Familial neonatal hypoproteinaemia with exudative enteropathy and intestinal lymphangiectasis.

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Abstract
A child with neonatal edema who was previously reported as a case of idiopathic hypoproteinemia was re-investigated 10 years later and shown to have protein-losing enteropathy and steatorrhea, and an abnormal small bowel histology with dilated lymphatic spaces and partial villous atrophy. The patient''s symptom-free sister was also shown to have protein-losing enteropathy. The relationship of this syndrome to intestinal malabsorption and gluten enteropathy is discussed.