Pediatric Rheumatology最新文献_第10页

Bone health and physical activity in adolescents with juvenile idiopathic arthritis: a cross-sectional case-control study 患有幼年特发性关节炎的青少年的骨骼健康和体育锻炼：一项横断面病例对照研究

IF 2.5 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology

Pub Date : 2024-04-19 DOI: 10.1186/s12969-024-00982-4

Egi Vasil, Colleen M. Nesbitt, Clodagh Toomey, Gregor Kuntze, Shane Esau, Carolyn A. Emery, Leigh Gabel

Adolescents with juvenile idiopathic arthritis (JIA) tend to engage in less physical activity than their typically developing peers. Physical activity is essential for bone development and reduced physical activity may detrimentally effect bone health. Thus, we examined differences in total body bone mineral content (BMC) and areal bone mineral density (aBMD) between adolescents with JIA and adolescent controls without JIA. We also examined associations between moderate-to-vigorous physical activity (MVPA), lean mass, and bone outcomes. Participants included 21 adolescents with JIA (14 females, 7 males) and 21 sex- and age-matched controls aged 10–20 years. Assessments included: height; weight; triple-single-leg-hop distance (TSLH); MVPA by accelerometry; and total body BMC, aBMD, and lean mass measured using dual X-ray absorptiometry. Height-adjusted z-scores were calculated for BMC and aBMD and used for all analyses. Multiple linear mixed effects models examined group differences in BMC and aBMD, adjusting for sex, maturity, MVPA, TSLH, and lean mass. Participants clusters, based on sex and age (within 18 months), were considered random effects. Adolescents with JIA had lower total body aBMD z-scores [β (95% CI); -0.58 (-1.10 to -0.07), p = 0.03] and BMC z-scores [-0.47 (-0.91 to -0.03), p = 0.04] compared with controls. Mean daily MVPA was 22.0 min/day lower in adolescents with JIA than controls; however, MVPA was not associated with aBMD [-0.01 (-0.01 to 0.01), p = 0.32] or BMC [0.00 (-0.01 to 0.00), p = 0.39]. Lean mass was positively associated with aBMD [0.05 (0.01 to 0.09) g/cm2, p = 0.03] and BMC [0.06 (0.03 to 0.10) g, p < 0.001]. Adolescents with JIA had lower total body aBMD and BMC compared with sex- and age-matched controls without JIA. Group differences in bone outcomes were not associated with the lower MVPA participation of adolescents with JIA. Despite this, physical activity should still be encouraged as it promotes physical well-being.

与发育正常的同龄人相比，患有幼年特发性关节炎（JIA）的青少年往往运动量较少。体力活动对骨骼发育至关重要，减少体力活动可能会对骨骼健康造成不利影响。因此，我们研究了患有 JIA 的青少年与未患有 JIA 的青少年对照组在全身骨矿物质含量（BMC）和骨矿物质密度（aBMD）方面的差异。我们还研究了中到剧烈运动（MVPA）、瘦体重和骨骼结果之间的关联。参与者包括 21 名患有 JIA 的青少年（14 名女性，7 名男性）和 21 名性别和年龄匹配的 10-20 岁对照组青少年。评估项目包括：身高、体重、三单腿跳跃距离（TSLH）、加速度测量法测量的MVPA、双X射线吸收测量法测量的全身BMC、aBMD和瘦体重。在所有分析中，都计算了BMC和aBMD的身高调整z分数。多重线性混合效应模型检验了 BMC 和 aBMD 的组间差异，并对性别、成熟度、MVPA、TSLH 和瘦体重进行了调整。基于性别和年龄（18 个月以内）的参与者分组被视为随机效应。与对照组相比，患有 JIA 的青少年全身 aBMD z 评分较低 [β（95% CI）；-0.58（-1.10 至 -0.07），p = 0.03]，BMC z 评分较低 [-0.47（-0.91 至 -0.03），p = 0.04]。与对照组相比，JIA 青少年的平均每日 MVPA 低 22.0 分钟/天；但是，MVPA 与 aBMD [-0.01 (-0.01 to 0.01)，p = 0.32] 或 BMC [0.00 (-0.01 to 0.00)，p = 0.39] 无关。瘦体重与 aBMD [0.05 (0.01 to 0.09) g/cm2, p = 0.03] 和 BMC [0.06 (0.03 to 0.10) g, p < 0.001] 呈正相关。与无 JIA 的性别和年龄匹配对照组相比，患有 JIA 的青少年全身 aBMD 和 BMC 较低。骨骼结果的群体差异与患有 JIA 的青少年较少参加 MVPA 无关。尽管如此，仍应鼓励体育锻炼，因为体育锻炼能促进身体健康。

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引用次数: 0

Risk perception, well-being, depression and anxiety in children and adolescents with rheumatic diseases during the COVID-19 pandemic - results from the prospective multicenter KICK-COVID study in Germany COVID-19 大流行期间患有风湿病的儿童和青少年的风险意识、幸福感、抑郁和焦虑--德国 KICK-COVID 前瞻性多中心研究的结果

IF 2.5 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology

Pub Date : 2024-04-18 DOI: 10.1186/s12969-024-00979-z

Claudia Sengler, Jens Klotsche, Malthe Jessen Pedersen, Martina Niewerth, Julia Göldel, Daniel Windschall, Johannes-Peter Haas, Frank Dressler, Ralf Trauzeddel, Anton Hospach, Frank Weller-Heinemann, Stefanie Lanzinger, Clemens Kamrath, Reinhard W. Holl, Petra Warschburger, Kirsten Minden

To investigate the psychosocial burden in children and adolescents with juvenile rheumatic diseases during the COVID-19 pandemic. As part of the multicentre observational KICK-COVID study linked to the National Pediatric Rheumatology Database, adolescents < 21 years and parents of children < 12 years with rheumatic diseases answered questions on perceptions of health risk (PHR) due to SARS-CoV2, stress, well-being (WHO-5) and symptoms of depression (PHQ-9) and anxiety (GAD-7). Data were collected at routine visits from June to December 2021 and assessed for association with demographic and clinical parameters, treatment and patient-reported outcomes by multivariable regression analyses. Data from 1356 individuals (69% female, 50% adolescents) were included. Median PHR on a numeric rating scale (NRS, 0–10) was 4 (IQR 2–6), median perceived stress was 3 (IQR 1–6). Adolescents reported a worse well-being with a significantly lower median WHO-5-score (60, IQR 40–76) than parents reported for their children < 12 years (80, IQR 68–84). Moderate to severe symptoms of depression and anxiety were reported by 14.3% and 12.3% of the adolescents, respectively. PHR was significantly higher in patients with systemic lupus erythematosus, methotrexate or biologic disease-modifying anti-rheumatic drug therapy than in patients without these characteristics, whereas lower WHO-5 or higher PHQ-9 or GAD-7 scores were only associated with poorer patient-reported health status and physical functioning. The perception of health risk due to SARS-CoV2 infection was not paralleled by an impairment of mental health, which were, however, significantly correlated with self-rated health status and functional capacity, highlighting the importance of patient-reported outcome assessment. German Clinical Trials Register (DRKS), no. DRKS00027974. Registered on 27th of January 2022.

调查COVID-19大流行期间患有幼年风湿病的儿童和青少年的社会心理负担。作为与全国儿科风湿病学数据库相连接的多中心观察性 KICK-COVID 研究的一部分，年龄小于 21 岁的青少年和年龄小于 12 岁的风湿病患儿的父母回答了有关 SARS-CoV2 导致的健康风险认知（PHR）、压力、幸福感（WHO-5）以及抑郁症状（PHQ-9）和焦虑症状（GAD-7）的问题。这些数据是在 2021 年 6 月至 12 月的例行就诊中收集的，并通过多变量回归分析评估了这些数据与人口统计学和临床参数、治疗和患者报告结果之间的关联。研究纳入了 1356 人（69% 为女性，50% 为青少年）的数据。按数字评分量表（NRS，0-10）计算的患者健康状况报告中位数为 4（IQR 2-6），感知压力中位数为 3（IQR 1-6）。青少年报告的健康状况较差，WHO-5 评分中位数（60，IQR 40-76）明显低于家长报告的 12 岁以下儿童的健康状况（80，IQR 68-84）。分别有 14.3% 和 12.3% 的青少年出现中度至重度抑郁和焦虑症状。接受过系统性红斑狼疮、甲氨蝶呤或生物改良抗风湿药物治疗的患者的PHR明显高于未接受过这些治疗的患者，而较低的WHO-5或较高的PHQ-9或GAD-7评分仅与患者报告的较差的健康状况和身体功能有关。SARS-CoV2感染导致的健康风险感知与心理健康受损并不同步，但心理健康受损与自评健康状况和功能能力显著相关，这凸显了患者报告结果评估的重要性。德国临床试验注册中心（DRKS），编号：DRKS00027974。DRKS00027974.注册日期：2022 年 1 月 27 日。

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引用次数: 0

Kawasaki disease in Kenya and review of the African literature 肯尼亚的川崎病和非洲文献综述

IF 2.5 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology

Pub Date : 2024-04-14 DOI: 10.1186/s12969-024-00977-1

A. Migowa, CM. Njeru, E. Were, T. Ngwiri, I. Colmegna, C. Hitchon, R. Scuccimarri

Kawasaki disease has been described across the globe, although publications from Africa are limited. To our knowledge, there are no publications on Kawasaki disease from Kenya, which triggered this report. A retrospective cross-sectional study was undertaken to identify in-patients with a discharge diagnosis of Kawasaki disease, over 2 different 5-year periods, at two pediatric hospitals in Nairobi, Kenya. We reviewed the medical records of all patients and report their clinical findings, diagnostic workup and treatment. In addition, we undertook a detailed review of the literature. Twenty-three patients with Kawasaki disease were identified, of those 12 (52.2%) had incomplete disease. The mean age was 2.3 years (SD+/-2.2) (range 0.3–10.3) with a male to female ratio of 1:1. The mean duration of fever at diagnosis was 8.3 days (SD+/-4.7) (range 2–20). Oral changes were the most common clinical feature and conjunctivitis the least common. Thrombocytosis at diagnosis was seen in 52% (12/23). Twenty-one patients (91.3%) were treated with intravenous immunoglobulin and all except 1 received aspirin. Baseline echocardiograms were performed in 95.7% (22/23) and found to be abnormal in 3 (13.6%). Follow-up data was limited. Our literature review identified 79 publications with documented cases of Kawasaki disease in children from 22 countries across the African continent with a total of 1115 patients including those from this report. Only 153 reported cases, or 13.7%, are from sub-Saharan Africa. This is the first publication on Kawasaki disease from Kenya and one of the largest reports from sub-Saharan Africa. It is the first to have a complete review of the number of published cases from the African continent. Challenges in the diagnosis and management of Kawasaki disease in many African countries include disease awareness, infectious confounders, access and cost of intravenous immunoglobulin, access to pediatric echocardiography and follow-up. Increasing awareness and health care resources are important for improving outcomes of Kawasaki disease in Africa.

全球各地都有关于川崎病的描述，但来自非洲的出版物非常有限。据我们所知，肯尼亚没有关于川崎病的出版物，因此引发了本报告。我们开展了一项回顾性横断面研究，以确定肯尼亚内罗毕两家儿科医院在两个不同的 5 年期间出院诊断为川崎病的住院病人。我们查阅了所有患者的病历，并报告了他们的临床发现、诊断工作和治疗情况。此外，我们还详细查阅了相关文献。共发现 23 名川崎病患者，其中 12 人（52.2%）病情不完全。患者平均年龄为 2.3 岁（SD+/-2.2）（范围为 0.3-10.3），男女比例为 1:1。确诊时的平均发热时间为 8.3 天（SD+/-4.7）（范围为 2-20）。口腔变化是最常见的临床特征，结膜炎是最不常见的临床特征。52%的患者（12/23）在确诊时出现血小板减少。21名患者（91.3%）接受了静脉注射免疫球蛋白治疗，除1名患者外，其余患者均接受了阿司匹林治疗。95.7%的患者（22/23）接受了基线超声心动图检查，其中3例（13.6%）发现异常。随访数据有限。我们在文献综述中发现了79篇有川崎病儿童病例记录的文献，这些病例来自非洲大陆的22个国家，包括本报告在内共有1115名患者。撒哈拉以南非洲地区的报告病例仅有 153 例，占 13.7%。这是第一份关于肯尼亚川崎病的出版物，也是撒哈拉以南非洲最大的报告之一。这也是首次对非洲大陆已发表病例数量的完整回顾。许多非洲国家在川崎病的诊断和管理方面面临的挑战包括对疾病的认识、感染性混杂因素、静脉注射免疫球蛋白的获取途径和成本、儿科超声心动图检查和随访。提高认识和增加医疗资源对于改善非洲川崎病的治疗效果非常重要。

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引用次数: 0

C-reactive protein to albumin ratio as a prognostic tool for predicting intravenous immunoglobulin resistance in children with kawasaki disease: a systematic review of cohort studies 将 C 反应蛋白与白蛋白比值作为预测川崎病儿童静脉注射免疫球蛋白耐受性的预后工具：队列研究的系统性回顾

IF 2.5 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology

Pub Date : 2024-04-12 DOI: 10.1186/s12969-024-00980-6

Jue Liu, Xingguang Chen, Minling Yang, Fangfang Shen, Feng Zhu, Jian Jin, Yiqun Teng

Intravenous immunoglobulin (IVIG) is the primary treatment for Kawasaki disease (KD). However, 10–20% of KD patients show no response to IVIG treatment, making the early prediction of IVIG resistance a key focus of KD research. Our aim is to explore the application of the C-reactive protein to albumin ratio (CAR) for predicting IVIG resistance in children with KD through meta-analysis. Cochrane Library, PubMed, MEDLINE, EMbase, CNKI, WanFang, the Chinese Biomedical Database, and CQVIP were searched up to November 2023 for cohort studies on predicting IVIG-resistant KD using the CAR. Articles were selected based on pre-established inclusion and exclusion criteria after extracting literature data and assessing them using the QUADAS-2.0 tool for evaluating the accuracy of diagnostic tests. Stata 15.0 software was used for meta-analysis. Four Chinese and English literature reports were included in this meta-analysis. The results revealed the presence of a threshold effect and high heterogeneity among the included studies. The combined sensitivity for CAR predicting IVIG-resistant KD was calculated as 0.65 (95% CI 0.58–0.72), specificity as 0.71 (95% CI 0.57–0.81), and the area under the curve (AUC) as 0.70 (95% CI 0.66–0.74) using the random-effects model. The combined positive likelihood ratio was 2.22 (95% CI 1.35–3.65), the combined negative likelihood ratio was 0.49 (95% CI 0.35–0.69), and the diagnostic odds ratio was 5 (95% CI 2–10). CAR is an auxiliary predictive indicator with moderate diagnostic value that provides guidance in the early treatment of the disease, demonstrating a certain predictive value that warrants further investigation. However, CAR cannot yet be considered as a definitive diagnostic or exclusionary marker for IVIG-resistant KD. Therefore, multi-center, large sample, and high-quality long-term follow-up trials are warranted to confirm the current findings.

静脉注射免疫球蛋白（IVIG）是治疗川崎病（KD）的主要方法。然而，10%-20% 的 KD 患者对 IVIG 治疗无反应，因此早期预测 IVIG 抗药性成为 KD 研究的重点。我们的目的是通过荟萃分析，探讨如何应用 C 反应蛋白与白蛋白的比值（CAR）来预测 KD 儿童对 IVIG 的耐药性。截至 2023 年 11 月，我们在 Cochrane Library、PubMed、MEDLINE、EMbase、CNKI、万方数据库、中国生物医学数据库和 CQVIP 中检索了使用 CAR 预测 IVIG 耐药 KD 的队列研究。在提取文献数据并使用用于评估诊断测试准确性的 QUADAS-2.0 工具对其进行评估后，根据预先确定的纳入和排除标准对文章进行筛选。使用 Stata 15.0 软件进行荟萃分析。本次荟萃分析共纳入了四篇中英文文献报告。结果显示，纳入的研究存在阈值效应和高度异质性。采用随机效应模型计算得出，CAR 预测 IVIG 耐药 KD 的综合灵敏度为 0.65（95% CI 0.58-0.72），特异度为 0.71（95% CI 0.57-0.81），曲线下面积（AUC）为 0.70（95% CI 0.66-0.74）。综合阳性似然比为 2.22（95% CI 1.35-3.65），综合阴性似然比为 0.49（95% CI 0.35-0.69），诊断几率比为 5（95% CI 2-10）。CAR是一个具有中等诊断价值的辅助预测指标，可为疾病的早期治疗提供指导，显示出一定的预测价值，值得进一步研究。然而，CAR 尚不能被视为 IVIG 耐药 KD 的明确诊断或排除标志物。因此，需要进行多中心、大样本和高质量的长期随访试验来证实目前的研究结果。

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引用次数: 0

Provider assessment of the temporomandibular joint in Juvenile idiopathic arthritis: a retrospective analysis from the CARRA database 幼年特发性关节炎患者颞下颌关节的医护人员评估：CARRA 数据库的回顾性分析

IF 2.5 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology

Pub Date : 2024-04-08 DOI: 10.1186/s12969-024-00968-2

Anna Costello, Marinka Twilt, Melissa A. Lerman

Temporomandibular joint (TMJ) involvement is an often underrecognized complication of juvenile idiopathic arthritis (JIA) that can cause decreased mandibular growth, altered facial morphology, and orofacial pain. It is estimated that the TMJ is affected in 30–45% of children with JIA. Standardized physical examination and imaging evaluations are important in accurately assessing active TMJ arthritis and sequalae. Little is known about the rate at which providers evaluate TMJ involvement in their clinical practice. Data were obtained from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. Data fields related to assessment for TMJ arthritis were added in 2019. Patients were included in the study if they had a diagnosis of JIA and had data recorded between January 2020 and August 2021. Standard descriptive statistics were used to describe demographic and clinical features. A total of 17,761 visits were reviewed for a total of 7473 patients with JIA. A total of 52.7% of patients had maximal mouth opening (MMO) recorded as finger breadths or total incisal distance (TID). Only 8% had TID measured. A total of 5.0% had MRI with contrast performed. A total of 939 patients had a diagnosis of TMJ arthritis. Of these, 28.5% had an MRI documented, 83% had an MMO documented, and 40% had TID measured. Few patient-level characteristics were statistically related to having MMO assessed. MRI was more likely to be obtained in older and in female patients. MMO was recorded at a given visit > 80% of the time at 17 sites, and it was recorded < 1% of the time at 8 sites. MRIs were infrequently performed at all sites, with 27 sites having no MRIs obtained and only 7 sites having an MRI obtained at > 10% of visits. MMO is not consistently measured in patients with JIA, and it is rarely measured quantitatively. Similarly, TMJ MRIs are rarely obtained in patients with JIA. Site of care is more associated with TMJ assessments than patient-level characteristics. These data suggest that provider education is needed to improve the assessment of the TMJ in patients with JIA to enable earlier recognition and prevent long-term complications.

颞下颌关节（TMJ）受累是幼年特发性关节炎（JIA）的一种并发症，通常未得到充分认识，它可导致下颌骨发育不良、面部形态改变和口面部疼痛。据估计，30-45% 的 JIA 患儿的颞下颌关节会受到影响。标准化的体格检查和影像学评估对于准确评估活动性颞下颌关节炎和后遗症非常重要。关于医疗机构在临床实践中对颞下颌关节受累情况的评估率，人们知之甚少。数据来自儿童关节炎和风湿病学研究联盟（CARRA）登记处。2019 年新增了与颞下颌关节炎评估相关的数据字段。如果患者确诊为 JIA，且在 2020 年 1 月至 2021 年 8 月期间有数据记录，则将其纳入研究范围。标准描述性统计用于描述人口统计学和临床特征。共对 7473 名 JIA 患者的 17761 次就诊进行了审查。共有 52.7% 的患者的最大张口度 (MMO) 被记录为指宽或总切口距离 (TID)。只有 8%的患者测量了总切口距离。共有 5.0% 的患者进行了带造影剂的核磁共振成像检查。共有 939 名患者被诊断为颞下颌关节炎。其中，28.5% 的患者有 MRI 记录，83% 的患者有 MMO 记录，40% 的患者测量了 TID。从统计学角度看，很少有患者的特征与 MMO 评估有关。年龄较大的患者和女性患者更有可能接受磁共振成像检查。在17个研究机构中，80%以上的患者在某次就诊时记录了MMO，10%的患者在就诊时记录了MMO。MMO在JIA患者中的测量并不一致，也很少进行定量测量。同样，JIA 患者也很少进行颞下颌关节 MRI 检查。就诊地点与颞下颌关节评估的相关性要高于患者层面的特征。这些数据表明，需要对医疗服务提供者进行教育，以改进对 JIA 患者颞下颌关节的评估，从而尽早识别并预防长期并发症。

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引用次数: 0

Exploring paediatric rheumatology care: a ten-year retrospective analysis of the patient population in Ghana. 探索儿科风湿病护理：对加纳患者群体的十年回顾性分析。

IF 2.5 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology

Pub Date : 2024-03-21 DOI: 10.1186/s12969-024-00975-3

Dzifa Dey, Bright Katso, Afia Baah, Saudatu Isaaka, Emmanuella Amoako

Background: Rheumatic diseases can seriously impact children's general health, development, and growth. However, due to a lack of resources, paediatric rheumatology is a largely underdeveloped speciality in many African nations. Children with rheumatic disorders face obstacles in accessing specialized medical care, including lack of specialists, care centres, medication access, and limited research and education to increase understanding of paediatric rheumatic disease among healthcare practitioners. This study described the disease characteristics, prevalence, and challenges faced by paediatric rheumatic disease patients receiving care at a teaching hospital in Accra, Ghana.

Methods: A retrospective record-based study was conducted among all paediatric cases presenting to the rheumatology clinic of the Korle Bu Teaching Hospital (KBTH) from January 2011 to December 2021. Data collected include clinical features, laboratory findings at disease presentation, andtherapeutic regimens prescribed per standard guidelines and experiences.

Results: A total of 121 cases were identified as of 2021, indicating a point prevalence of 0.0011%. The majority (73%) were females with a mean age of 13.4 ± 3.2 years. The mean duration of symptoms in months experienced by patients before being successfully referred to a rheumatologist was 18 months. There were significant differences between referred and confirmed diagnoses, especially in cases involving mixed connective tissue diseases (MCTD), systemic lupus erythematosus (SLE), and juvenile dermatomyositis (JDM), suggesting that these conditions may be under-recognised. Arthralgia and arthritis were the most common presenting symptoms. More than three-quarters (86.8%) of the cases studied were treated with steroids (oral or intravenous). In cases requiring immunosuppressive therapy, methotrexate was the most commonly prescribed in 33.9% of instances. Mortality was recorded at 8.3%, with the majority involving SLE cases. Most (95.7%) of the primary caregivers expressed positive experiences regarding care received at the adult rheumatology clinic.

Conclusion: There were significant delays in diagnosis and diagnostic accuracy for patients with paediatric rheumatic disease (PRD). This highlights the pressing need for strengthening paediatric rheumatology services in Africa, including increasing awareness about these conditions among the public and healthcare providers to improve early diagnosis and quality of life for children with these conditions.

背景：风湿病会严重影响儿童的总体健康、发育和成长。然而，由于缺乏资源，在许多非洲国家，儿科风湿病在很大程度上还是一门欠发达的专科。患有风湿性疾病的儿童在获得专业医疗护理方面面临重重障碍，包括缺乏专科医生、护理中心和药物，以及为提高医疗从业人员对儿科风湿病的认识而开展的研究和教育有限。本研究描述了在加纳阿克拉一家教学医院接受治疗的儿童风湿病患者的疾病特征、患病率和面临的挑战：研究对 2011 年 1 月至 2021 年 12 月期间在科勒布教学医院（KBTH）风湿病诊所就诊的所有儿科病例进行了回顾性记录研究。收集的数据包括临床特征、发病时的实验室检查结果以及根据标准指南和经验开出的治疗方案：结果：截至 2021 年，共发现 121 例病例，发病率为 0.0011%。大多数病例（73%）为女性，平均年龄为（13.4 ± 3.2）岁。在成功转诊至风湿免疫科之前，患者的平均症状持续时间为 18 个月。转诊病例与确诊病例之间存在明显差异，尤其是在涉及混合性结缔组织病（MCTD）、系统性红斑狼疮（SLE）和幼年皮肌炎（JDM）的病例中，这表明这些疾病可能未得到充分认识。关节痛和关节炎是最常见的症状。超过四分之三（86.8%）的病例接受了类固醇（口服或静脉注射）治疗。在需要接受免疫抑制治疗的病例中，甲氨蝶呤是最常用的处方药，占 33.9%。死亡率为8.3%，其中大部分为系统性红斑狼疮病例。大多数（95.7%）主要护理人员对在成人风湿病诊所接受的护理表示肯定：结论：儿科风湿病（PRD）患者的诊断和诊断准确性严重滞后。这凸显了加强非洲儿科风湿病服务的迫切需要，包括提高公众和医疗服务提供者对这些疾病的认识，以改善这些疾病的早期诊断和患儿的生活质量。

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引用次数: 0

Level and correlates of physical activity among children and adolescents with juvenile idiopathic arthritis compared to controls: results from a German nationwide prospective observational cohort study 与对照组相比，患有幼年特发性关节炎的儿童和青少年的体育锻炼水平及相关因素：一项德国全国性前瞻性观察队列研究的结果

IF 2.5 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology

Pub Date : 2024-03-20 DOI: 10.1186/s12969-024-00976-2

Florian Milatz, Sandra Hansmann, Jens Klotsche, Martina Niewerth, Tilmann Kallinich, Frank Dressler, Johannes-Peter Haas, Rainer Berendes, Gerd Horneff, Markus Hufnagel, Frank Weller-Heinemann, Daniel Windschall, Ralf Trauzeddel, Moritz Klaas, Hermann Girschick, Prasad T. Oommen, Ivan Foeldvari, Serdar Mustafa Cantez, Annette F. Jansson, Matthias Hartmann, Joachim Peitz-Kornbrust, Kirsten Minden

Physical active lifestyles are essential throughout growth and maturation and may offer potential preventive and therapeutic benefit in patients with juvenile idiopathic arthritis (JIA). Insufficient physical activity (PA), in contrast, can lead to aggravation of disease-related symptoms. This study aimed to i) examine PA levels in children and adolescents with JIA compared to general population controls and ii) investigate correlates of pronounced physical inactivity in order to identify risk groups for sedentary behaviour. Data from children and adolescents with JIA and population controls aged 3 to 17 years documented in the National Pediatric Rheumatologic Database (NPRD) and the German Health Interview and Examination Survey for Children and Adolescents (KiGGS) were used. Self-reported PA was collected from parents/guardians of children up to 11 years of age or adolescents 12 years of age and older. To compare PA-related data, age- and sex-specific pairwise analyses were conducted considering NPRD/KiGGS participants' data from 2017. Correlates of physical inactivity among patients were identified using a linear regression model. Data of 6,297 matched-pairs (mean age 11.2 ± 4.2 years, female 67%, patients’ disease duration 4.5 ± 3.7 years, persistent oligoarthritis 43%) were available for evaluation. Almost 36% of patients aged 3–17 years (vs. 20% of controls) achieved the WHO recommended amount of PA, while PA steadily decreased with age (18% of patients aged ≥ 12 years) and varied between JIA categories. Female adolescents and patients with enthesitis-related arthritis were least likely to achieve the minimum recommended level of PA. Physical inactivity was associated with female sex, higher age at disease onset, longer disease duration, more functional disability (C-HAQ) and higher disease activity (cJADAS-10). Depending on JIA category, children and adolescents with JIA were similarly or even more likely to achieve the WHO recommended minimum level of PA compared to general population controls. However, since a large proportion of young JIA patients appear to be insufficiently physically active, engagement in targeted efforts to promote PA is urgently needed.

积极锻炼身体的生活方式在整个生长和成熟过程中都是必不可少的，它可能对幼年特发性关节炎（JIA）患者起到潜在的预防和治疗作用。相反，体力活动（PA）不足会导致疾病相关症状加重。本研究旨在 i) 与普通人群对照组相比，检查患有幼年特发性关节炎的儿童和青少年的体力活动水平；ii) 调查明显缺乏体力活动的相关因素，以确定久坐行为的风险群体。研究使用了国家儿科风湿病数据库（NPRD）和德国儿童和青少年健康访谈与检查调查（KiGGS）中记录的 3 至 17 岁 JIA 儿童和青少年以及人群对照的数据。11 岁以下儿童或 12 岁及以上青少年的父母/监护人对自我报告的 PA 进行了收集。为了比较与运动量相关的数据，考虑到 NPRD/KiGGS 参与者 2017 年的数据，我们进行了特定年龄和性别的配对分析。采用线性回归模型确定了患者缺乏运动的相关因素。6297对配对数据（平均年龄（11.2 ± 4.2）岁，女性占67%，患者病程（4.5 ± 3.7）年，持续性少关节炎占43%）可供评估。近36%的3-17岁患者（对照组为20%）达到了世界卫生组织推荐的运动量，而运动量则随着年龄的增长而逐渐减少（≥12岁的患者为18%），不同JIA类别的患者运动量也不尽相同。女性青少年和与关节炎相关的关节炎患者达到最低建议运动量的可能性最小。缺乏运动与女性性别、发病年龄较高、病程较长、功能性残疾（C-HAQ）较多和疾病活动度（cJADAS-10）较高有关。根据 JIA 的类别，与普通人群对照组相比，患有 JIA 的儿童和青少年达到世界卫生组织建议的最低活动量水平的可能性相似，甚至更高。然而，由于很大一部分年轻的 JIA 患者似乎缺乏足够的体育锻炼，因此迫切需要采取有针对性的措施来促进体育锻炼。

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引用次数: 0

Efficacy and safety of canakinumab in systemic juvenile idiopathic arthritis, the first Chinese experience 卡那单抗治疗系统性幼年特发性关节炎的疗效和安全性--中国首次应用经验

IF 2.5 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology

Pub Date : 2024-03-19 DOI: 10.1186/s12969-024-00974-4

Lingzhi Qiu, Le Ma, Yifan Xie, Jing Jin, Yuting Pan, Shumin Li, Zhidan Fan, Haiguo Yu

Systemic juvenile idiopathic arthritis (sJIA) is a severe form of juvenile arthritis that is characterized by chronic joint inflammation and systemic symptoms such as fever, rash, and organ involvement. Anti-IL-6 receptor monoclonal antibody tocilizumab is an effective treatment. However, some patients still experience persisting or recurrent symptoms and the real-world effectiveness of canakinumab in Chinese patients with sJIA has never been reported. Therefore, this study aimed to assess the efficacy and safety of canakinumab in Chinese patients with sJIA using real-world data. We conducted a retrospective study on children with active sJIA. Clinical features, laboratory data, corticosteroid dosage, and adverse events (AEs) were collected at baseline and at 4, 8, 12, and 24 weeks after initiating canakinumab treatment. Seven female and four male patients were included in the study. All patients had previously been treated with tocilizumab and were administered canakinumab for 12.4 ± 3.4 months. Notably, significant improvements were observed in both clinical signs and symptoms as well as laboratory indicators. Four children under corticosteroid treatment were able to successfully discontinue their corticosteroid therapy: one at week 4, two at week 12, and one at week 24. Notably, there was a significant reduction in the number of tender and swollen joints (P = 0.0059) as well as the systemic juvenile arthritis disease activity score (P < 0.0001). The most common AE was infection, but no patients experienced serious AEs. No cases of macrophage activation syndrome or death were reported during the follow-up period. Canakinumab was found to be potentially efficacious and safe in Chinese patients with sJIA. No new AEs were observed with canakinumab treatment.

全身性幼年特发性关节炎（sJIA）是一种严重的幼年关节炎，其特点是慢性关节炎症和全身症状，如发热、皮疹和器官受累。抗IL-6受体单克隆抗体托西珠单抗是一种有效的治疗方法。然而，部分患者仍会出现持续或反复发作的症状，而卡那珠单抗在中国sJIA患者中的实际疗效尚未见报道。因此，本研究旨在利用真实世界的数据评估卡那珠单抗在中国sJIA患者中的疗效和安全性。我们对活动性sJIA患儿进行了一项回顾性研究。我们在基线和开始卡那单抗治疗后的 4、8、12 和 24 周收集了临床特征、实验室数据、皮质类固醇用量和不良事件（AEs）。研究共纳入了七名女性患者和四名男性患者。所有患者之前都接受过托西珠单抗治疗，卡那单抗的疗程为（12.4 ± 3.4）个月。值得注意的是，临床症状和体征以及实验室指标均有明显改善。四名接受皮质类固醇治疗的患儿成功地停止了皮质类固醇治疗：一名患儿在第4周停止治疗，两名患儿在第12周停止治疗，一名患儿在第24周停止治疗。值得注意的是，压痛和肿胀关节的数量（P = 0.0059）以及全身性幼年关节炎疾病活动度评分（P < 0.0001）均明显减少。最常见的不良反应是感染，但没有患者出现严重不良反应。随访期间没有出现巨噬细胞活化综合征或死亡病例。卡纳库单抗对中国sJIA患者具有潜在疗效和安全性。卡纳库单抗治疗未发现新的AEs。

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引用次数: 0

Multicentric carpotarsal osteolysis syndrome with variants of MAFB gene: a case report and literature review. 伴有 MAFB 基因变异的多中心腕跗骨溶解综合征：病例报告和文献综述。

IF 2.8 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology

Pub Date : 2024-03-13 DOI: 10.1186/s12969-024-00964-6

Xianfei Gao, Xiang Fang, Danping Huang, Song Zhang, Huasong Zeng

Background: Multicentric carpotarsal osteolysis (MCTO) is a rare genetic disorder characterized by the progressive loss of bone in the hands, feet, and other skeletal structures. It presents with symptoms that may resemble those of juvenile idiopathic arthritis, making diagnosis challenging for clinicians. The identification of MAF BZIP Transcription Factor B (MAFB) mutations as significant contributors to MCTO represents a major breakthrough in our understanding of the pathogenesis of this rare skeletal disorder.

Case presentation: Our objective was to present the phenotype, treatment, and outcome of a patient with a variant of MAFB-induced MCTO to broaden the range of clinical features associated with MCTO and share our clinical experience for improved diagnosis and treatment. In our case, early MRI examination of the bones and whole exome sequencing enabled an early and accurate MCTO diagnosis, and timely Denosumab administration resulted in no deterioration.

Conclusion: This suggests that MRI examination and whole exome sequencing should be considered when MCTO is suspected, and Denosumab might be an option in the treatment of MCTO.

背景：多中心腕跗骨溶解症（MCTO）是一种罕见的遗传性疾病，其特征是手、脚和其他骨骼结构的骨质逐渐流失。该病的症状可能与幼年特发性关节炎相似，这给临床医生的诊断带来了挑战。确定MAF BZIP转录因子B（MAFB）突变是导致MCTO的重要因素，是我们对这种罕见骨骼疾病发病机制认识的重大突破：我们的目的是介绍一名变异型 MAFB 诱导的 MCTO 患者的表型、治疗和结果，以扩大与 MCTO 相关的临床特征的范围，并分享我们的临床经验，从而改进诊断和治疗。在我们的病例中，早期的骨骼 MRI 检查和全外显子组测序使 MCTO 得到了早期、准确的诊断，及时使用地诺单抗也没有导致病情恶化：结论：这表明，当怀疑有 MCTO 时，应考虑进行 MRI 检查和全外显子组测序，而地诺单抗可能是治疗 MCTO 的一种选择。

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引用次数: 0

Serum and salivary inflammatory biomarkers in juvenile idiopathic arthritis-an explorative cross-sectional study. 幼年特发性关节炎的血清和唾液炎症生物标志物--一项探索性横断面研究。

IF 2.5 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology

Pub Date : 2024-03-09 DOI: 10.1186/s12969-024-00972-6

Lena Cetrelli, Anette Lundestad, Elisabet G Gil, Johannes Fischer, Josefine Halbig, Paula Frid, Oskar Angenete, Annika Rosén, Karin B Tylleskär, Keijo Luukko, Ellen Nordal, Anne N Åstrøm, Marit S Skeie, Astrid Kamilla Stunes, Athanasia Bletsa, Abhijit Sen, Astrid J Feuerherm, Marite Rygg

Background: Biomarkers may be useful in monitoring disease activity in juvenile idiopathic arthritis (JIA). With new treatment options and treatment goals in JIA, there is an urgent need for more sensitive and responsive biomarkers.

Objective: We aimed to investigate the patterns of 92 inflammation-related biomarkers in serum and saliva in a group of Norwegian children and adolescents with JIA and controls and in active and inactive JIA. In addition, we explored whether treatment with tumor necrosis factor inhibitors (TNFi) affected the biomarker levels.

Methods: This explorative, cross-sectional study comprised a subset of children and adolescents with non-systemic JIA and matched controls from the Norwegian juvenile idiopathic arthritis study (NorJIA Study). The JIA group included individuals with clinically active or inactive JIA. Serum and unstimulated saliva were analyzed using a multiplex assay of 92 inflammation-related biomarkers. Welch's t-test and Mann-Whitney U-test were used to analyze the differences in biomarker levels between JIA and controls and between active and inactive disease.

Results: We included 42 participants with JIA and 30 controls, predominantly females, with a median age of 14 years. Of the 92 biomarkers, 87 were detected in serum, 73 in saliva, and 71 in both biofluids. A pronounced difference between serum and salivary biomarker patterns was found. Most biomarkers had higher levels in serum and lower levels in saliva in JIA versus controls, and in active versus inactive disease. In serum, TNF and S100A12 levels were notably higher in JIA and active disease. The TNF increase was less pronounced when excluding TNFi-treated individuals. In saliva, several biomarkers from the chemokine family were distinctly lower in the JIA group, and levels were even lower in active disease.

Conclusion: In this explorative study, the serum and salivary biomarker patterns differed markedly, suggesting that saliva may not be a suitable substitute for serum when assessing systemic inflammation in JIA. Increased TNF levels in serum may not be a reliable biomarker for inflammatory activity in TNFi-treated children and adolescents with JIA. The lower levels of chemokines in saliva in JIA compared to controls and in active compared to inactive disease, warrant further investigation.

背景：生物标志物可能有助于监测幼年特发性关节炎（JIA）的疾病活动。随着 JIA 治疗方案和治疗目标的更新，迫切需要更灵敏、反应更快的生物标志物：我们的目的是调查一组挪威儿童和青少年JIA患者和对照组以及活动性和非活动性JIA患者血清和唾液中92种炎症相关生物标志物的变化规律。此外，我们还探讨了肿瘤坏死因子抑制剂（TNFi）的治疗是否会影响生物标志物的水平：这项探索性横断面研究由挪威幼年特发性关节炎研究（NorJIA研究）中患有非系统性JIA的儿童和青少年以及匹配的对照组组成。JIA组包括临床活动性或非活动性JIA患者。血清和未刺激唾液采用 92 种炎症相关生物标记物的多重检测方法进行分析。采用韦尔奇 t 检验和曼-惠特尼 U 检验分析 JIA 与对照组之间以及活动性与非活动性疾病之间生物标志物水平的差异：我们共纳入了42名JIA患者和30名对照组患者，其中女性居多，中位年龄为14岁。在 92 种生物标志物中，87 种在血清中检测到，73 种在唾液中检测到，71 种在两种生物流体中检测到。研究发现，血清和唾液中的生物标记物模式存在明显差异。大多数生物标记物在 JIA 和对照组之间，以及在活动性和非活动性疾病之间，血清中的含量较高，而唾液中的含量较低。在血清中，JIA 和活动性疾病患者的 TNF 和 S100A12 水平明显较高。如果不包括接受 TNFi 治疗的患者，TNF 的升高就不那么明显。在唾液中，趋化因子家族的几种生物标志物在 JIA 组中明显较低，在活动性疾病中甚至更低：在这项探索性研究中，血清和唾液中的生物标志物模式存在明显差异，这表明在评估JIA的全身炎症时，唾液可能无法替代血清。在接受TNFi治疗的儿童和青少年JIA患者中，血清中TNF水平的升高可能不是炎症活动的可靠生物标志物。与对照组相比，JIA患者唾液中趋化因子的水平较低；与非活动性疾病相比，活动性疾病患者唾液中趋化因子的水平较低，这值得进一步研究。

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引用次数: 0