Infant with cystic fibrosis (CF) are likely to develop –
Correct Answer: Meconiumileus
Description: Ans. is 'a' i.e., Meconium ileus Cystic fibrosis o Cystic fibrosis (mucoviscidosis) is a hereditary disease affecting the exocrine (mucus) glands of the lungs, liver, pancrease and intestines, causing progressive disability due to multisystem failure. o It has an autosomal recessive pattern of inharitance. Pathogenesis o The basic defect in CF is mutation in the cystic fibrosis transmembrane regulator (CFTR) gene that regulates chloride conductance channel in outer membrane of exocrine glands. Clinical manifestations 1) Lungs and sinus disease Sinusitis Bronchiactasis Lower respiratory tract infection Nasal polyps Chronic lung disease Pulmonary hypeension can cause hea failure Allergic bronchopulmonary aspergillosis 2) GIT, Liver and pancrease 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) Infeility In men due to absence of vas deference In women due to thick cervical mucus.
Category:
Pediatrics
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