不同病因急性心力衰竭患儿血清氨基末端脑钠素原水平变化及其与肺动脉高压的关系

目的探讨先天性心脏病、肺炎并心力衰竭患儿血清氨基末端脑钠素原(NT-proBNP)水平变化及其与肺动脉高压(PH)的关系。方法采用竞争性酶免疫法检测66例住院心力衰竭患儿(肺炎并心力衰竭25例,先天性心脏病并肺炎心力衰竭22例,先天性心脏病并中重度PH心力衰竭19例)及60例对照组患儿(肺炎、先天性心脏病、健康儿童各20例)外周血NT-proBNP水平。多普勒超声心动图检测心力衰竭患儿心室射血分数(LVEF)、缩短分数(FS),对先天性心脏病患儿估测肺动脉压力。结果1.肺炎心力衰竭组及先天性心脏病心力衰竭组NT-proBNP水平无显著差异(P>0.05),但二组均较相应对照组(肺炎对照组、先天性心脏病对照组)显著增高(Pa<0.001);先天性心脏病并中重度PH心力衰竭组较先天性心脏病无PH或轻度PH心力衰竭组水平亦明显增高(Pa<0.05)。2.三组心力衰竭患儿间LVEF、FS水平比较差异无显著性(Pa>0.05)。3.肺炎对照组、先天性心脏病对照组较正常对照组NT-proBNP水平略高,但无统计学意义(Pa>0.05)。结论血清NT-proBNP对肺炎心力衰竭、先天性心脏病并心力衰竭均有较敏感而特异的诊断价值,且随肺动脉压力增高而呈增高趋势。     

氨基末端脑钠素原在小儿先天性心脏病并肺动脉高压的诊断价值

目的探讨氨基末端脑钠素原(NT-proBNP)在小儿先天性心脏病(先天性心脏病)并肺动脉高压(肺高压)的诊断价值。方法2005年1月至2007年3月山东大学齐鲁儿童医院内科采用竞争性酶免疫法检测20例正常对照组小儿及65例先天性心脏病患儿血清NT-proBNP浓度。65例先天性心脏病患儿经用彩色多普勒超声心动图估测肺动脉压力,分为三组:无肺高压组20例、轻度肺高压组23例、中重度肺高压组22例。结果1、先天性心脏病无肺高压组较正常对照组NT-proBNP水平略有增高,但无明显统计学差异(P>0.05)。2、先天性心脏病并轻度肺高压组NT-proBNP水平明显高于先天性心脏病无肺高压组(P<0.001)。3、中重度肺高压组患儿NT-proBNP水平较轻度肺高压组明显增高(P<0.001)。4、以500 fmol/m l诊断轻度肺高压的敏感度为91%,特异度为90%,且经直线相关分析,显示NT-proBNP与肺高压高低呈正相关,r=0.80。结论血清NT-proBNP检测对先天性心脏病并肺高压具有敏感而特异的诊断价值,且可反映肺动脉压增高程度。     

支气管肺炎患儿血清脑钠肽水平与心功能的相关性

目的 探讨支气管肺炎患儿心功能状态与血清脑钠肽(BNP)水平的相关性.方法选取支气管肺炎患儿52例.其中并心力衰竭(心衰)患儿20例.男9例,女11例;年龄4个月～4岁.未并心衰患儿32例.男17例,女15例;年龄6个月～5岁.健康对照组婴幼儿30例.男16例,女14例;年龄6个月～5岁.3组儿童均抽取静脉血离心后留取血清,采用ELISA法测定其血清BNP水平,采用全自动生化分析仪测定其CK-MB水平,同时应用心脏超声诊断仪测定及计算心功能指标.结果支气管肺炎并心衰患儿血清BNP水平[(182.45±57.32) ng/L]、CK-MB水平[(48.19±16.08) IU/L],均高于健康对照组[(35.12±13.84) ng/L、(11.48±4.29) IU/L] (Pa＜0.01),支气管肺炎未并心衰患儿血清BNP及CK-MB水平分别为(43.69±19.55) ng/L、(13.06±5.64) IU/L,与健康对照组比较,均无显著性差异(Pa＞0.05).支气管肺炎并心衰患儿左心室射血分数(LVEF)、左室小轴缩短率(FS)、主动脉峰值流速(PFVA)及肺动脉峰值流速(PFVP)均较健康对照组明显降低(Pa＜0.01),支气管肺炎未并心衰患儿与健康对照组比较,均无显著性差异(Pa＞0.05).血清BNP水平与CK-MB水平呈正相关(r=0.28P＜0.05),与LVEF 、FS、PFVA、PFVP均呈负相关(r=-0.65,-0.58,-0.32Pa<0.01;r=-0.39P<0.05).血清CK-MB与LVEF、FS均呈负相关(r=-0.30,-0.26Pa＜0.05),与PFVA、PFVP均无相关性(Pa＞0.05).结论血清BNP水平可作为支气管肺炎患儿并心衰的实验室指标.     

先天性心脏病并肺动脉高压患儿血清基质金属蛋白酶-1、2与金属蛋白酶组织抑制物-1水平变化的意义

目的 探讨先天性心脏病(CHD)并肺动脉高压(PH)患儿血清基质金属蛋白酶(MMP-1、2)、金属蛋白酶组织抑制物(TIMP-1)水平变化的意义.方法 将81例左向右分流型CHD患儿按治疗前肺动脉收缩压(SPAP)分为4组:非PH组16例(SPAP＜4.0 kPa)、轻度PH组21例(4.0 kPa≤SPAP＜5.33 kPa)、中度PH组21例(5.33 kPa≤SPAP＜9.33 kPa)、重度PH组23例(SPAP≥9.33 kPa).中、重度PH组19例予前列腺素E1(PGE1)治疗,轻、中度24例接受手术治疗.选21例健康体检儿童为健康对照组.用ELISA检测其血清MMP-1、2和TIMP-1水平,并计算MMP-1/TIMP-1、MMP-2/TIMP-1比值.结果 CHD组MMP-1、2与健康对照组比较均显著增高,并PH后TIMP-1显著增高(Pa＜0.05).非PH、轻和中度PH组MMP-2/TIMP-1与对照组比较均显著增高(Pa＜0.05);MMP-1/TIMP-1与对照组比较均无统计学差异(Pa＞0.05).19例经PGE1治疗14 d后,MMP-1、2和TIMP-1与治疗前比较均显著下降(Pa＜0.01).24例手术治疗1周后,MMP-1、2 、TIMP-1和MMP-2/TIMP-1与术前比较均显著降低(Pa＜0.05).MMP-1与TIMP-1、MMP-1与MMP-1/TIMP-1、MMP-2与MMP-2/TIMP-1均呈显著正相关(Pa＜0.05).结论 1.MMP-1、2参与肺血管重构的全过程;2.PGE1可逆转肺血管重构;3.介入治疗、外科手术阻断了CHD异常的血流动力学这一肺血管重构的始动因子.     

先天性心脏病并心力衰竭患儿血浆氨基末端脑利钠肽前体水平变化的临床意义

目的 观察先天性心脏病(CHD)并心力衰竭(HF)患儿血浆氨基末端脑利钠肽前体(NT-proBNP)水平的变化,分析NT-proBNP与HF严重程度的关系及在心功能评估中的价值.方法 选择本院2008年6月-2011年6月住院CHD患儿46例,并根据纽约大学儿童HF指数(NYU PHFI)的评价标准将其分成3组:无HF组(15例)、轻度HF组(16例)、中重度HF组(15例).选取同期25例健康儿童作为健康对照组.分别留取各组儿童空腹静脉血2 mL,应用ELISA法测定其血浆NT-proBNP水平,同时测定其左心室射血分数(LVEF)、二尖瓣舒张早期流速峰值/二尖瓣舒张晚期流速峰值(E/A值).分析其血浆NT-proBNP水平与HF严重程度及LVEF、E/A值的相关性.结果 血浆NT-proBNP水平与HF严重程度呈明显正相关(r=0.82,P＜0.01),HF程度越重,血浆NT-proBNP水平升高越显著;中重度HF组NT-proBNP水平显著高于轻度HF组(P＜0.01),轻度HF组NT-proBNP水平显著高于无HF组(P＜0.01),无HF组NT-proBNP水平显著高于健康对照组(P＜0.05).血浆NT-proBNP水平与LVEF呈负相关(r=-1.20,P＜0.01).结论 血浆NT-proBNP水平随着HF的严重程度增加而升高,检测血浆NT-proBNP对评估CHD并HF患儿病情严重程度及判断预后具有重要临床意义.     

先天性心脏病合并肺动脉高压患儿血浆内皮素、降钙素基因相关肽的临床研究

目的 探讨先天性心脏病(congenital heart disease,CHD)患儿血浆内皮素(endothelin-1,ET-1)、降钙素基因相关肽(calcitonin gene-related peptide,CGRP)浓度的变化以及与肺动脉压力间的关系.方法 选择健康体检儿30例为对照组,无肺动脉高压(pulmonary hypertension,PH)的CHD 30例为无PH组,CHD合并轻度PH患儿20例为轻度PH组,CHD合并中重度肺动脉高压20例为中重度PH组.采用放射免疫法测定血浆ET-1、CGRP浓度.结果 CHD患儿血浆ET-1明显高于对照组(P<0.01),且轻度、中重度PH组明显高于无PH组及对照组(P<0.01);CHD无PH组CGRP浓度低于对照组(P<0.01),且轻度、中重度PH组明显低于无PH组及对照组(P<0.01);血浆ET-1与肺动脉压力呈正相关(r=0.977,P<0.01),CGRP与肺动脉压力呈负相关(r=-0.81,P<0.05);血浆ET-1与血浆CGRP水平呈显著负相关(r=0.843,P<0.01).结论 CHD患儿ET-1升高、血浆CGR降低,两者的变化与肺动脉压有相关性,可能参与了肺血管的重构及PH的形成.     

血清生长分化因子-15与先天性心脏病患儿心功能的关系及其对心力衰竭的诊断价值

目的：探讨生长分化因子-15（GDF-15）与先天性心脏病患儿心功能的关系及其对心力衰竭的诊断价值。方法：2011年3月至2012年5月成都市妇女儿童中心医院就诊的先天性心脏病患儿97例,依据改良Ross评分法分为心力衰竭组（71例）和无心力衰竭组（26例）。ELISA法检测两组血清GDF-15和B型脑钠肽前体(NT-proBNP)水平,超声心动图检测左室射血分数(LVEF)。采用Spearman相关分析对GDF-15与改良Ross评分、LVEF及NT-proBNP的相关关系进行分析。绘制ROC曲线并计算GDF-15曲线下面积及诊断心力衰竭的截断值。结果：心力衰竭组患儿血清GDF-15和NT-proBNP水平较无心力衰竭组显著增高,LVEF明显降低,差异有统计学意义(P<0.01)。血清GDF-15水平与改良Ross评分及血清NT-proBNP水平呈正相关（分别r=0.705和0.810,P<0.01）,与LVEF呈负相关（r=－0.421,P<0.01）。GDF-15诊断心力衰竭的ROC 曲线下面积为0.757,当GDF-15的诊断界值为1306 ng/L,敏感性和特异性分别为68.8%和71.2%。结论：先天性心脏病合并心力衰竭患儿血清GDF-15明显升高,血清GDF-15水平与先天性心脏病患儿心功能、LVEF及NT-proBNP水平均有不同程度的相关关系;GDF-15可能成为诊断小儿先天性心脏病合并心力衰竭的一项检测指标。     

心力衰竭患儿血浆脑钠肽变化及临床意义

目的 探讨心力衰竭患儿血浆脑钠肽(BNP)变化及其与心功能的关系.方法 研究对象为2005年4月至2007年1月在宜宾市第二人民医院住院的心力衰竭患儿43例,应用ELISA方法测定患儿血浆BNP浓度,M型超声心动图测量左室射血分数(LVEF)、短轴缩短率(FS),并比较BNP与LVEF、FS的相关性.结果与正常对照组比较,心力衰竭患儿血浆BNP水平明显升高,心功能参数LVEF、FS显著降低(t值分别为8.092、3.780、3.864.P值分别<0.001、<0.01、<0.01).心衰程度越重.血浆BNP水平升高越明显(F=4.26,P<0.01).心功能Ⅰ级组患儿血浆BNP水平已明显升高(t=4.708,P<0.01),心功能参数LVEF、FS降低不明显.不同原发病心力衰竭患儿血浆BNP无差别.血浆BNP水平与LVEF和FS存在负相关关系(r分别为-0.568,-0.325,P值分别<0.05,<0.001).结论 心衰惠儿血浆BNP水平显著升高,随着患儿心衰程度加重,血浆BNP水平增高越显著.血浆BNP水平是心衰患儿心脏功能更敏感的生化指标.     

肺炎并心力衰竭婴幼儿血清心肌肌钙蛋白Ⅰ和脑钠肽变化及其与心功能的关系

目的 探讨肺炎并心力衰竭(肺炎心衰)婴幼儿血清心肌肌钙蛋白I(cTnI)和脑钠肽(BNP)水平变化及其与心脏收缩功能的关系. 方法随机选取体检健康儿童30例(健康对照组).男17例,女13例;年龄5个月～3岁.同期选择重症肺炎心衰患儿30例(肺炎心衰组).男21例,女9例;年龄3个月～2岁.一般肺炎患儿30例(一般肺炎组).男16例,女14例;年龄3个月～3岁.抽取各组患儿静脉血2～3 mL分别以酶联免疫吸附法(ELISA)及微粒子免疫发光法(MEIH)测定血清BNP、cTnI水平.同时均行心脏超声测定其左室射血分数(LVEF)和左室短轴缩短率(LVFS).采用SPSS 11.0软件进行统计学分析.结果 肺炎心衰组血清cTnI[(0.389±0.030) ng/L]和BNP[(0.572±0.090) μg/L]水平升高,LVEF和LVFS水平降低;与一般肺炎组和健康对照组比较均有显著性差异(Pa＜0.05);肺炎心衰组血清cTnI与LVEF和LVFS均呈直线负相关(r=-0.87,-0.76 Pa＜0.05);肺炎心衰组血清BNP水平与LVEF和LVFS亦均呈直线负相关(r=-0.97,-0.85 Pa ＜0.05).结论 血清cTnI和BNP水平可作为判断肺炎心衰患儿心肌损害及心衰程度的指标.     

先天性心脏病患儿介入封堵术前后血浆血管紧张素Ⅱ和血清一氧化氮水平的变化

目的 观察先天性心脏病(CHD)介入封堵术前后血浆血管紧张素Ⅱ(AngⅡ)和血清一氧化氮(NO)水平的变化,探讨其在CHD并肺动脉高压(PH)中的作用及其意义.方法 选取90例CHD患儿.均经体检、辅助检查及心脏彩色B超确诊.其中单纯房间隔缺损40例.单纯室间隔缺损30例,单纯动脉导管未闭20例.依据心导管测压,将90例CHD患儿分为3组:中重度PH组(肺动脉收缩压6.66 kPa)30例和轻度PH组(3.99 kPa<肺动脉收缩压<6.66 kPa)30例、无PH组(肺动脉收缩压<3.99 kPa)30例.3个亚组的年龄及性别无统计学差异.分别采用放射免疫法和硝酸还原酶法测定各组术前、术后24 h、术后1、3、6及12个月血浆AngⅡ及血清NO水平.另选取年龄及性别与之相匹配的健康体检儿童30例作为健康对照组,用同样方法检测其血浆AngⅡ及血清NO水平.结果 介入封堵术前,CHD并PH组患儿血浆AngⅡ水平明显高于CHD无PH组(Pa<0.01);CHD并PH组患儿血清NO水平均明显低于CHD无PH组(Pa<0.01).CHD患儿介入封堵术24 h后,血浆AngⅡ逐渐下降,至12个月下降接近正常水平;CHD患儿介入封堵术24 h后,血清NO开始逐渐上升,至12个月上升接近正常水平.AngⅡ与肺动脉收缩压呈显著正相关(r=0.811 P<0.01);NO与肺动脉收缩压呈负相关(r=-0.421 P<0.05).结论 AngⅡ及NO可能参与CHD的发生、发展过程;血浆Ang Ⅱ及血清NO水平可作为判定其CHD患儿程度的参考指标之一.     

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 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     

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.     

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.