Doç. Dr. Demet CAN
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Dr. Behçet Uz Children HospitalİZMİR
Epidemiology of Bronchiectasis
Doç. Dr. Demet CAN
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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.
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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
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Definition
• Reduced airway mucus clearance,
• Stasis of infected airway secretions
• Regional or diffuse airway wall dilation and destruction with loss of airway structural integrity
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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
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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.
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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.
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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.
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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
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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
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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)
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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
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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
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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
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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.
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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.
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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 %)
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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
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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.
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• 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.
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Convalescence
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• Recurrent episodes of protracted bacterial bronchitis may lead to development bronchiectasis in some children such as at-risk populations (e.g., Indigenous children)
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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
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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.
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Indigenous Children• Indigenous children in Central Australia have the
highest rates of bronchiectasis in the world• Data on prevalence in Australian adults are lacking • 61 patients 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.
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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
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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%
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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
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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
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• 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.
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Thanks to HRCT
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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.
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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%).
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Mortality
• 1940- Most of the patients died before the age of 40 years
• 1960- Average age at death was not more than 55 years
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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.
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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%)
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