【摘要】 目的　探讨肺泡蛋白沉着症 (PAP)的临床特点和早期诊断。方法　对 4例经纤维支气管肺泡灌洗液 (BALF)病检证实的PAP患者病史进行回顾性分析 ,仔细观察BAL治疗前后各病例的症状 ,体征、影像等的特征性变化情况。结果　反复咳嗽、咳痰 ,进行性呼吸困难 ,慢性呼吸功能衰渴以及典型的影像表现与临床症状不相称是PAP的特征。BALF病检PAS染色阳性是确认PAP的重要依据。BALF可长期改善PAP患者的大部分症状。并使影像呈良性改变。结论　详询病史结合影像 ,BPLF的PAS染色对早期诊断本病至关重要。限制性肺通气功能障碍 ,CO2 弥漫散功能降低 ,血清LDH增高对诊断PAP有一定的参考价值。更多还原

【Abstract】 Objective To investigate clinical manifestation and early diagnosis of PAP.Methods 4 cases of PAP were identified by biopsy through PALE.The changes of clinical symptoms,signs, and post treatment.Results PAP was characterized of repeating cough,expectoration, dyspnea,slow respiratory failure,and clinical images which not matched with clinical symptoms.Positive of PAP dyeing provided a major basis of definite diagnosis .Most symptoms of PAP were released by PALE for long term, and PALE made clinical images present benign changes.Conclusion Enquiring carefuolly combining with clinical images,and positive of PAP dyeing were very important to early diagnosis of PAP.Restrictive disordr of ventilation function,decrasing diffusion function fo CO 2,and increasing LDH in serum had certain degree in diagnosis of PAP.更多还原