依地酸钙钠治疗儿童慢性中度铅中毒方案研究

目的研究依地酸钙钠(Ca Na2EDTA)治疗慢性中度铅中毒疗效,以优化儿童铅中毒的螯合治疗方案。方法回顾分析2014年9月—2016年12月接受Ca Na2EDTA连续3个疗程驱铅治疗的14例慢性中度铅中毒患儿的临床资料,分析住院期间每日24小时尿铅水平,以及治疗前、治疗3天和5天后血铅水平的变化。结果 14例患儿中,男4例、女10例,平均年龄(2.35±1.47)岁。Ca Na2EDTA治疗后血铅均显著下降。三疗程治疗3天的血铅下降值分别为治疗5天下降值的0.76、0.77、0.72。三疗程3天单位用药血铅下降值高于5天单位用药血铅下降值,差异均有统计学意义(P0.05)。三疗程治疗3天的尿铅下降值分别为治疗5天下降值的0.65、0.71、0.70。三疗程3天单位用药尿铅排出量高于5天单位用药尿铅排出量,差异均有统计学意义(P0.05)。结论 Ca Na2EDTA驱铅疗效明显,3天驱铅效率高于5天,治疗3天为一疗程的方案或许可作为原5天一疗程的替补方案。     

胎粪吸入幼兔外周血及支气管肺泡灌洗液中肿瘤坏死因子-α的表达

目的 探讨TNF-α在胎粪吸入幼兔肺部炎症反应中的表达水平及意义.方法 将20只日龄21～30 d幼兔随机分为模型组和对照组,每组10只.模型组经气管导管注入胎粪悬混液,复制胎粪吸入性肺炎模型;对照组手术操作与模型组相同,但无胎粪悬液注入,不复制模型.酶联免疫吸附法检测2组幼兔外周血和支气管肺泡灌洗液TNF-α表达水平,并行统计学分析.结果 模型组幼兔肺组织出现Ⅰ型肺泡上皮细胞变性,Ⅱ型肺泡内板层小体呈空泡样改变等炎性损伤表现,且外周血和支气管肺泡灌洗液中TNF-α表达水平均高于对照组(Pa<0.05).结论 TNF-α参与介导了胎粪吸入后肺部的炎症反应.     

托吡酯对戊四氮致(癎)大鼠认知功能及海马5-羟色胺、乙酰胆碱酯酶的影响

目的 探讨托吡酯(TPM)对戊四氮(PTZ)致(癎)大鼠认知功能及海马组织5-羟色胺(5-HT)和乙酰胆碱酯酶(AChE)的影响.方法 28日龄健康Wistar 大鼠60只,随机分为空白组、TPM一般剂量(0.018 g/kg)对照组(TPM对照A组)、TPM大剂量(0.036 g/kg)对照组(TPM对照B组)、PTZ(0.035 g/kg)点燃模型组(PTZ组)、TPM一般剂量(0.018 g/kg)治疗组(TPM治疗A组)和TPM大剂量(0.036 g/kg)治疗组(TPM治疗B组),每组10只.PTZ和TPM连用4周.模型组完全点燃后各组进行Y-型迷宫学习记忆测试,免疫组织化学检测其海马组织5-HT阳性神经元的表达,化学比色法检测其海马内AChE活性.结果 TPM 对照A、B与空白组比较,TPM对照B组与A组比较,PTZ与空白组比较,大鼠学习能力及记忆能力均明显降低(Pa<0.001),5-HT免疫阳性神经元数量明显减少(Pa<0.001),AChE活性明显增加(Pa<0.001); TPM治疗A、B组与PTZ组相比,TPM治疗A组与TPM 对照A组相比,TPM治疗B组与TPM对照B组相比,大鼠学习能力及记忆能力显著提高(Pa<0.001),5-HT免疫阳性神经元数量明显增加(Pa<0.001),AChE活性明显降低(Pa<0.001).结论 TPM可引起正常大鼠认知功能障碍,其机制可能与大鼠海马5-HT水平降低和AChE活性增加有关,其程度与TPM剂量有关; TPM良好的治疗效果掩盖了药物本身对认知功能的影响.     

黄芪对实验性IgA肾病大鼠Th1/Th2免疫失衡与核因子-κB表达的影响

目的探讨核因子-κB(NF-κB)和Th1/Th2免疫失衡在IgA肾病(IgAN)发病中的作用及黄芪防治IgAN的机制。方法32只SD大鼠分为模型组、干预组和对照组。模型组和干预组复制IgAN模型,干预组予黄芪颗粒剂混悬液口服,并以正常SD大鼠作为对照组。检测各组大鼠蛋白尿、血尿及肾脏病理改变,采用免疫组织化学技术检测各组大鼠肾组织NF-κB、转化生长因子-β1(TGF-β1)、单核细胞趋化蛋白-1(MCP-1)的表达;ELISA法检测其血清中Th1类细胞因子INF-γ和Th2类细胞因子IL-4水平。结果1.模型组大鼠尿红细胞、尿蛋白水平较对照组明显增高,差异有统计学意义(Pa<0.01);2.模型组大鼠肾组织NF-κB、MCP-1、TGF-β1的表达与干预组、对照组比较均明显增高,差异均有统计学意义(Pa<0.01);3.模型组大鼠血清INF-γ水平与干预组、对照组比较均显著下降(Pa<0.05),IL-4水平与干预组、对照组比较均显著升高(Pa<0.01);4.模型组大鼠肾小球系膜区、肾小管、肾间质病理损害较干预组和对照组加重。结论黄芪能降低IgAN大鼠尿红细胞数、尿蛋白水平,并减轻IgAN模型大鼠肾脏病...     

兔睾丸扭转复位后睾丸细胞凋亡的观察

目的　观察兔睾丸扭转复位后睾丸细胞凋亡情况及药物减轻睾丸凋亡的效果。方法　选用青春期雄性日本大耳白兔 2 0只 ,体重 90 0～ 110 0g ,月龄 2～ 3个月 ,随机分为 4组 ,每组 5只。手术制作幼兔睾丸扭转模型 (72 0° ,2h) ,对部分扭转组应用抗氧化剂 (抗坏血酸 )或钙离子通道拮抗剂 (维拉帕米 )。 2d后取出睾丸 ,TUNEL法观查各组动物睾丸组织细胞凋亡情况 ,HE染色检查睾丸病理组织学改变。结果　幼兔睾丸扭转复位后 ,精原细胞发生凋亡 ,而睾丸支持细胞及间质细胞未见凋亡现象。单纯扭转组精原细胞平均凋亡指数 (AI) (7.92± 1.2 9)‰ ,较对照组 (2 .6 0± 1.0 1)‰显著升高 ;睾丸扭转前后应用抗坏血酸及维拉帕米组的睾丸细胞平均AI ,分别为 (4.12± 0 .73)‰ ,(4.0 8± 0 .88)‰ ,较单纯扭转组 (7.92± 1.2 9)‰显著下降。HE染色的组织学改变与TUNEL法类似。结论　睾丸扭转损伤后可致使生精细胞凋亡。应用抗氧化剂及钙离子通道拮抗剂可以减轻扭转睾丸的精原细胞凋亡     

双歧杆菌减轻肠外营养幼兔肝损害的实验研究

目的长期肠外营养(PN)患儿容易发生肝损害并发症,如肝脂肪变、胆汁淤积,甚至肝衰竭,其机制仍不清楚,可能和肠屏障功能障碍有关。近来双歧杆菌作为一种益生菌,在调节肠道微环境、保护肝脏中的作用日益受到重视。本研究拟通过给PN幼兔添加双歧杆菌,探讨其保护机制。方法生后3周的新西兰种白兔27只,体重200~250g。分为3组,PN组10只,PN+双歧杆菌组8只,对照组9只。双歧杆菌组每日经胃管注入丽珠肠乐溶液1ml/只(含青春双歧杆菌0.5×108),PN组注入生理盐水1ml/只。PN持续10d,取血测肝功能、内毒素水平;作肝脏组织病理分级评分;作回肠黏膜显微测量;作内脏组织匀浆和血培养观察细菌移位。结果双歧杆菌组幼兔肝功能明显改善,血清总胆红素和胆汁酸含量较PN组显著下降(P<0.05)。病理切片显示双歧杆菌组幼兔肝小叶完整,细胞形态基本正常,个别存在轻度炎症细胞浸润和纤维组织增生。而PN组则出现明显肝细胞变性(主要为脂肪变性)、胆管增生和胆汁淤积。参照Loff肝脏病理学评分标准,显示PN组分值明显高于对照组和双歧杆菌组(P<0.05),而双歧杆菌组和对照组比较差异无显著性意义(P>0.05)。肠道病理切片的计算机显微测量结果显示,双歧杆菌组幼兔回肠绒毛高度、隐窝深度、绒毛面积显著高于PN组(P<0.05),和对照组比较差异无显著性意义(P>0.05)。PN组幼兔血浆内毒素水平显著升高(P<0.001),双歧杆菌组内毒素水平处于正常范围。内脏组织、器官细菌培养结果显示,PN组幼兔肠菌移位率明显高于双歧杆菌组(P<0.01)。结论双歧杆菌可能通过降低肠黏膜通透性,避免幼兔产生肠菌移位和内毒素血症,保护肝功能。     

小儿中重度铅中毒25例发病原因及疗效分析

目的 探讨儿童中重度铅中毒可能的危险因素和防治策略.方法 回顾分析2006年6月至2006年12月在新华医院铅中毒诊疗中心住院治疗的25例中重度铅中毒儿童的临床资料,其中中度铅中毒15例,重度10例.对病例进行病史询问和血铅、三大常规、血微量元素、肝肾功能和膝关节X摄片等检查,排查儿童可能的污染源.治疗方案依据铅中毒分级不同而变化.结果 25例中,婴幼儿占总病人数的84%.临床表现方面,贫血(21例,占84%)、纳差(16例,占64%)、总铁结合力升高(13例、占52%)、入院时微量元素检测发现血清锌偏低(13例,占52%)和血清铁偏低(10例,占加%)、经常腹痛(10例,占40%)、营养不良(7例,占28%)和经常便秘(5例,占20%)等临床表现较常见.常见的病因为日常生活接触和当地工业污染.中重度铅中毒儿童中多数儿童疗效较好.结论 婴幼儿是中重度铅中毒儿童的易感人群.中重度铅中毒儿童临床表现多样,危险因素主要为日常生活接触和周围环境污粢.大多数儿童疗效良好.治疗的同时须进行健康宣教.预防铅再污染和疗程间歇期保健.     

托吡酯联合叶酸对戊四氮致癫(癎)幼鼠海马神经元线粒体损伤的保护作用

目的探讨托吡酯(TPM)联合叶酸(FA)对慢性癫幼鼠海马CA3区神经元线粒体超微结构损伤的影响。方法将3周龄雄性Wistar大鼠随机分为阴性、阳性对照组、TPM组和TPM加FA组。予各组大鼠戊四氮(PTZ)腹腔注射,制造慢性癫模型,分别予等量蒸馏水、TPM40mg/(kg·d)和TPM40mg/(kg·d)加FA5mg/(kg·d)灌胃;阴性对照组先行9g/L盐水腹腔注射,再予等量的蒸馏水灌胃。连续用药2个月。观察各组行为学表现及海马CA3区神经元线粒体的超微结构。结果TPM和TPM加FA组大鼠惊厥发作次数及海马CA3区神经元线粒体的超微结构损伤程度较阳性对照组明显减轻。阳性对照、TPM和TPM加FA组的惊厥发作次数依次为:(48.4±3.7)、(44.3±3.1)、(40.8±3.7)次,3组间差异有统计学意义(Pa<0.01);阴性对照、阳性对照、TPM、TPM加FA组海马CA3区神经元线粒体的超微结构损伤程度依次为(0.34±0.09)、(3.76±0.28)、(2.85±0.21)、(2.09±0.31)级,4组间差异有统计学意义(Pa<0.01)。结论TPM对慢性癫发作所致的海马神经元线粒体损伤有保护作用,FA可加强TPM的线粒体保护作用。     

黄芪对幼兔肠缺血-再灌注肠黏膜屏障的保护作用

目的研究黄芪对幼兔肠缺血再灌注(IIR)肠黏膜屏障的保护作用。方法雄性幼兔20只。随机分成假手术组、IIR组(模型组)、黄芪组、生理盐水组(对照组)。黄芪组及对照组手术前7 d分别予黄芪6 mg/(kg.d)(腹腔注射)和生理盐水。观察幼兔缺血再灌注后小肠组织及血浆超氧化物歧化物(SOD)活性、肠黏膜形态损伤程度及黄芪对其损伤的作用。结果IIR后血浆及小肠组织SOD活性明显降低,小肠黏膜形态及肠黏膜屏障功能损害加重,黄芪能显著提高血浆及小肠组织SOD活性及减轻肠黏膜形态损伤。结论黄芪对幼兔IIR肠黏膜屏障损伤有明显的保护作用。     

丙戊酸对氯化锂-毛果芸香碱致癫(癎)大鼠海马神经元存活的影响

目的 探讨丙戊酸(VPA)对氯化锂-毛果芸香碱致癫(癎)大鼠神经元的保护作用.方法 出生35 d雄性Wistar大鼠分为空白对照组、癫(癎)模型组和VPA干预组,制作氯化锂-毛果芸香碱癫(癎)发作模型,VPA经口灌胃给药,每次350 mg·kg-1.VPA连续给药5 d后处死动物,取大鼠脑组织尼氏染色检测存活神经元,免疫组织化学检测脑源性神经营养因子(BDNF)蛋白和乙酰化H3组蛋白表达.结果 1.空白对照组海马形态学结构正常,细胞完整,神经元多呈三角形,核较大,胞质里可见深染尼氏小体;癫(癎)模型组大鼠海马CA1、CA3区可见神经元缺失、变性、坏死;尼氏小体数量较少,甚至出现尼氏小体溶解、消失;VPA干预组神经元坏死较少,改变较轻.2.癫(癎)模型组海马CA1区存活神经元数目显著少于空白对照组和VPA干预组;VPA干预组存活神经元数目较癫(癎)模型组增加(P<0.01),但仍少于空白对照组(P<0.01);癫(癎)模型组海马CA3区存活神经元数目显著少于空白对照组和VPA干预组(Pa<0.01);VPA干预组存活神经元数目较癫(癎)模型组增加(P<0.01),但仍少于空白对照组(P<0.01).3.癫(癎)模型组和VPA干预组海马CA1、CA3区BDNF蛋白的表达均较空白对照组高(P<0.05,0.01),但VPA干预组的表达高于癫(癎)模型组(P<0.05,0.01).4.VPA干预组CA1、CA3区乙酰化H3组蛋白表达较癫(癎)模型组和空白对照组均显著增加(Pa<0.01),癫(癎)模型组乙酰化H3组蛋白表达亦高于空白对照组(P<0.01).结论 VPA对氯化锂-毛果芸香碱致癫(癎)大鼠海马CA1、CA3区神经元具有保护作用;海马CA1 、CA3区乙酰化H3组蛋白表达增加,进而调控BDNF的表达增加,可能是VPA对癫(癎)后大鼠脑保护作用机制之一.     

Treatment with Probucol of Children with Familial Hypercholesterolemia

ABSTRACT. Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2±1.45 mmol/l to 7.17±0.84 mmol/l (12.6%). This level was further reduced to 5.92±0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.     

Treatment with probucol of children with familial hypercholesterolaemia

Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2 +/- 1.45 mmol/l to 7.17 +/- 0.84 mmol/l (12.6%). This level was further reduced to 5.92 +/- 0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.     

Growth of infants with osteogenesis imperfecta treated with bisphosphonate

Background:  Osteogenesis imperfecta (OI) is a heritable bone disease characterized by bone brittleness and various degrees of growth disorder. Cyclic pamidronate therapy is reportedly useful to prevent bone fracture in OI and in infants with OI, but, it remains unclear how infants with OI grow during bisphosphonate therapy.
Methods:  Height and weight measurements of OI infants treated with cyclic pamidronate therapy were taken before and every 6 months during therapy until 18 months. Vertebral morphometry and the concavity index were analyzed using X-ray films taken simultaneously.
Results:  Among OI patients, those in the group for which the height z- score decreased tended to have more femur fractures than those of the group for which the height z- score increased. Morphometry of the lumbar spine showed that compression fractures occurred less during cyclic pamidronate therapy, by which the lumbar bone mineral density increased.
Conclusions:  Bisphosphonate preserved vertebral morphometry during 18 months after starting therapy in infants. Prevention of femur fracture during the infantile period might help prevent short stature; therapeutic strategies during infancy must better emphasize prevention of long bone fracture before the beginning of gait.     

Treatment of patients with pineoblastoma with high dose cyclophosphamide

The outcome for patients with pineoblastoma has historically been very poor, with most patients dying of disseminated disease despite irradiation. Furthermore, the low incidence of this tumor has hindered progress toward defining better treatment strategies. Here we report the activity and toxicity of cyclophosphamide administered as a single agent at a dose schedule of 2 g/m²/day for 2 successive days at monthly intervals for a maximum of four courses. Eight patients were evaluated, six newly diagnosed and two recurrent. Amongst the six newly diagnosed patients, there were three patients demonstrating partial responses, and three had stable disease throughout the cyclophosphamide treatment period. All six patients are alive and disease free after further therapy. One patient with recurrent disease demonstrated tumor progression on cyclophosphamide, and the other had stable disease throughout the cyclophosphamide treatment period. Both patients subsequently died of progressive disease. The major toxicity of high dose cyclophosphamide was hematopoietic, with one patient requiring a dose reduction after three courses due to prolonged thrombocytopenia. One patient was also withdrawn from treatment with cyclophosphamide due to impaired pulmonary function. This study demonstrates the activity of high dose cyclophosphamide in the treatment of pineoblastoma and may serve as basis for the design of future studies of this tumor. © 1996 Wiley-Liss, Inc.     

Duration of isolation of children with influenza A treated with oseltamivir

The duration of fervescence (24 hours in children or 30 hours in adults) was shorter in the oseltamivir group than in the placebo group. According to a Japanese law enacted before oseltamivir therapy became available, schoolchildren with influenza must be isolated 48 hours after defervescence. Our data suggest isolation should be at least 84 hours for children with influenza A treated with oseltamivir and 108 hours for preschool children.     

Treatment of refractory hemorrhage with factor XIII in a patient with hemophilia A with inhibitor

An 11‐year‐old male with hemophilia A and a known high‐titer Factor VIII inhibitor was admitted with retroperitoneal hemorrhage. The patient was receiving infusions of recombinant activated Factor VII (rFVIIa) for a recent elbow hemorrhage when retroperitoneal bleeding commenced. Despite increased dosing of rFVIIa and a dose of activated prothrombin complex concentrate (aPCC), he continued to hemorrhage and required several blood transfusions. Factor XIII was administered 1 hour after rFVIIa and the patient demonstrated cessation of bleeding and normalization of clot strength. Factor XIII may act as an adjuvant in effective clot stabilization in patients with hemophilia and inhibitory antibodies. Pediatr Blood Cancer 2013; 60: E23–E25. © 2013 Wiley Periodicals, Inc.     

Comparison of patients with Kawasaki disease with retropharyngeal edema and patients with retropharyngeal abscess

Kawasaki disease with retropharyngeal edema (KD with RPE) is a rare complication, and it is diagnosed by neck CT. Most reported cases had a delayed diagnosis because those patients' conditions were misdiagnosed as retropharyngeal abscess (RPA). The purpose of this study was to differentiate KD with RPE from RPA. We performed a retrospective case–control study comparing children with KD with RPE to those with RPA hospitalized at the tertiary pediatric hospital in Tokyo between 2005 and 2011. The 39 patients revealing RPE on neck CT were divided into two groups: group A was classified as KD (n?=?21) and group B was classified as non-KD (n?=?18). Patients in group B were finally evaluated as having RPA clinically and were treated with antibiotic therapy. A significantly higher proportion of patients in group B complained of dysphagia (11 patients vs. 5 patients; p?=?0.0170) and neck pain (17 patients vs. 12 patients; p?=?0.0106). Neck CT revealed a ring enhancement (16 patients vs. no patients; p?<?0.0001) and mass effect in a greater proportion of patients in group B (11 patients vs. 1 patient; p?<?0.0003). Conclusion: Careful attention to manifestations and close analyses of CT imaging may allow clinicians to differentiate KD with RPE from RPA.     

Treatment of children with congenital cytomegalovirus infection with ganciclovir

BACKGROUND: Congenital cytomegalovirus (CMV) infection affects approximately 1% of live births in the US. Ten percent of these infants have symptoms at birth and another 10 to 15% acquire hearing loss or developmental problems. Congenital CMV is the most common cause of nonhereditary sensorineural hearing loss in children, and progressive hearing loss is common. To arrest the natural progression of congenital CMV, children referred to our center were treated with a prolonged course of ganciclovir. METHODS: Medical records of children with congenital CMV who were treated with ganciclovir were reviewed to tabulate their presenting symptoms, duration of treatment, audiologic and developmental assessments and complications. RESULTS: We treated nine children with symptomatic CMV with iv ganciclovir at a median age of 10 days (range, 3 days to 11 months). Findings at diagnosis included microcephaly (five of nine); petechiae (five of nine); thrombocytopenia (seven of nine); and intracranial calcifications (six of eight). Hearing loss was noted before therapy in five of nine. The median duration of iv and subsequent oral ganciclovir was 1 year and 0.83 year, respectively. Median follow-up was 2 years (range, 1 to 7 years). No child had progression of hearing loss; improvement occurred in two. Seven children had at least one complication of ganciclovir therapy: central venous catheter/site infection (six); catheter malfunction (three); and neutropenia (one). CONCLUSION: Of nine children none treated with ganciclovir for congenital CMV had detectable progressive hearing loss. Complications associated with iv therapy occurred frequently. Currently available oral analogues of ganciclovir may facilitate earlier and more prolonged therapy for children with symptomatic congenital CMV and should be subjected to randomized controlled trials.