Infant with cystic fibrosis (CF) are likely to develop – a) Meconitun ileusb) Loose motionsc) Vomitingd) Constipation

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.