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[儿童期肺部的间质病变]

[Interstitial processes of the lungs in childhood].

作者信息

Popper H

机构信息

Forschungseinheit für molekulare Lungen- und Pleurapathologie, Institut für Pathologie, Medizinische Universität Graz, Auenbruggerplatz 25, 8036, Graz, Österreich.

出版信息

Pathologe. 2017 Jul;38(4):260-271. doi: 10.1007/s00292-017-0280-2.

DOI:10.1007/s00292-017-0280-2
PMID:28349192
Abstract

Interstitial processes in the lungs of children can be due to several underlying diseases. Knowledge of the child's age is important as genetic aberrations play a major role in diseases in the first 2 years, whereas immunological diseases are more common starting in kindergarden age. In general lung diseases are rare in children, which makes the diagnostics difficult and results in a delayed diagnosis. In addition, pediatric pulmonologists are often very reluctant to perform lung biopsies due to a lack of a specialized pathologist. In order to make a contribution to the diagnostics of pediatric pulmonary diseases, pathologists should be specialized in pulmonary pathology, have a good knowledge of genetic methods and fetal lung development, which includes the genetic factors involved in lung growth and differentiation. A close cooperation with the pediatric pulmonologist is necessary and each patient should be discussed jointly on an interstitial lung disease board to promote the quality of diagnostics. The pathologist should be aware that the developing lungs of children are not just a smaller form of adult lungs and often react very differently. In this article, we mainly focus on diffuse infiltration patterns, such as ground glass and reticulonodular infiltrations as described in high-resolution computed tomography (HRCT). Localized interstitial processes, which can sometimes be tumor-like and malformations are not dealt with; however, vascular malformations are included as these often manifest as diffuse interstitial infiltrations and must therefore be taken into consideration for the differential diagnostics.

摘要

儿童肺部的间质性病变可能由多种潜在疾病引起。了解儿童的年龄很重要,因为基因异常在两岁以内的疾病中起主要作用,而免疫性疾病从幼儿园年龄开始更为常见。一般来说,肺部疾病在儿童中很少见,这使得诊断困难并导致诊断延迟。此外,由于缺乏专业病理学家,儿科肺科医生通常非常不愿意进行肺活检。为了对儿科肺部疾病的诊断做出贡献,病理学家应专门从事肺部病理学,对基因方法和胎儿肺发育有充分了解,这包括参与肺生长和分化的基因因素。与儿科肺科医生密切合作是必要的,每个患者都应在间质性肺病委员会上共同讨论,以提高诊断质量。病理学家应意识到,儿童发育中的肺部不仅仅是成人肺部的较小形式,而且反应往往非常不同。在本文中,我们主要关注高分辨率计算机断层扫描(HRCT)中描述的弥漫性浸润模式,如磨玻璃影和网状结节状浸润。局部间质性病变,有时可能类似肿瘤和畸形,本文不予讨论;然而,血管畸形包括在内,因为这些病变通常表现为弥漫性间质性浸润,因此在鉴别诊断中必须予以考虑。

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本文引用的文献

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Current Concepts and Controversies in Innate Immunity of Cystic Fibrosis Lung Disease.囊性纤维化肺病固有免疫的当前概念与争议
J Innate Immun. 2016;8(6):531-540. doi: 10.1159/000446840. Epub 2016 Jul 1.
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The Dynamics of Disease Progression in Cystic Fibrosis.囊性纤维化疾病进展的动态变化
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Pulmonary interstitial glycogenosis in a patient with trisomy 21.一名21三体综合征患者的肺间质糖原贮积症
J Neonatal Perinatal Med. 2016 May 17;9(2):227-31. doi: 10.3233/NPM-16915112.
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Pulmonary veno-occlusive disease.肺静脉闭塞病。
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