Document Detail

Renal amyloidosis in juvenile chronic arthritis: evolution after chlorambucil treatment.
MedLine Citation:
PMID:  2242306     Owner:  NLM     Status:  MEDLINE    
Over a 22-year period, eight patients affected with severe systemic or polyarticular juvenile chronic arthritis (JCA) developed systemic amyloidosis with nephrotic syndrome. They were treated with chlorambucil over 5-192 months (mean = 44 months). With treatment, an abrupt decrease in the severity of JCA was observed in six patients but two patients were chlorambucil resistant. After a mean follow-up period of 10 years from onset of renal symptoms, one chlorambucil-resistant patient died of end-stage renal failure; two patients have a persistent nephrotic syndrome; and five patients are free from proteinuria, of whom one has developed hypertension. A good correlation was observed between the response of the rheumatic disease to chlorambucil treatment and the clinical course of renal symptoms. Fourteen renal biopsies were performed in these eight patients. In all, amyloid deposits were of the AA type, which persisted on repeat biopsies. In addition, 15%-60% of glomeruli had become globally sclerotic by the second or third biopsies. At the ultrastructural level, modifications in the structure of amyloid deposits and reparative changes of the glomeruli, characterized by partial restoration of glomerular architecture, were observed in three patients with a favourable clinical course.
G Deschênes; A M Prieur; F Hayem; M Broyer; M C Gubler
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatric nephrology (Berlin, Germany)     Volume:  4     ISSN:  0931-041X     ISO Abbreviation:  Pediatr. Nephrol.     Publication Date:  1990 Sep 
Date Detail:
Created Date:  1991-01-03     Completed Date:  1991-01-03     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8708728     Medline TA:  Pediatr Nephrol     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  463-9     Citation Subset:  IM    
Service de Néphrologie Pédiatrique, Hôpital Necker-Enfants-Malades, Paris, France.
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MeSH Terms
Amyloidosis / drug therapy*,  etiology,  pathology
Arthritis, Juvenile Rheumatoid / complications*
Chlorambucil / therapeutic use*
Follow-Up Studies
Kidney Diseases / drug therapy*,  etiology,  pathology
Microscopy, Electron
Retrospective Studies
Reg. No./Substance:

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

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