Document Detail


Mechanism underlying early anaemia in children with familial juvenile nephronophthisis.
MedLine Citation:
PMID:  8897559     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Familial juvenile nephronophthisis (NPH) is a hereditary form of chronic tubulointerstitial nephritis with onset in childhood. About one-third of patients develop anaemia before renal insufficiency. We investigated the pathogenetic mechanisms leading to anaemia by comparing 6 patients with NPH and 12 reference patients with other renal diseases. We studied their iron metabolism and measured transferrin receptor-ferritin ratios. There was no evidence for iron deficiency or haemolysis. The serum erythropoietin concentrations of the patients with NPH (12 +/- 2.3 U/I) were low compared with the 12 reference patients (25 +/- 18.9 U/I). In the 2 patients with NPH who were fully investigated, the pharmacokinetics of recombinant human erythropoietin appeared normal. Thus, anaemia in patients with NPH does not result from iron deficiency or correlate with impaired iron status. The mechanism underlying the anaemia of NPH appears to affect the function or regulation of the cells producing erythropoietin.
Authors:
S Ala-Mello; S M Kivivuori; K A Rönnholm; O Koskimies; M A Siimes
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Pediatric nephrology (Berlin, Germany)     Volume:  10     ISSN:  0931-041X     ISO Abbreviation:  Pediatr. Nephrol.     Publication Date:  1996 Oct 
Date Detail:
Created Date:  1997-02-06     Completed Date:  1997-02-06     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8708728     Medline TA:  Pediatr Nephrol     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  578-81     Citation Subset:  IM    
Affiliation:
Department of Medical Genetics, University of Helsinki, Finland.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Anemia / etiology*
Child
Erythropoietin / blood
Female
Humans
Iron / metabolism
Male
Nephritis, Interstitial / complications*,  genetics
Chemical
Reg. No./Substance:
11096-26-7/Erythropoietin; 7439-89-6/Iron

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


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