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CYSTIC FIBROSIS- AT A GLANCE

CYSTIC FIBROSIS: A GENETIC AUTOSOMAL DISORDER

CYSTIC FIBROSIS: SYMPTOMS

CARRIER SCREENING TEST FOR CYSTIC FIBROSIS

CYSTIC FIBROSIS AND THE DIGESTIVE SYSTEM

 

 

CYSTIC FIBROSIS AND THE DIGESTIVE SYSTEM

The patient with cystic fibrosis shows abnormity in function of cystic fibrosis transmembrane conductance regulator gene in their body, control the flow of chloride ions and water across few body cells. The mutation in the gene causes the cell protein to behave abnormally thus altering the chloride flow in the body and thus excess of mucus, sweat and water is secreted as body reflex in patients. The patients with cystic fibrosis may have complications in their respiratory, digestive and reproductive system. Thick and sticky mucous is secreted by the cell of the affected system, which in turn clogs the passage and causes obstruction in respiration, digestion and reproduction.

The pancreas is mostly affected under the digestive system complication in patients with cystic fibrosis. Pancreas is an exocrine gland which secretes certain digestive enzymes which aids in food absorption and digestion. The deficiency of such enzymatic secretion by pancreas of patients with cystic fibrosis in turn lead to improper fat absorption in the body due to incomplete breakdown of fat content in the food. The pancreatic duct also gets obstructed due to increased thick secretions from pancreases, which causes difficulty in absorption of fats, certain proteins and vitamins like A, B, D, E and K. The pancreatic insufficiency in cystic fibrosis leads to passing large quantity, mushy and foul smelling stool. The stool is generally greasy and pale in color, which due to excess of fat in it, floats on the water of the toilet.

The occurrence of pancreatic insufficiency in patients with cystic fibrosis depends on the genotype of cystic fibrosis patient has and may not occur in all CF patients. The pancreatic sufficient patients show milder degree of respiratory complication and also lower value of sweat chloride as compared to patients with pancreatic insufficiency. In severe cases of cystic fibrosis the endocrine cells of the pancreases also gets destroyed thus causing insulin dependant diabetes. Thirty five percent of patients with cystic fibrosis developed this kind of diabetes in their 20s, where the chance increases to fifty persons in [patients in their 30s. The liver may also get affected in cystic fibrosis due to blockage in the flow if bile and manifests itself as swollen abdomen due to enlarged liver, vomiting of blood, and ultimately cirrhosis of liver. The gall bladder also gets affected in patients with cystic fibrosis and stones in gall bladder may develop.

 

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