Octreotide in the treatment of intestinal lymphangiectasia.

摘要: Primary intestinal lymphangiectasia is characterized by dilated small bowel lymphatics and loss of lymph into the lumen resulting in hypoproteinaemia oedema. Some patients have a more generalized lymphatic abnormality associated with lymphoedema limbs chylous pleural effusions. There no specific treatment although enteric protein may decrease low-fat diet. This report describes patient severe primary lymphangiectasia, limb oedema recurrent effusions, who responded to octreotide. Before starting octreotide she required weekly intravenous albumin infusions maintain serum above 20 g/l. Bilateral effusions repeatedly reaccumulated despite pleurectomy subsequently tetracycline pleurodesis. Treatment octreotide, 200 microg twice daily, resulted reduction from 16 4.1% 5 days (normal less than 1%) was maintained between 22 26 g/l without need for infusion. Oedema arms resolved completely did not reaccumulate. The mechanism action this condition appears be due gut another, as yet unidentified, action.