Cystic+Fibrosis

toc


 * =Pathophysiology =
 * Mutation in a single gene on chromosome 7à encodes the CF transmembrane conductance regulator.
 * Defective chloride transport in epithelial cells in lungs, pancrease, liver, GIT, reproductive organs.
 * a/w decreased Na/ water transport à dehydration / viscous secretionsà luminal obstruction + scarring of exocrine glands.
 * Present with :
 * Pancreatic insufficiency
 * Meconium ileus
 * DM
 * Obstructive hepatobiliary tract disease
 * Azoospermia
 * Chronic pulmonary infections ( 1ry cause of M& M)
 * =Diagnosis =
 * Sweat chloride concentration > 80 mEq/L + clinical manifestation ( cough, chronic purulent sputum, exertional dyspnea)
 * Family Hx of the disease
 * Chronic Sinusitis is universal
 * Malabsorption with a response to pancreatic enzymes
 * BPL shows neutophelia
 * =Treatment=
 * Directed towards alleviation of symptoms and correction of organ dysfunction.
 * Clearance of Airway Secretions: Chest PT, postural drainage.
 * Bronchodilators Therapy: consider when pt has an increase of 10% or more in FEV1 in response to an inhaled bronchodilator
 * Reduction in viscoelasticity of sputum: Recombinant human deoxyribonuclease I cleaves DNA from neutrophils
 * Antibiotic Therapy: based on sputum cultures, many pt are on long term maintenance Abx.
 * Pancreatic enzyme replacement
 * =Management of Anesthesia=
 * Elective surgery should be delayed until optimal pulmonary function
 * Vit K if hepatic function is poor
 * Volatile anesthetics dec bronchial SM tone, dec responsiveness of hyperreactive AW.
 * Humidification of inspired gas
 * Hydration
 * Avoidance of anticholinergic drugs
 * Frequent tracheal suctioning.
 * Frequent tracheal suctioning.

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