肺泡微石症临床分析1例并文献复习

Clinical analysis of 1 case of pulmonary alveolar calculus and literature review

目的:探讨肺泡微结石症(PAM)的临床特征,提高该病的诊断率。方法:收治肺泡微石症患者1例,对其进行临床分析,并结合国内发表的肺泡微石症病例进行文献复习。结果:共155例患者;患者来自17个家庭,父母为近亲婚配12例。体检发现64例,有临床症状92例。行胸部CT检查112例,其中典型的白描征20例,火焰征24例,微小结节41例。患者结合轻微的临床症状和典型的影像学表现而确诊82例,患者有明确的病理依据72例。结合基因检测确诊2例。结论:胸部典型的影像学改变是诊断PAM的主要依据,肺活检可明确诊断;该病目前无特殊治疗方法,基因检测给肺泡微石症的治疗提供了新的可能的药物作用靶点。

Objective：To investigate the clinical features of alveolar calculi（PAM）and to improve the diagnosis of this disease.Methods：1 patient with pulmonary alveolar calculus were selected,and we made a clinical analysis and literature review of the cases of pulmonary alveolar stones was carried out.Results：A total of 155 patients,patients from 17 families in 41 cases,12 cases of consanguineous parents.Physical examination revealed 64 cases and 92 cases with clinical symptoms.The chest CT examination in 112 cases,the typical line sign in 20 cases,the flame sign in 24 cases,41 cases of small nodules.Patients with mild clinical symptoms and typical imaging findings and confirmed in 82 cases,while patients with a clear pathological basis in 72 cases,2 cases were confirmed by gene detection.Conclusion：The typical imaging changes of chest are the main basis for the diagnosis of PAM,and the lung biopsy can be used for definite diagnosis.There is no specific treatment for this disease,and gene testing provides a new potential drug target for the treatment of pulmonary alveolar stones.