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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
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

slide3

Is an inherited disease RLT an autosomal recessive trait, the inherits of defective gene on chromosome 7 from both parents (incidence 1:4),

slide4
Defect of the gene on chromosome 7 responsible for functioning of

the cystic fibrosis transmembrane regulator (CFTR)

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The disease involves mechanical obstruction in several organs caused by the increased viscosity of mucous gland secretion.
pathophysiology
Pathophysiology
  • Absence of the CFTR as CL- channel interferes with NaCL transport, prohibiting movement of water across cell membrane.
  • Sweat test:- (pilocarpine intophoreses) analyse Na and CL, (Gold standard for dx)
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Pancreatic ducts clogged with mucous, prevent

Pancreatic enzymes from reaching duodenum,

Impairing digestion and absorption.

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MeconiumIlieus: abdominal distention, vomiting, failure to pass stools, rapid development of dehydration.

  • Gastrointestinal manifestations:
  • Large, bulky, loose, frothy, extremely foul-smelling stools.
  • Various appetite (early) then loss of appetite (later)
  • Weight loss, marked tissue wasting
  • Failure to growth
  • Distended abdomen
  • Thin extremities
  • Sallow skin
  • Anemia
  • Deficiency of fat soluble vitamins A,D, E
therapeutic management

Abx (prophylactic)

  • Chest physio-therapy &flatter mucous clearance device, exercise. mucolytics
  • Bronchodilator (aerosol).
  • Recombinant human deoxyribonuclease (decrease viscosity)
  • O2 therapy (co2,o2 narcosis pneumothorax)
  • Replacement of pancreatic enzymes –Cryon- (administer before or with meals to achieve normal growth and decrease stool.
  • High protein, high caloric diet.
  • Fat soluble vitamins (A, D, E and K)
Therapeutic Management
slide20
Pulmonary complications

Pancreatic deficiency is less problematic if adequate nutrition is insures.

Life expectancy=30yrs, terminal

Death= resistance pulmonary organism

Fibrosis + destruction of lung tissue.

Screening

Fetus: detection of two CF mutations, DNA analysis of amniotic fluid shows intestinal alkaline phosphates is reduced.

Siblings: carrier, DNA-study

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