Kidney biopsies were performed on fifteen patients with a long-standing history of familial Mediterranean fever (FMF) and evidence of renal involvement. On light microscopy, seven patients were found to have amyloidosis, six mesangial proliferative glomerulonephritis (MsPGN) and two patients rapid progressive glomerulonephritis (RPGN). Immunofluorescent studies of the six biopsies with MsPGN were positive for mesangial IgA deposits (IgA nephropathy) in three patients and IgM mesangial deposits in three (IgM nephropathy). We conclude that in patients with FMF and renal involvement, non-amyloid renal lesions (IgA nephropathy, IgM nephropathy and RPGN) should be considered in the differential diagnosis in addition to amyloidosis.
Fifteen cases of renal vein thrombosis were seen in patients with nephrotic syndrome, both adult and pediatric, and all had kidney biopsies. Six patients were found to have membranoproliferative glomerulonephritis, 3 membranous nephropathy, 2 focal segmental glomerulosclerosis, 2 renal amyloidosis and one each minimal change disease and diffuse mesangial proliferative glomerulonephritis. The diagnosis of renal vein thrombosis was made in all patients by utilizing digital subtraction venography. This method is simple, safe, noninvasive and quite efficient.
A 3-day-old male infant born to a non-diabetic mother was admitted to the University Hospital with gangrene of the right forearm and some gangrenous patches of the left forearm. This was associated with left renal vein thrombosis. Screening tests for coagulation disorders gave normal results. The gangrenous right forearm was amputated and subsequently grafted. The skin lesions of the left forearm were successfully grafted. The renal system recovered well on supportive measures. The course of hospitalization and subsequent follow-up assessment at 3 months of age were satisfactory.
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