Objective To improve the knowledge of kartagener syndrome and the understanding of primary ciliary dyskinesia ( PCD) . Methods Three cases of Kartagener syndrome were reported and analyzed on clinical manifestations, symptoms, imaging appearances of chest, and family history. Meanwhile the related literatures were reviewed. Results The three patients deserved special recognition because of recurrent upper and lower respiratory tract infection. Of them, the third case showed mainly the symptoms of nasosinusitis at an early age, and the distinct pulmonary symptoms appeared in his adulthood. Further examination showed that the triad of chronic sinusitis, bronchiectasis, and situs inversus is present in all cases. The parents of the two cases were consanguineous marriaged. Examination of the bronchial mucosal biopsy specimen of the first case under transmission electron microscopy showed cilia with the absence of inner dynein arms. The three cases got improvement after treatment of anti-infection and expectorant. Conclusion For patients with recurring infection in the upper and lower respiratory tract from infancy, the possibility of PCD should be considered no matter whether a situs inversus exists.
目的 全面介绍先天性囊性腺瘤样畸形(CCAM)其可能的病因,临床、病理特点及诊断手段,循证探讨针对CCAM的治疗方法及预后。 方法 对我院2011年11月收治的1例罕见的CCAM患者的临床资料进行分析,并对相关文献进行复习。 结果 患者数次误诊后最终诊断为CCAM,予手术治疗后痊愈,随访1年无复发。 结论 CCAM是一种少见的、非遗传性的、错构瘤样的肺发育异常,为一种良性的肺部畸形,其特点是局部肺终末呼吸性细支气管过度生长。CCAM多通过产前影像学检查、活组织检查或术后病检诊断。手术为治愈该病的最根本、最重要措施。
目的 提高对外源性过敏性肺泡炎的认识。 方法 回顾分析2011年10月报道1例外源性过敏性肺泡炎(过敏性肺炎)患者的诊断及治疗经过,总结其临床特征、诊疗要点及预后评价。 结果 患者数次误诊后最终诊断为外源性过敏性肺泡炎,予脱离变应原及激素治疗后痊愈,随访半年无复发。结论 该病临床表现无特异性,需结合患者病史、临床症状、血清学检查、影像学表现,甚至肺泡灌洗液及肺活检综合判断;脱离变应原为该病治疗的最根本、最重要措施;对于病情严重患者,短期全身性使用糖皮质激素可缩短病程或改善症状。