术前介入在肝母细胞瘤治疗中的应用评价

目的：探讨介入治疗在小儿肝母细胞瘤综合治疗中的地位及适应证。方法：对估计不能切除的肿瘤施以碘油化疗药物栓塞，然后再外科手术，9例中有7例施行了肝叶切除，右三叶3例，右半肝2例，左半肝2例。结果：介入后，小儿副反应轻，肿瘤体积缩小，6例AFP值下降，未施手术1例介入后肺转移死亡，探查术1例后改肝移植术后死亡，肝叶切除7例中，死亡5例（3例复发，1例脑转移，1例肝功能衰竭）存活2例健在。结论：介治疗可以使某些不能Ⅰ期切除的肝母细胞瘤有了手术切除的机会，但要掌握适应证和方法。     

重症肺炎合并心力衰竭患儿血清肌酸激酶同工酶、脑利钠肽及神经肽Y水平变化

目的探讨重症肺炎合并心力衰竭患儿血清肌酸激酶同工酶(CK-MB)、脑利钠肽(BNP)和神经肽Y(NPY)水平的变化。方法入选2010年12月至2014年12月住院普通肺炎患儿32例(普通组),重症肺炎患儿20例(重症组),重症肺炎合并心力衰竭患儿36例(心衰组),以及健康体检儿童26例(对照组),采用酶速率法在ADVIA1650全自动生化分析上测定CK-MB,酶联免疫吸附试验(ELISA)方法检测BNP及NPY的水平,其中18例重症肺炎合并心力衰竭患儿在恢复期再次检测。结果四组间血清CK-MB、BNP和NPY水平比较,差异均有统计学意义(F=25.19~277.94,P均0.01)。心衰组患儿血清CK-MB、BNP和NPY浓度均高于重症组、普通组和对照组,差异有统计学意义(P均0.05);重症组血清CK-MB浓度高于普通组和对照组,差异有统计学意义(P均0.05);普通组与对照组CK-MB浓度比较差异无统计学意义(P0.05);重症组、普通组和对照组三组间血清BNP和NPY浓度比较,差异均无统计学意义(P均0.05)。心衰组18例患儿治疗后的血清CK-MB、BNP和NPY水平均低于治疗前,差异有统计学意义(P均0.001)。心衰组血清BNP水平与CKMB、NPY水平均呈显著正相关(r=0.681、0.525,P均0.01);NPY与CK-MB水平亦呈显著正相关(r=0.545,P0.01)。结论血清CK-MB、BNP及NPY浓度检测有助于诊断儿童重症肺炎合并心衰,其中BNP更为敏感。     

B型脑钠肽与N端脑钠肽前体在新生儿窒息后心力衰竭辅助诊断中的比较

目的：观察并比较B型脑钠肽（ B type brain natriuretic peptide,BNP ）、N端脑钠肽前体（ N-terminal pro-brain natriuretic peptide,NT-proBNP）对新生儿窒息后发生心力衰竭的辅助诊断价值,优化早期临床诊断。方法回顾性分析2013年1月至2015年10月我院收治的124例出生后窒息新生儿的临床资料,根据有无心力衰竭分为心衰组53例和对照组71例,48 h内股静脉穿刺采血检测并比较,将存在差异的指标进行Logistic回归分析,计算BNP、NT-proBNP与窒息患儿左室射血分数的相关性,通过受试者工作特征（ ROC）曲线计算BNP、NT-proBNP对新生儿窒息后发生心力衰竭的诊断效能。结果心衰组患儿BNP[（835.8±154.7）pg／ml vs.（235.4±38.5）pg／ml]、NT-proBNP（25903.8 pg／ml vs.6974.5 pg／ml）、心肌肌钙蛋白T[（0.21±0.06）ng／ml vs.（0.11±0.03）ng／ml]、CK-MB[（61.3±11.7）U／L vs.（40.8±9.5）U／L]均显著高于对照组（P﹤0.05）;Logistic回归分析提示BNP、NT-proBNP与窒息新生儿发生心力衰竭密切相关（ ORBNP ＝3.013,P﹤0.001;ORNT-proBNP ＝3.808,P＝0.006）;BNP、NT-proBNP与左室射血分数呈明显负相关（ rBNP ＝-0.650,P ＝0.007;rNT-proBNP ＝-0.721,P ﹤0.001）。ROC曲线提示BNP、NT-proBNP对新生儿窒息后发生心力衰竭的诊断效能分别为0.868、0.911,截断值分别取268.8 pg／ml、3972.3 pg／ml时诊断一致性最高。结论血浆BNP与NT-proBNP均是反映新生儿窒息后发生心力衰竭的高效特异性指标,对新生儿心力衰竭具有辅助诊断价值,相比较而言NT-proBNP 的早期诊断意义更高。     

N端脑钠肽前体在小儿川崎病诊断中的价值

目的 观察N端脑钠肽前体(NT-proBNP)在小儿川崎病中的变化,探讨其在川崎病诊断中的价值.方法 检测36例典型川崎病患儿和20例不完全川崎病患儿急性期、恢复期血浆NT-proBNP,另检测30例伴有发热并排除了心肌受累的呼吸道感染患儿和30例健康儿童的血浆NT-proBNP,并进行相互比较.同时对川崎病患儿急性期行心彩超检查.结果 不完全川崎病组和典型川崎病组血浆NT-proBNP差异无统计学意义;两组川崎病组急性期血浆NT-proBNP明显高于呼吸道感染组和健康组,恢复期血浆NT-proBNP显著低于急性期;以血浆NT-proBNP＞300 pg/ml诊断川崎病,敏感性为80.35%,特异性为90.00%.川崎病组血浆NT-proBNP＞300 pg/ml的阳性率显著高于并发冠脉病变的阳性率.结论 血浆NT-proBNP可以作为一项早期诊断川崎病的参考指标.     

血N端脑钠肽前体在早产儿症状性动脉导管未闭诊治中的应用价值

目的探讨血浆N端脑钠肽前体(NT-pro BNP)在早产儿症状性动脉导管未闭(s PDA)诊治中的临床应用价值。方法选取2013年10月—2014年9月入住新生儿重症监护病房、胎龄28~32周、出生体质量??1 500 g的早产儿107例,分别于生后第4、7天检测NT-pro BNP,采血后30 min内行超声心动图检查。根据生后第4天超声心动图检查结果分PDA组(39例)与对照组(68例);PDA组根据有无超声血流动力学显著改变及临床表现分为症状性PDA(s PDA组,20例)和无症状性PDA(as PDA组,19例);s PDA组再根据是否服用布洛芬分为治疗组(13例)与非治疗组(7例)。结果生后第4天,s PDA组患儿血浆NT-pro BNP水平高于as PDA组,as PDA组高于对照组,差异均有统计学意义(P??0.05);生后第7天,s PDA组患儿血浆NT-pro BNP水平高于as PDA组和对照组,差异有统计学意义(P??0.05),as PDA组与对照组的差异则无统计学意义(P??0.05)。治疗组生后第7天血浆NT-pro BNP水平较第4天显著下降,差异有统计学意义(P??0.05);非治疗组生后第7天与第4天血浆NT-pro BNP水平的差异无统计学意义(P??0.05)。PDA患儿生后第4天血浆NT-pro BNP水平与动脉导管(DA)直径、左心房/主动脉根部内径比值(LA/AO)及DA直径与左肺动脉内径比值(TDD/LPA)呈正相关(r=0.498~0.670,P均??0.05)。生后第4天血浆NT-pro BNP水平预测s PDA的ROC曲线下面积(AUC)为0.969(95%CI:0.938~1.000),NT-pro BNP水平在13 964 pg/m L时,诊断s PDA的灵敏度为95%,特异度为95.4%。结论 s PDA早产儿血浆NT-pro BNP水平明显增高,治疗后下降。第4天血浆NT-pro BNP是预测s PDA的敏感指标,动态监测血浆NT-pro BNP水平变化对指导早产儿PDA治疗策略的选择有重要临床价值。     

N端脑钠肽前体对新生儿心力衰竭的早期诊断价值

目的检测血浆N端脑钠肽前体(NT-proBNP)在高危新生儿心力衰竭(HF)的表达水平,探讨NT-proBNP对新生儿HF的早期诊断价值。方法收集窒息后HF新生儿35例(HF组)及同期无窒息、无HF新生儿20例(对照组)。于出生第2天、第7天用电化学发光法检测血浆NT-proBNP水平,质量法检测CK-MB水平,采用SPSS16.0软件进行统计学分析。结果与对照组比较,治疗前HF组NT-proBNP、CK-MB水平均高于对照组,差异有统计学意义(P<0.05,0.01);治疗后CK-MB水平仍显著高于对照组(P<0.05),而NT-proBNP水平与对照组比较差异无统计学意义(P>0.05)。与治疗前比较,HF组治疗后NT-proBNP、CK-MB水平均显著降低,差异有统计学意义(P<0.05,0.001);治疗前后对照组CK-MB水平差异无统计学意义(P>0.05),NT-proBNP水平显著降低(P<0.01)。结论联合检测NT-proBNP、CK-MB水平可动态观察心肌损害程度及HF恢复情况,NT-proBNP检测可作为临床早期诊断新生儿HF的指标之一。     

N端脑钠肽原和糖原磷酸化酶同工酶BB在新生儿窒息合并心肌损伤中的变化

目的：探讨N端脑钠肽原(N-terminal pro-brain natriuretic peptide, NT-proBNP )和糖原磷酸化酶同工酶BB (glycogen phosphorylase isoenzyme BB , GPBB)在新生儿窒息合并心肌损伤中的变化及其临床意义。方法：随机选择64例窒息患儿为研究对象（轻度窒息39例,重度窒息25例,其中心肌损伤30例,非心肌损伤34例）,以25例正常新生儿为对照组。采用酶联免疫吸附法（ELISA）测定血浆NT-proBNP 和GPBB水平,同时检测心肌酶、肌钙蛋白I、心电图、X线胸片等。结果：心肌损伤组血浆NT-proBNP 和GPBB水平均明显高于非心肌损伤组和对照组（P<0.01）。重度窒息组血浆NT-proBNP 和GPBB水平明显高于轻度窒息组和对照组（P<0.01）。Spearman秩相关分析显示,窒息患儿血浆NT-proBNP 水平与GPBB、CK-MB、CK、LDH呈显著正相关（P<0.01）;GPBB水平与CK-MB、CK、LDH呈显著正相关（P<0.01）。结论： NT-proBNP 和GPBB均可作为窒息新生儿早期心肌损害的生化标志物,联合检测NT-proBNP 和GPBB对于早期发现窒息合并心肌损伤、判断病情程度、指导治疗具有重要的临床意义。［中国当代儿科杂志,2010,12（4）：252-255］     

川崎病患儿心率变异性与冠状动脉损害的相关性研究

目的 探讨川崎病(KD)患儿的心率变异性(HRV)指标与肌钙蛋白I(cTnI)和氨基末端脑钠肽前体(NT-proBNP)的相关性及其在预后中的应用价值。方法 将130 例KD 患儿分为冠状动脉损害组(n=47, CAL 组)和无CAL 组(n=83, NCAL 组), 同期选取110 例健康儿童为对照组, 29 例非心血管疾病恢复期患儿为非KD 组。各组儿童均行长程HRV 指标检测及分析。检测KD 组及非KD 组患儿血清NT-proBNP 及cTnI 水平。结果 同年龄性别KD 组患儿正常窦性N-N 间期标准差(SDNN)、相邻N-N 间期标准差的平均值(SDNNindex)、相邻N-N 间期之差>50 ms 的心搏数占心搏总数的百分数(PNN50)、极低频功率(VLF)、低频功率(LF)和高频功率(HF)值较对照组均明显下降, LF/HF 值较对照组升高(P<0.05)。CAL 组SDNN、全部记录中每5 min N-N 间期平均值的标准差(SDANN)、SDNNindex、相邻N-N 间期差值的均方根值(rMSSD)、PNN50、VLF、LF 和HF 值均低于对照组和非KD 组, LF/HF 值高于对照组(P<0.05)。CAL 组及NCAL 组的cTnI 和NT-proBNP 水平均高于非KD 组(P<0.05)。KD 患儿cTnI 与SDNN、HF 呈负相关, 与LF/HF 呈正相关(P<0.05);NT-proBNP 与SDNN、SDANN、HF 呈负相关(P<0.05)。结论 HRV 指标对KD 患儿的CAL 判断具有一定的临床意义。     

N端脑钠肽前体及Tei指数对新生儿缺氧性肺动脉高压右心室功能评价的意义

目的 探讨血浆N端脑钠肽前体(NT-proBNP)及Tei指数评价新生儿缺氧性肺动脉高压(HPH)患儿右心室功能的意义.方法 选择2012年6月至2013年11月本院新生儿重症监护病房收治的缺氧性新生儿为观察组,根据生后第1天是否合并肺动脉高压分为HPH组与非HPH组.HPH组依据其生后第7天的肺动脉压力(PASP)是否恢复正常分为HPH恢复组和HPH未恢复组.选择同期入院无缺氧病史的新生儿为对照组.所有研究对象均于生后第1天、HPH组于生后第7天测定PASP、右室Tei指数、右室射血分数(RVEF)、E/A比值;所有对象于超声检查后1h内采集静脉血,测定血浆NT-proBNP水平.结果 研究期间共收治有缺氧史的新生儿72例,合并肺动脉高压46例,其中轻度HPH组20例,中度HPH组18例,重度HPH组8例,非HPH组26例;共纳入对照组22例.生后第1天各组RVEF、E/A比值比较,中度及重度HPH组均低于对照组、非HPH组及轻度HPH组,重度HPH组低于中度组,差异有统计学意义(P＜0.05).生后第1天各组Tei指数及血浆NT-proBNP比较,HPH组和非HPH组均高于对照组,中度及重度HPH组高于非HPH组和轻度HPH组,重度HPH组高于中度HPH组,差异有统计学意义(P＜0.05).生后第7天HPH恢复组RVEF、E/A比值高于第1天,Tei指数及血浆NT-proBNP低于第1天,差异有统计学意义(P＜0.05);HPH未恢复组Tei指数及血浆NT-proBNP低于第1天,差异有统计学意义(P＜0.05),RVEF、E/A比值与第1天差异无统计学意义(P＞0.05).生后第1天Tei指数与血浆NT-proBNP呈正相关(r=0.826,P＜0.001),并且二者均与PASP正相关(r分别为0.555,0.504,P＜0.001).结论 新生儿HPH患儿存在右心功能障碍,其血浆NT-proBNP水平、右室Tei指数与PASP高低密切相关,有助于右心功能障碍的诊断.     

Radiological staging in children with hepatoblastoma

Hepatoblastoma is the most common malignant liver tumour of childhood. Accurate radiological staging is very important, especially in children who are treated according to the protocols of the International Childhood Liver Tumor Strategy Group (SIOPEL). These protocols use risk stratification, based almost entirely on imaging findings, to minimize the treatment for localized tumours and to intensify treatment for extensive tumours and those with extrahepatic spread.     

Survival trends in children with hepatoblastoma

Purpose  Hepatoblastoma (HB) is a relatively rare pediatric malignancy. In this study, we present demographic data and a survival analysis from the largest patient cohort with HB reported to date. Methods  The surveillance, epidemiology, and end results database was queried from 1973 to 2005 for all patients diagnosed with HB. Kaplan–Meier survival analysis was conducted to determine actuarial survival. Cox regression analysis was performed to determine hazard ratios (HR) for prognostic variables. Results   During the study period, 459 patients with HB were identified. Overall 1-, 3-, and 5-year survival rates for the entire patient cohort were 76, 63, and 60%, respectively. Five-year survival improved over time from 36 (1973–1982) to 63% (1983–2005). Predictors of poor survival include: age 2 years and greater (HR 1.566), black race (HR 1.910), diagnosis prior to 1983 (HR 3.327), inability to perform surgical resection (HR 3.857), regional disease (HR 1.939), and distant disease (HR 3.196). Conclusions  Hepatoblastoma continues to challenge surgeons and oncologists. Most children are diagnosed early in life and undergo surgical resection whenever possible. With the advent of efficacious chemotherapy, survival has improved. Older children, black patients, and those who present with advanced disease tend to have poor outcomes. Surgical resection is the single most important predictor of survival.     

Midterm results with hepatectomy after preoperative chemotherapy in hepatoblastoma

We evaluated the results of surgical treatment for hepatoblastoma in infants and children after intensive preoperative chemotherapy, with special reference to histology and extent of liver involvement. The clinical features of 10 children with hepatoblastoma were reviewed regarding response to neoadjuvant chemotherapy, histological subtypes, extent of hepatectomy, operative complications, and prognosis. Response to chemotherapy was measured by volumetric assessment of tumour size by computed tomography scan. Cisplatin and Adriamycin (PLADO regime) up to three cycles markedly reduced the tumour volume on computed tomography (mean regression rate 65.9%); alpha-foetoprotein (AFP) levels also decreased from an initial mean of 16,116.4 ng/ml to 2,050.9 ng/ml. Five patients underwent right hepatectomy, two had right trisegmentectomy, two had left hepatectomy, and one had left trisegmentectomy. Histopathology of resected specimens revealed foetal histology in four patients, poorly differentiated (anaplastic) subtype in three, and mixed histology with mesenchymal components and osteoid formation in three. There was 100% resectability including six unresectable tumours (prechemotherapy). Moreover, hepatic resection tended to be less invasive in patients whose tumours had been much reduced after preoperative chemotherapy. Preoperative administration of cisplatin and Adriamycin reduces the tumour size significantly so that a safe radical hepatectomy can be performed. It also allows early administration of postoperative chemotherapy. Although overall good results were obtained with the current protocol, we also document our experience of unfavourable outcomes in patients with bilobar tumours (despite trisegmentectomy), patients with tumours showing poor response to neoadjuvant chemotherapy, and patients with anaplastic histology. Overall, at a 60-month follow-up we report an 80% survival rate by a combined approach.     

14例儿童肝母细胞瘤综合治疗的疗效观察

目的：肝母细胞瘤是儿童时期最为常见的原发性肝脏恶性肿瘤。新辅助化疗﹢手术﹢术后化疗已成为肝母细胞瘤治疗的基本原则。该文旨在总结肝母细胞瘤综合治疗的疗效,进而探讨合理治疗的策略。方法：回顾性分析14例儿童肝母细胞瘤患者的临床资料,并追踪随访其治疗后的生存状况。结果：12例肝母细胞瘤患儿接受全程治疗,中位随访时间为18个月（1.5~74月）。9例无瘤存活,1例死亡,1例肿瘤转移,1例未发现肿瘤残留但术后甲胎蛋白持续不降。结论：手术及规范化疗能有效提高儿童肝母细胞瘤患者的生存率。［中国当代儿科杂志,2009,11（6）：456-459］     

Surgical treatment of hepatoblastoma in children

Hepatoblastoma is the most common liver malignancy in children. With rare exceptions, complete tumour resection is required to cure the patient. Radical tumour resection can be obtained either with standard partial hepatectomy or orthotopic liver transplantation. At present, the surgical approach to hepatoblastoma differs significantly between treatment groups in different parts of the world. Our aim was to review current surgical policy in hepatoblastoma. All aspects of surgery in hepatoblastoma are discussed, including biopsy, tumour resection principles, modern achievements in the field of liver surgery, and the indications and potential contraindications for liver transplantation. Every effort should be made to resect hepatoblastoma completely either by standard partial hepatectomy or by the use of liver transplantation in difficult or clearly unresectable cases.     

Neoadjuvant chemotherapy before surgery of hepatoblastoma

Though surgical resection is the main stay of treatment for childhood hepatoblastoma (HB), many are unsuitable for radical surgery at diagnosis due to extensive intrahepatic and/or extra hepatic disease. We report experience in five patients of HB from a single institution (2001–2005) with preoperative Neoadjuvant chemotherapy (NACT) followed by surgery. Three patients received cisplatin, doxorubicin; and two cisplatin/vincristine/5-fluorouracil. All showed more than 50% reduction in tumor size confirmed by CT scan. Hepatic resection R0 was performed in all. There was no chemotherapy related toxicity nor post surgical morbidity or mortality. All are disease free at median follow up of 4 years. NACT produces adequate down staging of the HB with acceptable toxicity. Though cisplatin with doxorubicin produced good results, new protocol with cisplatin, vincristine and 5FU is promising without cardiotoxicity.     

儿童初治肝母细胞瘤83例临床特征及预后分析

目的探讨肝母细胞瘤(HB)患儿的临床特征、生存情况及预后危险因素。方法回顾性分析2012年1月至2019年10月郑州大学第一附属医院儿童医院血液肿瘤科收治的83例初治HB患儿的临床资料, 记录患儿的性别、年龄、首发临床表现、治疗前病变范围(PRETEXT)分期、病理类型、病初甲胎蛋白(AFP)、治疗方法及治疗结果。2018年之前确诊的患儿采用"武汉方案"治疗, 2018年之后确诊的患儿采用"儿童肝母细胞瘤多学科诊疗专家共识(CCCG-HB-2016)"方案治疗。Kaplan-Meier生存分析法计算生存率, 单因素分析采用Log-Rank检验, 多因素预后分析采用Cox回归模型。结果 83例患儿中男51例、女32例;发病年龄25.2(9.0, 34.0)月龄, <3岁64例(77%)。最常见的首发临床表现为腹部包块(45例, 54%)。PRETEXT Ⅰ期8例, Ⅱ期43例, Ⅲ期20例, Ⅳ期12例。随访时间40(17, 63)个月, 全组HB患儿1年总体生存率(OS)和无事件生存率(EFS)分别为(84±4)%和(79±5)%, 5年OS和EFS分别为(78±5)%和(76±...     

14例肝母细胞瘤患儿的临床疗效观察

目的：通过对儿童肝母细胞瘤患儿的临床治疗结果的回顾总结,对ICE化疗方案的有效性和安全性进行评估。方法：自2000年6月至2008年6月,14例初发患儿入选,男7例,女7例,中位年龄：1.33岁（范围0.25～8.25岁）。临床分期：Ⅰ期6例,Ⅱ期1例,Ⅲ期5例,Ⅳ期2例。诊断时血甲胎蛋白（AFP）水平显著升高13例,1例AFP正常。采用多科室协作模式进行治疗,其中一期手术8例,3例进行了二期手术。化疗方案采用ICE方案,14例患儿共接受了73个疗程化疗,其中术前化疗25个疗程。结果：14例患儿治疗后有效12例（85.7%）,其中完全缓解10例（71.4%）,部分缓解2例,2例无效。随访至2008年7月31日,疾病处于长期完全缓解者9例（64.3%）,中位随访时间为35个月（范围：16～96个月）。5年总生存率（OS）为：（70.71±12.37）%,5年无事件生存率（EFS）为：（64.29±12.81）%。1例患儿复发,2例失访。结论：ICE化疗方案联合手术治疗能有效并且安全地治疗儿童肝母细胞瘤,Ⅳ期患儿的治疗有待于进一步研究。［中国当代儿科杂志,2009,11（8）：659-662］     

Successful multimodal therapy with chemotherapy and surgery for bilateral pulmonary metastases secondary to hepatoblastoma

Two children with hepatoblastoma and bilateral pulmonary metastases were effectively treated with an aggressive combination of chemotherapy and surgery. Chemotherapeutic agents included cyclophosphamide, vincristine, THP-adriamycin, and cis-platinum. The pulmonary metastases were temporarily responsive to a regimen of these agents, recurred, and were subsequently resected without further recurrence. Such multimodal therapy is advocated for the treatment of pulmonary metastases secondary to hepatoblastoma.