Total parenteral nutrition in familial hypercholesterolemia: importance of caloric control.

Effects of total parenteral nutrition (TPN) on serum cholesterol levels were examined in four patients with homozygous and three patients with heterozygous familial hypercholesterolemia and 13 patients without hypercholesterolemia. Fat-free TPN was administered in average doses of 26.5 cal/kg/day (43.6 cal/kg/day in a child) for 5 to 14 weeks. The serum cholesterol level decreased markedly by 47.5% and 58.6% in homozygous and heterozygous cases, respectively. Despite restriction in calories of TPN, protein nutrition was maintained in all patients. It became apparent that in familial hypercholesterolemia, whether the heterozygous or homozygous form, TPN performed at a well-adjusted caloric intake provided an adequate control of the serum cholesterol level as far as TPN was continued. In patients without hypercholesterolemia under TPN, serum cholesterol levels varied correlatively with the amount of calories to be administered. Compared with values before TPN, serum cholesterol level as far as TPN was continued. In patients without hypercholesterolemia under TPN, serum cholesterol levels varied correlatively with the amount of calories to be administered. Compared with values before TPN, serum cholesterol levels at the fourth week of TPN were elevated or maintained in patients receiving more than 40 cal/kg/day, while it was reduced in patients given less than 40 cal/kg/day. There was a statistically significant correlation between daily caloric intake per body weight and change of serum cholesterol level at the fourth week of TPN. This indicates that caloric control is of importance in TPN-induced change in serum cholesterol levels.