Ultra-rare cystic disease.
Ultra-rare cystic disease.
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非常罕见的囊性疾病
DOI：
10.1183/16000617.0163-2019
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发表时间：
2020-09-30
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影响因子：
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中科院分区：
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弥漫性囊性肺疾病包括一组异质性疾病，其特征在于肺实质内存在囊肿，有时显示特征性计算机断层扫描扫描模式，允许诊断。肺内囊肿形成的发病机制尚不清楚，根据不同的病因提出了许多假说：球阀效应、与供应终末细支气管的小血管和毛细血管浸润和阻塞有关的小气道和肺泡的缺血性扩张以及基质金属蛋白酶引起的结缔组织降解。根据病因学，大量肺囊肿疾病被分为六个诊断组：肿瘤性、先天性/遗传性、淋巴增生性、感染性、与间质性肺疾病相关以及其他原因。本文重点介绍淋巴管平滑肌瘤病、肺朗格汉斯细胞组织细胞增生症和Erdheim-Chester病、Birt-Hogg-Dubé、滤泡性细支气管炎和淋巴细胞性间质性肺炎、轻链沉积病和淀粉样变性、与异常肺发育和生长相关的先天性肺病以及与肿瘤性病变相关的囊性肺病。这些囊性疾病在流行病学上被认为是超罕见的疾病，因为它们影响每50 000人中不到1人或每百万人中不到20人。 尽管这组疾病的罕见，越来越多地使用高分辨率计算机断层扫描提高了诊断率，即使在无症状的患者允许及时和正确的治疗和管理，而不需要活检。弥漫性囊性肺疾病显示出特征性的CT扫描模式，即使在无症状的患者中也可以进行诊断，从而可以在不需要活检的情况下进行及时正确的治疗和管理。https://bit.ly/2wIUKet
Diffuse cystic lung diseases include a group of heterogeneous disorders characterised by the presence of cysts within the lung parenchyma, sometimes showing a characteristic computed tomography scan pattern that allows diagnosis. The pathogenetic mechanisms underlying cyst formation in the lung are still not clear and a number of hypotheses have been postulated according to the different aetiologies: ball-valve effect, ischaemic dilatation of small airways and alveoli related to infiltration and obstruction of small vessels and capillaries that supply the terminal bronchioles and connective tissue degradation by matrix metalloproteases. A wide number of lung cyst diseases have been classified into six diagnostic groups according to the aetiology: neoplastic, congenital/genetic, lymphoproliferative, infective, associated with interstitial lung diseases, and other causes. This article focuses on lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and Erdheim–Chester disease, Birt–Hogg–Dubé, follicular bronchiolitis and lymphocytic interstitial pneumonia, light-chain deposition disease and amyloidosis, congenital lung disease associated with aberrant lung development and growth, and cystic lung disease associated with neoplastic lesion. These cystic diseases are epidemiologically considered as ultra-rare conditions as they affect fewer than one individual per 50 000 or fewer than 20 individuals per million. Despite the rarity of this group of disorders, the increasing use of high-resolution computed tomography has improved the diagnostic yield, even in asymptomatic patients allowing prompt and correct therapy and management without the need for a biopsy. Diffuse cystic lung diseases show a characteristic CT scan pattern that often allows for diagnosis, even in asymptomatic patients, allowing prompt correct therapy and management without the needing of a biopsy https://bit.ly/2wIUKet
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DOI：
10.1084/jem.20130977
发表时间：
2014-04-07
期刊：
The Journal of experimental medicine
影响因子：
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作者：
Berres ML;Lim KP;Peters T;Price J;Takizawa H;Salmon H;Idoyaga J;Ruzo A;Lupo PJ;Hicks MJ;Shih A;Simko SJ;Abhyankar H;Chakraborty R;Leboeuf M;Beltrão M;Lira SA;Heym KM;Bigley V;Collin M;Manz MG;McClain K;Merad M;Allen CE
通讯作者：
Allen CE
DOI：
10.1164/rccm.200510-1620cr
发表时间：
2006-04-01
影响因子：
24.7
作者：
Colombat, M;Stern, M;Danel, C
通讯作者：
Danel, C
影响因子：
20.3
作者：
Badalian-Very, Gayane;Vergilio, Jo-Anne;Rollins, Barrett J.
通讯作者：
Rollins, Barrett J.
DOI：
10.4103/0189-6725.150987
发表时间：
2015-01-01
期刊：
African journal of paediatric surgery : AJPS
影响因子：
--
作者：
Barman, Shibsankar;Mandal, Kartik Chandra;Mukhopadhyay, Biswanath
通讯作者：
Mukhopadhyay, Biswanath
影响因子：
9.6
作者：
Cudzilo, Corey J.;Szczesniak, Rhonda D.;Young, Lisa R.
通讯作者：
Young, Lisa R.