Increased Fetal Iron Load in Rhesus Hemolytic Disease

There is little information about the iron overload caused by hemolysis in fetuses affected with rhesus hemolytic disease (RHD). The authors therefore studied the iron load in RHD by measuring cord blood ferritin levels in babies affected with RHD and gestational age- and weight-matched controls. Cord blood ferritin levels were higher in babies with RHD. Intrauterine transfusions did not affect the ferritin status of the babies with RHD and there was no correlation between hemoglobin and ferritin levels. The results indicate that there is an increased intrauterine iron load in babies with RHD, independent from intrauterine transfusions and rate of hemolysis.     

红细胞免疫抗体引起的新生儿溶血病4例报告

目的探讨红细胞免疫抗体引起的新生儿溶血病病例的母婴实验室检测结果、临床表现及治疗。方法回顾性分析4例红细胞免疫抗体引起的新生儿溶血病患儿及母亲产前相关实验室及临床资料。结果 4例患儿的母亲在孕期分别检测出IgG性质的红细胞抗E、抗D、抗Jkb及自身抗体;效价分别为16、2 048、1及16。患儿均足月分娩,但生后6 h~3 d出现不同程度的皮肤黄染,伴或不伴贫血。患儿红细胞放散试验发现存在与母体相同的抗体,效价分别为4、512、0及2。患儿均予光照治疗,2例重症患儿予换血及输血治疗,均预后良好。结论孕妇产前免疫血液学检查有助于及早发现红细胞不规则抗体以评估胎儿及新生儿溶血病风险。     

D-penicillamine therapy in AB0 hemolytic disease of the newborn infant

This study comprises 120 full-term infants with AB0 hemolytic disease admitted to the neonatal department during a period of 60 months from 1970 to 1975. During the first 30 months newborns (n=61) received no D-penicillamine therapy, whereas all infants (n=59) received this treatment (300–400 mg/kg/day, divided into 4 equal doses, for 2–5 days) during the last 30 months. The patients were further subdivided into two groups according to the point of time when D-penicillamine treatment was begun, viz. group I (34 treated and 34 control infants) within the first 24 h of life; group II (25 treated and 27 control infants) after the third day of life. In group I D-penicillamine therapy caused a marked decline of serum bilirubin concentrations at a time when such levels were rising in the control infants. The number of exchange transfusions per infant was 1.32 in the control and 0.11 in the D-penicillamine-treated infants.In group II D-penicillamine considerably reduced the number of exchange transfusions (0.700.24=controltreated) but the difference was statistically not significant. In the latter patients the mean bilirubin values showed a smaller difference compared to the controls than in group I.Since group I represented the results of early or preventive treatment, while group II those of late or therapeutic treatment, it is obvious that, for ensuring success, D-penicillamine treatment should be begun as early as possible in AB0 hemolytic disease of the newborn.     

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目的探讨不同比例成分血对新生儿母子ABO血型不合溶血病(HDN)换血治疗后内环境的影响。方法对2009年1月至2010年4月湖南省儿童医院40例HDN达到换血指征的患儿,采用不同比例成分血行换血术,血源均采用O型浓缩红细胞及AB型血浆。治疗组16例,换血时采用O型浓缩红细胞及AB型血浆的比例为3:1(即O型浓缩红细胞300mL:AB型血浆100mL);对照组24例,采用的比例为2:1。两组换血量均按150～180mL/kg双倍量换血,换血结束后对照组常规输注O型浓缩红细胞15～20mL/kg(总量约60mL),治疗组则不再输血,换血前后其他治疗相同。监测换血前后血清总胆红素、胆红素置换率、血红蛋白、红细胞压积、血清电解质、血气等指标变化。结果两组换血后血清总胆红素均较换血前明显下降,但治疗组下降较对照组更明显,差异有统计学意义(P<0.05)。两组换血前后血红蛋白、红细胞压积比较显示,治疗组换血前后比较差异无统计学意义(P>0.05),对照组比较则差异有统计学意义(P<0.05)。换血后两组血气指标pH、BE值及电解质如K+、Na+、Ca2+、Cl-较换血前差异无统计学意义(P>0.05)。结论采用O型浓缩红...     

ABO溶血病患儿应用丙种球蛋白的胆红素阈值探讨

目的 探讨选择应用丙种球蛋白治疗新生儿ABO溶血病的适宜胆红素阈值.方法 将我院新生儿科2011年7月至2012年9月收治的新生儿ABO溶血病患儿随机分为两组,试验1组患儿胆红素值达到Bhutani曲线第75百分位、试验2组达到第95百分位时,应用1次丙种球蛋白1 g/kg.比较两组患儿总光疗时间、治疗2天后胆红素下降幅度、住院时间、住院费用、血红蛋白值、应用丙种球蛋白的比例等资料.结果 试验1组(66例)和试验2组(63例)患儿总光疗时间、胆红素下降幅度、住院时间、住院费用、生后42天血红蛋白值差异均无统计学意义(P＞0.05),试验2组应用丙种球蛋白比例(34/63)小于试验1组(53/66),差异有统计学意义(P＜0.01),两组均没有需要换血或发生胆红素脑病的病例.结论 以Bhutani曲线第95百分位水平胆红素值作为应用丙种球蛋白治疗新生儿ABO溶血病的胆红素阈值更为合理.     

Severe late anemia of hemolytic disease of the newborn

Late anemia is a well-recognized complication of Rhesus hemolytic disease of the newborn (HDN). The incidence of Rhesus HDN is declining, with a tendency for more severely affected pregnancies to be managed in specialist centres. Consequently, many paediatric departments may see relatively few affected infants with comparatively mild disease, and the risk of late anemia in such cases may not always be appreciated. Two cases of infants born with evidence of Rhesus isoimmunization noted at birth and encountering no immediate problems other than mild hyperbilirubinemia are described. After an uneventful early neonatal course, both infants were discharged without follow-up and presented in the second to third weeks of life with severe, life-threatening anemia, leading to neurological sequelae in one case. The importance of close surveillance, including hemoglobin measurements, in all infants with Rhesus hemolytic disease, irrespective of initial severity, is reiterated.     

大剂量静脉滴注丙种球蛋白治疗新生儿ABO溶血病的疗效观察

目的　观察大剂量静脉滴注丙种球蛋白(IVIG)治疗新生儿ABO溶血病的临床效果。方法　对符合新生儿ABO溶血病诊断标准且无其它合并症的63例患儿,分为常规治疗组和大剂量IVIG治疗组。IVIG治疗组在常规治疗的基础上给予IVIG80 0mg/kg·d静脉滴注,每日一次,连续3日。结果　IVIG治疗组在治疗前血清胆红素水平比较高的情况下,黄疸消退时间为4 1 8±1 0 3天,常规治疗组为5 .2 8±1 .5 0天,t=3.72 4 ,P <0.0 1。结论　大剂量静脉滴注丙种球蛋白协同治疗新生儿ABO溶血病临床效满意     

Use of recombinant erythropoietin for the management of severe hemolytic disease of the newborn of a K0 phenotype mother

Very few people do not express any Kell antigens on their red blood cells (K0 phenotype). They can be immunized by transfusion or pregnancy and develop antibodies against Kell system antigens. These maternal antibodies can cause severe hemolytic disease of the fetus/newborn, as a result of the suppression of erythropoiesis and hemolysis. Multiple intrauterine transfusions in the management of severe hemolytic disease have been shown to cause erythropoietic suppression as well. Recombinant erythropoietin has been successfully used in the management of late anemia of infants with Rh hemolytic disease and in 1 case of KEL1 (Kell)-associated hemolytic disease. The authors present the case of severe hemolytic disease of a newborn due to KEL5 (Ku) isoimmunization of his K0 phenotype mother. Regular intrauterine transfusions were performed to manage the severe fetal anemia (Hb 3 g/dL). A male infant was born at the 36th week of gestation having normal hemoglobin (15.8 g/dL) and developed only mild hyperbilirubinemia. On the 15th day of life, the infant's hematocrit had fallen to 27.3%, with low reticulocyte count and low erythropoietin level. The infant was managed successfully with recombinant erythropoietin.     

Intravenous immunoglobulins in rhesus hemolytic disease

Objective: To evaluate the role of intravenous immunoglobulins in Rh hemolytic disease of newborn.Methods: The study included all DCT positive Rh isoimmunized babies admitted in the unit from August 2000 to February 2001. Intravenous immunoglobulins in the dose of 500 mg/kg on day 1 and day 2 of life in addition to the standard therapy. Babies who received IVIG were compared with those who did not receive IVIG for the peak bilirubin levels, duration of phototherapy, number of exchange transfusions, discharge PCV and the need for blood transfusions for late anemia till 1 months of age.Results: A total of 34 babies were eligible for the study. 8 babies received IVIG and 26 babies only standard treatment. The mean maximum bilirubin levels were significantly lower in the IVIG group compared to the group who received NO IVIG (16.52 ± 2.96 Vs 22.72 ± 8.84, p=0.004). Five babies in the IVIG group (62.5%) and 23 babies in the NO IVIG group required exchange transfusions (88.5%, p=0.014). 12 of the 26 babies in the NO IVIG group required multiple exchange transfusions while none of the babies in IVIG group required more one exchange transfusion (p=0.03). The mean duration of phototherapy was 165 ± 109 hours in the IVIG group as against 119 ± 56 hours in the NO IVIG group (p=0.29). Blood transfusion for anemia was more common in the IVIG group (37.5 % Vs 11.5% p=0.126) though the packed cell volumes at discharge were similar in both the groups (39.5 ±11 Vs 40 ± 5.1, P=0.92).Conclusion; Intravenous immunoglobulins is effective in decreasing the maximum bilirubin levels and the need for repeated exchange transfusions in Rh hemolytic disease of newborn. There is however an increased need for blood transfusions for late anemia in the babies treated with IVIG.     

Intravenous immune globulin in neonatal immune hemolytic disease: does it reduce hemolysis?

We studied the effect of intravenous immune globulin (IVIG) on hemolysis in term, hyperbilirubinemia, Coombs'positive infants utilizing measurement of carboxyhemoglobin fraction corrected for inhaled carbon monoxide (COHbc), a sensitive indicator of hemolysis. COHbc values were determined before and after IVIG infusion. In those babies who responded with a decrease in serum total bilirubin ( n = 19). no exchange transfusions were required and COHbc levels decreased significantly by 24 h post-IVIG from 1.37 ± 0.31 to 1.12 ± 0.26% tHb ( p < 0.0001). There were no corresponding decreases in COHbc levels (1.89 ± 0.54 to 1.82 ± 0.48% tHb; ( p > 0.05) among those whose serum bilirubin levels did not decrease in response to IVIG ( n = 7), and all of these infants required exchange transfusions. Furthermore, the extent of the decrease in COHbc was related to the degree of decrease in serum bilirubin levels, such that the percentage decrease of bilirubin at 24 h was directly correlated with the percentage decrease of COHbc at 24 h ( p = 0.007). We conclude that IVIG, when successful, inhibits hemolysis in these infants.     

Molecular characterization and multidisciplinary management of Gerbich hemolytic disease of the newborn

Gerbich (Ge) antigens are high frequency red cell antigens expressed on glycophorin C (GYPC) and glycophorin D. Hemolytic disease of the fetus and newborn (HDFN) due to Gerbich antibody is rare and presents a clinical challenge, as Gerbich negative blood is scarce. We report a case of HDFN due to maternal Ge3 negative phenotype and anti‐Ge3 alloimmunization, successfully managed by transfusion of maternal blood. Molecular testing revealed that the mother has homozygous deletion of exon 3 of GYPC, the father is homozygous wildtype for GYPC, and the infant is obligate heterozygote expressing Ge3.     

碳氧血红蛋白对新生儿溶血病的诊断价值

目的探讨碳氧血红蛋白（COHb）辅助诊断新生儿溶血病的价值。方法 选择2009年10月至2010年9月本科收治的足月儿,确诊新生儿溶血病者为溶血组,存在母子血型不合和病理性黄疸但未确诊溶血病者为非溶血性黄疸组,无母子血型不合及病理性黄疸者为对照组。分别于生后5天内测定COHb、血红蛋白（Hb）、网织红细胞（Ret）和总胆红素（STB）,并进行比较。建立受试者工作曲线（ROC曲线）,确定COHb、Hb、Ret的曲线下面积（AUC）,同时分析三者不同截断值的敏感度、特异度、阳性和阴性预测值。结果 （1）溶血组COHb、Ret和STB较非溶血性黄疸组及对照组增高,Hb降低（P均〈0.001）;非溶血性黄疸组STB较对照组增高（P〈0.001）,但COHb、Hb和Ret差异无统计学意义（P〉0.05）。（2）COHb、Hb和Ret的AUC分别为96.0%、74.9%、85.7%,三者比较差异有统计学意义（P〈0.05）。COHb预计溶血病的最佳截断值为1.2%（敏感度86.8%、特异度91.2%、阳性预测值93.0%、阴性预测值83.9%）,敏感度、特异度均优于Hb和Ret。三指标联合诊断的特异度可提高到98.2%。结论 COHb是敏感的新生儿溶血病诊断指标,与Hb和Ret联合检测,对于早期筛查和辅助诊断新生儿溶血病有重要意义。     

蓝光治疗对新生儿溶血病脑干听觉诱发电位的影响

目的　 探讨蓝光治疗在降低新生儿溶血病患儿的血清胆红素浓度时能否逆转脑干听觉诱发电位结果的改变。 方法 　对 2 0例新生儿ABO血型不和溶血病引起的高胆红素血症患儿光疗前后进行脑干听觉诱发电位检测 ,并与 2 2例正常新生儿作对照。 结果 　光疗后随着胆红素浓度的下降 ,新生儿溶血病患儿脑干听觉诱发电位值V波潜伏期 ,Ⅲ Ⅴ ,Ⅰ Ⅴ波间期较光疗前明显缩短 (P <0 0 1)。 结论 　光疗不仅能降低血清胆红素浓度 ,而且能逆转胆红素对脑干听觉诱发电位的影响 ,即光疗能逆转胆红素脑病发生     

流式法检测红细胞膜抗体含量在新生儿ABO溶血病的诊断价值

目的用流式细胞仪检测新生儿高胆红素血症患儿红细胞膜的抗体含量并进行计数,以探讨红细胞膜抗体计数对新生儿ABO溶血病的早期诊断价值。方法收集患儿静脉血标本70例,其中51例为临床确诊的新生儿溶血病,19例为临床疑似病例,采用流式细胞仪计数患儿红细胞膜被同种血型抗体致敏后的抗体含量水平。结果51例临床确诊的新生儿ABO溶血病标本均可检测到红细胞膜被同种血型抗体致敏后的相关抗体,但红细胞膜抗体含量及致敏比例有明显差异;19例临床疑似病例中,9例检测到红细胞膜相关抗体,并与临床最后诊断相符,10例结果阴性,其中6例临床排除ABO溶血所致的高胆红素血症,另4例病因不明。结论流式细胞仪可以直接检测到患儿红细胞被同种血型抗体致敏后的抗体含量,特别是对红细胞膜上结合抗体数量少,卡式法检测阴性、临床又高度怀疑的溶血病患儿有重要的临床诊断价值。     

不同剂量丙种球蛋白治疗ABO溶血病疗效比较

目的比较不同剂量静脉注射用丙种球蛋白(IVIG)治疗新生儿ABO血型不合溶血病的疗效。方法将出生后2 d内确诊的新生儿ABO血型不合溶血病患儿随机分为单剂组(70例)和多剂组(66例),单剂组静脉滴注IVIG 1 g/(kg.d),1 d;多剂组剂量500 mg/(kg.d),共3 d。生后第42天随访血红蛋白及生长发育等情况。结果单剂组需要双面光疗时间较多剂组短(P<0.01),两组患儿第42天血红蛋白水平、贫血发生率差异无显著性,两组患儿均不需换血治疗,均未发生胆红素脑病。结论单次大剂量IVIG(1 g/kg)治疗新生儿ABO血型不合溶血病是一种高效、经济、安全的治疗方法。     

不同血源对ABO溶血病外周动静脉同步换血疗效的比较

目的 　探讨不同血源对ABO溶血病外周动静脉同步换血的疗效。方法　对 2 3例ABO溶血病患儿依时间分为二组 ,同型血组和混合血组。同型血组采用与患儿血型相同的血为血源换血 ,混合血组采用AB型血浆和O型洗涤红细胞混合血为血源换血。换血途径均采用外周动静脉。结果　同型血组和混合血组换血后总胆红素下降显著 ,4 50 0± 1 4 4 2 4 μmol/L ,2 54 56± 87 58μmol/L ,393 4 8± 1 6 7 4 6vs2 36 6 0± 97 79μmol/L ;两组的换出率分别是 :4 2 2 5± 1 3 91 % ,38 4 6± 1 3 1 6 % ,t =0 70 ,P >0 0 5。换血后两组的血细胞 ,红细胞压积 (HCT) ,电解质 ,白蛋白改变相同。换血后无明显不良反应。结论　同型血和混合血为血源换血疗效相同 ,在紧急情况下采用同型血换血可以节约时间。     

A case of rhesus hemolytic disease with hemophagocytosis and severe iron overload due to multiple transfusions

BACKGROUND: A newborn with cholestatic hepatic disease and hemophagocytic lymphohistiocytosis due to rhesus hemolytic disease (RHD) is reported. OBSERVATION: A 34 weeks' gestation baby with RHD, who had received multiple intrauterine transfusions (IUT), developed cholestatic hepatic disease and secondary hemophagocytic lymphohistiocytosis (HLH). Her serum ferritin level increased to 5,527 ng/mL, and liver biopsy showed severe iron overload. Treatment with intravenous desferrioxamine resulted in a marked decrease in serum ferritin levels and normalization of liver function CONCLUSION: We suggest that patients who have undergone IUT be evaluated for hyperferritinemia. If hyperferritinemia is noted, chelation therapy should be considered. As another rare finding, HLH can complicate the course of RHD.