Blepharitis Disease

BASICS

DESCRIPTION
Chronic irreversible, abnormal dilatation of the bronchi, usually accompanied by infection and productive cough
  • System(s) affected: Pulmonary
  • Genetics: Associated with many conditions including some that are congenital or hereditary
  • Incidence/Prevalence in USA:
    • No reliable figures available
    • Less common than it once was, probably due to more effective treatment of childhood respiratory infections
  • Predominant age: Begins most often in early childhood, but symptoms may not appear until later in life
  • Predominant sex: Male = Female
SIGNS AND SYMPTOMS
  • Cough
  • Sputum - copious and purulent
  • Hemoptysis
  • Wheezing
  • Coarse or moist crackles
  • Cyanosis
  • Digital clubbing
  • Dyspnea
  • Barrel chest
  • Emaciation
  • Fatigue
  • Fever
  • Recurrent pneumonia
  • Tachycardia
  • Tachypnea
CAUSES
  • Alpha-1-antitrypsin deficiency
  • Allergic bronchopulmonary aspergillosis
  • Bronchial obstruction
  • Cystic fibrosis
  • Dyskinetic cilia syndromes
  • Hypogammaglobulinemia
  • Inhaling noxious chemicals
  • Kartagener's syndrome (situs inversus, sinusitis, immotile spermatozoa, bronchiectasis)
  • Necrotizing pulmonary infections
  • Pulmonary abscess
  • Severe lung infection in childhood (measles, adenovirus, influenza, pertussis, or bronchiolitis)
  • Tuberculosis
  • Congenital immunodeficiency syndromes
  • Chronic aspiration
  • Rheumatic diseases
  • Transplant graft rejection
RISK FACTORS
  • Repeated bouts of pneumonia
  • Any chronic respiratory illness
  • Retained foreign body
  • Immunodeficiency

DIAGNOSIS

LABORATORY
  • Positive sputum culture (yields H. influenzae, Streptococcus pneumoniae, staphylococcal, klebsiella, pseudomonas, or anaerobes)
  • Hypoxemia
  • Leukocytosis, usually
  • Serum immunoglobulins - check for hypogammaglobulinemia, IgE level helpful

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS
  • Bronchial dilation
  • Inflamed bronchi
  • Purulent bronchorrhea
  • Necrosis of bronchial mucosa
  • Peribronchial scarring
SPECIAL TESTS
  • Sweat test
  • Skin test for aspergillus
  • Bronchoscopy useful in locating bleeding site and to exclude adenoma or foreign body
  • Ciliary biopsy with electron microscopy (EM)
  • Pulmonary function tests show variable obstruction and restriction
  • Sputum culture/sensitivity, AFB, fungus
IMAGING
  • Bronchography
    • For definitive diagnosis, to help determine extent, and if surgery contemplated
    • Bronchial dilation, truncated
  • Chest x-ray
    • Can be normal
    • Coarse lung markings - honeycomb/tram tracks
    • Air-fluid level
    • Cystic lesions
    • Atelectasis
  • CT scan
    • Shows dilation of airways with signet rings
    • High resolution CT is best to establish diagnosis and extent of disease
    • Spiral CT helpful for questionable findings
DIAGNOSTIC PROCEDURES
  • Fiberoptic bronchoscopy
    • Recommended when disease is of recent onset or is unilateral
    • May be combined with bronchography
    • Obtain culture

TREATMENT

APPROPRIATE HEALTH CARE

Outpatient except for possible surgery

GENERAL MEASURES
  • Airway clearance techniques
    • Chest physical therapy
    • Percussion
  • Postural drainage
  • Hydration
  • Pulmonary rehabilitation to improve functional status
  • Noninvasive positive pressure ventilation, nocturnal or chronic
  • Bronchial artery embolization may be lifesaving for massive pulmonary hemorrhage
  • Avoid cigarette smoking
  • Bronchoscopy may be required for extraction of mucus or mycelial plugs, or if physiotherapy has failed
SURGICAL MEASURES

Segmental pulmonary resection for localized disease or refractory hemoptysis

ACTIVITY

As fully active as possible

DIET

No restrictions

PATIENT EDUCATION

Printed patient information available from: American Lung Association, 1740 Broadway, New York, NY 10019, (212)315-8700

FOLLOW UP

PREVENTION/AVOIDANCE
  • Treat all pneumonias adequately
  • Immunizations for viral illnesses (i.e., influenza)
  • Immunization for pneumococcal pneumonia
  • Routine childhood immunizations, e.g., pertussis, measles, Hib
  • Genetic counseling if inherited etiology
POSSIBLE COMPLICATIONS
  • Recurrent pulmonary infections
  • Pulmonary hypertension
  • Secondary amyloidosis
  • Cor pulmonale
  • Brain abscess
  • Massive hemoptysis
  • Atelectasis
  • Lung abscess
EXPECTED COURSE AND PROGNOSIS
  • Chronic. Surgery may be curative if disease localized.
  • Average life expectancy - 55 years

MISCELLANEOUS

ASSOCIATED CONDITIONS
  • Sinusitis
  • Cor pulmonale
  • Kartagener syndrome
  • Cystic fibrosis
AGE-RELATED FACTORS

Pediatric: Cystic fibrosis and other congenital disorders
Geriatric: Elderly more likely to need hospitalization for treatment
Others: N/A

PREGNANCY

N/A

OTHER NOTES

Conditions that may lead to bronchiectasis include severe pneumonia (especially measles, pertussis, adenoviral infections in children), necrotizing infections due to Klebsiella, staphylococci, influenza virus, fungi, mycobacteria, mycoplasma, bronchial obstruction from any cause (foreign body, carcinoma, enlarged mediastinal lymph nodes

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

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