Mucoviscidosis (cystic fibrosis of a pancreas).

Etiology, pathogenesis. Serious hereditary disease in which basis the lesion of excretory glands and the raised viscosity of secrets lays that leads to a lesion of lungs, a pancreas and an intestine, less often - a liver and kidneys. Substantial increase of concentration of chlorine and sodium in sweat of patients is pathognomonic. The essence of the metabolic block at a mucoviscidosis is not established yet; assume, that disease is bound to disturbance of synthesis of glycoproteids. In separated mucous glands there are products, interactions of glycoproteids and a calcium, raising viscosity of a secret. Disease is inherited on autosomally recessive type.

Clinical picture. There are some clinical forms of a mucoviscidosis: pulmonary, intestinal, admixed (is pulmonary -intestinal). Besides the abortal and erased forms of illness are known. The majority of patients in the first half-year of a life has changes from organs of respiration. The obsessional pertussoid is characteristic, early there are inflammatory and obstructive changes in lungs. At children of thoracal age the pneumonia, differing serious current and predilection to abscessing develops. At children more advanced age are observed fixing bronchites with the expressed bronchospasm. Development of a pneumosclerosis with bronchiectasias (a chronic pneumonia) is typical. In lungs wet rhonchuses are constantly listened, the emphysema is found out in all patients.