Watts G F, Mitropoulos K A, al-Bahrani A, Reeves B E, Owen J S
University Department of Medicine, University of Western Australia, Royal Perth Hospital.
J Intern Med. 1995 Aug;238(2):137-41. doi: 10.1111/j.1365-2796.1995.tb00911.x.
A 38-year-old Asian man presented with acute pancreatitis, marked hypertriglyceridaemia and macroproteinuria, 20 years after the diagnosis of lecithin-cholesterol acyltransferase (LCAT) deficiency. After recovery, he exhibited macroproteinuria and chylomicronaemia despite treatment with a very-low-fat diet. Infusion of normal plasma significantly increased the proportion of cholesterol esters in the patient's plasma and significantly lowered chylomicron-triglyceride levels, but not proteinuria. We conclude that renal dysfunction may be a late manifestation of LCAT deficiency and that it may lead to severe chylomicronaemia and acute pancreatitis. Infusion of normal plasma corrects the dyslipidaemia in LCAT deficiency, but in the short term does not improve renal function.
一名38岁的亚洲男性在被诊断为卵磷脂胆固醇酰基转移酶（LCAT）缺乏症20年后，出现了急性胰腺炎、明显的高甘油三酯血症和大量蛋白尿。康复后，尽管采用了极低脂肪饮食治疗，他仍表现出大量蛋白尿和乳糜微粒血症。输注正常血浆显著增加了患者血浆中胆固醇酯的比例，并显著降低了乳糜微粒甘油三酯水平，但对蛋白尿无影响。我们得出结论，肾功能障碍可能是LCAT缺乏症的晚期表现，并且可能导致严重的乳糜微粒血症和急性胰腺炎。输注正常血浆可纠正LCAT缺乏症中的血脂异常，但短期内并不能改善肾功能。
