当前位置: X-MOL 学术Indian J. Pediatr. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Juvenile Scleroderma
The Indian Journal of Pediatrics ( IF 4.3 ) Pub Date : 2023-11-15 , DOI: 10.1007/s12098-023-04894-1
Aruna Bhat 1
Affiliation  

Localised scleroderma and systemic sclerosis are rare chronic fibrosing disorders seen in children, and are collectively referred to as Juvenile Scleroderma. Histopathology of the two forms is non-distinct but they differ in terms of vasculopathy, internal organ involvement, morbidity and mortality. Raynaud’s phenomenon with digital tip ulcers is considered hallmark of systemic sclerosis. Quality of life gets greatly affected by these diseases. Early identification in the inflammatory phase of the disease, effective treatment and strict surveillance remain crucial for better outcomes. Emerging vascular and immunosuppressive strategies, coupled with efforts from scientific community to develop better biomarkers and monitoring tools, help constantly to improve survival rates.



中文翻译:

幼年型硬皮病

局限性硬皮病和系统性硬化症是儿童中罕见的慢性纤维化疾病,统称为青少年硬皮病。两种形式的组织病理学并无区别,但它们在血管病变、内脏器官受累、发病率和死亡率方面有所不同。伴有指端溃疡的雷诺现象被认为是系统性硬化症的标志。这些疾病极大地影响了生活质量。疾病炎症阶段的早期识别、有效治疗和严格监测对于获得更好的结果仍然至关重要。新兴的血管和免疫抑制策略,加上科学界开发更好的生物标志物和监测工具的努力,有助于不断提高生存率。

更新日期:2023-11-15
down
wechat
bug