肺泡蛋白沉积症12例临床分析

目的 提高临床医师对肺泡蛋白沉积症的认识，分别从临床表现、病理特点和诊断治疗等方面对该病加以探讨。方法 回顾12例肺泡蛋白沉积症病例资料，并结合文献进行其临床特点分析。结果 临床症状包括：活动性呼吸困难12例（100.00％）、咳嗽11例（91．67％），咯痰9例（75．00％）、胸闷5例（41．67％）、咯血1例（8．33％）。体征包括：吸气性爆裂音或裂帛音5例（41．67％）、叩诊浊音4例（33．33％）、紫绀3例（25．00％）、杵状指2例（16．67％）。影像学表现包括：地图样表现ll例（91．67％）、肺水肿样表现8例（66．67％）、碎石路样表现6例（50％）、肺间质纤维化样表现5例（41.67％）、肺实变样表现3例（25％）。结论 肺泡蛋白沉积后的X线胸片表现缺乏特异性，CT／HRCT可出现相对典型的改变（地图样表现、碎石路样表现）。肺泡蛋白沉积症特征地表现为临床症状与体征分离，影像学与体征不符的现象。支气管肺泡灌洗是简便有效的诊疗方法。

Clinical features of pulmonary alveolar proteinosis: experience with 12 cases and literature review

Objective To analyze and evaluate clinical features, pathological characteristics, diagnosis and treatment of the identified pulmonary alveolar proteinosis （PAP） patients. Methods Retrospective study of 12 patients identified by biopsy through BALF was conducted. Focused analysis with imaging studies （chest x - ray or CT/HRCT） and clinical presentations were aimed to determine the diagnostic methods of PAP. Results Nonspecific clinical representation of PAP was composed of progressive dyspnea （ 100% ） and nonproductive cough （91.67% ） . Physical examination showed signs of inner lungsawas seldom detected, such as inspiratory crackles or Velcro＇ s rales （41.67% ） and cyanosis （25 % ） . Although, absence of specific chest X - ray findings, some typical radiological changes contributed to diagnose PAP cor- rectly, such as patchy opacification（91.67% ） and crazy paving pattern（50% ）, especially in HRCT. Conclusion Separability of clinical symptoms and signs, radiological results and clinical features exist as characteristic representation of PAP. Diagnostic bronchoalveolar lavage is a practical and useful technology to determine PAP.