CHYLOUS EFFUSIONS INTO THE INTESTINE IN A PATIENT WITH PROTEIN-LOSING GASTROENTEROPATHY

Abstract

A girl had been affected with steatorrhea, lymphopenia, and hypoproteinemic edema from her fifth year of life; this edema was mainly localized in the legs. The hypoproteinemia was found to be due to abnormal loss of serum proteins into the intestine. A chylous fluid was occasionally obtained on duodenal intubation. Albumin levels were found to range from 20 to 500 mg/100 ml in this fluid; a considerable loss of protein into the intestine therefore might undoubtedly have resulted. Serum albumin was shown to pass fairly rapidly into the intestinal lumen. Fatty acid analysis of the chyle obtained, showed only the presence of high molecular saturated and unsaturated fatty acids. No low-molecular saturated fatty acids were found. It is suggested in the literature that the obstruction of chyliferous vessels may give rise to this clinical picture. In view of the localization of the edema and the family history, the present authors believe that there is a relationship between localized familial lymphedema (Milroy's disease) and protein-losing gastroenteropathy. The two conditions may be due to changes in the lymphatics, the localization of the changes determining which symptoms will appear. Treatment consisted in ordering a fat-free diet; this had a limited effect.