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Hemophagocytic Lymphohistiocytosis Gene Variants in Childhood-Onset SLE with Macrophage Activation Syndrome.
The Journal of Rheumatology ( IF 3.9 ) Pub Date : 2022-06-01 , DOI: 10.3899/jrheum.211200
Piya Lahiry 1 , Sergey Naumenko 2 , Madeline Couse 3 , Fangming Liao 3 , Daniela Dominguez 1 , Andrea Knight 4 , Deborah M Levy 1 , Melissa Misztal 3 , Lawrence W K Ng 1 , Linda T Hiraki 5
Affiliation  

Macrophage activation syndrome (MAS), a life-threatening complication of SLE, resembles familial hemophagocytic lymphohistiocytosis (fHLH), an inherited disorder of hyperinflammation. We compared the proportion of childhood-onset SLE (cSLE) patients with and without MAS, who carried low-frequency HLH non-synonymous variants.

中文翻译:

伴有巨噬细胞活化综合征的儿童期发病 SLE 中的噬血细胞性淋巴组织细胞增生症基因变异。

巨噬细胞活化综合征 (MAS) 是 SLE 的一种危及生命的并发症,类似于家族性噬血细胞性淋巴组织细胞增生症 (fHLH),一种遗传性过度炎症性疾病。我们比较了伴有和不伴有 MAS 的儿童期发病 SLE (cSLE) 患者的比例,这些患者携带低频 HLH 非同义变异。
更新日期:2022-06-01
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