CYSTIC FIBROSIS (G. Laksiteyner , 1905, Germany, G. Fanconi , 1936, America) -zabolevanie ha acterized universal time to zheniem exocrine glands.
Because of congenital enzyme tive failure (number and Drew GIH obscure reasons) exocrine glands secret becomes larvae fifth, viscous, hardly stands out, causing the blockage of small ducts. This causes Fibrocystic rebirth parenhi we glands in different organs: a stomach, salivary, similar changes also occur in the kidneys, liver. The lungs are often formed segmental atelectasis and bronchiectasis , while gastrointestinal race position – atrophy of the mucosa.
Etiology.
The reasons are not quite clear. Zabo Levan is hereditary recessive sive character.
There are a number before provisions: failure vitami on A , violation of the innervation of the glands.
Clinic.
Than earlier WHO Raste manifested disease, especially during unfavorable .
Distinguish:
- Meconium shape, which manifests itself in neonatal D result absence pischevaritel GOVERNMENTAL secrets: meconium (the first feces of newborn) are gu stymi, obstruct the intestines, vyzy -hand obstruction;
- Gastrointestinal, flowing with sympto Mami secretory failure;
- Lung – with shortness of breath, maps Noah bronchiectasis and atelectasis. Those chenie – unfavorable, especially in the development of disease in the WHO early rise.
With endocrinological point of view it is of particular interest cystic degeneration podzhel zling gland. At the same time, excretory cells are affected in the initial period, and later on, islands are also involved in the process, which is why the clinic of diabetes mellitus (DM) develops . In this diabetes develops slowly, but about the tech heavy.
TREATMENT.
- Treatment is symptomatic, its reduced to limit the fat in the diet,
- Prescribers containing digestive enzymes — zhelu dochy juice, pancreatin panzinorm , Festalum et al.,
- Foods rich vie Tamino.
- Treatment of Diabetes – com pensation disorders of carbohydrate metabolism, diet, insulin therapy.