It is a hereditary congenital abnormality of all mucus secreting glands particularly those of pancreas and bronchioles.
In this disease abnormally viscid mucus is secreted. This causes obstruction of the pancreatic ducts and retention of pancreatic secretions. This results in distension of the alveoli and subsequently ruptures. Pancreatic enzymes escape and pancreatitis results with subsequent fibrosis.
The other abnormality is that meconium becomes abnormally viscid and it causes obstruction of the intestine more frequently at the distal ileum. Viscid mucus into the bronchioles causes bronchiolar obstruction and predisposes to respiratory infection.
Sweat glands produce sweat containing four times more sodium chloride than the normal. So exocrine glands are also involved.
Symptoms-
2. In infants, the respiratory system is more often affected and bronchiolitis develops. Patients complain of dyspnoea with inspiratory indrawing of the lower chest and suprasternal notch. There may be brnochiectasis. · Steatorrhoea and stools are pale in color, greasy and with bad odour.· Infant is cachectic with distended abdomen.
3. In older children, there is steatorrhoea and wasting. Attacks of respiratory infection. Cirrhosis of liver with portal hypertension.
4. In adults, steatorrhoea and long standing pulmonary infection and choroiditis. There may be bilateral enlargement of submaxillary salivary glands. Excessive loss of salts through sweat.
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