Homozygous familial hypercholesterolaemia is a rare inherited condition with an incidence of approximately one in a million. It is associated with severe premature atherosclerosis and early death from cardiovascular complications. The results ofliver transplantation reported to date have suggested only partially effective reduction of the hypercholesterolaemia.Three boys with familial hypercholesterolaemia, aged 10-0 to 151 years, received liver grafts at Addenbrooke's Hospital. Their untreated fasting lipid concentrations were grossly raised. All three had angiographic evidence of coronary atheroma and two had exertional angina. One child had such severe atheroma that coronary artery bypass surgery was considered necessary before liver transplantation. All three had straightforward operative and postoperative courses and their lipid concentrations returned rapidly to normal. One boy developed chronic rejection requiring retransplantation. Currently all three boys are well, on normal diets, and with normal liver function.It is concluded that (1) liver transplantation offers highly effective treatment for this lethal condition, (2) timing the operation is difficult but it should be undertaken before coronary artery disease has progressed too far (when combined liver and heart transplantation may be the only possibility), and (3) We describe three children in whom liver transplantation fully corrected the lipid abnormality.Case reports PATIENT 1 This English boy developed xanthomata at 11 months of age and was found to have the typical lipid profile of homozygous familial hypercholesterolaemia. Initially he was treated with a low fat diet only but from the age of 9 years cholestyramine and nicotinic acid were added. Coronary angiography was normal at that time but when repeated four years later showed a 30% stenosis of the proximal left descending coronary artery. He remained asymptomatic and no ischaemic changes were seen on an electrocardiogram (ECG). His treatment was intensified and for two years he had weekly plasma exchanges. Although this produced a satisfactory fall in lipid concentrations he found the treatment extremely burdensome and it was complicated by multiple central line infections. He was therefore referred for consideration of liver transplantation.He received a whole liver graft in October 1990 and had an uncomplicated perioperative course. The donor was cytomegalovirus positive and the patient developed cytomegalovirus hepatitis which resolved fully after a course of ganciclovir. His lipid profile and liver function returned to normal two weeks after transplantation (table and figure). He was well and had a normal lipid profile and normal liver function 3-8 years after the operation. PATIENT 2 This Asian son of a first cousin marriage developed xanthomata at the age of 6 years and was found to have familial hypercholesterolaemia. In spite of treatment with a low fat diet, cholestyramine and simvastatin, his cholesterol remained greatly raised. Coronary angiography at the age of ...

Bromfenac sodium (Duract) is a phenylacetic acid-derived nonsteroidal anti-inflammatory agent introduced in the United States in 1997 and withdrawn in 1998. We describe the first case of fulminant hepatic failure associated with this agent treated successfully with liver transplantation. Similarities to hepatotoxicity with related agents is discussed.