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Background: Hyperbilirubinemia is one of the most common diagnosis in newborn nurseries in United States. Universal pre-discharge bilirubin screening decreased the incidence of extreme hyperbilirubinemia and risk of kernicterus., Objectives: We sought to assess temporal population trends of hyperbilirubinemia, kernicterus and usage of phototherapy, intravenous immunoglobulin (IVIG), and exchange transfusion., Design/methods: Data from Healthcare Cost and Utilization Project (HCUP)-the Kids' Inpatient Database (KID) obtained for years 1997-2012. All neonatal discharges with ICD-9 codes for neonatal jaundice (774.2, 774.6), kernicterus (773.4, 774.7) and procedure codes for phototherapy (99.83), IVIG infusion (99.14), exchange transfusion (99.01) were extracted. We compared the trends of diagnosis of hyperbilirubinemia, kernicterus, use of phototherapy, IVIG, and exchange transfusion., Results: During the study period, the proportion of infants diagnosed with hyperbilirubinemia increased by 65% (9.4% vs. 15.5%; p <.001) in term infants and 34.5% (33.5% vs. 45%; p <.001) in preterm infants, respectively. Rate of kernicterus discharges significantly reduced from 7 to 1.9 per 100,000 newborns. Overall, the number of exchange transfusions has decreased by 67% during study period while phototherapy and IVIG use increased by 83% and 170%, respectively., Conclusions: In last two decades, there was a significant decrease in neonatal discharges with a history of exchange transfusion or with a diagnosis of kernicterus. However, there was a significant increase in number of neonates discharged home with a history of phototherapy during birth hospitalization and decreased number of exchange transfusions were observed during the study period. Incremental implementation of universal predischarge bilirubin screening and treatments based on 2004 AAP recommended risk-based strategies might have contributed to timely interventions in infants with significant hyperbilirubinemia.

Objective: To understand the risk factors associated with adverse events during exchange transfusion (ET) in severe neonatal hyperbilirubinemia., Study Design: We conducted a retrospective study of infants with hyperbilirubinemia who underwent ET within 30 days of birth from 2015 to 2020 in a children's hospital. Both traditional statistical analysis and state-of-the-art explainable artificial intelligence (XAI) were used to identify the risk factors., Results: A total of 188 ET cases were included; 7 major adverse events, including hyperglycemia (86.2%), top-up transfusion after ET (50.5%), hypocalcemia (42.6%), hyponatremia (42.6%), thrombocytopenia (38.3%), metabolic acidosis (25.5%), and hypokalemia (25.5%), and their risk factors were identified. Some novel and interesting findings were identified by XAI., Conclusions: XAI not only achieved better performance in predicting adverse events during ET but also helped clinicians to more deeply understand nonlinear relationships and generate actionable knowledge for practice., (© 2022. The Author(s).)

Background: Intensive phototherapy (IPT) and exchange transfusion (ET) are the main treatments for extreme hyperbilirubinemia. However, there is no reliable evidence on determining the thresholds for these treatments. This multicenter study compared the effectiveness and complications of IPT and ET in the treatment of extreme hyperbilirubinemia., Methods: This retrospective cohort study was conducted in seven centers from January 2015 to January 2018. Patients with extreme hyperbilirubinemia that met the criteria of ET were included. Patients were divided into three subgroups (low-, medium-, and high- risk) according to gestational week and risk factors. Propensity score matching (PSM) was performed to balance the data before treatment. Study outcomes included the development of bilirubin encephalopathy, duration of hospitalization, expenses, and complications. Mortality, auditory complications, seizures, enamel dysplasia, ocular motility disorders, athetosis, motor, and language development were evaluated during follow-up at age of 3 years., Results: A total of 1164 patients were included in this study. After PSM, 296 patients in the IPT only group and 296 patients in the IPT plus ET group were further divided into the low-, medium-, and high-risk subgroups with 188, 364, and 40 matched patients, respectively. No significant differences were found between the IPT only and IPT plus ET groups in terms of morbidity, complications, and sequelae. Hospitalization duration and expenses were lower in the low- and medium-risk subgroups in the IPT only group., Conclusions: In this study, our results suggest that IPT is a safe and effective treatment for extreme hyperbilirubinemia. The indication of ET for patients with hyperbilirubinemia could be stricter. However, it is necessary to have a contingency plan for emergency ET as soon as IPT is commenced especially for infants with risk factors. If IPT can be guaranteed and proved to be therapeutic, ET should be avoided as much as possible., (Copyright © 2022 The Chinese Medical Association, produced by Wolters Kluwer, Inc. under the CC-BY-NC-ND license.)

Introduction: Phototherapy has reduced the need for exchange transfusion (ET) to manage jaundiced neonates. Hence there are concerns about increased risk of complication due to lack of opportunity to sustain skills in performing ET. We studied the complications and treatment outcomes of neonates treated for jaundice with ET., Methodology: A retrospective observational study was conducted from June 2013 to June 2020 in a tertiary care hospital in India. All neonates treated with ET for jaundice were included., Results: Twenty-eight neonates underwent 31 ET during the study period. Their mean gestational age and birth weight were 37 weeks and 3200 g, respectively. Predisposing factor for jaundice observed were Coomb's positive status (11), hepatosplenomegaly suggesting hemolysis (3), cephalhematoma (2) and birth asphyxia (1). Abnormal neurological status before ET was seen in seven neonates. Adverse clinical events that happened during or within 8 h after ET were desaturation (4), tachycardia (3), tachypnea (2), bradycardia (2), shock (2) and temperature instability (2). One neonate developed acute kidney injury after ET and required peritoneal dialysis. Abnormal lab parameters observed during or within 8 h after ET were hypocalcemia (20), anemia (8), hypokalemia (7), hypernatremia (3), thrombocytopenia (3) and hyperkalemia (2). Post ET sepsis was seen in five neonates: two had only blood culture positive sepsis, two had bone and joint infection and one had liver abscess., Conclusion: The neonates undergoing ET are at high risk of developing complications which may be life threatening. Hence careful monitoring during the procedure is needed., (© The Author(s) [2022]. Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Fat embolism syndrome in sickle cell disease is associated with great mortality, while more than half of survivors suffer severe neurological sequelae. Release of fat droplets leads to obstruction of the microcirculation as well as generation of proinflammatory cytokines that can cause direct tissue injury. Red cell exchange transfusion can be life-saving but the addition of therapeutic plasma exchange may further improve outcomes by removing such inflammatory mediators. Here, we describe the case of a 27-year-old male patient with sickle cell anaemia presenting with typical features of fat embolism syndrome including neurological involvement with greatly reduced level of consciousness. MRI of his brain showed multiple widespread microhemorrhages giving the characteristic "star field" pattern but also a cytotoxic lesion of the corpus callosum, known to be the result of direct neurotoxicity by proinflammatory cytokines. The patient underwent emergency red cell exchange transfusion leading only to modest clinical improvement but fully regained consciousness after three cycles of therapeutic plasma exchange. This case highlights the deleterious effect of the hyperinflammatory state characteristic of many sickle cell complications and supports further exploring the potential benefit from plasma exchange as an adjunct to red cell exchange in order to remove proinflammatory cytokines during acute complications of sickle cell disease., (Copyright © 2021. Published by Elsevier Ltd.)

Transfusion-related lung injury (TRALI) is a condition that develops suddenly within the first six hours after a blood transfusion and it is one of the most important causes of blood transfusion-related mortality. There are few data in the literature about TRALI in the neonatal period. We present two newborn patients who developed TRALI after exchange transfusion due to high bilirubin levels. Our first case was a late preterm LGA baby and was on CPAP. The baby was intubated due to sudden deterioration after the exchange transfusion. Our second case was born at term and, an exchange transfusion was performed on the 5th day of life. He developed respiratory distress unexpectedly soon after the exchange transfusion and was intubated. Glucose-6- phosphate dehydrogenase (G6PD) deficiency was detected in both of our cases. We wanted to emphasize that TRALI should be considered in the differential diagnosis of respiratory distress that develops soon after a transfusion in the newborn period and to draw attention to that TRALI may develop more frequently in patients with G6PD deficiency., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Background: Military experience has shown low-titer O whole blood (LTOWB) to be safe and beneficial in the resuscitation of hemorrhaging trauma patients. However, few civilian centers use LTOWB for trauma resuscitation. We evaluated the early experience and safety of a LTOWB program at a level 1 civilian trauma center., Methods: We retrospectively reviewed our trauma registry from January 2018 to June 2020 for patients admitted in shock (defined as ≥1 of the following: heart rate, >120 beats per minute; systolic blood pressure, <90 mm Hg; or shock index, >0.9) who received blood products within 24 hours. Patients were grouped by resuscitation provided: LTOWB (group 1), component therapy (CT; group 2), and LTOWB-CT (group 3). Safety, outcomes, and variables associated with LTOWB transfusion and mortality were analyzed., Results: 216 patients were included: 34 in Group 1, 95 in Group 2, and 87 in Group 3. Patientsreceiving LTOWB were more commonly male (p<0.001) and had a penetrating injury (p=0.005). Groups 1 and 3 had higher median ISS scores compared to Group 2 (19 and 20 vs 17; p=0.01). Group 3 received more median units of blood product in the first 4h (p<0.001) and in the first 24h (p<0.001). There was no difference between groups in 24h mortality or transfusion-related complications (all p>0.05). Arrival ED SBP was associated with LTOWB transfusion (odds ratio [OR] 0.98, 95% confidence interval [CI] 0.95-1.00, p=0.03). ED lactate was independently associated with 24h mortality. (OR 1.27, CI 1.02-1.58, p=0.03). LTOWB transfusion was not associated with mortality (p=0.49). Abstract., Conclusion: Severely injured patients received LTOWB-CT and more overall product units but had similar 24 h mortality when compared with the LTOWB or CT groups. No increase in transfusion-related complications was seen after LTOWB transfusion. Low-titer O whole blood should be strongly considered in the resuscitation of trauma patients at civilian centers., Level of Evidence: Retrospective, therapeutic, level IV., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Hemoglobin (Hb)-based oxygen (O 2 ) carriers (HBOCs) are being developed as alternatives to red blood cells and blood when these products are unavailable. Clinical trials of previous HBOC generations revealed side effects, including hypertension and vasoconstriction, that were not observed in preclinical studies. Large molecular weight (MW) polymerized bovine Hb (PolybHb) represents a new class of HBOC with promising results. We evaluated the safety profile of PolybHb after an exchange transfusion (ET) in guinea pigs (GPs). This study compares changes in indices of cardiac, inflammatory, and organ function after ET with high (R-state) and low (T-state) O 2 affinity PolybHb with high MW., Study Design and Methods: Guinea pigs underwent a 20% ET with PolybHb. To assess the implication of PolybHb ET on the microcirculation, hamsters instrumented with a dorsal window chamber were subjected to a similar volume ET., Results: T and R-state PolybHb did not induce significant alterations in cardiac function. T-state PolybHb induced mild vasoconstriction shortly after transfusion, while R-state did not have acute effects on microvascular tone., Conclusion: Large MW PolybHbs were found to be safe and efficacious in increasing O 2 carrying capacity and the O 2 affinity of the PolybHb did not affect O 2 delivery or extraction by tissues in relevant preclinical models. In conclusion, these results suggest that both T-state and R-state PolybHb are safe and do not impair O 2 delivery. The results are encouraging and support further evaluation of high MW PolybHbs and their future feasibility compared to allogenic blood in a trauma model., (© 2020 AABB.)

Background: Exchange transfusion (ET) is an established, efficacious, and reliable practice for severe neonatal hyperbilirubinemia, hemolytic disease of the newborn, and neonatal sepsis. This study assessed the indications and clinical outcomes of ET performed in a tertiary hospital in Korea., Materials and Methods: We studied 64 ET sessions performed on 23 neonates between March 1999 and March 2018. ET was performed based on estimated double volume exchange transfusion using fresh red blood cells and fresh frozen plasma. Patients' clinical information, including demographic data and ET indication, and laboratory data were collected pre- and post-ET., Results: The most common ET indication was hyperbilirubinemia with hemolytic anemia due to non-ABO maternal blood group discrepancies. In three preterm babies, ETs were performed for severe anemia, leukocytosis, and hyperkalemia cases. Before ET, the patients showed slightly high WBC counts, low hemoglobin levels, and low platelet counts. After ET, blood examination revealed normal WBC counts, increased hemoglobin levels, and decreased platelet counts (all P < 0.001). Bilirubin levels decreased immediately after ET (P < 0.001). Electrolyte and C-reactive protein levels showed no significant changes after ETs. Adverse events occurred in 11 (47.8 %) patients; the most common were hypoxemia and hypotension. One infant experienced cardiorespiratory arrest due to hypercalcemia and was successfully resuscitated. No one died within 24 h of ET. However, five infants showed hyperbilirubinemia aggravation., Conclusions: ET is an effective treatment modality for leukocytosis and hyperbilirubinemia with low mortality but involves common adverse events post-ET. This report provides an overview of current ET practices in Korea., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Aim: To evaluate the role of pathway to admission for jaundice among the risk factors for exchange transfusion in outborn infants in a low resource setting. Methods: This retrospective case-control study (1:1 ratio) was carried out at the Yankin Children's Hospital in Yangon (Myanmar). All cases were neonates admitted for treatment of jaundice between March 2013 and February 2014 and who required an exchange transfusion. Each control was the next noncase neonate admitted for treatment of jaundice and treated with phototherapy. Infant characteristics, pathways of admission and clinically relevant factors for exchange transfusion were collected. Results: One hundred thirty-four cases and 134 controls were included in the study. Among cases, home was the most common place of birth while public hospital was the most frequent source of referral. Among controls, private/public hospitals were the commonest places of birth and referral. At multivariable analysis, homebirth was associated with increased likelihood of receiving exchange transfusion at admission (OR 3.30, 95% C.I. 1.31-8.56). Conclusion: Homebirth was an independent risk factor for exchange transfusion at admission for jaundice in a low-resource setting. Appropriate health education of pregnant women and traditional/home birth attendants may contribute to reduce the need for exchange transfusion in low-resource settings.

Background: Mt a (MNS14) is a low-prevalence antigen of the MNS system. A few cases of hemolytic disease of the fetus and newborn caused by anti-Mt a have been reported in the literature, but up to now this antibody has never been associated with a hemolytic transfusion reaction (HTR)., Case Report: A 38-year-old male with sickle cell disease undergoing exchange transfusion presented with shivering, nausea, dyspnea, and pain in the lower limbs. Biologic parameters showed increased hemolysis. The administered red blood cell (RBC) units had been issued by electronic crossmatch due to a negative antibody screening test. In the posttransfusion investigations, crossmatch of the transfused RBC units with the patient's serum showed incompatibility of one unit. The presence of an antibody against a low-prevalence antigen was suspected and further serologic testing was performed for identification., Results: Anti-Mt a was identified in the patient's serum. The RBCs of the incompatible unit implicated in the HTR were Mt(a+). An eluate of a posttransfusion blood sample of the patient was nonreactive with the incompatible RBCs, and the direct antiglobulin test was negative., Conclusion: To our knowledge, this is the first case report of an HTR associated with anti-Mt a ., (© 2019 AABB.)

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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Children and adolescents with sickle cell disease (SCD) are at high risk of strokes and are frequently treated with red blood cell (RBC) transfusions. The goal is to suppress hemoglobin (Hb) S while minimizing transfusion-induced iron overload. RBCs may be given via simple transfusion, manual exchange transfusion (MET), or erythrocytapheresis (aRBCX). Chronic transfusion practices vary among institutions., Methods: This single-institution, retrospective cohort study compares Hb S control and therapy complication rates between MET and aRBCX in a cohort of children and adolescents with SCD and stroke during a 5-year period from 2008 through 2012. Duration and mode of transfusion therapy, achievement of Hb S suppression goal, iron burden by ferritin levels, and catheter complications were evaluated., Results: Thirty-seven children were included in analysis. The prevalence of catheter complications was 75% in aRBCX recipients compared with 0% in MET recipients (P < 0.001). There was no significant difference between modalities in achieving Hb S suppression or ferritin goals, but those receiving aRBCX had a greater likelihood of discontinuing chelation therapy. Among aRBCX recipients, adherence to >90% of transfusion appointments was associated with achieving Hb S suppression goals., Conclusion: aRBCX may have increased complication risks compared with MET for chronic transfusion therapy in SCD. Risks and benefits of aRBCX and MET should be considered when selecting a chronic transfusion modality. Transfusion therapy modalities should be compared in prospective studies for stroke prevention in children with SCD., (© 2017 Wiley Periodicals, Inc.)

Background: Exchange blood transfusion (EBT) is a form of whole blood transfusion in which the total blood volume is replaced within a few hours. In perinatal and neonatal medicine, EBT is most often used in the management of severe anaemia or severe hyperbilirubinaemia in the first week of life. Hypocalcaemia, one of the common morbidities associated with EBT, is thought to arise from the chelating effects of the citrate commonly used as an anticoagulant in the donor's blood. This disorder manifests with muscular and nervous irritability and cardiac arrhythmias., Objectives: To determine whether the use of prophylactic calcium reduces the risk of hypocalcaemia-related morbidities and death among newborn infants receiving EBT., Search Methods: We used the standard search strategy of the Cochrane Neonatal Review group to search the Cochrane Central Register of Controlled Trials (CENTRAL 2016, Issue 5), MEDLINE via PubMed (1966 to 29 June 2016), Embase (1980 to 29 June 2016), and CINAHL (1982 to 29 June 2016). We also searched clinical trials databases, conference proceedings, and the reference lists of retrieved articles for randomised controlled trials and quasi-randomised trials., Selection Criteria: All randomised and quasi-randomised trials of prophylactic intravenous calcium in EBT for newborns., Data Collection and Analysis: Two review authors independently assessed and extracted data on methods, participants, interventions, and outcomes (mean total and ionised serum calcium before and after EBT and the presence of adverse events such as hypoglycaemia, apnoea, cardiac arrest, and death immediately after EBT). We reported results as means difference (MD) with 95% confidence intervals (CI) for continuous outcomes and risk ratio (RR) and risk differences (RD) and 95% CIs for dichotomous outcomes. We assessed quality using the Cochrane 'Risk of bias' assessment tool and the GRADE system., Main Results: We found only one quasi-randomised trial with 30 participants that met our inclusion criteria. In the small trial, total and ionised serum calcium levels were measured immediately before and immediately after EBT. All the participants were included in the final analysis and all the important outcomes were reported. Primary outcomesThere was one death in each group (RR 1.00, 95% CI 0.07 to 14.55; RD 0.00, 95% CI -0.18 to 0.18; participants = 30; studies = 1). The study did not report the presence of cardiac arrhythmias within one week of EBT and the number of infants with serum calcium levels (total less than 8 mg/dL (2 mmol/L) or ionised less than 4.4 mg/dL (1.1 mmol/L)).Pair-wise comparison of EBT with intravenous 10% calcium gluconate versus EBT without intravenous calcium (change from baseline) showed mean total serum calcium was raised in the intervention group compared to the control group (MD -0.46, 95% CI -0.81 to -0.11; participants = 30; studies = 1). Very low-quality evidence also indicated an increase in the levels of mean ionised serum calcium in the intervention group compared to the control group (MD -0.22, 95% CI -0.33 to -0.11; participants = 30; studies = 1). Secondary outcomesAdverse reactions to intravenous calcium therapy included cardiac arrest in one neonate in the intervention arm (RR 3.00, 95% CI 0.13 to 68.26; RD 0.07, 95% CI -0.10 to 0.23; participants = 30; studies = 1). There was apnoea and hypoglycaemia (RR 1.00, 95% CI 0.07 to 14.55; RD 0.00, 95% CI -0.18 to 0.18; participants = 30; studies = 1) in the two neonates who died. Data were not available for other major secondary outcomes such as the number of infants with reduced serum magnesium, reduced parathormone, increased calcitonin, presence of seizures, carpopedal spasm, jitteriness and prolonged QTc interval on electrocardiography within one week of EBT., Authors' Conclusions: Very low-quality data from one quasi-randomised controlled trial suggested that the mean serum total and ionised calcium increased in the study group but decreased in the control group immediately after EBT. However, the mean values of total and ionised calcium in both arms of studies remained within international reference ranges. Unfortunately, data were not available to assess the trend of total and ionised serum calcium to the end of the first week after EBT. Therefore, due to the very low quality of evidence available, it is difficult to support or reject the continual use of prophylactic intravenous calcium in newborn infants receiving EBT. Researchers are encouraged to conduct more robustly designed trials with larger numbers of participants, and particularly, addressing the pattern of differences based on gestational age of participants, type of anticoagulant used, and the volume of blood used.

Septic shock, occurring in about 1% of neonates hospitalized in neonatal intensive care unit (NICU), is a major cause of death in the neonatal period. In the 1980s and 90s, exchange transfusion (ET) was reported by some authors to be effective in the treatment of neonatal sepsis and septic shock. The main aim of this retrospective study was to compare the mortality rate of neonates with septic shock treated only with standard care therapy (ScT group) with the mortality rate of those treated with ScT and ET (ET group). All neonates with septic shock admitted to our NICU from 2005 to 2015 were included in the study. Overall, 101/9030 (1.1%) neonates had septic shock. Fifty neonates out of 101 (49.5%) received one or more ETs. The mortality rate was 36% in the ET group and 51% in the ScT group (p = 0.16). At multivariate logistic regression analysis, controlling for potentially confounding factors significantly associated with death (gestational age, serum lactate, inotropic drugs, oligoanuria), ET showed a marked protective effect (Odds Ratio 0.21, 95% Confidence Interval: 0.06-0.71; p = 0.01). The lack of observed adverse events should encourage the use of this procedure in the treatment of neonates with septic shock.

Objectives: To identify the predictors of repeat exchange transfusion among infants with severe hyperbilirubinemia., Design: Retrospective cross-sectional study., Setting: A referral children's hospital in inner-city Lagos, Nigeria., Patients: Infants who received exchange transfusion for severe hyperbilirubinemia from January 2012 to December 2014., Intervention: None., Measurements and Main Results: The predictors of repeat exchange transfusion were identified among all infants who had at least one exchange transfusion using multivariable logistic regression. A total of 352 infants with mean peak total serum bilirubin of 26.32 ± 7.96 mg/dL received exchange transfusion; of these, 49 (13.9%) with mean peak total serum bilirubin of 32.85 ± 10.54 mg/dL had repeat exchange transfusion. More than two thirds of infants who received exchange transfusion and repeat exchange transfusion were male, and at least one third had ABO incompatibility. No infant had more than two exchange transfusions. The mean age of admission was approximately 5 days (range, 1-14 d). Peak total serum bilirubin greater than or equal to 30 mg/dL (odds ratio, 2.88; 95% CI, 1.46-5.70) and acute bilirubin encephalopathy (odds ratio, 2.37; 95% CI, 1.18-4.77) were predictive of repeat exchange transfusion., Conclusions: Acute bilirubin encephalopathy and excessive total serum bilirubin levels at least 30 mg/dL are predictive of repeat exchange transfusion. A risk assessment framework that combines total serum bilirubin levels, acute bilirubin encephalopathy status, and risk factors of neurotoxicity should be considered for the timely detection and monitoring of infants at risk of repeat exchange transfusion.

Background: Priapism is unwanted painful penile erection that affects about 36% of boys and men with sickle cell disease (SCD) most of whom have sickle cell anemia. Clinically, priapism could be stuttering, minor, or major. The first two types are mild, last < 4 h, are usually treated at home, have good prognosis with normal sexual function. The major type of priapism lasts >4 h, associated with severe pain, requires hospitalization; often does not respond to medical treatment and may require shunt surgery. Untreated major priapism and surgical intervention often cause impotence. In this study, we report our 15-year experience in treating adult patients with SCD and major priapism with blood exchange transfusion after being refractory to other medical therapies., Methods: Adult male African Americans patients with SCD and major priapism were enrolled in this study and followed for 15 years. A Haemonitics V-50 machine was initially used for whole blood exchange and was later replaced with Cobe Spectra machine for RBC exchange., Results: We used 239 blood exchanges requiring 1,136 RBC units. We maintained a post-exchange hemoglobin level of about 10 g/dL and hemoglobin S level < 30%. None of the patients had any neurological complications such as headache, seizures, neurological deficits, or obtundation post-exchange., Conclusion: Together, the data indicate that blood exchange transfusion for the treatment of patients with SCD and major priapism is efficacious and safe., (© 2015 Wiley Periodicals, Inc.)

Background: Exchange transfusion (ET) for severe neonatal hyperbilirubinemia (SNH) is frequently undertaken in low- and middle-income countries (LMIC), in sharp contrast to the prevailing practice in high-income countries. However, the criteria for initiating this procedure in settings with limited resources for treating infants with SNH have not been systematically explored., Objective: To identify key considerations for initiating ET in resource-poor countries to curtail its unnecessary use for the prevention of kernicterus., Methods: A review of the existing guidelines and literature on the management of neonatal hyperbilirubinemia worldwide was conducted to identify criteria and underlying factors for initiating ET., Results: There is a dearth of evidence from randomized clinical trials to support clear criteria for indicated ET worldwide. Because risk assessment for kernicterus based solely on the levels of total serum bilirubin (TSB) has often proved inadequate, a combination of plasma/serum bilirubin estimation and clinical evaluation for acute bilirubin encephalopathy (ABE) has been recommended for predicting the risk of kernicterus. However, there is a lack of consistency regarding the TSB levels for which ET should be initiated in relation to the clinical signs/symptoms of ABE and hemolytic disorders., Conclusions: A decision-making framework that combines TSB thresholds and evidence of neurotoxicity is needed for evaluating the risk of kernicterus and prioritising infants for ET in LMICs to curtail unnecessary interventions., (© 2015 S. Karger AG, Basel.)

Objective: Prophylactic intravenous immunoglobulin (IVIg) does neither reduce the need for exchange transfusion nor the rates of other adverse neonatal outcomes in neonates with rhesus hemolytic disease of the fetus and newborn (rhesus HDFN) according to our randomized controlled trial analysis. Our objective was to assess the long-term neurodevelopmental outcome in the children included in the trial and treated with either IVIg or placebo., Methods: All families of the children included in the trial were asked to participate in this follow-up study. The long-term neurodevelopmental outcome in children at least 2 years of age was assessed using standardized tests. The primary outcome was the incidence of neurodevelopmental impairment defined as at least one of the following: cerebral palsy, severe cognitive and/or motor developmental delay (with a test score of less than -2 SD), bilateral deafness or blindness., Results: Sixty-six of the 80 children (82.5%) who had been recruited to the initial randomized controlled trial participated in the follow-up study. The children were assessed at a median age of 4 years (range 2-7). The median cognitive score was 96 (range 68-118) in the IVIg group and 97 (range 66-118) in the placebo group (p = 0.79). There was no difference in the rate of neurodevelopmental impairment between the IVIg and the placebo group [3% (1/34) vs. 3% (1/32); p = 1.00]., Conclusions: The long-term neurodevelopmental outcome in children treated with IVIg was not different from that in children treated with placebo. Standardized long-term follow-up studies with large enough case series and sufficient power are needed to replicate these findings., (© 2015 S. Karger AG, Basel.)

Background: Massive exchange transfusion of 42-day-old red blood cells (RBCs) in a canine model of Staphylococcus aureus pneumonia resulted in in vivo hemolysis with increases in cell-free hemoglobin (CFH), transferrin-bound iron (TBI), non-transferrin-bound iron (NTBI), and mortality. We have previously shown that washing 42-day-old RBCs before transfusion significantly decreased NTBI levels and mortality, but washing 7-day-old RBCs increased mortality and CFH levels. We now report the results of altering volume, washing, and age of RBCs., Study Design and Methods: Two-year-old purpose-bred infected beagles were transfused with increasing volumes (5-10, 20-40, or 60-80 mL/kg) of either 42- or 7-day-old RBCs (n = 36) or 80 mL/kg of either unwashed or washed RBCs with increasing storage age (14, 21, 28, or 35 days; n = 40)., Results: All volumes transfused (5-80 mL/kg) of 42-day-old RBCs resulted in alike (i.e., not significantly different) increases in TBI during transfusion as well as in CFH, lung injury, and mortality rates after transfusion. Transfusion of 80 mL/kg RBCs stored for 14, 21, 28, and 35 days resulted in increased CFH and NTBI in between levels found at 7 and 42 days of storage. However, washing RBCs of intermediate ages (14-35 days) does not alter NTBI and CFH levels or mortality rates., Conclusions: Preclinical data suggest that any volume of 42-day-old blood potentially increases risks during established infection. In contrast, even massive volumes of 7-day-old blood result in minimal CFH and NTBI levels and risks. In contrast to the extremes of storage, washing blood stored for intermediate ages does not alter risks of transfusion or NTBI and CFH clearance., (© 2015 AABB.)

Objective: The aim of present study was to evaluate the indications and the complications associated with neonatal exchange transfusion (ET) performed for hyperbilirubinemia., Methods: This study included overall 306 neonates who underwent ET between 2005 and 2012. The demographic characteristics of patients, causes of jaundice and adverse events occurred during or within 1 week after ET were recorded from their medical files. Those newborns that underwent ET were classified as either "otherwise healthy" or "sick" group., Results: Of the 306 patients who underwent ET, 244 were otherwise healthy and had no medical problems other than jaundice. The remaining 62 patients were classified as sick that had medical problems other than jaundice ranging from mild to severe. The mean gestational age was 37.6 ± 2.5 weeks and the mean peak total bilirubin levels was 25.8 ± 6.6 mg/dl. The mean age at presentation was 5.4 ± 3.8 d for all infants. The most common cause of hyperbilirubinemia was ABO isoimmunization (27.8%). None of newborns died secondary to ET. Three infants had had necrotizing enterocolitis, and also three infants had had acute renal failure. The most common encountered complications of ET procedure were hyperglycemia (56.5%), hypocalcaemia (22.5%) and thrombocytopenia (16%)., Conclusions: Our data showed that ABO isoimmunization was the most common cause of hyperbilirubinemia. Even mortality was not seen, very rare but major gastrointestinal and renal complications were associated with ET. The majority of adverse events associated with ET were laboratory abnormalities mainly hyperglycemia, hypocalcaemia and thrombocytopenia which were asymptomatic and treatable.

Objective Red cell exchange transfusion is frequently used in the management of patients with sickle cell disease (SCD) either electively or chronically to maintain hemoglobin S (HbS) <30%. The purpose of this retrospective study was to evaluate the results of manual chronic partial exchange transfusion (MCPET) on level of Hb and HbS, on iron load and on the need for chelation, on risk of immunization, monitoring transfusion-transmitted viral infection, and clinical outcome. Methods We reviewed the long-term effect of MCPET in 10 children (six men and four women) with SCD and evaluated the iron balance during a median follow-up of 20 months (range: 6-36) in which 248 exchanges were performed. Results The pre-exchange median Hb value was 9.5 g/dl (range: 7.7-10.9 g/dl) and the median post-exchange value was 9.4 g/dl (range: 8.4-11.1 g/dl).The majority of patients reached an HbS of <50% with a median HbS value of 40.04% (range: 30-54). At start of the MCPET program, the median ferritin was 439 ng/ml (range: 80-1704 ng/ml). In the final evaluation, the median value of ferritin was 531 ng/ml (range: 84-3840 ng/ml). The annual calculated iron balance was 0. 28 ± 0.08 mg/kg/day. MCPET was well tolerated, and adverse effects were limited. Discussion MCPET in children with SCD is safe to prevent iron overload, and is effective and easy to use in our cohort. Conclusion Indication for chronic exchange blood transfusion is essential for patients with SCD with recurrent and frequent crises who do not respond to hydroxyurea. However, there is no consensual study for the period at which chronic transfusion can safely be stopped and further research in large population of patients with SCD will need to clarify this question.

Background: An exchange transfusion involves replacing patient's blood with donor blood in order to remove abnormal blood components and circulating toxins while maintaining adequate circulating blood volume., Objective: To observe the incidence, causes of jaundice requiring Exchange and any adverse event of exchange transfusion in newborns with unconjugated hyperbilirubinemia., Method: Prospective study undertaken at Neonatal Intensive Care Unit (NICU) of Manipal Teaching Hospital, Pokhara, Nepal from March 2014 to April 2015. For both mothers and neonates blood group and Rh typing and for all newborns pre and post exchange complete blood count with peripheral smear, serum bilirubin, hemoglobin, calcium, potassium, random blood sugar, C-reactive protein and blood culture and where ever required Direct Coombs test, reticulocyte count, G6PD activity and thyroid function test were done. The incidence, indications, positive outcome, complications and mortality were noted., Result: Out of 481 cases of unconjugated hyperbilirubinemia 29 (6%) required exchange transfusion. 55.2% Pathological Jaundice [13.8% ABO incompatibility, sepsis and hypothyroidism was commonest causes] and 44.8% exaggerated physiological jaundice [27.6% with no underlying pathology, 10.3% preterms 3.4% cephalhematoma] required exchange transfusion. Post transfusion, bilirubin level decreased significantly (p < 0.001). The commonest adverse events noted were anemia (89.7% / p < 0.018), hyperglycemia(51.7% / p < 0.001), hypocalcaemia (48.3% / p < 0.001)), sepsis(10.3%), hypernatremia (13.8%), hyperkalaemia, bradycardia, apnea and feed intolerance (6.9%). None of them had kernicterus and there was no mortalities., Conclusion: Exchange transfusion is an effective procedure to decrease bilirubin levels but is associated with many complications. Hypothyroidism was one of the commonest cause of jaundice requiring Exchange transfusion.

Objective: We compared intra-procedural neonatal pain, agitation and sedation scale (N-PASS) scores between a novel 'continuous arteriovenous exchange' (CAVE) and conventional pull-push (PP) techniques of partial exchange transfusion (PET) among neonates with polycythemia., Study Design: Neonates >32-0/7 weeks gestation, requiring PET for polycythemia, were randomized to PP or CAVE techniques. The procedure was video-recorded and edited to mask the technique. Intra-procedural N-PASS scores assigned by two trained and masked neonatal fellows were compared., Result: Twenty-two neonates were randomized to CAVE (n=12) or PP (n=10) method. The area under curve for cumulative N-PASS scores was significantly lesser in CAVE group (mean difference-11.9 (95% CI=-4.2, -19.6), P=0.005)). Decrease in hematocrit and complications of PET were comparable. Time for PET was longer with CAVE technique (16 (9, 29) min vs 10 (6, 12) min, P=0.016)., Conclusion: CAVE technique of PET was associated with lesser procedure-related pain (N-PASS scores) as compared with PP technique among neonates >32 weeks gestation.

Background: In experimental pneumonia we found that transfused older blood increased mortality and lung injury that was associated with increased in vivo hemolysis and elevated plasma cell-free hemoglobin (CFH), non-transferrin-bound iron (NTBI), and plasma labile iron (PLI) levels. In this study, we additionally analyze identically treated animals that received lower or higher bacterial doses., Study Design and Methods: Two-year-old purpose-bred beagles (n = 48) challenged intrabronchially with Staphylococcus aureus (0 [n = 8], 1.0 × 10(9) [n = 8], 1.25 × 10(9) [n = 24], and ≥1.5 × 10(9) [n = 8] colony-forming units/kg) were exchange transfused with either 7- or 42-day-old canine universal donor blood (80 mL/kg in four divided doses)., Results: The greater increases in CFH with older blood over days after exchange proved relatively independent of bacterial dose. The lesser increases in CFH observed with fresher blood were bacterial dose dependent potentially related to bacterial hemolysins. Without bacterial challenge, levels of CFH, NTBI, and PLI were significantly higher with older versus fresher blood transfusion but there was no significant measurable injury. With higher-dose bacterial challenge, the elevated NTBI and PLI levels declined more rapidly and to a greater extent after transfusion with older versus fresher blood, and older blood was associated with significantly worse shock, lung injury, and mortality., Conclusion: The augmented in vivo hemolysis of transfused older red blood cells (RBCs) appears to result in excess plasma CFH and iron release, which requires the presence of established infection to worsen outcome. These data suggest that transfused older RBCs increase the risks from infection in septic subjects., (© 2014 AABB.)

Although neonatal jaundice is common, acute bilirubin encephalopathy and kernicterus (i.e., chronic bilirubin encephalopathy) are rare. Universal screening for neonatal hyperbilirubinemia is controversial. The American Academy of Pediatrics recommends universal screening with bilirubin levels or targeted screening based on risk factors. However, the U.S. Preventive Services Task Force and the American Academy of Family Physicians found insufficient evidence that screening improves outcomes. Universal screening may also increase rates of phototherapy, sometimes inappropriately. Younger gestational age and exclusive breastfeeding are the strongest risk factors for the development of hyperbilirubinemia. Infants who appear jaundiced should be evaluated by a risk score or by measurement of total serum or transcutaneous bilirubin. Phototherapy is an effective treatment for hyperbilirubinemia, but the number needed to treat varies widely depending on sex, gestational age, and time since delivery. If indicated, phototherapy should be initiated based on gestational age and risk factors. Exchange transfusion leads to complications in about 5% of treated infants and has a mortality rate of three or four per 1,000 infants. Infants who breastfeed exclusively-particularly those who consume inadequate calories-are at increased risk of hyperbilirubinemia. However, interrupting breastfeeding for the treatment of jaundice increases the risk of early discontinuation of breastfeeding. Encouragement from health care professionals is important to promote breastfeeding in these situations.

The risk factors associated with the use of glucose-6-phosphate dehydrogenase (G6PD)-deficient blood in transfusion have not yet been well established. Therefore, the aim of this review was to evaluate whether whole blood from healthy G6PD-deficient donors is safe to use for transfusion. The study undertook a systematic review of English articles indexed in COCHRANE, MEDLINE, EMBASE, and CINHAL, with no date restriction up to March 2013, as well as those included in articles' reference lists and those included in Google Scholar. Inclusion criteria required that studies be randomized controlled trials, case controls, case reports, or prospective clinical series. Data were extracted following the Preferred Reporting Items for Systematic Reviews using a previously piloted form, which included fields for study design, population under study, sample size, study results, limitations, conclusions, and recommendations. The initial search identified 663 potentially relevant articles, of which only 13 studies met the inclusion criteria. The reported effects of G6PD-deficient transfused blood on neonates and children appear to be more deleterious than effects reported on adult patients. In most cases, the rise of total serum bilirubin was abnormal in infants transfused with G6PD-deficient blood from 6 hours up to 60 hours after transfusion. All studies on neonates and children, except one, recommended a routine screening for G6PD deficiency for this at-risk subpopulation because their immature hepatic function potentially makes them less able to handle any excess bilirubin load. It is difficult to make firm clinical conclusions and recommendations given the equivocal results, the lack of standardized evaluation methods to categorize red blood cell units as G6PD deficient (some of which are questionable), and the limited methodological quality and low quality of evidence. Notwithstanding these limitations, based on our review of the available literature, there is little to suggest that G6PD-deficient individuals should be excluded from donating red blood cells, although transfusions of such blood may potentially have negative impacts on premature neonates or patients who need repeated transfusions, and thus, for this group, screening for G6PD deficiency may be appropriate., (Crown Copyright © 2014. Published by Elsevier Inc. All rights reserved.)

Aim: In the 21st century, neonatal exchange transfusions (ETs) are uncommon procedures usually performed in tertiary neonatal units. As junior clinical staff now lack familiarity with the procedure, it is important to maintain awareness of its complications in order to manage clinical risks and counsel parents appropriately. The study aims to analyse the ET rate, its indications and its associated complications, in a single tertiary centre in the 21st century., Methods: This is a retrospective cohort study of all infants receiving ET from 1 January 2001 to 31 December 2010 at the Royal Women's Hospital, Melbourne., Results: Sixty-four ETs were performed in 51 infants, an average of 6.4 ETs per year. Forty-nine (96%) infants were exchanged for hyperbilirubinaemia and two (4%) for anaemia. Thirty-six (71%) infants had Rhesus haemolytic disease of the newborn and six (12%) had ABO incompatibility. Six infants were intubated and mechanically ventilated after ET; these infants were significantly more acidotic during the ET than those who were never on respiratory support (mean pH 7.153 and 7.309 respectively, mean difference -0.156, 95% CI -0.196 to -0.116, t = 7.85, P < 0.001). Overall mortality was 8% (n = 4)., Conclusions: Our current ET rate is very low compared with historical data. It is difficult to ascribe mortality and morbidity directly to ET as the procedure is now often performed on smaller, sicker or more premature infants whose risks of mortality and morbidity are high regardless of ET. Prospective multi-centre studies are needed to provide adequate data to analyse complications in greater detail., (© 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Background: Exchange transfusion (ET) has biologic plausibility as an adjunct to antimalarial drugs in treating severe malaria and has been used for decades despite limited evidence of its efficacy in improving survival. We examined the efficacy of ET as an adjunct treatment for severe malaria using US surveillance data and reviewed the literature to update recommendations., Methods: Patients with severe malaria reported to the US national malaria surveillance system during 1985-2010 were matched, and survival outcomes were compared between patients receiving and not receiving ET. The literature review used search terms "severe malaria" and "exchange transfusion." Case reports and series, observational and case-control studies, and meta-analysis were included., Results: One hundred one patients receiving ET were matched to 314 patients not receiving ET. There was no statistically significant association between ET and survival outcome (odds ratio, 0.84; 95% confidence interval, .44-1.60). We found 87 articles, mostly case reports or series, showing successful use of ET, likely reporting bias. There were 12 comparative studies, most of which were retrospective cohort studies, underpowered with no significant differences in survival. A previously published meta-analysis of 8 comparative studies found no significant survival differences. Adverse events were rarely reported but included acute respiratory distress syndrome, ventricular fibrillation, and hypotension., Conclusions: Despite rapid parasite clearance times resulting from ET, there is no evidence for efficacy of ET as adjunctive therapy in severe malaria. Adjunct ET cannot be recommended. When rapidly acting antimalarials, specifically artemisinins, become more widely available, the biologic plausibility argument for ET will become less relevant.

Red blood cell exchange (RBCEx) is frequently used in the management of patients with sickle cell disease (SCD) and acute chest syndrome or stroke, or to maintain target hemoglobin S (HbS) levels. In these settings, RBCEx is a category I or II recommendation according to guidelines on the use of therapeutic apheresis published by the American Society for Apheresis. Matching donor red blood cells (RBCs) to recipient phenotypes (e.g., C, E, K-antigen negative) can decrease the risk of alloimmunization in patients with multi-transfused SCD. However, this may select for donors with a higher prevalence of RBC disorders for which screening is not performed. This report describes a patient with SCD treated with RBCEx using five units negative for C, E, K, Fya, Fyb (prospectively matched), four of which were from donors with hemoglobin variants and/or glucose-6-phosphate dehydrogenase (G6PD) deficiency. Pre-RBCEx HbS quantification by high performance liquid chromatography (HPLC) demonstrated 49.3% HbS and 2.8% hemoglobin C, presumably from transfusion of a hemoglobin C-containing RBC unit during a previous RBCEx. Post-RBCEx HPLC showed the appearance of hemoglobin G-Philadelphia. Two units were G6PD-deficient. The patient did well, but the consequences of transfusing RBC units that are G6PD-deficient and contain hemoglobin variants are unknown. Additional studies are needed to investigate effects on storage, in-vivo RBC recovery and survival, and physiological effects following transfusion of these units. Post-RBCEx HPLC can monitor RBCEx efficiency and detect the presence of abnormal transfused units., (Copyright © 2013 Wiley Periodicals, Inc.)

Background: Allografts are used for vascular reconstruction in many forms of congenital heart disease. Although allografts induce anti-human leukocyte antibody (HLA) formation, much about this response is unknown., Methods: Three groups of patients aged 8 to 18 years old underwent analysis for class I and II anti-HLA antibodies using Luminex. Groups were defined by timing of allograft exposure and diagnosis at Norwood for hypoplastic left heart syndrome (neonatal group), at Glenn for single-ventricle lesions not requiring arch reconstruction (infant group), and cardiac defects repaired during infancy without allografts (controls). Patients had significant anti-HLA (sensitization) if mean fluorescence intensity was ≥ 1500., Results: The study enrolled 29 patients (median age, 10.1 years). Significant class I anti-HLA antibodies were seen in 44% (8 of 18) of the neonatal group, 25% (1 of 4) of the infant group, and 14% (1 of 7) of controls; class II anti-HLA antibodies were seen in 44% (8 of 18) of the neonatal group, 25% (1 of 4) of the infant group, and 29% (2 of 7) of controls. All patients received fresh whole blood, but the neonatal group had greater exposure (p = 0.001). There was less sensitization with increasing time from last receipt of allograft(s) or blood transfusion (p = 0.05)., Conclusions: Exposure to allograft at the Norwood procedure is associated with long-term sensitization to anti-HLA antibodies in 56% of patients. Sensitization also occurs in those without prior exposure to allografts, may decrease over time, and appears related to whole blood. These findings have implications for those in whom heart transplant is considered late in the clinical course., (Copyright © 2013 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Two-year-old purpose-bred beagles (n = 24) infected with Staphylococcus aureus pneumonia were randomized in a blinded fashion for exchange transfusion with either 7- or 42-day-old canine universal donor blood (80 mL/kg in 4 divided doses). Older blood increased mortality (P = .0005), the arterial alveolar oxygen gradient (24-48 hours after infection; P ≤ .01), systemic and pulmonary pressures during transfusion (4-16 hours) and pulmonary pressures for ~ 10 hours afterward (all P ≤ .02). Further, older blood caused more severe lung damage, evidenced by increased necrosis, hemorrhage, and thrombosis (P = .03) noted at the infection site postmortem. Plasma cell–free hemoglobin and nitric oxide (NO) consumption capability were elevated and haptoglobin levels were decreased with older blood during and for 32 hours after transfusion (all P ≤ .03). The low haptoglobin (r = 0.61; P = .003) and high NO consumption levels at 24 hours (r = −0.76; P < .0001) were associated with poor survival. Plasma nontransferrin-bound and labile iron were significantly elevated only during transfusion (both P = .03) and not associated with survival (P = NS). These data from canines indicate that older blood after transfusion has a propensity to hemolyze in vivo, releases vasoconstrictive cell-free hemoglobin over days, worsens pulmonary hypertension, gas exchange, and ischemic vascular damage in the infected lung, and thereby increases the risk of death from transfusion.

Background: Exchange transfusion (ET) is a high-risk procedure. The type and rate of complications in neonatal red cell alloimmune hemolytic disease exclusively are not clear., Objectives: Our aim was to study the type and rate of complications associated with ET in a large series of neonates with hemolytic disease of the fetus and newborn (HDFN) due to red cell alloimmunization., Methods: All neonates with HDFN due to red cell alloimmunization admitted to our center between January 2001 and June 2011 were eligible for this study. We recorded the number and rate of complications during admission in the group of neonates treated with ET (ET group) and not treated with ET (no-ET group). Multivariate logistic regression analysis was performed to measure the independent risk of complications of ET treatment., Results: A total of 347 infants with red cell alloimmune hemolytic disease were included; 39% (134/347) were treated with at least one ET (ET group), and 61% (213/347) did not require ET (no-ET group). Comparison between the ET group and no-ET group showed that ET treatment was independently associated with proven sepsis [8 vs. 1%, respectively; odds ratio (OR) 8.3, 95% confidence interval (CI) 1.7-40.3; p = 0.009], leukocytopenia (88 vs. 23%, respectively; OR 36.0, 95% CI 17.5-73.8; p < 0.001), severe thrombocytopenia (platelet count <50 × 10(9)/l; 63 vs. 8%, respectively; OR 31.4, 95% CI 14.0-70.4; p < 0.001), hypocalcemia (22 vs. 1%, respectively; OR 27.4, 95% CI 5.9-126.8; p < 0.001) and hypernatremia (8 vs. 0%, respectively; p < 0.001). There were no neonatal deaths in the ET group., Conclusion: ET in neonates with HDFN is associated with an increased risk of sepsis, leukocytopenia, thrombocytopenia, hypocalcemia and hypernatremia., (Copyright © 2012 S. Karger AG, Basel.)

Background: Severe falciparum malaria is associated with considerable rates of mortality, despite the administration of appropriate anti-malarial treatment. Since overall survival is associated with total parasite biomass, blood exchange transfusion has been proposed as a potential method to rapidly reduce peripheral parasitaemia. However, current evidence suggests that this treatment modality may not improve outcome. Automated red blood cell exchange (also referred to as "erythrocytapheresis") has been advocated as an alternative method to rapidly remove parasites from circulating blood without affecting patients' volume and electrolyte status. However, only limited evidence from case reports and case series is available for this adjunctive treatment. This retrospective cohort study describes the use of automated red blood cell exchange for the treatment of severe malaria at the Medical University of Vienna., Methods: Epidemiologic data for imported malaria cases in Austria are reported and data of patients treated for malaria at the General Hospital/Medical University of Vienna were extracted from electronic hospital records., Results: Between 2000 and 2010, 146 patients were hospitalized at the Medical University of Vienna due to malaria and 16 of those were classified as severe malaria cases. Eleven patients of this cohort were potentially eligible for an adjunctive treatment with automated red blood cell exchange. Five patients eventually underwent this procedure within a period of seven hours (range: 3-19 hours) after hospital admission. Six patients did not undergo this adjunctive treatment following the decision of the treating physician. The procedure was well tolerated in all cases and rapid reduction in parasite counts was achieved without occurrence of haemodynamic complications. One patient died within seven days, whereas four patients survived without any sequelae., Discussion and Conclusion: Automated red blood cell exchange was a safe and efficient procedure to rapidly clear peripheral parasitaemia. Whether the fast reduction in parasite biomass may ultimately improve patient survival remains however unclear. Randomized controlled trials are needed to conclusively appreciate the value of this adjunctive treatment.

Exchange transfusion is frequently used as an adjunctive treatment of severe malaria, although the efficacy of exchange transfusion as therapy for severe malaria remains controversial. The major perceived benefit of exchange transfusion is the rapid reduction of parasite load. However, no previous report has shown the dynamic change in parasitemia shortly following an acute load reduction. We report a 20-year-female who developed cerebral malaria and 30% parasitemia after traveling to Africa. In addition to antimalarial treatment, red cell exchange (RCX) was begun emergently with an automated blood-cell separator. Parasitemia dropped from 30 to 15% immediately after the procedure but rapidly increased to 25% after 50 min. The second procedure was performed 12 h after the first procedure. Her neurologic status returned to baseline on Day 2, and she was discharged on Day 6. Rapid increases in parasitemia can be observed after mechanical load reduction following RCX., (Copyright © 2011 Wiley Periodicals, Inc.)

Objectives: Our aim was to assess the efficiency of the peak systolic velocity in the middle cerebral artery (PSV-MCA) to predict neonatal anemia at the end of pregnancies after serial intravenous fetal exchange transfusions (IFET) for red-cell fetomaternal immunization., Patients and Methods: We conducted a retrospective study from 01/01/2004 to 31/12/2009 of 25 pregnancies after IFET for red-cell fetomaternal immunization, in Saint Vincent de Paul Hospital, Paris. The study assessed correlation between the last prenatal PSV-MCA measured and hemoglobin concentration at birth and other neonatal data., Results: Last prenatal PSV-MCA and hemoglobin concentration at birth were significantly correlated (r=-0.39, P<0.01)., Conclusion: There is a good correlation between last PSV-MCA measured before birth and neonatal haemoglobin and complexity of neonatal care linked to anemia. Cerebral Doppler is useful for the follow-up of pregnancies at risk for anemia even in the end of the pregnancy and after serial intravenous fetal exchange transfusions., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Evidence-based indications for the use of plasma products in neonatal medicine are limited to few conditions. In the setting of inherited disorders of hemostasis, fresh frozen plasma (FFP) and cryoprecipitate should be used as replacement therapy only if the specific factor concentrate is not available. FFP is indicated to treat disseminated intravascular coagulation (DIC), liver failure, vitamin K-dependent bleeding and to reconstitute whole blood for exchange transfusion. Despite the lack of evidence, the use of cryoprecipitate to treat neonates with acquired hypofibrinogenemia during DIC or liver failure is now considered standard therapy.

The objective of this study was to examine the use of partial exchange transfusion (PET) performed for polycythemia hyperviscosity syndrome (PHS) over time. A retrospective review of 141 infants who received a PET for PHS at Yale-New Haven Hospital between 1986 and 2007 was performed, querying maternal and neonatal medical records. Patient demographics, risk factors for PHS, indications for PET, and complications associated with PET and PHS were collected. Overall, there was no change in the number of PET performed over the study period ( R(2)=0.082, P=0.192). Eighty-eight percent of patients had at least one risk factor for PHS, most commonly maternal diabetes. Over time, there was a statistically significant decrease in maternal diabetes as a risk factor for PHS. Forty percent of patients had a significant complication attributed to PHS prior to PET. Eighteen percent of patients had a complication attributed to PET. Life-threatening complications of PHS or PET were rare. In conclusion, PHS continues to be a problem observed in neonatal intensive care units, particularly in at-risk populations. PHS and PET are associated with significant complications. Well-designed studies with long-term follow up are needed to assess the risks and benefits of PET for PHS., (© Thieme Medical Publishers.)

Our goal was to determine the indications for exchange transfusion (ECT) and the rates of ECT-related adverse events in neonatal hyperbilirubinemia. We reviewed retrospectively the medical charts of all newborns that had undergone ECT over three years from January 2006 to December 2008. Causes of jaundice, demographic data of the patients, and details of ECT and ECT-related adverse events were recorded. A total of 176 ECT procedures were performed in 150 neonates in the three-year study period. The mean total serum bilirubin before ECT was 29.59 +/- 6.88 mg/dl. Those infants requiring more than one ECT had higher total serum bilirubin than neonates with single ECT, but the difference was not significant (35.66 +/- 12.21 vs. 29.12 +/- 6.30 mg/dl, p = 0.09). The most common cause of ECT was ABO incompatibility (49.3%), Rh disease (7.3%) and idiopathic (28%). Among the adverse events related to ECT, thrombocytopenia (36.4%), hypocalcemia (25.5%), apnea (20%), and infection (10.9%) were noted commonly. No case of ECT-related mortality was observed. All of the adverse events resolved completely before discharge. ABO isoimmunization was the most common cause of ECT in this study. The majority of adverse events associated with ECT are asymptomatic and reversible.

Background: Even in circumstances where optimal antimalarial and supportive treatment is available, severe Plasmodium falciparum malaria is still associated with a significant case fatality. Although exchange transfusion (ET) has been considered as a controversial adjunct therapy, we have not encountered any case fatality since ET was introduced as a standard adjunct therapy for patients with severe malaria., Study Design and Methods: In this retrospective cohort study of 25 patients with severe malaria, the efficacy and safety of ET as an adjunct to parenteral antimalarial treatment (which was implemented in our hospital starting in 1998) were evaluated and compared with 31 historical control patients who were treated with conventional parenteral antimalarial treatment in the period before ET was added to the standard of care for severe malaria (generally before 1997)., Results: The parasite clearance times (PCT)(25%), PCT(50%), PCT(75%) and PCT(90%) were all significantly shorter for patients treated with ET than for patients treated with parenteral quinine only. The shorter PCTs in the ET group were the result of a more rapid parasite clearance in the early phases after initiation of ET., Conclusion: No case fatalities were observed in the ET group. The complications that were observed with ET were more likely related either to the multiorgan dysfunction associated with severe malaria or to side effects of parenteral quinine rather than to the ET procedure. ET may be safely executed in a setting with intensive care facilities and availability of safe blood products and should be considered as a beneficial adjunct treatment to parenteral antimalarial therapy.

Exchange transfusion (ECT) has an important role in preventing kernicterus in the treatment of indirect hyperbilirubinemia of the newborn. In present study, the etiology of hyperbilirubinemia and complications of ECT were studied over a five-year period in the Eastern Mediterranean region of Turkey. We describe our experience of 89 ECTs performed from 2003-2008 in 79 newborns with hyperbilirubinemia. The mean gestational age was 37 +/- 2.1 weeks and the mean of peak total bilirubin levels was 28.1 +/- 6.4 mg/dl. The most common cause of hyperbilirubinemia was ABO isoimmunization (38%). Complications of ECT developed in 17 neonates (21.5%), the most common being thrombocytopenia and seizure. None of newborns died secondary to ECT. Our data showed higher morbidity rates associated with ECT in the treatment of hyperbilirubinemia in our region. In order to prevent adverse effects of ECT, serum bilirubin levels should be closely monitored in newborns with ABO immunization.

Objectives: Acute intravascular hemolysis after exchange transfusion with glucose 6-phosphate dehydrogenase-deficient blood has been reported; however, it is not routine to screen donor blood for glucose 6-phosphate dehydrogenase deficiency while performing exchange transfusion. We hypothesized that exchange transfusion with glucose 6-phosphate dehydrogenase-deficient blood would lead to a less-than-expected decrease in total serum bilirubin. The objective of this study was to evaluate the effect of exchange transfusion with glucose 6-phosphate dehydrogenase-deficient blood in neonates with idiopathic hyperbilirubinemia on postexchange total serum bilirubin levels, duration of phototherapy, and need for repeat exchange transfusions., Methods: All neonates who were undergoing exchange transfusion for idiopathic hyperbilirubinemia were enrolled. A sample of donor blood was collected at the time of exchange transfusion for a glucose 6-phosphate dehydrogenase assay. The standard criteria for starting and stopping phototherapy and exchange transfusion were applied., Results: During the 1-year study period, 21 infants underwent exchange with glucose 6-phosphate dehydrogenase-deficient blood, and 114 neonates with similar baseline characteristics underwent exchange transfusion with glucose 6-phosphate dehydrogenase-normal blood. From 6 to 60 hours after exchange transfusion, there was a significantly lesser drop in total serum bilirubin in the recipients of glucose 6-phosphate dehydrogenase-deficient donor blood compared with recipients of glucose 6-phosphate dehydrogenase-normal blood. The mean duration of phototherapy in the postexchange period and number of infants who underwent repeat exchange transfusions were significantly higher in recipients of glucose 6-phosphate dehydrogenase-deficient donor blood in comparison with control subjects. Concurrently, there was a significantly higher drop in hematocrit and rise in plasma hemoglobin in the glucose 6-phosphate dehydrogenase-deficient donor group., Conclusions: Exchange transfusion with glucose 6-phosphate dehydrogenase-deficient donor blood leads to a lesser drop in postexchange total serum bilirubin. It prolongs the duration of phototherapy and increases the need for repeat exchange transfusions.

Severe unconjugated hyperbilirubinemia, seen mainly in neonates, may cause kernicterus and death. Conventional treatment for severe unconjugated hyperbilirubinemia consists of phototherapy and exchange transfusion. Phototherapy, however, has several known disadvantages while exchange transfusion is associated with a significant morbidity, and even mortality. These harmful effects indicate the need to develop alternative pharmacological treatment strategies for unconjugated hyperbilirubinemia. Generally, these strategies aim to decrease the plasma concentration of unconjugated bilirubin (UCB) by inhibiting production, stimulating hepatic clearance, or interrupting the enterohepatic circulation of the pigment. To be considered for routine clinical use, an alternative treatment strategy should be less invasive and at least as effective and safe as phototherapy. Several pharmacological therapies such as metalloporhyrins, clofibrate, bile salts, laxatives and bilirubin oxidase may meet these criteria in the future, but none of them has yet been evaluated sufficiently to allow routine application. This review aims to discuss the state of the art and future perspectives in pharmacological treatment of neonatal jaundice.