Herter infantilism


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coeliac disease

A malabsorptive syndrome caused by hypersensitivity of intestinal mucosa to alpha-gliadin, a gluten extract composed of glutamine and proline-rich proteins that is found in wheat, barley, rye, and oats.
 
Clinical findings
Diarrhoea, copious fatty stools, abdominal distension, weight loss, haemorrhage, osteopenia, muscle atrophy, peripheral neuropathy, CNS and spinal cord demyelination (sensory loss, ataxia), amenorrhoea, infertility, oedema, petechiae, dermatitis herpetiformis (especially if HLA B27), alopecia areata. Occult bleeding occurs in half of patients.
 
Lab
Transaminases (ALT, AST) are increased in ± 40% of patients with CD, which usually normalises with a gluten-free diet; iron deficiency, anaemia due to occult bleeding.

Diagnosis
Duodenal biopsy; anti-gliadin and anti-endomysial antibodies.
 
Management
Eliminate gliadin from diet.

Prognosis
Without treatment, 10–15% develop lymphoma (e.g., immunoblastic lymphoma; less commonly, T cell lymphoma), a risk that increases with disease duration. CD predisposes to gastrointestinal lymphoma and carcinoma of the oral cavity and oesophagus; the otherwise rare small intestinal adenocarcinoma is 80-fold more common in CD.

Herter infantilism

(hert'er)
[Christian Archibald Herter, U.S. physician, 1865–1910]
A form of infantilism resulting from defective fat and calcium absorption. It resembles sprue in adults. See: celiac sprue