Bronchiectasis
DR . ABDUL HAMEED AL QASEERBRONCHIECTASIS
Definition: Abnormal and permanent dilation of bronchi.Focal or diffuse distributionClinical consequences – chronic and recurrent infection and Pooling of secretions in dilated airways.Classification: Cylindrical (fusiform)SaccularVaricoseThe 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 AnaerobesAtypical mycobacteria Mycoplasma HIV Tuberculosis Fungi
AETIOLOGY : IMPAIRED HOST DEFENCE
Local causes: Endobronchial obstruction Generalised impairment: 1. Immunoglobulin deficiency 2. Primary ciliary disorders 3. Cystic fibrosisAETIOLOGY : NON-INFECTIOUS
Toxins or toxic substances NH3; gastric contentsImmune responses, ABPAInflammatory diseases: ulcerative colitis, rheumatoid arthritis, Sjцgren syndrome.-1-Antitrypsin deficiency Yellow nail syndromeCauses of Bronchiectasis
CLINICAL MANIFESTATIONS
Persistent or recurrent cough with purulent sputum.HaemoptysisInitiating 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 EXAMINATIONAny combination of rhonchi, creps or wheezes.Clubbing of digits.Chronic hypoxaemia cor pulmonale R heart failureAmiloidosis (rare)
DIAGNOSIS - 1
ClinicalRadiology: Chest XR: May be non-specificmild disease – normal XRC advanced disease – cysts + fluid levelsperibronchial 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 increasedSweat test – increased sodium and chloride in cystic fibrosisBronchoscopy: Obstruction – foreign body, tumor.ImmunoglobulinCilia function and structure – Kartagener syndrome.TREATMENT - 1
4 Goals: 1. Eliminate cause 2. Improve tracheo bronchial clearance 3. Control infection 4. Reverse airflow obstructionTREATMENT - 2
1. Immunoglobulin 2. Antituberculous drugs 3. Corticosteroids (ABPA) 4. Remove aspirated material Chest physical therapy Mucolytics BronchodilatorsTREATMENT - 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 diureticsLung transplantAntibiotic 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 treatmentSurgical 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 CFOther complications