반복 지속성 호흡기 질환 소아에서 비강 섬모의 미세구조 검사의 의의 = Ultrastructure of nasal cilia in children with recurrent or persistent respiratory diseases

다국어 초록 (Multilingual Abstract)

Purpose : Ciliary abnormalities of the respiratory system usually accompany recurrent or persistent respiratory diseases such as paranasal sinusitis, bronchiectasis, rhinitis, and/or otitis media, since they cause certain derangements in ciliary cleaning activities. This disease is usually inherited by autosomal recessive trait, but may also be found to be acquired or transient in rare cases after heavy exposure to pollutants, cigarette smoking or severe infection. We performed this study in children with frequently recurrent or persistent respiratory diseases to clarify if the ciliary abnormalities are preceding factors.
Methods : We enrolled 17 children with suspected respiratory ciliary abnormalities. The indications for evaluation of ciliary ultrastructure were recurrent or persistent respiratory infections. Children with immunologic abnormalities were excluded. From August 2000 to July 2003, we performed a biopsy on nasal mucosa and examined the structure of ciliary status by using an electron microscope.
Results : Of the subjects, there were seven males and 10 females, aged 2 to 10 years. Out of the 17 subjects, 12 cases of chronic paranasal sinusitis, nine chronic coughs, nine frequent upper respiratory infections, seven cases of recurrent otitis media, four cases of recurrent pneumonia, and four cases of bronchial asthma were found. Out of the 17 cases on which histologic examinations were conducted, four cases showed pathologic findings, including one case of inner dynein arm defect, one of microtubular transposition, one of supernumerous tubules, and one singlet, respectively.
Conclusion : It is essential for differential diagnosis and effective treatment to identify the abnormalities of ultrastructure of nasal cilia in children with symptoms of frequently recurrent or persistent respiratory diseases, if immunodeficiency or respiratory allergy could be excluded.

국문 초록 (Abstract)

목 적 : 호흡기계 섬모 이상은 점막 섬모의 청소 기능에 장애를 초래하므로 반복적 혹은 지속적인 호흡기 계통의 질환 즉, 부비동염, 기관지염, 기관지확장증, 비염 또는 중이염 등을 동반한다. 이 질환은 주로 선천적으로 발생하지만, 드물게는 후천성 혹은 일과성으로 발생되기도 한다. 본 연구에서는 자주 재발하거나 지속되는 호흡기 질환의 증상을 가진 환아들을 대상으로 호흡기계 섬모 이상이 선행하는지를 알아보고자 하였다.방 법 : 2000년 8월부터 2003년 7월까지 한양대학교병원 소아과에 입원하였거나 외래에 내원한 3개월 이상 지속되는 만성 또는 재발하는 호흡기 질환 환아들 중 17례에서 비강 내 점막 조직생검을 시행한 후 전자현미경을 이용하여 섬모의 상태를 검사하였다. 결 과 : 대상 환아 중 남아가 7례였고 여아가 10례였으며, 연령분포는 2세에서 10세 사이였다. 대상 환아 17례 중 만성 부비동염이 12례, 만성 기침이 9례, 잦은 상기도 감염이 9례에서 관찰되었으며 그 다음으로는 재발성 중이염(7례), 재발성 폐렴(4례), 기관지 천식(4례) 순으로 관찰되었다. 조직검사를 시행한 17례 중 4례가 병적인 소견을 나타냈는데 1례에서는 inner dynein arm 결손이 있었고 1례는 microtubular transposition, 1례는 supernumerous tubules, 1례는 singlet을 가지고 있었다.

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