Document Detail


Nephrotic syndrome accompanying familial hemophagocytic syndrome.
MedLine Citation:
PMID:  8846138     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
PURPOSE: We describe the first reported case of familial hemophagocytic syndrome (FHS) with concurrent minimal change nephrotic syndrome (MCNS).
PATIENTS AND METHODS: This is a case report of a 30-month-old girl who presented to Children's Memorial Hospital with pancytopenia and heavy proteinuria.
RESULTS: This patient presented with anemia, neutropenia, thrombocytopenia, hypertriglyceridemia, and proteinuria. A brother died at 2 months of age with similar findings. A bone marrow biopsy demonstrated histiocyte proliferation with marked erythrophagocytosis, consistent with FHS. Treatment was begun with corticosteroids and VP-16. The patient developed worsening peripheral edema and hypoalbuminemia, with heavy proteinuria. After 1 month of therapy with persistence of heavy proteinuria, a renal biopsy was performed, the results of which were consistent with MCNS.
CONCLUSION: This is the first reported case of FHS with coincident MCNS.
Authors:
M C Braun; R A Cohn; M Kletzel
Related Documents :
9617498 - A case of immunotactoid glomerulopathy with unusual microtubular deposits.
20842148 - Corin: an anp protease that may regulate sodium reabsorption in nephrotic syndrome.
3306428 - Membranous nephropathy accompanied by angiolymphoid hyperplasia of the skin.
463838 - Congenital glomerulosclerosis and nephrotic syndrome in two infants. speculations and p...
325678 - Spina bifida and so-called asplenia syndrome occurring separately in sibs.
20631828 - Current hypotheses on how microsatellite instability leads to enhanced survival of lync...
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  18     ISSN:  1077-4114     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:  1996 May 
Date Detail:
Created Date:  1996-10-21     Completed Date:  1996-10-21     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  195-7     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois, USA.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Child, Preschool
Female
Histiocytosis, Non-Langerhans-Cell / complications*
Humans
Nephrotic Syndrome / complications*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Acute renal failure with hyperuricemia as initial presentation of leukemia in children.
Next Document:  Unusual presentation of Ewing sarcoma with t(21;22) in a 3-year-old boy.