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A congenital enteropathy presenting with early-onset severe intractable diarrhea sometimes causing irreversible intestinal failure. Infants develop a watery diarrhea within the first days after birth and the diarrhea persists in spite of bowel rest and parenteral nutrition. Some infants are reported to have associated choanal, rectal or esophageal atresia. Autosomal recessive transmission but the causative gene has not been yet identified.
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A congenital enteropathy presenting with early-onset severe intractable diarrhoea sometimes causing irreversible intestinal failure. Infants develop a watery diarrhoea within the first days after birth and the diarrhoea persists in spite of bowel rest and parenteral nutrition. Some infants are reported to have associated choanal, rectal or oesophageal atresia. Autosomal recessive transmission but the causative gene has not been yet identified.
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