Helicobacter pylori - Portal Neonatal

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 transplantation.
51–53
Other diseases of the intestinal
epithelium
The classification of IDI is probably incomplete,
since other forms with abnormal small-bowel
mucosa have been described. These include mitochondrial
DNA rearrangements; congenital enterocyte
heparan sulfate deficiency, phosphomannose
isomerase deficiency; and a
carbohydrate-deficient glycoprotein syndrome
with hepatic-intestinal presentation. 54–59 Rare
diseases involving the immune system and smallbowel
mucosa, 60 or severe intractable enterocolitis
of infancy, 6 seem clearly different from the
above-described diseases. The so-called ‘phenotypic
diarrhea’ that is an IDI syndrome associated
with phenotypic abnormalities and immune deficiency
is one of these rare diseases recently
reported. The patients present with diarrhea starting
within the first 6 months of life (