儿童髓鞘少突胶质细胞糖蛋白G抗体相关疾病临床分析

目的：探讨儿童髓鞘少突胶质细胞糖蛋白G抗体相关疾病(MOGAD)的临床特点、影像学特征、治疗及转归,从而加深对该病的认识,提高疗效,改善预后。方法：收集我科收治的14例MOGAD患儿,总结其临床资料、治疗及转归,观察患者疗效及预后。结果：14例MOGAD患儿,男性儿童6例,女性儿童8例,发病年龄2岁至12岁。所有患儿外周血MOG-IgG滴度阳性(1∶10~1∶1000),3例同时合并抗NMDAR抗体阳性。10例表现为急性播散性脑炎,2例表现为视神经脊髓谱系疾病,2例表现为视神经炎,3例意识障碍、5例合并行为异常、6例惊厥发作。影像学累及广泛,主要累及脑白质、小脑、视神经、脊髓。所有患者急性期均给予大剂量甲基强的松龙联合丙种球蛋白冲击治疗,1例予血浆置换治疗。9例呈单相病程,5例患儿反复发作,其中3例予利妥昔单抗治疗,2例予环磷酰胺治疗,其余患者恢复较好。结论：儿童MOGAD临床表现复杂,影像学检查缺乏特异性。急性期大剂量激素联合丙种球蛋白冲击治疗有效,部分患儿呈多相病程,动态监测MOG-Abs水平有助于早期发现可能复发的患者,及时启动RTX和CTX等二线药物治疗可改善预后。     

56例MOG-IgG相关疾病首次发病临床特点回顾性分析

目的：探索抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病(anti-myelin oligodendrocyte glycoprotein-IgG associated disorder,MOGAD)首次发病的临床特点。方法：对56例临床确诊MOGAD患者首次发病临床资料进行回顾性分析，包括年龄、性别、症状、磁共振成像(magnetic resonance imaging,MRI)、眼底、脑脊液(cerebrospinal fluid,CSF)、抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体(anti-myelin oligodendrocyte glycoprotein-IgG,MOG-IgG)滴度水平、扩展功能障碍量表(expanded disability status scale,EDSS)评分、与第2次复发的间隔时间等。结果：首次发病年龄10～58岁，中位年龄23岁。男女比例为1.8∶1。主要临床表现是脑膜脑炎(32.14%)、视神经炎(25.00%)、脊髓炎(17.86%)、脑干脑炎(14.28%)及多症状(10.71%)。MRI表现无特异性，颅脑MRI显示病灶体积大、分布散，...     

抗髓鞘少突胶质细胞糖蛋白IgG抗体相关疾病临床及影像特征

目的 探讨抗髓鞘少突胶质细胞糖蛋白IgG抗体相关疾病(MOGAD)的临床及影像学特征。方法 选取2018年9月至2021年6月在山东第一医科大学附属省立医院确诊的MOGAD的临床及影像学资料,进行回顾性总结分析。结果 19例MOGAD患者中男9例,女10例,中位年龄10岁。患者临床表型为视神经脊髓炎谱系疾病者7例,急性播散性脑脊髓炎者6例,脑炎者4例[其中1例合并抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎],脊髓炎者1例,脑干脑炎者1例。17例(17/19)患者颅脑磁共振成像(MRI)检查发现异常信号,病变可累及脑组织任何部位,表现为多灶性、边界不清的点片状T₂WI高信号。4例(4/13)患者脊柱MRI检查发现脊髓异常信号。4例(4/9)患者眼眶MRI检查发现异常信号,表现为视神经T₂WI高信号;2例病变累及视交叉、视束。19例患者血清MOG-IgG抗体均为阳性,其中1例合并NMDAR抗体阳性,1例合并甲状腺过氧化物酶抗体阳性。结论 MOGAD多见于儿童青少年,临床表型多样且与年龄相关。病变可累及视神经、脊髓、颅脑等多个部位,影像学表现多...     

儿童抗N-甲基-D-天冬氨酸受体脑炎的临床特征及其复发相关因素分析

背景 目前抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎患儿复发比例较高,而相关研究较少,大部分医务人员对其认识不足。 目的 对复发和非复发的抗NMDAR脑炎患儿进行对比研究,以提高临床医生对抗NMDAR脑炎及其复发相关因素的认识,进而为抗NMDAR脑炎患儿的个性化治疗及降低复发率提供依据。 方法 回顾性分析2016年1月至2021年12月于河北医科大学附属河北省儿童医院神经内科诊断为抗NDMAR脑炎的54例住院患儿的临床资料,根据患儿复发情况分为非复发组和复发组。分析抗NDMAR脑炎患儿临床特征,包括性别和年龄分布、发病时间、临床症状及体征、脑脊液常规检查情况、影像学检查情况、脑电图检查情况、免疫学检查情况、治疗及预后情况,并对两组的上述相关指标进行比较。 结果 54例患儿男女比为1.16∶1,学龄期组（≥7岁）占比较高为51.8%;发病时间以夏季（6—8月）占比较高（33.3%）;临床症状表现多样,最常见的临床症状为癫痫发作,占61.1%;38例（70.4%）患儿出现脑脊液常规检查结果异常,主要表现为脑脊液白细胞升高和蛋白轻度升高;25例（46.3%）患儿颅脑磁共振成像（MRI）检查显示异常,异常信号最常见于额叶,其次为基底核及丘脑;6例（11.1%）给予免疫球蛋白治疗≥2轮,5例（9.3%）给予激素冲击治疗≥2轮,7例（13.0%）给予二线免疫治疗,31例（57.4%）给予抗癫痫药治疗;26例（48.1%）达到完全缓解。两组性别和年龄分布情况、发病时间、脑脊液常规检查结果、颅脑MRI检查结果异常比例、视频脑电图检查结果、CD8+T淋巴细胞、CD4/CD8、总B淋巴细胞、IgA、IgM、IgG比较、治疗前病程、免疫球蛋白治疗≥2轮比例、激素冲击治疗≥2轮比例、二线免疫治疗比例、应用抗癫痫药比例、出院时改良的Rankin量表评分（mRS）、完全缓解比例比较,差异无统计学意义（P>0.05）。复发组总T淋巴细胞及CD4+T淋巴细胞较未复发组降低（P<0.05）。 结论 抗NMDAR脑炎患儿的临床症状表现多样,但早期临床症状较重、入院后总T淋巴细胞及CD4+T淋巴细胞明显降低、给予规范免疫治疗后不易达到完全缓解的患儿应警惕复发的风险。     

以癫痫持续状态起病的儿童布鲁氏菌脑膜脑炎1例报道并文献复习

目的 探讨儿童布鲁氏菌脑膜脑炎临床特点及发病情况。方法 报道1例儿童马耳他布鲁氏菌脑膜脑炎（brucellosis meningoencephalitis）患儿的临床资料，并结合国内外2014年1月—2020年12月的相关文献，总结儿童布鲁氏菌脑膜脑炎的发病情况、诊断方法及治疗。结果 4岁女性患儿，2021年3月15日因“间断右侧肢体麻木16 h，抽搐2 h”以“癫痫持续状态”入院。入院3个月前曾有2次无热惊厥，曾诊断为癫痫病。本次发病急，伴有低热，以癫痫持续状态为主要表现，脑脊液检测提示中枢神经系统感染，但脑脊液常规及生化无法判断感染性质，进一步通过脑脊液宏基因组测序（metagenomic next generation sequencing）明确感染病原为马耳他布鲁氏菌（Brucella melitensis），通过外周血抗体检测进一步验证，经过有效的抗生素联合足量足疗程治疗，治疗半年后康复。数据库共检索到文献60篇，其中中文文献29篇；期间共报道7例儿童神经系统受累布鲁氏菌病，1例为病例报道，另6例为布鲁氏菌患儿综合分析所提及。结论 儿童布鲁氏菌脑炎或脑膜炎发病率低，发病的临床...     

儿童肺炎支原体脑炎14例临床分析

目的 探讨儿童肺炎支原体(MP)脑炎的临床特点、诊断与治疗.方法 回顾性分析我院2001～2005年确诊的14例MP脑炎患儿的临床资料.结果 MP脑炎大部分并发于呼吸道症状后,2例以神经系统症状首发,脑脊液细胞数一般＜500×106/L,蛋白轻到中度升高,糖和氯化物基本正常,脑脊液MP-IgM阳性率14.28%(2/14).采用抗感染等综合措施,全部病例加用激素,4例重症者辅以丙种球蛋白,14例患者均治愈,近期未见后遗症的发生.结论 MP脑炎可直接以中枢神经系统症状起病,与病毒性脑炎难以区别,脑脊液MP-IgM阳性率低.诊断要综合考虑,在综合治疗的基础上加用激素及丙种球蛋白可减少后遗症的发生.     

小儿病毒性脑炎30例临床分析

目的探讨小儿病毒性脑炎的临床诊断及治疗方法。方法回顾性分析30例临床确诊为病毒性脑炎患儿的临床表现、脑脊液的实验室检查结果、脑电图表现以及CT、MRI表现。结果 30例患儿住院治疗2～3周后,28例（93.3%）患儿获得临床治愈,1例（3.3%）患儿遗留癫痫后遗症,1例（3.3%）患儿死于呼吸衰竭。结论脑电图检查、脑脊液的实验室检查及头颅影像学检查有助于小儿病毒性脑炎的早期诊断,激素、抗病毒、高压氧、退热、抗惊厥、降颅压、维持水电解质平衡及营养支持等积极有效的综合治疗可改善患儿预后,降低病毒性脑炎患儿的致残率和病死率。     

儿童病毒性脑炎89例临床分析

目的探讨儿童病毒性脑炎的临床表现、治疗方法及预后。方法回顾性分析89例病毒性脑炎患儿的临床资料。结果 89例病毒性脑炎患儿以发热、头痛、呕吐为主要临床表现,脑脊液检查阳性率较高。89例中治愈69例,好转16例,4例转上级医院,无死亡病例。少数遗留脑积水、下肢功能障碍等后遗症。结论儿童病毒性脑炎的脑脊液检查较脑电图、脑CT检查阳性率高。临床确诊后及时给予抗病毒、对症治疗预后较好。个别患儿可能遗留神经系统后遗症。     

急性播散性脑脊髓炎30例临床特点及随访分析

目的探讨儿童急性播散性脑脊髓炎（ADEM）的临床特点及转归。方法对30例临床诊断为ADEM的患儿进行1～3a的跟踪随访，并对其临床过程治疗情况及结果进行分析。结果本组春季发病最多（占30％）其次为夏季（占27％）。首发神经系统症状以运动障碍为主（占13％）．28例头颅MPd均表现为颅内多发异常信号〈T1低信号，TZ及FLair像高信号）脑脊髓液常规异常16例（占53％）。均采用糖皮质激素治疗，经1～3a随访，仅4例复发，加用静脉丙种球蛋白治疗，未再复发。均未遗留神经系统后遗症。结论儿童ADEM春季发病多见，多有前期感染或预防接种史，神经系统表现具有多样性，经运动障碍最常见；早期应用糖皮质激素治疗，6个月左右，复发病例加用静脉丙种球蛋白治疗，临床效效果显著。     

儿童单病程及复发性抗N-甲基-D-天冬氨酸受体脑炎临床特征及预后分析

目的 分析儿童单病程及复发性抗N-甲基-D-天冬氨酸受体（N-methyl-D-aspartate receptor,NMDAR）脑炎的临床特征和预后。方法 选取2013年1月至2020年12月在首都儿科研究所附属儿童医院神经内科治疗的抗NMDAR脑炎患儿63例，回顾性分析其中复发（在随访过程中出现≥2次的脑炎表现的病情反复）和无复发（单病程）患儿的临床特征、诊疗情况和预后。结果 随访1～8年，其中复发患儿9例（14.3%），男6例，女3例；初发年龄6.9～13.1岁，平均（9.79±1.68）岁；复发的时间为5～63个月，平均（24.89±17.62）个月。2例（22.2%）患儿≥2次复发。复发患儿初次发病症状最常见的依次为言语障碍8例（88.9%），精神行为异常7例（77.8%），癫痫发作6例（66.7%），不自主运动6例（66.7%）。复发时患儿改良Rankin评分为（2.22±1.10）分，低于初次发病时评分[（3.77±1.12）分]，差异有显著性（P<0.05）。4例（44.4%）患儿初次发作时头颅MRI存在异常。6例（66.7%）患儿初次发病时存在脑脊液白细胞升高。...     

Value of D-Dimers in patients with acute aortic dissection

Objective: To evaluatel the value of D-dimers in patients with acute aortic dissection (AAD). Methods: This study consisted of 16 patients with AAD and 27 non-AAD patients. Serum D-dimets were measured by Sta-Liatest D-DI immunoturbidimetric assay. Results: D-dimer level was higher (P ＜ 0.001) in patients with AAD(7.91 ± 5.52 μg/ml) than that in non- AAD group(1.57±1.24 μg/ml). D-dimer was positive (＞0.4 μg/ml) in all patients with AAD and in 10 control group patients (37%). Among patients with acute AAD, D-dimers tended to be higher in Stanford A than in Stanford B (8.67 ± 4.31 μg/ml vs. 3.24±1.27 μg/ml, P ＜0.01). D-dimer values tended to be higher in more extended disease(3.84 ± 1.65 μg/ml, 8.57 ± 3.58 μg/ml and 11.87 ± 5.69 μg/ml in thoracic aorta, thoracic and abdominal aorta, thoracic and abdominal aorta and iliacal arteries, respectively, P ＜ 0.05 for both 8.57 ± 3.58 and 11.87 ± 5.69 vs. 3.84 ± 1.65 ). Including the control group into the analysis, we found a sensitivity of 100%, a negative predictive value of 100%, and a specificity of 66% and a positive predictive value of 64% for D-dimer in diagnosis of AAD in our patients with suspected AAD. Conclusion: D-dimer was elevated in patients with AAD. A negative D-dimer test result could be useful in excluding AAD.     

Research of combined liver-kidney transplantation model in rats

Objective： To set up a simple and reliable rat model of combined liver-kidney transplantation. Methods： SD rats served as both donors and recipients. 4℃ sodium lactate Ringer＇s was infused from portal veins to donated livers,and from abdominal aorta to donated kidneys, respectively. Anastomosis of the portal vein and the inferior vena cava （IVC） inferior to the right kidney between the graft and the recipient was performed by a double cuff method, then the superior hepatic vena cava with suture. A patch of donated renal artery was anastomosed to the recipient abdominal aorta. The urethra and bile duct were reconstructed with a simple inside bracket. Results： Among 65 cases of combined liver-kidney transplantation, the success rate in the late 40 cases was 77.5%. The function of the grafted liver and kidney remained normal. Conclusion： This rat model of combined liver-kidney transplantation can be established in common laboratory conditions with high success rate and meet the needs of renal transplantation experiment.     

BLOOD PRESSURE CHANGE WITH AGE IN SALT-SENSITIVE TEENAGERS

Objective To observe blood pressure change with age in salt-sensitive teenagers whose salt sensitivity were determined by repeated testing.Methods Salt sensitivity was determined through intravenous infusion of normal saline combined with volume-depletion by oral diuretic furosemide in 55 teenagers. After five years, salt sensitivity was re-examined and subject blood pressure was followed up. Blood pressure changes in salt-sensitive teenagers were compared to that of non-salt sensitive teenagers over five years.Results After 5 years, the repetition rate of salt sensitivity determined by intravenous saline loading is 92.7%. In teenagers with salt sensitivity on the baseline, both the systolic blood pressure increments and increment rates were much higher than non-salt sensitive teenagers (12.7±12.1 mmHg vs. 2.8±5.2 mmHg, P＜ 0.01; 12.2%± 12.0% vs. 2.5% ±4.4%, P＜ 0.001,respectively). There was a similar trend for diastolic blood pressure (8.4 ± 6.4 mmHg vs. 3.7 ± 6.4 mmHg, P = 0.052; 13.2% ±10.6 % vs. 6.8%± 10.1%, P = 0.053, respectively).Conclusions Salt sensitivity determined by intravenous saline loading showed good reproducibility. Blood pressure increments with age were much higher in salt-sensitive teenagers than non-salt sensitive teenagers, especially in terms of systolic blood pressure.     

安心颗粒对急诊经皮冠状动脉介入术后生活质量影响的研究

目的：评价使用安心颗粒对急诊经皮冠状动脉介入术（PPCI）术后生活质量的影响.方法：将160例接受PPCI的急性ST段抬高型心肌梗死患者随机分为安心颗粒组（术前顿服安心颗粒8.8g,术后安心颗粒4.4 g/次,每日2次）和对照组（仅接受基础药物治疗）.所有患者均服用阿司匹林、氯吡格雷和阿托伐他汀.分别在入院时、出院前1d、出院后180 d时,应用心肌梗死多维度量表（MIDAS）、中文版SF-36评价量表对患者生活质量评分.并观察术后30 d以内的出血并发症、血小板减少症发生情况.结果：入院时和出院前1d,两组患者的心肌梗死MIDAS、SF-36量表评分比较无差异（P＞0.05）;出院后180 d时,与对照组比较,安心颗粒组MIDAS、SF-36评分明显减低（P＜0.05）;组内与入院时比较,两组出院前1d、出院后180 d时,MIDAS、SF-36评分均降低（P＜0.05）.两组患者在随访期间均无大量出血、少量出血、重度和极重度血小板减少症发生,安心颗粒组有4例、对照组有7例发生不明显出血（P＞0.05）.两组发生轻度血小板减少症的患者数比较无差异（P＞0.05）.结论：PPCI使用安心颗粒,能改善急性ST段抬高型心肌梗死患者的生活质量,且不增加出血风险.     

The comparative studies of the influences of Urapidil and Nicardipine on sino-atrial node function, atrio-ventricular node function and hemodynamics

Objective:To investigate the influences of urapidil and nicardipine on rabbit sinus function,atrio-ventricular node function and hemodynamics.Methods:Thirty-two Angora's rabbits were selected and randomly divided into four groups.U1 group:urapidil 0.25 mg/kg;U2 group:urapidil 0.5 mg/kg;N1 group:nicardipine 10 μg/kg;N2 group:nicardipine 20 μg/kg.All these medicine were administrated within 30 seconds.Measurements were taken before and after the administration of urapidil or nicardipine for the following data:mean blood pressure(MAP),heart rate(HR),sino-atrial conduction time(SACT),maximal sinoatrial recovery time(SNRTmax)corrected sinus node recovery time(CSNRT),index of sinus node recovery time(SNRTI),Wenckebach A-V conduction frequency (WB),and P-R interval.Results:Significant MAP and HR changes were identified in all of the four groups before and after administration of both urapidil and nicardipine.No significant changes could be found in the rest of the parameters.Intergroup analysis showed that SACT and CSNRT of N1 and N2 groups were shorter than those of the U2 group(P＜0.01);the MAP decreased(P＜0.01)and the HR increased drastically(P＜0.01).Conclusions:Neither urapidil(0.25 mg/kg,0.5 mg/kg)nor nicardipine(10μg/kg,20μg/kg)has any significant influence on rabbit sinus function or rabbit atrio-ventricular node function.Nicardipine could be a better choice than urapidil for parafunctional sinus node patients.     

Expression of OPGmRNA and ODFmRNA in the patients of hip fracture due to osteoporosis

Objective：To investigate the gene expression of osteoprotegerin（OPG） and osteoclast differentiation factor（ODF） in the bone tissue of patients with hip fracture due to osteoporosis. Methods：OPGmRNA and ODFmRNA in the bone tissue in 50 cases of osteoporosis sufferers（over 50 years old） with hip fracture（Observer Group） and 30 cases of hip facture sufferers with no osteoporosis（Control group） were analyzed with the Semi-Quantitative RT-PCR method. Results：The mRNA expressed of ODF, OPG were both high in the patients with hip fracture. In the control group, the expression of OPG mRNA was observed, while the expression of ODF mRNA was very slight. Conclusion：Aged patients contained all signals including OPG, ODF that are essential for inducing osteoclastogenesis and promoting bone resorption.     

Dynamic Changes in Serum Estradiol and Progesterone levels in Patients of Premenstrual Syndrome with Adverse Flow of Liver-qi

Objective:To probe into the influence of changes of ovarian hormones on the pathogenesis of the specific sub-type premenstrual syndrome(PMS)and reveal partial microcosmic mechanisms of adverse flow of liver-qi.Methods:Estradiol(E2)and progesterone(P)levels in serum were determined at different phases of menstrual cycle by radioimmunoassay.Results:In the group of PMS with adverse flow of liver-qi.the secretive peak value Of E2 and P at the follicular phase significantly decreased,and the secretive peak value at the luteal phase did not come into being.Conclusions:Low E2 and P secretive peak at the follicular phase and absence of secretive peak at the luteal phase is one of the microcosmic mechanisms of PMS with adverse flow of liver-qi.One of the pathophysiologic mechanisms of specific sub-type PMS is probably the continuous low level of E2and P.     

Real-time Three-dimensional Echocardiography in Assessment of Left Ventricular and Right Ventricular Volumes

Real-time three-dimensional echocardiography (RT3DE)is a new ultrasound technique that enables dynamic threedimensional visualization and quantification of the heart in real time. Investigation of feasibility and methodology of RT3DE in determining left ventricular (LV) and right ventricular (RV) volumes, RT3DE was performed in 35 normal adults using Philips SONOS 7500 system with a 2-4 MHz matrix array transducer. The 60°×60° "pyramid" volume database was obtained and analyzed on a TomTec echo workstation. Both LV and RV volumes were calculated with four 3DE methods (i.e. apical 2, 4, 8, and 16-plane) through manually tracing ventricular endocardial borders in end diastole and end systole. Stroke volumes were then calculated. LV volume was also measured by 2DE Simpson's rule using GE VIVID 7 ultrasound machine.     

SCREENING FOR A 21-CHROMOSOME ABNORMALITY IN PREIMPLANTED EMBRYOS OF ELDERLY WOMEN

Increasing maternal age is the only etiological factor unequivocally linked to Down's syndrome in humans. The occurrence rate of newborns with Down's syndrome is about 1/220 in women over 35 years old. However, the occurrence rate in embryos fertilized in vitro, of the elder woman is unclear. Using FISH we screened the number of chromosome 21 in preimplanted embryos of 5 elderly women (average age, 38.4 years) to study the feasibility and necessity of screening trisomy 21 in embryos in patients over 35 years old at the in vitro fertilization (IVF) center.     

Scientific principles and rigorous processes should be followed in developing clinical guidelines for therapeutic interventions of integrative medicine

A clinical guideline for the therapeutic interventions of integrative medicine may be defined as a written document which states a series of recommendations on therapeutic interventions of integrative medicine for a special disease or condition. The guideline may provide assistance to medical professionals in making clinical decisions aimed at improving the clinical outcome of patients and reducing the costs of medical care（~＇4~. Recommendations issued by a guideline should be based on the best available evidence in both Western and Chinese medicine. For fulfilling this purpose, the development of clinical guidelines for therapeutic interventions in the field of integrative medicine should follow scientific principles and undergo a rigorous processes.