Querfeld U
University Children's Hospital, Cologne, Germany.
Pediatr Nephrol. 1999 Jan;13(1):77-84. doi: 10.1007/s004670050568.
The pathogenesis, clinical significance, and treatment options of the disturbances in lipid metabolism in children with persistent nephrotic syndrome are reviewed. The lipoprotein profile is characterized by elevations of total plasma cholesterol and often triglycerides, elevated very low-density lipoprotein and low-density lipoprotein cholesterol, whereas high-density lipoprotein-cholesterol levels are variable; plasma levels of the atherogenic and thrombogenic lipoprotein(a) are also elevated. The pathophysiology of nephrotic dyslipoproteinemia is multifactorial, including both an increased hepatic synthesis and a diminished plasma catabolism of lipoproteins. There is a rationale for treatment, since dyslipidemia may contribute to the development of atherosclerosis and the progression of chronic renal failure. However, the benefits of treatment with lipid-lowering drugs have not been proven. Short-term studies in adults with nephrotic syndrome have documented safety and efficacy of lipid-lowering drugs, including hydroxymethylglutaryl-CoA reductase inhibitors ("statins"), bile acid sequestrants, fibric acids, fish oil, and probucol. Statins are the most-effective mediation, resulting in a decrease of total cholesterol levels by about 30%-40%. Prospective controlled studies in children evaluating efficacy and safety of lipid-lowering drugs are needed.
本文综述了持续性肾病综合征患儿脂质代谢紊乱的发病机制、临床意义及治疗选择。脂蛋白谱的特征为血浆总胆固醇及常伴有甘油三酯升高、极低密度脂蛋白和低密度脂蛋白胆固醇升高，而高密度脂蛋白胆固醇水平则有所不同；致动脉粥样硬化和血栓形成的脂蛋白(a)的血浆水平也升高。肾病性血脂异常的病理生理学是多因素的，包括肝脏脂蛋白合成增加和血浆脂蛋白分解代谢减少。治疗是有理论依据的，因为血脂异常可能导致动脉粥样硬化的发展和慢性肾衰竭的进展。然而，降脂药物治疗的益处尚未得到证实。对成人肾病综合征的短期研究记录了降脂药物的安全性和有效性，这些药物包括羟甲基戊二酰辅酶A还原酶抑制剂（“他汀类药物”）、胆汁酸螯合剂、贝特类药物、鱼油和普罗布考。他汀类药物是最有效的药物，可使总胆固醇水平降低约30%-40%。需要对儿童进行前瞻性对照研究以评估降脂药物的疗效和安全性。
