Unusual cause of severe neonatal hyperbilirubinaemia: a twin case.

We present twins born to the 31-year-old, multigravida mother, who were referred to our centre at 90 hours of life for severe hyperbilirubinaemia. Twin 1 had already received two double volume exchange transfusions at 55 and 83 hours of life, in view of the persistent rise in bilirubin despite receiving phototherapy. Twin 2 had received phototherapy and 1 packed red blood cell transfusion in view of the fall in haematocrit. Mother's blood group was B positive and that of both twins was O positive. Both the twins were started on intensive phototherapy and their serum bilirubin and haematocrit were evaluated. On investigation, a minor blood incompatibility was found. Double volume exchange transfusion was done for twin 2 at 100 hours of life in view of the rapid rise in serum bilirubin. Both the babies were monitored for their serum bilirubin and treated for sepsis and discharged after 15 days.

Severe ABO Hemolytic Disease of the Newborn Requiring Exchange Transfusion.

ABO incompatibility (ABOi), the most common cause of hemolytic disease of the newborn (HDN), is nearly always mild and treatable with phototherapy. Reports of ABOi HDN requiring neonatal exchange transfusion are extremely rare since the inception of modern guidelines. Here, a case of ABOi HDN clearly met criteria for exchange transfusion. An O-positive African American mother delivered a B-positive neonate that quickly developed hyperbilirubinemia. The neonatal DAT was positive from anti-B and anti-A,B, and maternal IgG titer was 1024. Double volume exchange transfusion resulted in a favorable outcome. Given early discharge of newborns, further understanding of factors predicting severe disease is needed.

CD144+ endothelial microparticles as a marker of endothelial injury in neonatal ABO blood group incompatibility.

BACKGROUND: ABO antigens are expressed on the surfaces of red blood cells and the vascular endothelium. We studied circulating endothelial microparticles (EMP) in ABO haemolytic disease of the newborn (ABO HDN) as a marker of endothelial activation to test a hypothesis of possible endothelial injury in neonates with ABO HDN, and its relation with the occurrence and severity of haemolysis. MATERIAL AND METHODS: Forty-five neonates with ABO HDN were compared with 20 neonates with Rhesus incompatibility (Rh HDN; haemolytic controls) and 20 healthy neonates with matched mother and infant blood groups (healthy controls). Laboratory investigations were done for markers of haemolysis and von Willebrand factor antigen (vWF Ag). EMP (CD144(+)) levels were measured before and after therapy (exchange transfusion and/or phototherapy). RESULTS: vWF Ag and pre-therapy EMP levels were higher in infants with ABO HDN or Rh HDN than in healthy controls, and were significantly higher in babies with ABO HDN than in those with Rh HDN (p<0.05). In ABO HDN, pre-therapy EMP levels were higher in patients with severe hyperbilirubinaemia than in those with mild and moderate disease or those with Rh HDN (p<0.001). Post-therapy EMP levels were lower than pre-therapy levels in both the ABO HDN and Rh HDN groups; however, the decline in EMP levels was particularly evident after exchange transfusion in ABO neonates with severe hyperbilirubinaemia (p<0.001). Multiple regression analysis revealed that the concentrations of haemoglobin, lactate dehydrogenase and indirect bilirubin were independently correlated with pre-therapy EMP levels in ABO HDN. DISCUSSION: Elevated EMP levels in ABO HDN may reflect an IgG-mediated endothelial injury parallel to the IgG-mediated erythrocyte destruction and could serve as a surrogate marker of vascular dysfunction and disease severity in neonates with this condition.

Fetal intraperitoneal injection of immunoglobulin diminishes alloimmune hemolysis.

We report a case of severe fetal anemia associated with maternal anti-M antibody that was treated by direct injection of pooled human immunoglobulin into the fetal abdominal cavity. Four treatments at a dosage of 2 g per-kg estimated fetal body weight were performed, and no side effects were observed. A healthy baby girl was delivered transvaginally at 38 weeks, with neither exchange transfusion nor phototherapy required. Follow-up over 12 months found no indications of anemia or developmental delay in the child. This is believed to be the first report of fetal anemia in a blood-type-incompatible pregnancy being treated successfully with only direct immunoglobulin injection into the fetus. The immunoglobulin may have functioned as a neutralizing antibody causing the anemia to improve.

[Meta analysis of the effect of immunoglobulin infusion on neonatal isoimmune hemolytic disease caused by blood group incompatibility].

OBJECTIVE: Neonatal isoimmune hemolytic disease is still one of the major causes of neonatal hyperbilirubinemia. The infants with severe hemolysis even need phototherapy and exchange transfusion. Early intravenous immunoglobulin infusion may block hemolysis to some extent. This study aimed to investigate the efficacy and safety of immunoglobulin infusion on neonatal isoimmune hemolytic disease by meta analysis. METHOD: All randomized controlled trials on the effect of immunoglobulin infusion on neonatal Rh and ABO incompatible hemolytic disease obtained by searching MEDLINE, Cochrane Library, EMBASE, CNKI and CBM were included. Meta analysis was done by Review Manager 4.2 software. RESULTS: Six trials with totally 456 neonates were included. There were 109 infants with Rh blood group incompatible hemolysis in 4 studies and 347 infants with ABO blood group incompatible hemolysis in 4 studies. There was no significant difference in gestational age, weight and sex between the immunoglobulin infusion and control groups. Compared with those neonates treated with only phototherapy, the infants treated with immunoglobulin and phototherapy had shorter duration of phototherapy (weighted mean difference, WMD -15.42, 95%CI -29.00 to -1.85), less chance to be given exchange transfusion (RR 0.25, 95%CI 0.17 to 0.39) and shorter duration of hospitalization (WMD -25.44, 95%CI -36.93 to -13.94). While intravenous immunoglobulin could not decrease the maximum serum bilirubin level (WMD -29.91, 95%CI -78.24 to 18.42). There was no significant difference in the incidence of late anemia between the two groups. No adverse reaction was found in neonates who received immunoglobulin. CONCLUSIONS: The results of this meta analysis support that the intravenous immunoglobulin had some therapeutic effect on neonatal isoimmune hemolytic disease. The infants who received immunoglobulin had shorter duration of phototherapy and less chance to be given exchange transfusion. Well designed, double blind and randomized controlled trials with large sample size and long-term follow-up are needed for further evaluation of the efficacy and safety of the immunoglobulin therapy.

Management of anti-Jka alloimmunization.

OBJECTIVE: To determine whether prenatal management using guidelines established for anti-D is applicable to anti-Jka. STUDY DESIGN: A computerized database containing the records of all alloimmunized pregnancies at The Ohio State University Medical Center with due dates from 1959 to 2008 was used to identify pregnancies affected only by anti-Jka. Only cases with evidence that the newborn was Jka antigen positive were included. RESULTS: Twenty affected pregnancies met inclusion criteria. Of those, 16 pregnancies required monitoring with serum titers only and 4 were followed with more diagnostic tests as recommended during that time period. One pregnancy with the highest titer of 32 and elevated middle cerebral artery peak systolic velocity (MCA PSV) required 4 intrauterine transfusions for fetal anemia. Another pregnancy with a titer of 32 had an infant who required phototherapy for hemolytic disease of the fetus/newborn (HDFN), with a hemoglobin value of 15.9 g/dL. None of the other 18 infants required any therapy for HDFN. CONCLUSION: Our case series identified severe disease in 1 of 20 pregnancies from anti-Jka using maternal antibody titer and MCA PSV. Criteria used for monitoring RhD alloimmunization were effective in detecting severe HDFN resulting from to anti-Jka.

Intravenous immune globulin reduces the need for exchange transfusions in Rhesus and AB0 incompatibility.

AIM: To conduct a quality control review of a single institution experience with intravenous immune globulin in the treatment of Rhesus and AB0 incompatibility. METHODS: Intravenous immune globulin as treatment for Rhesus and AB0 incompatibility was introduced in our hospital in 1998. We performed a chart review of 176 infants with Rhesus or AB0 incompatibility treated in our hospital between 1993 and 2003, divided into a historical control group (1993-1998) and a treatment group (1999-2003). The project was approved through institutional ethics procedures. RESULTS: The use of exchange transfusion as a therapeutic modality was significantly reduced in the cohort treated with intravenous immune globulin (OR 0.11; 95% CI 0.046-0.26, p < 0.001). We found no difference between the intravenous immune globulin group and the infants receiving only exchange transfusion as far as the duration of phototherapy. Infants with Rhesus incompatibility had a higher need for top-up transfusions than those with AB0 incompatibility. CONCLUSION: This study supports the evidence from previous studies suggesting that intravenous immune globulin significantly reduces the need for exchange transfusion in infants with Rhesus and AB0 incompatibility.

Multiple dose IVIG treatment in neonatal immune hemolytic jaundice.

Isoimmune hemolytic jaundice due to ABO and Rh blood group incompatibility is an important problem in the neonatal period. Intravenous immune globulin (IVIG) treatment in isoimmune jaundice has been shown to be effective, but the response to treatment is variable. In this study, the effect of multiple doses IVIG therapy versus single dose MG therapy was investigated in 61 babies who had ABO and Rh hemolytic disease. Patients were divided into three groups. Group I received multiple dose IVIG treatment, group II received single dose MG treatment, and group III was not given any IVIG. All three groups received phototherapy. No exchange transfusion was needed in group I. The rate of exchange transfusion was 12 per cent in group II and 33 per cent in group III. Duration of phototherapy was shorter in group I than in groups II and III. It was concluded that IVIG treatment reduces the need of exchange transfusion in neonatal isoimmune hemolytic jaundice by lowering hemolysis. Multiple doses IVIG treatment appears to be better at blocking ongoing hemolysis.

Double volume exchange transfusion for ABO incompatible liver transplantation in paediatric patients: intraoperative and preoperative approach.

Double volume blood exchange transfusions (DBVET) were used to reduce the serum antibody levels in six paediatric patients receiving ABO incompatible hepatic allografts. In four patients, the exchange transfusions took place on the ward prior to surgery. In three of these four patients who had titres measured, the anti-A IgM titres fell from 1024 to 64, 64 to 8, and 128 to 16, respectively. The anti-A IgG titres fell from 32 to 16 and 512 to 64 in two patients, but rose from 16 to 32 in the third. In two patients DBVET were performed intraoperatively using a rapid infusion device. The IgM titres fell from 256 to 32 and 64 to 1, respectively, and the IgG titres fell from 16 to 4 and 2 to 0. Intraoperative DBVET can acutely and effectively reduce blood group antibodies. Intraoperative DBVET may reduce graft ischaemia time and allow red blood cell salvage.

Cryopreserved autologous platelet transfusions in alloimmunized patients with acute leukemia.

Autologous platelets of 5 alloimmunized patients with acute leukemia in remission were cryopreserved with 5% dimethylsulfoxide in liquid nitrogen and retransfused in the following therapy-induced thrombocytopenic phase. The mean platelet recovery after freezing, thawing and washing was 85 +/- 6%. The mean corrected 1-hour increment in platelet counts was 11 (4-27) x 10(9)/l, i.e. 61% in comparison with fresh platelet transfusions in other patients. In vivo function of frozen platelets was documented by improvement of the posttransfusion bleeding time. Cryopreserved autologous platelets function hemostatically and can be used even for completely refractory patients and thus permit curative antileukemic therapy.

[Current validity of the exchange transfusion intervention by the classic indication. Our 15 years' experience]. / Validità attuale dell'intervento di exanguinotrasfusione secondo l'indicazione classica. Nostra esperienza di 15 anni.

In this paper the Authors report their personal experience of the use of exchange transfusion, secondary to classic indication, in the treatment of neonatal hyperbilirubinemia, in order to: 1) determine the trend over the past wears in the number of exchange transfused neonates, both from a global point of view and in relation to indications; 2) critically assess the risks, in terms of complications and mortality, correlated to exchange transfusion. Four hundred and eighty-eight neonates, who were subjected to 693 exchange transfusions in the Pediatric Clinic and Neonatal Division of the Policlinico Gemelli in Roma, were studied over a period of 15 years (1972-1986), according to the following indications: 214 cases of neonatal hyperbilirubinemia with MEN-Rh, 106 cases of neonatal hyperbilirubinemia with MEN-ABO and 168 cases of idiopathic hyperbilirubinemia. The total number of exchange transfused neonates decreased drastically from 304 in the period 1972-76 to 65 in the period 1982-86. The frequency of exchange transfused neonates because of idiopathic hyperbilirubinemia decreased significantly (p greater than 0.001), booth in comparison to the number of live births and in comparison to the number of exchange transfused neonates, probably due to the gradual introduction of phototherapy. The frequency of exchange transfused neonates with iso-Rh and iso-ABO decreased or remained stationary on account of the confirmed relative lesser efficacy of phototherapy on hemolytic jaundice. With regard to mortality and morbidity, 27 out of 488 neonates died during the neonatal period, but only 4 within six hours of exchange transfusion; the majority of those who died had a reduced gestational age and low birthweight, and were affected by a severe associated pathology.

Phototherapy in full-term infants with hemolytic disease secondary to ABO incompatibility.

Current guidelines for treatment of hemolytic disease of the newborn make no differentiation between ABO and Rh incompatibility. A protocol that prolonged the observation period in full-term, ABO-incompatible infants with positive Coombs' tests who were otherwise healthy was tested. Postponement of treatment made it possible to determine more accurately which infants needed phototherapy. This dramatically decreased the number of infants treated without increasing their risk of requiring exchange transfusion.

The treatment of neonatal isoimmune thrombocytopenia with intravenous immunoglobin (IgG i.v.).

We present a report of the use of IgG i.v. to treat clinically manifest neonatal immune thrombocytopenia. The IgG i.v. was administered at a daily dosage of 0.4 g/kg body weight for 5 days. Treatment was started when the child was 3 days old and had a platelet count of 2 X 10(9)/l. Four days later the platelet count had risen to 200 X 10(9)/l. The diagnosis of immune thrombocytopenia was confirmed by platelet typing of the mother's and child's platelets and identification of anti-platelet antibodies in maternal serum.