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的发病机制及诊治进展

儿童神经精神性狼疮(neuropsychiatric systemic lupus erythematosus,NPSLE)是系统性红斑狼疮(systemic lupus erythematosus,SLE)的严重并发症,发生率高达95％.目前认为NPSLE发病机制是多种因素作用的结果,包括血脑屏障破坏、血管栓塞、神经内分泌功能紊乱、自身抗体的产生以及炎性介质的参与.临床表现复杂多样,主要表现为头痛、癫痫样发作及精神障碍.国际上尚无统一的诊断标准,必须综合应用免疫血清学、脑脊液检测、神经影像学和神经精神的评估,治疗主要应用免疫抑制剂、生物治疗和对症治疗,早期诊断和规范治疗有助于改善预后.     

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.     

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。     

儿童创伤临床特点及死亡危险因素分析

目的 分析儿童创伤的临床特点及死亡危险因素,为探索预防措施及降低病死率提供参考.方法 回顾性分析2014年4月至2016年3月我院急诊科收治的7 936例创伤患儿的临床特点,利用Logistic回归分析对可能引起创伤患儿死亡的危险因素进行分析.结果 创伤患儿男性多于女性(1.73∶1),外地患儿(5535例)多于本地患儿(2401例)(χ2=14.314,P＜0.05).婴儿期(2024例)和幼儿期(3097例)病例数最多.四肢[41.9%(3324/7936)]和皮肤创伤[38.5%(3058324/7936)]最多见.儿童创伤原因因年龄段不同而有所差异,坠落、跌倒、交通事故是引起儿童创伤的常见原因.存活组1933例、死亡组5例的儿童创伤评分分别为8.53±2.17、3.17±1.29,普通住院组1852例、ICU组86例的儿童创伤评分分别为9.72±1.25、5.23±1.84.休克失代偿、机械通气、Glasgow评分≤7分、儿童创伤评分＜8分是引起创伤儿童死亡的危险因素.结论 家长应提高安全意识、加强对儿童的看护、遵守交通规则以降低儿童创伤发生率.临床医生应对休克失代偿、机械通气、Glasgow评分、儿童创伤评分等因素予以足够重视,尽早识别危重患儿,提高抢救成功率.     

儿童消化性溃疡穿孔临床特征及其危险因素分析

目的　探讨小儿消化性溃疡并发穿孔的临床特征及其危险因素,为临床干预提供参考依据。方法　回顾性分析2014年12月至2019年12月郑州大学附属儿童医院688例胃镜检查诊断为消化性溃疡患儿的临床资料。患儿分为消化性溃疡合并穿孔组47例和非穿孔组641例。分析人口统计学、发病率、临床特征、病因、症状、内镜或外科处理方法及临床转归,通过多因素Logistic分析消化性溃疡并发穿孔的危险因素。结果　本组共14916例行胃镜检查,筛查消化性溃疡688例（4.61%）,其中合并穿孔47例（6.83%）。男性412例、女性276例,中位年龄8.5岁（1月龄～14岁）。溃疡患儿居住农村为主485例（70.49%）;无家族史353例（51.31%）,穿孔不伴腹膜刺激征424例（61.63%）;溃疡位置主要为胃窦228例（33.14%）,十二指肠球部264例（38.37%）;溃疡多以直径＜10 mm为主（461例,67.01%）,单发较多（465例,67.59%）;溃疡因幽门螺旋杆菌（Hp）感染（306例,44.48%）和非甾体类抗炎药（109例,15.84%）为主。穿孔组年龄明显低于非穿孔组［（5.3±1.2）岁 vs.（9.8±3.5）岁,P＜0.001］;溃疡合并穿孔多发生于农村患儿（38例,80.85%）;穿孔组更易出现发热、 白细胞及C反应蛋白升高,两组比较差异有统计学意义（P＜0.05）;穿孔好发位置为胃窦、十二指肠球部,与溃疡好发位置基本重叠;穿孔组溃疡直径≥10 mm（35例,74.47%）,穿孔组多发溃疡比例（33例,70.21%）显著高于非穿孔组（P < 0.001）;引起儿童消化性溃疡穿孔主要原因为Hp感染（28例,59.57%）和NSAID（8例,17.02%）。开腹手术预后好,但腹腔镜住院周期明显缩短。多因素Logistic回归分析提示年龄、溃疡位置及大小、病因是穿孔发生的独立危险因素。结论　消化性溃疡并发穿孔存在复杂的危险因素,尽早合理治疗利于缩短病程、提高预后。     

儿童消化性溃疡穿孔临床特征及其危险因素分析

目的　探讨小儿消化性溃疡并发穿孔的临床特征及其危险因素,为临床干预提供参考依据。方法　回顾性分析2014年12月至2019年12月郑州大学附属儿童医院688例胃镜检查诊断为消化性溃疡患儿的临床资料。患儿分为消化性溃疡合并穿孔组47例和非穿孔组641例。分析人口统计学、发病率、临床特征、病因、症状、内镜或外科处理方法及临床转归,通过多因素Logistic分析消化性溃疡并发穿孔的危险因素。结果　本组共14916例行胃镜检查,筛查消化性溃疡688例（4.61%）,其中合并穿孔47例（6.83%）。男性412例、女性276例,中位年龄8.5岁（1月龄～14岁）。溃疡患儿居住农村为主485例（70.49%）;无家族史353例（51.31%）,穿孔不伴腹膜刺激征424例（61.63%）;溃疡位置主要为胃窦228例（33.14%）,十二指肠球部264例（38.37%）;溃疡多以直径＜10 mm为主（461例,67.01%）,单发较多（465例,67.59%）;溃疡因幽门螺旋杆菌（Hp）感染（306例,44.48%）和非甾体类抗炎药（109例,15.84%）为主。穿孔组年龄明显低于非穿孔组［（5.3±1.2）岁 vs.（9.8±3.5）岁,P＜0.001］;溃疡合并穿孔多发生于农村患儿（38例,80.85%）;穿孔组更易出现发热、 白细胞及C反应蛋白升高,两组比较差异有统计学意义（P＜0.05）;穿孔好发位置为胃窦、十二指肠球部,与溃疡好发位置基本重叠;穿孔组溃疡直径≥10 mm（35例,74.47%）,穿孔组多发溃疡比例（33例,70.21%）显著高于非穿孔组（P < 0.001）;引起儿童消化性溃疡穿孔主要原因为Hp感染（28例,59.57%）和NSAID（8例,17.02%）。开腹手术预后好,但腹腔镜住院周期明显缩短。多因素Logistic回归分析提示年龄、溃疡位置及大小、病因是穿孔发生的独立危险因素。结论　消化性溃疡并发穿孔存在复杂的危险因素,尽早合理治疗利于缩短病程、提高预后。     

儿童意外损伤危险因素分析

意外损伤系指由意想不到的原因所造成的损伤或死亡,是生活中对人体生命安全有严重威胁的一类疾病。加利福尼亚州１９９３年疾病与损伤情况分析显示意外损伤是潜在寿命损失最重要的原因［１］。大量研究表明意外损伤已构成儿童期严重健康问题,有人认为意外损伤是儿童的头号杀手［２］。调查发现在５４０００名儿童中,一年中有６人因意外损伤死亡,９０４人住院治疗,１１６８２人次因意外损伤急诊。根据妇幼卫生项目县的局部抽样调查,意外死亡居我国０～１４岁儿童死亡顺位的第一位［３］。意外损伤的发生,既有外部原因,也有其内在规律…     

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。     

��ͯ�񾭾������Ǵ�14���ٴ��ص㼰Σ�����ط���

??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.     

��ͯ�񾭾������Ǵ�ͷ­�����ӷ���ϲ�������������

??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.     

��ͯϵͳ�Ժ���Ǵ��񾭾�������о�

??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。     

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。     

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。