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[两兄弟患肺泡微石症]

[Pulmonary alveolar microlithiasis in 2 brothers].

作者信息

Radosavljevic G, Rebic P, Mandaric D

机构信息

Institut des Maladies pulmonaires et de la Tuberculose, Centre clinique de de Faculté de Médecine de Belgrade, Yougoslavie.

出版信息

Rev Pneumol Clin. 1988;44(4):202-4.

PMID:3217655
Abstract

Pulmonary alveolar microlithiasis is a rare disease of unknown cause and pathogenesis, where the pathological substrate is composed of calcium microconcrements inside the alveoli. The lungs are endured with deterioration of respiratory mechanics, ventilation and perfusion disorders and finally pulmonary hypertension and chronic cor pulmonale. The disease follows a slow course extending approximately over 20 years. The authors present two cases of pulmonary alveolar microlithiasis affecting two brothers aged 14 and 19 years respectively. Radiographic findings were typical and impressive. Lung changes were discovered on recruitment in the elder brother and on examination of family members in the younger brother. Both has no complaint and normal respiratory findings. The histopathological diagnosis was made from a sample obtained by bronchoscopic-parabronchial lung biopsy. This report is completed with data from the literature.

摘要

肺泡微结石症是一种病因和发病机制不明的罕见疾病,其病理基础是肺泡内的钙微结石。肺部会出现呼吸力学恶化、通气和灌注障碍,最终导致肺动脉高压和慢性肺源性心脏病。该病病程缓慢,大约持续20年。作者报告了两例肺泡微结石症病例,分别影响了一名14岁和一名19岁的兄弟。影像学表现典型且令人印象深刻。在哥哥征兵体检时以及弟弟的家庭成员检查中发现了肺部病变。两人均无不适,呼吸检查结果正常。组织病理学诊断来自经支气管旁肺活检获取的样本。本报告结合了文献数据。

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