案例报告:异常发病的多发性膜纤维缩症
Selsabil Daboussi1,2, Ben Hmida Lenda1,2, Samira Mhamedi1,2
1Department of Pneumology, Military Hospital, University of Tunis El Manar, Tunis, 1008, Tunisia.
F1000Research
|July 19, 2024
概括
异形性多发膜性纤维生化症 (IPPFE) 是一种罕见的肺部疾病. 这一案例凸显了病理学在诊断IPPFE方面的重要性,因为它往往具有误导性的放射性呈现.
科学领域:
- 肺部病理学 肺部病理学
- 病理学 病理学 病理学
- 放射学 放射学是一门学科.
背景情况:
- 异形性多发性膜纤维生化 (IPPFE) 是一种罕见的,缓慢进展的慢性肺病,影响上肺叶.
- 它的特征是多发性和亚多发性肺纤维化与弹性质.
- 首次在2013年被认可,理解和诊断仍然具有挑战性.
研究的目的:
- 报告通过病理发现诊断的IPPFE病例.
- 为了更好地了解这种罕见的肺部疾病.
- 突出诊断挑战和病理学的作用.
主要方法:
- 一个73岁的男性吸烟者的病例报告,呼吸障碍和咳恶化.
- 高分辨率计算机断层扫描 (HRCT) 显示上叶异常和肺气.
- 扫描指导的跨parietal 肺活检与组织病理学和 orcein 染色分析.
主要成果:
- 在HRCT检测中,发现了双面腹膜下腔凝结,支气管切开和腹膜收缩.
- 病理学显示重塑的肺膜与炭病,纤维化和过多的弹性纤维.
- 肺功能测试显示,患者患有可逆阻塞性呼吸系统障碍.
结论:
- 该案强调了IPPFE的非典型和误导性的放射性表现.
- 病理学结果对于确认IPPFE的诊断至关重要.
- 为了更好地理解,诊断和管理指南,需要进一步的研究.
相关概念视频
Pleural Effusion I: Introduction
828
Pleural effusion is an abnormal fluid accumulation in the pleural cavity, a narrow space between the lungs and the chest wall. It is not a disease per se but rather a symptom or indication of an underlying disease. In normal circumstances, this space contains a small amount of fluid (5 to 15 mL), a lubricant facilitating the non-frictional movement of the pleural surfaces.
There are two main types of pleural effusion: transudative and exudative. They are differentiated using Light's...
There are two main types of pleural effusion: transudative and exudative. They are differentiated using Light's...
828
Pleural Effusion II: Symptoms and Management
162
Pleural Effusion Overview
A pleural effusion is the abnormal collection of fluid between the parietal and visceral pleura layers of tissue that form the lining of the lungs and chest cavity. It can occur independently or due to surrounding parenchymal diseases, such as infection, malignancy, or inflammatory conditions.
Clinical Manifestations:
A pleural effusion is the abnormal collection of fluid between the parietal and visceral pleura layers of tissue that form the lining of the lungs and chest cavity. It can occur independently or due to surrounding parenchymal diseases, such as infection, malignancy, or inflammatory conditions.
Clinical Manifestations:
162
Introduction to Fibroblasts
3.1K
Rudolph Virchow discovered spindle-shaped cells called fibroblasts in 1858. Inactive fibroblasts, called fibrocytes, become activated by various stimuli, such as growth factors and inflammatory cytokines. Activated fibroblasts play a crucial role in wound healing, inflammation, formation of new blood vessels, and cancer progression. Uncontrolled activation of fibroblasts results in fibrosis, the excess deposition of fibrous tissue, which can lead to scarring and affect normal organs. This...
3.1K
Pleural Disorders: Types and Brief Description
196
The pleura is a vital part of the respiratory system. It's a double-layered membrane surrounding the lungs and lining the chest cavity. The two layers of the pleura are:
196
Pleura of the Lungs
1.7K
The lungs are nestled in a cavity, shielded by the pleura. The pleura, a form of serous membrane, wraps around each lung. This membrane arrangement consists of two layers: the visceral and parietal pleurae. The visceral pleura lines the surface of the lungIn contrast, the parietal pleura is the outer layer and contacts to the thoracic wall, the mediastinum, and the diaphragm. The hilum is the point of connection between the visceral and parietal layers. The space between the parietal and...
1.7K


