Long term r espiratory d ysfunction
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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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Long Term R espiratory D ysfunction

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Long term r espiratory d ysfunction

Long Term Respiratory Dysfunction

Cystic Fibrosis CF


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


Long term r espiratory d ysfunction

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


Long term r espiratory d ysfunction

Defect of the gene on chromosome 7 responsible for functioning of

the cystic fibrosis transmembrane regulator (CFTR)


Long term r espiratory d ysfunction

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)


Long term r espiratory d ysfunction

Pancreatic ducts clogged with mucous, prevent

Pancreatic enzymes from reaching duodenum,

Impairing digestion and absorption.


Long term r espiratory d ysfunction

Medium for bacterial growth


Diagnostic evaluation

Diagnostic evaluation


Long term r espiratory d ysfunction

  • 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


Long term r espiratory d ysfunction

Pulmonary manifestation


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


Long term r espiratory d ysfunction

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


Respiratory

Respiratory


Respiratory1

Respiratory


Digestive

Digestive


Chest physiotherapy cpt

Chest physiotherapy CPT


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