儿童神经精神性狼疮的研究进展

儿奄神经精神性狼疮(NPSLE)是系统性红斑狼疮(SLE)的严重并发症,可发生于SLE病程的任何阶段,其可能的发病机制是多方面的,包括自身抗体的产生、微血管病变、鞘内前炎性因子的产生和过早的动脉粥样硬化等.其临床表现复杂多样,中枢神经系统症状在儿童常见,可以出现严重的反复发作性头痛、癫痫发作、腩血管意外及精神症状.综合运用血清学、脑脊液检查、神经影像学检查有助于NPSLE的诊断.治疗主要应用免疫抑制剂和对症处置.早期诊断、积极治疗可能有助于改善患儿的预后.     

儿童神经精神性狼疮临床特征及危险因素分析

目的总结儿童神经精神性狼疮(NPSLE)的临床特征及危险因素。方法回顾分析2013年1月至2018年12月住院治疗的SLE患儿的临床资料。结果纳入315例SLE患儿中,58例确诊NPSLE,男11例、女47例,平均年龄(10.45±2.63)岁;共出现14种神经精神症状,依次为头痛21例、癫痫发作19例、脑血管意外15例、脱髓鞘综合征13例、情绪失调10例。58例NPSLE患儿均行头颅影像学检查,异常53例(91.4%);44例行脑电图检查,异常38例(86.4%);39例行脑脊液检查,异常34例(87.2%)。首次发病发生NPSLE较未发生NPSLE的患儿,血小板计数100×10~9/L的发生率高,差异有统计学意义(P0.05)。2013年1月至2015年12月发病的23例患儿病死率为21.7%,高于2016年1月至2018年12月发病的35例患儿(病死率2.9%),差异有统计学意义(P0.05)。多元logistic回归分析表明,血小板计数降低、抗心磷脂抗体阳性、激素治疗不规范是发生NPSLE的独立危险因素(P0.05)。结论 NPSLE以头痛、癫痫发作最多见,血小板计数降低的NPSLE患儿更早出现神经精神症状。近年NPSLE患儿病死率较前有所降低。血小板计数降低、抗心磷脂抗体阳性、激素治疗不规范是SLE患儿发生NPSLE的危险因素。     

小儿神经精神性狼疮

目的：探讨小儿神经精神性狼疮（ＮＰＬＥ）的临床表现、诊断和发病机理。方法：对１２年来诊断为系统性红斑狼疮２６例中有神经精神症状的８例ＮＰＬＥ进行系统的临床观察，分析其ＣＳＦ、ＥＥＧ和影像学检查所见。结果：临床表现有精神症状、器质性脑功能障碍、颅神性损害、外周神经病变和惊厥发作；ＣＳＦ变化主要是蛋白增高，ＥＥＧ均有不同程度的脑电波异常，颅脑影像学检查４例有３例异常。尸检１例示脑及多个脏器血管病变。结论：ＮＰＬＥ的临床表现多样化，需结合ＣＳＦ、ＥＥＧ和影像学检查在排除其他神经精神症状的病因后作出诊断。其发病机理是血管病变、免疫损伤和抗神经原的自身抗体等多种因素的作用。     

儿童神经精神性狼疮12例影像学表现及临床分析

目的 探讨儿童神经精神性狼疮的临床特点与影像学的关系.方法 回顾性分析我院小儿肾脏风湿免疫科2005年1月至2013年1月间收治的12例确诊儿童神经精神性狼疮患儿的临床资料及影像学资料.结果 12例患儿均处于狼疮重度活动期,均行头部MRI或CT检查.3例行头部CT检查,2例异常;11例行头部MRI检查,10例发现异常,弥漫型病变4例,病变主要分布在双侧大脑半球、基底节区、半卵圆中心及小脑半球,多发点、片状T1低信号、T2高信号影,FLAIR与DWI表现为高信号或等信号;局灶型病变4例,表现为单一部位的T1低信号、T2高信号,FLAIR及DWI表现为相同部位高信号;病变部位在大脑半球2例、脑干1例、小脑1例.脑萎缩病变1例,脑出血病变1例.结论 头部MRI诊断儿童神经精神性狼疮敏感性高,抗心磷脂抗体阳性可能与系统性红斑狼疮中枢神经系统损害有密切关系.狼疮重度活动时需警惕神经精神性狼疮的发生,结合患儿病史特点、临床表现及影像学改变,除外其他原因引起的神经系统症状,早期诊断及积极治疗,患者预后可能良好.     

儿童神经精神性狼疮14例临床特点及危险因素分析

目的 总结儿童神经精神性狼疮（NPSLE）的临床特点,并探讨儿童系统性红斑狼疮（SLE）并发NPSLE的相关危险因素。方法 对2005年1月至2013年8月在中国医科大学附属第一医院儿科住院的14例NPSLE患儿临床资料进行回顾性分析,同时选择同期入院的31例无NPSLE的SLE患儿作对照,对两组患儿的发病年龄、性别、临床表现、实验室检查等因素进行单因素及多因素分析。结果 NPSLE患儿的主要临床表现为头痛2例（50.0%）、偏侧型运动障碍7例（50.0%）、精神异常5例（35.7%）、癫痫样发作5例（35.7%）、不自主运动2例（14.3%）、视物不清2例（14.3%）等。13例行头颅磁共振成像（MRI）检查均有异常表现。单因素分析显示患儿口腔溃疡、血淋巴细胞减少、血小板减少、C反应蛋白（CRP）升高、抗心磷脂抗体阳性、抗中性粒细胞胞浆抗体阳性、抗u1-RNP抗体阳性、尿红细胞增多、血K+升高、血尿素氮升高、血肌酐升高为有统计学意义的危险因素;进一步行非选择性多因素回归分析结果显示抗心磷脂抗体阳性、CRP升高、血肌酐升高是发生NPSLE的独立危险因素（OR=8.296、11.170、30.415,P＜0.05）。结论 头痛、运动障碍、精神异常以及癫痫样发作是NPSLE患儿最常见的临床症状,抗心磷脂抗体阳性、CRP升高、血肌酐升高是SLE患儿发生NPSLE的危险因素。     

儿童神经精神狼疮早期诊断和治疗

系统性红斑狼疮（systemic lupus erythematosus,SLE）侵犯中枢神经系统和外周神经系统,导致出现相应神经精神性症状及体征,称为神经精神性狼疮（neuropsychiatric lupus erythematosus,NPSLE）。NPSLE并不少见,可见于1/4～3/4的SLE患者,且病死率为7%～19%［1-3］。NPSLE已成为SLE的重要死亡原因,目前国内对儿童NPSLE关注不多,因此在某种程度上未能及时对NPSLE作出正确诊断。     

儿童精神性排便异常

精神性排便异常是儿童排便异常中一个容易被忽视的课题。近年来随着小儿肛肠外科的发展,特别是在检测手段上对直肠测压及其他电生理技术的推广与应用,使功能性及器质性排便异常的诊断更加明确,而对于小儿直肠肛门畸形治疗手段的深入研究也使各种并发症的发生大幅度减少。这些成果已使更多的儿童从排便异常的痛苦中解救出来。尽管如此,排便异常因其极具社会性,今后仍然需要进一步深入研究。在研究排便异常时,人们对器质性排便异常给予了过多的重视,立足于改进多种手术方法,进行一系列的术后功能练习,这些措施无疑对促进恢复起到了重…     

儿童脓毒症发病机制及诊治进展

脓毒症是一种常见的临床综合征.其不同于既往的败血症或菌血症,具有更加复杂的病理生理过程.其发病机制复杂,包括促炎/抗炎因子平衡失调、肠道细菌易位、免疫抑制、相关信号通路激活等.进一步对脓毒症发病机制的研究是探索脓毒症治疗新方法的理论基础.该文就脓毒症的诊断、发病机制、治疗原则及常见病毒感染所致脓毒症进行综述.     

儿童精神性尿频的尿动力学变化

目的 探讨儿童精神性尿频的病因、病理生理变化及其治疗。方法 本组38例，应用尿流动力仪分别记录排尿量、尿流曲线、膀胱压力容积及压力－流率－肌电图。结果 38例中，4例尿动力学安全正常；34例出现膀胱功能异常，占89.5%(34/38)，其中逼尿肌不稳定性收缩者12例，低顺应性膀胱者6例，低顺应性膀胱合并逼尿肌不稳定性收缩者16例；最大膀胱测量容量百分数下降14例。排尿期异常仅5.3%(2/38)，为尿道括约肌过度活跃。尿动力学检测后，84.2%的患儿症状完全消失或好转。结论 逼尿肌不稳定性收缩是最主要的病理生理学改变；排尿训练是主要治疗措施。     

儿童咳嗽变异性哮喘发病机制及诊治进展

近年来,儿童慢性咳嗽的发病率及就诊率逐年增加,其中咳嗽变异性哮喘是儿童慢性咳嗽主要病因之一。全球哮喘防治创议指出咳嗽变异性哮喘是以咳嗽为主要或唯一症状,并伴有气道高反应性的疾病。该病是哮喘的一个亚型,主要的临床表现是反复或持续的咳嗽。由于缺乏典型的喘息症状,儿童咳嗽变异性哮喘在临床上易被误诊或漏诊,进而缺乏规范治疗。此...     

儿童狼疮性脑病临床特征

目的探讨儿童狼疮性脑病(NPSLE)的临床特点、辅助检查及治疗。方法对11例NPSLE患儿的临床资料进行分析。结果11例NPSLE患儿均有不同程度的头痛症状,意识障碍及失语各1例,抽搐、记忆力减退和共济失调各3例,出现病理反射及脑膜刺激征各4例。血清学均呈狼疮活动表现。3例脑脊液常规无异常,寡克隆抗体明显升高。其中10例行头颅CT检查,异常9例;10例患儿行脑电图检查,正常1例,且与CT无交叉。经个体化综合治疗,11例患儿神经精神症状均有不同程度的改善。结论系统性红斑狼疮累及神经系统症状最常表现为头痛。目前NPSLE主要根据临床表现作出诊断,血清学、影像学及脑电图检查有助于狼疮性脑病的诊断。对NPSLE的治疗强调个体化的综合治疗。     

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??Objective??To analyse the positron emission computed tomography??PET/CT?? findings of neuropsychiatric systemic lupus erythematosus??NPSLE?? in children. Methods??Patients diagnosed with NPSLE treated at Department of Pediatrics??Peking Union Medical College Hospital from April 2012 to February 2014 were recruited. PET/CT using F-18-labelled fluorodeoxyglucose??18F-FDG?? was performed in patients with NPSLE??and the results were analyzed. Results??A total of 19 patients received 18F-FDG PET/CT and 13 of them were females. Age was from 7to14 years and median age was 11.3 years. The most common neuropsychiatric manifestation was headache??8/19???? followed by seizure??7/19???? cognition dysfunction??2/19????psychiatric disease??1/19?? and ataxia??1/19??. All patients under MRI examination??and only 4 of them were abnormal. 18F-FDG PET/CT revealed significant glucose hypometabolism in all patients. The temporal lobe??17/19?? was the most susceptible brain regions??followed by the parietal lobe??16/19????occipital lobe??14/19?? and frontallobe??10/19??. In contrast??glucose hypermetabolism was detected in basal ganglia??8 patients?? and thalamus??3 patients??. Conclusion??PET reveals significant glucose hypometabolism with or without local hypermetabolism in patients with NPSLE.     

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??Objective The aim of this study was to summarize the clinical features in children with neuropsychiatric systemic lupus erythematosus??NPSLE?? and to investigate the related risk factors of in systemic lupus erythematosus??SLE?? patients with NPSLE. Methods The clinical data of 14 children with NPSLE were retrospectively analyzed. The indicators including the age?? sex?? clinical manifestations?? laboratory tests were compared with 31 randomly selected SLE patients with non-NPSLE manifestations by univariate and multivariate analysis. Results The main clinical manifestations in children with NPSLE were headache??50%????lateral type motor disorder??50%????mental disorders??35.7%????epilepsy??35.7%????involuntary movement ??14.29%????blurred vision??14.29%?? etc. 13 patients had abnormalities in brain MRI. Univariate analysis showed that children with oral ulcers??blood lymphopenia??thrombocytopenia??elevated CRP??anticardiolipin antibodies positive??antineutrophil cytoplasmic antibody positive??anti-u1-RNP antibody positive??urine red blood cells increased??elevated serum K+ elevated??blood urea nitrogen??elevated serum creatinine were the risk factors with statistical significance. Further non-selective multivariate regression analysis showed that only the anticardiolipin antibody positive?? elevated CRP?? elevated serum creatinine into the multiple regression equation?? a positive correlation. Conclusion Headache?? movement disorders?? mental disorders and seizures were the most common symptoms in children with NPSLE. The brain MRI is a sensitive indicator of checking NPSLE. Anticardiolipin antibody positive?? elevated CRP and elevated serum creatinine were the most important risk factor for SLE children with NPSLE.     

系统性红斑狼疮患儿低钠血症诊治分析

目的探讨系统性红斑狼疮(SLE)患儿低钠血症的临床特征及影响因素。方法回顾分析自2011年1月至2019年1月收治的83例SLE患儿的临床资料,并根据其血钠水平分为低钠血症组和非低钠血症组,比较两组患儿的临床表现及实验室检查结果。结果与非低钠血症组相比,低钠血症组患儿有较高的肾脏病变发生率,血沉、C-反应蛋白、白介素-6(IL-6)、IL-1、SLE疾病活动指数(SLEDAI)明显升高,血氯、白蛋白和补体C3水平降低,差异均有统计学意义(P均0.05);而白细胞计数、血天冬氨酸转氨酶、乳酸脱氢酶、尿素氮和肌酐的差异无统计学意义(P均0.05)。结论 SLE 合并低钠血症患儿更容易出现肾脏病变,低钠血症可能与严重炎症反应有关。     

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??Objective??To observe the incidence??clinical types??and prognosis of neuropsychiatric systemic lupus erythematosus??NPSLE?? in children??and to probe the relationship between the onset and the relacted factors. Methods??
The clinical data of 87 children with SLE treated in Children’s Hospital of Shanghai from Jan. 2002 to Dec. 2015 were analyzed retrospectively. The patients were divided into NPSLE group and non-NPSLE group. Collect the data of the patients??including the course of the disease??activity index??SLEDAI scores????blood sedimentation??the values of serum complement and the positive rate of dsDNA??anti-ribosomal P-protein autoantibody and anticardiolipin antibody in autoantibodies??and make a comparison and statistical analysis. Results??Nervous system injury occurred in 23 cases??with an incidence rate of 26.4%??and the average course of the disease was 3??11 months. Most of 23 cases had headache. Most of the patients presented SLE activity in serology. Totally 39 cases of all patients had abnormal cranial imaging findings. Electroencephalogram was performed in 21 patients??which presented 12 abnormalities. There were no statistical differences between patients with and without NPSLE in the disease course??blood sedimentation??the values of serum complement??the positive rate of dsDNA or activity index. Clinical symptoms were improved in all 39 patients after treatment??but 2 patients died of heart failure in follow-up. Conclusion??Neuropsychiatric manifestation in children with SLE is common??and it can happen at any stage of the disease. NPSLE in children presents different kinds of symptoms. Early diagnosis and treatment can improve the short-term prognosis. Psychiatric symptoms may also occur during therapy.     

小儿系统性红斑狼疮的特点

目的了解小儿系统性红斑狼疮(SLE)的特点。 方法对85例SLE儿的临床资料进行分析。 结果85例中起病年龄10a以上者66例(77．6％),男女比为16．1,12例(14．1％)家族中有结缔组织病史。最常见的表现是抗核抗体(ANA)阳性(91．8％)、血沉增快(90．6％)、肾脏受累(82．3％)、发热(82．3％)、低补体血症(81．2％)、蝶形红斑(69．4％)、关节症状(62．3％)及血液系统损害(62．3％)。18例(21．2％)起病时表现为单一系统损害。6例(7．0％)肾损害始终为唯一临床表现。经激素联合免疫抑制剂治疗,随访0．5～23a,临床缓解或病情波动者49例,无1例进入慢性肾功能衰竭,死亡9例,失访27例。 结论本病临床表现多样,肾损害发生率高,早期正规治疗疗效显著。     

Antineuronal antibodies and electroneurophysiological studies in pediatric patients with neuropsychiatric systemic lupus erythematosus

Neuropsychiatric systemic lupus erythematosus (NPSLE) is one of the most difficult manifestations of lupus to diagnose. Measurement of serum brain autoantibodies and assessment of cognitive function by electroneurophysiological studies (electroencephalogram (EEG) and P300) have contributed to an earlier and a more specific diagnosis of NPSLE. Thus, we were stimulated to assess the value of serum antineuronal antibodies and electroneurophysiological studies in diagnosis and early prediction of NPSLE. To investigate this, assessment of serum antineuronal antibodies and cognitive function (clinically and by electroneurophysiological studies) was done in 30 lupus patients [14 (46.7%) with and 16 (53.3%) without clinical evidence of NPSLE] in comparison with 30 healthy matched subjects. Patients without clinical evidence of NPSLE were followed-up clinically by monthly neuropsychiatric evaluation for 18 months. Seropositivity for antineuronal antibodies and abnormalities of EEG and P300 (prolonged latency and/or low amplitude) were found in 60%, 50% and 70%, respectively of lupus patients. During follow-up, 8 out of the 16 patients without clinical evidence of NPSLE developed such evidence [six (75%) had antineuronal seropositivity, five (62.5%) had abnormal EEG, six (75%) had P300 abnormalities and all had at least one abnormal result of these parameters at the time of initial evaluation before clinical presentation of NPSLE]. In conclusion, serum antineuronal antibodies and electroneurophysiological studies may be reliable parameters for diagnosis and early prediction of NPSLE, especially when combined together, before clinical manifestations ensue. Further studies on a large scale are warranted to evaluate the predictive value of these parameters in NPSLE.     

抗心磷脂抗体与儿童系统性红斑狼疮及狼疮性肾炎相关性研究

目的探讨抗心磷脂抗体(ACA)与儿童系统性红斑狼疮(SLE)及狼疮性肾炎(LN)的相关性。方法选取2005年4月至2013年10月住院治疗116例SLE患儿,收集并分析患儿的临床资料。结果 116例SLE患儿中,男20例、女96例;ACA-Ig M、Ig G或Ig A的阳性率分别为37.1%、35.3%、26.7%,阳性率差异无统计学意义(P>0.05)。116例SLE患儿中LN 75例,非LN 41例,LN与非LN患儿之间ACA-Ig M、Ig A阳性率差异无统计学意义(P均>0.05);而LN患儿ACA-Ig G阳性率(42.7%)高于非LN患儿(22.0%),差异有统计学意义(P=0.026)。男性和女性患儿间,LN及ACA各亚型比例的差异无统计学意义(P均>0.05)。结论各亚型ACA对于SLE的诊断价值无差别,但ACA-Ig G阳性提示易并发LN。     

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??Abstract??Objective??To observe the efficacy and safety of Rituximab ??Roche Pharmaceuticals RTX?? trade name?? rituximab?? treatment for children with systemic lupus erythematosus??and try to find a useful alternative therapeutic approach to those with bad response to traditional therapies. Methods??Produced by Roche Pharmaceuticals??CD20 monoclonal antibody rituximab was used by intravenous injection. Initial dose was 188 mg/m2?? 2 weeks later the second injection was given at 375 mg/m2. Thirty minutes before each injection 5mg Dexamethasone and 10mg Promethazine were given to prevent drug allergy. Results??Twelve cases of SLE in children were performed flow cytometry detection of CD20+ 4 weeks after administration??10 cases were 0??85.71%?? in B cell depletion?? and gradually rose after 6??8 months. IgG?? IgM and IgA plasma had no significant difference from the baseline. Clinical manifestations and clinical signs in 12 cases of children were evaluated??and the average score dropped from 16.0±2.95 to 8.67±1.83. ANA?? Anti-ds-DNA?? C3 and C4 had different degrees of improvement. In 6 cases of lupus nephritis children with urinary protein was significantly improved after 6 months of Rituximab treatment??4 cases of elevated serum creatinine and blood urea nitrogen was also returned to normal after 6 months of treatment. One case had severe pulmonary infection. Conclusion??Significant effects of CD20+ monoclonal antibody ??rituximab?? treatment are shown for children with SLE and lupus nephritis?? especially in severe children. This provides a new alternative treatment for those with poor tolerance to traditional Prednisone and cyclophosphamide treatment and with poor clinical effect. However?? infection problem can not be ignored. The Rituximab replacement treatment for children with SLE still need further study.     

A 10 year review of systemic lupus erythematosus in Singapore children

A 10 year retrospective analysis of the clinical features and survival of 24 Singapore children with systemic lupus erythematosus was made. The female to male sex ratio was 11:1. The median age at diagnosis was 9.3 years (range: 3.5–17.6 years), and the median duration of follow-up was 3.6 years (range: 3 months - 10 years). The common modes of presentation were prolonged fever and malar rash (both 46%). Renal involvement (71 %) was frequent. There were six deaths, three from chronic renal failure, two from infection, and one from carditis. The overall survival at 5 years was 0.800 (s.e.m. = 0.090), and at 10 years 0.698 (s.e.m. = 0.103). The survival for lupus nephritis was 0.727 at 5 years (s.e.m. = 0.116), and 0.586 at 10 years (s.e.m. = 0.130). Although the 5 year survival rate is comparable with other series, there were more deaths after the first 5 years, and morbidity from the disease as well as from therapy was considerable.