Paediatric Pulmonology and Allergology

2004 April, Vol. VII, No. 1 (pp.2419-2430)

What is New in Cystic Fibrosis

Andrew Bush

A bet­ter un­ders­tan­ding of the bio­lo­gy of CFTR is le­a­ding to spe­ci­fic the­ra­pies, bet­ter pre­ven­ti­ve stra­te­gies of non-res­pi­ra­to­ry com­pli­ca­tions, and mo­re ra­tio­nal ma­na­ge­ment of new in­fec­tions. The­re we­re a plet­ho­ra of stu­dies con­fir­ming that mac­ro­li­des ha­ve a lar­ge num­ber of im­mu­no­mo­du­la­to­ry pro­per­ties, for exam­ple sup­pres­sing the sec­re­tion of pro-in­flam­ma­to­ry cy­to­ki­nes by bron­chial epit­he­lial cells. Three ran­do­mi­sed, pla­ce­bo con­trol­led stu­dies in three dif­fe­rent con­ti­nents, using dif­fe­rent met­hods and dif­fe­rent po­pu­la­tions, ha­ve con­fir­med be­ne­fit for mac­ro­li­des in CF.  The Bromp­ton trial fai­led to de­monst­ra­te any re­duc­tion in spu­tum IL-8 or neut­rop­hil elas­ta­se. A small pi­lot stu­dy in CF de­monst­ra­ted no chan­ge in in­du­ced spu­tum neut­rop­hil count, IL-8, neut­rop­hil elas­ta­se, TNF-a, or my­e­lo­pe­ro­xi­da­se. Sub­se­qu­ent to the pub­li­ca­tion of the­se trials, a ret­ros­pec­ti­ve ob­ser­va­tio­nal re­view com­pa­red the pro­gress of 20 CF pa­tients who we­re de­te­rio­ra­ting on stan­dard the­ra­py, with 20 stab­le pa­tients. The ad­di­tion of mac­ro­li­des to the tre­at­ment of the de­te­rio­ra­ting group sta­bi­li­sed their con­di­tion. The pro­blems of CF re­la­ted dia­be­tes, CF bo­ne di­se­a­se and cros­sin­fec­tion are al­so dis­cus­sed in this ar­tic­le.

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