Rate of glucose-6-phosphate dehydrogenase deficiency in neonatal indirect hyperbilirubinemia at a private tertiary centre.

OBJECTIVES: To investigate the rate of hospitalized neonates with glucose-6-phosphate dehydrogenase (G6PD) deficiency presented with indirect hyperbilirubinemia at a private tertiary center in Al-Ahsa, Saudi Arabia, over 4 years and to compare the characteristics of G6PD-deficient and normal neonates admitted for indirect hyperbilirubinemia. METHODS: The retrospective case control study was carried out at Almoosa Specialist Hospital, Al-Ahsa, Saudi Arabia. Data were collected from Yassasi Medical System from 2018-2021 and finalized in 2024. The study included 2 groups: G6PD-normal and G6PD-deficient neonates with indirect hyperbilirubinemia not having recognizable triggers of hemolysis. The analysis focused on serum bilirubin levels, direct bilirubin levels, hematocrit levels, hemoglobin levels, reticulocyte percentage, G6PD levels, duration of phototherapy, and the need for exchange transfusion. RESULTS: The study enrolled 3200 neonates with hyperbilirubinemia, of whom 274 met inclusion criteria. A total of 103 (37.6%) neonates were G6PD-deficient, with 77 (74.8%) being male and 26 (25.2%) female. Glucose-6-phosphate dehydrogenase-deficient neonates exhibited significantly higher initial total bilirubin levels and earlier sampling times. There was no significant correlation between G6PD deficiency and hematocrit or hemoglobin levels in hyperbilirubinemic neonates, but 4 neonates required exchange transfusion, demonstrating statistical significance (p=0.009). CONCLUSION: High rate of G6PD deficiency in neonates with indirect hyperbilirubinemia, requiring close monitoring to prevent exchange transfusions, with no significant differences in hematocrit or hemoglobin levels.

Managing neonatal hyperbilirubinemia: An updated guideline.

ABSTRACT: More than 80% of newborn infants experience jaundice as a result of elevated bilirubin during the first few weeks after birth. In most cases, hyperbilirubinemia is physiologic, but persistent and extreme elevations can lead to serious long-term complications, such as kernicterus. To avoid these complications and help clinicians in the successful assessment, evaluation, and treatment of hyperbilirubinemia, the American Academy of Pediatrics updated its clinical practice guideline for neonatal hyperbilirubinemia. This article reviews the guideline and highlights significant updates, such as an elevation in the threshold for phototherapy and exchange transfusion, inclusion of gestational age, and removal of racially based norms.

Fetomaternal Hemorrhage and Choriocarcinoma: A Case Report.

BACKGROUND: This case describes chronic anemia of a late preterm infant secondary to maternal-fetal hemorrhage and subsequent findings of maternal choriocarcinoma. CLINICAL FINDINGS: This infant was born at 35 6/7 weeks gestational age via cesarean section for non-reassuring fetal heart tones. The mother presented with decreased fetal movement and the biophysical profile was 4/8. Following delivery, the infant did not require respiratory support, was vigorous with extreme pallor, and had a hemoglobin of less than 5 on cord gas. PRIMARY DIAGNOSIS: Chronic anemia secondary to fetomaternal hemorrhage. INTERVENTIONS: The infant's initial hemoglobin was 2.4 and hematocrit was 8.1. The mother's Kleihauer-Betke test was elevated at 7%. The infant required a partial exchange transfusion following admission to the neonatal intensive care unit. Following the partial exchange transfusion, the infant began to experience increasing respiratory distress and required respiratory support. An echocardiogram showed severe persistent pulmonary hypertension of the neonate. The mother was subsequently diagnosed with choriocarcinoma. OUTCOMES: The infant fully recovered from chronic anemia and persistent pulmonary hypertension of the neonate and was discharged home with the mother. The infant required follow-up testing for choriocarcinoma outpatient. PRACTICE RECOMMENDATIONS: Newborns diagnosed with early chronic anemia should be evaluated, the cause investigated, and appropriate treatment considered. If the cause of blood loss is unknown, a maternal Kleihauer-Betke test should be considered. In this case, a partial exchange transfusion was performed to avoid cardiovascular volume overload, but another course of treatment could include small aliquots of packed red blood cell transfusions.

Prophylactic exchange transfusion in sickle cell disease pregnancy: a TAPS2 feasibility randomized controlled trial.

ABSTRACT: Serial prophylactic exchange blood transfusion (SPEBT) is increasingly used in sickle cell disease (SCD) pregnancy, despite a lack of robust evidence. The Transfusion Antenatally in Pregnant Women with Sickle Cell Disease (TAPS2) study assessed the feasibility and acceptability of conducting a definitive randomized controlled trial of SPEBT (intervention) vs standard care (control) in this population. Women aged ≥18 years with SCD, between 6+0 and 18+6 weeks of singleton gestation, were randomized 1:1 every 6 -10 weeks throughout pregnancy in 7 hospitals in England. The main outcomes were recruitment rate (primary outcome), acceptability, and retention. Secondary outcomes were safety and maternal/infant outcomes. In total, 194 women were screened over 42 months (extended because of the pandemic), 88 were eligible, and 35 (39.8%) consented to participate; 18 participants were randomized to intervention, and 17 to control. Follow-up data were collected on all participants. Twelve patients in the intervention group received at least 1 SPEBT, of these, 11 received ≥3. The remaining patient was withdrawn from SPEBT because of transfusion reaction. Sixteen control participants required at least 1 transfusion. There were no statistically significant differences in maternal, infant, and postnatal outcomes. A trend toward a lower incidence of vaso-occlusive crisis, preterm delivery, and improved birthweight was observed in the intervention. The study achieved satisfactory recruitment and retention, confirming its acceptability to participants. TAPS2 demonstrates that it is feasible to perform a definitive international trial of SPEBT in SCD pregnancy. These trials were registered at www.ClinicalTrials.gov as #NCT03975894 and International Standard Randomized Controlled Trial Number (www.isrctn.com; #ISRCTN52684446).

Severe Hemolytic Disease of a Newborn with Bilirubin of 37.3 mg/dL and High anti-A Titer: Corrected with Reconstituted Whole Blood Exchange.

High neonatal bilirubin is a common phenomenon responding to phototherapy. We report a case of a newborn with a highly elevated bilirubin of 37.3 mg/dL due to ABO incompatibility between the mother (Group O) and the newborn (Group A) requiring whole blood exchange, a procedure performed rarely to treat newborn hyperbilirubinemia. The newborn (38.8 weeks of gestation) initially showed a total bilirubin of 8.4 mg/dL and was discharged after being stabilized by phototherapy. However, the baby returned to the hospital with highly elevated bilirubin and was admitted to the Neonatal Intensive Care Unit (NICU). Emergent reconstituted whole blood exchanger therapy was initiated due to refractoriness to phototherapy and IVIG. Markedly elevated anti-A titer was found in the mother's blood (1:512) and cord blood (1:128). The baby was stabilized and eventually discharged with a serum bilirubin of 13.8 mg/dL. This case demonstrates the possible predictive value of mother/cord blood anti-A titers in severe newborn hyperbilirubinemia, which may prevent premature discharge and trigger early initiation of lifesaving therapy.

Exchange Transfusion Trends and Risk Factors for Extreme Neonatal Hyperbilirubinemia over 10 Years in Shiraz, Iran.

Background: Exchange transfusion (ET) is an effective treatment for acute bilirubin encephalopathy and extreme neonatal hyperbilirubinemia (ENH). It can reduce mortality and morbidity. This study aimed to investigate the trends and risk factors of ENH requiring ET in hospitalized neonates in Iran. Methods: A retrospective analysis of medical records of neonates who underwent ET due to ENH was conducted from 2011 to 2021, in Shiraz, Iran. Clinical records were used to gather demographic and laboratory data. The quantitative data were expressed as mean±SD, and qualitative data was presented as frequency and percentage. P<0.05 was considered statistically significant. Results: During the study, 377 ETs were performed for 329 patients. The annual rate of ET decreased by 71.2% during the study period. The most common risk factor of ENH was glucose-6-phosphate dehydrogenase (G6PD) deficiency (35%), followed by prematurity (13.06%), ABO hemolytic disease (7.6%), sepsis (6.4%), Rh hemolytic disease (6.08%), and minor blood group incompatibility (3.34%). In 28.52% of the cases, the cause of ENH was not identified. 17 (5.1%) neonates had acute bilirubin encephalopathy, of whom 6 (35.29%) had G6PD deficiency, 6 (35.29%) had ABO incompatibility, and 2 (11.76%) had Rh incompatibility. Conclusion: Although the rate of ET occurrence has decreased, it seems necessary to consider different risk factors and appropriate guidelines for early identification and management of neonates at risk of ENH should be developed. The findings of the study highlighted the important risk factors of ENH in southern Iran, allowing for the development of appropriate prevention strategies.

Exchange transfusion combined with artesunate (ET-AS) as a safe and effective therapy in severe P. falciparum malaria: a case series.

BACKGROUND: the mortality associated with severe malaria due to Plasmodiun falciparum remains high despite improvements in malaria management. Case prensentation: this case series aims to describe the efficacy and safety of the exchange transfusion combined with artesunate (ET-AS) regimen in severe P. falciparum malaria. Eight patients diagnosed with severe P. falciparum malaria were included. All patients underwent ET using the COBE Spectra system. The aimed for a post-exchange hematocrit of 30%. Half the estimated blood volume was removed and replaced using fresh frozen plasma. The regimen was well-tolerated without complications. The parasite clearance time ranged from 1 ~ 5 days. Five patients with cerebral malaria exhibited full improved consciousness within 3 days, while patient2 with hemolysis improved on day 2. Liver function improved within 1 ~ 6 days, and patient 1 and patient 6 showed improvements renal function on days 18 and 19, respectively. The length of intensive care unit stay range from 2 ~ 10 days, and all patients treated with ET-AS remained in the hospital for 3 ~ 19 days. CONCLUSIONS: these preliminary results suggest that ET-AS regimens are a safe and effective therapy for severe P. falciparum malaria and can benefit patients in clinical settings.

Postexchange transfusion-related acute lung injury in a term neonate.

We report a successful case where a newborn with transfusion-related acute lung injury following an exchange transfusion was effectively treated using conservative methods, eliminating the need for surfactant therapy. Very few instances of this complication have been documented globally. A low birth weight, small for gestational age, term neonate, diagnosed with hyperbilirubinaemia due to Rh incompatibility, experienced sudden respiratory distress in the form of severe retractions, tachypnoea and cyanosis 3 hours after the procedure. Neonate required mechanical ventilation on the grounds of mixed acidosis and diffuse alveolar infiltrates on the chest radiograph. The medical team suspected and treated the baby for transfusion-related acute lung injury through conservative measures. Transfusion-related acute lung injury, an acute life-threatening complication of blood component transfusion, can exhibit symptoms in neonates that are frequently misinterpreted as sepsis. The baby was discharged in good health after successful management after 19 days.

Suppression of Hb Bart's to improve tissue oxygenation and fetal development in homozygous alpha-thalassemia.

Intra-uterine reduction of Hb Bart's only reached with exchange transfusions.

Glucose-6-phosphate dehydrogenase deficiency and neonatal indirect hyperbilirubinemia: a retrospective cohort study among 40,305 consecutively born babies.

BACKGROUND AND OBJECTIVE: Glucose-6-phosphate dehydrogenase deficiency (G6PDD) being highly prevalent in the Middle East, the primary objective was to estimate the incidence of neonatal jaundice among G6PD-deficient neonates and to explore its association with various risk factors. METHODS: This retrospective cohort study includes 7 years data of neonates diagnosed with G6PDD between 1st January 2015, and 30 September 2022, from Al Wakra Hospital, HMC Qatar. RESULTS: Among the 40,305 total births, 1013 had G6PDD with an incidence of 2.51%. Of all the G6PDD babies, 24.6% (249/1013) received phototherapy and three babies required exchange transfusion. Statistically significant associations were noted between the need for phototherapy and gestational age, gestational age groups, birth weight, and birth weight groups, but logistic regression analysis showed significant association for phototherapy only with the gestational age group. CONCLUSION: Universal screening and proper follow-up is essential for G6PDD as it plays crucial role in neonatal jaundice.

Neurodevelopmental outcome at 6 months of age of full-term neonates with hyperbilirubinemia necessitating exchange transfusion.

BACKGROUND: Bilirubin neurotoxicity involves a spectrum of varying severity that could result in adverse long-term sequelae. AIMS: To compare the neurodevelopmental outcome of full-term neonates who underwent exchange transfusion with those who did not. STUDY DESIGN: A retrospective cohort study. SUBJECTS: This study included a retrospective review of records of sixty neonates who were matched in admission ages and serum bilirubin levels and the comparison groups were those who received an exchange transfusion (n = 30) versus those where exchange transfusion was planned, but the bilirubin levels dropped sufficiently during the period where the exchange blood was being prepared (n = 30). History, clinical examination, and laboratory investigations were documented. OUTCOME MEASURES: Neurodevelopmental outcome, at 6 months of age, using Bayley scales of infant development was assessed. RESULTS: The exchange group had statistically significant lower cognitive scores (p-value 0.005). The higher the rate of bilirubin decline, the better the language and motor scores in the phototherapy group (p-values 0.020 and 0.024 respectively). Infants with longer duration to exchange transfusion had lower cognitive, language, and motor scores (p-values 0.01, 0.001, and 0.003 respectively). CONCLUSIONS: Slower rates of bilirubin decline and longer duration before intervention increase the chances of adverse neurodevelopmental outcomes.

Erythrokinetic mechanism(s) causing the "late anemia" of hemolytic disease of the fetus and newborn.

A transfusion-requiring "late anemia" can complicate the management of neonates convalescing from hemolytic disease of the fetus and newborn (HDFN). This anemia can occur in any neonate after HDFN but is particularly prominent in those who received intrauterine transfusions and/or double-volume exchange transfusions. Various reports describe this condition as occurring based on ongoing hemolysis, either due to passive transfer of alloantibody through breast milk or persistence of antibody not removed by exchange transfusion. However, other reports describe this condition as the result of inadequate erythrocyte production. Both hypotheses might have merit, because perhaps; (1) some cases are primarily due to continued hemolysis, (2) others are primarily hypoproductive, and (3) yet others result from a mixture of these two mechanisms. We propose prospective collaborative studies that will resolve this issue by serially quantifying end-tidal carbon monoxide. Doing this will better inform the assessment and treatment of neonates recovering from HDFN.

A Description of IVIG Use in Term Neonates with ABO Incompatibility.

OBJECTIVE: This study aimed to determine if treatment with IVIG of neonates with ABO incompatibility (without Rh incompatibility) results in decreased number of packed red blood cell (pRBC) transfusions and phototherapy use. STUDY DESIGN: An Institutional Review Board (IRB)-approved, single-institution retrospective study was conducted. Neonates ≥38 weeks' gestational age born between January 1, 2007, and December 31, 2016, with ABO incompatibility were included. The comparison among groups was performed using chi-square and Fisher's exact tests for categorical variables; continuous variables were assessed by Kruskal-Wallis test. RESULTS: Six hundred and sixty-eight neonates with ABO incompatibility met inclusion criteria, 579 were included in the analyses. From these, 431 (74%) neonates had positive Direct Antiglobulin Test (DAT); 98 (17%) received IVIG and 352 (61%) received phototherapy. Thirty-six (6%) neonates received pRBC and 6 (1%) required exchange transfusions. Only 3 (0.5%) infants received pRBC transfusions postdischarge, by 3 months of age. Neonates requiring IVIG had lower initial hemoglobin (13.6 vs. 16.0 g/dL, p ≤ 0.0001) and higher bilirubin at start of phototherapy (9.1 vs. 8.1 mg/dL, p = 0.0064). From the 42 (7%) neonates who received simple and exchange transfusions, IVIG use was not associated with decreased use or number of transfusions (p = 0.5148 and 0.3333, respectively). Newborns with A+ and B+ blood types had comparable initial hemoglobin, DAT positivity, APGAR, and bilirubin. However, infants with B+ blood group were more likely (than A + ) to require phototherapy (p < 0.001), receive IVIG (p = 0.003), and need phototherapy for a longer duration (p = 0.001). CONCLUSION: The results of this large retrospective study reveal that giving IVIG to neonates with ABO incompatibility was associated with increased simple or exchange transfusions. Newborns with B+ blood type required more phototherapy and IVIG. Further studies are needed to better stratify neonates who would benefit from IVIG use in order to optimize treatment strategies and avoid unnecessary risks and adverse events. KEY POINTS: · IVIG use not associated with decreased use of pRBC or exchanges.. · Phototherapy duration associated with increased IVIG and pRBC use.. · Newborns with B+ blood type had worse hemolytic anemia..

Massive fetomaternal haemorrhage warranting novel use of tandem isovolumetric partial exchange transfusion and therapeutic hypothermia.

A newborn male infant was pale, hypotonic, and had respiratory distress after delivery. Venous cord blood gas revealed a severe metabolic acidosis. His initial examination was consistent with moderate encephalopathy and laboratory testing uncovered severe congenital anaemia (haematocrit 0.127 L/L). He met the clinical criteria for therapeutic hypothermia (TH) and required red blood cell transfusions, but due to the severity of his anaemia, an exchange transfusion was favoured to prevent transfusion-associated circulatory overload. There are no previous reports of these procedures completed in tandem, but the benefits were perceived to outweigh the risks. During the 72 hours of TH, the infant received an isovolumetric partial exchange transfusion and tolerated both treatments without any adverse clinical events.Kleihauer-Betke testing detected a massive chronic fetomaternal haemorrhage with 475 mL (164 mL/kg) of blood. A brain MRI completed prior to discharge was normal. At 6 months of age, he is growing and developing normally.

Current status of neonatal jaundice management in Japan.

BACKGROUND: This nationwide survey aimed to determine the status of jaundice management in Japan. METHODS: A questionnaire about bilirubin level measurements and neonatal jaundice treatment was sent to 330 institutions providing neonatal care. The responses were analyzed according to institution level. RESULTS: Of 330 institutions, 172 responded (52.1% response rate). Total bilirubin levels were measured in the central laboratory using spectrophotometry at 134 institutions and a blood gas analyzer at 81 institutions. Unbound bilirubin (UB) levels were measured by 79 institutions, while transcutaneous bilirubin measurements were taken at 63 institutions. There was no association between institution level and UB or transcutaneous bilirubin measurement. For phototherapy criteria, the Murata-Imura criteria were adopted by 67 institutions, Nakamura criteria by 36, and Morioka criteria by 39. Light-emitting diodes (LED) were used by 160 institutions versus fluorescent lights by 31. When a blue LED was used, 119 institutions used the high mode. There is no standard for increasing light intensity. No association was found between institution level and phototherapy criteria. UB was measured in 14 of 63 institutions using the Murata-Imura criteria. CONCLUSIONS: There is a large variation in the management and treatment of neonatal jaundice among institutes in Japan.

ABO-incompatible orthotopic heart transplant: a case report☠.

BACKGROUND: ABOi heart transplant has become routine for the majority of children <2 years old. An 8-month-old child with complex congenital heart disease presented to the Medical University of South Carolina Shawn Jenkins Children's Hospital in need of transplantation. METHODS: This case report describes the use of ABOi transplantation and describes the details of the total exchange transfusion prior to cardiopulmonary bypass. RESULTS: After a successful intraoperative total exchange transfusion following the ABOi protocol, the patient's isohemagglutinin titers were 1 VC on postoperative day (POD) 1, and isohemagglutinin titer was <1 VC on POD 14. The patient had no signs of rejection and continued to recover. CONCLUSIONS: Successful ABOi transplantation requires planning, an interdisciplinary approach, and clear closed-loop communication. Planning with the surgical and anesthesia teams is necessary for the hemodynamic stability of the patient during the total volume exchange as well as precautions put in place to ensure the blood products used in this procedure are correct. Planning with the lab and blood bank is also necessary to ensure they are prepared with enough blood products and can run isohemagglutinin titers.

Preparation and exchange transfusion effect of a double polymerization human umbilical cord haemoglobin of red blood cell substitute.

The development of haemoglobin-based oxygen carrier (HBOC) is an excellent supplement to pre-hospital emergency blood transfusions. In this study, a new type of HBOC was prepared by using human cord haemoglobin (HCHb) and glutaraldehyde (GDA) and Bis(3,5-dibromosalicyl) fumarate (DBBF) to modify (DBBF-GDA-HCHb), the changes of physicochemical indexes during its preparation were evaluated, while a traditional type of GDA-HCHb was prepared, and the oxygen-carrying capacity of two type of HBOC was evaluated by a rat model of 135.0% exchange transfusion (ET). Eighteen SD male rats were selected, and were randomly divided into control group (5.0% albumin), DBBF-GDA-HCHb group and GDA-HCHb group. The 12 h survival rate of the C group was 16.67%, and the two HBOC groups were both 83.33%. Compared with GDA-HCHb, DBBF-GDA-HCHb can reduce lactic acid content by supplying oxygen to hypoxic tissues in a more timely manner, and can also can improve the reduction of MAP due to ischaemia.