The bronchopulmonary pathology of Alpha-1 Antitrypsin (AAT) deficiency: Findings of the death review Committee of the National Registry for individuals with severe deficiency of Alpha-1 antitrypsin
The bronchopulmonary pathology of Alpha-1 Antitrypsin (AAT) deficiency: Findings of the death review Committee of the National Registry for individuals with severe deficiency of Alpha-1 antitrypsin
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DOI：
10.1016/j.humpath.2004.08.013
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发表时间：
2004-12-01
期刊：
影响因子：
3.3
通讯作者：
Stoller, JK
中科院分区：
文献类型：
--
作者：
Tomashefski, JF;Crystal, RG;Stoller, JK
为了评估入组α-1抗胰蛋白酶（AAT）严重缺乏个体登记研究时死亡的42名个体的肺和肝脏病理学变化，由死亡审查委员会的病理学家成员审查了所有可用的组织病理学手术或尸检标本。死亡的根本原因是肺气肿34例，肝硬化2例。使用各自的图像评分系统，对肺气肿切片和肝标本进行分级。评价病理异常程度与临床特征之间的相关性。所有肺均表现出重度全腺泡肺气肿（平均肺气肿评分，7.9 +/- 1.06 [标准差]，其中10代表最严重程度），下叶占优势。腺泡中心肺气肿极轻微。肺气肿的病理严重程度与肺功能指标无相关性，肺气肿的程度与肝纤维化程度无明显相关性。轻度增加的支气管腺壁比伴随轻度炎症和杯状细胞增生。小气道变化极小。膜性细支气管扩张是一个常见的发现，然而，支气管扩张较大的气道是一个轻微的特点，只有6例（15%）。气道形态学特征与慢性支气管炎或哮喘的临床表现无关。虽然肝脏和肺部病理变化之间缺乏相关性可能反映了肝脏疾病和肺部疾病的不同发病机制，但肺气肿级别与肺功能之间缺乏相关性可能反映了一系列晚期肺部疾病患者的样本偏态。(C)2004年爱思唯尔公司All rights reserved.
To assess the pathological changes in the lungs and liver of 42 individuals who died while enrolled in the Registry of Individuals with Severe Deficiency of Alpha-1 Antitrypsin (AAT), all available histopathologic surgical or postmortem-derived specimens were reviewed by the pathologist member of the Death Review Committee. The underlying cause of death was emphysema in 34 patients and cirrhosis in 2 patients. Slides of lung were graded for emphysema, and liver specimens were graded for fibrosis, using respective pictorial scoring systems. Correlations between the degree of pathological abnormality and clinical features were evaluated. All lungs exhibited severe panacinar emphysema (mean emphysema score, 7.9 +/- 1.06 [standard deviation], where 10 represents the greatest severity) with a lower lobe predominance. Centriacinar emphysema was minimal. No correlation was found between the pathological severity of emphysema and pulmonary function measurements, and no significant correlation was found between the degree of emphysema and the degree of hepatic fibrosis. Mildly increased bronchial gland-to-wall ratio accompanied mild inflammation and goblet cell hyperplasia. There were minimal changes in small airways. Dilatation of membranous bronchioles was a frequent finding; however, bronchiectasis of larger airways was a minor feature in only 6 patients (15%). Airway morphological features did not correlate with the clinical presence of chronic bronchitis or asthma. Although the lack of correlation between liver and lung pathological changes may reflect different pathogenetic mechanisms of liver disease and lung disease, the lack of correlation between emphysema grade and lung function likely reflects the skewed sample in a series of patients with advanced lung disease. (C) 2004 Elsevier Inc. All rights reserved.