Jankowski Krzysztof, Wyzgał Anna, Wierzbicka Aldona, Tronina Olga, Durlik Magdalena, Pruszczyk Piotr
Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland.
Department of Biochemistry and Experimental Medicine, Children's Memorial Health Institute, Warsaw, Poland.
Acta Biochim Pol. 2015;62(3):621-3. doi: 10.18388/abp.2015_971. Epub 2015 Aug 28.
Hypercholesterolemia is a common disorder in adult population, but total cholesterol concentrations beyond 1000 mg/dl occur rarely, and are found in patients with homozygous familial hypercholesterolemia and familial lecithin-cholesterol acyltransferase deficiency, in chronic graft-versus-host disease of the liver, after intravenous infusion of fat emulsion (intralipid), in newborn infants with immature liver function, and in obstructive biliary cholestasis. Cholestasis induces a dramatic increase in plasma cholesterol and the appearance of an abnormal lipoprotein, lipoprotein X (LpX), in the plasma. We report a case of severe hypercholesterolemia mediated by LpX in a patient transplanted for primary biliary cirrhosis (PBC), who was qualified for liver re-transplantation (re-LTx) due to chronic cholestasis. Four months after re-LTx, the cholesterol concentration was normal. The problems in diagnosis and treatment are discussed.
高胆固醇血症在成年人群中是一种常见病症，但总胆固醇浓度超过1000mg/dl的情况很少见，见于纯合子家族性高胆固醇血症和家族性卵磷脂胆固醇酰基转移酶缺乏症患者、肝慢性移植物抗宿主病患者、静脉输注脂肪乳剂（英脱利匹特）后、肝功能不成熟的新生儿以及梗阻性胆汁淤积患者。胆汁淤积会导致血浆胆固醇显著升高，并在血浆中出现异常脂蛋白——脂蛋白X（LpX）。我们报告一例因原发性胆汁性肝硬化（PBC）接受移植的患者，由LpX介导的严重高胆固醇血症病例，该患者因慢性胆汁淤积符合再次肝移植（re-LTx）条件。再次肝移植四个月后，胆固醇浓度恢复正常。本文讨论了诊断和治疗方面的问题。
