Congenital
nephrotic syndrome of the Finnish type (CNF) is associated with
protein deficiency despite substantial
protein supplementation in the nephrotic state before
nephrectomy. Different
protein intakes (2.5 vs. 3.7 g/kg per day) in hypoproteinaemic children on continuous cycling
peritoneal dialysis (CCPD) were studied.
Lipids were also measured to determine whether severe atherogenic abnormalities seen during
nephrosis improved after
nephrectomy. Growth was normal or became normal with both
protein intakes. Serum pre-
albumin and
transferrin concentrations became normal. Total
protein (57 +/- 3.0 vs. reference limits 60-75 g/l) and
albumin (28 +/- 5.0 vs. reference limits 30-50 g/l) concentrations improved but remained below normal, even with the higher
protein intake. Muscle mass determined by measuring femoral quadriceps muscle thickness using ultrasound was markedly reduced in all patients at
nephrectomy. It improved (P < 0.05) in all but 2 patients who had several
bacterial infections, but reached normal level in only 3 patients within 6 months. Plasma total,
very low-density lipoprotein (VLDL) and
low-density lipoprotein (
LDL) triglyceride concentrations decreased (P < 0.05, P < 0.05 and P < 0.01, respectively) and VLDL,
LDL and
high-density lipoprotein (HDL) particles contained less
triglyceride than in the nephrotic state.
HDL cholesterol concentrations increased by 28% [0.58 +/- 0.22 mmol/l during
nephrosis, 0.81 +/- 0.21 mmol/l on CCPD after
nephrectomy (P < 0.05)] but remained below the level of 1.38 +/- 0.75 mmol/l in normal controls (P < 0.001). If compared with controls there were still significant abnormalities in
lipoprotein concentrations on CCPD.(ABSTRACT TRUNCATED AT 250 WORDS)