Congenital bronchiectasis

Alternative names
Acquired bronchiectasis; Bronchiectasis

Definition
Bronchiectasis is an abnormal destruction and dilation (widening) of the large airways. A person may be born with it (congenital bronchiectasis) or may acquire it later in life as a result of another disorder.

Causes, incidence, and risk factors

Bronchiectasis is often caused by recurrent inflammation or infection of the airways. It may be present at birth, but most often begins in childhood as a complication from infection or inhaling a foreign object.

Cystic fibrosis causes about 50% of all bronchiectasis in the United States today. Recurrent, severe lung infections (pneumonia, tuberculosis, fungal infections), abnormal lung defenses, and obstruction of the airway by a foreign body or tumor are some of the predisposing factors.

Symptoms often develop gradually, and may occur months or years after the event that causes the bronchiectasis.

Symptoms

     
  • Chroniccough with large amounts of foul-smelling sputum production  
  • Coughing up blood  
  • Cough worsened by lying on one side  
  • Shortness of breath worsened by exercise  
  • Weight loss  
  • Fatigue  
  • Clubbing of fingers may be present (abnormal amount of tissue in the fingernail beds)  
  • Wheezing  
  • Skin discoloration, bluish  
  • Paleness  
  • Breath odor

Signs and tests

When listening to the chest with a stethoscope, the doctor may hear small clicking, bubbling, rattling, or other sounds, usually in the lower lobes of the lungs.

Tests may include:

     
  • A chest X-ray  
  • A chest CT  
  • A sputum culture  
  • A CBC, may reveal anemia and differential may show evidence of fungus infection  
  • A sweat test or other cystic fibrosis testing  
  • Serum Immunoglobulin analysis  
  • Serum precipitins (testing for antibodies to the fungus, aspergillus)  
  • A PPD (purified protein derivative) skin test for prior TB infection

Treatment

Treatment is aimed at controlling infections and bronchial secretions, relieving airway obstruction, and preventing complications.

Regular, daily drainage to remove bronchial secretions is a routine part of treatment. A respiratory therapist can teach postural drainage and effective coughing exercises to patients and their families.

Antibiotics, bronchodilators, and expectorants are often prescribed for infections. Childhood vaccinations and a yearly influenza vaccine help reduce the chance of some infections. Avoiding upper respiratory infections, smoking, and pollution may lessen the susceptibility to infection.

Surgical lung resection may be indicated for those who fail to respond to therapy or for massive bleeding.

Expectations (prognosis)

With treatment, most people can lead normal lives without major disability.

Complications

     
  • Cor pulmonale  
  • Recurrent pneumonia  
  • Coughing up blood (hemoptysis)

Calling your health care provider
Call your health care provider if:

     
  • Symptoms worsen or do not improve with treatment  
  • There is a change in color or amount of sputum, or bloody sputum  
  • Chest pain or shortness of breath increases

Prevention

The risk may be reduced if lung infections are promptly treated.

Johns Hopkins patient information

Last revised: December 5, 2012
by Potos A. Aagen, M.D.

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