儿童地中海贫血复合缺铁性贫血表型及临床意义

目的了解地中海贫血(地贫)患儿合并缺铁性贫血(IDA)时血液学各指标的变化,为临床治疗和筛查提供理论依据。方法通过外周血细胞计数和毛细管电泳筛查确定可疑阳性患儿,采用反向点杂交(RDB)和跨越缺口PCR(Gap-PCR)方法确定初筛阳性患儿的基因突变类型。同时应用化学发光方法检查患儿的血清铁蛋白情况。利用统计学方法比较单纯地贫患儿与地贫复合IDA患儿以及单纯缺铁患儿在血液学各参数上的差异。结果静止型地贫或轻型地贫复合IDA后会加重地贫患儿的症状,中重度地贫患儿在其复合缺铁后血液学指标改变并不明显。结论应该关注地贫患儿的缺铁情况,达到有效预防,合理治疗目的。     

先天性心脏病心肌损害临床分析

目的 探讨先天性心脏病(先心病)患儿心肌损害程度与缺氧及心功能的关系,为心肌保护提供理论依据。方法 测定115例先心病患儿心肌酶谱和58例先心病心肌肌钙蛋白I(CTnI)。结果 115例中A、D、E组心肌酶谱改变以乳酸脱氢酶(LDH)、乳酸脱氢酶同工酶1(LDH1)、肌酸激酶同工酶(CK-MB)、α-羟丁酸脱氢酶(α-HBDH)增高明显,在各组心肌酶谱结果比较及与正常值比较中,两组有显著差异或非常显著差异(P<0.05或P<0.01);CK-MB／CK>0.05。先心病患儿58例中A、D、E组cTnI检出阳性率明显升高(P<0.05),且A、D、E组中cTnI与LDH、LDH1、CK、α-HBDH阳性率比较,各组间有显著差异(P<0.05),与CK-MB比较,无显著差异(P>0.05)。结论 LDH、LDH1、CK-MB、CK-MB／CK、α-HBDH、cTnI是判断先心病患儿心肌损害重要指标;CK-MB与cTnI是诊断心肌损害的血清金标准。     

肺炎支原体肺炎患儿心肌酶谱动态分析

目的 对婴幼儿与儿童肺炎支原体(MP)肺炎的心肌酶谱的动态变化进行检测,了解二者在发病早期与恢复期的不同。方法 婴幼儿组和儿童组MP肺炎均在发病后1周和3周早晨取空腹血测定血清心肌酶谱及其同工酶。结果 心肌酶谱在发病第1周时儿童组血清门冬氨酸氨基转氨酶(AST)、肌酸激酶(CK)、肌酸磷酸激酶同工酶(CK-MB)、α-羟丁酸脱氢酶(α-HBD)均高于婴幼儿组,两组比较有显著性差异(P<0.01)。发病后3周经治疗血清心肌酶和同工酶婴幼儿组均高于儿童组,差异有显著性(P<0.01)。婴幼儿组中2次测定乳酸脱氢酶(LDH),均高于儿童组,且差异有显著性(P<0.01)。结论 MP肺炎感染早期儿童组心肌损害较婴幼儿组明显。发病后3周经治疗婴幼儿心肌损害较儿童组明显且迁延。婴幼儿组病程中LDH均高于儿童组,可能与LDH敏感性高、恢复慢、特异性差及婴幼儿肺部表现较儿童严重、且缺氧也更明显有关。     

珠蛋白生成障碍性贫血并缺铁性贫血患儿的临床特征

目的观察珠蛋白生成障碍性贫血(地贫)高发区小儿地贫并缺铁性贫血(IDA)的临床特征。方法回顾性分析25例地贫并IDA的地贫突变类型和治疗效果。比较地贫并IDA组与单纯地贫组、单纯IDA组的血常规参数。结果 25例患儿中,轻型β-地贫14例,轻型α-地贫7例,中间型α-地贫3例,中间型β地贫1例。补铁治疗有效,血红蛋白升高(21.24±7.62)g.L-1。单纯地贫组红细胞容积、红细胞分布宽度和血小板计数较地贫并IDA组与单纯IDA组稍高(P<0.05),2组平均血红蛋白水平无明显差异(P>0.05)。结论在地贫高发区地贫患儿并IDA较常见,对地贫患儿需进行常规铁代谢指标测定。     

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。     

血清酶学变化在评估新生儿缺氧缺血性脑病病情和预后中的价值

目的：了解缺氧缺血性脑病(HIE)时血清酶学改变及对病情判断、预后的相关性。方法：对住院治疗的99例HIE患儿进行血清酶学检测,并选择同期住院的非HIE患儿38例为对照组。结果：①HIE病情越重,血清酶升高越显著。②天冬氨酸转氨酶、乳酸脱氢酶、肌酸激酶、肌酸激酶同工酶于疾病初期升高非常显著,病情越重,第1日升高越明显,5日后均显著下降,仅γ-谷氨酰转肽酶呈升高趋势。③CT分度越重,血清酶升高越显著。CT分度轻度与重度之间,乳酸脱氢酶、肌酸激酶同工酶升高水平差异有显著性意义,P<0.05及<0.01。④血清酶升高与预后有显著相关性,天冬氨酸转氨酶、乳酸脱氢酶、肌酸激酶血清水平治愈组与死亡及病重组、新生儿期后治疗组对比,经显著性检验,P<0.01及0.05,表明HIE预后不良者升高尤为显著。结论：血清酶水平对病情、预后判断具有一定的价值。     

肿瘤标记物联合检测在儿童神经母细胞瘤诊治中的意义

目的探讨24 h尿香草扁桃酸(VMA)、神经元特异性烯醇化酶(NSE)、乳酸脱氢酶(LDH)、血清铁蛋白(SF)及C-反应蛋白(CRP)在儿童神经母细胞瘤(NB)的诊断及疗效评估中的作用。方法对33例确诊为NB患儿的临床资料进行回顾性分析,应用高效液相色谱仪通过化学比色法测定24 h尿VMA;采用放射免疫法检测血清NSE和SF;应用全自动生化分析仪通过速率法测定血清LDH;采用固相免疫双抗体夹心法测定血CRP。分析以上患儿在初诊时5种肿瘤标记物的阳性率,对比初诊与化疗2个疗程后各指标的检测值;对比出现疾病进展的10例患儿在初诊、化疗2疗程后及进展时各标记物的检测值,并对比分析进展患儿与未进展患儿的检测值。结果初诊时5种肿瘤标记物阳性率均在65%以上,其中24 h尿VMA阳性率最高,达92.3%,其次为NSE,阳性率83.6%,LDH、SF及CRP阳性率分别为82.2%、75.6%及67.9%。经2个疗程化疗后,尿VMA、NSE、LDH及SF水平均初诊时明显下降(P<0.01)。在出现疾病进展时,尿VMA、NSE、LDH及SF较化疗2疗程后均升高(P<0.01),且均高于正常值,而疾病进展组的尿VMA、NSE、LDH明显高于未进展组(P<0.01)。结论 5种肿瘤标记物VMA、NSE、SF、LDH及CRP联合检测对NB早期诊断及评价疗效有一定价值。     

重型β地中海贫血合并急性免疫性溶血──附4例报告

重型β地中海贫血主要表现为慢性血管外溶血,目前较常用的治疗方法是反复输血,尤其高量输血,使血红蛋白Ｈｂ维持在１００ｇ／Ｌ以上［１］。合并感染常加重贫血,但罕见引起急性血管内溶血。β地贫出现血管内溶血较多见于ＡＢＯ或其他血型错型输血的溶血反应,关于β地贫和并免疫性溶血的报道国内外均极少［２］［５］,作者等曾报道２例［３］,现将近期临床诊断２例,一共４例分析报告如下。一、临床资料１．一般资料４例患儿均为１９９６～１９９８年本院住院病人,男３例,女１例,年龄９个月～２岁,确诊重刑β地中海贫血１个月～２…     

血清心肌肌钙蛋白Ⅰ诊断川崎病急性期心肌损伤的临床价值

为探讨血清心肌肌钙蛋白Ⅰ(cTnI)诊断川崎病(KD)急性期心肌损伤的临床价值。检测KD组(n=40)及对照组(n=23)患儿血清cTnI、肌酸激酶(CK)、肌酸激酶同功酶(CK-MB)、乳酸脱氢酶(LDH)与谷草转氨酶(GOT)浓度。结果显示:①KD组与对照组血清CK、LDH、GOT浓度无显著性差别(P>0.05);而血清cTnI,CK-MB浓度明显高于对照组水平(P<0.001)。②在诊断KD患儿急性期心肌损伤上cTnI优于CK-MB(P<0.05),。结果表明:cTnI与CK-MB对KD患儿急性期心肌损伤有诊断价值;与CK-MB比较,cTnI具有高特异性、灵敏度。     

新生儿硬肿症血清心酶谱研究

新生儿硬肿症是国内较常见的疾病,病死率较高。本院曾报道新生儿硬肿症患儿血清肌酸激酶(CK)、乳酸脱氢酶(LDH)、天冬氨酸转氨酶(AST)活性升高。但     

Serum LDH values in childhood acute leukemias and non-Hodgkin's lymphoma

Serum lactate dehydrogenase activity (LDH) was examined in 66 children with acute lymphoblastic leukemia (ALL), 26 with acute non-lymphocytic leukemia (ANLL), and 116 with non-Hodgkin's lymphoma (NHL). The mean serum LDH value for the ALL and ANLL groups was not significantly different: 970 +/- 105 units/L (mean +/- standard error of the mean) and 817 +/- 161 units/L, respectively. The difference between the LDH values in patients with ALL (970 +/- 105 units/L) and NHL (551 +/- 51 units/L) was significant (P = .001). In 32% of the patients with ALL and 23% of the patients with ANLL, serum LDH values were above 1000 units/L, whereas only 13% of the cases with NHL had values above 1000 units/L. In patients with ALL the LDH levels were correlated with white blood cell counts at the time of diagnosis. In NHL, there was no difference in serum LDH levels among the various histologic subtypes. Values of LDH in stage IV NHL and in ALL were similar.     

Increased cytosol aminopeptidase and lactate dehydrogenase in serum originating from lymphocytes in measles and rubella infection

We determined cytosol aminopeptidase (c-AP; EC 3.4.11.1) and lactate dehydrogenase (LDH) levels in serum; these enzymes are known to originate from lymphocytes in patients with measles and rubella. In patients with measles (n = 19), both enzyme levels increased markedly with the onset of rash: mean (+/- SD) c-AP was 269.7 +/- 103.5 U/L and LDH was 1149.5 +/- 255.2 U/L. In patients with rubella, activities of both enzymes increased mildly: c-AP (n = 18) was 81.6 +/- 24.4 U/L and LDH (n = 13) was 674.0 +/- 168.8 U/L. Increased c-AP and LDH levels in patients with measles and rubella presumably originate from the destruction of infected, activated lymphocytes, especially T lymphocytes.     

应用血液学指标诊断新生儿地中海贫血

目的　成人中应用血液学指标筛查地中海贫血的报道较多,但成人的血液学诊断值不能应用于新 生儿。本研究旨在评价红细胞平均体积(MCV)、红细胞脆性、红细胞体积分布宽度(RDW)对新生儿地中海贫血的 诊断价值。方法　以386例在本院新生儿科接受治疗的高未结合胆红素血症患儿为研究对象,根据地贫基因诊断 结果分为地贫组(n=35)和非地贫组(n=351)。检测患儿MCV、红细胞脆性、RDW等血液学指标,作出ROC曲 线,分别计算曲线下面积和各指标的最佳临界值以及相应的敏感度、特异度。结果　地贫组的MCV、RDW和脆性 分别是80±8fL、16.2%±1.0%、31%±13%,而非地贫组为94±9fL、15.8%±1.0%、46%±14%,其中两组 MCV和红细胞脆性的差别具有显著意义(均P<0.01)。在诊断地贫时,MCV的ROC曲线下面积(AUCROC)为 0.877,最佳临界值为88fL,该临界值的敏感度和特异度分别是92%和73.5%;红细胞脆性的AUCROC为0.796,最 佳临界值为37.5%,该临界值的敏感度和特异度分别是85%和75%;RDW的AUCROC为0.630,最佳临界值为 15.9%,该临界值的敏感度和特异度分别是73%和58%。结论　MCV和红细胞脆性均可作为新生儿地贫诊断的 有效指标,且MCV的诊断价值优于红细胞脆性。     

Elevated tricuspid regurgitation velocity in congenital hemolytic anemias: Prevalence and laboratory correlates

Elevated tricuspid valve regurgitation jet velocity (TRV ≥ 2.5 m/s) is associated with mortality among adults with sickle cell disease (SCD), but correlative biomarkers are not studied according to treatment exposure or genotypes. To investigate the associations between biomarkers and TRV elevation, we examined the relationship between TRV and hemolytic, inflammatory, and cardiac biomarkers, stratified by disease‐modifying treatments and SCD genotype. In total, 294 participants with SCD (mean age, 11.0 ± 3.7 years) and 49 hereditary spherocytosis (HS; mean age, 22.9 ± 19.75 years) were included for comparison and enrolled. TRV was elevated in 30.7% of children with SCD overall: 18.8% in HbSC/HbSβ⁺‐thalassemia, 28.9% in untreated HbSS/HbSβ⁰‐thalassemia, 34.2% in HbSS/HbSβ⁰‐thalassemia hydroxyurea‐treated, and 57% in HbSS/HbSβ⁰‐thalassemia chronic transfusion treated. TRV was elevated in 10.7% and 27.8% in HS children and adults, respectively. In children with SCD, elevated TRV was correlated with hemoglobin (odds ratio [OR] = 0.78, P = 0.004), lactate dehydrogenase (LDH; OR = 2.52, P = 0.005), and N‐terminal pro‐brain natriuretic peptide (NT‐pro BNP; OR = 1.003, P = 0.004). In multivariable logistic regression, adjusting for genotype, sex, hemolytic index, and treatment, hemoglobin concentration remained the only significant variable associated with elevated TRV in untreated HbSS/HbSβ⁰‐thalassemia participants. TRV was not associated with inflammatory markers, other markers of hemolysis, or NT‐pro BNP in untreated HbSS/HbSβ⁰‐thalassemia. Neither hemoglobin nor LDH was associated with TRV in HbSC/HbSβ⁺‐thalassemia. These results suggest that elevated TRV is influenced by the degree of anemia, possibly reflecting sickling as part of the disease pathophysiology. Prospective studies should monitor hemoglobin concentration as children with SCD age into adulthood, prompting initiation of TRV screening and monitoring.     

儿童难治性肺炎支原体肺炎临床特征分析

目的 通过比较难治性肺炎支原体肺炎（RMPP）和一般肺炎支原体肺炎（MPP）患儿临床特征的差异,旨在为儿童RMPP的早诊断、早治疗提供科学依据。方法 选取2015年10月至2016年12月期间MPP住院患儿703例为研究对象。根据诊断标准将患儿分为RMPP组（n=152）和MPP组（n=551）。对两组患儿在基本情况、临床表现、感染指标和心肌酶谱上的差异进行统计学比较分析。结果 两组患儿性别构成及年龄比较差异均无统计学意义（P > 0.05）。RMPP组入院第1天的热峰高于MPP组（P < 0.01）;听诊哮鸣音的比例则低于MPP组（P=0.009）。RMPP组的中性粒细胞百分比和降钙素原水平均高于MPP组（P < 0.05）;淋巴细胞百分比明显低于MPP组（P < 0.05）。RMPP组的天门冬氨酸氨基转移酶和乳酸脱氢酶（LDH）水平均高于MPP组（P < 0.05）。Logistic回归分析结果显示,热峰和LDH水平与儿童RMPP密切相关（P < 0.05）。受试者工作特征曲线（ROC）分析结果显示,热峰和LDH诊断儿童RMPP的ROC曲线下面积分别为0.647和0.637。在 ≤ 2岁患儿中,当临界值取LDH为400 U/L时,诊断儿童RMPP的灵敏度为52.63%,特异度为54.84%;在 > 2岁患儿中,当临界值取LDH为335 U/L时,诊断儿童RMPP的灵敏度为69.92%,特异度为51.55%。结论 RMPP患儿早期高热,且伴随多种实验室指标异常;尤其在 > 2岁患儿中,血清LDH水平升高对儿童RMPP具有较高的早期临床诊断价值。     

Anemia and transfusion requirements among Ugandan children with severe malaria treated with intravenous artesunate

Abstract

Parenteral artesunate for the treatment of severe malaria in non-immune travelers is associated with late-onset hemolysis. In children in sub-Saharan Africa, the hematologic effects of malaria and artesunate are less well documented. Here we report a prospective case series of 91 children with severe malaria treated with parenteral artesunate, managed at a resource-poor hospital in Africa, with longitudinal data on hemoglobin (Hb), lactate dehydrogenase (LDH), haptoglobin, and erythrocyte morphology. The median (range) age was 2 (1–8) years and 43 (47%) were female. The median (IQR) admission Hb level was 69 (55–78) g/L and 20 patients (22%) had severe malarial anemia (Hb?<?50?g/L). During hospitalization, 69 patients (76%) received one or more blood transfusions. Fatal outcome in 8 patients was associated with severe anemia in 6/8 cases. Follow-up Hb measurement was performed on 35 patients (38%) at day 14 after initial hospital admission; the remaining patients had no clinical evidence of anemia at the follow-up visit. The convalescent Hb was median (range) 90 (60–138) g/L, which was significantly higher than the paired admission levels (median increase +28?g/L, p?<?.001). Evidence of hemolysis (elevated LDH and low haptoglobin) was common at admission and improved by day 14. No patient met the standardized definition of post-artemisinin delayed hemolysis (PADH). In this cohort of young children with severe malaria treated with artesunate, anemia was common at admission, required one or more transfusions in a majority of patients, and markers of hemolysis had normalized by day 14.     

Evaluation of lactate dehydrogenase, creatine kinase and hepatic enzymes for the retrospective diagnosis of perinatal asphyxia among sick neonates

It is difficult to make a retrospective diagnosis of perinatal asphyxia in symptomatic neonates delivered non-institutionally. We studied serum creatine kinase muscle-brain fraction (CK-MB), lactate dehydrogenase (LDH), glutamic oxaloacetic transaminase (SGOT) and glutamic pyruvate transaminase (SGPT) for differentiating asphyxiated (n=25) from non-asphyxiated (n=20) neonates who present with non-specific signs of sickness. CK-MB was assayed at 8 and 24 h; and LDH, SGOT and SGPT at 72 h of life. On comparing cases and controls, median 8-hr CK-MB [80 U/L vs. 26 U/L respectively, P< 0.001], median 24-hr CK-MB [33.5 U/L vs. 21.5 U/L respectively, P=0.009] and median LDH [965 U/L vs. 168 U/L respectively, P< 0.001] were higher in asphyxiated neonates. Raised LDH had 100% sensitivity, while CK-MB had 100% specificity for asphyxia. LDH had the highest area under ROC curve (0.998). We conclude that LDH at 72 hr of life is most accurate at differentiating asphyxiated from non-asphyxiated symptomatic neonates.     

人参皂苷Rb1激活蛋白酪氨酸激酶2/信号传导及转录激活蛋白3通路减轻川崎病小鼠心肌损伤

目的:探讨人参皂苷Rb1对川崎病小鼠心肌损伤的治疗作用及其信号通路。方法:将5～6周龄BALB/C小鼠随机分为对照组、模型组、阿司匹林组、人参皂苷Rb1低剂量组(50 mg/kg)和高剂量组(100 mg/kg),每组12只。除对照组外,其他各组均间断性腹腔注射10%牛血清白蛋白生理盐水溶液,以诱发川崎病心肌损伤病理模...     

Serum ALT: LDH ratio in typhoid fever and acute viral hepatitis

In 100 consecutive children aged below 18 years with confirmed typhoid fever, 29 had moderate hepatitis. Serum alanine amino transferase: lactate dehydrogenase (ALT: LDH) ratios of these 29 children at the time of hospitalization were compared with that of 29 children with acute viral hepatitis. The serum ALT: LDH ratio levels (expressed in multiples of upper limit of normal) was found to be less than 9 in typhoid hepatitis and more than 9 in acute viral hepatitis. Serum ALT: LDH ratio helps to differentiate typhoid hepatitis from acute viral hepatitis.     

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.