Comparison of different treatment regimens in a case of homozygous familial hypercholesterolemia

The laboratory results of five periods of different treatment regimens were compared in a 19-year-old girl with homozygous familial hypercholesterolemia (FH): weekly low-density lipoprotein (LDL) apheresis sessions with dextran sulfate columns (LA 15, Kaneka Corporation, Osaka, Japan) without statin administration: weekly LDL apheresis with polyacrylate columns (DALI Fresenius Adsorber Technology. Bad Homburg. Germany) without statin: LDL apheresis as in Period 2 with 40 mg atorvastatin daily: LDL apheresis as in Period 2 with 80 mg atorvastatin daily and fortnightly LDL apheresis sessions with polyacrilate and administration of 80 mg atorvastatin daily. The five treatments were given in the above order. and each lasted at least 2 months. To compare the effectiveness of the different methods, the blood levels of total cholesterol. LDL-cholesterol and high-density lipoprotein (HDL)-cholesterol were measured before each session, and the percentage decreases in the blood levels of total cholesterol and LDL-cholesterol were recorded during sessions in Periods 1 and 2. In Periods 1 and 2. the biological effectiveness of LDL apheresis was comparable. Atorvastatin (40 mg daily) improved the blood levels of total cholesterol and LDL-cholesterol. but lowered HDL-cholesterol values. Increasing the daily dose of atorvastatin from 40 mg to 80 mg did not significantly improve LDL-cholesterol levels. When the time between two sessions was longer (Period 5). the total cholesterol and LDL-cholesterol values worsened and were comparable to those of Period 2 during which there was no atorvastatin treatment. In this case of homozygous FH. weekly sessions of LDL apheresis in association with atorvastatin at dose of 40 mg per day gave the best results.