Infant with cystic fibrosis (CF) are likely to develop – a) Meconitun ileusb) Loose motionsc) Vomitingd) Constipation
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Description: Cystic fibrosis
Cystic fibrosis (mucoviscidosis) is a hereditary disease affecting the exocrine (mucus) glands of the lungs, liver, pancreas and intestines, causing progressive disability due to multisystem failure.
It has an autosomal recessive pattern of inheritance.
Pathogenesis
The basic defect in CF is a mutation in the cystic fibrosis transmembrane regulator (CFTR) gene that regulates chloride conductance channel in the outer membrane of exocrine glands.
Mutation in the CFTR gene
↓
Dysfunction of chloride conductance channel of exocrine glands
↓
Lack of chloride conduction
↓
Secretion of glands becomes more viscous and thick
↓
Blockade of respiratory tract and ducts of exocrine
glands by thick viscid mucus
↓
Manifestations of CF
Clinical manifestations
1) Lungs and sinus disease
Sinusitis
Bronchiectasis
Lower respiratory tract infection
Nasal polyps
Chronic lung disease
Pulmonary hypertension → can cause heart failure
Allergic bronchopulmonary aspergillosis
2) GIT, Liver and pancreas
Meconium ileus
Rectal prolapse
Malabsorption
Pancreatitis
Constipation
Intussusception
Meconium peritonitis
Bile duct obstruction and biliary cirrhosis
3) Endocrine and growth
Diabetes
Osteoporosis
Poor growth
4) Infertility
In men due to the absence of vas deference
In women due to thick cervical mucus.
Category:
Pediatrics
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