Familial Homozygous Hypercholesterolemia treated with Intravenous Hyperalimentation: A Case Report

Abstract

Familial homozygous hypercholesterolemia is a fatal disease in consequence of coronary insufficiency or vascular disease. In addition, drug and diet therapy is often ineffective. Intravenous hyperalimentation is recognized as one of therapeutic procedures available for this disease. We treated one patient with familial homozygous hypercholesterolemia using intravenous hyperalimentation followed by good result in decreasing serum cholesterol level.
This 18 year-old female patient was suffered from anginal attack, xanthoma and her exercised-ECG showed ST-depressing in leads II, III, _aV_f and v_2-4. Coronary angiography showed the 90% stenosis in LAD with other stenotic lesions. Serum cholesterol level ranged from 758mg/dl to 1003mg/dl. On August 23, 1977 intravenous hyperalimentation was started with solution which contains 21% dextrose and 4% crystalline amino acids and electrolytes. Initially hyperalimentation provided 1, 800cal/day. However, serum cholesterol did not decrease so much while body weight increased. For that reason callory was reduced to 1200cal/day. After calloric reduction, serum cholesterol fluctuantly decreased into 363mg/dl on the 106th day after intravenous hyperlimentation therapy. Xanthoma, especially in the subcutane of Achilles tendon read, reduced as the decreasment of serum cholesterol level.
But anginal attacks showed no change in frequency. During this therapy, her general condition was kept in good status and serum total protein and albumin were maintained around 6.6-8.6g/dl and 3.3-4.2g/dl, respectively. On November 29, 1977, A-C bypass operation was done. After the 8th day of this surgery, the oral intake was started and intravenous hyperalimentation was ended and then serum cholesterol level again increased to 800mg/dl. As described above, intraveous hyperalimentation cause decline of serum cholesterol level, but the mechanism of this effect is not well known.