Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome masquerading as juvenile idiopathic arthritis: a case series with a novel PRG4 variant and emphasis on bone health [0.03%]
以关节健康为重点的伪装成幼年特发性关节炎的卡普托屈指症-关节病-股骨颈畸形-心包炎(CACP)综合征病例系列及新颖的PRG4变异型
Mohammadreza Fathi,Kosar Chitzan-Zadeh,Mohammadamin Darbandi et al.
Mohammadreza Fathi et al.
Background: Camptodactyly-arthropathy-coxa vara-pericarditis syndrome is a rare autosomal recessive disorder caused by loss-of-function variants in the proteoglycan 4 gene, which encodes lubricin. It is frequently misdiag...
Factors associated with fatigue in children with enthesitis/spondylitis-related juvenile idiopathic arthritis: an exploratory cross-sectional study [0.03%]
与附着点炎/脊柱关节炎相关幼年特发性关节炎患儿疲劳相关的因素:一项探索性的横断面研究
Mei Zhang,Yue Xing,Xiangfeng He et al.
Mei Zhang et al.
Background: Enthesitis/spondylitis-related juvenile idiopathic arthritis (ESR-JIA) is a subtype of JIA characterized by peripheral arthritis and enthesitis, with possible axial skeleton involvement. Fatigue is a common an...
Surgical versus medical management of PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis): a systematic review and network meta-analysis [0.03%]
PFAPA(周期性发热、咽扁桃体炎、口炎、腺病)的手术治疗与药物治疗:系统评价和网络Meta分析
Mohammed Alsabri,Shree Rath,Mohamed Amr Elkarargy et al.
Mohammed Alsabri et al.
Background: Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome is the most common autoinflammatory periodic fever disorder of childhood. This study aimed to compare the effectiveness and safet...
Janus kinase inhibitors are of limited use in refractory JIA-associated uveitis: retrospective data from a tertiary uveitis center [0.03%]
jak抑制剂对难治性JIA相关葡萄膜炎的疗效有限:一家三级葡萄膜炎诊疗中心的回顾性数据分析
Karoline Baquet-Walscheid,Charlotte Wortmann,Kai Rothaus et al.
Karoline Baquet-Walscheid et al.
Background: Juvenile idiopathic arthritis-associated uveitis (JIAU) frequently follows a chronic and complicated course. Janus kinase inhibitors (JAKi) represent a promising new therapeutic option. ...
Elevated nil values in TB-IGRA: a potential diagnostic clue for pediatric histiocytic necrotizing lymphadenitis [0.03%]
结核感染T细胞斑点试验的阴性结果对儿童郎格罕组织细胞增生症淋巴结炎的诊断价值
Qing Wang,Xingyun Wang,Yu Gong et al.
Qing Wang et al.
Background: Histiocytic necrotizing lymphadenitis (HNL) is a benign condition in children that is often misdiagnosed due to its clinical overlap with tuberculosis, rheumatic diseases, and lymphadenitis. The QuantiFERON-TB...
Spectrum of MEFV variants in 8,252 patients from Eastern and Southeastern Anatolia: a large single-center cohort study (2019-2026) [0.03%]
安纳托利亚东部和东南部8252例患者中MEFV变异的光谱:一项单中心大样本队列研究(2019-2026)
Abdullah İhsan Gürler,Uğur Gümüş
Abdullah İhsan Gürler
Background: Familial Mediterranean Fever (FMF) is a monogenic autoinflammatory disorder caused by variants in MEFV. Although Türkiye carries a considerable FMF burden, large contemporary single-center genetic datasets fr...
Variation in the access and use of biologic and targeted synthetic DMARD in juvenile idiopathic arthritis: insights from a national UK survey [0.03%]
英国全国性调查发现:生物制剂和靶向合成DMARD在儿童关节炎中的应用使用存在差异
Samantha Cooray,Kalindi Rajani,Sandrine Compeyrot-Lacassagne et al.
Samantha Cooray et al.
Background: Biologic and targeted synthetic disease-modifying anti-rheumatic drugs (b/tsDMARDs) have transformed outcomes for juvenile idiopathic arthritis (JIA). However, contemporary understanding of real-world treatmen...
Two- and 3-year outcomes of the childhood arthritis and rheumatology research alliance FiRst Line Options for sJIA Treatment (FROST) trial [0.03%]
儿童关节炎和风湿病研究联盟FiRSt Line Options for sJIA治疗(FROST)试验的两年及三年结果
Timothy Hahn,George Tomlinson,Yukiko Kimura et al.
Timothy Hahn et al.
Background: Systemic juvenile idiopathic arthritis (sJIA) is a severe autoinflammatory disease associated with substantial morbidity, including macrophage activation syndrome (MAS), sJIA-associated lung disease, and gluco...
Critical care outcomes of hemophagocytic lymphohistiocytosis in children: a single centre retrospective observational study [0.03%]
儿童噬血细胞性淋巴组织细胞病重症监护结局:一项单中心回顾性观察研究
Manuel Murciano,Gabriella Bottari,Denise Pires Marafon et al.
Manuel Murciano et al.
Background: Hemophagocytic lymphohistiocytosis (HLH) is a challenging and potentially life-threatening condition characterized by an uncontrolled inflammatory response resulting from hyperactivation of T lymphocytes and m...
"Why did you choose that score?" - Associations between patient global assessment scores and mental health symptoms in patients with juvenile idiopathic arthritis and systemic lupus erythematosus [0.03%]
患者总体评估得分及其与青年特发性关节炎和系统性红斑狼疮患者精神健康症状之间的关联
Inna Powers,Sonja I Ziniel,Jeanelle Sheeder et al.
Inna Powers et al.
Background: Rheumatic disease significantly alters a child's quality of life. Rheumatologists often apply a patient global assessment score (PGA) in real time to gauge quality of life, effectiveness of treatment, and dise...