The clinical characteristics of pulmonary alveolar proteinosis: experience at Seoul National University Hospital, and review of the literature.
The clinical characteristics of pulmonary alveolar proteinosis: experience at Seoul National University Hospital, and review of the literature.
复制标题
DOI：
10.3346/jkms.1999.14.2.159
复制
发表时间：
1999-04
影响因子：
4.5
通讯作者：
Kim YW
中科院分区：
文献类型：
--
作者：
Kim G;Lee SJ;Lee HP;Yoo CG;Han SK;Shim YS;Kim YW
肺泡蛋白沉积症在韩国是一种极其罕见的疾病，仅报道了少数病例。同时，自1987年以来，在首尔大学医院的十多年里，也发生了5例病例，我们总结了这些病例的临床特点和病程。尽管只有少数病例报告提到了患者的病程，但文献中报告的 7 例病例被纳入其中，以添加有关临床特征和病程的数据。主要受影响的是中年男性患者。未发现与特定环境或职业暴露的关联。劳累时呼吸困难是主要症状。双侧爆裂音一致，胸部平片上双侧肺门旁模糊浸润和高分辨率 CT 上毛玻璃样混浊是特征性的。诊断时未发现任何患者存在叠加感染。几乎每个病例都存在弥散能力下降和缺氧的情况。全肺灌洗被证明是一种有效的治疗措施。治疗反应良好。疾病的长期病程，例如复发率，尚不清楚。
Pulmonary alveolar proteinosis is such an extremely rare disease in Korea, that only a few cases have been reported. Meanwhile five cases were experienced at Seoul National University Hospital over ten years since 1987. We summarized the clinical characteristics and courses of them. Seven cases reported in the literature were included to add data about clinical characteristics and courses although only a few case reports mentioned patient's course. Middle aged male patients were mainly affected. No association with particular environmental or occupational exposure was identified. Dyspnea on exertion was the main symptom. Bilateral crackles were consistent, and bilateral parahilar hazy infiltrations on plain chest radiograph and ground glass opacity on high-resolution CT were characteristic. Superimposed infection was not identified in any patient at the time of diagnosis. Decreased diffusing capacity and hypoxia were present in almost every case. Whole lung lavage proved to be an effective therapeutic measure. The response to treatment was good. Long-term course of the disease, e.g. recurrence rate, is not yet known.
登录
查看更多内容
影响因子：
39.2
作者：
LARSON, RK;GORDINIER, R
通讯作者：
GORDINIER, R
影响因子：
9.6
作者：
CLAYPOOL, WD;ROGERS, RM;MATUSCHAK, GM
通讯作者：
MATUSCHAK, GM
影响因子：
158.5
作者：
ROSEN, SH;CASTLEMAN, B;HUNT, RTN
通讯作者：
HUNT, RTN
影响因子：
5
作者：
KARIMAN, K;KYLSTRA, JA;SPOCK, A
通讯作者：
SPOCK, A
影响因子：
39.2
作者：
RAMIREZR, J;KIEFFER, RF;BALL, WC
通讯作者：
BALL, WC