Ballinger A B, Farthing M J
Digestive Diseases Research Centre, St Bartholomew's and The Royal London School of Medicine and Dentistry, UK.
Eur J Gastroenterol Hepatol. 1998 Aug;10(8):699-702.
Primary intestinal lymphangiectasia is characterized by dilated small bowel lymphatics and loss of lymph into the bowel lumen resulting in hypoproteinaemia and oedema. Some patients have a more generalized lymphatic abnormality associated with lymphoedema of the limbs and chylous pleural effusions. There is no specific treatment although enteric protein loss may decrease with a low-fat diet. This report describes a patient with severe primary intestinal lymphangiectasia, associated with limb oedema and recurrent pleural effusions, who responded to treatment with octreotide. Before starting octreotide she required weekly intravenous albumin infusions to maintain the serum albumin above 20 g/l. Bilateral pleural effusions repeatedly reaccumulated despite pleurectomy and subsequently tetracycline pleurodesis. Treatment with octreotide, 200 microg twice daily, resulted in a reduction in enteric protein loss from 16 to 4.1% in 5 days (normal less than 1%) and the serum albumin was maintained between 22 and 26 g/l without the need for albumin infusion. Oedema in the arms resolved completely and the pleural effusions did not reaccumulate. The mechanism of action of octreotide in this condition appears to be due to a reduction in gut protein loss and another, as yet unidentified, action.
原发性肠淋巴管扩张症的特征是小肠淋巴管扩张，淋巴液进入肠腔导致低蛋白血症和水肿。一些患者存在更广泛的淋巴异常，伴有肢体淋巴水肿和乳糜性胸腔积液。目前尚无特异性治疗方法，尽管低脂饮食可能会减少肠道蛋白丢失。本报告描述了一名患有严重原发性肠淋巴管扩张症、伴有肢体水肿和复发性胸腔积液的患者，其对奥曲肽治疗有反应。在开始使用奥曲肽之前，她每周需要静脉输注白蛋白以维持血清白蛋白水平高于20g/L。尽管进行了胸膜切除术及随后的四环素胸膜固定术，双侧胸腔积液仍反复再积聚。每天两次给予200μg奥曲肽治疗，5天内肠道蛋白丢失从16%降至4.1%（正常低于1%），血清白蛋白维持在22至26g/L之间，无需输注白蛋白。手臂水肿完全消退，胸腔积液未再积聚。奥曲肽在这种情况下的作用机制似乎是由于肠道蛋白丢失减少以及另一种尚未明确的作用。
