Longterm outcome

MVID is a congenital constitutive intestinal epithelial cell disorder leading, in its typical early-onset form, to permanent intestinal failure. The largest multicenter survey, of 23 MVID patients,15 revealed an extremely reduced life expectancy with a 1-year survival rate of less than 25%. Most children died of septic complications, liver failure, or metabolic decompensation. Few cases of MVID, especially with the late-onset form, may survive with limited stool output and may require only partial PN.29 Treatment with corticosteroids, colostrum or epidermal growth factor has not been successful, but octreotide has been used with partial success in one patient.15 In contrast to the initial outcomes before the 1980s, PN now allows most infants and children to survive. However, complications related to inadequate PN do limit long-term survival. These include recurrent catheter-related sepsis, extensive thrombosis, fat overload syndrome and cholestasis. In addition, without evidence of an associated renal disease, some of these infants and children present chronic hydro-electrolytic imbalance and acidosis, with subsequent impaired length growth. Others, because of repeated dehydration episodes associated with unadapted phosphocalcic intakes, present with nephrocalcinosis. Finally, even with adequate long-term PN and normal growth, most children remain with high and uncomfortable stool output. This requires daily fluid replacement with the high risk of severe dehydration. Intestinal transplantation therefore became the only definitive treatment of this rare intestinal disease.30-33

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