米力农治疗婴幼儿先天性心脏病并肺动脉高压的疗效观察

目的 探讨米力农治疗婴幼儿先天性心脏病(CHD)并肺动脉高压 (PAH)的临床疗效及血流动力学、心功能指标的变化和意义.方法 选择广西壮族自治区人民医院2004年7月-2007年7月收治的45例婴幼儿CHD并PAH患儿.随机分为米力农治疗组(米力农组,n=25)及多巴胺加酚妥拉明治疗组(多巴胺组,n=20).米力农组予米力农0.5 μg /(kg·min);多巴胺组予多巴胺和酚妥拉明,均为5 μg/(kg·min).二组均持续静脉给药5 d,观察临床疗效、血流动力学和心功能指标变化,并进行比较.结果 米力农组用药后5 d,患儿临床症状、体征好转,血流动力学及心功能指标较治疗前显著改善(Pa<0.05),总有效率为92.0%;多巴胺组治疗前后血流动力学及心功能指标改善不明显(Pa>0.05),总有效率为75.0%.米多农组与多巴胺组治疗后临床疗效、血流动力学和心功能指标比较差异均有统计学意义(Pa<0.05).米力农组患儿心率、血压较治疗前均无显著变化(Pa>0.05),无严重心律失常发生.结论 米力农具有正性肌力和扩血管的作用,对肺血管床有选择性扩张作用,能明显改善CHD婴幼儿并PAH的心功能,降低其肺动脉压力,不影响其血压和心率,其临床疗效优于多巴胺.     

米力农对先天性心脏病并肺动脉高压及心力衰竭患儿的术前干预作用

目的 探讨米力农对小儿先天性心脏病(CHD)并肺动脉高压(PAH)及心力衰竭(CHF)术前治疗的效果.方法 收集2006年1月-2008年7月上海交通大学附属儿童医院ICU收治的CHD患儿40例.年龄1个月～3岁.均为左向右分流型CHD,并PAH及CHF.将患儿随机分为研究组与对照组,各20例,二组病种、年龄、心功能及PAH程度比较无显著性差异.二组患儿均应用强心、利尿及扩血管治疗,另外,研究组20例加米力农静脉维持[0.5 μg/(kg·min)],对照组20例加多巴胺和多巴酚丁胺各5μg/(kg·min)静脉维持.分别于用药前、静脉维持用药72 h及停药后2 h通过彩色多普勒超声心动图检测二组各项心脏收缩、舒张功能及肺动脉压力指标.结果 研究组用药72 h各项心脏收缩功能指标(心脏指数、左室射血分数及左事短轴缩短率)和舒张功能指标(左室收缩时间间期、右室收缩时间间期及二尖瓣E峰和A峰比值)均显著优于对照组(Pa<0.05),肺动脉压力下降程度显著高于对照组(P<0.05);研究组改善心功能有效率显著高于对照组(P<0.05).结论 米力农对CHD并PAH及CHF患儿具有正性肌力和扩血管作用,能明显改善心功能和降低肺动脉压力,为手术纠正心脏畸形提供良好时机.     

咪达唑仑持续静脉泵入治疗儿童癫(癎)持续状态的疗效观察

目的 观察咪达唑仑治疗儿童癫(癎)持续状态的疗效及不良反应.方法 将癫(癎)持续状态患儿按随机分配原则分为2组:研究组给予咪达唑仑0.3 mg/kg静注,15 min后若发作未能控制,以1～2 μg/(kg·min)持续静脉泵入,每15min增加1 μg/(kg·min),最大量20 μg/(kg·min).对照组给予安定0.3mg/kg静注,15 min后若发作未能控制,以5 μg(kg·min)持续静脉泵入.每15分钟增加.5 μg/(kg·min),最大量50 μg/(kg·min).结果 咪达唑仑组7例癫(癎)发作完全控制,1例在药量达20 μg/(kg·min)癫(癎)发作仍未控制,本组患儿控制癎痴持续状态的有效率为87.5%.安定组6例癫(癎)发作完全控制,1例在药量达50 μg/(kg·min)癫(癎)发作未控制,本组患儿控制癫(癎)持续状态的有效率为83.3%.两组控制惊厥疗效及控制惊厥药物起效时间的比较无显著性.安定组1例肌张力减低和1例呼吸抑制.咪达唑仑组未发现血压、心率、血氧饱和度和呼吸状态的变化.结论 咪达唑仑治疗癫(癎)持续状态起效快,效果好,不良反应小,可用于治疗癫(癎)持续状态.     

1,6-二磷酸果糖治疗新生儿窒息并心肌损害

目的探讨1,6-二磷酸果糖治疗新生儿窒息后心肌损害的效果。方法新生儿窒息并血清心肌酶谱变化85例患儿,随机分为治疗组45例和对照组40例;二组患儿均采用综合治疗。治疗组予1,6-二磷酸果糖0.10~0.25g/(kg.次),2次/d,快速静脉滴注(10min内滴完),疗程7~10d,入院12h内及治疗后查血清心肌酶谱。结果治疗与对照组治疗后肌酸激酶(CK)、肌酸激酶同工酶(CK-MB)、α-羟丁酸脱氢酶(HBDH)比较差异均具有显著性意义(Pa<0.05)。结论1,6-二磷酸果糖对新生儿窒息心肌损害有一定预防及治疗作用。     

儿童肺动脉高压276例病因学分析

目的 探讨儿童肺动脉高压(PAH)的病因及相关因素,以提高儿童PAH的早期诊断和治疗.方法 回顾性分析1995年5月-2007年5月北京大学第一医院儿科住院患儿中包含PAH诊断并经超声心动图检查证实为肺动脉压力升高的病例,记录其年龄、性别、临床主要诊断、PAH相关症状及超声心动图估测的肺动脉压力,按病因进行分组统计,对不同病因组间经多普勒超声心动图三尖瓣返流速度估测的肺动脉收缩压(sPAP)进行比较.不属于威尼斯会议PAH临床诊断分类中第一类的PAH未统计在内.结果 共诊断PAH 276例.男168例,女108例;年龄1个月～17岁,中位年龄9个月.其中特发性PAH 9例(3.3%),余267例(96.7%)均为相关性PAH,其中以先天性心脏病(先心病)相关性PAH为主,共245例(88.7%),其中又以室间隔缺损等左向右分流先心病为主,共217例(88.6%),复杂先心病相关PAH 28例(11.4%).结缔组织病相关PAH 19例(6.9 %),PAH在SLE、幼年型类风湿关节炎及多发性大动脉炎中的发病率分别为10.3 %(13/126)、8.7%(4/46)、15.4%(2/13),余2例与门脉高压相关,1例与珠蛋白生成障碍性贫血相关.经三尖瓣返流速度估测的8例特发性PAH患儿 sPAP为(74.6±23.9) mmHg(1 mmHg=0.133 kPa),显著高于33例先心病相关PAH[(58.0±19.7) mmHg]及12例结缔组织病相关PAH[(49.6±18.9) mmHg](t=-2.052,-2.609 Pa<0.05).结论 儿童PAH主要是由多种病因引起的相关性PAH,常见病因为先心病和结缔组织病,特发性PAH患儿确诊时病情已较严重.     

N-乙酰半胱氨酸对先天性心脏病患儿体外循环心肌缺血再灌注损伤的保护作用

目的 探讨N-乙酰半胱氨酸(NAC)预处理在体外循环(CPB)心脏直视手术中的心肌保护作用.方法 先天性心脏病患儿26例(房间隔缺损和室间隔缺损各13例),随机分为对照组和NAC组,每组各13例.NAC组患儿在术前常规处理的基础上于术前3 d开始口服NAC[10 mg/(kg·次),2次/d,连用3 d],对照组术前常规处理同NAC组.二组分别于术前(T1)、主动脉阻断15 min(T2)、主动脉开放后30 min(T3)抽取静脉血2 mL,用于CK、CK-MB测定.同时于主动脉开放约30 min取其心肌组织,观察其心肌超微结构变化,记录其术后血管活性药物应用情况及术后拔管时间、ICU留置时间. 采用SPSS 13.0软件进行统计学分析.结果 二组患儿血清CK、CK-MB在T2 、T3 时间点显著升高,与T1时间点比较均有显著性差异(Pa<0.05),且在T3 时间点NAC组血清CK、CK-MB水平均显著低于对照组(Pa<0.05).NAC组心肌超微结构受损较对照组轻.NAC组术中、术后多巴胺、多巴酚丁胺应用剂量均低于对照组(Pa<0.05),术后拔管时间、ICU留置时间与对照组比较均无显著性差异(Pa>0.05).结论 NAC预处理可使CPB心内直视术中患儿心肌超微结构损害减轻,心肌酶漏出减少,减轻CPB心内直视术中心肌的缺血再灌注损伤.     

静脉营养对早产儿免疫功能的影响

目的 观察早产儿60例静脉营养对血清三酰甘油(TG)水平及体液、细胞免疫功能的影响.方法 早产儿随机分为A、B、C 3组,分别在出生第2天开始静脉滴注氨基酸和脂肪乳剂.A组:单用氨基酸;B组:氨基酸加中/长链脂肪乳;C组:氨基酸加长链脂肪乳.各组氨基酸和脂肪乳剂均从1.0 g/(kg·d)开始,每日递增0.5 g/kg,直至3.0 g/(kg·d),共用7 d.分别于出生第1天治疗前、第9天治疗后采集静脉血,监测TG,免疫球蛋白IgA、IgM、IgG,补体C3、C4和T淋巴细胞亚群CD3、CD4、CD4/CD8及NK细胞值.结果 治疗后3组患儿TG均有不同程度增高(Pa<0.05),B组、C组均高于A组(Pa<0.05).与治疗前比较,3组IgG水平均显著下降(Pa<0.01),IgM水平均显著升高(Pa<0.05),治疗后除A组C3、C4、CD3、CD4/CD8和B组CD3有所增高(Pa<0.05)及C组NK细胞有所下降外(P<0.05),余各指标均无显著性差异(Pa>0.05).与A、B组比较,C组NK细胞明显下降(Pa<0.05).3组治疗前后IgA、IgM、IgG、C3、C4、CD3、CD4/CD8的变化均无统计学意义.结论 早产儿出生第2天开始输注脂肪乳剂,7 d后均可引起TG明显增高,但在安全范围内;氨基酸及中/长链脂肪乳剂对体液免疫、细胞免疫均无明显影响,长链脂肪乳剂输注7 d后可引起NK细胞下降.     

吸入伊洛前列素对先天性心脏病患儿术后肺动脉高压的治疗作用

目的 评价吸入伊洛前列素对先天性心脏病术后肺动脉高压的疗效,并初步探讨其机制.方法 以中国医学科学院阜外心血管病医院小儿心脏中心PICU收治的体外循环下双心室矫治手术后合并肺动脉高压13例患儿为研究对象,在术后48 h内常规治疗的基础上,给予有效剂量伊洛前列素25 ng/(kg·min),每次吸入10 min,每4小时1次.观察患儿的血流动力学改变,采用酶联免疫吸附法检测血浆cAMP和cGMP浓度.结果 13例患儿平均年龄(17.88±12.56)个月,平均体质量(9.29±3.59)kg.治疗前右房压、肺动脉收缩压、肺动脉收缩压/主动脉收缩压比值分别是(11.5±1.41)mm Hg(1 mm Hg=0.133 kPa)、(61.64±13.6)mm Hg和0.66±0.16;治疗后20 min分别降至(9.88±1.88)mmHg、(47.67±12.18)mm Hg和0.52±0.15;治疗前后差异均有显著性(P＜0.01).治疗后120 min伊洛前列素疗效仍然存在.治疗前患儿血浆cAMP浓度为(335.75±127.31)μg/L,治疗后20 min升至(519.68±148.54)μg/L,治疗前后差异有显著性(P＜0.01).治疗前后血压、呼吸机条件和血浆cGMP浓度没有明显变化(P＞0.05).结论 对于先天性心脏病术后合并肺动脉高压患儿,吸入伊洛前列素可以显著降低肺动脉压力、改善肺循环血流动力学状态,可能与增高血浆cAMP浓度有关系.吸入伊洛前列素对体循环和呼吸功能影响小,未观察到明显不良反应.     

痰热清注射液治疗合并鹅口疮的婴儿急性上呼吸道感染40例疗效分析

目的 探讨合并鹅口疮的婴儿急性上呼吸道感染(简称上感)治疗方法.方法 79例住院治疗的合并鹅口疮婴儿急性上感患儿随机分为两组.两组入院后均常规给予制霉菌素碳酸氢钠混悬液涂抹患儿口腔黏膜,观察组应用痰热清注射液0.5 ml/(kg·d)静脉滴注,对照组应用阿莫西林100mg/(kg·d)、病毒唑10 mg/(kg·d)分别静脉滴注,治疗5 d观察疗效.结果 观察组显效27例(67.50%),有效8例,无效5例,总有效35例(87.50%);对照组显效14例(35.90%).有效9例,无效16例,总有效23例(58.97%).两组显效率、总有效率比较差异均有非常显著性(χ2分别为7.90、8.23,P均<0.01).出院4周鹅口疮复发情况:观察组3例(7.50%)、对照组13例(33.33%),两组比较差异同样有非常显著性(χ2=8.16,P<0.01).结论 痰热清治疗合并鹅口疮的婴儿上感有效率和总有效率均明显优于对照组,观察组鹅口疮复发率明显低于对照组,而且没有明显不良反应,提示痰热清治疗合并鹅口疮的婴儿上感效果较好,值得临床推广应用.     

感染性休克兔胃黏膜二氧化碳分压变化的意义

目的 观察感染性休克兔胃黏膜与动脉血二氧化碳分压差[pg-a(CO2)]的变化.方法 日本大耳白兔16只,麻醉后压力控制通气,将动物随机分成休克组和对照组,每组各8只.休克组静脉输注稀释的大肠杆菌内毒素(2 mg/kg)复制感染性休克模型,对照组输入等量9 g/L盐水.多导生理记录仪连续监测平均动脉血压和心率.多普勒超声流量计持续测定心排出量和肠系膜上动脉血流量.气体张力测定法每10分钟测定1次胃黏膜二氧化碳分压.每小时测定Hb水平、动静脉血气分析、动脉血乳酸.结果 对照组各参数维持稳定,休克组变化明显.与基础状态比较,休克组兔静脉注射内毒素后2 h,动脉血压由(78±5)mmHg降至(50±2)mmHg(1 mmHg=0.133 kPa)(F=145.3 P<0.01),心脏指数由(201±17)mL/(kg·min)降至(112±11)mL/(kg·min)(F=80.5 P<0.01),肠系膜上动脉血流指数由(20.0±1.0)mL/(kg·min)降至(10.6±1.3)mL/(kg·min)(F=60.0 P<0.05),p(g-a)(CO2)由(11.4±2.8)mmHg升至(57.4±7.1)mmHg(F=120.9 P<0.01).氧输送则由(31.4±2.9)mL/(kg·min)降至(16.6±2.0)mL/(kg·min)(F=75.5 P<0.01),氧消耗由(11.2±1.8)mL/(kg·min)降至(5.1±0.7)mL/(kg·min)(F=46.4 P<0.01).休克后2组各参数比较均有显著性差异(Pa<0.01).结论 p(g-a)(CO2)可作为感染性休克兔胃肠低灌注的指标.     

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.     

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.     

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.     

Treatment of Children with Advanced-Stage Lymphoblastic Lymphoma with Pegaspargase

Objective: To evaluate the feasibility of Pegaspargase instead of L-asparaginase to treat children with advanced-stage lymphoblastic lymphoma (LBL) on the Berlin-Frankfurt-Munster (BFM)-95 protocol. Methods: Fifty-four newly diagnosed patients with stage III or IV LBL and without any treatment were enrolled in this study. Pegaspargase took place of L-asparaginase in BFM-95. The complications and treatment responses of patients treated on the BFM-95 protocol and modified BFM-95 protocol were then evaluated respectively. Findings : For LBL patients treated with BFM-95 protocol or modified BFM-95 protocol, the complete response, event-free survival, overall survival were similar. Stage 4 myelosuppression was the most common complication in both groups. Besides that, among 31 patients receiving modified BFM-95 protocol, coagulation defects were the most common complication. In contrast, anaphylactic reaction was the most common complication in the other 23 patients receiving BFM-95 protocol. Conclusion: Modified BFM-95 protocol is available to children with advanced-stage LBL with an equal outcome and enhances its compliance and decreases the incidence of anaphylactic reaction, compared to BFM-95 protocol. Coagulation defects are the major complication and tolerable in modified one.Key Words: Pegaspargase, L-Asparaginase, Lymphoblastic Lymphoma, Chemotherapy     

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.