黄芪及其复方治疗小儿中间型β地中海贫血的临床观察及护理体会

目的:探讨黄芪及其复方治疗小儿中间型β地中海贫血的护理方法。方法:将40例中间型β地中海贫血的患儿按1∶1∶1随机分为复方中药组、单味黄芪组及安慰剂对照组。90 d为1个疗程,治疗前及治疗30,60,90 d,分别取患儿的手指末梢血检测血常规﹝观察血红蛋白(Hb)、外周血红细胞总数(RBC)、红细胞平均体积(MCV)、平均血红蛋白含量(MCH)﹞。以碱性血红蛋白电泳,检测胎儿血红蛋白(HbF)比例及含量。结果:复方中药组9例中有效6例,无效3例,总有效率66.74%;单味黄芪组12例中显效1例,有效7例,无效4例,总有效率66.74%;安慰剂对照组9例中有效6例,无效3例,总有效率11.30%,复方中药组和单味黄芪组与安慰剂对照组的总有效率比较,差异有统计意义(P0.05)。患儿的Hb,RBC,MCH血液指标明显提高,各组治疗前后患儿Hb,RBC和MCH差异均有统计意义(P0.05)。结论:黄芪及其复方对小儿中间型β地中海贫血有较好疗效,同时结合耐心细致的护理十分重要,可减少并发症,提高临床疗效。     

Identification of two new synthetic histone deacetylase inhibitors that modulate globin gene expression in erythroid cells from healthy donors and patients with thalassemia

We have identified two new histone deacetylase (HDAC) inhibitors (9 and 24) capable of inducing the expression of gamma-globin and/or beta-globin promoter-driven reporter genes in a synthetic model of Hb switch. Both compounds also increased, with different mechanisms, the gamma/(gamma+beta) ratio expressed in vitro by normal human erythroblasts. Compound 9 increased the levels of gamma-globin mRNA and the gamma/(gamma+beta) ratio (both by 2-fold). Compound 24 increased by 3-fold the level of gamma-globin and decreased by 2-fold that of beta-globin mRNA, increasing the gamma/(gamma+beta) ratio by 6-fold, and raising (by 50%) the cell HbF content. Both compounds raised the acetylation state of histone H4 in primary cells, an indication that their activity was mediated through HDAC inhibition. Compounds 9 and 24 were also tested as gamma/(gamma+beta) mRNA inducers in erythroblasts obtained from patients with beta(0) thalassemia. Progenitor cells from patients with beta(0) thalassemia generated in vitro morphologically normal proerythroblasts that, unlike normal cells, failed to mature in the presence of EPO and expressed low beta-globin levels but 10 times higher-than-normal levels of the alpha hemoglobin-stabilizing protein (AHSP) mRNA. Both compounds ameliorated the impaired in vitro maturation in beta(0) thalassemic erythroblasts, decreasing AHSP expression to normal levels. In the case of two patients (of five analyzed), the improved erythroblast maturation was associated with detectable increases in the gamma/(gamma+beta) mRNA ratio. The low toxicity exerted by compounds 9 and 24 in all of the assays investigated suggests that these new HDAC inhibitors should be considered for personalized therapy of selected patients with beta(0) thalassemia.     

儿童静脉滴注头孢曲松钠出现急性溶血性贫血

1例1．5岁男孩首次静脉滴注头孢曲松钠1．0g治疗上呼吸道感染时出现寒战、发热，而后转入我院。入院时精神萎靡、面色苍黄。血常规示：Hb92g／L，RBC4．21×10^12／L，红细胞平均体积（MCV）68．4fl，红细胞压积（PCV）0．288，网织红细胞（Ret）0．01。入院第2天再次给予头孢曲松钠1．0g加入5％葡萄糖注射液100ml静脉滴注。5h后，患儿全身皮肤黄染，又2h后出现酱油色尿，T39℃。实验室检测：Hb45g／L，RBC1、83×10^12／L，PCV0．12，Ret0．03，直接Coombs试验（＋）。诊断为急性溶血性贫血，立即停药。患者经静脉注射地塞米松、静脉注射丙种球蛋白及输注红细胞后，上述症状缓解，实验室检测示：RBC3．60×10^12／L，Hb91g／L，PCV0、289，Ret0．036。     

Medicinal chemistry of fetal hemoglobin inducers for treatment of beta-thalassemia

In this review we summarize the achievements of medicinal chemistry in the field of pharmacological approaches to the therapy of beta-thalassemia using molecules able to stimulate the production of fetal hemoglobin (HbF). We first describe the molecular basis of the pathology and the biochemical rational of using HbF inducers for therapy; we then outlined the in vitro and in vivo experimental systems suitable for screening of such potential drugs, and finally we describe the different classes of compounds with emphasis on their advantages and disadvantages in the treatment. The results of these reviewed studies indicate that: (a) HbF inducers can be grouped in several classes based on their chemical structure and mechanism of action; (b) clinical trials with some of these inducers demonstrate that they are effective in ameliorating the symptoms of beta-thalassemia; (c) a good correlation was found between HbF stimulation in vivo and in vitro indicating that in vitro testing might be predictive of the in vivo response; (d) combined use of different inducers might maximize the effect, both in vitro and in vivo. However, (e) the response to HbF inducers, evaluated in vitro and in vivo, is variable, and some patients might be refractory to HbF induction by certain inducers; in addition, (f) several considerations call for caution, including the fact that most of the inducers exhibit in vitro cytotoxicity, predicting side effects in vivo following prolonged treatments.     

Effect of epoetin on HO-1 mRNA level and plasma antioxidants in hemodialysis patients

OBJECTIVE: Patients with renal failure and undergoing hemo- (HD) or peritoneal dialysis are under oxidative stress which is thought to contribute to the long-term complications noted in this patient population. One effect of HD-induced oxidative stress is via red blood cell (RBC) membrane lipid peroxidation leading to RBC destruction and anemia. Interaction of this oxidative stress with epoetin (EPO) treatment to increase RBC number and Hb concentration remains unexplored. PATIENTS AND METHODS: This preliminary study used RT-PCR as well as colorimetric based assay approaches to evaluate the effect of EPO-alpha treatment on markers of oxidative stress in hemodialysis patients. Eighteen patients (12 males, 6 females, age range 45 - 68), were treated with EPO-alpha (Eprex) 50 UI/kg thrice weekly over an 8-month study period. Monocytes were isolated at baseline, then monthly thereafter, monocyte heme-oxygenase-1 (HO-1) and plasma Hb and antioxidant power (AOP) were determined. RESULTS AND CONCLUSIONS: Treatment with EPO increased Hb (9.4 +/- 0.7 g/dl to 10.9 +/- 0.5, mean +/- SD p < 0.001). In addition, both monocyte HO-1 mRNA (0.34 +/- 0.08 vs. 0.59 +/- 0.02 d.u. p < 0.001) and plasma AOP (1,379.8 +/- 175 micromol/l to 1,624 +/- 170, p < 0.04) increased. While AOP changes showed no correlation with other indices, increases in HO-1 and Hb were positively correlated using 2 different measures: delta Hb (peak Hb - baseline Hb) vs. delta HO-1 (peak HO-1 mRNA - baseline HO-1 mRNA) as well as delta Hb(5 months-baseline) vs. delta HO-1 (5 months - baseline) mRNA (r = 0.81, p < 0.001 and r = 0.76, p < 0.001; respectively). In conclusion, the increases upon EPO treatment of both HO-1 gene expression and plasma AOP as well as the significant correlation between delta Hb and delta HO-1 mRNA suggest that EPO treatment reduces oxidative stress via a combination of effects. These could potentially include effects on oxidative stress directly as well as effects on the levels and types of antioxidants present in plasma.     

右旋糖酐氢氧化铁在治疗血液透析患者肾性贫血中的应用

目的:评价右旋糖酐氢氧化铁注射液治疗维持性血液透析患者肾性贫血的有效性与安全性.方法:120例维持性血透病人,随机分为静脉组与口服组,分别采用静脉注射右旋糖酐氢氧化铁及口服琥珀酸亚铁进行补铁治疗,总疗程8周.检测治疗前后血清铁及红细胞相关指标并对不良反应进行监测.结果:治疗8周时,静脉组RBC、Hb、Hct、血清铁蛋白(SF)与转铁蛋白饱和度(TSAT)显著升高,幅度明显高于口服组(P＜0.01).静脉组3例有不良反应,1例出现药物相关皮疹,停药后消失,2例轻度胃肠道反应.口服组19例出现明显胃肠道症状.静脉组总不良反应发生率(3/60,5.0%)明显低于口服组(19/60,31.6%),P＜0.01.结论:静脉注射右旋糖酐氢氧化铁可有效纠正维持性血透病人的铁缺乏、提高铁利用率及rHuEPO的治疗效果.且不良反应发生率低、安全性良好.     

离子交换高效液相色谱法血红蛋白分析筛查地中海贫血的研究

目的探讨离子交换高效液相色谱法（HPLc）血红蛋白分析用于地贫筛查的应用价值和基本规律。方法收集50例血常规表型正常、151例小细胞低血素症外周全血,及7例含Hb变异体的α-地贫外周全血或脐带血标本,用VARIANTTMⅡ离子交换HPLCHb分析系统及配套β-地贫短程序试剂进行Hb组分分析,再根据Hb分析及DNA分子诊断结果分组统计和全面评价。结果50例正常标本HbA．2．0％～4．0％、HbF〈2．0％。151例小细胞低血素症标本中,40例HbA,〉4．0％、HbF〈5．0％的标本均确诊为p一地贫基因携带者,筛查符合率100％109例HbA,〈4．0％、HbF〈2．0％的标本中确诊108例为α-地贫;2例HbA．〈4．0％、HbF≥5．0％的标本分别确诊为δβ-地贫和HPFH。HPLC图谱显示能检出但不能定量的Hb变异体为HbH、HbBart’s和HbCS,不能检出HbWS。结论离子交换HPLCHb分析操作简单,结果稳定可靠,人为因素影响小,适合大规模人群筛查及常规检测;并提示实践应用中,须注意特定仪器及配套试剂所采用技术的适应范围,总结经验,丰富及完善其分析数据库及色谱图,避免误诊和漏检。     

重组人促红素联合蔗糖铁用于妇科围手术期红细胞动员的疗效观察

目的观察重组人促红素(rhEPO)联合蔗糖铁用于妇科围手术期红细胞动员的效果。方法选取2012年5-12月我科收治的中度贫血患者156例,随机分为对照组与观察组,每组78例。对照组按照患者血红蛋白(Hb)情况,给予口服铁剂、饮食补充。观察组于手术前一日开始给予rhEPO 20 000 IU皮下注射,同时静脉补充蔗糖铁100 mg,每周2次。手术日开始,给予rhEPO 10 000 IU/支,每日皮下注射,连续7 d。分别于注射后1、3、5、7 d观察两组患者的Hb的变化情况。结果手术后,观察组患者的Hb未受手术影响;对照组患者的Hb与手术前比较,均有不同程度下降;治疗组患者的Hb值明显高于对照组,两组比较,差异有统计学意义(P<0.05)。结论 rhEPO联合蔗糖铁应用于妇科围手术期红细胞动员效果满意,既能缓解目前血液紧张的状态,又可以避免异体输血的隐患。     

琥珀酸亚铁片对妊娠合并缺铁性贫血的疗效及血红蛋白、红细胞、血清铁蛋白的影响

目的观察琥珀酸亚铁片对妊娠合并缺铁性贫血(IDA)的治疗效果及其对血红蛋白(Hb)、红细胞(RBC)、血清铁蛋白(SF)的影响。方法选择我院2011年1月至2012年12月的妊娠合并缺铁性贫血患者120例(均符合入选标准),其中轻度贫血107例,中度贫血13例,重度贫血0例,给患者口服琥珀酸亚铁片,1²片/次,3次/d。分别测定治疗前后孕妇的Hb、RBC和SF,观察并记录实验过程中出现的不良反应,评价药物的安全性。结果 120例妊娠合并缺铁性贫血患者39例痊愈,57例显效,22例有效,2例无效,总有效率为98.3%。治疗后Hb、RBC和SF与治疗前比较差异有统计学意义(P<0.01)。治疗过程中部分患者出现轻微的不良反应,均未影响治疗效果。结论琥珀酸亚铁片对妊娠合并缺铁性贫血有显著的治疗效果,能明显提高妊娠合并缺铁性贫血患者的Hb、RBC和SF。     

β地中海贫血基因携带者及G6PD缺乏的产前筛查与血液学指标评价

目的调查本地区围生期妇女β地中海贫血(地贫)基因携带率和G6PD缺乏的发病率;以及评价血液学指标在β地贫及G6PD酶缺乏筛查中的意义.方法对产前检查的孕妇,进行了β地贫基因诊断及G6PD定量分析,血常规红细胞参数检测及血红蛋白电泳HbA2定量.对已明确诊断的85例β地贫组,76例IDA组及75例G6PD酶缺乏组,进行红细胞参数(RBC、Hb、HCT、MCV、MCH、MCHC、RDW、RET)结果的比较;并将各方法检测结果与地中海贫血基因诊断结果比较,分析各筛查方法的敏感性和特异性.结果2 450例孕妇共检出β地贫阳性85例,β地贫基因携带率为3.5%;共检出G6PD缺乏75例,G6PD缺乏的发病率为3.1%.地贫组、IDA组及G6PD缺乏组RET均增高,与正常对照组比较,差异均有显著性(P＜0.01);G6PD缺乏组RET显著增高,与地贫组及IDA组比较,差异均有显著性(P＜0.01).在地贫组和IDA组中,MCV均减低,RDW均增高,与正常对照组比较,均有显著性差异(P＜0.01).地贫组与IDA组比较,地贫组MCV减低较IDA组明显,两者之间差异有显著性(P＜0.01).对照基因检测结果,MCV诊断地贫基因携带者的敏感性为97.6%,特异性为54.5%.HbA2定量对β地贫基因携带者的敏感性为96.8%,特异性为97.4%.结论CD41-42(-CTTT)是本地区孕妇最常见的β地贫基因突变类型,MCV测定可作为产前地贫携带者筛查的敏感而有价值指标,而HbA2定量是β地贫基因携带者筛查可靠的血液学指标.     

维生素A联合重组人促红细胞生成素预防早产儿贫血的疗效

目的在给予重组人促红细胞生成素的基础上,观察口服维生素A预防早产儿贫血的效果。方法选取120例早产儿,分成3组,对照组给予常规治疗,观察A组给予重组人促红细胞生成素(rHu-EPO)及维生素D滴剂,观察B组给予重组人促红细胞生成素及维生素AD滴剂。3组患儿均在出生后第14天开始给予维生素E、赖氨肌醇维生素B12及依地酸铁钠口服溶液,疗程共4周;对比3组早产儿治疗前后体质量、血红蛋白(Hb)、红细胞比容(Hct)、血清铁蛋白(SF)及网织红细胞(Ret)的变化情况,统计其输血例数。结果治疗后,患儿Hb、Hct、Ret水平及输血例数各组间均有显著差异(P<0.05);其SF水平观察B组高于观察A组及对照组(P<0.05),观察A组与对照组无显著差异;观察组W水平显著高于对照组(P<0.05),但观察A组及B组间差异不明显。结论在给予重组人促红细胞生成素的基础上加用维生素A能有效地预防早产儿贫血。     

Interaction of mercury and water deprivation on the hematology of chickens

Four-week-old male chickens were subjected to one of the following treatments: 0 ppm mercury (Hg) plus water ad libitum, 0 ppm Hg plus limited water (LW), 500 ppm Hg plus water ad libitum, and 500 ppm Hg plus LW. Exposure was for each of the following time intervals: 0-3, 0-6, 0-9, 0-12, and 0-15 d. LW significantly increased red blood cell (RBC) numbers, hematocrit (HCT), mean corpuscular volume (MCV), and hemoglobin (Hb) level and significantly decreased mean corpuscular hemoglobin (MCH) level within 3 d of treatment. Mercury produced variable results, with no significant trends developing during the treatment period. The effects caused by Hg appeared to be masked by the effects of dehydration. The birds receiving water ad libitum had numerically greater RBC numbers and HCT and Hb levels than birds receiving water ad libitum that was contaminated with Hg, while the effects caused by Hg in the groups that received limited water were not consistent. Each group was given a recovery period of 14 d, during which time all birds received uncontaminated water and feed ad libitum. The hematologic effects caused by Hg and water deprivation did not return to a normal condition. Reduced RBC numbers, HCT, and Hb levels were observed at the end of the recovery period in the birds that were exposed to Hg and/or water deprivation.     

老年慢性心力衰竭患者贫血相关指标分析

目的分析老年慢性心力衰竭患者贫血相关指标变化情况。方法对比分析48例老年慢性心力衰竭患者（观察组）和48例健康人（对照组）的外周血红细胞计数（RBC）、血红蛋白（Hb）、红细胞比容（Hct）等贫血相关指标。结果观察组RBC、Hb、Hct分别为（3．37±0．49）×10^12／L、（103．64±14．33）g／L、（0．31±0．08）,对照组分别为（4．64±0．51）×10^12／L、（121．33±17．69）g／L、（0．37±0．07）,两组差异均有统计学意义（t=4．46、3．06、4．18,P=0．02、0．04、0．04）;治疗后,观察组心力衰竭症状缓解,RBC为（3．76±0．58）×10^12／L,与对照组差异有统计学意义（t=0．48,P=0．03）;观察组Hb、Hct分别为（123．77±17．26）g／L、（0．37土0．05）,与对照组差异均无统计学意义（P=0．06、0．11）。结论老年心力衰竭患者贫血相关指标均低于健康人,心力衰竭与贫血之间存在明显的相关性。     

The effect of methanolic extract of Buchanania lanzan Spreng seeds on hematological indices

Objective:

The objective of the current study was carried out to investigate the effects of methanolic extract of Buchanania lanzan Spreng seeds on hematological indices.

Materials and Methods:

Eighteen male albino Wistar rats were divided into three groups, six in each. Group I animals received distilled water, Group II and III were treated with an oral dose of 1000 mg oil/kg and 2000 mg oil/kg of extract, respectively, for 7 days. At the end of the study, blood was collected and evaluated for packed cell volume (PCV), hemoglobin (Hb) concentration, and red blood cell (RBC) and white blood cell (WBC) counts.

Results:

There was a significant dose-dependent increase in the hematological indices such as PCV, Hb, RBC, and WBC count in the treatment group.

Conclusions:

The improvement of PCV, Hb, and RBC values is an indication of the anti-anemic effect which may be due to the stimulation of RBC production in bone marrow. Further, stimulated production of WBC could be as a result of possible stimulus of the immune system. Hence, this study confirms that the extract of B. lanzan could be useful for the treatment of anemia.KEY WORDS: Buchanania lanzan, hemoglobin concentration, packed cell volume, red blood cell, white blood cell     

依普定治疗肾性贫血45例疗效观察

目的 :观察依普定治疗肾性贫血的疗效。方法 :小剂量依普定皮下注射治疗45例肾性贫血患者 ,采用自身对照的方法观察患者治疗前、后红细胞、血红蛋白、红细胞压积、网织红细胞等的变化情况。结果 :经依普定治疗后 ,患者红细胞、血红蛋白、红细胞压积、网织红细胞计数均较治疗前明显升高且副作用较少。结论 :小剂量依普定皮下注射治疗肾性贫血有较好疗效     

左卡尼汀对促红细胞生成素治疗血液透析患者肾性贫血疗效的影响

目的 探讨左卡尼汀对促红细胞生成素治疗血液透析患者肾性贫血疗效的影响.方法 将40例进行血液透析治疗（HD）的慢性肾衰尿毒症患者,随机分为治疗组和对照组,每组20例.对照组给予促红细胞生成素9 000 u/周,于血液透析后皮下注射,同时常规口服铁剂、叶酸.治疗组在对照组基础上予左卡尼汀1.0g加入20 ml 0.9％氯化钠注射液静脉缓慢注射3～5 min,每周2次.两组均治疗3个月.治疗前和治疗4周、3个月后检测血红蛋白（Hb）、血细胞比容（Hct）、血清铁蛋白（SF）、血清全段甲状旁腺素（iPHT）并比较.结果 治疗组治疗4周后,Hb、Hct水平较治疗前上升（均P＜0.05）.3个月后两组Hb较治疗前均显著升高（均P＜0.05）,而且治疗组Hb的升高明显优于对照组（P＜0.01）.结论 左卡尼汀能提高促红细胞生成素治疗血液透析患者肾性贫血的疗效.     

NaFe-EDTA强化酱油对学龄前儿童贫血干预效果评价

目的评价NaFe EDTA营养强化酱油对学龄前儿童贫血改善效果。方法按随机对照双盲法选择 36 4名 3～ 6岁的农村学龄前儿童 ,分干预组 (2 0 8人 )和对照组 (15 6人 )分别食用NaFe EDTA营养强化酱油和空白对照酱油连续追踪观测 6个月 ,检测血红蛋白变化水平和贫血发生率。结果服用营养强化酱油 6个月后 ,干预组男、女学龄前儿童的血红蛋白浓度升高明显 ,与食用前血红蛋水平相比差异有显著性 (P <0 .0 0 1) ;血红蛋白净增值与对照组相比差异有显著性 (P <0 .0 1) ;干预组男、女学龄前儿童的贫血发生率明显降低 ,与食用前的贫血发生率相比差异有显著性 (P <0 .0 5 ,P<0 .0 1)。结论NaFe EDTA强化酱油可明显改善和降低儿童贫血发生率 ,是一种有效预防控制学龄前儿童贫血的营养干预措施。     

重组人类促红细胞生成素防治极低出生体重儿贫血的临床观察

目的：探讨重组人类促红细胞生成素（rHuEPO）防治极低出生体重儿贫血的疗效。方法：将42例极低出生体重儿按入院次序分成治疗组21例，对照组21例，治疗组于出生第8天，即予rHuEPO600IU／（kg.w），隔日1次，每周3次，皮下注射，共5周；对照组未予rHuEPO治疗。结果：治疗组较对照组出现贫血率低（19．04％vs61.90%），两组比较有显著性差异（P＜0．01）；两组极低出生体重儿生后血红蛋白、红细胞数、红细胞压积均逐步下降，但治疗组较对照组上述指标下降程度明显减轻，差异有显著性（P＜0．01或0．05）。结论：早期大剂量rHuEPO能够低极低出生体重儿贫血发生率，并能减轻其贫血的程度。