A 6-year-old girl with anasarca, bilateral pleural effusions, and ascites was found to have severe hypoproteinemia secondary to gastrointestinal protein loss. This protein-losing enteropathy was originally felt to be idiopathic in origin, but re-examination some months later led to the recognition of chronic constrictive pericarditis. Pericardiectomy resulted in a complete relief of symptoms with a return of plasma proteins to normal and disappearance of the enteropathy. The 9 previously reported patients with protein-losing enteropathy and constrictive pericarditis are discussed briefly and the preoperative and postoperative data in our patient, including cardiac catheterizations and studies of protein metabolism, are presented.
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