Bronchiectasiz

Question 1

Definition

Answer 1

Abnormal dilatation of the bronchi after a chronic suppurative airway infection with sputum production, progressive scarring and lung damage

Question 2

Causes of bronchiectasis( congenital , adult and children )

Answer 2

Congenital defect like cystic fibrosis, ciliary dysfunction syndrome (primary ciliary dyskinesia, kartageners syndrome ) , primary hypogammaglobulinaemia

Children - Damage of airways by infection ( whooping cough, measles) inhaled toxin or foreign body, Tuberculosis ( most common)

Adults - suppurative pneumonia, pulmonary tuberculosis, allergic bronchopulmonary aspergillosis complicating asthma , bronchial tumors

Question 3

Pathophysiology of bronchiectasis

Answer 3

Damage of the airway, chronic inflammation and infection in the airways -> sputum hypersécrétion -> impaired mucociliary clearance -> lung damage

Question 4

Symptoms of bronchiectasis

Answer 4

 Chronic daily, persistent cough
Copious,, continuously purulent sputum
Pleuritic chest pain
Hemoptysis
Infective exacerbation with fever, malaise, and anorexia
Halitosis
General debility

Question 5

Physical signs of bronchiectasis

Answer 5

Only present if secretions are present or there is lobar collapse

Coarse crackles
Diminished breath sounds
Hemoptysis

Question 6

Investigation of bronchiectasis

Answer 6

Sputum culture for pseudonomas aeruginosa and staphylococcus aureus and mycobacteria

Chest x-ray in advanced dx with thick airway walls , cystic bronchiectatic spaces from pneumonic consolidation or collapse.

Screening test for congenital disease like ciliary dysfunction syndrome, ( saccharin test )

Biopsy for ciliary beats frequency, taken from the nose

Question 7

Management of bronchiectasis

Answer 7

Physiotherapy for drainage of excess bronchial secretion

Antibiotic therapy

Surgical treatments with excision of bronchictic area

Question 8

Most fatal genetic disease in Caucasian

Answer 8

Cystic fibrosis

Question 9

Type of inheritance of cystic fibrosis

Answer 9

Autosomal recessive inheritance

Question 10

Incidence of cystic fibrosis

Answer 10

One in 2500 live births

Question 11

Chromosome affected in cystic fibrosis

Answer 11

Chromosome seven

Question 12

Name of channel mutated in cystic fibr

Answer 12

CFTR cystic fibrosis transmembrane, conductance regulator

Question 13

Pathophysiology of cystic fibrosis

Answer 13

Gene defect -> increased sodium and chloride in sweat and increased resorption of sodium and water from respiratory epithelium-> dehydration of the airway Epithelium -> predisposing individual to chronic bacterial infection and ciliary dysfunction-> bronchiectasis.

Question 14

Clinical features of cystic fibrosis

Answer 14

Normal lung size with bronchiolar information, infection, and bronchiectasis symptoms
Infertility in men

Question 15

Management of cystic fibrosis

Answer 15

Chest physiotherapy, if sputum
Antibiotics if infection Although antibiotic resistant is common
Inhaled bronchodilators and glucocorticoids
Lung transportation in severe cases oxygenation at home