肺泡蛋白沉积症的临床研究

目的探讨肺泡蛋白沉积症(PAP)的临床特点、确诊方法和治疗效果。方法对7例经病理证实的PAP患者病史进行回顾性分析,观察支气管肺泡灌洗(BAL)和全肺灌洗前后症状、体征、影像学等的改变。结果PAP的临床表现有反复咳嗽、咳痰,进行性呼吸困难,慢性呼吸功能衰竭以及与临床症状不相称的影像学表现。BAL和全肺灌洗后PaO2显著提高(P<0.05),肺功能虽然有所改善,但无统计学意义(P>0.05)。大部分临床症状和X线表现有所改善。结论X线影像和CT扫描有助于早期诊断PAP,肺组织活检仍是确诊PAP的最可靠手段,支气管肺泡灌洗液的过碘酸雪夫染色阳性可作为确认PAP的重要依据。BAL和全肺灌洗能够有效地改善临床症状和血气交换功能。

Diagnostic and therapeutic analysis of pulmonary alveolar proteinosis

Objective To investigate clinical manifestation,definite diagnosis and therapeutic efficacy of pulmonary alveolar proteinosis(PAP).Methods 7 cases of PAP identified by biopsy were studied retrospectively.The changes of clinical symptoms,signs,and images were surveyed after bronchoalveolar lavage(BAL) and total pulmonary lavage treatment.Results PAP was characterized by recurrent cough,expectoration,dyspnea,chronic respiratory failure and dismatch of clinical symptoms with radiographic findings.After BAL and total pulmonary lavage treatment,the patients' arterial partial pressure of oxygen(PaO_2) were elevated significantly((P<)0.05),lung function were improved without statistically significance(P>0.05).Symptoms and radiographic alterations of PAP were relieved by BAL and total pulmonary lavage.Conclusions Chest X ray film and chest CT scan are helpful to early detection of PAP.Although lung biopsy is still the most reliable approach,positive stain with periodic aicd-Schiff of bronchoalveolar lavage fluid provides direct evidence for PAP diagnosis.