小儿特发性血小板减少性紫瘢T细胞亚群的观察

小儿特发性血小板减少性紫瘢Ｔ细胞亚群的观察田根全，袁文菊，程岩青海省儿童医院ＴＨＥＴ－ＣＥＬＬＳＵＢ－ＧＲＯＵＰＭＥＡＳＵＲＥＭＥＮＴＯＦＩＤＩＯＰＡＴＨＩＣＴＨＲＯＭＢＯＣＹＴＯＰＥＮＩＣＰＵＲＰＵＲＡ（ＩＴＰ）ＩＮＰＥＤＩＡＴＲＩＣＰＡＴＩＥＮＴ...     

不同日龄、不同病情新生儿血小板数量的观察

不同日龄、不同病情新生儿血小板数量的观察秦振庭，王广方北京医科大学第一临床医学院妇儿医院ＰＬＡＴＥＬＥＴＣＯＵＮＴＳＡＴＤＩＦＦＥＲＥＮＴＡＧＥＧＲＯＵＰＳＯＦＮＥＷＢＯＲＮＳ￥Ｃｈｅｎ－ＴｉｎｇＣｈｉｎｅｔａｌ（Ｗｏｍｅｎ＆Ｃｈｉｌｄｒｅｎｈｏｓｐ...     

Y-1型计测板对红细胞微量压积测量效果的初步评价

Ｙ－１型计测板对红细胞微量压积测量效果的初步评价袁光孚，保菊英，王冀湘，耿广献西宁市一医院ＥＶＡＬＵＡＴＩＯＮＯＦＡＣＡＬＣＵＬＡＴＩＮＧＰＬＡＴＥＭＯＤＥＬＹ－ＩＦＯＲＴＨＥＭＥＡＳＵＲＥＭＥＮＴＯＦＭＩＣＲＯ－ＨＥＭＡＴＯＣＲＩＴ．￥ＹｕａｎＧｕ...     

血小板质控初探

血小板质控初探袁光孚，张玉芳，保菊英，秦振庭西宁市第一人民医院ＣＡＭＰＡＲＩＳＩＯＮＯＦＤＩＦＦＥＲＥＮＴＭＥＴＨＯＤＳＦＯＲＰＬＡＴＥＬＥＴＣＯＵＮＴＩＮＧＡＴＨＩＧＨＡＬＴＩＴＵＤＥＱＩＮＧＨＡＩ￥Ｋｕａｎｇ－ＦｕＹｕａｎ．ｅｔａｌ（Ｘｉ－Ｎｉｎ...     

小儿白血病形态学、免疫学分型研究

小儿白血病形态学、免疫学分型研究程岩，田根全，袁文菊，杨小红青海省儿童医院ＴＨＥＲＥＳＥＡＲＣＨＯＦＭＯＲＰＨＯＬＯＧＩＣＡＮＤＩＭＭＵＮＯＬＯＧＩＣＡＬＣＬＡＳＳＩＦＩＣＡＴＩＯＮＯＦＴＨＥＰＥＤＩＡＴＲＩＣＬＥＵＫＥＭＩＡ￥ＹａｎＣｈｅｎｇｅｔａ...     

小儿急性白血病患者血浆纤维结合蛋白测定的临床意义

小儿急性白血病患者血浆纤维结合蛋白测定的临床意义张宝玺，郭稳捷河北医学院附属二院儿科ＴＨＥＣＬＩＮＩＣＡＬＳＩＧＮＩＦＩＣＡＮＣＥＯＦＰＬＡＳＭＡＦＩＢＲＯＮＥＣＴＩＯＮＤＥＴＥＣＴＩＯＮＩＮＣＨＩＤＨＯＯＤＡＣＵＴＥＬＥＵＫＥＭＩＡ￥ＺｈａｎｇＢａ...     

新生儿依赖维生素K凝血因子活性的动态观察

新生儿依赖维生素Ｋ凝血因子活性的动态观察楼金吐，俞锡林，孔宏伟浙江医科大学附属儿童医院ＴＨＥＣＨＡＮＧＩＮＧＰＡＴＴＥＲＮＯＦＶＩＴＡＭＩＮＫ－ＤＥＰＥＮＤＥＮＴＦＡＣＴＯＲＳＩＮＮＥＯＮＡＴＥＳ￥ＬｏｕＪｉｎｔｕ，ｅｔａｌ．（Ｉｎｓｔｉｔｕｔｅｏｆ...     

小儿恶性组织细胞增生症血象及骨髓象分析

小儿恶性组织细胞增生症血象及骨髓象分析郭艺杰，王令仪武汉同济医科大学同济医院儿科ＡＮＡＬＹＳＩＳＯＦＨＥＭＯＧＲＡＭＡＮＤＢＯＮＥＭＡＲＲＯＷＰＩＣＴＵＲＥＳＩＮＭＡＬＩＧＮＡＮＴＨＩＳＴＯＣＹＴＯＳＩＳ￥ＧｕｏＹｉＪｉｅ，ｅｔａｌ（Ｄｅｐａｒｔｍｅ...     

重组人集落刺激因子在白血病中的临床应用

重组人集落刺激因子在白血病中的临床应用顾立达，艾孜买提，孔佩艳，严媚，赵勒新疆医学院一附院儿科血液组ＣＬＩＮＩＣＡＬＡＰＰＬＩＣＡＴＩＯＮＯＦＲＥＣＯＭＢＩＮＡＮＴＨＵＭＡＮＧＲＡＮＮＬＯＣＹＴＥＣＯＬＯＮＹＳＴＩＭＵＬＡＴＩＮＧＦＡＣＴＯＲＯＮＬＥ...     

小儿血液病骨髓活检的临床意义

小儿血液病骨髓活检的临床意义屠立明，沈亦逵，王若洁广东省人民医院儿科ＣＬＩＮＩＣＳＩＧＮＩＦＩＣＡＮＣＥＯＦＢＯＮＥＭＡＲＲＯＷＢＩＯＰＳＹＩＮＣＨＬＬＤＲＥＮＨＥＭＡＴＯＬＯＧＩＣＤＩＳＥＡＳＥＳ￥ＴｕＬｉｍｉｎｇ，ｅｔａｌ．（ＧｕａｎｇｄｏｎｇＰ...     

Severe hemolysis and methemoglobinemia following fava beans ingestion in glucose-6-phosphatase dehydrogenase deficiency—case report and literature review

Introduction  Reduced concentrations of glucose-6-phospate dehydrogenase (G6PD) render erythrocytes susceptible to hemolysis under conditions of oxidative stress. In favism, the ingestion of fava beans induces an oxidative stress to erythrocytes, leading to acute hemolysis. Discussion  The simultaneous occurrence of methemoglobinemia has been reported only scarcely, despite the fact that both phenomena are the consequence of a common pathophysiologic mechanism. The presence of methemoglobinemia has important diagnostic and therapeutic consequences. We report a previously healthy boy who presented with combined severe hemolytic anemia and cyanosis due to methemoglobinemia, following the ingestion of fava beans. His condition was complicated by the development of transient acute renal failure. A G6PD-deficiency was diagnosed. We review the literature on the combination of acute hemolysis and methemoglobinemia in favism. Pathophysiologic, diagnostic, and therapeutic aspects of this disorder are discussed.     

儿童再发川崎病77例临床特征回顾性分析

目的 探讨再发川崎病(KD)的临床特点及其预后。方法 收集1994年1月至2012年10月重庆医科大学附属儿童医院收治的KD再发病例,比较初发时和复发时的临床特征、实验室指标和随访资料。并选取5～10年未再发KD病例作为对照组,探讨KD再发可能的危险因素。结果 19年间收治KD 4 875例,其中再发KD 77例,再发1次74例,再发2次3例,男∶女为1.4∶1。再发平均间隔时间1.6年,1年以内再发45.4%（35/77）。发热病程再发时较初发时缩短 (7.6±3.1) vs ( 8.9 ± 3.8) d,P＜0.05;WBC和CRP水平再发时较初发时显著降低,(14.3±5.7) vs (16.2 ±5.4)×109·L-1和(61±58) vs ( 95±76) mg·L-1,P均<0.05。急性期（病程≤30 d）冠状动脉病变（CAL ）发生率初发时为17.8%（13/73) ,再发时为13.3%( 10/75);应用IVIG后亚急性期（病程>30 d）CAL发生率初发时为11.0%（8/73),再发时为9.3%（7/75),组间比较差异均无统计学意义。7例初发时与再发时均有CAL,其中1例初发时与再发时均合并冠状动脉瘤（CAA）。52例KD再发患儿有出院后随访资料,随访时间平均2.1年,其中1 例再发时合并左侧冠状动脉主干小型CAA的患儿随访中出现新发部位左前降支瘤样扩张,冠状动脉内径回缩至正常后4年7个月再次出现左侧冠状动脉扩张。多因素Logistic分析显示,年龄＜3岁、性别、热程＞10 d、并发CAL及WBC>20×109·L-1均与KD再发无统计学关联。结论 KD再发多发生在1年内。再发KD的CAL总发生率并未升高,初发时合并CAL患儿,再发时更易发生CAL。     

Double-blind, randomized trial of diazepam versus placebo for prevention of recurrence of febrile seizures

The aim of this study was to evaluate the efficacy and tolerance of intermittent oral administration of diazepam during hyperthermia for reducing the recurrence of febrile seizure: 185 children, between 8 months and 3 years of age, with a first febrile seizure and normal neurologic development, were randomly assigned in a double-blind fashion to receive orally administered diazepam (0.5 mg/kg, then 0.20 mg/kg, every 12 hours) or placebo, whenever the rectal temperature was more than 38 degrees C. The main criterion of efficacy was the seizure recurrence rate 1 year after the first seizure. The duration of the study was 3 years; eight different centers in France participated. There were 462 febrile episodes and 1000 days with prophylactic treatment. The recurrence rates did not differ between the diazepam group (16%) and the placebo (19.5%) group. The children with recurrent seizures were significantly younger at the time of the first seizure (17 +/- 6.9 months) than children without a recurrent seizure (21 +/- 8.5 months). In children with recurrent seizures, prophylactic treatment was correctly administered to only 1 of 15 children in the diazepam group and to 7 of 18 children in the placebo group. The following were the reasons for this poor cooperation: convulsion being the first manifestation of the fever (seven cases in each group), parents neglecting to give treatment (nine cases), and refusal to take treatment by two children. Side effects were similar in the two groups except for hyperactivity, which was more frequent in the diazepam (138 days) than in the placebo (34 days) group. Intermittent oral administration of diazepam at the onset of fever offered no advantage over placebo in preventing recurrence of seizure. This finding probably reflects a lack of efficacy of the intermittent method rather than of diazepam itself.     

Accidental epileptic seizures in children

The authors report a series of 71 children admitted to a general pediatric hospital for a first non febrile, non symptomatic seizure and observed for an average of 6 years and 5 months. Among these patients, 19 cases corresponded to an isolated unexplained seizure without paroxysmal E.E.G. abnormalities, which did not reappear without treatment in a mean follow-up period fo 5 years and 3 months. The typical features of these "accidental seizures" are compared with other types of epilepsy. Finally, these "accidental seizures" can be classified into 2 groups: atonic seizures in the young child (1-4 years) and partial seizures in older children. A statistical analysis was undertaken to define the risk factors for recurrence after the first epileptic attack. A low recurrence risk is expected for children between 1 to 4 years with atonic type of seizures without paroxysmal E.E.G. abnormalities while there is a high recurrence risk for children under 1 year with generalized seizures and paroxysmal E.E.G. intercritical abnormalities.     

AML-XH-99方案治疗婴幼儿急性髓系白血病临床总结

目的探讨婴幼儿急性髓系白血病(AML)的临床特征及采用AML-XH-99方案的疗效。方法 1998年5月至2009年4月诊断的0～3岁AML(除外M3)26例,采用AML-XH-99方案化疗。应用SPSS13.0软件统计,采用Kaplan-Meier生存分析法进行无事件生存(EFS)分析。结果 26例患儿中M5居首(61.54%),其次为M2(26.92%)。年龄>12个月22例,<12个月4例,7例(26.92%)起病时外周血白细胞>50×109/L,5例(19.23%)染色体核型异常,5例(19.23%)在诱导治疗结束后48 h的骨髓涂片中幼稚细胞比例>15%。24例(92.31%)第1疗程获完全缓解(CR),总缓解率为96.15%。5例(19.23%)骨髓复发,中位复发时间为完全持续缓解(CCR)15.8个月。3例在强化疗后接受异体造血干细胞移植。26例中1例失访,7例死亡(5例死于疾病复发,2例死于治疗相关并发症)。中位CCR时间为4.08年,7年EFS为60.72%。结论 AML-XH-99方案治疗婴幼儿急性髓系白血病缓解率高,预后良好。     

Risk factors for recurrence of febrile convulsions

A cohort of hundred children with febrile convulsions, in the age group of 3 months to 5 years were followed up prospectively for one year to study the natural course of the illiness, and to determine if specific factors would increase the risk of recurrence of febrile convulsions. The risk factors studied were age of onset under one year, long duration of convulsion (more than 15 minutes), family history of febrile convulsion or epilepsy and combination of two or all of the above factors. Four groups of children with different risk factors were followed up for recurrence of convulsion, after the first attack. A group of children without any risk factor was considered as control and they were also followed up for recurrence of convulsions. Though all the groups with the risk factors, showed a trend towards a higher recurrence rate when compared to controls, the difference observed clinically was not significant statistically. This could be due to the small sample size of each group. A larger study could throw light on the predictive value of these risk factors and narrow down the use of long term anticonvulsant prophylaxis.     

儿童间变性大细胞淋巴瘤20例临床疗效分析

目的总结分析儿童间变性大细胞淋巴瘤(anaplastic large-cell lymphoma,ALCL)治疗方案的远期疗效及影响预后的因素。方法回顾性分析2002年1月-2010年4月在我院诊断并接受治疗和随访的20例ALCL患儿,采用本院制定的B-NHL 2001方案进行危险度分组和治疗,采用SPSS软件对数据进行处理。结果 ALCL患儿20例,男9例,女11例,诊断时中位年龄8.4(1.9～15.4)岁,>10岁者7例。诊断时Ⅱ期、Ⅲ期和Ⅳ期患儿分别有1例、11例和8例。8例原发于颈部淋巴结,而纵隔、腹腔、腹股沟和腋窝等部位各2例,右腰大肌和左上臂肌各1例,髋骨和右肱骨各1例。15例发病时有B症状,6例ECT显示有骨骼侵润,2例骨髓穿刺见肿瘤细胞,初诊时血清乳酸脱氢酶和铁蛋白增高1倍以上者各6例。病理分析显示17例为T细胞型,16/18例免疫组化间变性大细胞淋巴瘤激酶(ALK)阳性。所有患儿均接受了R3组治疗,有2例初诊为横纹肌肉瘤,其中1例给予AVCP方案一疗程后明确诊断转入R3,1例Ⅱ期患儿接受R2期治疗未缓解转入R3,另有1例初诊T-NHL,以PVDL方案治疗一疗程转入R3。5年无事件生存率和总生存率分别为(69.3±10.5)%和(74.0±10.1)%。单因素分析发现年龄>10岁组患儿复发风险更大,而性别、病理类型、分期、原发部位、是否有骨骼或骨髓侵润、血清乳酸脱氢酶和铁蛋白水平等,对复发风险在统计学上无显著影响。结论儿童ALCL的临床表现多样,其疗效受年龄影响,目前按照B-NHL2001治疗方案能取得较好的远期疗效。     

Risk factors for recurrent urinary tract infection in preschool children

OBJECTIVE: Children with urinary tract infections (UTI) are at risk of renal scarring which may lead to impaired renal function and hypertension. This study examines the risk factors that predispose to recurrent UTI in children and the role of recurrent UTI in renal scarring. METHODOLOGY: A group of 290 children under 5 years of age with a first symptomatic UTI were studied. Micturating cystourethrogram and dimercaptosuccinic acid (DMSA) renal scintigraphy were performed at entry, and DMSA was repeated 1 year later. Two hundred and sixty-one children (90%) were followed up at 1 year. RESULTS: There were 46 confirmed recurrent infections in 34 children, a recurrence rate of 12%. Multiple recurrence occurred in 14/34 (34%) children. Age of less than 6 months on entry independently predicted for recurrent UTI (odds ratio (OR): 2.9)). Compliance with prophylactic antibiotics fell throughout the year of follow up. Vesicoureteric reflux (VUR) was present in 14/34 (34%) of the group with recurrent UTI, 69/256 (27%) without recurrence. Urinary tract infection was significantly associated with bilateral and intrarenal reflux; grade 3-5 reflux independently predicted for recurrent UTI (OR: 3.5). Recurrent UTI was significantly associated with high grade DMSA defects on entry, renal parenchymal defects at 1 year follow up, and new defects at 1 year. CONCLUSION: The independent risk factors for recurrent UTI identified by this study were an age of less than 6 months at the index UTI and grade 3-5 VUR. These findings suggest more selective targeting may minimize problems associated with prophylaxis and improve outcomes for children with urine infection.     

小儿再发性化脓性脑膜炎28例分析

目的探讨小儿再发性化脓性脑膜炎(以下简称再发化脑)可能的病因及相应治疗对策。方法回顾分析1995—2004年在天津市儿童医院神经科住院的28例再发化脑患儿的临床资料,以及根据可能病因分别行头MR、脊髓MR、颞骨CT、脑干电测听、血IG和血培养等检查。结果28例再发化脑的病因:脑脊液鼻漏13例、脑脊液耳漏8例、脑脊液耳鼻漏1例、潜毛窦5例、体液免疫缺陷病1例。给予急性期抗感染治疗,恢复期根据病情分别给予脑脊液鼻漏修补术,脑脊液耳漏、脑脊液耳鼻漏、内耳修补术,潜毛窦切除术,随诊6个月至2年未再复发。结论化脑是中枢神经系统严重感染性疾病,重者可危及生命。首发化脑就应注意寻找病因,再发化脑更应积极寻找病因并祛除病因,防止再发。     

Subureteral collagen injection versus antireflux surgery in primary vesico-ureteral reflux grade III

Endoscopic subureteral collagen injection (SCIN) was performed in 24 girls and 5 boys (mean/range: 3.2/0.7–12.2 years) with primary grade III vesicoureteral reflux. Clinical outcome was compared to 20 girls and 6 boys treated by antireflux surgery (mean/range: 3.0/0.2–9.4 years). Forty-one ureters were treated by endoscopy, 37 by surgery. Patients were followed for 12 months. SCIN was not associated with severe persistent complications. No reflux was detected immediately after SCIN. After 6 months recurrent grade II reflux was present in 1 (4%) surgically treated patient and in 12/29 (41%) patients treated by collagen injection. This corresponded to a recurrence rate of 1/37 (3%) of ureters following surgery and 15/41 (37%) following SCIN. 8/15 refluxing ureters after collagen showed decreased reflux grade (two grade I, six grade II). After 12 months 24/26 (92%) patients with antireflux surgery remained free of urinary tract infections as compared to 25/29 (86%) children following endoscopy. Following SCIN, 4/29 (14%) patients required secondary antireflux surgery because of recurrent urinary tract infections.Conclusion On the basis of this study endoscopic SCIN appears to be safe in children. It is less effective than surgery with regard to elimination of primary grade III reflux. However, clinical success rate is comparable to surgery with regard to the frequency of recurrent urinary tract infections after the procedure. This may be due to the reduction of refluxing urine volume in those patients who showed recurrence of reflux after collagen injection.