Lymphangioleiomyomatosis and multifocal micronodular pneumocyte hyperplasia in Japanese patients with tuberous sclerosis complex
Lymphangioleiomyomatosis and multifocal micronodular pneumocyte hyperplasia in Japanese patients with tuberous sclerosis complex
复制标题
DOI：
10.1016/j.resinv.2015.06.003
复制
发表时间：
2016-01-01
影响因子：
3.1
通讯作者：
Katayama, Ichiro
中科院分区：
文献类型：
--
作者：
Tanaka, Mari;Hirata, Haruhiko;Katayama, Ichiro
背景资料：结节性硬化症（TSC）的肺部受累包括肺血管平滑肌瘤病（LAM）和多灶性小结节性肺细胞增生（MMPH）。本回顾性研究调查了日本TSC患者的肺部受累情况和TSC-LAM.Methods的肺功能测试：研究对象包括59例TSC患者（年龄范围：13-66岁，15名男性和44名女性）。根据计算机断层扫描图像上囊肿数量的增加，将女性患者分为3组（第1组：症状性LAM，第2组：无症状性LAM，第3组：无LAM）和3个囊性分级（无LAM的患者，1级患者和2级+3级患者）。结果：LAM患者中男性1例（6.7%），女性19例（43.2%）; MMPH患者中男性7例（43.2%），女性23例（52.3%）。多发性肺结节患者的肾血管平滑肌脂肪瘤发生率和癫痫发作史均高于无结节患者。虽然所有6名青少年患者均未显示肺部症状，但在3名患者中发现了MMPH，在1名13岁女孩中发现了LAM。一氧化碳弥散量（DLCO）之间的3组和DLCO和一氧化碳弥散量除以肺泡容积（DLCO/VA）之间的差异显着3囊性grades.Conclusions：有没有国家之间的TSC患者肺部受累的患病率差异显着。LAM和MMPH甚至发生在TSC患者的青春期。DLco和囊肿数量是TSC-LAM发作和进展的有用预测因子。(C)2015日本呼吸学会Elsevier B. V.出版，保留所有权利。
Background: Pulmonary involvement in tuberous sclerosis complex (TSC) includes lym-phangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH). This retrospective study investigated pulmonary involvement in Japanese TSC patients and pulmonary function testing in TSC-LAM.Methods: The study subjects included 59 TSC patients (age range: 13-66, 15 males and 44 females). Female patients were divided into 3 groups (Group 1: symptomatic LAM, Group 2: asymptomatic LAM, Group 3: without LAM) and 3 cystic grades according to increasing cyst numbers on computed tomography images (patients without LAM, Grade 1 patients, and Grade 2+Grade 3 patients). The results of pulmonary function tests were compared among the groups and the grades.Results: One male (6.7%) patient and 19 female (43.2%) patients were diagnosed with LAM and 7 male (43.2%) and 23 female (52.3%) patients were diagnosed with MMPH. Patients with multiple pulmonary nodules had higher rates of renal angiomyolipoma and history of seizures than patients without nodules. Although all 6 adolescent patients displayed no pulmonary symptoms, MMPH was found in 3 patients and LAM was found in a 13-year-old girl. Carbon monoxide diffusing capacity (DLco) differed significantly among the 3 groups and DLco and carbon monoxide diffusing capacity divided by the alveolar volume (DLco/VA) differed significantly among the 3 cystic grades.Conclusions: There was no difference in the prevalence of pulmonary involvement in TSC patients among countries. LAM and MMPH occur even during adolescence in TSC patients. DLco and the number of cysts are useful predictors of onset and progression of TSC-LAM. (C) 2015 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.