CAJ | 학술논문

目的探讨21-三体综合征相关肺间质疾病的肺组织病理、影像和临床特点.方法报道1例经影像及病理证实的21-三体综合征相关肺间质疾病,并复习文献总结其肺组织病理、影像及临床特点.结果该例临床表现为重症肺炎,感染反复;病情稳定期无明显气促、发绀,胸部CT仍表现为磨玻璃样影、区域性肺气肿、肺实质带等肺间质病变征象.胸腔镜可见双肺各叶充气不均,表面呈弥漫性小气泡样改变.病理可见肺泡、肺泡管、肺泡囊扩张,肺泡间隔增宽.复习文献,国内未见报道.国外分别报道7例和24例21-三体综合征病理资料,86％～ 88％病例可见肺泡生长异常.影像学特征性表现为胸膜下肺囊肿.临床上21-三体综合征高发呼吸道感染并容易加重引起呼吸衰竭;术后可引起持续低氧血症,需长时间呼吸支持.结论 21-三体综合征存在染色体相关的肺泡生长异常,病理表现肺泡简单化,影像表现肺间质疾病,临床应警惕感染、手术导致其呼吸衰竭的风险.
목적 탐토21-삼체종합정상관폐간질질병적폐조직병리、영상화림상특점.방법 보도1례경영상급병리증실적21-삼체종합정상관폐간질질병,병복습문헌총결기폐조직병리、영상급림상특점.결과 해례림상표현위중증폐염,감염반복;병정은정기무명현기촉、발감,흉부CT잉표현위마파리양영、구역성폐기종、폐실질대등폐간질병변정상.흉강경가견쌍폐각협충기불균,표면정미만성소기포양개변.병리가견폐포、폐포관、폐포낭확장,폐포간격증관.복습문헌,국내미견보도.국외분별보도7례화24례21-삼체종합정병리자료,86％～ 88％병례가견폐포생장이상.영상학특정성표현위흉막하폐낭종.림상상21-삼체종합정고발호흡도감염병용역가중인기호흡쇠갈;술후가인기지속저양혈증,수장시간호흡지지.결론 21-삼체종합정존재염색체상관적폐포생장이상,병리표현폐포간단화,영상표현폐간질질병,림상응경척감염、수술도치기호흡쇠갈적풍험.
Objective To study the pathology,imaging and clinical features of a child with trisomy 21 syndrome associated interstitial lung disease.Method Data of a case with trisomy 21 syndrome associated interstitial lung disease confirmed by lung imaging and pathology were collected,analyzed and the related reports in literature were reviewed.Result The patient was a one year and 7 months old boy who suffered from severe pneumonia and recurrent infection during his hospital stay.When his disease was stable,he did not have shortness of breath and cyanosis,but a chest computed tomography (CT) showed ground-glass opacity,regional emphysema,band-like change in lung parenchyma,which indicated interstitial lung diseases.Unequal air inflation in bilateral lungs and diffuse overdistension of peripheral air spaces in lung surface were seen through thoracoscope.Pathological examination indicated that alveolar,alveolar ducts and alveolar sac were enlarged,alveolar septa was expanded.There were two reports in lung pathology of trisomy 21 syndrome,alveolar growth abnormalities was seen in 86％-88％ cases.The multiple subpleural cysts in chest CT was characteristic.Clinically,trisomy 21 syndrome had high morbidity of respiratory tract infection and progress to respiratory failure frequently.Prolonged postoperative desaturation was constant which required long duration of respiratory support.Conclusion Trisomy 21 syndrome associated alveolar growth abnormalities were confirmed,which manifest as alveolar simplification in pathology and interstitial lung diseases in imaging.The risk of respiratory failure in these cases caused by infection and surgery should be considered.