Long Term R espiratory D ysfunction. Cystic Fibrosis CF. Cystic Fibrosis CF. A multi-system disorder of exocrine glands, leading to increased production of thick mucous in bronchioles, small intestines and pancreatic and bile ducts.
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Long Term Respiratory Dysfunction
Cystic Fibrosis CF
A multi-system disorder of exocrine glands, leading to increased production of thick mucous in bronchioles, small intestines and pancreatic and bile ducts
Defect of the gene on chromosome 7 responsible for functioning of
the cystic fibrosis transmembrane regulator (CFTR)
Pancreatic ducts clogged with mucous, prevent
Pancreatic enzymes from reaching duodenum,
Impairing digestion and absorption.
Medium for bacterial growth
Pancreatic deficiency is less problematic if adequate nutrition is insures.
Life expectancy=30yrs, terminal
Death= resistance pulmonary organism
Fibrosis + destruction of lung tissue.
Fetus: detection of two CF mutations, DNA analysis of amniotic fluid shows intestinal alkaline phosphates is reduced.
Siblings: carrier, DNA-study