The definition and prognosis of severe intractable diarrhea (SID) in i
nfants have changed since the condition was first described in 1978. S
ID due to abnormalities of the intestinal mucosa is discussed in this
article. Microvillous atrophy and villous atrophy can be individualize
d based on clinical data (age at onset, family history, characteristic
s of the diarrhea) and on findings from histologic (degree of atrophy,
appearance of the glands and epithelium), ultrastructural, and immuno
histochemical (evidence of T cell activation) studies. Some cases of S
ID with villous atrophy are due to an immune or autoimmune process or
to epithelial dysplasia, whereas others are associated with a characte
ristic dysmorphic syndrome. These forms of SID carry a grim prognosis
and usually require prolonged or life-long parenteral nutrition.