Changing perspective of lipoid nephrosis in Iranian children.
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Biomedical subjects
Publications and source records attributed to M T Honarmand.
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A total of 411 children, aged from 0.3 to 18 years, suffering from glomerular diseases, were studied by renal biopsy between 1976 and 1985. The clinical presentation included nephrotic syndrome (79% of cases), renal failure (43%), and arterial hypertension (38%). In all, 177 cases presented with primary nephrotic syndrome; all had complicated courses and most were either corticosteroid-dependent or -resistant. Only 26.6% had minimal change disease on renal biopsy; 56.5% had focal-segmental sclerosis; and immunofluorescent deposits were observed in half of the group. Acute poststreptococcal (36 cases), mesangiocapillary (80 cases), and lupus (34 cases) glomerulonephritis occurred frequently; IgA glomerulopathy (10 cases) and haemolytic uraemic syndrome (6 cases) were uncommon. Glomerular crescents were observed in 71 cases. These observations illustrate the types of glomerular diseases seen in Iranian children.
We describe the case of a 22-month-old girl who presented with a severe nephritic-nephrotic syndrome associated with arterial hypertension, remained symptomatic throughout the follow-up period and met advanced renal failure at 6 3/4 years of age. The initial renal biopsy revealed a mesangiocapillary glomerulonephritis with mesangial deposits of immunoglobulins (IgA, IgG, IgM), C3 and fibrin, extending into the capillary wall. Diffuse glomerular sclerosis was observed in the second biopsy. To our knowledge there are no reports of similar cases in the literature.
Juvenile nephronophthisis is a common cause of end stage renal failure in children and adolescents. The early occurrence with rapid progression to advanced renal failure has not previously been described. In this report we present three babies in whom failure to thrive, dehydration and a renal concentrating defect presented soon after birth; they were subsequently investigated for renal failure during the first year of life. In two cases the association with congenital hepatic fibrosis was noted. A family history of nephronophthisis was not present but the parents were consanguineous and one sibling had died in infancy.