Doç. Dr. Demet CAN

1. Epidemiology of Bronchiectasis Doç. Dr. Demet CAN Dr. Behçet Uz Children Hospital İZMİR

2. Bronchiectasis Laennec first described bronchiectasis in 1819 when he presented the case of a 3-year-old who died of pertussis. At autopsy the child had marked bronchiectasis. Hasse used the term “bronchiectasis” in 1846.

3. Definition • Bronchiectasis is a chronic disease of the conducting airways that produces persistent productive cough, recurrent respiratory infectious exacerbations and obstructive lung disease. Redding GJ. Pediatr Clin N Am 2009;56: 157-171

4. Definition • Reduced airway mucus clearance, • Stasis of infected airway secretions • Regional or diffuse airway wall dilation and destruction with loss of airway structural integrity

5. Incidence • Most series are between 1900-1950s • Up to half of the children developed bronchiectasis following severe pneumonia • Estimated annual incidence of bronchiectasis to be 1.3/1000 Tsang KW, Tipoe GL. Int J Tuberc Lung Dis 2004: 8: 691-702 Clark NS. BR Med J 1963; 1: 80-87

6. Incidence • Beginning in 1947 • 160 children with bronchiectasis • A fall in the annual hospitalization rate for bronchiectasis in five hospitals from approximately 48/10,000 to 10/10,000 Field CE. Pediatrics 1949; 4: 21-46.

7. Incidence • Nigeria • 1975-1979 • 1150 children with thoracic and cardiovascular diseases • Suppurative lung disease: 42% • Bronchiectasis:6% Adebonojo SA, et al. Ann Thorac Surg 1982; 33: 40-47.

8. Incidence • 1994 • Royal Brompton National Heart and Lung Hospital • Children referred for evaluation of respiratory complaints • Only 1% of 4,000 children had bronchiectasis unrelated to CF • Bronchiectasis unrelated to CF was rare Nikolaizik WH, Warner JO. Arch Dis Child 1994; 70: 141-142.

9. Incidence • Finland • Admissions and new cases during the period of 1983-1992 • There were 5,710 admissions for bronchiectasis and 1,928 new cases • The occurrence of cases treated in hospital was 4.9 per million person-years at age 0-14 years, 103.8 at 65 years or over and 38.9 in the total population • The estimated incidence of bronchiectasis was low in Finland, especially in childhood, Saynajakangas O, et al. Cent Eur J Public Health 1998;6:235-237

10. Incidence • Incidence of bronchiectasis is related to socioeconomic status • 4.2 / 100,000 (USA) • Improved sanitation, childhood immunizations against measles and pertussis, better nutrition, effective antimicrobial medication, the decline in the incidence of tuberculosis

11. Orphan Disease • In developed countries classifacation is as with CF BE and non-CF BE • Prevalance of non-CF bronchiectasis is low in developed country (Orphan Disease)

12. Is it an Orphan Disease? “orphan disease“ • It is so rare in the developed countries • Sanitation improved • Measles and pertussis is prevented as a result of childhood immunization • It is not considered commercially viable to develop drugs to treat the condition Barker AF, Bardana EJ Jr. Am Rev Respir Dis 1988; 137: 969-978

13. 2000 • Bronchiectasis in children was low in developed countries • Bronchiectasis in adult was more frequent • 145 adult with BE • In 40% of cases productive cough has begun at age 10 Pasteur MC, et al. Am J Respir Crit Care Med 2000; 162: 1277-1284

14. 2001 • 23 patient with BE • 1993-1999 • 13 boys (57%), 10 girls (43%) • A mean age of 8.45±4.02 years • Infection (4 patients had TB, 4 patients had postpneumonia BE) • A considerable population in Adana was living in neighbors with poor conditions

15. 2002 • Reports from developing countries suggest that childhood bronchiectasis may not be disappearing • Quantitative estimate of prevalence among populations of children worldwide is lacking • More epidemiologic data about childhood bronchiectasis is needed to define high-risk regions and socioeconomic groups.

16. 2005 • 1987-2001 • 111 children with bronchiectasis • 50.5% boys • The mean age of patients: 7.4 ± 3.7 • Mean age of the patients when they had their first respiratory symptoms was 2.5 ± 2.7 • Post-infectious: 29.7% • High rate of consanguinity among patients, with 42.6% of children’s parents being first or second degree relatives.

17. 2005 • Bronchiectasis remains common among some developing countries including Turkey • To evaluate the risk factors that cause BE • 204 patients, 99 females, 105 males • The mean age of patients was 7.16±3.72 • Consanguinity of parents in 76 patients (37.2 %)

18. 2006 • 1995-2004 • 50 patients with bronchiectasis • 20 females (40%), 30 males (60%) • Onset of symptoms: 6.9±3.5 years • 10 (20%) children with TB

19. 2007 • Saudi Arabia • A retrospective review • 1993-2005 • 151 patients with non-CF bronchiectasis • 75 (49.7%) males • 76 (50.3%) females • Start of symptoms: 5±3.2 years Banjar HH. Indian J Pediatr 2007; 74: 149-152.

20. 108 children • 6-14 years of age • A cough that lasted for >4 weeks • Bronchiectasis: 2.7% • 1680 asthmatics • The prevalence of bronchiectasis among the asthmatics is 3%. The impact of bronchiectasis in clinical presentation of asthma. Oğuzülgen IK, Kervan F, Ozis T, Turktas H. South Med J 2007; 100: 468-471.

21. Convalescence

22. Recurrent episodes of protracted bacterial bronchitis may lead to development bronchiectasis in some children such as at-risk populations (e.g., Indigenous children)

23. Indigenous Children • Alaska natives • Australian aboriginal • New Zealand’s Maori children • 14 – 20/ 1000 children • 40 times greater than the prevalence of CF among nonindigenous children

24. Indigenous Children • Alaska native children • 110/10,000 in the 1940s • 140/10,000 in the 1980s • Part of a developed nation, adequate immunization programs and access to medical care • Relative poverty: Small, crowded houses heated by wood-burning stoves in isolated villages, with limited access to running water • Passive smoking: 70% Singleton R, et al. Pediatr Pulmonol 2000; 29: 182-187.

25. Indigenous Children • Indigenous children in Central Australia have the highest rates of bronchiectasis in the world • Data on prevalence in Australian adults are lacking • 61patients and 166 admissions were identified. • 59 patients were indigenous (97%). • Recurrent respiratory infection is the major cause of illness. Associated factors include indigenous ethnicity, HTLV-1 pozitivity Steinfort DP. Respir Med 2008; 102: 574-578.

26. A Genetic Cause • A genetic disease can predispose to bronchiectasis in Turkey, where consanguinity of parents is common. • Transporter associated with antigen presentation (TAP) deficiency syndrome is characterized by recurrent bacterial lower respiratory tract infections. • Results indicate that TAP gene polymorphisms may have had a role in the development of bronchiectasis

27. Non-tuberculous mycobacteria • NTM is the cause or simply a complication of bronchiectasis • A prospective study of 100 patients with bronchiectasis in the UK. The prevalence of NTM infection was low at 2%

28. 2009 • 2001-2005, 2 centers • 105 children with bronchiectasis • 50 boys, 55 girls • 7 children not vaccinated against measles or pertussis • Patients had a higher prevalence of asthma compared with the general population

29. 2010 • 2003-2008 • 66 children with bronchiectasis • 44 (66.7%) boys • Mean age of the patients: 9.20±4.38 years • İzmir is located in the western coast of Turkey • Asthma was found to be the second common cause of bronchiectasis

30. There is a significant under-diagnosis because of low clinical suspicion • 10-fold increase in the rate of HRCT-diagnosed non-CF bronchiectasis • Non-CF bronchiectasis should not be orphan any more. Ann Thorac Med 2007; 2: 2 & Thorax 2009; 64: 246-251.

31. Thanks to HRCT

32. CF-Bronchiectasis • 47 CF • Median age: 10.1 years • HRCT and CT scoring system • Bronchiectasis: 36 ( 79.2%) Helbich T, et al. Radiologe 1993; 33: 142-146.

33. CF-Bronchiectasis • Children were diagnosed with CF after NBS. • Computed tomography and bronchoalveolar lavage were performed • The prevalence of bronchiectasis was 22% and increased with age (P = 0.001) • İnfants (8.5%) • 4-6 year olds (56%).

34. Mortality • 1940- Most of the patients died before the age of 40 years • 1960- Average age at death was not more than 55 years

35. Mortality Hong Kong Government statistics for 1990 showed a hospital admission rate of about 16.4/100 000 population, and a mortality rate of 1/100 000.

36. Mortality • 842 patient with bronchiectasis • There had been 239 deaths (28%) • The risk of death was 1.28 times greater for the male bronchiectatic patients than for the females • Bronchiectasis was the main cause of death in 13% of bronchiectatic patient • The main cause of death was cardiac disease (22%)