Cystic fibrosis (cystic fibrosis of the pancreas) - a systemic hereditary disease that affects exocrine glands. The cause of cystic fibrosis is a genetic mutation, transmitted in an autosomal - retsissivnomu type.
Distinguish the following basic forms of cystic fibrosis:
mixed (pulmonary-intestinal, 75-80%)
respiratory (15-20%)
E. (5%).
The mixed form is the most severe manifestation of cystic fibrosis. From the first weeks of a life marked recurrent severe bronchitis and pneumonia with prolonged duration, persistent cough, often koklyushepobny with viscous mucus, intestinal disorders and eating disorders. Violations of bronchial conductivity is an integral part of the broncho-pulmonary changes. Violation of self-purification process leads to blockage of the bronchioles and small bronchi. Typical changes are permanent raznokolibernye wheezing, which usually are heard from both sides. Percussion sound is a box color.
X-ray picture is characterized by the prevalence and diversity of large pulmonary changes. The combination of emphysema and a sharp increase in pulmonary and deformed figure, even without exacerbation creates a fairly typical pattern.
When bronhologicheskom study identifies common bronchial deformation with a number of typical cystic features: teardrop bronchiectasis, changing the course of the bronchi, as well as the unevenness of bronchi chetkoobraznaya III-VI of the order. In the study of respiratory function normally, there are marked obstructive disorders.
In the long cystic fibrosis is almost always observed in the defeat of the nasopharynx, adenoids, nasal polyps. Almost all children revealed sinusitis, clinical manifestation of which is an abundant secretion, headache, nasal voice.
Clinical symptoms of intestinal syndrome consists of symptoms of dysfunction of the pancreas and intestines. In the intestine there putrefactive processes accompanied by the accumulation of gases, resulting in permanently swollen belly. Characterized by abundant, thick, foul-chair zamaskoobrazny putrid odor, which can sometimes be suspected cystic fibrosis at the first examination of the patient.
Mikonialny ileus occurs in 10-15% of patients in the neonatal period. In the first days of life poyavlyayutsyasimptomy intestinal obstruction: vomiting bile, neothozhdenie meconium, increased stomach. Very dangerous complication is mekonievy peritonitis. Meconium ileus is considered to be a manifestation of a severe form of cystic fibrosis.
Treatment of cystic fibrosis.
Apply mucolytic drugs, postural drainage and vibratory massage for at least 3 times a day, therapeutic bronchoscopy and course of antibiotics.
