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Clinico-pathological features of the nephrotic syndrome in South African children
Insights
African children with nephrotic syndrome often have severe glomerular lesions unresponsive to steroids, unlike Indian children who typically have minimal change disease and respond well to steroids.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
- Immunopathology
Background:
- Nephrotic syndrome presents with diverse clinicopathological patterns.
- Understanding ethnic variations in nephrotic syndrome is crucial for effective management.
Purpose of the Study:
- To compare the clinicopathological patterns of nephrotic syndrome in African and Indian children.
- To investigate the association between histological types and steroid responsiveness in these populations.
Main Methods:
- Retrospective analysis of 130 children with nephrotic syndrome (74 African, 56 Indian).
- Histopathological examination of kidney biopsies.
- Correlation of histological findings with clinical presentation and treatment response.
Main Results:
- African children predominantly showed structural glomerular lesions (86%) with steroid unresponsiveness.
- Indian children mostly had minimal change nephrotic syndrome (75%) which was steroid responsive.
- Extramembranous and proliferative lesions were common in Africans; Indians showed patterns similar to other global populations, but with more frequent relapses.
Conclusions:
- Significant clinicopathological differences exist in nephrotic syndrome between African and Indian children.
- These differences impact steroid responsiveness and treatment strategies.
- The etiology of nephrotic syndrome remains largely unidentified in this cohort.
Abstract:
One hundred and thirty children of whom 74 were Africans and 56 Indians with contrasting clinicopathological patterns of the nephrotic syndrome are described. Eighty-six per cent of African children had obvious structural glomerular lesions which were associated with unresponsiveness to steroids while 75 per cent of Indians had minimal change nephrotic syndrome which was steroid responsive. The treatment history of a further 41 unbiopsied children with nephrotic syndrome (nine Africans, 32 Indians) support and emphasize this difference. Extramembranous and a tropical variety of extramembranous (36.5 per cent) together with proliferative (20.2 per cent) lesions accounted for most of the histological types in African children. The disease in Indian children was similar to that in other countries in age of onset, sex distribution, frequency of histological types and steroid responsiveness although there was a preponderance of frequent relapsers (69 per cent). Africans differed from children in other continents in the frequency of histological categories (therefore in steroid responsiveness) and occasionally in clinical behaviour. They also differed from children in tropical Africa in a lower incidence of the disease, male dominance and absence of malarial nephropathy. The aetiology of nephrotic syndrome in nearly all the children remains unidentified.