Absence of 'true' minimal change nephrotic syndrome in African children in South Africa.
Minimal change nephrotic syndrome (MCNS) which is a well defined clinical, histopathological and therapeutic entity accounts for the majority of childhood nephrosis throughout the world except Africa. We describe 15 (13%) of 115 biopsy-confirmed African children with nephrotic syndrome who had minimal change on light microscopy. These are compared to 42 biopsied Indian children with typical MCNS. The critical differences between African children and children with classical MCNS were the lack of a predictable response in eight of the former to steroids and cyclophosphamide and in a later peak age of presentation (7-8 years). These patients differed in most respects from minimal change seen in the malarial zones of North Africa except for clinical presentation. Minimal change in West and East Africa more closely approximates typical MCNS. Two of the African patients with minimal change had endemic syphilis and responded clinically to penicillin therapy. The clinical presentation in all the African children, the favourable short-term outcome in 12 of these patients, immunofluorescent (13 patients) and electron microscopic (three patients) findings, and selectivity of proteinuria (eight patients) paralleled that of classical MCNS. The implications of this study are that African children in South Africa with MCNS should not be treated with steroids or cyclophosphamide. There is no clear benefit in the use of these drugs in nephrotic children with obvious glomerular lesions and therefore it follows that such therapy should be avoided in all African children in South Africa with this disease.
D001741:Black or African American; D044383:Black People; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007194:India / Q000208:ethnology; D007223:Infant; D007668:Kidney / Q000473:pathology; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D011507:Proteinuria / Q000209:etiology; D013019:South Africa; D013256:Steroids / Q000627:therapeutic use; D013590:Syphilis, Congenital / Q000150:complications
D013256:Steroids; D003520:Cyclophosphamide