地中海贫血患儿血清乳酸脱氢酶活性分析及临床意义

目的探讨血清乳酸脱氢酶(LDH)在地中海贫血患儿中的变化及临床意义。方法对近年住我院的地中海贫血患儿的血清乳酸脱氢酶进行检测,并与急性溶血组与非溶血组比较。结果地中海贫血组LDH活性较非溶血组明显升高(P<0.01)。地贫合并感染时LDH明显升高,与地贫非感染状态下比较有差异性(P<0.01),与急性溶血组比较无差异性(P>0.05)。地贫组中,Hb<60 g/L时LDH活性较Hb>60 g/L时明显升高(P<0.05)。结论地中海贫血患者血清乳酸脱氢酶活性升高,合并感染时升高明显,与溶血程度呈正相关,可作为病情观察的重要指标。     

乳酸脱氢酶在儿童难治性肺炎支原体肺炎诊断和治疗中的意义

目的 探讨乳酸脱氢酶(lactic dehydrogenase,LDH)在儿童难治性肺炎支原体肺炎(refractory Mycoplasma pneumoniae pneumonia,RMPP)诊断和治疗中的意义.方法 对2013年6月至2016年6月南方医科大学附属深圳妇幼保健院儿科收治的肺炎支原体肺炎(Mycoplasma pneumoniae pneumonia,MPP)患儿,均给予红霉素治疗.根据RMPP的定义,将其分为普通MPP组和RMPP组.RMPP组采用红霉素基础上添加甲泼尼龙治疗.根据疗效,将RMPP患儿分为有效组和无效组.所有患儿均检测血LDH、WBC计数、C-反应蛋白、红细胞沉降率(erythrocyte sedimentation rate,ESR)、丙氨酸氨基转移酶(ALT)、天门冬氨酸氨基转移酶(AST)及肌酸激酶(CK).分别比较RMPP组和普通MPP组、有效组和无效组之间在治疗前后上述指标有无差异.结果 共纳入253例受试者,其中普通MPP组161例,RMPP组92例.与普通MPP组相比,RMPP组患儿年龄更大,LDH、ESR、ALT、AST水平更高(P＜0.05).Logistic回归分析显示,LDH(OR=1.029,95%CI 1.020~1.037)、ESR(OR=1.063,95%CI 1.009~1.120)为RMPP的预测因素(P＜0.05).受试者工作特征曲线表明,LDH临界值为400.50U/L时,曲线下面积最大为0.959,95%CI 0.936~0.983.RMPP组添加甲泼尼龙治疗后,患儿临床症状迅速改善,有效组LDH、ESR水平显著降低(P＜0.05).结论 血清LDH可能为早期识别RMPP和判断疗效的一个重要指标.     

Lactate dehydrogenase in hypothermia-treated newborn infants with hypoxic-ischaemic encephalopathy

Aims: We investigated whether plasma lactate dehydrogenase (LDH) predicts outcome in hypothermia (HT)‐treated term infants with moderate/severe hypoxic‐ischaemic encephalopathy (HIE) and additionally whether LDH differs between infants with evidence for acute and nonacute perinatal insults and postnatal collapse (PNC). Methods: Data from HT‐treated infants with HIE (n = 39) were analysed retrospectively. Adverse outcome was defined as a Mental and/or Psychomotor Developmental Index (Bayley Scales of Infant Development II), at 18 months <70. The likely timing of insult onset was assessed in infants with an LDH sample obtained within 6 h of birth or PNC (n = 20). Results: LDH differed between the favourable/adverse outcome groups at the end of HT treatment (median (IQR) 1540 (1400–1950)U/L vs. 3555 (3003–8705)U/L, (p < 0.01)). All infants (n = 22) with LDH <2085U/L had a favourable outcome while 6 of 11 infants with LDH ≥ 2085U/L had an adverse outcome. LDH in those who died (n = 4) was higher than the favourable outcome group (5090 (2915–12222)U/L, (p < 0.01)) but sampled earlier. Early LDH differed significantly (p < 0.01) between infants with evidence for acute or nonacute insults or PNC. Conclusion: These results offer a biomarker, with high negative predictive value in the assessment of outcome in HT‐treated term infants, needing prospective validation.     

Abnormal lactic dehydrogenase isoenzymes in patients of Indian childhood cirrhosis and their siblings

In fifteen patients with Indian childhood cirrhosis (I.C.C.) total lactic dehydrogenase (LDH) activity was increased as compared with controls (P<0.001). The isoenzyme pattern in I.C.C. patients was quite distinctive from that observed in normal controls and in patients with acute viral hepatitis. In I.C.C. there was a two fold increase in LDH isoenzyme-1, and a decrease in LDH-2 and LDH-4. Sixty per cent patients with I.C.C. had abnormal bands between LDH-1 and 2 and LDH-2 and 3. Siblings of the patients with I.C.C. had normal total LDH activity, and showed double ringed bands in the region of LDH 1–3. The abnormal bands in patients of I.C.C. are explained on the basis of hybridization of LDH with other serum components.     

Changes in serum lactate dehydrogenase activity in children with atopic dermatitis

Background: In recent years an increase has been seen in the number of patients with severe atopic dermatitis (AD) accompanied with generalized typical eruptions. Some markers indicating the severity of the disease and symptom changes are very useful, and therefore the purpose of the present study was to investigate serum lactate dehydrogenase (LDH) as such a marker. Methods: A total of 58 children with AD were enrolled. The severity of the disease was graded on the basis of the extent of eruptions and the severity of atopic symptoms. The fraction of serum LDH, number of eosinocytes in the peripheral blood, and serum IgE levels were also determined. Results and Conclusion: There was a close correlation between the severity of cutaneous symptoms and serum LDH activity, and between severity and eosinocyte count, but no relationship was seen between serum IgE levels and severity of the disease. The aforementioned factors were determined in a time‐related way. As the patients' condition improved, serum LDH activity tended to decline, but there were no consistent changes in eosinocyte count in the peripheral blood or serum IgE level. On LDH isozyme the levels of LDH4 and LDH5 were high. Tissue showed high LDH activity, especially in epidermides. These results suggest that serum LDH activity is a useful marker.     

先天性神经母细胞瘤发病机制与临床特点的研究进展

神经母细胞瘤是儿童最常见的颅外实体肿瘤,来源于肾上腺髓质或交感神经节.先天性神经母细胞瘤约占神经母细胞瘤患儿总数的5％,大多数患儿于出生后1个月内确诊.与1岁以上神经母细胞瘤患儿相比,新生儿神经母细胞瘤有其独特的病程.本文就先天性神经母细胞瘤的发病机制、临床表现、治疗方法以及预测患儿长期预后的生物学因素进行综述.     

C-反应蛋白、血沉、乳酸脱氢酶及血清铁蛋白联合检测对儿童发热待查病因诊断价值的探讨

目的探讨C-反应蛋白(CRP)、红细胞沉降率(ESR)、乳酸脱氢酶(LDH)及血清铁蛋白(SF)联合检测对发热待查患儿病因诊断的临床应用价值。方法回顾性分析热程2周以上的发热待查住院患儿154例的临床资料,并根据出院诊断分为感染组(n=54)、风湿组(n=67)、恶性肿瘤组(简称为肿瘤组,n=33),对3组患儿血清CRP、ESR、LDH及SF 4项指标的均值进行比较,并通过ROC曲线分析其单独及联合检测对发热待查患儿病因的诊断价值。结果感染组、风湿组、肿瘤组3组患儿血清CRP和ESR均升高,其中风湿组升高最明显;血清LDH在肿瘤组升高最明显;SF在风湿组和肿瘤组均明显升高。LDH对风湿性疾病、CRP和ESR对恶性肿瘤诊断的ROC曲线下面积(AUC)0.7(P0.05)。CRP诊断感染和风湿性疾病的AUC分别为0.861、0.782;ESR诊断感染和风湿性疾病的AUC分别为0.770、0.743;LDH诊断感染和恶性肿瘤的AUC、灵敏度、特异度及约登指数均较低;SF诊断感染的AUC、灵敏度、约登指数均为最高,但特异度最低;SF诊断风湿性疾病的AUC、灵敏度、特异度、约登指数都较高;SF诊断恶性肿瘤的AUC较低。4项指标联合检测对诊断风湿性疾病和恶性肿瘤的AUC、灵敏度、特异度比单独检测时高。结论在发热待查患儿的病因诊断中,CRP、ESR、LDH及SF对初步诊断风湿性疾病有一定临床意义,对感染性疾病和恶性肿瘤的诊断和鉴别价值有限;4项指标联合检测对发热待查患儿的病因诊断价值优于单独检测。     

基于决策曲线和剂量反应分析评估乳酸脱氢酶对儿童难治性肺炎支原体肺炎的预测价值

目的 探讨乳酸脱氢酶（LDH）对儿童难治性肺炎支原体肺炎（RMPP）的预测价值。方法 通过倾向性匹配法获得73例RMPP患儿为难治组,146例非难治性的普通MPP患儿为普通组,利用logistic回归、限制性立方样条模型和决策曲线分析评估LDH对RMPP的临床预测价值。结果 难治组和普通组高热发生率、WBC计数、血小板计数、中性粒细胞百分比及血清C反应蛋白、降钙素原、血红蛋白、白蛋白、谷氨酸-丙酮酸氨基转移酶、天门冬氨酸氨基转移酶、LDH含量的比较差异有统计学意义（P < 0.05）。两组鼻咽抽吸物MP-DNA载量及胸腔积液、肺实变、肺不张、气促、皮肤损害发生率的比较差异有统计学意义（P < 0.05）。多因素logistic回归分析显示,高热、血红蛋白水平、LDH水平、肺实变是RMPP发生的独立预测因素（OR分别为10.097、0.956、1.006、3.756,均P < 0.05）。限制性立方样条分析结果显示,LDH连续性变化与RMPP发生的关联强度呈非线性剂量反应关系（P < 0.01）。决策曲线分析显示LDH对RMPP的预测有重要临床价值。结论 LDH是儿童RMPP发生的独立预测因素,与RMPP发生的关联强度呈非线性剂量反应关系。     

Intensive chemotherapy in children with stage IV neuroblastoma

A retrospective analysis of effectiveness of sequential chemotherapy with cyclophosphamide, doxorubicin, cisplatin and etoposide in children with stage IV neuroblastoma was undertaken. Study group included 17 children of mores than one year old with median age of 3 years (range 18 months to 7 years). Fourteen were males and three females. Sites of primary tumor were abdomen in 12 patients, pelvis in 3, paravertebral in 1 and unknown in 1. Metastatic sites included bone marrow (88%), bone (82%), orbit (29.4%) and lymph node (11.7%). One patient had brain parenchymal disease and another had cerebrospinal fluid positivity for malignant cells. Fifteen of the 17 patients had major response with chemotherapy (complete response in two and partial response in 13). Ten of the 15 patients completed four courses of chemotherapy and five patients progressed while on chemotherapy and died. Only two of the ten patients, who had four courses chemotherapy are alive after 2 years. Hence the 2-year survival in this series is 11.7%. There was no toxic death in this study.     

血乳酸清除率在小儿脓毒性休克中的应用

血乳酸是反映全身组织灌注情况和细胞内是否缺氧的敏感标志物.近年来研究发现,脓毒性休克时血乳酸明显增高,当休克得到改善,血乳酸可很快下降.因此,测定体内乳酸的清除率可准确提示脓毒性休克的血流动力学改变情况并对其预后作出有效评估.该文就血乳酸清除率在小儿脓毒性休克中的研究进展作一概述.     

microRNA在神经母细胞瘤的研究进展

神经母细胞瘤是儿童最常见的颅外恶性实体肿瘤,肿瘤转移及复发是其死亡的重要原因.神经母细胞瘤的发生与胚胎发育异常密切相关,但其确切的发病机制、分化成熟及转移的机制并未完全阐明.微小RNA在机体胚胎发育过程和肿瘤的发生中起到重要作用,并与肿瘤发展及预后密切相关.该文就微小RNA在神经母细胞瘤的研究进展作一综述.     

Lactate dehydrogenase as an indicator of severe illness in neonatal intensive care patients: a longitudinal cohort study

Aim: Lactate dehydrogenase (LDH) increases in several critical conditions that cause cell damage and could potentially be used for early detection of serious illness in the newborn. Our aim was to investigate the relationship between the early clinical course of NICU infants and LDH in plasma at admission. Methods: LDH was measured in a cohort of patients consecutively admitted to a major NICU in Hanoi. The infants were classified as ‘obviously needing intensive care during the first week’ (n = 83) or ‘not receiving intensive care measures during the first week’ (n = 260) by a senior neonatologist blinded to the LDH and lactate activity. Results: LDH differed significantly between the groups in infants born after 32 gestational weeks. LDH differed with the vitality of the patient (F = 26.25, p < 0.0001) at admittance and correlated with lactate (R = 0.496, p < 0.0001). Also, the predictive value for obvious need of intensive care was higher for LDH than for lactate assessed by area under the curve calculated with ROC-curves [0.82 (0.77–0.88) vs. 0.67 (0.60–0.75)]. Conclusion: There is a strong relationship between bad clinical condition of infants during first week of life and elevated plasma LDH. The results suggest that LDH might be a valuable support in decision making in the neonatal period.     

神经母细胞瘤中hTR、hTERT基因的表达

目的探讨人端粒酶RNA（hTR）、人端粒酶逆转录酶（hTERT）基因在神经母细胞瘤自然逆转成熟及恶性增殖中的表达及其意义。方法应用原位杂交方法检测34例神经母细胞瘤、5例神经节神经母细胞瘤、8例神经节细胞瘤、16例胎儿肾上腺髓质、20例新生儿肾上腺髓质中hTR、hTERT基因的表达。结果hTR、hTERT基因在病理分型F型中的阳性表达均为46．15％（6／13）；uF型中阳性表达分别是85．71％（18／21）、90．48％（19／21）；两组比较差异有显著性意义（X^2=6．05，P〈0．05；X^2=8．10，P〈0．05）。在神经节神经母细胞瘤中hTR、hTERT基因的阳性表达均为20％。神经节细胞瘤中hTR、hTERT基因的阳性表达分别是25％、12．5％；二组与神经母细胞瘤比较差异有显著性意义（X^2=11．70，P〈0．05；X^2=10．81，P〈0．05）。在胎儿及新生儿肾上腺髓质中hTR、hTERT基因为弱阳性及阴性。结论在神经母细胞自然逆转成熟及恶性增殖是时hTR、hTERT起了重要的作用，当hTR、hTERT基因表达减弱，神经母细胞具有自然逆转成熟的趋势，当hTR、hTERT基因表达阳性，提示神经母细胞增殖力增强，因此hTR、hTERT基因的表达可作临床判断预后的指标。     

血乳酸和乳酸清除率评估脓毒性休克患儿预后的临床分析

目的 评价血乳酸及乳酸清除率与脓毒性休克患儿预后的关系.方法 2009年10月至2011年6月我院PICU收治的脓毒性休克患儿42例,所有患儿均按照脓毒性休克诊疗方案进行早期目标指导治疗,测定每例患儿复苏前及复苏后1h、2h、6h血乳酸值,计算复苏后1h、2h、6h的乳酸清除率.按照预后将患儿分为存活组(n=20)和死亡组(n=22),评价早期乳酸值和乳酸清除率与预后的关系.结果 (1)存活组复苏前及复苏后1h、2h、6h的乳酸值明显低于死亡组[(3.92±2.58) mmol/Lvs (6.91 ±4.16) mmol/L,(2.79±1.89) mmol/L vs (7.93±4.39) mmol/L,(2.20±1.83) mmol/L vs(9.20±4.97) mmol/L,(1.32±0.51) mmol/L vs (9.94±5.02) mmol/L],差异具有统计学意义(P＜0.05).(2)存活组复苏后1h、2h、6h的乳酸清除率明显高于死亡组[(26.31 ±20.82)％ vs(-24.28±53.39)％,(43.46±17.85)％ vs(-34.31±58.98)％,(61.04±16.71)％ vs(-45.33±83.51)％],差异具有统计学意义(P＜0.05).(3)通过受试者工作特征曲线分析,复苏后6h血乳酸值和乳酸清除率的曲线下面积分别为99.4％、96.7％;复苏后6h血乳酸＞2.20 mmol/L及复苏后6h乳酸清除率＜18.65％,患儿病死率高.结论 脓毒性休克患儿动态监测血乳酸具有重要意义,复苏后6h的血乳酸值及乳酸清除率可作为预后判断的指标.     

儿童神经母细胞瘤预后因子及危险度分级计划探讨

目的对23例NB和17例GNB临床资料进行生存分析，确定独立预后因子，制定新的可行性危险度分级计划。方法运用Cox回归模型，引入年龄、性别、INSS、病理类型、血NSE、24h尿VMA、血LDH和原发部位相关预后因子，进行多因素分析，确立独立预后因子。以独立预后因子为依据，计算每例患儿的预后指数（prognostic index，PI），计算公式为：PI=b1x1＋b2x2＋…bnxn（b：回归系数，x：独立预后因子变量等级值）。以PI中位数为界值，PI≤中位数为低危组，PI〉中位数为高危组，采用Kaplan-Meir生存曲线和Log-rank检验进行生存分析。结果年龄、INSS和24h尿VMA为独立预后因子，PI中位数为10．5145，低危组和高危组对生存率的影响差异具有统计学意义（P=0．001），说明低危组较高危组预后好。新的危险度分级计划为：确定年龄、INSS和24h尿VMA等级；计算PI，具体计算公式：PI=2．066X age＋1．285X VMA＋1．268X INSS；确定危险度组别，低危组：PI≤10．5145；高危组：PI〉10．5145。结论年龄、INSS和24h尿VMA为独立预后因子。由于上述独立预后因子临床上较易获得且Log-rank检验具有统计学意义，所以由此确立的新的危险度分级计划具有实用性和科学性，可以作为基层医生选择化疗强度和判断预后的依据。     

Lactate dehydrogenase activity is increased in plasma of infants with advanced necrotizing enterocolitis

In infants with necrotizing enterocolitis (NEC), intestinal gangrene defines advanced disease. Since intestinal ischemia is considered a pathogenetic factor for intestinal gangrene, serum activity of mucosal and seromuscular enzymes may be elevated in these patients. Our aim was to evaluate if serum enzymes activity is increased in infants with NEC associated with intestinal gangrene. We performed a retrospective review of the case notes of infants operated on for NEC between 1998 and 2006. Patients with preoperative determination of serum enzymes were included in the study, and were divided into Group A and Group B based on the presence or absence of intestinal gangrene, respectively. Serum activities of alkaline phosphatase (ALP), glutamic oxaloacetic transaminase (GOT), creatine kinase (CK), and lactate dehydrogenase (LDH) were compared in the two Groups. Values are medians (interquartile range). Thirty-five infants were operated on for NEC in the study period. Eighteen patients fulfilled the inclusion criteria: 12 in Group A and six in Group B. Group A patients had significantly higher LDH activity [1131.0 (1092.0-1300.0) vs. 482.0 (440.0-624.5) IU/L; P < 0.005]. Our findings suggest that LDH activity may be increased in infants with NEC and intestinal gangrene. Its evaluation could be a further tool in the surgical decision making process in infants with NEC.     

Management of Wilms' tumor and neuroblastoma

Wilms' tumor and neuroblastoma resemble each other in usual presentation and by their occurrence in young children. However they represent a dichotomy in advances in treatment. In Wilms' tumor, survival of patients has dramatically improved as a result, of multimodal therapy, whereas in neuroblastoma little advance in treatment has occurred. Development of rational chemotherapeutic regimens through better understanding of drugs and of cell cycle kinetics as well as investigation of other modalities of therapy, i.e. immunotherapy, etc., may be helpful in improving survival in the years to come.