Annals of Thoracic Medicine
: 2007  |  Volume : 2  |  Issue : 1  |  Page : 2-

Noncystic fibrosis bronchiectasis: Is it an orphan disease?

Khalid F Mobaireek 
 College of Medicine, King Saud University, Saudi Arabia

Correspondence Address:
Khalid F Mobaireek
College of Medicine, King Saud University
Saudi Arabia

How to cite this article:
Mobaireek KF. Noncystic fibrosis bronchiectasis: Is it an orphan disease?.Ann Thorac Med 2007;2:2-2

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Mobaireek KF. Noncystic fibrosis bronchiectasis: Is it an orphan disease?. Ann Thorac Med [serial online] 2007 [cited 2020 Nov 25 ];2:2-2
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Bronchiectasis is a chronic lung disease, defined pathologically as irreversible dilatation of the bronchi; it was first described by Laennec in 1819.[1],[2] Orphan disease is a disease that is so rare that it is not considered commercially viable to develop drugs to treat it.[3] Disease is considered orphan either because it is rare or it is more prevalent in developing countries than in the developed world. Bronchiectasis unrelated to cystic fibrosis (CF) was termed an 'orphan disease.'[4] The hospital admissions for bronchiectasis declined in the developed countries since the 1950s. This decline has been attributed to improved sanitation, nutrition, childhood immunization, and the early and frequent use of antibiotics.[5] However, over the last decade more children are being diagnosed as non-CF bronchiectasis due to the increased use of high resolution computerized tomography.[5] There is a significant under-diagnosis because of low clinical suspicion. The current prevalence in the developed countries is estimated to be 172-335/million children.[5] Though infections remained as an important cause, the causative organisms have changed from tuberculosis, measles and pertussis in the past to adenovirus, herpes and HIV in the present time. Adam[6] reviewed cases of recurrent or persistent pneumonia without apparent cause in all children admitted to King Khalid University Hospital in Riyadh from 1986 to 1989. Out of 781 patients admitted with pneumonia, 18 children met the study inclusion criteria. Children known to have hematological diseases, malignancy, nutritional disorders and immune disorders were excluded. All had associated etiologies: 33% had immunodeficiency, 28% had primary structural and functional anomalies and tumors of the respiratory tract and great vessels, 22% had infections, 11% had idiopathic pulmonary fibrosis and 6% had asthma. In this issue, Dr. Banjar[7] reviewed the experience at King Faisal Specialist Hospital and Research Centre from 1993 to 2005. She found associated disease in 91 (60%) of the cases. These associated diseases included pulmonary diseases in 48 (32%), immunodeficiency in 27 (18%), CNS anomalies in 10 (7%) and cardiac abnormalities in 10 (7%). These are not different from the causes in the developed countries. Measles, pertussis and tuberculosis are no longer a major cause of bronchiectasis in Saudi Arabia as immunization is almost universal and nutrition has improved. The availability of more sophisticated, less invasive and more sensitive radiological studies for bronchiectasis will lead to increased detection of the disease throughout the world. Earlier diagnosis of even mild disease will also change the outcomes, including the possibility of complete resolution. The definition of bronchiectasis may also need revision as the disease may not be permanent in all cases. With these changes, research needs to be encouraged and this frustrating disease should not be orphan any more.[8],[9] For this the Annals of Thoracic Medicine published a review article[1] in its first issue; and in this issue, an original study[7] besides this editorial - all about bronchiectasis.


1La'λnnec RT. A treatise in the diseases of the chest and on mediate auscultation. 4th ed. (1819). Translation by J Forbes. Longman: London; 1834.
2AL-Shirawi N, AL-Jahdali Hamdan H, Al Shimemeri A. Pathogenesis, etiology and treatment of bronchiectasis. Ann Thorac Med 2006;1:41-51.
3The American HeritageŽ Stedman's Medical Dictionary, 2nd ed. Houghton Mifflin Company: 2004.
4Barker AF, Bardana EJ Jr. Bronchiectasis: Update of an orphan disease. Am Rev Respir Dis 1988;137:969-78.
5Eastham KM, Fall AJ, Mitchell L, Spencer DA. The need to redefine non-cystic fibrosis bronchiectasis in childhood. Thorax 2004;59:324-7.
6Adam KA. Persistent or recurrent pneumonia in Saudi children seen at King Khalid University Hospital, Riyadh: Clinical profile and some predisposing factors. Ann Trop Paediatr 1991;11:129-35.
7Banjar HH. A review of 151 cases of pediatric non-cystic fibosis bronchiectaisis in a tertiary care center. Ann Thorac Med 2007;2:7-12.
8Karakoc GB, Yilmaz M, Altintas DU, Kendirli SG. Bronchiectasis: Still a problem. Pediatr Pulmonol 2001;32:175-8.
9Callahan CW, Redding GJ. Bronchiectasis in children: Orphan disease or persistent problem? Pediatr Pulmonol 2002;33:492-6.