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
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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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