心脏结节病

结节病是一种至今病因未明的全身性肉芽肿疾病。本病国外常见,近年来国内报道也逐渐增多。在临床上医生们往往注意到肺、淋巴结、皮肤结节病,而忽视了心脏结节病。心脏结节病是结节病的主要死亡原因,并且近年来认为,临床上不能解释的一些现象     

以浅表淋巴结肿大为表现的结节病2例

结节病在临床上少见,其病理、生理基础为非干酪样坏死性肉芽肿浸润,确诊需依赖肺、淋巴结的活组织病理检查。本文回顾性分析了2例结节病的临床资料,重点讨论结节病的临床特征和鉴别诊断要点,以提高本病的诊断及治疗水平。     

胸内结节病临床诊治体会

目的:探讨胸内结节病的临床特点及诊疗方法。方法:结合11例结节病的临床诊疗过程,并对相关文献进行复习,了解结节病的诊疗方法及误诊因素。结果:胸内结节病临床表现缺乏特异性,易造成漏诊、误诊误治。结论:掌握胸内结节病的临床特点,影像学检查并与实验室检查特别是病理组织学检查结合是减少漏诊、诊误诊误治的主要措施。     

结节病症状与支气管肺泡灌洗液相关性分析

目的:通过对有或无临床症状的胸部结节病患者支气管肺泡灌洗液(BALF)中淋巴细胞表型的分析,探讨结节病患者临床症状与肺部免疫反应之间的关系。方法:有明显症状就诊的结节病患者44例,无症状体检发现的结节病患者30例,分别行纤维支气管镜检查并肺泡灌洗测定BALF中淋巴细胞比例、T淋巴细胞CD4+/CD8+比值。另对有临床症状者17例进行激素治疗前后BALF中淋巴细胞比例、CD4+/CD8+比值的比较。结果:(1)结节病有临床症状的患者BALF中淋巴细胞比例、CD4+/CD8+比值显著高于无症状组(P<0.05,P<0.05)。(2)有症状的结节病经激素治疗后BALF中淋巴细胞比例、CD4+/CD8+比值较治疗前明显下降。结论:(1)结节病患者存在CD4+T细胞升高为主的细胞免疫异常,免疫活性高者多伴有明显临床症状,激素可调节结节病的免疫异常,BALF细胞学检测有助于判断结节病的免疫活动性。     

结节病11例分析

目的 提高临床医生对结节病的认识。方法 对11例结节病患者临床资料进行回顾性分析。结果 11例结节病患者呼吸系统表现8例，肺外表现3例。结论 结节病多见于中青年，临床表现多样，缺乏特异性。     

结节病12例临床分析

目的：探讨提高结节病诊断率的方法。方法：回顾性分析2005年6月～2006年6月笔者在进修期间见到的经病理确诊的结节病12例患者的临床资料。结果：由于结节病临床表现缺乏特异性及部分临床医生对该病认识不足，因而其诊断率低、误诊率高。结论：掌握结节病临床特点及影像学特点，结合实验室检查可提高其诊断率。     

纤维化型肺结节病的发病机制及其影响因素的研究进展

结节病是一种病因不明的多系统累及的肉芽肿性疾病,好发于呼吸系统,如不及时治疗,约20%的患者最终可能会发展为肺纤维化,严重时并发呼吸衰竭,最终导致死亡。结节病发展为肺纤维化后严重影响患者的生活质量及生存期。因此,深入了解纤维化型肺结节病的发病机制具有重要的临床意义。纤维化型肺结节病的发生与遗传学有关联,基因多态性、转录水平的调控和表观遗传改变等对纤维化型肺结节病的进展有重要影响。促纤维化因素、信号转导过程和免疫机制也参与促进结节病发展为肺纤维化。此外,结节病相关的肺纤维化患者可同时并发肿瘤,2种疾病之间可能存在关联,结节病可能会增加肿瘤的发病风险。结节病的临床分期及分型不同,治疗方案也不同。现对纤维化型肺结节病的发病机制中基因遗传学机制、促纤维化因素、免疫学机制、与特发性肺纤维化(IPF)和肿瘤的相关性及治疗方面的新进展进行综述,以进一步提高临床医生对该病的认识。     

肺结节病与中心型肺癌的鉴别诊断

目的探讨肺结核病与中心型肺癌的鉴别依据。方法分析２１例肺结节病和１１６例中心型肺癌的临床特别。结果肺结节病多发于４０岁结节病临床症状较少，可有皮损，眼部病变，而肺癌咳嗽、咯痰、咯血症状明显增多，病变侧呼吸音常有减低。Ｘ线及纤支镜所见肺结节病与肺癌各有其特点。肺结节病患者多数ＳＡＣＥ升高，而肺癌患者多为ＣＥＡ升高。结论应结合临床症状、体症、Ｘ线、纤支镜等辅助检查，进行鉴别。     

以急性肾损伤为表现的结节病一例

结节病是一种多系统受累的疾病,以肺部受累最为常见,肾脏受累较为少见.本文报道1例以急性肾损伤起病的结节病,并就其进行文献复习,以及临床特征、病理特点和治疗疗效的探讨,以加深临床医师对结节病所致肾损伤的认知.     

以双肺弥漫病变为表现的肺内结节病1例报告

结节病是一种病因不明以非干酪样肉芽肿为病理特征的多脏器受累的疾病,诊断包括临床和影像学表现,确诊依赖病理学检查。但结节病在我国发病率低,临床和影像表现可无特异性,经常遇到病理取材受限,并且随着研究进展,非干酪样肉芽肿即为结节病观点受到质疑等,使结节病容易误诊和漏诊。因此结节病的诊断仍是个难题。本文对我院收治的     

结节病误诊结核病18例分析

目的：本文对结节病误诊结核病18例进行分析，以提高对结节病认识。方法：通过临床观察，胸部x线，纤支镜活检，实验室检查分析。结果：造成误诊原因为①对结节病认识不足，②胸部x线易混淆，③病理检查取材困难，Keveim试剂来源限制，难以取得病理依据。结论：胸内结节病难以排除结核病时，可作SACE活性测定，纤支镜内肺活检及肺功能检查，以提高结节病诊断与结核病的鉴别。     

Serial estimation of serum angiotensin converting enzyme activity during and after pregnancy in a woman with sarcoidosis

Serum angiotensin converting enzyme activities were estimated during pregnancy and the puerperium in a woman with sarcoidosis and a series of normal women. In the patient with sarcoidosis angiotensin converting enzyme activity was raised during pregnancy, particularly at 21 weeks' gestation, yet she remained well with no symptoms to suggest relapse of sarcoidosis. Serum angiotensin converting enzyme activity may not be of value in monitoring sarcoidosis activity during pregnancy.     

Characteristics of patients with a diagnosis of sarcoidosis: a comparison of the 2006 and 2015 versions of diagnostic criteria for sarcoidosis in Japan

Objective: Histological verification of epithelioid cell granuloma is important in diagnosing sarcoidosis; tissue sampling is a worldwide requirement. In 2006, to reduce medical expenses and avoid invasive procedures, diagnostic criteria without histological verification were permitted by the Japanese government. In 2015, new diagnostic criteria, allowed clinical diagnoses based on only respiratory, ocular, and cardiac systems with at least a two-system involvement, increasing the need to sample tissue from clinically unevaluable organs in suspected sarcoidosis. This study aimed to compare the characteristics of patients who were diagnosed with sarcoidosis according to the 2006 and 2015 criteria.Materials and Methods: Using the 2015 version, we re-evaluated the characteristics of 264 patients with diagnosed or suspected sarcoidosis according to the 2006 criteria, at Jichi Medical University Hospital between 2004 and 2012 (clinical diagnosis, 84; histological diagnosis, 117; suspected sarcoidosis 63).Results: Thirty-nine patients were diagnosed with suspected sarcoidosis due to the absence of at least a two-system involvement; two patients had insufficient laboratory data suggestive of sarcoidosis. Six patients moved from suspected sarcoidosis to a histological diagnosis because of a greater leniency in the criteria for supportive findings. The 2015 diagnostic criteria excluded patients with organ involvement without a requirement for systemic steroids from the clinical diagnosis group. A case of schwannoma, erroneously placed in the clinical diagnosis group by the 2006 criteria, was reclassified according to the 2015 criteria.Conclusion: The 2015 version is preferable for clinically diagnosing sarcoidosis, even without histological specimens, and provides guidance for indications for systemic treatment.     

结节病肾损害1例并文献复习

目的:通过分析结节病累及肾脏患者的临床资料,旨在提高结节病肾损害诊断和治疗水平。方法:回顾性分析1例女性结节病肾损害患者的临床表现、实验室检查及疗效,结合文献复习,总结结节病肾损害的诊治经验。结果:患者以不明原因的高钙血症、慢性肾功能不全为首发症状,1年后经淋巴结活检诊断为结节病,给予糖皮质激素和免疫抑制剂治疗,肾功能、高钙血症好转。结论:结节病肾损害常常廷误诊断,明确诊断后治疗首选糖皮质激素治疗,疗效欠佳时可联合应用免疫抑制剂。     

结节病患者外周血Th17细胞表达及临床意义

目的研究不同时期结节病患者外周血淋巴细胞亚群Th17细胞的表达变化,并探讨其临床意义。方法选取结节病患者38例,其中初诊并伴有咳嗽、乏力、消瘦、发热等明显临床症状的活动期结节病患者18例,复诊的稳定期结节病患者20例。另选取15例健康志愿者为对照组。流式细胞术检测所有人选者外周血Th17细胞表达,紫外分光光度法检测所有结节病患者血管紧张素转化酶（SACE）水平。所有初诊结节病患者行支气管肺泡灌洗液细胞分类及CD4＋／CD8＋比例检查。结果活动期结节病患者外周血Th17／CD3＋ T细胞百分比较稳定期结节病患者及健康对照组明显增高[（1．59±0．44）％比（0．564-0．32）％和（0．49±0．23）％,P均〈0．05],而稳定期结节病患者外周血Th17／CD3＋T细胞百分比与健康对照组无明显差异（P〉0．05）。活动期结节病患者SACE水平较稳定期结节病患者SACE水平明显增高[（56．6±14．6）IU／L比（35．8±18．3）IU／L,P〈0．05],结节病患者外周血Th17细胞表达与SACE水平无明显相关性（P〉0．05）;活动期结节病外周血Th17细胞表达与BALF中淋巴细胞百分比无明显相关性（P〉0．05）,但与CD4＋／CD8＋呈明显正相关（r=0．63,P〈0．05）。结论活动期结节病患者外周血Th17细胞表达增高,结节病患者外周血Th17细胞表达增高可能与结节病的病情活动有关。     

大麻素Ⅱ型受体在结节病、环状肉芽肿中的组织分布

目的：研究大麻Ⅱ型受体（CB2）在人正常皮肤及结节病、环状肉芽肿的组织分布。方法：用兔抗人CB2受体抗体做免疫组化,检测人皮肤及结节病、环状肉芽肿的组织CB2受体表达分布情况。结果：CB2受体在人皮肤及结节病、环状肉芽肿均有表达分布,且结节病、环状肉芽肿的CB2受体过度表达。结论：大麻素受体CB2的表达与皮肤结节病、环状肉芽肿关系密切。     

Evaluation of the clinical and histological features of paratracheal and hilar gland enlargement

Sixty-nine patients with a chest radiograph showing enlargement of the hilar and/or paratracheal lymph nodes were analysed. Ten of these, mainly Asian, were found to be suffering from tuberculosis. Of the remaining fifty-nine, who had sarcoidosis, 12% were tuberculin-positive. All the cases diagnosed as sarcoidosis had either a positive Kveim test and/or mediastinal lymph node histology compatible with sarcoidosis. 10% of cases with sarcoidosis showed evidence of uveitis and 10% showed bone cysts and there was also a high proportion of other extrathoracic lesions.     

结节病的药物治疗进展

结节病是一种以非干酪性上皮样肉芽肿为病理表现、全身多系统受累的疾病,以肺脏和胸内淋巴结受累最为常见。目前糖皮质激素仍是结节病的首选治疗药物。但长期使用激素可带来诸多的不良反应,急需研发新的治疗药物和方法。本文就结节病药物治疗方面的研究进展作一综述。     

Association of angiotensin-converting enzyme/DD genotype with sarcoidosis susceptibility in Slovenian patients.

BACKGROUND: Sarcoidosis is a multisystemic chronic inflammatory disorder of unknown etiology with multifactorial genetic predisposition. An elevated ACE serum level is considered to be the activity marker of the disease. The involvement of the ACE I/D polymorphism in sarcoidosis susceptibility has been investigated in different populations, but results have been inconclusive. The purpose of this study was to evaluate the possible association of angiotensin-converting enzyme (ACE) gene insertion (I)/deletion (D) polymorphism with sarcoidosis in the Slovene population. MATERIAL/METHODS: In 105 sarcoidosis patients (69 female, 36 male, mean age: 41+/-1 years) and in 80 sex- and age-matched control subjects, genotyping for the ACE gene I/D polymorphism was performed by PCR and restriction enzyme digestion. RESULTS: An increased frequency of DD homozygotes vs. II homozygotes + ID heterozygotes was found in the group of sarcoidosis patients compared with the control group (OR: 2.19, 95%CI: 1.12-4.26, p=0.02). No differences in genotype frequencies were found in the group of sarcoidosis patients when considering the clinical course or presentation of the disease. CONCLUSIONS: These results indicate that the ACE gene I/D polymorphism might be a risk factor for sarcoidosis susceptibility in the Slovene population and imply the possible role of population origin in the modulation of the influence of ACE gene variability in the pathophysiology of sarcoidosis.     

肺结节病支气管肺泡灌洗液中血管紧张素转化酶测定及临床意义

测定肺结节病患者支气管肺泡灌洗液中血管紧张素转化酶（ＢＡＬＦ－ＡＣＥ）的水平。结果显示，活动性结节病的ＢＡＬＦ－ＡＣＥ水平明显高于对照组（Ｐ＜０．０５）。相关性分析表明，ＢＡＬＦ－ＡＣＥ与支气管肺泡泡灌洗液中淋巴细胞百分率有明显正相关（γ＝０．８７２３，Ｐ＜０．０１）。提示ＢＡＬＦ－ＡＣＥ在肺结节病的病理机制中起着重要作用，并可作为诊断肺结节病的一项敏感的生物学指标。