Severe hyperlipoproteinemia in congenital nephrotic syndrome of the Finnish type: effect of dialysis and kidney transplantation.

Abstract

Two children with congenital nephrosis of the Finnish type were studied successively at the three stages of the disease: (A) nephrosis, (B) renal insufficiency/peritoneal dialysis and (C) post-transplantation; two additional patients were studied at two stages. Plasma lipoprotein profiles were determined by density gradient ultracentrifugation and lipids by enzymatic methods. Stage A was characterized by hyperchylomicronemia, low high density lipoprotein (HDL) cholesterol and the presence of dense low density lipoprotein (LDL) and HDL particles. Total cholesterol and triglycerides showed great daily variation (5-14 and 5-33 mmol/l, respectively). During stage B, hyperlipidemia weakened. Yet HDL concentration remained low and the concentration of intermediate density lipoproteins (IDL) increased. At stage C, hyperlipidemia had almost subsided, but the presence of IDL persisted. In conclusion, severe hyperlipoproteinemia of congenital nephrosis at the nephrotic stage is attenuated during renal insufficiency and dialysis, and essentially normalizes after kidney transplantation. Yet the presence of IDL implies an increased risk of atherosclerosis.