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Bronchiectasis

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Bronchiectasis
Classification and external resources
ICD-10 J47., Q33.4
ICD-9 494, 748.61
DiseasesDB 1684
MedlinePlus 000144
eMedicine med/246
MeSH D001987

Bronchiectasis is a disease state defined by localized, irreversible dilation of part of the bronchial tree. It is classified as an obstructive lung disease, along with emphysema, bronchitis and cystic fibrosis. Involved bronchi are dilated, inflamed, and easily collapsible, resulting in airflow obstruction and impaired clearance of secretions. Bronchiectasis is associated with a wide range of disorders, but it usually results from necrotizing bacterial infections, such as infections caused by the Staphylococcus or Klebsiella species or Bordetella pertussis.[1]

Contents

Signs and symptoms

People with bronchiectasis produce frequent green/yellow sputum (what is distinctive is that patients with bronchiectasis can fill 240ml (8 oz) glasses with their daily sputum production). There may have bad breath and, when associated with cystic fibrosis, diarrhoea (because patients with bronchiectasis often develop chronic pancreatitis).

Diagnosis

The diagnosis of bronchiectasis is based on the review of clinical history and characteristic patterns in high-resolution CT scan findings. Such patterns include "tree-in-bud" abnormalities and cysts with definable borders. In one small study, CT findings of bronchiectasis and multiple small nodules were reported to have a sensitivity of 80%, specificity of 87%, and accuracy of 80% for the detection of bronchiectasis. Bronchiectasis may also be diagnosed without CT scan confirmation if clinical history clearly demonstrates frequent respiratory infections as well as confirmation of an underlying problem via blood work and sputum culture samples.[2]

Causes

Bronchiectasis, gross pathology.
Bronchiectasis secondary to a large carcinoid tumor (not shown) that was completely obstructing the bronchus proximally. The yellowish discoloration of lung parenchyma reflects obstructive pneumonia.

Bronchiectasis has both congenital and acquired causes, with the latter more frequent.

Acquired causes

Acquired Immune Deficiency Syndrome (AIDS) is the leading cause of bronchiectasis, especially in children. AIDS predisposes patients to a variety of pulmonary ailments, such as pneumonia and other opportunistic infections.[3]

Tuberculosis is another major cause. [4]

Bronchiectasis can sometimes be an unusual complication of inflammatory bowel disease, especially ulcerative colitis. It can occur in Crohn's disease as well, but does so less frequently. Bronchiectasis in this situation usually stems from various allergic responses to inhaled fungus spores.[5]

Recent evidence has shown an increased risk of bronchiectasis in patients with rheumatoid arthritis who smoke. One study stated a tenfold increased prevalence of the disease in this cohort.[6] Still, it is unclear as to whether or not cigarette smoke is a specific primary cause of bronchiectasis.

Other acquired causes of bronchiectasis involving environmental exposures include respiratory infections, obstructions, inhalation and aspiration of ammonia and other toxic gases, pulmonary aspiration, alcoholism, heroin (drug use), various allergies,[7] and allergic bronchopulmonary aspergillosis.[8]

Congenital causes

Kartagener syndrome, which affects the mobility of cilia in the lungs,[9] aids in the development of the disease. Another common genetic cause is cystic fibrosis, in which a small number of patients develop severe localized bronchiectasis.[10] Young's syndrome, which is clinically similar to cystic fibrosis, is thought to significantly contribute to the development of bronchiectasis. This is due to the occurrence of chronic, sinopulmonary infections.[11] Patients with alpha 1-antitrypsin deficiency have been found to be particularly susceptible to bronchiectasis, for unknown reasons.[12] Other less-common congenital causes include primary immunodeficiencies, due to the weakened or nonexistent immune system response to severe, recurrent infections that commonly affect the lung.[13] Several other congenital disorders can also lead to bronchiectasis, including Williams-Campbell syndrome and Marfan syndrome.[14]

Prevention

In order to prevent bronchiectasis, children should be immunized against measles, pertussis and other acute respiratory infections of childhood. While smoking has not been found to be a direct cause of bronchiectasis, it is certainly an irritant that all patients should avoid in order to prevent the development of infections (such as bronchitis) and further complications.[15]

A healthy body mass index, vaccination (especially against pneumonia and influenza) and regular doctor visits may have beneficial effects on the prevention of progressing bronchiectasis. The presence of hypoxemia, hypercapnia, dyspnea level and radiographic extent can greatly affect the mortality rate from this disease.[16]

Treatment

Treatment of bronchiectasis includes controlling infections and bronchial secretions, relieving airway obstructions, removal of affected portions of lung by surgical removal or artery embolization and preventing complications. This includes the prolonged usage of antibiotics to prevent detrimental infections,[17] as well as eliminating accumulated fluid with postural drainage and chest physiotherapy. Surgery may also be used to treat localized bronchiectasis, removing obstructions that could cause progression of the disease.[18]

Inhaled steroid therapy that is consistently adhered to can reduce sputum production and decrease airway constriction over a period of time, and help prevent progression of bronchiectasis. One commonly used therapy is beclometasone dipropionate, which is also used in asthma treatment.[19] Use of inhalers such as albuterol (salbutamol), fluticasone (Flovent/Flixotide) and ipratropium (Atrovent) may help reduce likelihood of infection by clearing the airways and decreasing inflammation.[20]

Although not approved for use in any country, Mannitol dry inhalation powder, under the name Bronchitol, has been granted orphan drug status by the FDA for use in patients with bronchiectasis and with cystic fibrosis.[21]

Combination therapies, long acting bronchodilators and inhaled corticosteroids such as Symbicort and Advair Diskus are also commonly used inhaled medicines which has in many cases been effective in clearing the airways, reducing sputum and reducing inflammation.

History

Rene Laënnec, the man who invented the stethoscope, used his invention to first discover bronchiectasis in 1819.[22] The disease was researched in greater detail by Sir William Osler in the late 1800s; it is suspected that Osler actually died of complications from undiagnosed bronchiectasis.[23]

See also

References

  1. ^ Hassan, Isaac (8 December 2006). "Bronchiectasis". eMedicine Specialties Encyclopedia. Gibraltar: WebMD. http://www.emedicine.com/radio/topic116.htm. Retrieved 2007-06-22. 
  2. ^ Miller, JC (2006). "Pulmonary Mycobacterium Avium-Intracellular Infections in Women". Radiology Rounds 4 (2). 
  3. ^ Sheikh S, Madiraju K, Steiner P, Rao M (1997). "Bronchiectasis in pediatric AIDS". Chest 112 (5): 1202–7. doi:10.1378/chest.112.5.1202. PMID 9367458. 
  4. ^ Catanzano, Tara (5 September 2005). "Primary Tuberculosis". eMedicine Specialties Encyclopedia. Connecticut: WebMD. http://www.emedicine.com/radio/topic411.htm. Retrieved 2007-06-22. 
  5. ^ Ferguson HR, Convery RP (2002). "An unusual complication of ulcerative colitis". Postgrad. Med. J. 78 (922): 503. doi:10.1136/pmj.78.922.503. PMID 12185236. 
  6. ^ Kaushik, VV, Hutchinson D, Desmond J, Lynch MP, and Dawson JK (2004). "Association between bronchiectasis and smoking in patients with rheumatoid arthritis". Annals of the Rheumatic Diseases 63 (8): 1001–2. doi:10.1136/ard.2003.015123. PMID 15249329. 
  7. ^ Lamari NM, Martins ALQ, Oliveira JV, Marino LC, Valério N (2006). "Bronchiectasis and clearence physiotherapy: emphasis in postural drainage and percussion" (in Portuguese). Braz. J. Cardiovasc. Surg. 21 (2). 
  8. ^ Allergic Bronchopulmonary Aspergillosis
  9. ^ Morillas HN, Zariwala M, Knowles MR (2007). "Genetic Causes of Bronchiectasis: Primary Ciliary Dyskinesia". Respiration 72 (3): 252–63. doi:10.1159/000101783. PMID 17534128. 
  10. ^ Dalrymple-Hay MJ, Lucas J, Connett G, Lea RE (1999). "Lung resection for the treatment of severe localized bronchiectasis in cystic fibrosis patients". Acta Chir Hung. 38 (1): 23–5. PMID 10439089. 
  11. ^ Handelsman DJ, Conway AJ, Boylan LM, & Turtle JR (1984). "Young's syndrome. Obstructive azoospermia and chronic sinopulmonary infections". NEJM 310 (1): 3–9. doi:10.1056/NEJM198401053100102. PMID 6689737. 
  12. ^ Shin MS, Ho KJ (1993). "Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A rare occurrence?". Chest 104: 1384–86. doi:10.1378/chest.104.5.1384. 
  13. ^ Notarangelo LD, Plebani A, Mazzolari E, Soresina A, Bondioni MP (2007). "Genetic causes of bronchiectasis: primary immune deficiencies and the lung". Respiration + 74 (3): 264–75. doi:10.1159/000101784. PMID 17534129. 
  14. ^ {{cite web|url=http://www.marfantrust.org/what_is_marfan_syndrome/what_causes_marfan_syndrome/|accessdate=2010-12-08|title=Medical Problems and Treatments | The Marfan Trust|publisher=The Marfan Trust|
  15. ^ Crofton J (1966). "Respiratory tract disease. Diagnosis and treatment of bronchiectasis. I. Diagnosis". Br Med J 1 (5489): 721–3 contd. PMID 5909486. 
  16. ^ Onen ZP, Eris Gulbay B, Sen E, Akkoca Yildiz O, Saryal S, Acican T, Karabiyikoglu G (2007). "Analysis of the factors related to mortality in patients with bronchiectasis". Respir Med. 101 (7): 1390–97. doi:10.1016/j.rmed.2007.02.002. PMID 17374480. 
  17. ^ Evans DJ, Bara AI,Greenstone M (2007). "Prolonged antibiotics for purulent bronchiectasis in children and adults". Cochrane Database Syst Rev (2): CD001392. doi:10.1002/14651858.CD001392.pub2. PMID 17443506. 
  18. ^ Otgün I, Karnak I, Tanyel FC, Senocak ME, Büyükpamukçu N (2004). "Surgical treatment of bronchiectasis in children". J. Pediatr. Surg. 39 (10): 1532–6. doi:10.1016/j.jpedsurg.2004.06.009. PMID 15486899. http://linkinghub.elsevier.com/retrieve/pii/S0022346804004026. 
  19. ^ Elborn JS, Johnston B, Allen F, Clarke J, McGarry J, Varghese G. (1992). "Inhaled steroids in patients with bronchiectasis". Respir Med 86 (2): 121–4. doi:10.1016/S0954-6111(06)80227-1. PMID 1615177. 
  20. ^ Reports, Consumer (15 March 2007). "Ipratropium and Albuterol Inhalation - Drug Review". Consumer Reports of U.S.. http://www.consumerreports.org/mg/drug-reports/ipratropium-and-albuterol-inhalation.htm. Retrieved 2007-06-22. 
  21. ^ Waknine, Yael (27 July 2005). "Orphan Drug Approvals: Bronchitol, Prestara, GTI-2040". Medscape today for WebMD. http://www.medscape.com/viewarticle/509116. Retrieved 2007-06-22. 
  22. ^ Roguin, A (2006). "Rene Theophile Hyacinthe Laënnec (1781–1826): The Man Behind the Stethoscope". Clin Med Res 4 (3): 230–35. doi:10.3121/cmr.4.3.230. PMID 17048358. 
  23. ^ Wrong O (2003). "Osler and my father". J R Soc Med 96 (6): 462–64. doi:10.1258/jrsm.96.9.462. PMID 12949207. 

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