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February 1969

Intestinal Lymphangiectasia: Metabolic Studies

Author Affiliations

Rochester, NY
From the departments of pediatrics (Drs. Amirhakimi, Bryson, and Forbes) and medicine (Dr. Samloff), University of Rochester School of Medicine and Dentistry, Rochester, NY.

Am J Dis Child. 1969;117(2):178-185. doi:10.1001/archpedi.1969.02100030180010

INTESTINAL lymphangiectasia is associated with enteric loss of protein and malabsorption of fat.1-6 As a consequence, children with this disease fail to thrive,7,8 and display steatorrhea and hypoproteinemia with secondary edema of variable extent. Although the exact pathogenesis of this condition is as yet not completely understood, the response of a few patients with this syndrome to diet in which the fat is primarily medium-chain-triglycerides (MCT) has been remarkable.1,7-11 The purpose of this report is to present metabolic data obtained in a 15-year-old boy with isolated intestinal lymphangiectasia prior to and during MCT therapy.

Report of a Case  This white boy (SMH, 63-91-58), was the product of a normal full-term pregnancy. Delivery and neonatal period were uneventful. His growth, development, and general health were normal until age 4 years. At that time he underwent surgery for the repair of a right inguinal hernia. During the operation, milky