PULMONARY ALVEOLAR PROTEINOSIS - PROSPECTIVE CLINICAL-EXPERIENCE IN 23 PATIENTS FOR 15 YEARS
PULMONARY ALVEOLAR PROTEINOSIS - PROSPECTIVE CLINICAL-EXPERIENCE IN 23 PATIENTS FOR 15 YEARS
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作者：
KARIMAN, K;KYLSTRA, JA;SPOCK, A
对患有肺泡蛋白沉积症 (PAP) 的患者 (23)（包括 3 名儿童）进行了为期 15 年的研究。根据总体过程和结果，他们被分为两组。 I 组（24%）自发缓解，未接受任何治疗。第二组（76%）由于进行性呼吸困难和肺功能测试恶化，接受了肺灌洗。在接受肺灌洗并随访的患者中，76% 的患者有良好反应（II-a 组），其中相当多的人出现完全缓解，而 21%（II-b 组）对肺灌洗没有反应。与 II 组相比，I 组的动脉 PO2 [O2 张力] 较高，肺泡-动脉 O2 分压差 [P (A-a)O2] 较低（72 .+-. 5 和 38 .+-. 3 vs. 57 .+-. 4 和 51 .+-. 3，P < 0.05）。同样，II-a 组的动脉 PO2 和 P (A-a)O2 值优于 II-b 组（57.+-.4 和 51.+-.3 对比 40.+-.6 和 65.+-.11）。在所研究的 16 名患者的灌洗材料中，巨噬细胞出现异常增大，其数量（分类计数的百分比）显着低于正常值 (90-95%) (60.+-.4%)。没有证据表明活动性感染与异常或机会性微生物有关。没有观察到与 PAP 相关的死亡。这项对大量 PAP 患者的前瞻性长期随访记录了该疾病的总体病程、预后和治疗。
Patients (23) , including 3 children, with pulmonary alveolar proteinosis (PAP) were studied over a 15 yr period. Based on the overall course and outcome they were divided into 2 groups. Group I (24%) had spontaneous remission and received no treatment. Group II (76%), becuase of progressive dyspnea and deterioration of pulmonary function tests, underwent lung lavage. Of those who underwent lung lavage and were followed, 76% had a favorable response (group II-a) and a significant number of them developed a complete remission, while 21% (group II-b) had no response to lung lavage. Group I had higher arterial PO2 [O2 tension] and lower alveolar-arterial O2 partial pressure difference [P (A-a)O2] than group II (72 .+-. 5 and 38 .+-. 3 vs. 57 .+-. 4 and 51 .+-. 3, P < 0.05). Similarly, the values of arterial PO2 and P (A-a)O2 were better in group II-a than group II-b (57 .+-. 4 and 51 .+-. 3 vs. 40 .+-. 6 and 65 .+-. 11). In lavage material of 16 patients studied, macrophages appeared unusually enlarged and their number (% of differential count) was significantly lower (60 .+-. 4%) than normal (90-95%). There was no evidence of active infection related to unusual or opportunistic organisms. No death related to PAP was observed. This prospective long-term follow-up of large series of patients with PAP documents the overall course, prognosis and management of this illness.