Cystic Fibrosis: Indian Experience

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Cystic Fibrosis: Indian Experience

Indian Pediatrics 2002; 39:813-818

A. S. Ahuja., Consultant Pediatrician, Royal Albert

Infirmary Wigan, Lancs., UK

S. K. Kabra ,Additional Professor,Department of

Pediatrics, All India Institute of Medical Sciences,

New Delhi.

Cystic fibrosis (CF) is the most common life limiting

recessive genetic disorder in Caucasians with an

incidence of approximately 1 in 2500 children born in

the United Kingdom(1). It is less common in African

Americans (1 in 15000) and in Asian Americans

(1:31000). It also affects other ethnic groups such as

black population with an incidence of 1 in 17,000 and

the native American population with an approximate

incidence of 1 in 80,000 (2,3).

Cystic fibrosis was thought to be extremely rare in

India. However published reports, reviews and comments

indicate that CF is probably far more common in people

of Indian origin than previously thought but is under

diagnosed or missed in the majority of cases

(4–25)(Table I). The precise incidence of CF among

Indians is unknown. The incidence in migrant Indian

populations in the USA has been estimated to be 1 in

40000(8), and in the UK between 1 in 10000 to 12000

(9,10). As a result of the widespread belief that CF

does not occur in Indians, the disease is rarely

suspected and even if it is suspected the diagnosis is

rarely confirmed due to the poor availability of

facilities for diagnosis. The median age of diagnosis

among Indian Americans is 12 months compared with 6

months among Caucasian American children and reflects

a low index of suspicion for the disease even among

Indians in western countries(8). Recent reports

suggest that genetic and clinical profile of Indian

children with CF may be different(7-9). These reports

suggest that the diagnosis of CF is delayed in Indian

children which may result in severe malnutrition by

the time the condition is eventually diagnosed — one

of the bad prognostic indicators for survival(12).

Becki

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