idiopathic scoliosis. Spine (Phila Pa 1976); 2008 Sep 15;33(20):2237-42
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  • Total perioperative blood loss and transfusion requirements (cell saver, autologous, directed, and allogeneic blood) were: 3442.8 +/- 1344.0 mL and 1537.1 +/- 905.1 mL in Group 1; 2089.8 +/- 684.0 mL and 485.2 +/- 349.8 mL in Group 2; and 2184.1 +/- 1163.7 mL and 531.5 +/- 510.5 mL in Group 3.


32. Pereira Pinto L, de Souza LB, Gordón-Núñez MA, Soares RC, de Brito Costa EM, de Aquino AR, Fernandes MZ: Prevention of oral lesions in children with acute lymphoblastic leukemia. Int J Pediatr Otorhinolaryngol; 2006 Nov;70(11):1847-51
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  • [Title] Prevention of oral lesions in children with acute lymphoblastic leukemia.
  • Acute lymphoblastic leukemia (ALL) is the most common form of cancer in children and is responsible for severe stomatologic complications.
  • [MeSH-major] Anti-Infective Agents / therapeutic use. Chlorhexidine / analogs & derivatives. Precursor Cell Lymphoblastic Leukemia-Lymphoma / complications. Stomatitis / prevention & control

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  • (PMID = 16914211.001).
  • [ISSN] 0165-5876
  • [Journal-full-title] International journal of pediatric otorhinolaryngology
  • [ISO-abbreviation] Int. J. Pediatr. Otorhinolaryngol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Ireland
  • [Chemical-registry-number] 0 / Anti-Infective Agents; 0 / Antimetabolites, Antineoplastic; MOR84MUD8E / chlorhexidine gluconate; R4KO0DY52L / Chlorhexidine; YL5FZ2Y5U1 / Methotrexate
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33. Vastert SJ, Kuis W, Grom AA: Systemic JIA: new developments in the understanding of the pathophysiology and therapy. Best Pract Res Clin Rheumatol; 2009 Oct;23(5):655-64
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  • [Title] Systemic JIA: new developments in the understanding of the pathophysiology and therapy.
  • Systemic juvenile idiopathic arthritis (sJIA) is a rare, systemic inflammatory disease classified as a subtype of JIA.
  • Besides arthritis, it is characterised by systemic features such as spiking fever, skin rash, hepatosplenomegaly or serositis.
  • It is becoming clear now that abnormalities in the innate immunity (cytokines such as interleukin (IL)-1, IL-6 and IL-18, and neutrophils and monocytes/macrophages rather than lymphocytes) play a major role in the pathogenesis of sJIA, distinguishing it from other JIA subtypes.
  • Another distinctive feature of sJIA is its strong association with macrophage activation syndrome (MAS).
  • Based on this, consensus is emerging that sJIA should be viewed as an autoinflammatory syndrome rather than a classic auto-immune disease.
  • As a consequence of the progression in understanding the underlying mechanisms of sJIA, major changes in the management are evolving.
  • So far, treatment has been based on glucocorticosteroids in combination with disease-modifying drugs such as methotrexate.
  • Recently, remarkable improvement has been observed with IL-1 and IL-6 targeted therapies.
  • These therapies might also change the long-term outcome of this disease.
  • However, controlled trials set up in international collaboration are needed to determine the optimal treatment strategies for all sJIA patients.

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  • (PMID = 19853830.001).
  • [ISSN] 1532-1770
  • [Journal-full-title] Best practice & research. Clinical rheumatology
  • [ISO-abbreviation] Best Pract Res Clin Rheumatol
  • [Language] ENG
  • [Grant] United States / NIAMS NIH HHS / AR / P01 AR048929; United States / NIAMS NIH HHS / AR / AR048929-01A10004; United States / NIAMS NIH HHS / AR / AR047784-070007; United States / NIAMS NIH HHS / AR / P01 AR048929-01A10004; United States / NIAMS NIH HHS / AR / P60 AR047784; United States / NIAMS NIH HHS / AR / P60 AR047784-070007
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antirheumatic Agents; 0 / Cytokines; 0 / Glucocorticoids
  • [Number-of-references] 78
  • [Other-IDs] NLM/ NIHMS146803; NLM/ PMC2774820
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34. Sud S, Marcon M, Assor E, Palmert MR, Daneman D, Mahmud FH: Celiac disease and pediatric type 1 diabetes: diagnostic and treatment dilemmas. Int J Pediatr Endocrinol; 2010;2010:161285
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  • [Title] Celiac disease and pediatric type 1 diabetes: diagnostic and treatment dilemmas.
  • Despite the advent of sensitive and specific serologic testing, routine screening for celiac disease (CD) in diabetic populations may not be universal practice, and many clinicians struggle to find the optimal approach to managing CD in pediatric Type 1 diabetes (T1D) patients.

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  • (PMID = 20652072.001).
  • [ISSN] 1687-9856
  • [Journal-full-title] International journal of pediatric endocrinology
  • [ISO-abbreviation] Int J Pediatr Endocrinol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2905696
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35. García-Nieto V, Flores C, Luis-Yanes MI, Gallego E, Villar J, Claverie-Martín F: Mutation G47R in the BSND gene causes Bartter syndrome with deafness in two Spanish families. Pediatr Nephrol; 2006 May;21(5):643-8
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  • [Title] Mutation G47R in the BSND gene causes Bartter syndrome with deafness in two Spanish families.
  • Bartter syndrome (BS) is a heterogeneous group of autosomal recessive hypokalaemic salt-losing tubulopathies.
  • Five types of BS caused by different genetic defects have been identified, and one of them is associated with sensorineural deafness (BSND).
  • Mutations in the recently described BSND gene, mapped in chromosome 1p31, have been reported to be associated with BSND.
  • This gene encodes barttin, an essential beta-subunit subunit for ClC-Ka and ClC-Kb channels.
  • Both subunits are co-expressed in basolateral membranes of renal tubules, in the ascending limb of the loop of Henle, and in the stria vascularis of the inner ear.
  • We studied two apparently unrelated Spanish families from the Canary Islands, with five members showing this pathology.
  • Sequence analysis of the BSND gene showed that the affected members were homozygous for a C-to-T transition in exon 1, while their parents were heterozygous.
  • This alteration results in a missense mutation, G47R, which has been previously shown to abolish the stimulatory effect on the subunit barttin of the ClC-Kb channel.
  • Our results indicate that families with the G47R mutation indeed present polyhydramnios, premature birth and salt loss.
  • Nevertheless, glomerular filtration rate was normal in all patients.
  • Clinical manifestations are moderate in patients with the G47R mutation compared to other published data form patients with BSND.
  • This constitutes the first report of BSND cases in Spain.
  • [MeSH-major] Bartter Syndrome / genetics. Hearing Loss, Sensorineural / genetics. Membrane Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Child, Preschool. Chloride Channels / genetics. Chloride Channels / physiology. Deafness / genetics. Female. Humans. Male. Mutation, Missense / genetics. Pedigree. Phenotype. Sequence Analysis, DNA. Spain

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  • (PMID = 16572343.001).
  • [ISSN] 0931-041X
  • [Journal-full-title] Pediatric nephrology (Berlin, Germany)
  • [ISO-abbreviation] Pediatr. Nephrol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / BSND protein, human; 0 / CLCNKB protein, human; 0 / Chloride Channels; 0 / Membrane Proteins
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36. Zalewski G, Wasilewska A, Zoch-Zwierz W, Chyczewski L: Response to prednisone in relation to NR3C1 intron B polymorphisms in childhood nephrotic syndrome. Pediatr Nephrol; 2008 Jul;23(7):1073-8
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  • [Title] Response to prednisone in relation to NR3C1 intron B polymorphisms in childhood nephrotic syndrome.


37. Jaing TH, Yang CP, Hung IJ, Tsay PK, Tseng CK, Chen SH: Clinical significance of central nervous system involvement at diagnosis of childhood T-cell acute lymphoblastic leukemia. Pediatr Blood Cancer; 2005 Aug;45(2):135-8
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  • [Title] Clinical significance of central nervous system involvement at diagnosis of childhood T-cell acute lymphoblastic leukemia.
  • BACKGROUND: Patients with T-cell acute lymphoblastic leukemia (T-ALL) frequently present with unfavorable features at diagnosis.
  • We sought to correlate initial central nervous system (CNS) disease at diagnosis with shortened survival in childhood T-ALL.
  • The introduction of early and effective CNS-directed therapy might no longer portend a poor prognosis for CNS leukemia.
  • [MeSH-major] Central Nervous System Neoplasms / diagnosis. Leukemia, T-Cell / diagnosis. Precursor Cell Lymphoblastic Leukemia-Lymphoma / diagnosis


38. Sharma R, Tepas JJ 3rd, Hudak ML, Mollitt DL, Wludyka PS, Teng RJ, Premachandra BR: Neonatal gut barrier and multiple organ failure: role of endotoxin and proinflammatory cytokines in sepsis and necrotizing enterocolitis. J Pediatr Surg; 2007 Mar;42(3):454-61
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  • METHODS: Blood samples from infants with signs of NEC and/or sepsis were collected for culture and determination of complete blood cell counts and concentrations of CKs (interleukin [IL]-1beta, tumor necrosis factor [TNF] alpha, and IL-6) and ETX at the onset of illness.


39. Balgobind BV, Van Vlierberghe P, van den Ouweland AM, Beverloo HB, Terlouw-Kromosoeto JN, van Wering ER, Reinhardt D, Horstmann M, Kaspers GJ, Pieters R, Zwaan CM, Van den Heuvel-Eibrink MM, Meijerink JP: Leukemia-associated NF1 inactivation in patients with pediatric T-ALL and AML lacking evidence for neurofibromatosis. Blood; 2008 Apr 15;111(8):4322-8
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  • [Title] Leukemia-associated NF1 inactivation in patients with pediatric T-ALL and AML lacking evidence for neurofibromatosis.
  • Patients with NF1 have a higher risk to develop juvenile myelomonocytic leukemia (JMML) with a possible progression toward acute myeloid leukemia (AML).
  • In an oligo array comparative genomic hybridization-based screening of 103 patients with pediatric T-cell acute lymphoblastic leukemia (T-ALL) and 71 patients with MLL-rearranged AML, a recurrent cryptic deletion, del(17)(q11.2), was identified in 3 patients with T-ALL and 2 patients with MLL-rearranged AML.
  • Since the NF1 protein is a negative regulator of the RAS pathway (RAS-GTPase activating protein), homozygous NF1 inactivation represent a novel type I mutation in pediatric MLL-rearranged AML and T-ALL with a predicted frequency that is less than 10%.
  • NF1 inactivation may provide an additional proliferative signal toward the development of leukemia.
  • [MeSH-major] Leukemia, Myeloid, Acute / genetics. Leukemia-Lymphoma, Adult T-Cell / genetics. Mutation / genetics. Neurofibromatoses / genetics. Neurofibromin 1 / genetics


40. Toporski J, Garkavij M, Tennvall J, Ora I, Gleisner KS, Dykes JH, Lenhoff S, Juliusson G, Scheding S, Turkiewicz D, Békássy AN: High-dose iodine-131-metaiodobenzylguanidine with haploidentical stem cell transplantation and posttransplant immunotherapy in children with relapsed/refractory neuroblastoma. Biol Blood Marrow Transplant; 2009 Sep;15(9):1077-85
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  • [Title] High-dose iodine-131-metaiodobenzylguanidine with haploidentical stem cell transplantation and posttransplant immunotherapy in children with relapsed/refractory neuroblastoma.
  • We evaluated the feasibility and efficacy of using high-dose iodine-131-metaiodobenzylguanidine ((131)I-MIBG) followed by reduced-intensity conditioning (RIC) and transplantation of T cell-depleted haploidentical peripheral blood stem cells (designated haplo-SCT) to treat relapsing/refractory neuroblastoma (RRNB).
  • Granulocyte-colony stimulating factor (G-CSF)-mobilized, T cell-depleted haploidentical paternal stem cells were infused on day 0 together with cultured donor mesenchymal stem cells.
  • No primary acute graft-versus-host disease (aGVHD) was observed.
  • [MeSH-major] 3-Iodobenzylguanidine / administration & dosage. Hematopoietic Stem Cell Transplantation / methods. Neoplasm Recurrence, Local / therapy. Neuroblastoma / therapy
  • [MeSH-minor] Antineoplastic Agents / administration & dosage. Cell Culture Techniques. Child. Child, Preschool. Cohort Studies. Combined Modality Therapy. Female. Graft vs Host Disease / immunology. Haplotypes. Humans. Infant. Male. Mesenchymal Stem Cell Transplantation. Radiopharmaceuticals / administration & dosage. T-Lymphocytes / immunology. Transplantation Chimera

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  • (PMID = 19660720.001).
  • [ISSN] 1523-6536
  • [Journal-full-title] Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation
  • [ISO-abbreviation] Biol. Blood Marrow Transplant.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
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41. Yazi D, Akkoc T, Yesil O, Ozdemir C, Aydoğan M, Koksalan K, Bahceciler NN, Barlan IB: Treatment with Mycobacterium vaccae ameliorates airway histopathology in a murine model of asthma. Allergy Asthma Proc; 2008 Jan-Feb;29(1):67-73
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  • Furthermore, smooth muscle and epithelial thickness in small and large airways and hyperplasic goblet cell numbers in all sized airways of this treatment group were not significantly different from controls.

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  • (PMID = 18302841.001).
  • [ISSN] 1088-5412
  • [Journal-full-title] Allergy and asthma proceedings
  • [ISO-abbreviation] Allergy Asthma Proc
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Bacterial Vaccines; 0 / Interleukin-5; 0 / SRP 299; 130068-27-8 / Interleukin-10; 82115-62-6 / Interferon-gamma; 9006-59-1 / Ovalbumin
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42. Mcheik JN, Dichamp I, Levard G, Ragot S, Beby-Defaux A, Grosos C, Couvrat V, Agius G: Infantile hypertrophic pyloric stenosis: are viruses involved? J Med Virol; 2010 Dec;82(12):2087-91
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  • Nasopharyngeal fluids, stools, vomit, and surgical pyloric muscle fragments and swabs were tested by cell culture, viral antigen assay and PCR.

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  • (PMID = 20981797.001).
  • [ISSN] 1096-9071
  • [Journal-full-title] Journal of medical virology
  • [ISO-abbreviation] J. Med. Virol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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43. Büyükka Bay S, Corapcioglu F, Kavurt S, Müezzinoğlu B, Anik Y, Tugay M: Oligodendroglioma arising in a mature cystic ovarian teratoma in a child. Pediatr Hematol Oncol; 2010 Nov;27(8):636-40
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  • In childhood mature cystic teratomas are the most common type of ovarian germ cell tumors.
  • On histopatological examination, various samples from cystic areas had mature tissues from all 3 germ cell layers, including skin, bone, bronchial structures, and cerebellum.
  • To the authors' knowledge, there are a few reports in the literature of an oligodendroglioma arising in an ovarian teratoma in adults and this presented patient is the first case in childhood.


44. Small D: Targeting FLT3 for the treatment of leukemia. Semin Hematol; 2008 Jul;45(3 Suppl 2):S17-21
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  • [Title] Targeting FLT3 for the treatment of leukemia.
  • FLT3 is a receptor tyrosine kinase with important roles in hematopoietic stem/progenitor cell survival and proliferation.
  • It is frequently overexpressed in acute leukemias and is frequently mutated in acute myeloid leukemia (AML).
  • FLT3 internal tandem duplication (ITD) mutations in AML portend poor prognosis in both adult and pediatric patients.
  • Anti-FLT3 antibodies may also prove to be an excellent way of targeting FLT3 in AML and acute lymphocytic leukemia (ALL) by inhibiting signaling and through antibody-dependent cell-mediated cytotoxicity.

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  • (PMID = 18760705.001).
  • [ISSN] 0037-1963
  • [Journal-full-title] Seminars in hematology
  • [ISO-abbreviation] Semin. Hematol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA090668; United States / NCI NIH HHS / CA / P01 CA070970; United States / NCI NIH HHS / CA / R01 CA090668-01A1; United States / NCI NIH HHS / CA / CA090668-01A1; United States / NCI NIH HHS / CA / P01 CA070970-10A16432; United States / NCI NIH HHS / CA / CA070970-10A16432
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies; 0 / Protein Kinase Inhibitors; EC 2.7.10.1 / FLT3 protein, human; EC 2.7.10.1 / fms-Like Tyrosine Kinase 3
  • [Number-of-references] 34
  • [Other-IDs] NLM/ NIHMS69701; NLM/ PMC2597087
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45. Kaler SG, Tang J, Donsante A, Kaneski CR: Translational read-through of a nonsense mutation in ATP7A impacts treatment outcome in Menkes disease. Ann Neurol; 2009 Jan;65(1):108-13
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  • [Title] Translational read-through of a nonsense mutation in ATP7A impacts treatment outcome in Menkes disease.
  • Protein translation ends when a stop codon in a gene's messenger RNA transcript enters the ribosomal A site.
  • Mutations that create premature stop codons (nonsense mutations) typically cause premature translation termination.
  • An alternative outcome, read-through translation (or nonsense suppression), is well known in prokaryotic, viral, and yeast genes but has not been clearly documented in humans except in the context of pharmacological manipulations.
  • Here, we identify and characterize native read-through of a nonsense mutation (R201X) in the human copper transport gene, ATP7A.
  • Western blotting, in vitro expression analyses, immunohistochemistry, and yeast complementation assays using cultured fibroblasts from a classic Menkes disease patient all indicated small amounts of native ATP7A(R201X) read-through and were associated with a dramatic clinical response to early copper treatment.
  • [MeSH-major] Adenosine Triphosphatases / genetics. Cation Transport Proteins / genetics. Codon, Nonsense / genetics. Menkes Kinky Hair Syndrome / genetics
  • [MeSH-minor] Cells, Cultured. DNA Mutational Analysis. Fibroblasts / metabolism. Fibroblasts / pathology. Genetic Complementation Test / methods. Humans. Infant. Magnetic Resonance Imaging. Models, Molecular. Peptide Chain Termination, Translational / genetics

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  • (PMID = 19194885.001).
  • [ISSN] 1531-8249
  • [Journal-full-title] Annals of neurology
  • [ISO-abbreviation] Ann. Neurol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 HD008768-04
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cation Transport Proteins; 0 / Codon, Nonsense; EC 3.6.1.- / Adenosine Triphosphatases; EC 3.6.3.4 / ATP7A protein, human
  • [Other-IDs] NLM/ NIHMS86171; NLM/ PMC2917729
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46. Yu DC, Grabowski MJ, Kozakewich HP, Perez-Atayde AR, Voss SD, Shamberger RC, Weldon CB: Primary lung tumors in children and adolescents: a 90-year experience. J Pediatr Surg; 2010 Jun;45(6):1090-5
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  • Rare pediatric lung tumors including small cell carcinoma, adenocarcinoma, and pulmonary capillary hemangiomatosis were also seen.

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20620301.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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47. Bax DA, Mackay A, Little SE, Carvalho D, Viana-Pereira M, Tamber N, Grigoriadis AE, Ashworth A, Reis RM, Ellison DW, Al-Sarraj S, Hargrave D, Jones C: A distinct spectrum of copy number aberrations in pediatric high-grade gliomas. Clin Cancer Res; 2010 Jul 01;16(13):3368-77
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  • [Title] A distinct spectrum of copy number aberrations in pediatric high-grade gliomas.
  • Toward this end, we sought to validate and extend investigations of the differences between pediatric and adult tumors.
  • Despite this, aberrations targeting the "core signaling pathways" in adult glioblastomas are significantly underrepresented in the pediatric setting.
  • CONCLUSIONS: These data highlight that although there are overlaps in the genomic events driving gliomagenesis of all ages, the pediatric disease harbors a distinct spectrum of copy number aberrations compared with adults.

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  • (PMID = 20570930.001).
  • [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
  • [Grant] United Kingdom / Cancer Research UK / / A6718
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2896553; NLM/ UKMS30567
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48. Angiolillo AL, Whitlock J, Chen Z, Krailo M, Reaman G, Children's Oncology Group: Phase II study of gemcitabine in children with relapsed acute lymphoblastic leukemia or acute myelogenous leukemia (ADVL0022): a Children's Oncology Group Report. Pediatr Blood Cancer; 2006 Feb;46(2):193-7
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  • [Title] Phase II study of gemcitabine in children with relapsed acute lymphoblastic leukemia or acute myelogenous leukemia (ADVL0022): a Children's Oncology Group Report.
  • BACKGROUND: To determine the response rate and toxicity to gemcitabine administered as 10 mg/m2/min x 360 min weekly for 3 weeks in children with relapsed acute lymphoblastic leukemia (ALL) or acute myelogenous leukemia (AML).
  • [MeSH-major] Antimetabolites, Antineoplastic / administration & dosage. Deoxycytidine / analogs & derivatives. Leukemia, Myeloid, Acute / prevention & control. Precursor Cell Lymphoblastic Leukemia-Lymphoma / prevention & control


49. Hockenberry M, Krull K, Moore K, Gregurich MA, Casey ME, Kaemingk K: Longitudinal evaluation of fine motor skills in children with leukemia. J Pediatr Hematol Oncol; 2007 Aug;29(8):535-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Longitudinal evaluation of fine motor skills in children with leukemia.
  • BACKGROUND: Improved survival for children with acute lymphocytic leukemia (ALL) has allowed investigators to focus on the adverse or side effects of treatment and to develop interventions that promote cure while decreasing the long-term effects of therapy.
  • [MeSH-major] Motor Skills. Precursor Cell Lymphoblastic Leukemia-Lymphoma / physiopathology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / therapy

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  • (PMID = 17762494.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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50. Steigman SA, Oh JT, Almendinger N, Javid P, LaVan D, Fauza D: Structural and biomechanical characteristics of the diaphragmatic tendon in infancy and childhood: an initial analysis. J Pediatr Surg; 2010 Jul;45(7):1455-8
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  • [Title] Structural and biomechanical characteristics of the diaphragmatic tendon in infancy and childhood: an initial analysis.
  • CONCLUSIONS: In neonates and infants, the diaphragmatic tendon has increased cell density and higher levels of major extracellular matrix components than in older children, in whom the diaphragmatic tendon tends to have higher tensile strength.

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20638523.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Glycosaminoglycans; 9007-34-5 / Collagen; 9007-49-2 / DNA; 9007-58-3 / Elastin
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51. Grate LR: Many accurate small-discriminatory feature subsets exist in microarray transcript data: biomarker discovery. BMC Bioinformatics; 2005;6:97
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  • We also find small gene sets that distinguish leukemia subtypes in the large pediatric acute lymphoblastic leukemia cancer set of Yeoh et al.
  • [MeSH-minor] Biomarkers, Tumor. Carcinoma, Hepatocellular / diagnosis. Carcinoma, Hepatocellular / genetics. Cell Line, Tumor. Cluster Analysis. Data Interpretation, Statistical. Databases, Genetic. Gene Expression Regulation, Neoplastic. Humans. Liver Neoplasms / diagnosis. Liver Neoplasms / genetics. Markov Chains. Models, Molecular. Models, Theoretical. Neoplasms / diagnosis. Neoplasms / genetics. Pattern Recognition, Automated. Precursor Cell Lymphoblastic Leukemia-Lymphoma / diagnosis. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics. Protein Conformation. Sequence Analysis, DNA. Software Design

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  • (PMID = 15826317.001).
  • [ISSN] 1471-2105
  • [Journal-full-title] BMC bioinformatics
  • [ISO-abbreviation] BMC Bioinformatics
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers; 0 / Biomarkers, Tumor
  • [Other-IDs] NLM/ PMC1090559
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52. Kunisaki SM, Freedman DA, Fauza DO: Fetal tracheal reconstruction with cartilaginous grafts engineered from mesenchymal amniocytes. J Pediatr Surg; 2006 Apr;41(4):675-82; discussion 675-82
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  • The amniotic fluid can be a practical cell source for engineered tracheal reconstruction.
  • [MeSH-major] Amniotic Fluid / cytology. Cartilage / transplantation. Cell Transplantation. Fetus / surgery. Mesoderm / cytology. Tissue Engineering. Trachea / surgery

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  • (PMID = 16567175.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Grant] United States / NIDDK NIH HHS / DK / DK065406-02
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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53. Vartzelis G, Lancaster D, Fallon P: Intracranial hypertension in pediatric patients with acute lymphoblastic leukemia. Pediatr Blood Cancer; 2009 Mar;52(3):418-20
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  • [Title] Intracranial hypertension in pediatric patients with acute lymphoblastic leukemia.
  • Acute lymphoblastic leukemia (ALL) remains one of the most common malignancies of childhood.
  • [MeSH-major] Intracranial Hypertension / complications. Precursor Cell Lymphoblastic Leukemia-Lymphoma / complications


54. Marie-Cardine A, Divay F, Dutot I, Green A, Perdrix A, Boyer O, Contentin N, Tilly H, Tron F, Vannier JP, Jacquot S: Transitional B cells in humans: characterization and insight from B lymphocyte reconstitution after hematopoietic stem cell transplantation. Clin Immunol; 2008 Apr;127(1):14-25
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  • [Title] Transitional B cells in humans: characterization and insight from B lymphocyte reconstitution after hematopoietic stem cell transplantation.
  • However, their precise role in human B cell differentiation has not been established.
  • Therefore, besides characterizing them further in the blood of healthy adults and children and cord blood, we used the immune reconstitution after hematopoietic stem cell transplantation (HSCT) model to define their role in human B cell development.
  • Similarly, after HSCT, all B cells first appearing in peripheral blood are transitional B cells; afterwards, the transitional B cell percentage progressively decreases while the mature naïve B cell proportion rises.
  • Our results now formally demonstrate that transitional B cells are necessary developmental intermediates for human mature B cell generation.
  • [MeSH-major] B-Lymphocyte Subsets / cytology. B-Lymphocytes / cytology. Cell Differentiation / immunology. Hematopoietic Stem Cell Transplantation. Precursor Cells, B-Lymphoid / cytology

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  • (PMID = 18191619.001).
  • [ISSN] 1521-6616
  • [Journal-full-title] Clinical immunology (Orlando, Fla.)
  • [ISO-abbreviation] Clin. Immunol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD
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55. Alcasabas P, Ravindranath Y, Goyette G, Haller A, Del Rosario L, Lesaca-Medina MY, Darga L, Ostrea EM Jr, Taub JW, Everson RB: 5,10-methylenetetrahydrofolate reductase (MTHFR) polymorphisms and the risk of acute lymphoblastic leukemia (ALL) in Filipino children. Pediatr Blood Cancer; 2008 Aug;51(2):178-82
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  • [Title] 5,10-methylenetetrahydrofolate reductase (MTHFR) polymorphisms and the risk of acute lymphoblastic leukemia (ALL) in Filipino children.
  • This may be due to a difference in leukemia biology or to a high prevalence of folate deficiency in Filipinos.
  • [MeSH-major] Methylenetetrahydrofolate Reductase (NADPH2) / genetics. Polymorphism, Genetic. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics

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  • (PMID = 18421714.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NIEHS NIH HHS / ES / P30 ES06639; United States / NICHD NIH HHS / HD / R01HD0394281
  • [Publication-type] 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] EC 1.5.1.20 / Methylenetetrahydrofolate Reductase (NADPH2)
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56. Ivanovski P, Nikolić D, Dimitrijević N, Ivanovski I, Perišić V: Erythrocytic transglutaminase inhibition hemolysis at presentation of celiac disease. World J Gastroenterol; 2010 Nov 28;16(44):5647-50
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  • Previously it was considered to be a rare childhood disorder, but is actually considered a relatively common condition, present at any age, which may have multiple complications and manifestations.

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57. Wolf E, Schneider MR, Zhou R, Fisch TM, Herbach N, Dahlhoff M, Wanke R, Hoeflich A: Functional consequences of IGFBP excess-lessons from transgenic mice. Pediatr Nephrol; 2005 Mar;20(3):269-78
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  • The functions of insulin-like growth factor-binding proteins (IGFBPs) have been studied extensively in vitro, revealing IGF-dependent and also IGF-independent effects on cell growth, differentiation, and survival.

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  • (PMID = 15602658.001).
  • [ISSN] 0931-041X
  • [Journal-full-title] Pediatric nephrology (Berlin, Germany)
  • [ISO-abbreviation] Pediatr. Nephrol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Insulin-Like Growth Factor Binding Proteins
  • [Number-of-references] 68
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58. Dengel DR, Ness KK, Glasser SP, Williamson EB, Baker KS, Gurney JG: Endothelial function in young adult survivors of childhood acute lymphoblastic leukemia. J Pediatr Hematol Oncol; 2008 Jan;30(1):20-5
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  • [Title] Endothelial function in young adult survivors of childhood acute lymphoblastic leukemia.
  • BACKGROUND: Adult survivors of childhood acute lymphoblastic leukemia (ALL) have an earlier than expected mortality from cardiovascular disease.
  • This study examined endothelial function in 75 young (age 30.2+/-7.1 y) adult survivors of childhood ALL who received chemotherapy without cranial radiation (n=25) or chemotherapy combined with cranial radiation (n=50) compared with a healthy control group of similar sex, age, and weight (n=59).
  • CONCLUSIONS: These data suggest that young adults treated for ALL during childhood are at risk for impaired FMD regardless of whether or not they received cranial irradiation.
  • The extent to which this mechanism relates to early development of cardiovascular disease in long-term childhood ALL survivors remains to be determined.
  • [MeSH-major] Brachial Artery / physiopathology. Endothelium, Vascular / physiopathology. Nitroglycerin / administration & dosage. Precursor Cell Lymphoblastic Leukemia-Lymphoma / physiopathology. Vasodilation / drug effects. Vasodilator Agents / administration & dosage

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  • (PMID = 18176175.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Grant] United States / NCRR NIH HHS / RR / M01-RR00400; United States / NCI NIH HHS / CA / R21-CA106778; United States / NCI NIH HHS / CA / U24-CA55727
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Vasodilator Agents; G59M7S0WS3 / Nitroglycerin
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59. Moreno L, Bautista FJ, Zacharoulis S: Outcome of teenagers and young adults with ependymoma: the Royal Marsden experience. Childs Nerv Syst; 2009 Sep;25(9):1047-52
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  • [Title] Outcome of teenagers and young adults with ependymoma: the Royal Marsden experience.
  • BACKGROUND: The outcome and clinical characteristics of teenagers and young adults (TYA) with ependymoma have not been well documented.
  • We report the Royal Marsden Hospital experience treating TYA with ependymoma.
  • MATERIALS AND METHODS: Sixteen TYA were treated for ependymoma from 1971 to 2004 and are compared to 24 children (not infants) treated in the same period.
  • RESULTS: Twelve TYA (75%) received treatment in a neuro-oncology unit.
  • Average time from symptoms to diagnosis was 183 days for TYA vs. 61.2 for children (p = 0.005).
  • Two TYA (12.5% vs. 41.6% for children, p = 0.08) were enrolled in a clinical trial.
  • Only 25% of TYA achieved gross total resection, all of them received radiotherapy and five of them received chemotherapy.
  • There were five relapses; all of them were local.
  • Five-year overall survival was 84.6% +/- 10 for TYA vs. 78.1% +/- 8.7 for children (p = 0.15), and 5-year progression-free survival was 66.6% +/- 12.3 for TYA vs. 44.4% +/- 10.3 for children (p = 0.08).
  • Up to 56% of patients treated in the paediatric unit received psychosocial support vs. 42.9% of patients treated in the adult unit.
  • DISCUSSION: Ependymoma in adolescents and young adults is an infrequent entity, with perhaps better outcome compared to children.
  • The extent of surgical resection as seen in children is an important prognostic factor.
  • Providing adolescents with ependymoma the appropriate neuro-oncologic care, including access to multidisciplinary teams, full access to clinical trials and age-appropriate neuro-oncologic ancillary support services, remains a challenge.
  • [MeSH-major] Brain Neoplasms / therapy. Ependymoma / therapy
  • [MeSH-minor] Adolescent. Age Factors. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Kaplan-Meier Estimate. Male. Neoplasm Recurrence, Local / therapy. Social Support. Treatment Outcome. Young Adult

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  • (PMID = 19533154.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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61. Paris C, Kopp K, King A, Santolaya ME, Zepeda AJ, Palma J: Cytomegalovirus infection in children undergoing hematopoietic stem cell transplantation in Chile. Pediatr Blood Cancer; 2009 Sep;53(3):453-8
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  • [Title] Cytomegalovirus infection in children undergoing hematopoietic stem cell transplantation in Chile.
  • BACKGROUND: Cytomegalovirus (CMV) infection remains as an important cause of morbidity and mortality in children undergoing hematopoietic stem cell transplantation (HSCT).
  • Risk factors identified were pre-transplant serologic status (positive recipient), acute and chronic graft versus host disease (GvHD), GvHD prophylaxis, and treatment with antithymocyte globulin.
  • [MeSH-major] Cytomegalovirus Infections / etiology. Hematopoietic Stem Cell Transplantation / adverse effects

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  • [Copyright] (c) 2009 Wiley-Liss, Inc.
  • (PMID = 19418548.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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62. Laudadio RE, Wolfson MR, Shaffer TH, Driska SP: Developmental differences in the contractile response of isolated ovine tracheal smooth muscle cells. Pediatr Pulmonol; 2009 Jun;44(6):602-12
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  • This technique was used to study the change in contractile response of the airway smooth muscle (ASM) cell during development.
  • When velocity was normalized to the cell length, there was no difference between the adult and PT cells, but there was a significant difference between the NB (0.30 sec(-1)) and adult (0.54 sec(-1)) cells.

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  • (PMID = 19434687.001).
  • [ISSN] 1099-0496
  • [Journal-full-title] Pediatric pulmonology
  • [ISO-abbreviation] Pediatr. Pulmonol.
  • [Language] ENG
  • [Grant] United States / NCRR NIH HHS / RR / P20 RR020173; United States / NCRR NIH HHS / RR / 1 P20 RR 020173
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS368109; NLM/ PMC4367129
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63. Galinier P, Carfagna L, Delsol M, Ballouhey Q, Lemasson F, Le Mandat A, Moscovici J, Guitard J, Pienkowski C, Vaysse P: Ovarian torsion. Management and ovarian prognosis: a report of 45 cases. J Pediatr Surg; 2009 Sep;44(9):1759-65
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  • BACKGROUND/PURPOSE: Ovarian torsion in childhood and adolescence is a rare entity.
  • Underlying pathology was benign in 22 cases and low-grade malignancy in 2 (one grade II immature teratoma and one steroid cell tumor).

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  • (PMID = 19735822.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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64. Hwang IG, Yoo KH, Lee SH, Park YH, Lim TK, Lee SC, Park S, Park BB, Ko YH, Kim K, Koo HH, Kim WS: Clinicopathologic features and treatment outcomes in malignant lymphoma of pediatric and young adult patients in Korea: comparison of korean all-ages group and Western younger age group. Clin Lymphoma Myeloma; 2007 Nov;7(9):580-6
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  • [Title] Clinicopathologic features and treatment outcomes in malignant lymphoma of pediatric and young adult patients in Korea: comparison of korean all-ages group and Western younger age group.
  • PURPOSE: The aim of this study is to define distinctive clinicopathologic features of malignant lymphoma in pediatric and young adult patients, particularly in Korea.
  • PATIENTS AND METHODS: From May 1993 to November 2005, 294 pediatric and young adult patients (age range, 0-31 years) with malignant lymphoma were analyzed in this study at Samsung Medical Center.
  • Among patients with non-Hodgkin lymphoma (NHL), T/natural killer cell immunophenotype is more common in the present younger age group than the all-ages group (45.5% vs. 25%; P = .001) and Western younger age group (45.5% vs. 13.3%; P = .001).
  • Lymphoblastic lymphoma and T-anaplastic large-cell lymphoma included relatively higher proportions in the younger age group.
  • CONCLUSION: The incidence of Hodgkin disease and T-cell NHL is relatively higher in pediatric and young-adult population group than the all-ages group.
  • However, treatment outcome of the younger age group, excluding lymphoblastic lymphoma, seems to be similar to those in any age group.

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  • (PMID = 18186966.001).
  • [ISSN] 1557-9190
  • [Journal-full-title] Clinical lymphoma & myeloma
  • [ISO-abbreviation] Clin Lymphoma Myeloma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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65. Bhuiyan ZA, Al-Shahrani S, Al-Khadra AS, Al-Ghamdi S, Al-Khalaf K, Mannens MM, Wilde AA, Momenah TS: Clinical and genetic analysis of long QT syndrome in children from six families in Saudi Arabia: are they different? Pediatr Cardiol; 2009 May;30(4):490-501
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  • Onset of arrhythmia was more severe in the recessive carriers and occurred during early childhood in all recessive LQT1 patients.

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  • (PMID = 19184172.001).
  • [ISSN] 1432-1971
  • [Journal-full-title] Pediatric cardiology
  • [ISO-abbreviation] Pediatr Cardiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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66. Günşar C, Vatansever S, Var A, Aygören R, Yilmaz O, Türköz E, Sencan A, Mir E: Antibiotic treatment is superior to ursodeoxycholic acid on total parenteral nutrition associated hepatic dysfunction. Pediatr Surg Int; 2010 May;26(5):479-86
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  • All TUNEL-positive cell number data were statistically significant except between G2 and G3(p < 0.05).

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  • (PMID = 20405274.001).
  • [ISSN] 1437-9813
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Fas Ligand Protein; 0 / Gentamicins; 0 / bcl-2-Associated X Protein; 140QMO216E / Metronidazole; 4Y8F71G49Q / Malondialdehyde; 724L30Y2QR / Ursodeoxycholic Acid; EC 1.11.1.7 / Peroxidase; EC 3.4.22.- / Caspase 3; RFM9X3LJ49 / Bilirubin
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67. Al Balushi Z, Bulduc S, Mulleur C, Lallier M: Desmoplastic small round cell tumor in children: a new therapeutic approach. J Pediatr Surg; 2009 May;44(5):949-52
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  • [Title] Desmoplastic small round cell tumor in children: a new therapeutic approach.
  • PURPOSE: Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histologic and immunohistochemical features occurring in a young population with a male predominance.
  • [MeSH-major] Abdominal Neoplasms / therapy. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow Transplantation. Pelvic Neoplasms / therapy. Radiotherapy, Adjuvant. Sarcoma, Small Cell / therapy

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  • (PMID = 19433176.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / EWS1-WT1 fusion protein, human; 0 / Oncogene Proteins, Fusion; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; BG3F62OND5 / Carboplatin; UM20QQM95Y / Ifosfamide
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68. Boncimino A, Bertaina A, Locatelli F: Cord blood transplantation in patients with hemoglobinopathies. Transfus Apher Sci; 2010 Jun;42(3):277-81
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  • Despite the optimization of conventional treatment, both thalassemia and sickle cell disease are still associated with significant morbidity and mortality, especially in developing countries.
  • [MeSH-major] Anemia, Sickle Cell / therapy. Cord Blood Stem Cell Transplantation. Directed Tissue Donation. Thalassemia / therapy

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  • [Copyright] Copyright 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 20382570.001).
  • [ISSN] 1473-0502
  • [Journal-full-title] Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis
  • [ISO-abbreviation] Transfus. Apher. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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69. Terui E, Matsunaga T, Yoshida H, Kouchi K, Kuroda H, Hishiki T, Saito T, Yamada S, Shirasawa H, Ohnuma N: Shc family expression in neuroblastoma: high expression of shcC is associated with a poor prognosis in advanced neuroblastoma. Clin Cancer Res; 2005 May 1;11(9):3280-7
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  • The biological features and prognosis of neuroblastoma, a neural crest-derived pediatric tumor, are closely associated with expression of the Trk receptor.
  • Because the Shc family proteins (ShcA, ShcB, and ShcC) are adaptors for various receptors, including Trk receptors, and are regulators of neuronal cell development, we speculated that they may play a role in neuroblastoma.
  • Therefore, in this study, we used semiquantitative reverse transcription-PCR to examine the expression of these three genes in 15 neuroblastoma cell lines, an all-trans-retinoic acid-treated neuroblastoma cell line, and 52 tumor samples.
  • In neuroblastoma cell lines and tumor samples, shcA was ubiquitously and highly expressed.
  • Also, shcB was hardly expressed in neuroblastoma cell lines, but its expression in RT-BM-1 cells was enhanced after all-trans-retinoic acid-induced differentiation, and it was highly expressed in low-stage tumors (P = 0.0095).
  • Finally, the expression of shcC was observed in most of the neuroblastoma cell lines and in some stage 4 patients.
  • [MeSH-minor] Cell Differentiation / drug effects. Cell Differentiation / genetics. Cell Line, Tumor. Child. Child, Preschool. Female. Gene Expression Regulation, Neoplastic. Humans. Infant. Male. Nuclear Proteins / genetics. Oncogene Proteins / genetics. Prognosis. RNA, Messenger / genetics. RNA, Messenger / metabolism. Receptor, trkA / genetics. Receptor, trkB / genetics. Reverse Transcriptase Polymerase Chain Reaction. Shc Signaling Adaptor Proteins. Survival Analysis. Survival Rate. Tretinoin / pharmacology

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  • (PMID = 15867224.001).
  • [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
  • [Chemical-registry-number] 0 / Adaptor Proteins, Signal Transducing; 0 / MYCN protein, human; 0 / Nuclear Proteins; 0 / Oncogene Proteins; 0 / RNA, Messenger; 0 / SHC1 protein, human; 0 / SHC2 protein, human; 0 / Shc Signaling Adaptor Proteins; 5688UTC01R / Tretinoin; EC 2.7.10.1 / Receptor, trkA; EC 2.7.10.1 / Receptor, trkB
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70. Herrmann D, Seitz G, Warmann SW, Bonin M, Fuchs J, Armeanu-Ebinger S: Cetuximab promotes immunotoxicity against rhabdomyosarcoma in vitro. J Immunother; 2010 Apr;33(3):279-86
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  • Multidrug resistance is a common problem in the treatment of childhood rhabdomyosarcoma (RMS).
  • Expression of EGFR and binding of its specific antibody Cetuximab to embryonal RMS cell lines RD and A-204 and alveolar RMS Rh30 were monitored by flow cytometry.
  • However, cell dependent cytotoxicity of PBMCs to RMS cells such as Rh30 and RD was enhanced specifically by Cetuximab.
  • [MeSH-major] Antibodies, Monoclonal / pharmacology. Antibody-Dependent Cell Cytotoxicity / drug effects. Rhabdomyosarcoma, Alveolar / immunology. Rhabdomyosarcoma, Embryonal / immunology
  • [MeSH-minor] Antibodies, Monoclonal, Humanized. Antineoplastic Agents / immunology. Antineoplastic Agents / pharmacology. Cell Line, Tumor. Cell Proliferation / drug effects. Cetuximab. Dose-Response Relationship, Drug. Gene Expression Profiling. Gene Expression Regulation, Neoplastic / drug effects. Gene Regulatory Networks. Humans. Leukocytes, Mononuclear / cytology. Leukocytes, Mononuclear / drug effects. Leukocytes, Mononuclear / immunology. Models, Genetic. Oligonucleotide Array Sequence Analysis. Receptor, Epidermal Growth Factor / genetics. Receptor, Epidermal Growth Factor / immunology

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  • (PMID = 20445348.001).
  • [ISSN] 1537-4513
  • [Journal-full-title] Journal of immunotherapy (Hagerstown, Md. : 1997)
  • [ISO-abbreviation] J. Immunother.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antineoplastic Agents; EC 2.7.10.1 / Receptor, Epidermal Growth Factor; PQX0D8J21J / Cetuximab
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71. Vangipuram SD, Wang ZJ, Lyman WD: Resistance of stem-like cells from neuroblastoma cell lines to commonly used chemotherapeutic agents. Pediatr Blood Cancer; 2010 Mar;54(3):361-8
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  • [Title] Resistance of stem-like cells from neuroblastoma cell lines to commonly used chemotherapeutic agents.
  • BACKGROUND: Cancer stem cell theory suggests that the presence of tumor initiating stem-like cells in cancers may be responsible for cancer progression and relapse.
  • CD133 cell surface maker expression has been used to identify stem-like cells in cancer cell lines.
  • Our goal was to identify such cells in neuroblastoma cell lines and to study the cytotoxicity of common anticancer drugs for those cells.
  • MATERIALS AND METHODS: CD133+ cells from SK-N-SH and SK-N-BE cell lines were isolated using magnetic microbeads.
  • [MeSH-minor] Antigens, CD / biosynthesis. Blotting, Western. Cell Line, Tumor. Dose-Response Relationship, Drug. Drug Resistance, Neoplasm. Drug Screening Assays, Antitumor. Glycoproteins / biosynthesis. Humans. Peptides. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / pathology


72. Sharma S, Free A, Mei Y, Peiper SC, Wang Z, Cowell JK: Distinct molecular signatures in pediatric infratentorial glioblastomas defined by aCGH. Exp Mol Pathol; 2010 Oct;89(2):169-74
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  • [Title] Distinct molecular signatures in pediatric infratentorial glioblastomas defined by aCGH.
  • To investigate this we performed high resolution array comparative genomic hybridization (aCGH) analysis on three pediatric infratentorial GBM, ages 3.5, 7 and 14 years.
  • While histologically typical, one brainstem tumor showed mainly pleomorphic astrocytic cells, whereas the other brainstem and spinal tumors showed a GFAP positive small cell component.
  • Segmental loss involving chromosome 8 was seen in all three tumors (Chr8;133039446-136869494, Chr8;pter-3581577, and Chr8;pter-30480019 respectively), whereas loss involving chromosome 16 was seen in only 2 cases with small cell components (Chr16;31827239-qter and Chr16;pter-29754532).
  • None of the frequent losses, gains and amplifications known to occur in adult GBM were identified, suggesting that pediatric infratentorial glioblastomas show a molecular karyotype that was more characteristic of pediatric embryonal tumors than adult GBM.

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  • [Copyright] Copyright © 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20621092.001).
  • [ISSN] 1096-0945
  • [Journal-full-title] Experimental and molecular pathology
  • [ISO-abbreviation] Exp. Mol. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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73. de Fátima Soares M, Braga FT, da Rocha AJ, Lederman HM: Optic nerve infiltration by acute lymphoblastic leukemia: MRI contribution. Pediatr Radiol; 2005 Aug;35(8):799-802
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  • [Title] Optic nerve infiltration by acute lymphoblastic leukemia: MRI contribution.
  • We describe the clinical presentation and imaging features of a patient with acute lymphoblastic leukemia (ALL) that was complicated by optic nerve infiltration.
  • [MeSH-major] Optic Nerve Diseases / etiology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / complications

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  • (PMID = 15815901.001).
  • [ISSN] 0301-0449
  • [Journal-full-title] Pediatric radiology
  • [ISO-abbreviation] Pediatr Radiol
  • [Language] eng
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  • [Publication-country] Germany
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74. Hendershot E: Treatment approaches for metastatic Ewing's sarcoma: a review of the literature. J Pediatr Oncol Nurs; 2005 Nov-Dec;22(6):339-52
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  • The Ewing's sarcoma family of tumors (ESFT) is an aggressive group of neoplasms that represent approximately 3% of all pediatric malignancies.
  • [MeSH-minor] Child. Humans. Immunotherapy. Male. Oncology Nursing. Risk Assessment. Risk Factors. Stem Cell Transplantation. Stem Cells

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  • (PMID = 16216896.001).
  • [ISSN] 1043-4542
  • [Journal-full-title] Journal of pediatric oncology nursing : official journal of the Association of Pediatric Oncology Nurses
  • [ISO-abbreviation] J Pediatr Oncol Nurs
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 43
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75. Smith SD, Fischer G: Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series. Pediatr Dermatol; 2009 Nov-Dec;26(6):725-9
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  • [Title] Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series.
  • However when it occurs in adult women it is accepted that remission is unlikely and that in addition untreated or inadequately treated disease may be complicated by significant disturbance of vulvar architecture and less commonly squamous cell carcinoma.
  • Even when diagnosed early and treated effectively, childhood onset lichen sclerosus may be complicated by distortion of vulvar architecture.

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  • (PMID = 20199450.001).
  • [ISSN] 1525-1470
  • [Journal-full-title] Pediatric dermatology
  • [ISO-abbreviation] Pediatr Dermatol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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76. Addeo R, Caraglia M, Baldi A, D'Angelo V, Casale F, Crisci S, Abbruzzese A, Vincenze B, Campioni M, Di Tullio MT, Indolfi P: Prognostic role of bcl-xL and p53 in childhood acute lymphoblastic leukemia (ALL). Cancer Biol Ther; 2005 Jan;4(1):32-8
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  • [Title] Prognostic role of bcl-xL and p53 in childhood acute lymphoblastic leukemia (ALL).
  • Molecular parameters involved in the prediction of response of childhood acute lymphoblastic leukemia (ALL) are still unclear.
  • [MeSH-major] Gene Expression Profiling. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics. Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology. Proto-Oncogene Proteins c-bcl-2 / biosynthesis. Tumor Suppressor Protein p53 / biosynthesis


77. Krishnamoorthy S: Receptor tyrosine kinase (RTK) mediated tyrosine phosphor-proteome from Drosophila S2 (ErbB1) cells reveals novel signaling networks. PLoS One; 2008 Aug 06;3(8):e2877
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  • [Title] Receptor tyrosine kinase (RTK) mediated tyrosine phosphor-proteome from Drosophila S2 (ErbB1) cells reveals novel signaling networks.
  • Protein phosphorylation mediates many critical cellular responses and is essential for many biological functions during development.
  • About one-third of cellular proteins are phosphorylated, representing the phosphor-proteome, and phosphorylation can alter a protein's function, activity, localization and stability.
  • Tyrosine phosphorylation events mediated by aberrant activation of Receptor Tyrosine Kinase (RTK) pathways have been proven to be involved in the development of several diseases including cancer.
  • To understand the systems biology of RTK activation, we have developed a phosphor-proteome focused on tyrosine phosphorylation events under insulin and EGF signaling pathways using the PhosphoScan technique coupled with high-throughput mass spectrometry analysis.
  • Comparative proteomic analyses of all these tyrosine phosphorylation events revealed that around 70% of these pY events are conserved in human orthologs and paralogs.
  • A careful analysis of published in vivo tyrosine phosphorylation events from literature and patents revealed that around 38% of pY events from Drosophila proteins conserved on 185 human proteins are confirmed in vivo tyrosine phosphorylation events.
  • Hence the data are validated partially based on available reports, and the credibility of the remaining 62% of novel conserved sites that are unpublished so far is very high but requires further follow-up studies.
  • The novel pY events found in this study that are conserved on human proteins could potentially lead to the discovery of drug targets and biomarkers for the detection of various cancers and neurodegenerative diseases.

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  • (PMID = 18682802.001).
  • [ISSN] 1932-6203
  • [Journal-full-title] PloS one
  • [ISO-abbreviation] PLoS ONE
  • [Language] ENG
  • [Grant] United States / NICHD NIH HHS / HD / P01 HD039942; United States / NIGMS NIH HHS / GM / R01 GM061707; United States / NICHD NIH HHS / HD / P01-HD-39942; United States / NIGMS NIH HHS / GM / R01 GM-61707
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Drosophila Proteins; 0 / Insulin; 0 / Phosphopeptides; 0 / Phosphoproteins; 0 / Proteome; 62229-50-9 / Epidermal Growth Factor; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor, Epidermal Growth Factor
  • [Other-IDs] NLM/ PMC2488400
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78. Bonanomi S, Gaiero A, Masera N, Rovelli A, Uderzo C, Fichera G, Mulas R, Zecca S, Pozzi L, Cohen A: Distinctive characteristics of diabetes mellitus after hematopoietic cell transplantation during childhood. Pediatr Transplant; 2006 Jun;10(4):461-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Distinctive characteristics of diabetes mellitus after hematopoietic cell transplantation during childhood.
  • We report on six patients who developed diabetes mellitus after hematopoietic cell transplantation (HCT).
  • [MeSH-major] Cell Transplantation. Diabetes Mellitus / diagnosis. Hematopoietic System


79. Kleinschmidt-DeMasters BK, Lovell MA, Donson AM, Wilkinson CC, Madden JR, Addo-Yobo SO, Lillehei KO, Foreman NK: Molecular array analyses of 51 pediatric tumors shows overlap between malignant intracranial ectomesenchymoma and MPNST but not medulloblastoma or atypical teratoid rhabdoid tumor. Acta Neuropathol; 2007 Jun;113(6):695-703
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  • [Title] Molecular array analyses of 51 pediatric tumors shows overlap between malignant intracranial ectomesenchymoma and MPNST but not medulloblastoma or atypical teratoid rhabdoid tumor.
  • Intracranial malignant ectomesenchymoma (MEM) is a pediatric tumor postulated to arise from neural crest cells that contain divergent neuroectodermal and mesenchymal tissues, principally mature ganglion cells and rhabdomyosarcoma (RMS).
  • We investigated a case of MEM by molecular, cytogenetic, and gene array analyses and compared results with our previously unpublished series of 51 pediatric tumors including conventional RMS, Ewing sarcoma (EWS), medulloblastoma (MED), atypical teratoid rhabdoid tumor (ATRT), and malignant peripheral nerve sheath tumor (MPNST); the latter is a sarcoma also with potential for divergent differentiation.
  • Gene expression microarray analyses showed tight clustering of the MEM with the MPNST (n = 2), but divergence from other pediatric tumors.
  • This suggests a common stem cell origin or embryonic gene recapitulation for these tumors and provides novel insights into their underlying biology.


80. Cajaiba MM, Reyes-Múgica M: [Renal tumors of childhood and adolescence associated with chromosomal anomalies]. Actas Urol Esp; 2007 Oct;31(9):966-77
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  • [Title] [Renal tumors of childhood and adolescence associated with chromosomal anomalies].
  • The pediatric kidney is a common site for tumors carrying specific chomosomal alterations.
  • Other neoplasms with chromosomal rearrangements, such as Renal Cell carcinomas (CRs) are much less frequent in children (between 1.8 and 6.3% of all malignant renal tumors).

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  • (PMID = 18257366.001).
  • [ISSN] 0210-4806
  • [Journal-full-title] Actas urologicas españolas
  • [ISO-abbreviation] Actas Urol Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 62
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81. Kerkar N: Hepatitis B in children: complexities in management. Pediatr Transplant; 2005 Oct;9(5):685-91
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  • Efficient and multi-specific helper and cytotoxic T-cell response is essential for controlling HBV infection.
  • Chronic HBV infection is characterized by a state of HBV-specific T-cell hyporesponsiveness.
  • Future treatments should be based on the restoration of HBV-specific T-cell responses to levels similar to that seen in subjects controlling HBV.

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  • (PMID = 16176431.001).
  • [ISSN] 1397-3142
  • [Journal-full-title] Pediatric transplantation
  • [ISO-abbreviation] Pediatr Transplant
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antiviral Agents
  • [Number-of-references] 63
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82. Schraders M, van Reijmersdal SV, Kamping EJ, van Krieken JH, van Kessel AG, Groenen PJ, Hoogerbrugge PM, Kuiper RP: High-resolution genomic profiling of pediatric lymphoblastic lymphomas reveals subtle differences with pediatric acute lymphoblastic leukemias in the B-lineage. Cancer Genet Cytogenet; 2009 May;191(1):27-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] High-resolution genomic profiling of pediatric lymphoblastic lymphomas reveals subtle differences with pediatric acute lymphoblastic leukemias in the B-lineage.
  • Lymphoblastic lymphoma (LBL) is one of the most frequent occurring pediatric non-Hodgkin lymphomas.
  • In the WHO classification scheme, pediatric LBL is considered to be the same disease entity as pediatric acute lymphoblastic leukemia (ALL).
  • However, it is unclear whether the genetic basis of pediatric LBL development is similar to that of pediatric ALL.
  • We performed genome-wide analyses of copy number aberrations in 12 T-LBL and 7 precursor B-cell LBL pediatric cases using high-resolution SNP-based array CGH.
  • These results show that, similar to T-ALL, the genomic alterations in T-LBL predominantly target genes involved in cell cycle progression.
  • The majority of precursor B-cell LBL was characterized by high-hyperdiploidy (71%), and showed high resemblance with high-hyperdiploid precursor B-cell ALL.
  • Taken together, our data suggest that pediatric LBL and ALL exhibit similar genomic abnormalities within confined immunophenotypic and cytogenetic subgroups, but that the representations of these subgroups differs between the two entities.
  • [MeSH-major] B-Lymphocytes / pathology. Cell Lineage. Gene Expression Profiling. Genome, Human / genetics. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics. Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology
  • [MeSH-minor] Cell Cycle. Child. Chromosomes, Human, Pair 13 / genetics. Chromosomes, Human, Pair 9 / genetics. Gene Duplication. Gene Expression Regulation, Leukemic. Humans. Polyploidy. Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / genetics. Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / pathology. T-Lymphocytes / pathology

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  • (PMID = 19389505.001).
  • [ISSN] 1873-4456
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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83. Koyama S, Cobb LJ, Mehta HH, Seeram NP, Heber D, Pantuck AJ, Cohen P: Pomegranate extract induces apoptosis in human prostate cancer cells by modulation of the IGF-IGFBP axis. Growth Horm IGF Res; 2010 Feb;20(1):55-62
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  • The IGF axis is critical for the regulation of apoptosis in many human cancer cell lines.
  • Treatment of LAPC4 prostate cancer cells with 10microg/ml POMx, a highly potent pomegranate extract prepared from skin and arils minus seeds and standardized to ellagitannin content (37% punicalagins by HPLC), resulted in inhibition of cell proliferation and induction of apoptosis.
  • Interestingly, co-treatment with POMx and IGFBP-3 revealed synergistic stimulation of apoptosis and additive inhibition of cell growth.

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  • [Copyright] Copyright 2009 Elsevier Ltd. All rights reserved.
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  • (PMID = 19853487.001).
  • [ISSN] 1532-2238
  • [Journal-full-title] Growth hormone & IGF research : official journal of the Growth Hormone Research Society and the International IGF Research Society
  • [ISO-abbreviation] Growth Horm. IGF Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P50 CA092131-08; United States / NIA NIH HHS / AG / AG020954-04; United States / NCI NIH HHS / CA / 1R01CA100938; United States / NCI NIH HHS / CA / P50 CA092131; United States / NCI NIH HHS / CA / R01 CA100938-05; United States / NIA NIH HHS / AG / R01 AG020954-04; United States / NIA NIH HHS / AG / R01 AG020954; United States / NCI NIH HHS / CA / CA092131-01A10003; United States / NICHD NIH HHS / HD / R01 HD047013; United States / NICHD NIH HHS / HD / R01HD047013; United States / NCI NIH HHS / CA / R01 CA100938; United States / NCI NIH HHS / CA / P50CA92131; United States / NCI NIH HHS / CA / P50 CA092131-01A10003
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Scotland
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 0 / Hydrolyzable Tannins; 0 / Insulin-Like Growth Factor Binding Protein 3; 0 / Intracellular Signaling Peptides and Proteins; 0 / Plant Extracts; 0 / ellagitannin; 67763-96-6 / Insulin-Like Growth Factor I; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 2.7.11.24 / JNK Mitogen-Activated Protein Kinases
  • [Other-IDs] NLM/ NIHMS154342; NLM/ PMC2815223
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84. Van Vlierberghe P, Palomero T, Khiabanian H, Van der Meulen J, Castillo M, Van Roy N, De Moerloose B, Philippé J, González-García S, Toribio ML, Taghon T, Zuurbier L, Cauwelier B, Harrison CJ, Schwab C, Pisecker M, Strehl S, Langerak AW, Gecz J, Sonneveld E, Pieters R, Paietta E, Rowe JM, Wiernik PH, Benoit Y, Soulier J, Poppe B, Yao X, Cordon-Cardo C, Meijerink J, Rabadan R, Speleman F, Ferrando A: PHF6 mutations in T-cell acute lymphoblastic leukemia. Nat Genet; 2010 Apr;42(4):338-42
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  • [Title] PHF6 mutations in T-cell acute lymphoblastic leukemia.
  • T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive hematological malignancy with an increased incidence in males.
  • In this study, we report the identification of inactivating mutations and deletions in the X-linked plant homeodomain finger 6 (PHF6) gene in 16% of pediatric and 38% of adult primary T-ALL samples.

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  • (PMID = 20228800.001).
  • [ISSN] 1546-1718
  • [Journal-full-title] Nature genetics
  • [ISO-abbreviation] Nat. Genet.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA120196-03; United States / NIAID NIH HHS / AI / U54-AI057158; United States / NCI NIH HHS / CA / R01 CA129382; United States / NCI NIH HHS / CA / R01 CA129382-03; United States / NCI NIH HHS / CA / CA129382-03; United States / NCI NIH HHS / CA / CA120196-03; United States / NCI NIH HHS / CA / R01CA120196; United States / NIAID NIH HHS / AI / U54 AI057158; United States / NCI NIH HHS / CA / R01CA129382; United States / NCI NIH HHS / CA / R01 CA120196; United States / NLM NIH HHS / LM / 1R01LM010140-01; United States / NCI NIH HHS / CA / U24 CA114737; United States / NLM NIH HHS / LM / R01 LM010140; United States / NCI NIH HHS / CA / R01 CA155743
  • [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 / Carrier Proteins; 0 / Homeodomain Proteins; 0 / PHF6 protein, human; 0 / Proto-Oncogene Proteins; 0 / TLX3 protein, human; 143275-75-6 / TLX1 protein, human
  • [Other-IDs] NLM/ NIHMS176587; NLM/ PMC2847364
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85. Weintrob N, Stern E, Klipper-Aurbach Y, Phillip M, Gat-Yablonski G: Childhood obesity complicating the differential diagnosis of maturity-onset diabetes of the young and type 2 diabetes. Pediatr Diabetes; 2008 Feb;9(1):60-4
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  • [Title] Childhood obesity complicating the differential diagnosis of maturity-onset diabetes of the young and type 2 diabetes.
  • RESULTS: The proband showed elevated C-peptide level and was negative for beta-cell antibodies.


86. Wang J, Wu X, Xi ZJ: Langerhans cell histiocytosis of bone in children: a clinicopathologic study of 108 cases. World J Pediatr; 2010 Aug;6(3):255-9
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  • [Title] Langerhans cell histiocytosis of bone in children: a clinicopathologic study of 108 cases.
  • BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disease that is characterized by abnormal proliferation of pathological Langerhans cells (LCs).
  • In this study, a total of 108 pediatric patients with LCH of bone were evaluated retrospectively for illustrating the clinicopathologic features of this disease, with a goal of improving the diagnosis, treatment, and prognosis.
  • [MeSH-major] Bone Diseases / pathology. Histiocytosis, Langerhans-Cell / pathology


87. Brown VI, Seif AE, Reid GS, Teachey DT, Grupp SA: Novel molecular and cellular therapeutic targets in acute lymphoblastic leukemia and lymphoproliferative disease. Immunol Res; 2008;42(1-3):84-105
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  • [Title] Novel molecular and cellular therapeutic targets in acute lymphoblastic leukemia and lymphoproliferative disease.
  • While the outcome for pediatric patients with lymphoproliferative disorders (LPD) or lymphoid malignancies, such as acute lymphoblastic leukemia (ALL), has improved dramatically, patients often suffer from therapeutic sequelae.
  • (ii) block the interaction of ALL cells with stromal cells or lymphoid growth factors secreted by the bone marrow microenvironment; or (iii) stimulate innate and adaptive immune responses.

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  • (PMID = 18716718.001).
  • [ISSN] 0257-277X
  • [Journal-full-title] Immunologic research
  • [ISO-abbreviation] Immunol. Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / NIH 1 K08 CA104882-01A1; United States / NCI NIH HHS / CA / K08 CA104882; United States / NCI NIH HHS / CA / NIH CA102646; United States / NCI NIH HHS / CA / K08 CA104882-05; United States / NCI NIH HHS / CA / R01 CA102646; United States / NCI NIH HHS / CA / NIH R03 CA123554; United States / NCI NIH HHS / CA / CA104882-05; United States / NCI NIH HHS / CA / R03 CA123554; United States / NCI NIH HHS / CA / CA1116660
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adjuvants, Immunologic; 0 / Enzyme Inhibitors; 0 / Receptor, Notch1; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases
  • [Number-of-references] 158
  • [Other-IDs] NLM/ NIHMS209775; NLM/ PMC2890314
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88. Lang P, Handgretinger R: Haploidentical SCT in children: an update and future perspectives. Bone Marrow Transplant; 2008 Oct;42 Suppl 2:S54-9
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  • This review focuses on current results and new strategies, especially in pediatric patients with malignant diseases.
  • CD34(+) positive selection was the most common procedure for graft manipulation in the past years, whereas T and B cell depletion is a promising new method.
  • GVHD could herewith be effectively reduced and primary engraftment was reported in 83-100% of patients after transplantation of high stem cell doses.
  • TRM, mainly because of viral infections, was improved by the use of reduced-intensity conditioning (which helped to speed up T cell recovery) and by close monitoring of viral loads and prophylactic/preemptive therapy.
  • [MeSH-major] Lymphocyte Depletion / methods. Precursor Cell Lymphoblastic Leukemia-Lymphoma / therapy. Stem Cell Transplantation. Transplantation Conditioning / methods

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  • (PMID = 18978746.001).
  • [ISSN] 1476-5365
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Number-of-references] 57
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89. Karakas Z, Tugcu D, Unuvar A, Atay D, Akcay A, Gedik H, Kayserili H, Dogan O, Anak S, Devecioglu O: Li-Fraumeni syndrome in a Turkish family. Pediatr Hematol Oncol; 2010 May;27(4):297-305
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  • The adrenocortical carcinoma (ACC) association with acute leukemia is unusual in childhood, even in LFS.
  • The authors here present a family with pR337P mutation in TP53 gene who had a child with acute lymphoblastic leukemia (ALL) and associated adrenocortical carcinoma as a case 1 and his cousin with brain tumor as a case 2.
  • [MeSH-major] Adrenocortical Carcinoma / genetics. Brain Neoplasms / genetics. Li-Fraumeni Syndrome / genetics. Mutation, Missense. Neoplasms, Second Primary / genetics. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics. Tumor Suppressor Protein p53 / genetics

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  • (PMID = 20426520.001).
  • [ISSN] 1521-0669
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53
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90. Connell MG, Corbett HJ, Purvis A, Losty PD, Jesudason EC: Sex and congenital diaphragmatic hernia. Pediatr Surg Int; 2006 Jan;22(1):95-8
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  • [Title] Sex and congenital diaphragmatic hernia.
  • BACKGROUND AND PURPOSE: Human studies note sex reversal syndromes and sex difference(s) in the incidence of congenital diaphragmatic hernia (CDH).
  • Epidemiology surveys record a higher incidence of CDH in females, whilst other reports cite a higher frequency in males.
  • Nitrofen, a teratogen, produces experimental CDH.
  • This agent is speculated to interfere with retinoid acid-steroid signalling pathways and may also be linked with sexual differentiation.
  • This study was designed therefore to test the hypothesis that nitrofen may influence sexual phenotype and frequency of CDH.
  • METHODS: Time mated Sprague Dawley rats were dosed with nitrofen at day 9.5 to generate predominantly left sided CDH.
  • Fetuses were delivered by caesarean section on days 20 or 21 of gestation (term=day 22).
  • External genitalia were examined to define external genital phenotype.
  • The abdominal cavity was opened and the genito-urinary system carefully examined.
  • The internal genital organs were assigned a phenotype and findings correlated with external appearances.
  • The diaphragm of each fetus was studied for the absence or presence of CDH and the laterality of defect recorded.
  • Controls (non nitrofen fed) were used for all comparative analysis.
  • RESULTS: Control (n=600) and nitrofen exposed offspring (n=504) had equal frequencies of males and females.
  • CDH occurred with similar incidence in male and female nitrofen treated pups.
  • In all nitrofen exposed fetuses and normal controls, internal and external genitalia concorded without evidence of significant genital tract malformations or intersex states.
  • CONCLUSIONS: Prenatal nitrofen exposure is not associated with significant gender differences (or prenatal loss) in the risk of CDH.
  • Genital tract malformations do not appear to accompany CDH in the nitrofen model.
  • [MeSH-major] Disorders of Sex Development. Hernia, Diaphragmatic / physiopathology. Hernias, Diaphragmatic, Congenital. Phenyl Ethers / pharmacology. Sex Differentiation / drug effects
  • [MeSH-minor] Animals. Disease Models, Animal. Female. Male. Maternal Exposure. Pregnancy. Rats. Rats, Sprague-Dawley

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  • (PMID = 16292652.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Grant] United Kingdom / Medical Research Council / / G0802057; United Kingdom / Medical Research Council / / G84/6485
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Phenyl Ethers; N71UYG034A / nitrofen
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91. Schiffman JD, Chun N, Fisher PG, Dahl GV, Ford JM, Eggerding FA: Identification of a novel p53 in-frame deletion in a Li-Fraumeni-like family. Pediatr Blood Cancer; 2008 Apr;50(4):914-6
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  • We describe a 2-year-old female with a completely resected cerebral pilocytic astrocytoma who subsequently developed B-progenitor acute lymphoblastic leukemia (ALL).
  • [MeSH-minor] Astrocytoma / genetics. Base Sequence. Brain Neoplasms / genetics. Child, Preschool. DNA Mutational Analysis. Female. Germ-Line Mutation. Humans. Male. Middle Aged. Pedigree. Polymerase Chain Reaction. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 17554785.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Tumor Suppressor Protein p53
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92. Brandow AM, Brousseau DC, Pajewski NM, Panepinto JA: Vaso-occlusive painful events in sickle cell disease: impact on child well-being. Pediatr Blood Cancer; 2010 Jan;54(1):92-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Vaso-occlusive painful events in sickle cell disease: impact on child well-being.
  • BACKGROUND: This study describes how painful events affect the health-related quality of life (HRQL) of children with sickle cell disease (SCD) and determines the responsiveness of a generic HRQL measure in SCD.
  • (1) HRQL is significantly impaired at presentation to the emergency department for a painful event and (2) PedsQL 4.0 Acute Version Generic Core Scales is responsive to change in the evolution of a painful event.
  • HRQL was measured with the Acute Version of the PedsQL 4.0 Generic Core Scales, completed by child (self-report) and caregiver (proxy report) at presentation and 7 days post-discharge.

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  • [Copyright] Copyright 2009 Wiley-Liss, Inc.
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  • (PMID = 19653296.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] ENG
  • [Grant] United States / NHLBI NIH HHS / HL / K23 HL080092; United States / NHLBI NIH HHS / HL / K23 HL80092; United States / NHLBI NIH HHS / HL / K23 HL080092-05; United States / NHLBI NIH HHS / HL / T32 HL007209; United States / NHLBI NIH HHS / HL / T32 HL072757
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS144359; NLM/ PMC3114448
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93. Buccoliero AM, Castiglione F, Rossi Degl'Innocenti D, Ammanati F, Giordano F, Sanzo M, Mussa F, Genitori L, Taddei GL: Hox-D genes expression in pediatric low-grade gliomas: real-time-PCR study. Cell Mol Neurobiol; 2009 Feb;29(1):1-6
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  • [Title] Hox-D genes expression in pediatric low-grade gliomas: real-time-PCR study.
  • HOX genes encode transcription factors, which play a key role in morphogenesis and cell differentiation during embryogenesis.
  • Actually, several HOX genes are aberrantly expressed in many tumors and cell lines derived from them.
  • In the present work, we study the relative expression of HOX-D genes (D1, D3, D4, D8, D9, D10, D11, D12, D13) with real-time polymerase chain reaction in a group of 14 pediatric low-grade gliomas.
  • The observation that all but three HOX-D genes studied are expressed with different pattern in neoplastic and non-neoplastic brain tissue may support the hypothesis that HOX-D genes play a role in the pathogenesis of pediatric low-grade gliomas.

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  • [ISSN] 1573-6830
  • [Journal-full-title] Cellular and molecular neurobiology
  • [ISO-abbreviation] Cell. Mol. Neurobiol.
  • [Language] eng
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94. El-Sheikh A, Fan R, Birks D, Donson A, Foreman NK, Vibhakar R: Inhibition of Aurora Kinase A enhances chemosensitivity of medulloblastoma cell lines. Pediatr Blood Cancer; 2010 Jul 15;55(1):35-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Inhibition of Aurora Kinase A enhances chemosensitivity of medulloblastoma cell lines.
  • BACKGROUND: Medulloblastoma comprises approximately 20% of all primary pediatric brain tumors.
  • The objective of this study was to evaluate the impact of Aurora Kinase A inhibition in medulloblastoma cell lines.
  • PROCEDURE: Cell proliferation was measured using an MTS assay after adding an Aurora Kinase inhibitor (C1368) at different concentrations.
  • Cell cycle analysis was carried out by Flow Cytometry using propidium iodide (PI).
  • RESULTS: Inhibition of Aurora Kinase A induces cell death in medulloblastoma cells and lowers the IC(50) of other chemotherapeutic agents (etoposide and cisplatin) used in medulloblastoma treatment.
  • Cell arrest at G2/M phase was significantly increased in medulloblastoma cell lines treated with C1368 Sigma at IC(30) or transfected siRNA.
  • CONCLUSIONS: These data indicate that inhibition of Aurora Kinase A inhibits cell growth in medulloblastoma through inhibition of pro-proliferative signaling pathways Wnt, Myc, and RB.
  • [MeSH-minor] Aurora Kinase A. Aurora Kinases. Cell Cycle / drug effects. Cell Line, Tumor. Cell Proliferation / drug effects. Cell Survival / drug effects. Drug Screening Assays, Antitumor. Humans. RNA, Small Interfering / pharmacology. Signal Transduction / drug effects


95. Longaker MT: Regenerative medicine: a surgeon's perspective. J Pediatr Surg; 2010 Jan;45(1):11-7; discussion 17-8
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  • [Title] Regenerative medicine: a surgeon's perspective.
  • More than 200 million incisions are made in the world each year on children and adults.
  • They all end up with a scar unless there is an unusual situation where we are operating on an early gestation fetus.
  • The question is, "why do we not regenerate?
  • " and "why do we always heal with either a 'normal amount of scarring' or, approximately 15% of the time, with a pathologic amount of scarring (hypertrophic scar or keloid)? "

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
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  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] ENG
  • [Grant] United States / NIDCR NIH HHS / DE / R21 DE019274; United States / NIDCR NIH HHS / DE / DE018727-02; United States / NIDCR NIH HHS / DE / R21 DE019274-02; United States / NIDCR NIH HHS / DE / R21 DE018727-02; United States / NIDCR NIH HHS /