印度儿童肝硬化:一项回顾性研究 - - 重新定义旧的神话!
Surbhi Goyal1, Akanksha Singh1, Shivanshu Gangwar1
1Pathology, GB Pant Institute of Postgraduate Medical Education and Research, Delhi, Delhi, India.
Journal of clinical pathology
|January 8, 2024
概括
印度儿童肝硬化 (ICC) 仍然被低诊断,往往呈现出欺骗性. 组织病理学是诊断儿童这种迅速致命的肝病的关键,特别是在密码性肝硬化病例中.
科学领域:
- 儿科肝病学 儿科肝病学
- 胃肠病学 胃肠病学
- 病理学 病理学 病理学
背景情况:
- 印度儿童肝硬化 (ICC) 是一种严重的肝病,曾经被认为已经根除,但仍然存在.
- 由于欺骗性的临床表现和缺乏意识,低诊断很常见.
研究的目的:
- 突出印度儿童肝硬化 (ICC) 的临床病理特征.
- 强调及时诊断和管理ICC的重要性.
- 提高临床医生对这种诊断不足的疾病的认识.
主要方法:
- 从2016年1月到2022年12月的肝脏档案组织的回顾性分析.
- 包括儿科活检病例,怀疑铜过载.
- 由三个病理学家进行的独立本病理学审查,与临床和放射学数据相关联.
主要成果:
- 五名儿童出现了黄和腹部膨胀,表明慢性肝病.
- 所有病例都显示出坚固的肝扩大,利的边缘和透氨酸炎;自身免疫/病毒/代谢反应大多是负面的.
- 气球变性,Mallory Denk体,细胞周纤维化和全球体铜沉积的组织学发现证实了ICC,不包括威尔逊病.
结论:
- 印度儿童肝硬化 (ICC) 仍然是一个重要的,尽管未被确诊的儿科肝病.
- 这种疾病迅速致死,遗传模式不确定,铜的作用受到争议.
- 组织病理学检查对于诊断ICC至关重要,特别是在密码性肝硬化病例中.
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