Heubner-Herter disease


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Heubner-Herter disease

 [hoib´ner her´ter]
celiac disease of infants.

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.

Heubner, Johann Otto L.

(hoyb′nĕr)
German pediatrician, 1843–1926.

Heubner's disease

Syphilitic endarteritis of the brain.

Heubner-Herter disease

Nontropical sprue in infants.

Herter,

Christian A., U.S. physician, 1865-1910.
Gee-Herter disease - Synonym(s): Gee disease
Herter disease - gluten sensitivity, manifested by diarrhea, malabsorption, steatorrhea, nutritional and vitamin deficiencies. Synonym(s): celiac disease
Heubner-Herter disease - Synonym(s): Gee disease

Heubner,

Johann O.L., German pediatrician, 1843-1926.
artery of Heubner - a cerebral artery. Synonym(s): medial striate artery
Heubner arteritis - inflammation of arteries within the circle of Willis secondary to chronic basal meningitis from tubercle bacillus or particular fungi.
Heubner-Herter disease - Synonym(s): Gee disease