Intractable diffuse pulmonary diseases: Manual for diagnosis and treatment

This manual has been compiled by a joint production committee with the Diffuse Lung Disease Assembly of the Japanese Respiratory Society (JRS) to provide a practical manual for the epidemiology, diagnosis, and treatment of intractable diffuse pulmonary diseases. The contents are based upon the results of research into these diseases by the Diffuse Pulmonary Diseases Study Group (principal researcher: Sakae Homma) supported by the FY2014–FY2016 Health and Labor Sciences Research Grant on Intractable Diseases.This manual focuses on: 1) pulmonary alveolar microlithiasis, 2) bronchiolitis obliterans, and 3) Hermansky-Pudlak Syndrome with interstitial pneumonia. As these are rare/intractable diffuse lung diseases (2 and 3 were first recognized as specified intractable diseases in 2015), there have not been sufficient epidemiological studies made, and there has been little progress in formulating diagnostic criteria and severity scales; however, the results of Japan's first surveys and research into such details are presented herein. In addition, the manual provides treatment guidance and actual cases for each disease, aiming to assist in the establishment of future modalities.The manual was produced with the goal of enabling clinicians specialized in respiratory apparatus to handle these diseases in clinical settings and of further advancing future research and treatment.     

超声造影对甲状腺影像报告和数据系统4类结节的诊断价值

目的 探讨超声造影(contrast-enhanced ultrasound, CEUS)对甲状腺影像报告和数据系统(thyroid imaging reporting and data system,TI-RADS)4类结节的恶性风险及临床诊断价值。方法 分析226例甲状腺患者的结节特征,依据Kwak制定标准进行分类,其中TI-RADS 4A、4B和4C类结节纳入研究中,所有结节均行CEUS检查。CEUS结果与病理结果对照。结果 226个TI-RADS 4类结节中,CEUS诊断恶性为106个,良性为120个,术后病理诊断恶性110为个,良性为116个,CEUS诊断4类结节的准确性、敏感度、特异度、阳性预测值及阴性预测值,分别为91.15%、89.09%、93.10%、89.91%和89.74%。CEUS诊断4类结节结果和病理结果比较,具有较高的一致性,Kappa值0.823(P＜0.001)。CEUS诊断4A、4B和4C类结节的实际恶性率,分别为14.06%(9/64)、47.53%(48/101)和86.89%(53/61)。 结论 CEUS检查对TI-RADS 4类甲状腺结节具有较高的诊断价值。有助于指导TI-RADS 4类甲状腺可疑结节临床后续决策。     

甲状腺髓样癌超声特征分析

[摘要] 目的：探讨甲状腺髓样癌（MTC）超声特征。方法：回顾性分析40个MTC超声表现,以60个甲状腺乳头状癌（PTC）及97个甲状腺良性结节为对照,分别比较MTC与PTC及甲状腺良性结节在病灶数量、直径、边界、形态、纵横比、囊性成分、实性部分回声、钙化、血供及颈部淋巴结等方面的差异性,归纳其声像图特征。结果：MTC体积较大,低回声（97.5%）,实性（92.5%）,A/T＜1（97.5%）,边界清晰（62.5%）,形态规则（45%）,可有钙化（32.5%为粗钙化、25%为微钙化）,血供丰富（95%）,淋巴结转移（65％）。7例MTC术前检测血清降钙素均显著升高。结论：MTC具有甲状腺恶性肿瘤的一般超声特征,但又与常见的PTC不同,具有其自身特点,不典型MTC易误诊为甲状腺良性结节,可结合血清降钙素检测及细胞学穿刺活检综合判断。     

Cor pulmonale due to embolization of metastatic chondrosarcoma

Summary A 25-year-old woman with acute dyspnea was found to have nodular shadows in a chest X-ray film. Multiple defects in a perfusion scan indicated pulmonary embolization. The cause of acute cor pulmonale was extensive metastatic embolization from chondrosarcoma of the calf. Embolization by metastasis as a rare occurrence resulting in acute cor pulmonale should be considered in patients with a malignant tumor.     

我国肺功能检测应用现状的调查和分析

目的：对肺功能检测在我国的应用现状进行首次调查。方法：对我国29个省，直辖市，自治区212家医院进行问卷调查。结果：检测回函率为81．6％，已开展肺功能检测为91．9％，其中三级医院为69．2％，肺功能检测在20世纪70年代后期开展较快，90年代产期达到高峰，国产肺功能检测仪仅为12．4％，进口检测仪为87．6％，平均每月肺功能应用次数及每病床使用数总体较低，肺功能检测项目和临床应用在三级和二级医院间有较大差异，肺功能在内，外科应用较为广泛，儿科，妇科，耳鼻喉科等应用较少，检测的基本项目是通气功能，所有已开展肺功能检测的医院都已开展，支气管扩张试验（74．2％）和激发试验（65．4％）应用也较为广泛，肺容量（60．0％），弥散（58．2％）及气道阻力（42．7％）测定分别在三级医院开展，但二级和以下医院开展较少，运动心肺功能试验在三级医院中只有16．4％的医院开展，三级以下医院均为空白，对所选用的肺功能正常预计值能明确说明的医院只有21．4％，能明确适用适于儿童正常值的医院只占开展儿科检测的16．9％，引用国人预计值的医院仅16．3％，结论：我国肺功能检测发展仍不平衡，对肺功能检测临床应用的认识有待加强，肺功能仪器国产化亟待加强，肺功能检测应更广泛的开展（对象和项目），并应重视正确的选用肺功能预计值。     

The Relationship Between HRCT and Pulmonary Function in Behcet’s Disease

The purpose of this study was to compare the pulmonary functions in female Behcets patients with or without pathological pulmonary lesions using high resolution computerized tomography (HRCT). Twenty-nine female patients aged 19–54 yrs and 20 healthy females aged 19–50 yrs (control group) were accepted into the study. HRCT images were taken and according to HRCT patients were divided into HRCT (+) and HRCT (–) groups (18 patients in each). Pulmonary function tests (PFT) through a spirometer were performed and FVC, FEV₁, FEF_25–75%, PEF, VC, RV, FRC, DLCO and DLCO/VA were determined. No statistically significant difference was observed when the PFT values were compared between HRCT (+) and HRCT (–) patients for the obtained results and the percentage of the expected values. When the PFT values were compared for both HRCT (+) and HRCT (–) patients with the control group separately there was no statistical difference between the best values. A statistically low DLCO/VA value was observed between the percentage of the expected values. It is concluded that though there is a pulmonary restriction in Behcets disease, this restriction has no relation to pulmonary functions. It would be useful to perform pulmonary function tests in the patients with Behcets disease both with or without any pathological findings in HRCT for obtaining information about pulmonary functions. We suggest that even when the results of the pulmonary function tests are normal, considering some pathological changes in HRCT, HRCT investigations may be useful for following up the disease.     

Histoplasmosis of bone

A case of histoplasmosis of bone manifested by a single punched-out lesion in the humerus is reported in a man with asymptomatic mild chronic disseminated histoplasmosis. Roentgenologically evident lesions of bone due to Histoplasma capsulatum are rare, and this is the only report of a large punched-out lytic lesion. Such lesions are common in disseminated histoplasmosis due to the African strain H. duboisii. Although H. duboisii has been accorded species differentiation, there are only two clearly defined differences, compared to H. capsulatum. One is morphologic (size of yeast cell), and the other is a different clinical disease spectrum. Many believe that this organism is best viewed as a stable variant of H. capsulatum. At times, the two strains are indistinguishable morphologically, and at times, as in the case reported, there may be clinical similarities in their respective disease entities.     

反义基质金属蛋白酶抑制剂-1基因转染对氧化应激反应介导的肺纤维化大鼠的影响

目的:探讨反义基质金属蛋白酶抑制剂-1(TIMP-1cDNA)基因转染对氧化应激反应介导的肺纤维化大鼠的影响。方法:取40只大鼠,其中30只建立肺纤维化模型,随机分为模型组、反义组和空载组,其余10只为对照组。术后第1天,反义组和空载组向气管分别注入反义TIMP-1cDNA逆转录病毒载体和空载体,模型组和对照组注入生理盐水溶液。观察4组大鼠一般体征和肺组织病理学变化,对比肺系数、丙二醛(MDA)、超氧化物歧化酶(SOD)水平及谷胱甘肽/氧化型谷胱甘肽(GSH/GSSG)比值;对比肺组织中基质金属蛋白酶-9(MMP-9)、TIMP-1、Ⅳ型胶原mRNA和蛋白相对表达量及MMP-9/TIMP-1比值。结果:对照组大鼠一般状态良好,但模型组和空载组状态逐渐变差,反义组有所改善;对照组肺组织正常,模型组和空载组肺纤维化及组织结构损伤严重,反义组较模型组减轻,但仍有炎症细胞。肺系数、肺组织MDA水平、TIMP-1、Ⅳ型胶原mRNA和蛋白相对表达量对照组最低,反义组其次,模型组和空载组最高;肺组织SOD水平、GSH/GSSG对照组最高,反义组其次,模型组和空载组最低;肺组织MMP-9/TIMP-1 mRNA相对表达量及MMP-9/TIMP-1比值组间比较,反义组最高,模型组和空载组其次,对照组最低。上述指标模型组和空载组均相近(P0.05),其余每2组间比较差异均显著(均P0.05)。结论:反义TIMP-1基因转染可减轻氧化应激反应介导的肺纤维化,可能与下调TIMP-1、上调MMP-9 mRNA和蛋白表达有关。     

不同肺静脉电隔离策略治疗阵发性心房颤动的对比研究

目的对现阶段阵发性心房颤动（房颤）肺静脉电隔离治疗最常采用的两种导管消融策略进行比较。方法回顾性分析本院连续完成的第51～100例肺静脉节段性消融术（SPVA组）和第51～100例肺静脉环形消融术（CPVA组）的相关资料,比较两组的操作相关指标、安全性及临床效果。所有患者均为发作频繁、症状严重的阵发性房颤。结果两组的基线资料和平均操作时间差异无统计学意义,但SPVA组的平均X线透视时间较长（P〈0．01）,平均放电时间较短（P〈0．01）。术后3个月内CPVA组和SPVA组症状性房性快速心律失常（ATa）的复发率分别为30％和48％（P=0．10）。术后随访6个月时,CPVA组有41例（82％）患者在未服用抗心律失常药物的情况下已经至少连续3个月未再发作症状性ATa,而SPVA组仅有30例（60％）达此标准（P〈0．05）。两组术中各出现1例无症状性右上肺静脉狭窄,无其他重要并发症。结论肺静脉电隔离治疗阵发性房颤的CPVA策略在临床效果方面显著优于SPVA策略,且二者的安全性相当。     

慢性缺氧对肺外动脉胶原堆积及胶原基因表达的影响

模拟海拔５０００米连续低压缺氧７天复制大鼠肺动脉高压模型，观察结构重建的肺外动脉胶原含量及其基因表达的变化。结构表明：缺氧７天末，大鼠肺动脉压、右心室／左心室＋室间隔重量比值及肺外动脉湿干重量和羟脯氨酸含量均明显升高；经Ｎｏｒｔｈｅｒｎ杂交和斑点杂交分析无交叉和非特异性杂交的编码人Ｐｒｏａ１（Ｉ），Ｐｒｏａ１（Ⅲ）肽链的ｃＤＮＡ探针，进行ＤＮＡ－ＲＮＡ的斑点杂交显示，缺氧组织的Ｐｒｏａ１（Ｉ），ｐ