| Nephrotic syndrome associated with hypocomplementemia in a 4-year-old boy with hemophilia B. | |
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MedLine Citation:
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PMID: 9256835 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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PURPOSE: The case of a 4-year-old boy with hemophilia B with inhibitor who developed nephrotic syndrome is described. The possible association between factor IX therapy and nephrotic syndrome in patients with hemophilia B is discussed. PATIENT AND METHODS: A chart review of a 4-year-old boy with hemophilia B and an inhibitor who developed nephrotic syndrome with transient hypocomplementemia was performed. In addition, a literature search was undertaken to determine the prevalence of this association and possible etiologic factors. RESULTS: Although the nephrotic syndrome was resistant to steroid therapy and Bebulin (Osterreichisches Institut für Haemoderivate Ges.M.B.H., Subsidiary of Immuno AG, Vienna, Austria) infusions were continued, the edema resolved and proteinuria decreased. Seven month later, proteinuria, accompanied by transient hypocomplementemia, increased again. A rise in factor IX inhibitor level was observed. The patient received no immunosuppressive therapy, and exhibited a continuous decrease in urinary protein excretion over the following months. DISCUSSION: A discussion about possible differential diagnoses and a review of the literature are presented. |
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Authors:
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A R Constantinescu; L S Weiss; P Saidi; J Eisele; L J Ettinger |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of pediatric hematology/oncology Volume: 19 ISSN: 1077-4114 ISO Abbreviation: J. Pediatr. Hematol. Oncol. Publication Date: 1997 Jul-Aug |
Date Detail:
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Created Date: 1997-08-29 Completed Date: 1997-08-29 Revised Date: 2011-10-06 |
Medline Journal Info:
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Nlm Unique ID: 9505928 Medline TA: J Pediatr Hematol Oncol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 345-7 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, UMDNJ-Robert Wood Johnson Medical School, New Brunswick 08901, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Child, Preschool Complement Inactivator Proteins / metabolism Complement System Proteins / metabolism* Factor IX / adverse effects, therapeutic use Hemophilia B / blood*, complications* Humans Male Nephrotic Syndrome / blood*, complications* |
| Chemical | |
Reg. No./Substance:
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0/Complement Inactivator Proteins; 9001-28-9/Factor IX; 9007-36-7/Complement System Proteins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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