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??Gastrointestinal??GI?? endoscopy plays an indispensable role in the diagnosis and management of various pediatric GI disorders. Complications during the operation of digestive endoscopy are unavoidable. The incidence of complications of diagnostic endoscopy in children is very low??but the incidence of complications is obviously increased in recent years??because of the development of endoscopic treatment in children. Following strict operation indications and contraindications of GI endoscopy??standardized operation process??and skilled pediatric endoscopists are the key to reducing complications. Early identification and timely management of complications after endoscopy is essential to ensuring the safety of children and maintaining harmonious doctor-patient relationship.     

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(上接本刊2006年第6期477页)4表皮样囊肿、皮样囊肿和畸胎瘤表皮样囊肿、皮样囊肿和畸胎瘤属先天性肿瘤。其中表皮样囊肿较多见,又称表皮样瘤、胆脂瘤、真性胆脂瘤或珍珠瘤,由残留原始外胚层细胞发展而来。占中枢神经系统肿瘤的1.39%。男性略多于女性,男女比例为1.2∶1。各年龄段均可发病,以20～50岁为最多见。皮样囊肿比表皮样囊肿更少见,约占颅内肿瘤的1%,可发生于任何年龄,20岁以下多见。畸胎瘤只占颅内肿瘤的0.5%,可发生于任何年龄,但小儿和青年人约占70%,男女比例为2.06∶1。4.1临床与病理表皮样囊肿常位于中线外侧。可位于脑内,如第四…     

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??Children partial epilepsy manifesting hypermotor seizures?? analysis of 7 cases and literatures review.????CHEN Qian??ZHANG Gui-zhen??JIANG Li-li??WANG Yang??ZHONG Di-xiao??XU Ke-ming.Capital Institute of Pediatrics??Beijing 100020??China
Absract??Objective??To approach the clinical characteristics of childhood partial epilepsy presenting HMS. Methods ??From Jun. 2003 to Jua. 2009 in Capital Institute of Pediatrics??Video-EEG was performed on 7 children who met the H. Lüders semiology diagnosis criteria??analyzed the clinical episode by reviewing the videotapes??observe the ictal and interictal EEG manifestations and follow up the effect on antiepileptic drugs. Results??Totally 29 attacks were recorded in 7 cases by Video-EEG monitor. The seizures lasted from 10 to 84 seconds?? 22 of which occurred during sleep and 7 during awakeness.HMS consisted of mild or marked agitation and HM behavior including claping??waving arms??boxing??tapping??scratching??kicking or horizontal movements of trunk and pelvis while lying on the bed.The non-HM signs included eyes and head??neck??trunk and limbs deviation??dystonic posturing??flushing and fear or angry expression on the face. Lateral or bilateral sharp or spike waves at frontalpole and frontal leads were found in all cases except one in the interictal EEG??while ictal EEG demonstrated frontalpole or frontal onset rhythmic low amplitude fast activity at lateral or bilateral leads in all the cases ??immediately covered by motor artefacts and recovered by primary background activity after the episode. Conclusion??HMS??as a manifestation of partial epilepsy presents marked agitation and HM behavior characteristics that may accompany with dystonic posturing and expression??ictal and interictal EEG demonstrates lateral or bilateral epileptic waves at frontalpole or frontal leads. Seizures can be controlled satisfactorily by carbamazepine or oxcarbazepine.     

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??Objective??To summarize the efficacy and safety of arsenic trioxide combined with chemotherapy for children with recurrent/refractory neuroblastoma. Methods??Retrospective analysis was made in seven cases of neuroblastoma treated with traditional chemotherapy combined with venous arsenic trioxide after diagnosis with poor prognosis or the condition deteriorated. Results??Among the seven patients treated with chemotherapy combined with venous arsenic trioxide for 3 to 7 courses??4 were effective and 3 were ineffective. Patient 3 was treated with arsenic trioxide combined with following regimen after tumor was not resected totally??and patient 5??6 and 7 were given arsenic trioxide combined with preoperative chemotherapy. Patient 3 and patient 5 achieved complete remission after combination chemotherapy??and patient 6 and patient 7 showed decreased tumor volume and tumor marker. Patient 1??2 and 4 were given arsenic trioxide after failure of conventional chemotherapy. Metastasis in patient 1 disappeared at first??but the disease relapsed after 11 months. The tumor responded poorly and increased in size and number in patient 2 and patient 4. Patient 1 the use of arsenic trioxide was stopped in patient 1 because of prolonged QT interval??0.51s????who returned normal after symptomatic treatment. Five patients are still alive and they have lived for 3.5 y??3.0 y??1.2 y??1.0 y and 0.7 y??respectively. Conclusion??Venous arsenic trioxide combined with chemotherapy can be a choice of treatment for recurrent/refractory neuroblastoma??whose effect and safety requires further investigation.     

溶血危象7例

对我科 1998～ 2 0 0 0年收治的 7例溶血危象患儿的临床资料进行回顾性分析。发现 7例中 2例因感染诱发 ,5例因食用蚕豆诱发。 7例均有发热、贫血、黄疸等临床表现。治疗予输同型血或成份血 ,加用地塞米松 0 .5ｍｇ/(ｋｇ·ｄ)静滴 ,并给予对症处理 ,7例均好转出院。溶血危象是小儿血液系统急症 ,应尽早诊治。溶血危象7例$宜昌市中心人民医院儿科!443003@寇宏     

乳糜胸7例

小儿乳糜胸临床少见，1984年～1996年我们共治疗7例，现报告分析如下。临床资料一、一般资料：男4例，女3例。年龄＜28dl例，～la2例，～6a2例，～12a2例。二、原发病：先天性肠淋巴管扩张症、缩窄性心包炎、先天性心脏病并心力衰竭、纵隔肿瘤、肾病、肺炎、产伤各1例。三、乳糜积液部位：双侧胸腔3例（其中2例并乳糜腹），左、右胸腔各2例。四、临床表现及实验室检查：除原发病以及胸闷、气促、衰弱等临床表现外均有胸腔积液X线表现，胸水乳糜试验均阳性，李凡他试验阳性，比重1．O12～1．026，胸水细胞数（0．028～0．22）×109／L，其…     

急性淋巴细胞白血病患儿中可逆性脑病临床总结

目的探讨急性淋巴细胞白血病患儿化疗后可逆性脑病的临床和影像学特点。方法回顾分析2015年9月1日至2018年9月1日住院时发生脑病的急性淋巴细胞白血病患儿的临床资料。结果研究期间共收治新发急性淋巴细胞白血病582例,9例患儿发生10次可逆性脑病(1例患儿发生2次),其中男6例、女3例,脑病发生中位年龄6.55岁(3.9~12.5岁)。最常见的神经系统临床表现是抽搐,其次是肌无力和感觉异常。7例患儿曾接受培门冬治疗;5例患儿在脑病发生前有急性高血压病史;6例患儿在脑病发生时有低钠血症,部分有低纤维蛋白原血症。头颅磁共振成像检查均提示T1和T2信号异常,累及部位多见于顶枕叶。结论联合化疗、化疗药物鞘内注射和急性高血压是可逆性脑病发生的高危因素;监测血压、血钠、纤维蛋白原,以及头颅磁共振成像检查有助于早期发现可逆性脑病。     

Progression of methotrexate-induced leukoencephalopathy in children with leukemia

From 1972 - 1974, 228 children began treatment for acute lymphocytic leukemia and were prospectively assessed for neurologic complications. After CNS irradiation (2,400 rad) and intrathecal methotrexate (MTX), they received weekly intravenous maintenance therapy with MTX alone (40–60 mg/m²; 20 patients) or MTX (10-30 mg/m²) with other drugs (208 patients). Signs of leukoencephalopathy appeared in 11 children (nine without CNS leukemia) after 4-15 months of IV MTX alone, and included lethargy, seizures, spasticity, paresis, drooling, and dementia. Before or during the clinical onset, EEG frequencies slowed (all ten patients tested). Radionuclide scans showed periventricular accumulation of ^99mTc (9/11 patients) and remained abnormal for ≥ six months in eight patients. Cranial computed tomograms or neuropathology findings (five patients each) demonstrated leukoencephalopathy (nine patients) and radiation-related microangiopathy (ten patients). Severe neurologic and neuropsychologic dysfunctions were present in four long-term survivors.     

儿童桥本脑病5例临床分析

目的通过总结儿童桥本脑病的临床特点,提高儿科医师对该病的认识,以期早期诊断及治疗、改善预后。方法回顾性分析2014年1月至2018年12月重庆医科大学附属儿童医院临床诊断的5例儿童桥本脑病临床资料,总结其临床表现、辅助检查结果、诊治经过及预后。结果5例桥本脑病患儿中男2例,女3例,年龄10岁6月龄至14岁4月龄;主要临床表现包括精神行为异常(4/5)、认知障碍(4/5)、癫痫发作(4/5)、不自主运动(2/5)及睡眠障碍(2/5);5例患儿血清抗甲状腺过氧化物酶抗体(TPO-Ab)和抗甲状腺球蛋白抗体(TG-Ab)均明显升高;脑电图均提示背景弥漫性慢化、头颅磁共振成像(MRI)均无异常、血及脑脊液自身免疫性脑炎抗体检测均为阴性,3例患儿脑脊液生化检测提示蛋白轻度升高;5例患儿在予以激素治疗后病情均逐渐好转。结论儿童桥本脑病罕见,主要以精神行为异常、认知障碍、癫痫发作为主要表现,血清TPO-Ab和TG-Ab显著升高为其特点,早期激素治疗,效果显著、预后良好。     

儿童急性淋巴细胞白血病伴白质脑病临床及影像学特点

目的探讨急性淋巴细胞白血病(ALL)患儿并发白质脑病的临床特点及影像学特点,以提高对ALL伴白质脑病的认识。方法回顾性分析2011年5月至2021年4月发生白质脑病的28例ALL患儿的临床资料。结果 28例患儿中男18例、女10例;ALL中位发病年龄为6.63(1.37～12.87)岁,白质脑病中位发病年龄为7.81(1.45～14.01)岁。24例患儿出现神经系统表现,以癫痫发作(13例)、瘫痪(8例)、肢体麻木(6例)、视力障碍(6例)及口齿不清(4例)等为主。影像学上,病灶主要分布在顶叶(10例)、侧脑室周围及半卵圆中心白质区(9例)、基底节区(6例)、额叶(4例)及枕叶(4例)等部位,病灶呈多发性、不规则小片状或大片状分布。ALL伴白质脑病发生后立即停用化疗药物并予对症处理,大多数病例临床症状及影像学病灶逐渐消失。随访1年,28例中19例复查了头颅MRI,其中16例(84.2%)病灶较第1次明显改善,11例(57.9%)异常信号完全消失。结论白质脑病可能是ALL治疗期间重要并发症。虽然ALL伴白质脑病的临床与影像学表现多样,但当神经系统症状出现时,给予早期诊断与治疗后预后较好。     

儿童桥本脑病5例临床分析

目的　通过总结儿童桥本脑病的临床特点，提高儿科医师对该病的认识，以期早期诊断及治疗、改善预后。方法　回顾性分析2014年1月至2018年12月重庆医科大学附属儿童医院临床诊断的5例儿童桥本脑病临床资料，总结其临床表现、辅助检查结果、诊治经过及预后。结果　5例桥本脑病患儿中男2例，女3例，年龄10岁6月龄至14岁4月龄；主要临床表现包括精神行为异常（4/5）、认知障碍（4/5）、癫痫发作（4/5）、不自主运动（2/5）及睡眠障碍（2/5）；5例患儿血清抗甲状腺过氧化物酶抗体（TPO-Ab）和抗甲状腺球蛋白抗体（TG-Ab ）均明显升高；脑电图均提示背景弥漫性慢化、头颅磁共振成像（MRI）均无异常、血及脑脊液自身免疫性脑炎抗体检测均为阴性，3例患儿脑脊液生化检测提示蛋白轻度升高；5例患儿在予以激素治疗后病情均逐渐好转。结论 儿童桥本脑病罕见，主要以精神行为异常、认知障碍、癫痫发作为主要表现，血清TPO-Ab和TG-Ab显著升高为其特点，早期激素治疗，效果显著、预后良好。     

Immunocompetence and prognosis in children with acute lymphoblastic leukemia: Combination of two different maintenance therapies

Intensive chemotherapy in patients with leukemia produces immunosuppression. The level of immunocompetence correlates with prognosis. The immunological function of 29 children with acute lymphoblastic leukemia (ALL) in complete remission and on 2 different maintenance therapies was evaluated and compared with 16 normal children (Group A). Sixteen children (Group B) with ALL received 6 mercaptopurine (6MP) daily and methotrexate (MTX) twice a week, and 13 children (Group C) received 6MP and MTX weekly for maintenance. There was depression of both cellular immunity, measured by the number of T cells and skin tests, and humoral immunity, measured by number of B cells, primary antibody production to typhoid vaccine, and levels of immunoglobulins. However, continuous maintenance therapy (Group B) produced significantly more severe immunosuppression of cellular immunity than the intermittent therapy (Group C). Humoral immunity was equally depressed in both groups of leukemia patients, but was less altered than cellular immunity. Concomitantly, patients with intermittent maintenance chemotherapy had less hematologic depression, fewer episodes of infection, and fewer died in complete remission. Patients of both groups with higher levels of immunocompetence had better prognosis with longer duration of complete remission than patients with severe immunosuppression. Out of 6 patients with “favorable immunocompetence” only 1 relapsed at 7 months and the other 5 remain in complete remission from 8 to 31 months. Among 23 leukemic patients with “unfavorable immunocompetence,” 15 relapsed and 8 remain in complete remission from 9 to 26 months.     

儿童急性淋巴细胞白血病化疗后合并肺部感染的临床特征

目的分析化疗后合并肺部感染的急性淋巴细胞白血病(ALL)患儿的临床特征,为ALL合并肺部感染的早期诊断提供依据。方法对115例次化疗后合并肺部感染且行肺部CT的ALL患儿(108例)进行回顾性分析,收集患者一般临床资料及肺部螺旋CT结果,探究肺部感染发生的危险因素,以及病原体与肺部CT的影像学特点。结果儿童ALL化疗后的肺部感染77.4%发生于诱导缓解阶段,多发生于化疗后的31~60?d,以粒缺患儿所占比例(67.0%)最高。病原学拟诊或确诊的41例肺部感染患儿以细菌感染(36%)与真菌感染(41%)的发生率较高。细菌或真菌感染所致肺部病变CT表现的差异无统计学意义(P0.05)。结论 ALL患儿在化疗诱导缓解阶段,尤其是粒细胞缺乏时肺部感染发生率高。细菌和/或真菌是主要病原体,难以根据肺部影像学改变明确肺部感染的性质。     

大剂量甲氨喋呤治疗急性淋巴细胞白血病的血药浓度研究

目的　通过对甲氨喋呤 (MTX)治疗急性淋巴细胞白血病的血药浓度研究 ,探讨MTX剂量、血药浓度、效应、毒副作用等方面的关系 ,以及血药浓度检测的临床意义。方法　荧光偏振免疫分析法检测不同剂量MTX治疗的血药浓度 ,结合临床资料综合分析。结果　 1.第 2 4hMTX的血清浓度随剂量增加而增加 ;白血病复发率随浓度增加而下降 ,分别为 4 0 %、33.3%、2 5 % ;2 .鞘注前脑脊液 (CSF)浓度均低于有效浓度 ,因此须加鞘注治疗 ;3.本研究中MTX血药浓度均低于发生严重毒副作用的高危浓度 ,毒副作用均较轻微。结论　MTX血药浓度研究为实施合理的个体化治疗方案提供客观依据