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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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
Is an inherited disease RLT an autosomal recessive trait, the inherits of defective gene on chromosome 7 from both parents (incidence 1:4),
the cystic fibrosis transmembrane regulator (CFTR)
Pancreatic enzymes from reaching duodenum,
Impairing digestion and absorption.
MeconiumIlieus: abdominal distention, vomiting, failure to pass stools, rapid development of dehydration.
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