Bronchiectasis. Cystic Fibrosis Flashcards
what is the definition of bronchiectasisis ?
irreversible abnormal dilation of the bronchi
what is the etiology of bronchectasisis ?
consequence of cycles of inflammation and destruction of the structural components of the bronchial wall and mucus plugging
infection usually the cause of inflammation - pseudomonas , homophilus
adenovirus and influenza virus
staphylococcus and klebsiella
disorder of mucus plugging clearing
cystic fibrosis
primary cillary dyskinesia
COPD
smoking = poor Hillary movement’s
what are the clinical features of bronhciectasisi ?
CHRONIC PRODUCTIVE COUGH LASTING MONTHS TO YEARS
copious mucopurlent sputum
dyspnea
hemopptysis
physical examination of bronchiectasisis ?
wheezing - due to obstruction
crackles and rhonchi (in obstruction)
how to diagnose bronchiectasisis ?
chest x ray
bronchi - tram trac lines
thin walled cysts - dilated bronchi formic sacs
late stage bronchietasisi - honey combing
CT - NEEDED
dilated bronchi with thickened walls - signet ring appearance and
tram track lines
cysts at bronchial ends and honeycombing
spirometry
obstructive pulmonary disease
sputum culture = determine infectious etiology
or bronchoalveolar lavage
treatmnet of bronchiectasis?
cannot be reversed so conservative
antibiotic therapy of exacerbations
smoking cessation
influenza and pneumococcal seasonal vaccinations
if obstructive symptoms - bronchodilators , corticosteroids and nebuliser hypertonic saline can e used
invasive - surgical resection of bronchiectatc lung or lobectomy = indicated in pulmonary hemrrhafe , inviability of the bronchus and substantial prutum production
complications of bronchiectasis ?
recurrent bronchopulmonary infections - COPD - respiratory failure and cor pulmonate
pulmonary hemorrhage
lung abcess
etiology of cystic fibrosis ?
hereditary autosomal recessive disorder caused by defective CFTR
long arm of chromosome 7
delta F508 nucleotide deletion
what are the clinical features of cystic fibrosis ?
Signs of chronic respiratory insufficiency: digital clubbing associated with chronic hypoxia
Meconium ileus (in newborns)
Failure to thrive (due to malabsorption)
Pancreatic disease
Pancreatitis
Exocrine pancreatic insufficiency
Foul-smelling steatorrhea
Abdominal distention
Diarrhea
Deficiency of fat-soluble vitamins
CF-related diabetes mellitus (CFRD)
Cholecystolithiasis, cholestasis
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COPD with bronchiectasis
Chronic sinusitis: nasal polyps
Recurrent or chronic productive cough and pulmonary infections
S. aureus is the most common cause of recurrent pulmonary infection in infancy and childhood.
P. aeruginosa is the most common cause of recurrent pulmonary infections in adulthood.
obstruction: expiratory wheezing and/or dyspnea.
Barrel chest ,
Hemoptysis
salty sweat
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Urinary
Nephrolithiasis, nephrocalcinosis
Frequent urinary tract infections
Genital
Men: usually infertile
Obstructive azoospermia is common,
Women: reduced fertility
physical examination of CF
moist rales (indicate pneumonia),
hyperresonance to percussion
diagnosis of CYStic fibrosis?
sweat chloride test.
chloride value ≥ 60 mmol/L
30–59 mmol/L are considered intermediate range
DNA testing for the two CFTR mutations is indicated to confirm the diagnosis
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Stool: ↓ chymotrypsin and pancreatic elastas
Blood: Contraction alkalosis and hypokalemia
spirometry is obstructive
treatment of cystic fibrosis?
mucolytic.
Aerosolized dornase alpha
N-acetylcysteine)
Bronchodilator therapy (e.g., albuterol)
Chest physiotherapy (e.g., postural drainage with percussion)
High-dose ibuprofen has been shown to reduce respiratory disease progression
In chronic respiratory insufficiency
Long-term oxygen inhalation therapy
Lung transplantation is a treatment option for patients with end-stage lung disease
Diet
Additional sodium chloride intake
High-energy diet to compensate for increased demand
Pancreatic enzyme supplements
Oral supplementation of fat-soluble vitamins
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ΔF508 mutation in the CFTR gene
Ivacaftor
Lumacaftor
Tezacaftor
Elexacaftor
