Textbook of Pediatric Gastroenterology, Hepatology and Nutrition
Textbook of Pediatric Gastroenterology, Hepatology and Nutrition_ A Comprehensive Guide to Practice
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- Mã sản phẩm: TEX150847
- Tình trạng: 2
Before focusing on microvillus inclusion disease and tufting
enteropathy, we briefly review similarly presenting entities.
In 1968 Avery, Villavicencio and Lilly were the first to describe
a severe chronic diarrhea in 20 infants and they named
it “infantile intractable diarrhea”; according to their description
“(it) was prolonged and intractable despite extensive
hospital therapy” .
This syndrome was defined on the basis of some clinic
characteristics, namely: (1) diarrhea of more than 2 weeks
duration, (2) age, less than 3 months, (3) three or more stool
cultures negative for bacterial pathogens, (4) necessity of
intravenous rehydration, and (5) prolonged and intractable
diarrhea despite hospital therapy.
The death rate was very high: 9 out of the 20 babies
(45 %) in Avery et al.’s record had died; it was even higher in
Hyman et al.’s (70 %) record .
Heterogeneity and lack of specificity are evident in Avery’s
original report: different pathologies were grouped in
it, some of which with a diagnosis were well defined even at
that time. Only autoptic material was available for the first
cases, and only after the introduction of total parenteral nutrition
(TPN) at the beginning of the 1970s  it was possible
to study the matter more in depth, thanks to proximal
small-intestinal biopsy  and later on to the development of
endoscopic techniques, which were safe and adequate for the
infant as well. It became consequently possible to discriminate
different causes for the so-called intractable diarrhea of
infancy  but its definition superimposes on the definition
of “protracted diarrhea of infancy”: The latter has duration
in common with it, but a failure to gain weight is enough to
define the picture .
Many cases of “protracted diarrhea of infancy” are diet
associated, as a consequence of cow milk or lactose intolerance
or malnutrition. Malnutrition causes intestinal atrophy
and consequently a malabsorption syndrome and diarrhea,
which apparently gets better with fasting. These features
have almost disappeared in the developed countries.
The main causes of “intractable diarrhea of infancy,” including
more severe and longer forms, can thus be summed
up (see also Table 1.1):