Pancreatic damage in cystic fibrosis

Cystic fibrosis (pancreofibrosis, congenital pancreatic steatorrhea, etc.) is a hereditary disease characterized by cystic changes in the pancreas, intestinal glands, respiratory tract, major salivary glands, etc. due to the secretion of a very viscous secretion by the corresponding glands. It is inherited in an autosomal recessive manner. It is believed that 2.6-3.6% of the adult population are heterozygous carriers of the cystic fibrosis gene.

Cystic fibrosis occurs in different regions of the world with quite different frequencies - from 1:2800 to 1:90000 newborns (the latter figure applies mainly to people of the Mongoloid races).

The pancreas in cystic fibrosis is compacted, the connective tissue layers are excessively developed. The excretory ducts are cystically dilated. In older children, the acini are dilated, cystic dilation of individual ducts and acini is noted - up to a complete cystic transformation of the entire glandular parenchyma. The number of pancreatic islets is the same as in healthy individuals. The development of the disease is associated with a violation of transmembrane ion transport, 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 pulmonary emphysema, chronic pneumonia with frequently occurring foci of abscess formation, the presence of chronic rhinitis, sinusitis with polyposis.

Such a combination of quite different symptoms, observed since childhood, allows the doctor to suspect cystic fibrosis. X-ray examination of the chest and paranasal sinuses allows to reveal changes in them, quite characteristic of cystic fibrosis. During ultrasound examination of the pancreas, it can be compacted, 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 an accessory pancreas is gastroduodenoscopy, if necessary - with a biopsy. The so-called sweat test with determination of the content of sodium and chlorine in sweat is considered very reliable. Evidence in favor of cystic fibrosis is an increase in the content of these ions in sweat over 40 mmol / l in children and 60 mmol / l in adults.

A low-fat, high-protein diet is recommended for the treatment of cystic fibrosis. Frequent (4-6 times a day) fractional meals are recommended. Enzyme preparations are prescribed to compensate for the insufficiency of the exocrine function of the pancreas (pancreatin, panzinorm, pancitrate, festal, solizyme, somilase, etc.). Acetylcysteine (a mucolytic drug) is prescribed to thin thick mucous secretions. In case of significant weight loss, anabolic steroid hormones are prescribed along with enhanced nutrition. Patients should be under the dispensary supervision of a gastroenterologist with periodic (once every 1-2 months) coprological examinations (the degree of digestion disorder is determined, mainly concerning fats, and the dose of enzyme preparations is selected accordingly). Patients with cystic fibrosis are usually recommended multivitamins, especially B vitamins.

Patients with cystic fibrosis should also be under the regular supervision of a pulmonologist, so as not to trigger the bronchopulmonary process, and an otolaryngologist. They should avoid hypothermia in every possible way.

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