DR . ABDUL HAMEED AL QASEER Bronchiectasis DR . ABDUL HAMEED AL QASEER

BRONCHIECTASIS Definition: Abnormal and permanent dilation of bronchi. Focal or diffuse distribution Clinical consequences – chronic and recurrent infection and Pooling of secretions in dilated airways. Classification: Cylindrical (fusiform) Saccular Varicose

The induction of bronchiectasis required : The affected airways show a variety of changes including transmural inflammation ,mucosal edema ,& ulcerations . The induction of bronchiectasis required : 1. an infectious insult. 2. airways obstruction . 3. reduced clearance , &/or 4. a defect in host defense .

Aetiology: A. Infections-Micro-organisms Measles and Pertussis Adeno & Influenza virus Bacterial infection with virulent organisms: S.aureus, Klebsiella Anaerobes Atypical mycobacteria Mycoplasma HIV Tuberculosis Fungi

AETIOLOGY : IMPAIRED HOST DEFENCE Local causes: Endobronchial obstruction Generalised impairment: 1. Immunoglobulin deficiency 2. Primary ciliary disorders 3. Cystic fibrosis

AETIOLOGY : NON-INFECTIOUS Toxins or toxic substances NH3; gastric contents Immune responses, ABPA Inflammatory diseases: ulcerative colitis, rheumatoid arthritis, Sjögren syndrome. -1-Antitrypsin deficiency Yellow nail syndrome

Causes of Bronchiectasis

CLINICAL MANIFESTATIONS Persistent or recurrent cough with purulent sputum. Haemoptysis Initiating episode: Severe pneumonia, or insidious onset of symptoms or asymptomatic or non-productive cough – dry bronchiectasis in upper lobe, Dyspnoea, wheezing – widespread bronchiectasis or underlying COPD. Exacerbation of infection: Sputum volume increase, purulence or blood.

Symptoms of Bronchiectasis

PHYSICAL EXAMINATION Any combination of rhonchi, creps or wheezes. Clubbing of digits. Chronic hypoxaemia  cor pulmonale  R heart failure Amiloidosis (rare)

DIAGNOSIS - 1 Clinical Radiology: Chest XR: May be non-specific mild disease – normal XRC advanced disease – cysts + fluid levels peribronchial thickening, “tram tracks”, “ring shadows” CT Scan: Peribronchial thickening, dilated bronchioles. Sputum culture: Pseudomonas aeuruginosa, H.influenzae.

DIAGNOSIS - 2 Lung function: Airflow obstruction – FEV1 decreased. Air trapping - RV increased Sweat test – increased sodium and chloride in cystic fibrosis Bronchoscopy: Obstruction – foreign body, tumor. Immunoglobulin Cilia function and structure – Kartagener syndrome.

TREATMENT - 1 4 Goals: 1. Eliminate cause 2. Improve tracheo bronchial clearance 3. Control infection 4. Reverse airflow obstruction

TREATMENT - 2 2. Antituberculous drugs 3. Corticosteroids (ABPA) 1. Immunoglobulin 2. Antituberculous drugs 3. Corticosteroids (ABPA) 4. Remove aspirated material Chest physical therapy Mucolytics Bronchodilators

TREATMENT - 3 Antibiotics – short course, prolonged course, intermittent regular courses, inhalation. Initial empiric Rx: Ampi, Amox, Cefaclor, Septran Ps.aeruginosa – Quinolone, aminoglycoside, 3rd generation cephalosporin, pipracillin. Surgery: Oxygen and diuretics Lung transplant

Antibiotic therapy For most patients with bronchiectasis , the appropriate antibiotic are the same as those used in COPD ; however , in general , larger doses & longer courses are required . If the patients not improved antibiotic therapy should be guided by the microbiological sensitivity for example , Pseudomonas species , oral ciprofloxacine ( 250-750mg / 12h) or ceftazidime I.V. (1-2g /8h ) .

Complications

Surgical treatment Surgical excision in bronchiectasis is indicated in few cases . Young patients with unilateral disease & confined to a single lobe or segment who not controlled by medical therapy may get benefit from surgery . Unfortunately ,many patients in whom medical treatment unsu – ccessful are also unsuitable for surgery because of either extensive disease or coexisting chronic lung disease .

Cystic Fibrosis CF is an autosomal recessive disease as a result of mutation affecting a gen on the long arm of chromosome 7 , which codes for a chloride known as cystic fibrosis transmembrane cond - uctance regulator CFTR. CF is a monogenic disorder that presented as a multisystemic . The first signs & symptoms occur in childhood , but ~ 5% of patients in US diagnosed in adulthood . Because of improvement in therapy ~ 46% now > 18 years & > 16% are > 30 years old .

Clinical manifestation The upper & lower respiratory tract are commonly affected in CF

Other complications

Diagnosis of CF The diagnosis of CF rests on the combination of clinical features & abnormal CFTR function. Sweat test : Elevated sweat chloride are nearly pathognomonic in CF ( > 70 meq /l in adult) 2. Nasal transepithelial electric PD 3. CFTR mutation analysis .

Management of CF The management of CF is that of severe bronchiectasis . Regular nebulised antibiotics therapy ( colomycin or tobramycin ) is used to suppress chronic Pseudomonas infection .. Aspergillus & atypical mycobacterium are also frequently found . Some patients with CF have coexistent asthma . There is a clear link between good nutrition& prognosis in CF . Malabsorption is treated with oral pancreatic enzymes & vitamins Diabetes eventually appears in 25% . Osteoporosis secondary to malabsorption should be treated . Somatic gene therapy may the near future therapy .

Treatment that reduced exacerbations &/or improved lung function in CF

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