Document Detail


Nephrotic syndrome in Nigerian children with homozygous sickle cell disease.
MedLine Citation:
PMID:  9557431     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Two hundred and seventy two children with the nephrotic syndrome were seen and followed up at the Paediatric Renal Unit of the University of Nigeria Teaching Hospital, Enugu over 12 1/2 year period, between June 1983 and December 1995. Of these, there were nine (3.3%) children with homozygous sickle cell disease (SS). This latter group had a mean age of 9.6 +/- 3.2 years, male to female ratio of 1:2 and serum cholesterol of 3.13 +/- 1.48 mmol/l at the time of diagnosis. The mortality rate was 55.6% in these sicklers, death occurring within one and a half to five years of diagnosis. Causes of death were attributable to sickle cell disease in 60%, renal diseases in 20%, and other causes in 20%. Renal biopsy in two of the sicklers showed membrano-proliferative glomerulonephritis (MPGN) and focal glomerulosclerosis (FGS) respectively. Homozygous sickle cell disease does seem to predispose to the development of the nephrotic syndrome and those that do develop nephrotic syndrome exhibit some special characteristics, when compared to non-sicklers with nephrotic syndrome. These include older age of onset of the nephrotic syndrome, reverse sex ratios, lower serum cholesterol, higher mortality rate and sickle cell complications rather than chronic renal failure as the major cause of death.
Authors:
B A Okoro; H U Okafor
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  East African medical journal     Volume:  74     ISSN:  0012-835X     ISO Abbreviation:  East Afr Med J     Publication Date:  1997 Dec 
Date Detail:
Created Date:  1998-05-14     Completed Date:  1998-05-14     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0372766     Medline TA:  East Afr Med J     Country:  KENYA    
Other Details:
Languages:  eng     Pagination:  819-21     Citation Subset:  IM    
Affiliation:
Department of Paediatrics, University of Nigeria Teaching Hospital, Enugu.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Anemia, Sickle Cell / complications*,  genetics,  mortality
Biopsy
Case-Control Studies
Causality
Cause of Death
Child
Female
Follow-Up Studies
Hospitals, Teaching
Humans
Male
Nephrotic Syndrome / etiology*,  pathology
Nigeria

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