斯琼综合征合并幼年特发性关节炎1例的护理

总结1例斯琼综合征合并幼年特发性关节炎患儿的护理体会。护理要点包括加强皮肤、眼部、口腔黏膜的局部护理,保护受损关节,积极做好特殊用药甲泼尼龙和丙种球蛋白的护理,做好患儿及家长的心理护理。患儿经治疗和护理,病情好转出院。     

全身型幼年特发性关节炎1例

1例报告 男,7岁.因间歇发热1个月余于2003-10入院.1个月前无明显诱因出现发热、咽痛,无其他伴随症状,体温最高41℃,在当地医院静滴抗生素治疗效果差,仍有持续高热,发热时伴一过性全身皮疹,呈红斑丘疹,以躯干部为主,不痒,热退后皮疹可消退,应用氢化考的松后体温可下降,发热半个月后出现全身水肿、胸腔积液、心包积液,在当地医院多次查血常规WBC (20.1～34.3)×109/L,N0.89～0.91,Hb123～96 g/L,PLT正常.     

幼年性特发性关节炎的膝关节MRI表现

目的幼年性特发性关节炎的早期诊断更易于治疗及改善预后。回顾性研究幼年性特发性关节炎的膝关节MR表现特征以提高对于幼年性特发性关节炎的诊治水平。方法分析42例幼年性特发性关节炎患儿的膝关节磁共振表现。患者中女18例、男24例,平均年龄10.5岁（5-16岁）。结果42例JIA患者的42个膝关节可见不同程度的MR异常改变（100%）,主要影像学表现包括滑膜增生及血管翳形成、关节腔渗出积液、骨髓水肿、半月板形态及信号异常改变、关节软骨破坏、腘窝淋巴结肿大等。结论在幼年性特发性关节炎的膝关节MRI表现中,滑膜增厚、关节腔积液及骨髓水肿较为常见;关节软骨破坏及软骨下骨质侵蚀则较为少见。     

血清细胞因子水平监测在托珠单抗治疗幼年特发性关节炎全身型疗效评价中的应用

目的探讨血清细胞因子水平对评价托珠单抗治疗幼年特发性关节炎全身型(systemic-onset juvenile idiopathic arthritis,SoJIA)疗效的意义。方法选取2016年6月至2018年10月在首都儿科研究所附属儿童医院住院的SoJIA患儿30例,其中男20例(67%),女10例(33%),诊断时年龄为0.84至13岁。使用白细胞介素-6受体拮抗剂(托珠单抗注射液)治疗,观察治疗前、用药2周后、用药6周后及用药22周后的血白细胞(WBC)、C反应蛋白(CRP)、动态红细胞沉降率(AESR)、血清白细胞介素(IL-6、IL-2R、IL-8、IL-10、IL-1β)及肿瘤坏死因子α(TNF-α)水平。采用Mann-Whitney非参数检验以及卡方检验分析用药后不同时间上述细胞因子水平的差异。结果30例患儿,用药前均有发热,其中28例患儿应用托珠单抗后体温降至正常,治疗期间未再出现发热,1例患儿用药后出现过敏反应后停药,1例用药后疗效不佳停药。在用药后体温正常的28例患儿中,其关节炎及皮疹表现均有明显改善,且WBC、AESR及CRP均较用药前下降。28例SoJIA患儿,血清IL-6水平在第2周为107.50(28.03~281.50)pg/mL,与治疗前[168.50(67.40~589.25)pg/mL]相比差异无统计学意义(Z值为-1.754,P>0.05),用药6周后为64.05(19.90~130.75)pg/mL,用药22周后为24.80(3.45~95.40)pg/mL,浓度均低于治疗前,差异有统计学意义(Z值分别为-2.942、-3.334,P值均为<0.01);血清IL-2R水平第2周为740.50(510.00~1161.00)U/mL,第6周为796.50(534.00~1008.00)U/mL,第22周为688.00(527.00~889.50)U/mL,分别与治疗前[1322.50(812.00~1659.00)U/mL]相比,均低于治疗前水平,差异有统计学意义(Z值分别为-2.818、-3.130、-3.466,P值均<0.01);TNF-α浓度在用药2周后为23.70(20.30~41.23)pg/ml,6周后为26.75(16.83~47.03)pg/ml,22周后为18.60(13.10~34.90)pg/ml,分别与治疗前的26.50(20.55~37.43)pg/ml比较差异无统计学意义(Z值分别为0、-0.560、-1.954,P值均>0.05);IL-8浓度在用药2周后为200.85(95.43~364.00)pg/ml,6周后为194.50(50.75~433.00)pg/ml,22周后为161.50(38.98~308.00)pg/ml,分别与治疗前的96.20(59.75~371.75)pg/ml比较差异无统计学意义(Z值分别为-0.86、-0.131、-0.186,P值均>0.05);IL-10水平在用药2周后与用药前比较差异无统计学意义(χ^2值为2.33,P>0.05),用药6周后及22周后分别与用药前比较差异有统计学意义(χ^2值分别为4.08、4.08,P值均<0.05);IL-1β水平用药2周后、6周后及22周后分别与用药前比较差异无统计学意义(χ^2值分别为0.084、2.504、3.818,P值均>0.05)。结论应用托珠单抗治疗后,血清细胞因子IL-6和IL-2R水平有助于监测SoJIA病情活动及药物疗效。     

幼年特发性关节炎患儿出院计划研究进展

幼年特发性关节炎（JRA ）,是儿童时期常见的风湿免疫性疾病,以慢性关节滑膜炎为主要特征,伴全身多脏器功能损害。在国外的发病率约为113／10万。Ravelli等［2］研究证实,25％～70％的JIA患者发病后10年中仍处于疾病活动状态。JIA逐渐成为儿童致残的主要原因之一［1-3］。目前认为JRA比较常见的病因有感染因素、遗传因素、免疫因素和其他如寒冷、潮湿、疲劳、营养不良、精神等因素。常常是急性发作与缓解交替出现。幼年类风湿性关节炎的治疗提倡尽可能保存患儿关节功能和减轻关节外症状;对患儿及其家长进行心理支持,以保证患儿正常的生长发育。所以,对患儿的健康教育和出院管理在患儿的疾病控制方面起到举足轻重的作用。     

肌骨超声诊断幼年特发性关节炎的临床研究

目的:探讨肌骨超声诊断幼年特发性关节炎(JIA)的价值。方法:2017年7月-2019年1月本院收治的40例JIA患儿作为研究对象,另选取40例健康儿童作为对照。超声扫查掌指关节、近端指间关节、腕关节、膝关节和踝关节,计算关节滑膜炎个数,并与体格检查结果进行比较。结果:40例JIA患儿共检查了400个关节,体格检查检出滑膜炎30例(138个关节)、肌骨超声检出38例(183个关节)。肌骨超声将4例患儿(体格检查仅关节痛、无滑膜炎)诊断为持续性少关节型JIA,将2例患儿(体格检查诊断为少关节型)诊断为类风湿因子阳性的多关节型,将2例患儿(体格检查诊断为少关节型)诊断为类风湿因子阴性的多关节型。与健康儿童比较,JIA患儿所有受测关节的软骨厚度均较低(P<0.05)。结论:肌骨超声诊断JIA 的灵敏度比体格检查高,有较高的临床应用价值。     

幼年特发性关节炎的诊治进展

幼年特发性关节炎（JIA）是小儿常见的风湿病之一。以全身表现及反复发作的关节炎症为特征。JIA是由国际风湿病联盟在2001年加拿大埃得蒙顿第三次会议上统一命名的，从而取代了以往的幼年类风湿性关节炎（JRA）和幼年慢性关节炎（JCA）这两个分类标准。本病的治疗目前多采用早期治疗，联合用药，以缓解症状，控制疾病的进展。     

全身型幼年特发性关节炎临床及治疗特点

目的 探讨全身型幼年特发性关节炎（SOJIA）临床与治疗特点.方法 回顾性分析65例SOJIA患儿的临床表现、实验室检查及治疗方法与随访结果.结果 仅22例（33.8％）临床同时出现典型三联征,43例（66.2％）患儿临床表现不典型,其中12例（18.5％）患儿因临床表现不典型而贻误诊断.采用个体化治疗方案：非甾体类抗炎药（NSAIDs）＋糖皮质激素7例（10.8％）；NSAIDs＋甲氨蝶呤（MTX） 15例（23.1％）；NSAIDs＋MTX＋糖皮质激素43例（66.2％）,其中8例应用大剂量甲泼尼龙冲击治疗,8例因病情反复而加用环孢素A治疗.23例（35.4％）联合应用丙种球蛋白治疗.64例（98.5％）患儿初治均获缓解.53例随访4个月～7年,29例（54.7％）持续缓解,24例（45.3％）复发.结论 SOJIA非典型病例早期诊断存在困难,NSAIDs＋抗风湿药物＋糖皮质激素联合用药是目前较为推崇的治疗方案,但远期疗效差,复发率较高,生物制剂有望改善预后.     

幼年特发性关节炎二例误诊分析

1 病例资料 [例1] 女,8岁.因持续发热3月余入院.3个月前出现发热,热型呈弛张热,同时出现颈部和躯干淡红色斑点状充血疹,热退后消失,不留痕迹.发热时精神欠佳,热退后精神好,无全身中毒症状.病程中2次出现右肘部关节肿痛,颈痛伴活动受限,并有一过性膝、踝关节疼痛.     

误诊为幼年特发性关节炎54例分析

对我院1998-2005年曾诊断为幼年特发性关节炎（JIA）而最终排除的54例分析如下。     

Refractory systemic onset juvenile idiopathic arthritis: current challenges and future perspectives

Systemic juvenile idiopathic arthritis (SJIA) is a rare disease with distinct features not seen in other categories of juvenile idiopathic arthritis. In recent years, advances in the understanding of disease immunopathogenesis have led to improved targeted therapies with significant improvement in patient outcomes. Despite these advances, there remain subsets of SJIA with refractory disease and severe disease-associated complications. This review highlights existing options for treatment of refractory SJIA and explores potential future therapeutics for refractory disease.

Key Points:

• Despite targeted Interleukin IL-1 and IL-6 inhibitors a subset of SJIA remains refractory to therapy. About 1 in 7 SJIA patients will be refractory to targeted IL-1 or IL-6 therapy.
• There is no current agreed upon definition for refractory SJIA and we propose in this review that refractory SJIA is presence of active systemic or arthritic features despite treatment with anti-IL-1 or anti-IL-6 therapy or disease requiring glucocorticoids for control beyond 6 months.
• SJIA disease associated complications include presence of associated macrophage activation syndrome (MAS), interstitial lung disease (ILD) or amyloidosis and management of each differs.
• Refractory SJIA treatment options currently include additional conventional synthetic disease modifying anti-rheumatic drugs (csDMARDS), biologic (bDMARDS), combination biologic therapy, targeted synthetic (tsDMARDS) or other immunomodulatory therapies.

     

From childhood to adult life with juvenile idiopathic arthritis (JIA): a pilot study

Purpose. To explore the experiences of the health service provision in the transition process from childhood to adult life from the perspective of young patients with juvenile idiopathic arthritis (JIA) and health professionals working in this field.

Method. Two groups of young persons with JIA and two groups of health professionals were interviewed in focus groups. The data were analysed according to approved guidelines for qualitative analysis.

Results. 'Capability to lead a meaningful adult life' emerged as a core category; capability as a consequence of 'being enabled' from the youths' point of view, and 'enabling the young patients' from the health professionals' perspective. Preparation for the transition and transfer process was perceived as inadequate and seemed dependent on enthusiastic health professionals both in the children's and adult wards. Suggestions for formal improvements were required in four main areas.

Conclusion. This pilot study has identified inadequacies in the transition management provided for young people with JIA. Acceptance of the young persons as active participants in their own transition process might strengthen these young persons' experience of an autonomous identity and enable participation socially and in society, thus enhancing the ability to achieve a meaningful adult life. Further investigations should focus on adults living with JIA, how they lead their lives physically and psychosocially.     

超声鉴别诊断儿童化脓性关节炎与幼年特发性关节炎的临床价值

探讨超声鉴别化脓性关节炎（SA）与幼年特发性关节炎（JIA）关节病变的临床价值。方法 回顾性分析经临床确诊的100例化脓性关节炎（SA组,共100个关节）和45例幼年特发性关节炎（JIA组,共105个关节）患儿资料,分析并比较两组超声表现;采用多因素二元Logistic回归分析筛选鉴别SA与JIA的影响因素;绘制受试者工作特征（R0C）曲线分析各超声表现单独及联合应用鉴别SA与JIA的诊断效能。结果 单因素分析显示,SA组与JIA组关节腔积液、滑膜增厚、滑膜厚度及滑膜血流分级比较,差异均有统计学意义（均P＜0.05）。多因素二元Logistic回归分析显示,关节腔积液、滑膜厚度及滑膜血流分级均为鉴别SA与JIA的影响因素（均P＜0.05）。ROC曲线分析显示,关节腔积液、滑膜厚度及滑膜血流分级鉴别SA与JIA的曲线下面积分别为0.781、0.935、0.910;三者联合应用鉴别SA与JIA的曲线下面积（0.972）,以0.732为截断值,灵敏度为90.5%,特异度为99.0%。结论 超声在鉴别儿童SA与JIA关节病变中有一定的临床价值,联合应用多个超声表现能提高鉴别诊断效能。     

高频超声对幼年特发性关节炎膝关节病变的诊断价值

目的探讨高频超声在幼年特发性关节炎(JIA)膝关节病变中的诊断价值.方法应用高频超声检查45例JIA活动期患者76个患侧膝关节和22例缓解期患者34个膝关节,观察二维声像图和彩色多普勒血流特征.结果①JIA活动期膝关节滑膜增厚显示率94.7%,髌上囊积液出现率89.5%,滑膜厚度与缓解期比较有显著性差异(P<0.01);②活动期滑膜厚度与关节积液的程度呈显著正相关(r=0.461);③增厚的滑膜内血流信号的显示率为31.9%,与滑膜厚度有关.结论高频超声检查为幼年特发性关节炎提供可靠的诊断指标,是临床诊断JIA和评估疗效敏感、客观的检查方法.     

From childhood to adult life with juvenile idiopathic arthritis (JIA): A pilot study

Purpose. To explore the experiences of the health service provision in the transition process from childhood to adult life from the perspective of young patients with juvenile idiopathic arthritis (JIA) and health professionals working in this field.

Method. Two groups of young persons with JIA and two groups of health professionals were interviewed in focus groups. The data were analysed according to approved guidelines for qualitative analysis.

Results. ‘Capability to lead a meaningful adult life’ emerged as a core category; capability as a consequence of ‘being enabled’ from the youths' point of view, and ‘enabling the young patients’ from the health professionals’ perspective. Preparation for the transition and transfer process was perceived as inadequate and seemed dependent on enthusiastic health professionals both in the children's and adult wards. Suggestions for formal improvements were required in four main areas.

Conclusion. This pilot study has identified inadequacies in the transition management provided for young people with JIA. Acceptance of the young persons as active participants in their own transition process might strengthen these young persons' experience of an autonomous identity and enable participation socially and in society, thus enhancing the ability to achieve a meaningful adult life. Further investigations should focus on adults living with JIA, how they lead their lives physically and psychosocially.     

磁共振成像在诊断幼年特发性关节炎中的应用

目的:探讨幼年特发性关节炎(JIA)患者膝关节磁共振成像(MRI)的主要表现及检查技术要点。方法:探讨T1加权自旋回波序列(T1W/SE)、T2加权快速自旋回波序列(T2W/TSE)、增强后T1W脂肪抑制三维快速梯度回波序列(SPIR/3D/FFE)等技术及其在膝关节MRI中的作用,并分析42例JIA患儿的膝关节MRI表现。结果:增强后T1脂肪抑制三维快速梯度回波序列结合T1W、T2W序列能综合评价JIA造成的关节病变程度,其主要表现包括滑膜增生、血管翳形成、关节腔渗出积液、骨髓水肿、半月板变性、关节软骨破坏、腘窝淋巴结肿大等。结论:增强后T1脂肪抑制三维快速梯度回波序列结合传统T1W、T2W成像对判断JIA膝关节病变有重要价值。