- Cystic fibrosis a respiratory system
Respiratory disorders affect more than 90% of patients with cystic fibrosis, leading to the most serious complications of this disease. Patients suffer from chronic cough, progressive dyspnea and limited exercise tolerance.
Thick and sticky mucus settles in the airways and blocks their light, while limiting the possibility of penetration of immune cells and drugs. Such conditions are conducive to the multiplication of bacteria and the development of infections.
Recurrent pneumonia and bronchitis are often caused by bacteria of the genus Pseudomonas aeruginosa, which is highly resistant to antibiotics.
Cystic fibrosis is often accompanied by polyps and chronic inflammation in the nasal cavity and paranasal sinuses. Typically, there is a purulent runny nose, fever and characteristic headaches. Chronic inflammation of the airways leads to a gradual weakening and destruction of their walls, resulting in progressive expansion of their lumen (bronchospasm) and fibrosis of the lungs.
An increase in pulmonary resistance causes an overgrowth and overload of the right ventricle of the heart, known as the pulmonary heart (cor pulmonale). The so-called drumsticks are an exponent of chronic hypoxia. The final stage of lung damage is advanced respiratory failure.
- Mucoviscidosis a gastrointestinal tract
Some of the earliest symptoms of cystic fibrosis may be prolonged jaundice and delayed neonatal donation. Blockage of the intestine by dense tar can be the cause of neonatal tar occlusion. Bowel obstruction caused by hard stools in elderly patients is known as distal intestine obstructive syndrome (Dios). Symptoms include stools, vomiting and abdominal pain, and a characteristic picture of fluid levels on an abdominal X-ray.
Blockage of the pancreatic ducts by dense secretions causes the trapping of digestive enzymes in them, which gradually destroy the pancreatic pulp and lead to its recurrent inflammation. Pancreatic enzymes do not reach their target site (the small intestine) and cannot function properly.
This causes a violation of digestion and absorption of fats and carbohydrates. Nutritional deficiencies result in a slowdown in the growth and development of the child. Decreased protein absorption may lead to generalised oedema.