Protein-Losing Enteropathy Following Adrenocorticotropic Hormone Therapy in an Infant with West Syndrome.

Abstract

Protein-losing enteropathy (PLE) is characterized by hypoproteinemia caused by the leakage of plasma proteins from the gastrointestinal tract wall into the lumen. We report a case of PLE occurring during low-dose adrenocorticotropic hormone (ACTH) therapy for West syndrome. A 6-month-old boy diagnosed with West syndrome was initially treated with sodium valproate and vitamin B6. Although the frequency of seizures decreased, electroencephalography findings did not improve. Consequently, low-dose ACTH therapy and a ketogenic diet were introduced. On day 5 (X + 5 days) after initiating ACTH therapy, scrotal edema and hypoalbuminemia were observed. Scintigraphy revealed leakage of radionuclides into the gastrointestinal tract, leading to a diagnosis of PLE. ACTH therapy was discontinued on X + 6 days, while valproate, vitamin B6, and the ketogenic diet were continued. Following the cessation of ACTH therapy, the patient showed improvement. We identified ACTH therapy as the likely cause of PLE in this case. This represents the first report of PLE diagnosed during ACTH therapy.