Pancreatic lesions in cystic fibrosis

Cystic fibrosis (pancreofibrosis, pancreatic congenital steatorrhea, etc.) is a hereditary disease characterized by a cystic change in the pancreas, glands of the intestine, respiratory tracts, large salivary glands, etc. Due to the secretion of a very viscous secretion corresponding to the glands. It is inherited by autosomal recessive type. It is believed that 2.6-3.6% of the adult population are heterozygous carriers of the cystic fibrosis gene.

Cystic fibrosis is found in different regions of the world with quite a different frequency - from 1: 2800 to 1: 90000 newborns (the latter figure refers primarily to persons of Mongoloid races).

The pancreas in cystic fibrosis is densified, the interlayers of connective tissue are excessively developed. The excretory ducts are cystically dilated. In older children, the acini are enlarged, cystic enlargement of individual ducts and acini is noted - until the entire glandular parenchyma is completely cystic. The number of pancreatic islets is the same as in healthy individuals. The development of the disease is associated with a violation of the transmembrane transport of ions, which is believed to be caused by a defect in the "calcium-dependent regulatory protein".

The main symptoms of cystic fibrosis in adults are weight loss, "pancreatogenic" diarrhea, significant steatorrhea, persistent pulmonary diseases with the formation of purulent bronchiectasis, compensatory emphysema, chronic pneumonia with frequently occurring foci of abscess formation, the presence of chronic rhinitis, sinusitis with polyposis.

This combination of quite different symptoms, observed from childhood, allows the doctor to suspect cystic fibrosis. X-ray examination of the thorax and paranasal sinuses reveals changes in them, which are quite typical for cystic fibrosis. With ultrasound of the pancreas, it can be densified, enlarged in size, cystically degenerated with the presence of echo-negative contents in the cysts. The liver can be enlarged. An important method for detecting the additional pancreas is gastroduodenoscopy, in necessary cases - with biopsy. Very reliable is the so-called sweat test with the determination in the pot of sodium and chlorine. Evidence in favor of cystic fibrosis is an increase in the content of these ions in the pot over 40 mmol / l in children and 60 mmol / L in adults.

As a treatment for mucovacidosis, a low-fat diet and a protein-rich diet are recommended. Recommended frequent (4-6 times a day) fractional meals. Enzyme preparations are prescribed that compensate for the insufficiency of the exocrine function of the pancreas (pancreatin, panzinorm, pancitrat, festal, soluzim, somilase, etc.). To dilute thick mucous secretion, acetylcysteine is prescribed (mucolytic drug). With a large loss of body weight, along with increased diet prescribe anabolic steroid hormones. Patients should be under the clinical supervision of a gastroenterologist with periodic (1 time in 1-2 months) carrying out coprological studies (the extent of digestion disturbance is determined, mainly it concerns fats, and accordingly the dose of enzyme preparations is selected). Patients with cystic fibrosis usually recommend multivitamins, especially B vitamins.

Patients with cystic fibrosis should also be under dispensary supervision at the pulmonologist in order not to start the bronchopulmonary process, and in the otolaryngologist. They should avoid hypothermia in every possible way.

[1], [2], [3], [4], [5], [6], [7]