Failure of Complete Bile Diversion and Oral Bile Acid Therapy in the Treatment of Homozygous Familial Hypercholesterolemia

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Abstract
Two patients with homozygous familial hypercholesterolemia, refractory to medical therapy, underwent complete bile diversion by common-duct ligation and Cholecystotomy, in an attempt to arrest the progression of their xanthomatosis and atherosclerosis by depletion of body cholesterol. Clofibrate was given after operation to one patient, and cholic acid to both, in an effort to enhance further the negative sterol balance.