Outcome

This neonatal diarrhea, which resists all treatments, requires permanent PN. However, it seems that some infants have a rather milder phenotype than others.50 Thus, because of partial intestinal function and limited amount of stool output, some patients only need partial long-term PN, with three to four weekly infusions. Careful monitoring should be performed, in order to avoid progressive growth retardation. In most patients, the severity of intestinal malabsorption and diarrhea make them totally dependent on daily long-term PN with subsequent risk of complications. This is therefore another indication for intestinal trans-plantation.51-53

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