Coeliac disease

Coeliac disease

an enteropathy
1. where gliadin fraction of gluten provokes a damaging immunological response
  1. in prox small intestinal mucosa
    1. so rate of migration of enterocytes moving up villi from crypts raises markedly
      1. but not enough to compensate for increased cell loss from villous tips
        villi get progressively shorter & absent-> flat mucosa
incidence of classical coeliac disease
1. 1 in 3000 in Europe
presentation
1. age at presentation partly influenced by age of intro of gluten into diet
2. classically profound malabsorptive syndrome
  1. @ 8-24 months old
  2. after intro of wheat-containing weaning foods
  3. failure to thrive
  4. abdo distension
  5. buttock wasting
  6. abnormal stools
  7. general irritability
3. nowadays children more likely to present less acutely later in childhood
4. highly variable clinical features
  1. mild, non-specific GI sx
  2. growth failure
  3. anaemia
    1. iron &/or folate def
5. on screening higher risk kids
  1. type 1 diabetes mellitus
  2. autoimmune thyroid disease
  3. Down syndrome
  4. 1st degree relatives of ppl w/ known coeliac disease
  5. serological screening tests
    1. v sensitive & specific
    2. IgA tissue transglutaminase antibodies
    3. endomysial antibodies
diagnosis
1. serology
  1. highly suggestive but not confirmatory
  2. not sensitive nor specific enough to replace biopsy
2. endoscopy
  1. small intestinal biopsy
    1. mucosal changes
      1. increased intraepithelial lymphocytes
      2. variable degree of villous atrophy
      3. crypt hypertrophy
  2. and
    1. resolution of sx and catch-up growth upon gluten withdrawal
      1. confirms diagnosis
3. can't use gluten-free diet as diagnostic test w/o small intestinal biopsy
Mx
1. remove all wheat, rye & barley-containing products from diet
  1. -> symptom resolution
  2. adhere to gluten-free diet for life
  3. probably reduces risk of small bowel malignancy to normal
2. supervision by dietician is essential
3. gluten challenge later in childhood
  1. for those whose initial biopsy or response to gluten withdrawal was doubtful
  2. for those in whom CD presents when < 2 yrs old
  3. give food containing gluten or gluten powder
    1. at start of challenge, serological markers should be -ve
      1. gluten challenge is +ve if serology becomes +ve again
4. NO REPEAT SMALL INTESTINAL BIOPSY
increased incidence of small bowel malignancy in adulthood

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	Criado por v.djabatey quase 11 anos atrás