Celiac disease (Guy-Herter-Heubner disease, Glu-Tenenteropathy, Intestinal Infantilism),

Celiac disease (Hy-Herter-Heubner disease, Glu-Tenenteropathy, Intestinal Infantilism)

Celiac disease is characterized by impaired intestinal absorption, sub- or atrophy of the mucous membrane of the jejunum and an unconditionally positive reaction to a gluten-free diet (exclusion of gluten-containing cereals from food). The disease occurs in approximately 1 in 3,000 people and is inherited in an autosomal dominant manner.

Etiology and pathogenesis
A connection has been established between the disease and the consumption of gliadin, a protein in the cereals wheat, rye, oats and barley. However, the exact mechanism of the pathological interaction of gliadin with the mucosa is unclear. The presence of an enzyme defect is assumed - the absence or deficiency of gliadinaminopeptidase or another enzyme involved in the breakdown of gluten.

An immunological reaction (humoral and cellular) to gluten has also been described, occurring in the lining of the small intestine.

Clinical picture
There are true celiac disease and celiac disease syndrome, which can develop with various intestinal diseases. The onset of celiac disease often coincides with the introduction of complementary foods containing flour into the child’s diet, so children aged 6-12 months are more often affected.

Frequent, foamy stools with a pungent odor, light or grayish, and greasy appear. Pathogenic microflora is usually not found in feces. Standard treatment of dyspepsia (antibiotics, enzymes, diet) is ineffective.

The child becomes lethargic, pale, loses body weight, and decreases appetite. Dystrophy develops and a typical appearance is acquired: severe exhaustion, sunken eyes, swollen abdomen. Swelling of the legs and bone fractures may occur. Pseudoascites is determined.

With a long course, signs of multivitamin deficiency appear. Short stature is typical. The course is wavy, secondary infection is possible.

Diagnostics
The diagnosis is suspected based on the history, appearance and character of the stool. It is confirmed by improvement on a gluten-free diet and worsening when it is violated.

A scatological and biochemical blood test, x-ray and histology of a small intestine biopsy are performed. Specific antibodies can be detected in the blood.

Differentiate with cystic fibrosis, disaccharidase deficiency, and gastrointestinal abnormalities.

Treatment
The basis is a lifelong gluten-free diet with the exclusion of grains and products containing gliadin. Prescribe vitamins, Ca, Fe preparations, enzymes, massage and gymnastics.

If you follow a diet and proper treatment, the prognosis is favorable. Children must be registered with a dispensary.