Helicobacter pylori - Portal Neonatal

Figure 1.5 Epithelial dysplasia. Disorganization of
surface epithelium showing tufts with apical rounding
epithelial cells.
crypts often have an abnormal aspect with dilatations
such as pseudocysts and abnormal regeneration
with branching 11 (Figure 1.6). The study of
basement membrane components demonstrated
an abnormal laminin and heparan sulfate proteoglycan
deposition at that level, compared to biopsy
specimens from patients with celiac disease or
autoimmune enteropathy. 11 Relative to the
controls, there was faint and irregular laminin
deposition at the epithelial–lamina propria interface,
while heparan sulfate proteoglycan depositions
were large and lamellar, suggestive of abnormal
development of basement membrane at the
origin of the epithelial abnormalities. On the other
hand, we observed an increased immunohistochemical
expression of desmoglein in IED, and
ultrastructural changes of desmosomes, which
were increased in length and number (Figure
1.7). 37
Diagnosis
Diagnosis of IED is sometimes difficult for several
reasons. The early onset of severe and permanent
diarrhea and the lack of features diagnostic of
microvillus inclusion disease are important diagnostic
elements. However, the characteristic tufts
of extruding epithelium may not be obvious, especially
early on. At the onset of the clinical course,
IED is most often suspected after elimination of
MVID, and the final diagnosis is made rather late,
by performing repeated intestinal biopsies which
Intestinal epithelial disease 7
Figure 1.6 Epithelial dysplasia. Partial villus atrophy with
crypt hyperplasia and/or pseudocystic crypt appearance,
branching and disorganization of surface epithelium.
Figure 1.7 Desmoglein staining. Increased expression of
desmoglein in the tight junctions.
change from normal in early life (only non-specific
villus atrophy with or without mononuclear cell
infiltration of the lamina propria) to the characteristic
tufts. In addition, it is difficult to show the
rare, specific abnormalities of basement
membrane components, integrins or desmosomes
in part of the mucosa, in the absence of tufts.
Another difficulty is related to the infiltration of
the lamina propria by T cells, a finding that would
support the hypothesis of an immune-related
enteropathy, especially when tufts are missing.
One might speculate that defective cell adhesion
increases intestinal permeability, with a subse-