Complications during home parenteral nutrition in children affected by congenital microvillous atrophy

Abstract

Among the heterogeneous group of intractable diarrheas in infancy is a now better-identified new clinical entity, intestinal epithelial dysplasia (IED). These forms, together with autoimmune enteropathies, enteropathies described in immunological deficits,2 and those associated to some rare inborn errors of metabolism and/or in some genetical syndromes represent the most severe microvillous atrophies. Intestinal epithelial dysplasias reveal themselves with intractable diarrhea starting in the neonatal period: the diarrhea is severe, watery, with electrolitic losses and without extradigestive manifestations. It is resistent to all the most common dietetic and pharmacological treatments and also to total bowel rest. An histological picture of severe microvillous atrophy with normal or hyperplastic cryptae is always present but is only sometimes associated with microvillous inclusions. These conditions that represent the more typical example of irreversible intestinal failure may be considered today to be one of the more frequent indications to small bowel transplant (Sb-Tx) in childhood. Parenteral nutrition (PN) has completely modified prognosis of this disease assuring to the patients not only survival but also normal growth while waiting for Sb-Tx. In these cases PN is started soon after birth and has a prolonged duration with home parenteral nutrition (HPN) programs. It is a total PN that assures the whole nutritional need and that has to cover the persistent severe fecal losses. In these patients, PN dependence and its complications in particular influence Sb-Tx timing. The aim of this study is to evaluate the complications we observed during HPN in three cases of congenital microvillous atrophy with epithelial dysplasia waiting for Sb-Tx.