Doç. Dr. Demet CAN

Dr. Behçet Uz Children HospitalİZMİR

Epidemiology of Bronchiectasis

Doç. Dr. Demet CAN

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.

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

Definition

• Reduced airway mucus clearance,

• Stasis of infected airway secretions

• Regional or diffuse airway wall dilation and destruction with loss of airway structural integrity

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

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.

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.

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.

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

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

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)

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

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

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

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.

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.

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 %)

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

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.

• 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.

Convalescence

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

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

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.

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.

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

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%

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

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

• 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.

Thanks to HRCT

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.

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%).

Mortality

• 1940- Most of the patients died before the age of 40 years

• 1960- Average age at death was not more than 55 years

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.

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%)