肺泡微石症1例

肺泡微石症（pulmonary alveolar microlithiasis)首先由Tlidveppen(1993年）报道并命名。我们在近10年的尸检标本中发现1例，现结合文献复习讨论其病变特点。     

肺泡微石症一例

患者男,43岁。因“突发胸闷10h”入院,临床诊断“右肺大泡,右自发性气胸”。患者系农民,多年来行走时微感气促,活动后加剧,曾两侧反复多次发生气胸,其父母及兄妹中未有此病症。体检：双肺听诊呼吸音降低,肝脾未触及。人院CT检查示两肺弥漫性高密度灶,胸膜下可见素描样勾勒钙化（图1）,考虑肺泡微石症伴间质纤维化,两侧气胸。实验室检查：氧分压76hamHg（1mmHg=0．133kPa）,肺泡动脉氧分压差28mmHg（〈20mmHg）。于2006年9月4日行VATS下右肺大泡烧灼术＋胸膜剥脱固定＋肺活检。     

肺泡微石症2例报告

肺泡微石症２例报告山东莱阳中心医院（２６５２００）张型竹，贺桂凤临床病例例１：女，４５岁，干部。患者６年前在一次体力劳动时发现气短，曾几次去基层医院就诊都诊为“慢性支气管炎”给予对症治疗。平日在没有发上呼吸道感染时，在室内坚持日常工作无问题。既往无职...     

一个肺泡微结石症家系中SLC34A2基因的新突变

子.此外在2例患者及先证者女儿的第2内含子发现一纯合性单核苷酸多态.结论 本家系中患者SLC34A2基因第8外显子出现新的纯合突变,提前出现终止密码子,编码截短蛋白,导致发病.所发现的第2内含子多态性意义有待深入研究.     

肺泡蛋白沉着症1例

肺泡蛋白沉着症１例陈新，王仪，唐秀如，魏霖患者男，５０岁。２个月前受凉后出现咳嗽、咳痰、气促症状。痰量少，灰白色，体温３７．５～３８℃。随着气促逐渐加剧，出现进行性呼吸困难、紫绀明显、无寒战、高热咯血。胸片示：双肺弥漫性病变。痰液细菌培养阴性。当地医...     

支气管肺泡灌洗液细胞学诊断肺泡蛋白沉着症1例

患者女性 ,43岁 ,工人。因慢性咳嗽、咳痰、进行性呼吸困难 4年余 ,加重伴低热 1年入院。患者既往有高分子粘合剂粉尘接触史 11年 ,油漆接触史 7年。胸部Ｘ线示 :两肺下野可见肺纹理增粗并连成网状 ,右下外带呈毛玻璃状改变 ,心肋膈角锐利。胸部ＣＴ示两肺中下肺野可见分布不均匀的片状毛玻璃样病灶。肺功能检查 :提示肺通气功能中度障碍。病理检查　送检支气管灌洗液标本 ,标本底部肉眼可见米黄色淤泥样沉积物。涂片镜下所见 :在纤毛柱状上皮细胞、炎细胞及少许吞噬细胞背景中 ,可见均匀粉染无结构球形小体 ,直径 2 0～ 5 0 μｍ ,球形小体…     

遗传性石骨症

石骨症属常染色体遗传的家族性骨硬化疾病 ,主要表现为骨质代谢障碍、骨纤维异常增生 ,常伴有口腔症状。本文对石骨症的临床表现、病因病理及遗传学研究进展作一综述     

家族性肺泡微结石症

家族性肺泡微结石症是肺泡内形成以钙为主要成发的小结石，病理呈慢性进行性经过。本文报告姐姝２例，姐９岁，妹７岁，临床、化验、胸片均证实，一般认为常染色体隐性遗传，２例随诊１０年，生长发育良好。     

肺泡蛋白沉积症2例报道及文献复习

肺泡蛋白沉积症（pulmonary alveolar proteinosis,PAP）是以肺泡及细支气管腔内无定形PAS染色阳性蛋白物质为特征的肺部疾病,病因不详。大多数情况下,不引起肺泡的炎症反应,且肺的纤维化极其轻微。该病的主要影响是病灶部位肺泡内的氧气向血液转运受损害,患者在静息时常常存在低氧血症。此病自1958年Rosen首次报道以来,国内亦有报道,我们报道2例并结合文献复习。     

幼儿石骨症1例并文献复习

病例 :　患儿 ,男 1岁 6个月 ,因“面色苍白 3 月 ,双下肢瘫痪 1 0天 ,“鼻衄半天”入院。入院前 3 月 ,患儿出现进行性面色苍白。 1 月前患儿全身出现针尖大小出血点 ,并有间断发热。曾就诊于本市某教学医院 ,诊治经过不详。 1 0天前患儿双下肢出现瘀点瘀斑 ,半天前发生鼻     

The occurrence of extracellular matrix vesicles in pulmonary alveolar microlithiasis

Summary Mineralization in pulmonary alveolar microlithiasis was studied by transmission electron microscopy. The disease is characterized by psammoma like calcifications composed of hydroxyapatite crystals. The calcifications were surrounded by typical forming cells and matrix composed of collagen fibers arranged in a longitudinal pattern. Abundant calcifying extracellular matrix vesicles were found between the cells and the calcified fronts. The type of calcification found in the present lesion is similar to ectopic primary mineralization in other diseases, embryonal ossification and bone wound healing.This study was supported by a grant from the Government of Israel, The Ministry of Health     

Pulmonary alveolar microlithiasis: Clinical features,evolution of the phenotype,and review of the literature

Pulmonary alveolar microlithiasis (PAM) (MIM 265100) is a rare, autosomal recessive pneumopathy characterized by intra‐alveolar formation and accumulation of tiny, roundish corpuscles called “microliths”. The name “alveolar microlithiasis” was first used by Puhr in 1933; since then, several reports have appeared, and over 300 individuals with this condition have been reported. We have reviewed the PAM cases in the literature in light of personal experience, focusing on medical implications, disease diagnosis and progression over time, familial predisposition, and geographical and sex distribution. This study confirms autosomal recessive inheritance and does not support the role of other, non‐genetic, factors in the pathogenesis of PAM. © 2002 Wiley‐Liss, Inc.     

Pulmonary alveolar microlithiasis. A new analytical approach

A case of pulmonary alveolar microlithiasis is presented which has been investigated using X-ray energy spectroscopy and microscopic infrared spectroscopy. These methods gave precise details of the chemical composition of the microliths seen in this unusual disease. The pathogenesis of this disorder is discussed in the light of these findings.     

A case of pulmonary alveolar microlithiasis

Pulmonary alveolar microlithiasis (PAM) is a rare disease with unknown etiology and pathogenesis. It is characterized by diffuse, innumerable, and minute calculi, called microlithiasis in the alveoli. More than half of reported cases are asymptomatic at the time of diagnosis. We describe the first case of PAM in Korea. A 19-yr-old man without respiratory symptoms presented with interstitial thickening on the chest radiograph. His chest high resolution CT scan showed diffusely scattered, ill defined tiny micronodules and interstitial thickening. Open lung biopsy confirmed the diagnosis of PAM. He was followed up for 6 months without treatment, and no progression was noticed.     

A case of pulmonary alveolar proteinosis treated with whole lung lavage

Pulmonary alveolar proteinosis represents a rare syndrome characterized by the accumulation of proteinaceous phospholipid-laden material in the alveoli. This leads to impaired gas exchange and arterial hypoxemia of varying degrees. The diagnosis is confirmed by lung biopsy. Sequential whole-lung lavage (WLL) first described in 1963 is the standard of care. We report a case of a male diagnosed of having pulmonary alveolar proteinosis (PAP) on transbroncial lung biopsy (TBLB). He was treated with sequential WLL (Left followed by right, Left being more involved on chest X-ray) followed by recombinant GM-CSF, with good result.     

Visualization of phosphatidylcholine (16:0/16:0) in type II alveolar epithelial cells in the human lung using imaging mass spectrometry

Imaging mass spectrometry (MS) is an emerging technique that can detect numerous biomolecular distributions in a non‐targeting manner. In the present study, we applied a mass imaging modality, mass microscopy, to human lung tissue and identified several molecules including surfactant constituents in a specific structure of the lung alveoli. Four peaks were identified using imaging MS, and the ion at m/z 772.5, in particular, was localized at some spots in the alveolar walls. Using an MS/MS analysis, the ion was identified as phosphatidylcholine (PC)(16:0/16:0), which is the main component of lung surfactant. In a larger magnification of the lung specimen, PC (16:0/16:0) was distributed in a mottled fashion in a section of the lung. Importantly, the distribution of PC (16:0/16:0) was identical to that of anti‐SLC34A2 antibody immunoreactivity, which is known to be a specific marker of type II alveolar epithelial cells, in the same section. Our experience suggests that imaging MS has excellent potential in human pathology research.