낭성섬유증(cystic fibrosis)은 상염색체 열성유전질환으로 침샘,땀샘,췌장,대장,기관기관지 등의 외분비선의 이상에 의해 나타나는 질환이다.백인에게는 비교적 흔한 유전질환이나 한국 등의 아시아 국가에서는 드문 질환이다.1988년에 한국에서 한 예가 발표되었으나 진단에초점이 맞춰져 있었고 방사선학적 소견에 대한 발표는 없어 당시 낭성섬유증으로 진단되었던 소아의 추적검사를 중심으로 방사선학적 소견을 보여주고자 한다.
Cystic fibrosis is a hereditary disease involving recessive transmission. The fundamental abnormality consists of the production of abnormal secretion from a variety of exocrine glands such as salivary and sweat glands and those of the pancreas, colon, and tracheobronchial trees. Cystic fibrosis is the most common lethal genetically transmitted disease among whites, but is uncommon in Asians, including Koreans. Although a case involving a Korean was reported in 1988, the focus was diagnosis rather than the radiological findings. In the case of cystic fibrosis we now describe, the focus is inverted: we enphasise the reported in 1988, focusing on radiologic findings
