Page 170 - Medicine and Surgery

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166 Chapter 4: Gastrointestinal system

Elements Pancreatic enzymes Bile Intestinal mucosa
Breakdown of Solubilisation of Secretion and
Function
macromolecules fats absorption
Pancreatic resection Liver disease causing a Coeliac disease
Chronic pancreatitis cessation of bile Ileal resection
Causes of Pancreatic obstruction production Bacterial overgrowth
malabsorption Whipple’s disease
Abetalipoproteinaemia
Figure 4.8 Malabsorption syndromes.

Age is shared) and other organ specific autoimmune condi-
Can present at any age. tions, e.g. primary biliary cirrhosis.

Sex Microscopy
F > Minadult diagnosed disease. There is lymphocytic infiltration of the lamina propria,
and an increase in intra-epithelial lymphocytes (which
Geography bear the γδ eceptor). Loss of normal villous architecture
Common in Europe, (1 in 300 in Ireland) rare in Black ranges from blunting (partial villous atrophy) to com-
Africans. plete loss (total villous atrophy) with crypt hyperplasia
in an attempt to compensate.
Aetiology
Investigations
Thought to be an autoimmune disease with genetic and
Serology: Screening by IgG gliadin and IgG anti-
environmental components.
reticulin antibodies is sensitive but not specific.
Genetic: 90% of patients have the HLA A1,B8,DR3,

Screening by IgA gliadin and IgA anti-reticulin an-
DR7,DQW2haplotype.70%concordanceofmonozy-
tibodies is specific but not sensitive. Antiendomysial
gotic twins.
antibodies (IgA) are found in the serum of most pa-
Environmental: There is amino acid sequence ho-

tients (sensitivity 98% specificity 93–99%), but total
mology between gliadin and adenovirus 12, increased
IgAmustalsobemeasuredascoexistentIgAdeficiency
childhood incidence in those exposed to gluten con-
is not uncommon. ELISA for transglutaminase anti-
taining foods from a young age.
body has been shown to be sensitive and specific.
Small bowel biopsy is diagnostic when taken at diag-
Pathophysiology
nosis, later when on a gluten free diet showing recov-
Gluten ingestion results in mucosal damage causing a
ery of architecture and after gluten challenge showing
flattening of villi (subtotal villous atrophy) which in turn
villous loss again.
leads to a more generalised malabsorption.
Management
Clinical features
Aglutenfree diet leads to a restoration of normal villous
Patients may present with irritability and failure to thrive
structure and resolution of dermatitis herpetiformis (see
in childhood, delayed puberty, short stature, or vomit-
page 394). Haemoglobin and antiendomysial antibodies
ing, diarrhoea, anorexia or abdominal distension at any
may be checked at routine follow-up to look for inad-
age.
vertent gluten intake.
Complications Whipple’s disease
There is an association with development of small bowel
lymphomaandasmallincreasedriskinthedevelopment Definition
of small bowel adenocarcinoma. There is also an asso- Rare multisystem disorder with malabsorption, lym-
ciation with dermatitis herpetiformis (HLA B8 linkage phadenopathy and arthritis.