丘脑肿瘤的临床特征和外科治疗

目的 研究儿童丘脑肿瘤的临床特征和外科治疗效果。方法 回顾性研究手术治疗丘脑肿瘤儿童22例的临床资料，并进行6个月～9年随访。结果 丘脑肿瘤儿童平均年龄9岁，男童略多于女童，病程约2个月，头痛和视乳头水肿为最常见的症状和体征，病理类型以低级别的胶质瘤最常见，大部分丘脑肿瘤影像表现边界较清。显微外科治疗肿瘤全切9例，次全切8例，部分切除3例，活检2例。术后症状和体征好转10例，不变8例，加重4例，无手术死亡。结论 儿童丘脑肿瘤临床特征明显，手术切除近期疗效肯定，远期疗效取决于病理类型。     

急性播散性脑脊髓炎94例临床与影像学分析

目的 探讨急性播散性脑脊髓炎(ADEM)的临床特征、影像学表现及与临床的关系.方法 分析2006年1月-2009年8月94例ADEM患儿的流行病学、临床表现、实验室检查、影像学特点、治疗及预后转归情况.将患儿分为昏迷组和无昏迷组,分别统计2组的病变部位及例数,采用χ2检验比较2组病变部位之间有无统计学差异.结果 患儿发病年龄1岁1个月～16 岁,平均7.0岁.男53例,女41例,脑病是突出症状,发生率为87.2%.重症出现昏迷,从发病至昏迷平均3.1 d.主要症状有发热、头痛/呕吐、抽搐、肢体瘫痪、颅神经麻痹、视力受累等.常出现锥体束征和脑膜刺激征.61例行脑脊液检查,其中34例(55.7%)脑脊液细胞数升高[(10～385)×106 L-1)],主要为淋巴细胞升高.颅内多个部位可受累,其中受累频率高者有多个脑叶、双侧背侧丘脑、双侧基底核、侧脑室旁、中脑、脑桥及小脑.昏迷组双侧丘脑受累的发生率为56.5%,无昏迷组为25.4%,差异有统计学意义(χ2=7.152,P<0.01).其他部位受累的发生率2组比较差异无统计学意义.绝大部分患者(88例)接受甲泼尼龙冲击治疗及口服泼尼松维持治疗,自起病至接受治疗平均 15.1 d.66%的患儿出院时恢复较好,行走无需帮助.结论 儿童ADEM男童略多于女童,临床特征复杂多样,病情轻重不一;颅脑MRI检查有助于及时明确诊断;双侧丘脑同时受累者常出现昏迷.及时恰当的治疗对预后极为重要.     

儿童关节内/近关节与关节外骨样骨瘤的特点及手术疗效分析

目的通过对比骨骼未成熟儿童关节内/近关节与关节外骨样骨瘤的临床及影像学特点,探讨儿童关节内/近关节部位骨样骨瘤的临床特点、手术疗效及预后。方法对复旦大学附属儿科医院收治的43例儿童骨样骨瘤病例的临床资料进行分析,其中男31例,女12例,平均年龄8.9岁(1.8～16岁)。根据骨样骨瘤瘤巢部位分成关节内/近关节组(16例)和关节外组(27例),回顾以上病例的临床特点、影像学特点及治疗方法,运用SPSS22.0进行统计学分析。结果关节内/近关节组16例中仅6例存在骨样骨瘤的典型症状,13例存在关节滑膜炎症状,10例误诊,9例存在特征性瘤巢,8例关节周围骨骼出现异常改变;关节内/近关节组平均延迟诊断17个月,与关节外组对比在延迟诊断时间上存在统计学差异(P 0.05)。关节外的骨样骨瘤临床症状和影像学表现更典型,肿瘤受累骨骼存在过度生长现象,但未见跛行等显著临床症状。结论儿童关节内/近关节骨样骨瘤与关节外的骨样骨瘤存在不同的临床表现及影像学特征,同时缺乏典型的骨样骨瘤临床特征,而滑膜炎表现相对较多,跛行或者骨膜反应程度轻,硬化骨不显著,需要与炎症、剥脱性骨软骨炎等鉴别诊断,同时需要更精准的手术治疗。     

抗MOG抗体阳性儿童脑皮质脑炎1例报告并文献复习

目的探讨抗MOG抗体阳性皮质脑炎的临床特征。方法回顾分析1例确诊MOG抗体阳性皮质脑炎患儿的临床资料,并以"MOG"、"脑炎"为检索词,检索并复习近10年报道的相关病例。结果女性患儿,10岁,以癫痫发作(局灶性发作)为主要表现。颅脑磁共振成像(MRI)示额顶叶皮质受累。予抗癫痫药物及糖皮质激素治疗后临床症状及颅脑MRI改变消失。结论癫痫发作、发热及皮质综合征且头颅MRI示脑皮质损害者,应注意筛查MOG抗体。     

以小脑及脑干损害为首发表现的山豆根中毒一例并文献复习

目的探讨山豆根中毒并小脑及脑干损害的临床特点和治疗。方法对安徽医科大学第一附属医院儿童重症监护病房2021年5月确诊1例山豆根中毒并脑干及小脑损害患儿的临床资料、实验室检查、影像学检查等资料进行收集及回顾性分析。以"山豆根""中枢神经损害""小脑齿状核"为检索词, 分别在"中国期刊全文数据库""万方数据知识服务平台"建库至2021年4月进行文献检索并文献复习, 排除成人病例、资料不全儿童病例。总结山豆根中毒合并脑干及小脑损害的临床特点、影像学改变、治疗及预后。结果患儿, 女, 5岁, 因"吞咽困难、嗜睡、站立不稳2 d"入院。伴饮水呛咳、言语不清、表情呆滞。无抽搐发作。既往史及家族史无异常。辅助检查示脑脊液常规、生化正常, 宏基因二代测序阴性, 头颅核磁共振成像加增强:双侧齿状核及脑桥片状T1低信号T2高信号, 诊断脑干脑炎, 小脑炎, 给予降颅压、抗病毒治疗后症状缓解缓慢。完善基因检测阴性, 毒物筛查提示山豆根中毒。文献检索加本例共收集18例病例, 分别为山豆根中毒并小脑损伤15例, 山豆根中毒并小脑及脑干损伤3例。表现为步态不稳、言语不清、意识改变等, 神经系统受累程度重。头颅核磁...     

婴儿流行性乙型脑炎45例

目的探讨婴儿流行性乙型脑炎(乙脑)的临床特点。方法随机选择1岁以下的乙脑患儿45例为观察组,1岁以上的乙脑患儿85例为对照组,分别对其流行病学特征、临床表现、实验室检查结果等进行对比分析。结果1岁以下乙脑发生意识障碍、呼吸衰竭、体征及血白细胞升高比例与1岁以上乙脑比较具有明显差异(Ρ均<0.05)。两组发病季节、主要临床特征、脑脊液变化及特异性乙脑抗体IgM阳性率比较均无明显差异(Ρ均>0.05)。结论婴儿乙脑脑膜刺激征、巴氏征、神经反射及典型的血象改变与1岁以上婴儿乙脑临床表现不完全相符,临床医师不应以脑膜刺激征、巴氏征和神经反射的改变作为婴儿乙脑诊断的必备体征。     

肺炎支原体肺炎患儿血清NO、TNF-α及IL-2水平测定及意义

临床资料 :选择 1998年 7月至1999年 7月在我院住院确诊肺炎支原体肺炎 (ＭＰＰ)患儿共 38例为研究对象(病例组 ) ,男 18例 ,女 2 0例 ,年龄 5～14岁 ,平均 (9± 3)岁。ＭＰＰ临床诊断标准依第 6版《实用儿科学》 ,微量颗粒凝集法测定血清ＭＰ ＩｇＭ抗体≥ 1∶40阳性。急性期为自发病到入院时间≤10ｄ ,恢复期为临床症状、体征及胸部Ｘ线征像消失或明显减轻。根据有无肺外并发症分为Ⅰ组 (2 4例 )与Ⅱ组 (14例 )。Ⅰ组 :单纯肺部受累和 (或 )伴轻微皮肤、消化道受累 ;Ⅱ组 :除肺部受累外伴其它如神经、心脏、肾、肝及血液等肺外系统受累。…     

白质消融性白质脑病临床分析

目的分析白质消融性白质脑病的临床特点及诊断方法。方法对9例临床诊断为该病的患儿的临床及头颅影像学特点、实验室检查等进行分析,结合病例对该病进行综述介绍。结果（1）临床表现：9例中8例出现神经系统症状和体征,1例仅有MRI异常。发病时间为生后6个月-3岁;5例家族史阳性,病前智力运动发育多正常;多以运动症状起病,下肢为著。6例发病前或每遇病情加重前有呼吸道感染史,3例病前有轻微头部外伤史;到目前为止,有症状的7例呈进行性加重病程,其中4例同时有发作性加重现象。所有病例智力受损相对轻。7例头围正常,8例有上运动单元病变体征,4例有共济失调体征。3例双侧视神经萎缩。（2）头颅MRI：表现为双侧对称弥漫性深部白质长T1长T2信号,可累及皮层下白质,额、顶叶为主,Flair像为对称性白质高信号伴部分白质低信号甚至囊性变或大部分白质低信号仅存少量线状稍高信号,为此症特征性改变。（3）其他遗传性白质脑病相关酶学或生化检查均正常。结论以运动障碍起病、运动障碍重于智力障碍、神经影像学改变显著重于临床表现、MRI表现为双侧对称弥漫性深部白质长T1长T2病变伴早期出现白质消融征象是本症的临床特点,临床诊断还需除外其他遗传性及获得性脑白质病。找到相应基因的致病突变可最终确诊。     

儿童肺炎支原体感染相关性脑梗死

目的探讨儿童肺炎支原体(MP)感染相关性脑梗死的发病机制、临床表现、影像学特点及预后。方法回顾性总结分析2008年6月-2011年12月本院收治的22例MP感染并脑梗死患儿的临床表现、影像学改变、实验室检查结果、治疗及预后,复习相关文献,并进行比较。结果患儿中女童(15例)多于男童(7例),平均发病年龄为6.82岁(13个月～13岁),68.2%(15/22例)的患儿存在呼吸道感染表现。血清MP抗体阳性检出率为100%,而脑脊液阳性率相当低(1/22例)。肺部影像学提示肺炎改变7例(32%)。头部影像学以缺血性梗死为主(20/22例),出血性梗死少见(3/22例)。病灶主要位于基底核,占63.6%(14/22例)。头颅磁共振脑血管成像(MRA)大多(11/18例)异常,以大脑前循环动脉异常为主,其中大脑中动脉占54.5%。经内科保守治疗,患儿临床症状、体征均明显改善。结论儿童MP感染可并脑梗死,呼吸道感染表现可有可无,临床主要表现为病变对侧肢体活动障碍及中枢性面瘫,头颅MRA多表现为大脑前循环异常,基底核、颞叶、丘脑为常见梗死区,经内科保守治疗预后良好。     

肺炎支原体肺炎286例临床特点及肺外并发症分析

目的分析肺炎支原体肺炎临床特点及肺外器官受损的特点及预后。方法采用ELISA在病程4-11天测血清MP-IgM均阳性结合X线胸片、心电图、血尿粪常规、痰培养等临床及化验室资料进行综合分析。结果286例中单纯肺炎者180例，合并肺外器官受累者106例占37．1％，其中以消化、泌尿、心血管系统及浆膜腔积液多见。均经红霉素、阿奇霉素治疗，疗程3-4周。结论肺炎支原体肺炎临床多表现症状重，体征轻，且可引起较多系统的肺外并发症，应引起临床医生对该病的重视。     

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??Abstract?? Objective To identify the clinical features and the prognosis of anti-NMDAR encephalitis in children??so as to improve the pediatrician’s awareness about this disease. Methods The clinical features??therapeutic regimen and prognosis of 17 children with anti-NMDAR encephalitis were summarized by a retrospective and follow-up study. Results Among the 17 patients with anti-NMDAR encephalitis??12 were males and 5 were females.Their age varied from 2 to 12 years??and the average age was 5 years 8 months.The most common initial symptoms were convulsions ??7 cases?? and psychiatric symptoms ??7 cases??.The common symptoms in the course of the disease were psychiatric symptoms??17 cases????sleep disorders ??16 cases????extrapyramidal symptoms ??14 cases????and convulsion ??11 cases??.Only 4 patients had autonomic symptoms.The anti-NMDAR antibody were found in all the patients’CSF.The EEG test of 16 patients showed diffused slow background.Tumors were not found in any patient. Fourteen cases were treated with IVIg and methylprednisolone??2 cases only received IVIg therapy and 1 case gave up.Rituximab ??2 cases?? and cyclophosphamide ??1 cases?? were administrated in 3 cases with no improvement after the above treatment.The patients had been followed up for 3 to 15 months.Twelve of them recovered completely??2 cases died??2 cases had epilepsy??and 1 case had speech disorder. Conclusion Psychiatric symptoms??sleep disorders??extrapyramidal symptoms and convulsion are the common symptoms of anti-NMDAR encephalitis in children.Tumors and autonomic nervous system dysfunction rarely happen. Immunotherapy is effective in the majority of the patients.The prognosis of anti-NMDAR encephalitis in children is optimistic.     

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??Objective??To analyze the features of clinical manifestions and gene mutations of Chinesse children with X-linked Alport syndrome??XLAS??. Methods??Retrospectively analyze the clinical and pathological features of 31 patients with Alport syndrome with COL4A5 mutations??who were treated in Shanghai Children’s Hospital from June 2011 to June 2016. Results??Of these 31 cases??there were 12??38.7%?? females and 19??61.3%?? males??and the average age of onset was 2.6 years old. Thirteen patients had an onset of hematuria and proteinuria and 22??70.9%?? patients had family medical history. One patient presented ocular changes and 2 patients had hearing loss. Renal pathology showed that 15 of them had minimal change disease??MCD?? and 5 mesangial proliferative glomerulonephritis??and only 6 had typical pathologic changes of Alport syndrome. We identified 31 different mutations in all patients??and there were 19??61.3%?? cases of missense mutations??2??6.5%??cases of large deletion mutations??4??12.9%?? cases of spice-site mutations and 6??19.3%?? cases of frame shift mutations. Among these 19 missense mutations??16??84.2%?? cases were Gly-X-Y mutations. Conclusion??Most of XLAS children show MCD in renal biopsy and had missense mutations in COL4A5 gene. Clinical symptoms and pathology are not typical?? resulting in the difficulities in diagnosis of Alport syndrome.     

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??Abstract??Objective??To identify the clinical features of opsoclonus-myoclonus syndrome and its response to adrenocorticotropic hormone ??ACTH??. Methods??Fourteen OMS cases who were diagnosed during 2006??2010 in Peking University First Hospital were enrolled in this study. Data on medical history?? neurological signs??laboratory tests??response to ACTH and the relapse were evaluated. Results??Among 14 cases?? eight were male and six were female. Age at presentation ranged from 12 to 44 months ??average 20.7 months??. Main symptoms at presentation were opsoclonus ??14/14????myoclonus and ataxia ??14/14????poor sleeping ??14/14????irritability ??14/14??. Neuroblastoma was found in one of the fourteen cases. EEGs of all showed no epileptic discharges. Before the diagnosis of OMS??7 cases were ever misdiagnosed as acute cerebellar ataxia??4 were misdiagnosed as epilepsy?? 4 were misdiagnosed as encephalitis and 1 was misdiagnosed as hereditary metabolic disease. All patients received ACTH therapy. Neurologic function improved in all children?? but seven children had relapse within 3??12months ??average 5.7 months??. Conclusion??OMS is a rare autoimmune neurological disorder which is often present in young chlidren.Neuroblastoma is common in children with OMS.OMS is characterized by rapid??involuntary and irregular conjugate eye movements ??opsoclonus????myoclonic jerking of the limbs and trunk??ataxia??poor sleep and behavioral disturbances. Due to the possible immune-mediated mechanisms?? treatment with ACTH can be successful?? but relapse rate is high and the outcome is unfavourable. Neurological sequelae such as behavioral?? language and cognitive problems occur in the majority.     

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??Abstracts?? Objective To analyze the clinical characteristics and prognosis of acute encephalitis with visual impairment in children. Methods The clinical features?? including clinical manifestations??laboratory tests??Flash visual evoked potential and cranial MRI?? in 22 patients diagnosed with acute encephalitis with visual impairment in children were retrospectively analyzed. Results The disease course was less than one week in 10 cases. The disease course of eight cases was between one week and two weeks?? two cases were between two weeks and four weeks?? two cases were over four weeks. Seven cases had visual impairment as the first symptom. One case was with the diagnosis of viral encephalitis with cortical blindness. Nine cases were with the diagnosis of viral encephalitis with optic neuritis. One case was with the diagnosis of encephalitis with neuropapillitis. One case was with the diagnosis of disseminated encephalomyelitis with optic neuritis. Two cases were with the diagnosis of disseminated encephalomyelitis with optic neuritis. One case was with the diagnosis of brainstem encephalitis with exposure keratitis. One case was with the diagnosis of encephalitis with lens opacity and retinal dysplasia. One case was with the diagnosis of ophthalmoneuromyelitis. One case was likely with the diagnosis of Vogt-Koyanagi-Harada syndrome. In all the cases of Flash visual evoked potential??eighteen were abnormal. In the ten cases of fundus photography??seven were abnormal. In all the cases of cranial MRI??ten were abnormal and six suffered from extensive pathological changes in the brain. In the eighteen cases of hormone therapy??fourteen had good effects. The time of post hospitalization follow-up ranged from 6 months to 8 years. The prognosis of 20 patients was good. Two patients’ prognosis was bad. Conclusions There are different kinds of etiology and diseases in children who are diagnosed with acute encephalitis with visual impairment. Flash visual evoked potential and fundus photography are of important significance in the early stages. High doses of IVIG corticosteroids and hyperbaric oxygen therapy play a key role in improving the prognosis of disease.     

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??Objective??To analyze the clinical features and the results of genetic diagnosis in children with hypokalemic renal tubular diseases. Methods??The clinical data of 38 patients with hypokalemic renal tubular diseases were analyzed retrospectively??who were treated in Children’s Hospital Affiliated to Shanghai Jiao Tong University from Jan. 2010 to Jan. 2016. Results??Totally 38 patients with hypokalemic renal tubular diseases were enrolled in this study. There were 18 cases of renal tubular acidosis??RTA?? including 17 cases of type??RTA and 1 case of type?? RTA. There were 11 cases of Bartter syndrome??5 cases of Gitelman syndrome and 4 cases of Fanconi syndrome. The common clinical manifestations of hypokalemic renal tubular diseases included myasthenia??nausea??vomiting??polydipsia??polyurine and growth retardation. One case of Fanconi syndrome progressed to chronic Kidney disease??phase ????while the other
children had normal renal function. Glomerular proteinuria was found in 1??1 and 3 children with Bartter syndrome??Gitelman syndrome and Fanconi syndrome??respectively. Additionally??1 case with Fanconi syndrome has tubular proteinuria. However??urinary trace proteins associated with glomerular and tubular injury commonly elevated in these hypokalemic renal tubular diseases. Genetic analysis showed a new potential heterozygous mutations of ATPV0A4 in type??RTA and three heterozygous mutations of SLC12A3 in Gitelman syndrome. Conclusion??The clinical symptoms vary in patients and are featured mainly by myasthenia??nausea??vomiting??polydipsia??polyurine and growth retardation. Glomerular and tubular injuries are commonly found in hypokalemic renal tubular diseases. Moreover??genetic diagnosis may be helpful in diagnosis??treatment and genetic counseling.     

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??Objective To study the features of lung ultrasonography??LUS?? in lung diseases and evaluate the value of LUS in pediatric intensive care unit??PICU??. Methods Totally 137 children hospitalized from May 1??2015 to August 31??2015??in pediatric intensive care unit of Bayi Children’s Hospital?? General Hospital of Beijing Military Command?? were included in this study. All the children were examined by LUS and diagnosed by medical history?? clinical manifestation?? laboratory examination?? signs of X-ray or chest CT. The results of LUS were compared with them. Results There were 89 cases??66.7%?? who were lung diseases and 48 cases??33.3%?? without lung diseases. The cases with lung diseases were as follows?? 76 cases??85.4%?? with pneumonia?? 8 cases??9.0%?? with capillary bronchitis and 5 cases??5.6%?? with acute respiratory distress syndrome??ARDS??. The main characteristics of children with lung diseases on LUS were as follows?? A-lines disappearance?? pleural line abnormalities?? lung consolidation?? interstitial syndrome?? lung pulse and pulmonary edema. The signs of pleural line?? A-lines?? lung consolidation?? interstitial syndrome?? pulmonary edema and lung pulse could be used to monitor the conditions of children with lung diseases. Futhermore?? compared with by X-ray?? it was much easier to find the lesions of patients by LUS who failed to be withdrawn ventilator. The features of LUS in children with no lung diseases included pleural line and A-lines??but did not include abonormal signs of B-lines?? interstitial syndrome and lung consolidation. Conclusion The diagnosis of LUS is precise in lung diseases. LUS has more practical value?? especially for patients with condition waving?? and those with withdrawingofmechanical ventilation. Moreover?? LUS has more advantages?? including no radiation and monitoring the diseases on bed without interfering the treatment. Therefore?? it is necessary that LUS should be routinely used in PICU.     

儿童病毒性脑炎合并呼吸衰竭64例诊断与治疗

目的：总结儿童病毒性脑炎合并呼吸衰竭的临床特点,为早期诊断和有效治疗提供经验。方法回顾性分析2005年5月至2015年5月天津市儿童医院神经内科病房收治的64例病毒性脑炎合并呼吸衰竭患儿的临床资料。结果所有患儿均为急性起病,以发热(60例,93．7%),抽搐(50例,78．1%),意识障碍(46例,71．8%),锥体束征阳性(30例,46．8%)等为主要临床表现,多于急性期发生呼吸衰竭。中枢性呼吸衰竭56例,中枢性呼吸衰竭合并周围性呼吸衰竭6例,呼吸循环衰竭2例。46例行腰椎穿刺术,32例异常,颅内压增高者10例,白细胞数增多19例,蛋白定量升高17例,脑脊液病原学阳性10例,其中单纯疱疹病毒8例,EB病毒1例,柯萨奇病毒1例。所有患儿行头CT检查,6例异常;34例行MRI检查,29例异常。所有患儿脑电图均不正常,48例(75%)患儿首次脑电图示全导弥漫性δ活动;22例患儿治疗期间监测伴有局灶或全导痫性/样放电。5例患儿电生理检查示颈段脊髓前角损害。24例患儿并发应激性溃疡,肝损害4例,心脏损害6例,肾损害4例,肺损害1例。所有患儿均行机械通气,带机时间2~50 d。33例(51．5%)好转出院,14例死亡,放弃治疗17例。25例患儿遗留多种神经功能障碍,14例继发性癫痫患儿于门诊随诊3个月至6年,其中8例为药物难治性癫痫。结论儿童病毒性脑炎合并呼吸衰竭患儿起病急,进展快,致残率、病死率高。早期识别,及时呼吸支持,注意保护脏器功能,有助于改善预后。     

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??Objective To compare the etiology and clinical characteristics of clinically mild encephalitis/encephalopathy with a reversible splenial lesion of corpus callosum in Chinese children with those cases from different countries. Methods The clinical and imaging features of two children with the diagnosis of MERS were analyzed and pediatric MERS cases were screened from “China National Knowledge Infrastructure”??“China Biology Medicine”??“PubMed ” and “Web of Science” databases for analysis between January??2000 and February??2015. Results A total of 115 cases were collected and the male to female ratio was 1.1 to 1?? with onset age of 6 months to 14 years old. There was usually a certain infection before the onset. About 58.3% of infectious pathogens were detected?? and influenza virus and rotavirus infection were very common pathogens?? followed by bacterial infections and mycoplasma pneumoniae infection. The neurological symptoms manifested as mental and behavioral disorders ??42.6%???? seizures ??37.4%???? decreased consciousness ??5.2%?? or consciousness??25.2%?? and dysarthria ??6.9%??. The MR imaging ??MRI?? findings exhibited lesions in an isolated splenial or the white matter and entire corpus callosum?? and completely disappeared at follow-up MRI. With anti-infection and symptomatic treatments??the clinical symptoms of most patients were relieved without leaving any serious neurological sequelae. Conclusion Infection is a prevalent predisposing factor of MERS in children?? with nonspecific clinical characteristics. The MRI examination plays a role of implication for diagnosis. And all clinical symptoms of patients will disappear within a few days and the prognosis of patients is good.     

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??Objective??To explore the clinical characteristics of anti-N-methyl-D-aspartate receptor??anti-NMDAR??encephalitis in children. Methods??Between April 2015 and December 2015??fifteen children were diagnosed with this disorder at the Department of Neurology??Children’s Hospital of Fudan University. We retrospectively analyzed the data of clinical characteristics and laboratory examinations. Results????1??There were seven females and eight males??the median age of onset was 7 years old??ranging from 8 months to 12 years.??2??Clinical characteristics were as follows?? 3/15??20%?? had impaired consciousness along with convulsion?? and 4/15??26.7%?? had emotional and behavioral changes at the onset of disease. Seizures occurred in all the 15 children and status epilepticus in 3 cases. Facial and limb involuntary movements occurred in 12/15??80%??. Seven cases??46.7%?? demonstrated uroschesis. Three had hypoventilation. Two??13.3%?? were secondary to virus infection.??3??Imaging and laboratory examination showed that the white blood cell of cerebrospinal fluid??CSF?? in 5/15 cases??33.3%?? were moderately elevated. The protein of cerebrospinal fluid??CSF?? was obviously increased in 2 patient??13.3%??. IgG index was detected in 10 patients and increased in 4/10??40%??. All had cerebrospinal fluid antibodies. MRI was abnormal in 10/15??66.7%??. EEG typically showed diffuse background slowing??while no extreme delta brush was observed. One case??6.7%?? had a mass in ovary. All patients received intravenous methylprednisolone and immunoglobulins??IVIG??. One case??6.7%?? received plasma exchange. Follow-ups lasted for 1 to 6 months. The functional outcomes of 9 patients were favorable??mRS being 0 to 2. Conclusion??Due to the lack of specificity of clinical symptoms and laboratory examination??this disorder is difficult to diagnose at the onset. The disease can be diagnosed by the specific anti-NMDAR antibody in the cerebrospinal fluid. Tumors and delt brush are rarely found in children with anti-NMDAR encephalitis. Early diagnosis and timely initiation of immunosuppressive treatment will result in favorable outcome in the majority of the patients.     

儿童冠状动脉起源异常合并心肌损害临床特征及诊治分析（附72例报告）

目的　探讨冠状动脉起源异常合并心肌损害的临床特点及相应治疗策略,提高临床医师对此类疾病的认识和诊治水平。方法　回顾性分析2015-2017年首都医科大学附属北京儿童医院心脏内科诊治的72例冠状动脉起源异常合并心肌损害患儿的临床资料,总结其临床特点和诊疗方法。结果　本组42例（58.3%）患儿以胸痛、胸闷、心悸为主要临床表现,心电图异常以ST-T改变（62.5%）为主,超声心动图结果示13例（18.1%）患儿合并心脏扩大,4例（5.6%）合并左室收缩功能减低。冠状动脉CT血管造影检查（CTA）结果示左冠状动脉起源异常最为常见（73.6%）,其次为双侧冠状动脉起源异常（13.9%）,单纯右冠状动脉起源异常最少见（12.5%）。所有患儿诊治过程顺利,无死亡病例。结论　冠状动脉起源异常可导致不同程度心肌供血问题,引起缺血性心肌损害,在患者的检查中更应重视CTA对冠状动脉开口位置和走行的判断,在诊断治疗中更应注意其与儿童心肌炎的鉴别。