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Long Term R espiratory D ysfunction

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 R espiratory D ysfunction

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  1. Long Term Respiratory Dysfunction Cystic Fibrosis CF

  2. 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

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

  4. Defect of the gene on chromosome 7 responsible for functioning of the cystic fibrosis transmembrane regulator (CFTR)

  5. The disease involves mechanical obstruction in several organs caused by the increased viscosity of mucous gland secretion.

  6. 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)

  7. Pancreatic ducts clogged with mucous, prevent Pancreatic enzymes from reaching duodenum, Impairing digestion and absorption.

  8. Medium for bacterial growth

  9. Diagnostic evaluation

  10. 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

  11. Pulmonary manifestation

  12. 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

  13. 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

  14. Respiratory

  15. Respiratory

  16. Digestive

  17. Chest physiotherapy CPT

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