以抗生素治療囊性纖維化病人肺部惡化之洋蔥伯克氏 ... | 合法醫療器材資訊網

2020年4月2日—Burkholderiacepaciacomplexinfectionspresentasignificantchallengeforpeoplewithcysticfibrosisandtheirclinicians.Theincidenceislikely ...

Cystic fibrosis (CF) is a complex multisystem disease, caused by defects in a single gene (Ratjen 2003[1]). It is the most common life‐shortening genetic disorder in those of European descent, affecting 1 in 2500 live births in the UK and Western Europe, although it is also found in all other major ethnic groups (Bobadilla 2002[2]). The effects in the lungs are responsible for most of the morbidity and mortality, and the hallmark of the condition is progressive and irreversible bronchiectasis and respiratory failure.

The primary defect in CF is in an epithelial chloride ion channel (Riordan 1989[3]). This is expressed throughout the body, and is involved in secretory function in a number of organs, including the lung, liver, gut, pancreas and sweat glands (Hoogeveen 1991[4]). Defective ion transport in CF is responsible for the effects of the condition in these organs. In the lung, loss of chloride excretion and uncontrolled sodium absor...