Paediatrics: Coeliac disease

Coeliac disease

Coeliac disease is an enteropathy due to lifelong intolerance to gluten pro-tein (present in wheat, barley, rye, and oats by cross contamination).

Prevalence is approximately 1% when populations are screened. It is asso-ciated with:

•   A positive family history.

•   Type 1 diabetes.

•   Down syndrome.

•   IgA deficiency.

Presentation

The condition may present at any age after starting solids containing glu-ten.

The ‘classic’ initial features include:

•   Pallor.

•   Diarrhoea.

•   Pale, bulky floating stools.

•   Anorexia.

•   FTT.

•   Irritability.

Later, there is:

•   Apathy.

•   Gross motor developmental delay.

•   Ascites.

•   Peripheral oedema.

•   Anaemia.

•   Delayed puberty.

•   Arthralgia.

•   Hypotonia, muscle wasting.

•   Specific nutritional disorders.

Increased recognition, and the widespread practice of antibody screening of children at high risk, has changed considerably the clinical spectrum of cases seen, with less classical and severe symptoms now more common at time of initial diagnosis.

There are three settings in which the diagnosis of coeliac disease should be considered and screened for:

•   Children with frank gut symptoms.

•   Children with the non-gastrointestinal manifestations described here.

•   Asymptomatic individuals with conditions that are associated with coeliac disease.

Coeliac crisis

Life-threatening dehydration due to diarrhoea accompanying malabsorp-tion. This condition is now very rare except in the less-developed world.

Investigations

• Measurement of serum tissue transglutaminase IgA antibody (TTG) is recommended for initial testing for coeliac disease. IgA sensitivity and specificity approach 100%, although false positives are occasionally seen. Anti-endomysium IgA antibody is observer-dependent and expensive. Anti-gliadin antibody tests are less accurate and are now not advised. It is important to exclude IgA deficiency as a cause of falsely negative serology.

• Endoscopic small bowel biopsy of the third part of the duodenum shows diffuse, subtotal villus atrophy, increased intraepithelial lymphocytes, and crypt hyperplasia. The villi return to normal on a gluten-free diet.

Most clinicians consider positive mucosal histology and full clinical recov-ery on gluten-free diet +/– positive IgA antibodies sufficient to make a diagnosis. Antibody levels should return to normal on treatment and nega-tive serology is a marker of compliance. Avoid gluten challenge (>10g oral gluten per day for 3–4mths and re-biopsy) unless diagnosis is in doubt, e.g. initial biopsy is inadequate or not typical, or alternative diagnosis is possible, e.g. transient gluten intolerance may occur after gastroenteritis, giardiasis, or cow’s milk protein intolerance.

Treatment

• Gluten-free diet under the supervision of a paediatric dietitian.

• Gluten-free foods are prescribable in UK.

• Gluten avoidance should be life-long if coeliac disease is confirmed.

•   Nutritional supplements may be required.

Prognosis

Excellent if patient is compliant with strict, life-long gluten-free diet. There is a possible increased risk of intestinal lymphoma if gluten is ingested, even in asymptomatic coeliac disease.