SEVERE HYPERLIPOPROTEINEMIA IN CONGENITAL NEPHROTIC SYNDROME OF THE FINNISH TYPE - EFFECT OF DIALYSIS AND KIDNEY-TRANSPLANTATION

Two children with congenital nephrosis of the Finnish type were studie d 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 hyperchylomi cronemia, low high density lipoprotein (HDL) cholesterol and the prese nce of dense low density lipoprotein (LDL) and HDL particles. Total ch olesterol and triglycerides showed great daily variation (5-14 and 5-3 3 mmol/l, respectively). During stage B, hyperlipidemia weakened. Yet HDL concentration remained low and the concentration of intermediate d ensity lipoproteins (IDL) increased. At stage C, hyperlipidemia had al most subsided, but the presence of IDL persisted. In conclusion, sever e 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 impl ies an increased risk of atherosclerosis.