The significance of focal sclerotic lesions of glomeruli in children.
To establish the relationship between the type of focal sclerotic lesion of glomeruli and the development of progressive renal disease, the clinical courses of 20 children with focal segmental and 7 with focal global sclerosis were analyzed. Only five patients, all of them with focal segmental sclerosis, did not have the nephrotic syndrome, although all had proteinuria. Results suggest that patients with focal global sclerosis have a course identical to that of children with the minimal lesion form of nephrotic syndrome: onset in early childhood, response to steroid therapy, and a relapsing, nonprogressive course. Focal segmental sclerosis, in constrast, is characterized by older age at onset, high incidence of nephritic symptoms, lack of response to steroid therapy, and a progressive course with histologic and functional deterioration. Since most published reports have not distinguished between these two entities, a more favorable prognosis in focal segmental sclerosis may be inferred than is actually the case.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D007223:Infant; D007674:Kidney Diseases / Q000473:pathology; D007678:Kidney Glomerulus / Q000473:pathology*; D008297:Male; D009400:Nephrosclerosis / Q000209:etiology / Q000473:pathology*; D009404:Nephrotic Syndrome / Q000150:complications / Q000188:drug therapy / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D011507:Proteinuria / Q000209:etiology; D013866:Thioguanine / Q000627:therapeutic use
D007136:Immunoglobulins; D003520:Cyclophosphamide; D013866:Thioguanine; D011241:Prednisone