Paediatric Pulmonology and Allergology

2004 April, Vol. VII, No. 1 (pp.2389-2396)

The changing face of non-cystic fibrosis bronchiectasis in childhood

David A. Spencer

Non-Cys­tic fib­ro­sis bron­chiec­ta­sis is ge­ne­ral­ly thought to be both un­com­mon and pro­gres­si­ve in chil­dren li­ving in wes­tern so­cie­ties. Ma­ny ca­ses we­re pre­vio­us­ly mis­diag­no­sed as ha­ving ast­hma be­cau­se of chro­nic cough. This ar­tic­le re­ve­al fin­dings ob­tai­ned from 93 chil­dren with high re­so­lu­tion com­pu­ter to­mog­rap­hy de­fi­ned non-CF bron­chiec­ta­sis pre­sen­ting up to 9.6 proc. of all new re­fer­rals to a ter­tia­ry pa­e­diat­ric res­pi­ra­to­ry cen­tre sin­ce 1996.  Ma­le to fe­ma­le ra­tio was 2:1. Me­dian age at symp­tom on­set was 1,1 years (0 – 16) and of diag­no­sis was 7,2 years (1,6 – 18,8). The re­fer­ral diag­no­sis of ast­hma was re­fu­ted in 39 of 45 ca­ses. As­so­cia­tions we­re pre­vio­us pneu­mo­nic il­lness (30 perc.), im­mu­no­com­pro­mi­se (21 perc.), ob­li­te­ra­ti­ve bron­chio­li­tis (9 perc.), con­ge­ni­tal lung ab­nor­ma­li­ty (5 perc.), chro­nic as­pi­ra­tion (3 perc.), eosi­nop­hi­lic oesop­ha­gi­tis (2 perc.), fa­mi­lial syn­dro­me (2 perc.), pri­ma­ry ci­lia­ry dys­ki­ne­sia (1 perc.) and right mid­dle lo­be syn­dro­me (1 perc.). Chest ra­dio­graph and high re­so­lu­tion com­pu­ter to­mog­rap­hy scan ag­re­ed for diag­no­sis and lo­be af­fec­ted in on­ly 5 perc. ca­ses. A ma­jor diag­nos­tic va­lue of high re­so­lu­tion com­pu­ter to­mog­rap­hy in chil­dren su­spec­ted as ha­ving non-CF bron­chiec­ta­sis was ap­pro­ved. Non-CF bron­chiec­ta­sis diag­no­sis de­lay in chil­dren is a pro­blem. Dif­fe­ren­ces in ori­gin and cour­se of the di­se­a­se of non-CF bron­chiec­ta­sis in chil­dren and adults should be ta­ken in­to ac­count, and  high re­so­lu­tion com­pu­ter to­mog­rap­hy scan should be con­si­de­red even in small chil­dren if ne­ces­sa­ry.

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