title: Portacaval shunt in four patients with homozygous hypercholesterolaemia.
abstract: Four patients with homozygous hyperbetalipoproteinaemia who had proved resistant to intensive medical therapy have undergone portacaval shunt. During 3 weeks of total parenteral alimentation before the operation, the serum-cholesterol decreased significantly in each of the four patients. During the fat-emulsion phase of hyperalimentation, one patient experienced a rise in cholesterol. Postoperatively, the size of xanthomas decreased in all cases; serum-cholesterol levels rose above those achieved during hyperalimentation, and this rise continued for as long as 3 months before a further reduction was recorded. In patients with angina, this improved considerably; and in most cases bruits decreased in itensity. The results at this stage do not, however, suggest portacaval shunt for all patients with homozygous hypercholesterolaemia.
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002784:Cholesterol / Q000097:blood; D002792:Cholestyramine Resin / Q000627:therapeutic use; D002994:Clofibrate / Q000627:therapeutic use; D004041:Dietary Fats / Q000008:administration & dosage; D005260:Female; D005947:Glucose / Q000627:therapeutic use; D006720:Homozygote; D006801:Humans; D006937:Hypercholesterolemia / Q000097:blood / Q000235:genetics / Q000628:therapy*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D009539:Nicotinic Acids / Q000627:therapeutic use; D010288:Parenteral Nutrition; D010375:Pedigree; D011167:Portacaval Shunt, Surgical; D011300:Preoperative Care; D011492:Protein Hydrolysates / Q000627:therapeutic use; D014280:Triglycerides / Q000097:blood
chemical_list: D004041:Dietary Fats; D009539:Nicotinic Acids; D011492:Protein Hydrolysates; D014280:Triglycerides; D002792:Cholestyramine Resin; D002784:Cholesterol; D002994:Clofibrate; D005947:Glucose