Extremely rare inherited enteropathy that is typically
apparent within hours or days after birth. The disorder is characterized by
chronic, severe, watery diarrhea and malabsorption caused by hypoplasia
and/or atrophy of the wall of the small intestine (e.g., hypoplastic villus
atrophy, defective brush-border assembly and differentiation). In infants,
chronic diarrhea and malabsorption may result in severe dehydration,
malnutrition, failure to thrive, and acidosis.