肺淋巴管平滑肌瘤病的临床及CT表现

目的:探讨肺淋巴管平滑肌瘤病(PLAM)的临床及CT表现特征,提高对本病的认识.方法:回顾性分析3例PLAM的临床表现特征、肺功能检查结果及CT征象.结果:3例均为女性,年龄分别为28、40和42岁,临床主要表现为进行性呼吸困难,实验室检查3例均有低氧血症.CT显示两肺弥漫分布的多个小囊状透亮影,囊壁薄,厚度均匀,壁厚约1mm左右,HRCT囊腔影薄壁显示清晰.结论:PLAM主要表现为进行性呼吸困难,常伴有咯血、自发性气胸、乳糜胸及低氧血症,HRCT对本病的早期诊断具有重要意义.     

肺淋巴管平滑肌瘤病的病理与胸部放射表现

肺淋巴管肌瘤病或称肺淋巴管平滑肌增生(pulmonary lymphangioleiomyomatosis,PLAM)是一种病因不明、持续发展的弥漫性肺间质疾病[1],主要累及肺、纵隔淋巴结及腹部淋巴结的少见疾病.肺淋巴管平滑肌瘤病是发生在育龄期妇女.由Burrel及Ross于1937年首选描述,Cornog及Enterline在1966年将此病命名为PLMA,是一类少见的、发生于特殊人群的、具有特别临床经过和预后的疾病[2].其病理特征是肺支气管及细支气管壁、肺泡间隔、肺血管、淋巴管及胸膜的平滑肌进行性不规则增生,导致弥漫性肺间质病变.临床以反复发作的自发性气胸、乳糜性胸水或腹水、间断性咯血及进行性呼吸困难为特征.该病是一种罕见的综合征.以前只有病理检查才能发现,但是随着高分辨率CT(HRCT)技术的出现和应用,使得影像科医师可以直接观察到这种改变,它在HRCT上表现为小囊状影及囊状影的壁.笔者将其有关病理及HRCT特征综述如下.     

肺淋巴管平滑肌瘤病影像学分析

目的 探讨肺淋巴管平滑肌瘤病(PLAM)胸部X线片、多排螺旋CT(MSCT)的影像学表现特点及诊断价值.方法 回顾性分析经病理证实的4例PLAM的临床和胸部X线片、MSCT表现.并复习文献进行分析.结果 PLAM临床主要表现为呼吸困难(4例),咳嗽、咳痰(2例),咯血(2例),反复出现的气胸(2例)和乳糜胸(1例)等.胸部X线片主要表现为弥漫性分布的网状阴影(2例)、蜂窝状透亮区(1例)、气胸(2例)和胸腔积液(1例)等;MSCT特征性表现:2例见双肺弥漫性分布、大小不一、薄壁、囊状透亮区,囊腔直径3～15 mm.1例广泛性囊变合并肺间质纤维化并胸膜增厚、粘连,确诊后3个月死亡,余症状缓解出院.结论 PLAM是一种罕见的弥漫性肺间质病变,胸部X线片表现缺乏特征;MSCT特征性表现对临床诊断有重要价值.     

肺淋巴管肌瘤病的HRCT诊断

目的 探讨肺淋巴管肌瘤病（PLAM）高分辨率CT（HRCT）特征性表现,以期提高对该病的认识水平.方法 回顾性分析7例经病理证实的肺淋巴管肌瘤病影像学及临床资料,所有病例均行胸部常规CT及HRCT扫描.结果 常规胸部CT见两肺密度减低,偶见囊状影;HRCT清晰显示两肺弥漫、均匀分布的圆形薄壁囊状阴影,病灶之间的肺组织正常.随着病程进展,囊状阴影有增大、增多趋势,部分融合成肺大泡样改变,亦可成形态怪异的囊腔.结论 HRCT上表现为两肺弥漫、均匀分布的圆形薄壁囊状阴影,病灶之间的肺组织正常,再结合性别、年龄、进行性呼吸困难等临床资料,应考虑PLAM可能.     

肺淋巴管肌瘤病的影像学分析

目的探讨肺淋巴管肌瘤病的影像学表现，提高诊断水平。方法回顾性分析7例经病理证实的肺淋巴管肌瘤病的胸部X线片、常规CT及高分辨CT（HRCT）表现。结果胸部X线片无特异性，1例胸部X线片无异常发现，1例表现为两肺弥漫性的网状和线条状阴影，小蜂窝状或多发小囊状影，3例表现为气胸，2例表现为右侧胸腔积液。无一例发现肺门或纵隔淋巴结肿大。常规CT扫描虽然发现直径较大的囊腔样病变，但囊壁显示不清，HRCT扫描病变显示清晰，表现为两肺从肺尖至肺底弥漫分布的囊状改变，均有薄壁，壁厚约1mm，边缘清楚，厚薄均匀。大部分囊腔直径〈20mm。结论肺淋巴管肌瘤病的HRCT表现具有特征性，HRCT对明确诊断以及预后判断具有非常重要的应用价值。     

弥漫性肺疾病高分辨CT与多排螺旋CT薄层重组的对照研究

目的：探讨高分辨CT（HRCT）与多排螺旋CT（MSCT）薄层重组对弥漫性肺疾病的诊断价值,以及HRCT是否能被MSCT薄层重组所替代。方法：搜集同时做HRCT及MSCT薄层重组的弥漫性肺疾病患者51例进行回顾性分析。结果：51例患者在MSCT薄层重组中48例有不同程度的肺内改变：双肺弥漫性或散在小结节影12例;肺内弥漫分布网格状改变22例,可显示小叶间隔线增厚;肺内弥漫分布囊状透亮区病变14例,可显示弥漫分布的囊性病变。HRCT图像可更加清晰地显示肺内小结节影分布情况,弥漫分布网格状影像,对于弥漫分布囊状透亮区,HRCT能够明确分辨小叶中央型及全小叶型病变,51例均有阳性发现。结论：MSCT薄层重组对弥漫性肺疾病的诊断阳性率与HRCT无明显差别,但细节分辨率及图像质量低于HRCT,因此不能完全替代HRCT扫描。     

肺淋巴管平滑肌瘤病CT诊断

目的探讨肺淋巴管肌瘤病(PLAM)的临床特征及常规CT与高分辨率CT(HRCT)表现,以便临床诊断。方法回顾性分析2008年1月—2011年12月期间经该院经病理证实的12例肺淋巴管平滑肌瘤患者的CT资料。结果所有患者常规CT上均可见广泛小透亮影、囊状影均匀分布于两肺,没有中央与周围型之分,也没有上中下野的差别,囊性气腔以圆形或椭圆形为主;HRCT上表现为两肺均匀薄壁小囊腔,所有囊腔囊壁完整,囊壁厚度为1～2mm,囊气腔被正常肺组织环绕。结论肺淋巴管平滑肌瘤病是一种好发于育龄期妇女的疾病,CT尤其是HRCT扫描呈网状阴影或均匀分布的囊性气腔影应高度怀疑为PLAM,临床确诊有赖于病理检查。     

铁及其化合物粉尘肺沉着病2种影像诊断比较研究

目的 比较高千伏X射线胸片和胸部高分辨率计算机断层扫描(HRCT)对铁及其化合物粉尘肺沉着病(以下简称“铁肺沉着病”)的诊断价值。方法 采用偶遇抽样方法,选择某磁材厂80名接触铁氧体粉尘的工人进行高千伏X射线胸片和胸部HRCT检查。比较2种方法对铁肺沉着病诊断结论的差异。结果 80名工人中,仅5人(占6.2%)胸部HRCT未见异常改变,其余均呈现不同程度弥漫性分布的气腔结节、磨玻璃影病变。高千伏X射线胸片、胸部HRCT诊断铁肺沉着病,极轻度分别占8.8%(7/80)和37.5%(30/80),轻度者分别占21.3%(17/80)和26.3%(21/80);对铁肺沉着病患者的诊断率分别为30.0%(24/80)和63.8%(51/80)。采用2种方法诊断铁肺沉着病的结果存在一致性(Kappa值=0.411,P0.01);与高千伏X射线胸片比较,胸部HRCT对铁肺沉着病的诊断分度和诊断率均较高(P0.01)。结论 胸部HRCT对铁肺沉着病的诊断分级和诊断率均高于高千伏X射线胸片;胸部HRCT有助于该疾病的早期诊断。     

淋巴管肌瘤病的诊疗指南(2019)

1 概述 淋巴管肌瘤病(lymphangioleiomyomatosis, LAM),又称淋巴管平滑肌瘤病.是一种罕见的以肺部弥漫性囊性病变为特征的多系统肿瘤性疾病.几乎所有LAM均发生于女性,尤其以育龄期女性为主,男性LAM病例极其罕见.LAM的主要临床特征包括呼吸困难、气胸、乳糜胸、肾血管平滑肌脂肪瘤(AML).L...     

慢性阻塞性肺病并发自发性气胸58例临床观察

目的：了解慢性阻塞性肺病（COPD）并发自发性气胸的临床特点，找出易误诊的原因，诊断及治疗要点，方法：所有的商例均拍摄X线胸片，必须时加拍左，右侧位胸片或X线多轴透视或胸部CT检查，测定肺压缩的程度，并用人工气胸器测压确定气胸的类型，确诊后立即给予胸穿抽气或胸腔闭式引流治疗，结果：COPD并发气胸患者年龄较大，临床表现不典型，容易误诊为原发病加重，误诊率为22．4％，大部分病例（75．9％）需行的胸腔闭式引流治疗，病死率较高（5．2％），结论：对经积极治疗无效的COPD患者，要想到是否并发气胸，要常规拍摄胸片或X线多轴透视，一旦确诊为气胸，多需紧急胸穿抽气或胸腔闭式引流治疗。     

肺淋巴管肌瘤病的高分辨率CT表现

目的 探讨肺淋巴管肌瘤病的高分辨率CT（HRCT）特点,以提高对本病的诊断水平.方法 回顾性分析13例经病理证实的肺淋巴管肌瘤病患者胸部HRCT改变.结果 13例均为女性,年龄28 ～ 57岁,平均40岁.11例患者胸部HRCT表现为两肺均匀分布、多发的薄壁气囊影,直径数毫米至2 cm,囊壁厚1～2mm,气囊影无分布差异;囊腔周围为正常肺组织;2例患者弥漫分布大小不一囊腔、周围未见明显正常肺组织.1例患者左侧胸腔积液、腹膜后多发淋巴结肿大.结论 肺淋巴管肌瘤病的HRCT表现具有特征性,是诊断本病首选的影像学检查方法.     

Secondary spontaneous pneumothorax in rapidly progressive forms of silicosis: characterization of pulmonary function measurements and clinical patterns

BACKGROUND: A secondary spontaneous pneumothorax is a complication of an underlying pulmonary disease. In recent years, there have been only a few scattered reports of patients with silicosis also having a pneumothorax. Silicosis, a form of disabling pulmonary fibrosis, is a well-known occupational disease resulting from high-level exposure to silica or silica-containing dusts. The objective of the present study was to elucidate any associations between the occurrence of a pneumothorax, and pulmonary function tests and clinical observations performed prior to the pneumothorax; these two factors may be predictors for a pneumothorax among workers exposed primarily to silica-containing respirable dust. METHODS: A diagnosis of silicosis was made on several factors: silica dust exposure, appropriate interval of time after exposure, clinical findings, pulmonary function tests and chest radiological findings. A checklist was designed for collecting data of occupational history, respiratory signs, and symptoms from onset of dust exposure to the occurrence of a pneumothorax. Spirometery was conducted in accordance to the recommendations of standard protocols and guidelines posited by the American Thoracic Society. Autopsies were performed in three cases where the patient had suffered a pneumothorax due to silicosis. Mann-Whitney U-tests and Fisher's exact tests were used to determine any associations between pneumothorax and predictor factors. RESULTS: An association between a progressive decrease in pulmonary function test values and a pneumothorax was observed. The occurrence of a pneumothorax was associated with complaints of pleuretic chest pain, resting dyspnea, respiratory distress, paroxysmal nocturnal dyspnea, orthopnea and crackle. CONCLUSION: A characteristic decline in pulmonary function test values and the severity of respiratory impairment may facilitate the occurrence of a pneumothorax in silicosis.     

Rare association of rare diseases: Langerhans-cell histiocytosis and lymphangioleiomyomatosis in the lung]

This is a case history of a 24-year-old female patient in whom two rare pulmonary diseases occurred: Langerhans cell histiocytosis and four years later lymphangioleiomyomatosis were diagnosed. Both diseases were verified by the examination of the lung tissue removed by the surgery for pneumothorax. The patient's symptoms were characterised by coughing, dyspnoe, repeated pneumothorax. The authors summarised the characteristics and treatment of these diseases. This case history is worth of reporting for its unique rareness.     

High resolution computed tomography of diffuse interstitial pneumonia during treatment

HRCT was carried out in twenty patients with diffuse interstitial pneumonia: 13 cases of IIP, 3 of BOOP, 2 of drug-induced pneumonia, 1 of rheumatoid lung and acute interstitial pneumonia of unknown origin. With special attention to inflammatory activity, the patients underwent HRCT periodically during the treatment. Correlative investigation between HRCT image and grade of accumulation in 67Ga scintigraphy was also performed. Response to steroid therapy was clearly reflected on HRCT image, that was shown as decreasing pulmonary density or thinning of honeycomb wall. HRCT is considered to be useful in assessing the activity of diffuse interstitial pneumonia.     

30例特发性肺纤维化的临床特征分析

目的探讨特发性肺纤维化（IPF）的临床特征与诊断方法。方法对30例IPF患者临床表现、胸部高分辨CT（HRCT）、肺功能检查进行回顾性分析。结果30例IPF患者临床表现为咳嗽、进行性呼吸困难和Velcro音。胸部高分辨CT表现为磨玻璃样、网状、结节状、条索状及蜂窝状阴影。肺功能检查示24例限制性通气功能障碍，6例混合性通气功能障碍，均有弥散功能障碍。动脉血气分析示低氧血症。结论对长期咳嗽伴有呼吸困难的患者，及时行胸部高分辨CT、肺功能、动脉血气分析及肺活检，能对本病做出正确的诊断。