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Filtered-sunlight phototherapy (FSPT) that blocks ultraviolet light and reduces infrared radiation is safe and non-inferior to intensive electric phototherapy (IEPT) for treating mild-to-severe neonatal hyperbilirubinemia. In this randomized non-inferiority trial, the safety, efficacy, exchange transfusion (ET), and mortality rates of FSPT versus IEPT among Nigerian neonates with severe-to-hazardous hyperbilirubinemia were investigated. Safety was defined as the absence of hyperthermia, hypothermia, dehydration, or sunburn; efficacy by the proportion of assessable treatment days during which total serum or plasma bilirubin (TSB) increased by < 0.2 mg/dL/hr for newborns aged ≤ 72 h-old or decreased for newborns > 72 h-old. A treatment day was deemed assessable if a neonate received phototherapy for ≥ 4 h, and non-inferiority was inferred for differences within a 10% margin. We enrolled 192 newborns (admission TSB ≤ 62 mg/dL), assigned to FSPT (n = 98) or IEPT (n = 94). FSPT was effective on 94.2% of the assessable treatment days compared with 97.1% for IEPT. The mean difference in efficacy between FSPT and IEPT was -2.9%, 95% CI: -7.6, 1.9). 2.6% of newborns who received FSPT developed controlled hyperthermia, and no baby met the criteria for withdrawal for safety reasons. Overall, 50.6% (39/77) of newborns who received FSPT and 53.7% (51/95) of newborns who received IEPT had ET (p = 0.89) and 7 in each group (9.1% vs 7.4%; p = 0.86) died. In conclusion, FSPT is safe and non-inferior to IEPT for treating neonates with severe-to-hazardous hyperbilirubinemia, it is not associated with significantly higher rates of ET and mortality and should be considered where practicable when IEPT cannot be assured. Clinical Trials.gov Number: NCT02612727 (24/11/2015)., (© 2024. The Author(s).)

Introduction: There are few reports of severe hematological involvement in children with neonatal lupus erythematosus (NLE) treated with exchange transfusion. In this case report, we present a female patient with NLE admitted to the Children's Hospital of Soochow University. The main clinical manifestations were pancytopenia and congenital heart block (CHB). Her condition was serious and could not be improved by conventional treatment; however, she responded well to exchange transfusion therapy., Case Presentation: A female infant, aged 1 month and 3 days, was admitted to the Children's Hospital of Soochow University owing to the "discovery of thrombocytopenia over 1 month." She tested positive for anti-SSA IgG, anti-Ro-52 IgG, and anti-mitochondrial M2 antibodies. In contrast, her mother tested positive for ANA (1:320) and anti-Ro/SSA antibodies. The patient was diagnosed with NLE and presented with pancytopenia and CHB. Her cardiac function was normal and no intervention was performed; however, her hematological involvement was more severe, without significant improvement after steroid, intravenous immunoglobulin, and transfusion treatments. After exchange transfusion therapy, the patient significantly improved, and the short-term follow-up prognosis was good., Conclusion: For patients with NLE presenting with hematological involvement that cannot be improved by conventional treatment or whose condition is serious, exchange transfusion therapy should be considered to reduce antibody titers and improve their condition., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Zhou, Sun, Peng and Zhu.)

Objective: To assess the utility of jaundice surveillance and routine 24 hour bilirubin screening in identifying neonates who qualify for phototherapy (PT) at ≤24 hours after birth., Study Design: In this retrospective, single-center observational study, records of neonates ≥35 0/7  weeks gestation born to O+, antibody negative mothers (n = 6098) were screened to identify who received PT at ≤24 hours after birth. The hour specific TSB at which neonates qualified for PT, blood type, direct antiglobulin test (DAT), and whether treatment was triggered by jaundice detection at <24 hours or the 24-hour bilirubin screen were determined., Results: 59 neonates (1.0%) qualified for PT ≤ 24 hours after birth; 10 (17%) were identified by jaundice detection at <24 hours, whereas 49 (83%) were identified on 24-hour bilirubin screening. Forty-eight of the 59 (81%) were ABO incompatible and DAT+; 11 were DAT negative, one of whom had glucose-6-phosphate dehydrogenase deficiency. Among the ≤24 hour PT group, 17 had a PT qualifying TSB within 3 mg/dL of exchange transfusion (ET); 14 of whom were only identified first on 24-hour bilirubin screening. Six exceeded ET thresholds, 4 of whom were identified on 24-hour bilirubin screening., Conclusions: Neonates who qualified for PT at ≤24 hours were identified mostly by 24-hour bilirubin screening, a fraction of whom had a TSB that approached or exceeded ET thresholds. Our findings support routine birth hospitalization bilirubin screening and suggest screening no later than 24 hours after birth may be beneficial., Competing Interests: Declaration of Competing Interest Dr. Watchko disclosed a financial relationship with McGraw-Hill. The other authors have no conflict of interest relevant to this article to disclose., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: The objectives were to evaluate the descriptive features of newborns with a diagnosis of Rhesus (Rh) hemolytic disease, to determine the morbidity and mortality rates, to evaluate the treatment methods and the factors affecting treatment requirements and clinical outcomes during a ten-year period at a tertiary center., Methods: Newborn infants who had a positive direct Coombs test and/or had a history of intrauterine transfusion (IUT) due to Rh hemolytic disease were included. The data regarding the prenatal, natal and postnatal periods were collected from hospital records., Results: A total of 260 neonates were included of which 51.2% were female. The mean ± standard deviation gestational age was 36.9 ± 2.7 weeks. The rate of preterm birth was 41.2%. Of 257 mothers whose obstetric medical history could be accessed, 87.2% were multigravida, whereas 76.3% were multiparous. Among mothers who had a reliable history of anti-D immunoglobulin prophylaxis (n=191), 51.3% had not received anti-D immunoglobulin prophylaxis in their previous pregnancies. The antenatal transfusion rate was 31.7% and the frequency of hydrops fetalis was 8.8%. While combined exchange transfusion (ET) and phototherapy (PT) was performed in 15.4% of the babies, the majority either needed phototherapy only (51.1%) or no treatment (33.5%). The mortality rate was 3.8 % (n = 10), and nine babies out of these 10 were those with severe hydrops fetalis., Conclusion: This study showed that Rh hemolytic disease is still a major problem in developing countries. Multiple comorbidities may occur in addition to life threatening complications, including hydrops fetalis, anemia and severe hyperbilirubinemia. High rates of multiparity and low rates of anti-D immunoglobulin prophylaxis are potential barriers for the eradication of the disease. It should be remembered that Rh hemolytic disease is a preventable disease in the presence of appropriate antenatal follow-up and care facilities., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Taiwan Pediatric Association. Published by Elsevier B.V. All rights reserved.)

Objectives: To identify indications for exchange transfusions, assess the use and waste of exchange transfusion products (ie, reconstituted whole blood exchange transfusions), and determine nationwide distribution and prevalence of these transfusions in the Netherlands., Study Design: All 9 neonatal intensive care units and 15 non-neonatal intensive care unit hospitals participated in this retrospective, observational, cohort study. We retrieved data on the indications for and use of all exchange transfusion products ordered by participating centers over an 11-year period., Results: A total of 574 patients for whom 1265 products were ordered were included for analyses. Severe ABO (32.6%) and non-ABO (25.2%) immune hemolysis and subsequent hyperbilirubinemia were the most frequent indications. Rare indications were severe leukocytosis in Bordetella pertussis (2.1%) and severe anemia (1.5%). Approximately one-half of all ordered products remained unused. In 278 of 574 neonates (48.4%), ≥1 products were not used, of which 229 (82.7%) were due to the resolving of severe hyperbilirubinemia with further intensification of phototherapy. The overall prevalence of neonates who received an exchange transfusion was 14.6:100 000 liveborn neonates., Conclusions: A considerable proportion of products remained unused, and annually a limited number of patients are treated with an exchange transfusion in the Netherlands, highlighting the rarity of the procedure in the Netherlands., Competing Interests: Declaration of Competing Interest This study is researcher initiated and not externally funded. No funds, grants, or other support were received. D.P.d.W. received funding from Momenta Pharmaceuticals, Inc., which was acquired by Johnson & Johnson, and is an investigator for a phase 2 trial (NCT03842189) of a new drug for the treatment of HDFN. E.J.T.V. is the principal investigator for a phase 2 trial (NCT03842189) and a phase 3 trial (NCT05912517) of a new drug for the treatment of HDFN, which is sponsored by Janssen Pharmaceuticals. E.L. is a sub-investigator for a phase 2 trial (NCT03842189) of a new drug for the treatment of HDFN, which is sponsored by Janssen Pharmaceuticals. All other authors reported to have no conflict of interest or financial disclosures., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Hemolytic disease of the fetus and newborn (HDFN) is caused by maternal antibodies attacking fetal blood cell antigens. Despite routine antenatal anti-D prophylaxis, intrauterine transfusions (IUTs) are still needed in some HDFN cases. Methods: We conducted a retrospective cohort study on newborns with HDFN born in the 1st Department of Obstetrics and Gynecology of the Medical University of Warsaw. We analyzed 274 neonates with HDFN, identifying 46 who required IUT due to fetal anemia and 228 who did not. The laboratory results, management, and outcomes were compared between these groups. Results: Comparative analysis showed that newborns treated with IUT were more likely to have significant anemia, hyperbilirubinemia, and iron overload, indicated by a high ferritin concentration. These neonates more often required top-up transfusions, phototherapy, intravenous immunoglobulin infusions, and exchange transfusions. The length of stay was longer for newborns who received IUT. Conclusions: HDFN requiring IUT is associated with a greater number of complications in the neonatal period and more often requires additional treatment compared to HDFN not requiring IUT.

Background: The data to support chronic automated red cell exchange (RCE) in sickle cell disease (SCD) outside of stroke prevention, is limited, especially in adults., Study Design and Methods: A retrospective analysis was conducted of patients with SCD who were referred for chronic RCE at our institution over a 10-year period. Data that were evaluated included patient demographics, referral indications, and procedural details (e.g., vascular access, adverse events, etc.). In a subanalysis, the number of annual acute care encounters during 3 years of chronic RCE was compared with that in the year preceding the first RCE., Results: A total of 164 patients were referred for chronic RCE: median age was 28 years (interquartile range [IQR] = 22-36) at referral and 60% were female. Seventy (42.6%) were naïve to chronic transfusion (simple or RCE) prior to referral. The leading indications for referral were refractory pain (73/164, 44.5%) and iron overload (57/164, 34.7%). A total of 5090 procedures occurred during the study period (median = 19, IQR = 5-45). Of the 138 patients who had central vascular access, 8 (6%) and 16 (12%) had ≥1 central-line-related thrombosis and/or infection, respectively. Of those who were not RBC alloimmunized at initiation of RCE, 12/105 (11.4%) developed new antibodies during chronic RCE. In those 30 patients who were adherent to therapy for 3 years, there was no significant difference in acute care encounters following initiation of RCE., Conclusion: Prospective clinical trials are needed to determine which patients are most likely to benefit from chronic RCE and refine selection accordingly., (© 2024 AABB.)

Background: Exchange transfusion due to hyperbilirubinemia is performed in neonates with signs of encephalopathy or if the level of bilirubin is more than the exchange threshold and not responding to intensive phototherapy. Bilirubin passage through the blood-brain barrier can cause injury to different sites of the brain and may have long-life effects. In this study, we aimed to evaluate the neonates who underwent exchange transfusion and investigated their developmental problems. By recognizing their developmental delay, we can recommend screening time and early occupational therapy if needed., Methods and Material: This is a retrospective study on neonates who underwent exchange transfusion due to hyperbilirubinemia in Namazi and Hafez hospitals, in Shiraz, Iran, between 2016 and 2021. The exclusion criteria were the unwillingness of the parents to participate in the study or incomplete data. Children who died were also excluded from the study. Demographic and clinical data were obtained from hospital records. Children were invited to the clinic for examination, and development was assessed by Ages and Stages Questionnaires (ASQ). All neonates had done auditory brainstem response. The result was obtained. Quantitative data are reported as mean standard deviation (SD) and qualitative data with frequency and percentage. Spearman's correlation coefficient and Chi-square test were used, and the P value was significant below 0.05., Results: Eighty-seven neonates were enrolled. Forty-nine (56.3%) were female, and 38 (43.7%) were male. Glucose-6-phosphate dehydrogenase(G6PD) deficiency was the most prevalent hematologic cause of hyperbilirubinemia (23%). Auditory disorder, speech disorder, motor disorder, and encephalopathy were seen in four (4.6%), two (2.3%), three (3.4%), and four infants (4.6%), respectively., Conclusion: Bilirubin neurotoxicity can cause developmental impairment including auditory, speech, and motor disorders besides encephalopathy. Early recognition and proper early intervention can lead to better outcomes for the child, family, and society., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Journal of Education and Health Promotion.)

Babesiosis is a potentially life-threatening tick-borne parasitic infection. Severe disease in splenectomized individuals may require exchange transfusion. A 58-year-old male with a history of splenectomy presented with 2 weeks of subjective fever, weakness, and abdominal pain. He denied any rashes, tick bites, or recent travel. He had a motor vehicle accident a few years ago and had undergone an emergency splenectomy. On examination, the patient was febrile (39.3 °C), tachycardic (106/min), and jaundiced. Labs revealed anemia and thrombocytopenia. Computed tomography (CT) abdomen revealed asplenia. As it was summer, there was concern for a tick-borne illness. A peripheral smear showed schistocytes, and labs revealed hyperbilirubinemia, high lactate dehydrogenase (LDH), low haptoglobin, and reticulocytosis (13%), consistent with hemolysis. Testing for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Ehrlichia, Borrelia, Anaplasma , and viral hepatitis was negative. Antibody testing for Babesia microti was positive. A blood parasite smear confirmed Babesia microti with a parasitemia of 9.5%. The patient received intravenous azithromycin and atovaquone for severe babesiosis. On day 2 of hospitalization, parasitemia increased to 14.7%. Hemoglobin and platelets dropped further on day 3. His parasite load remained consistently above 10% despite medical treatment. A decision was made for a red blood cell (RBC) exchange transfusion for severe disease, which was performed on the fourth day of hospitalization. Clinical improvement was seen after one session of exchange RBC transfusion. Hemoglobin remained stable, and thrombocytopenia improved 1 day after RBC exchange transfusion. Parasitemia dropped to 1.2% after 4 days of exchange transfusion, and azithromycin was switched to oral. He received 9 days of inpatient azithromycin and atovaquone. He was discharged with a plan to continue the oral antimicrobials for 3 more weeks. Asplenia and parasitemia > 10% are associated with severe babesiosis. Asplenia, in particular, is associated with severe infection, hospitalization, and prolonged duration of therapy. Exchange transfusion in severe babesiosis can be lifesaving., Competing Interests: The authors declare no conflict of interest., (Copyright 2024, Gondal et al.)

Vaso-occlusive crisis is a sequela of sickle cell disease that can lead to severe pain and infarction at the location of occlusion. In men, genitourinary complications include priapism, hematuria, and very rarely, testicular infarction. Few cases have been previously reported in the literature, but in all of those cases, partial or complete orchiectomy was performed. We report the first known case of segmental testicular infarct secondary to sickle cell disease treated with medical management without need for surgical intervention., (© 2024 The Authors.)

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., (© 2024. The Author(s).)

Introduction: Unconjugated hyperbilirubinemia is part of the everyday life of the neonatal period as it reflects the adaptation of the metabolism of bilirubin. The neonatal hyperbilirubinemia usually resolves spontaneously, but it can also be the cause of an acute or chronic encephalopathy known as kernicterus. Regardless of the cause, the goal of therapy is to prevent this neurotoxicity while not causing undue harm. Phototherapy and, if it is unsuccessful, exchange transfusion (ECT) remain the primary treatment modalities used to keep the maximal total serum bilirubin (TSB) below pathologic levels., Materials and Methods: This is a descriptive retrospective cohort study of 69 live neonates hospitalized in the Department of Neonatology and Neonatal Resuscitation of Mohammed VI University Hospital with unconjugated hyperbilirubinemia requiring ECT and treated with intensive phototherapy instead, spanning five years from March 2016 to March 2021. We aim to demonstrate the effectiveness of phototherapy in achieving prolonged reduction of bilirubin levels and the prevention of neurological complications and to compare our results with those in the literature., Results: The use of intensive phototherapy in the treatment of neonatal unconjugated hyperbilirubinemia is very effective in lowering total serum bilirubin when its level is in the range of exchange transfusion, and it has succeeded in preventing the neurological complications of severe hyperbilirubinemia., Conclusion: Through this study, it can be seen that phototherapy is an efficacious, simpler, and less hazardous alternative to exchange transfusion in achieving a sustained reduction of bilirubin levels and preventing neurological complications., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Ouerradi et al.)

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., Competing Interests: Declaration of competing interest The authors declare that they have no competing interests., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Background and Objectives: Management of severe allergic transfusion reactions (ATR) is challenging. In this study, we investigate the usefulness of skin tests and basophil activation tests (BAT) in chronically transfused patients for the prevention of future ATR., Materials and Methods: BAT and skin tests were carried with the supernatant of red blood cell (RBC) units for a sickle-cell disease patient under chronic exchange transfusion who has presented a severe ATR, in order to prevent potential future ATR. If the results for both BAT and skin tests were negative, the RBC units could be transfused to the patient. If either one of the results was positive, the tested RBC unit was discarded for the patient., Results: 192 RBC units were tested with both tests. The level of results concordance between the two tests was 95%. Out of the 169 negative units with both tests, 118 units were transfused to the patient for which he presented no ATR., Conclusion: In our study, combining both BAT and skin tests was associated with a good negative predictive value since we were able to safely transfuse our patient. Further studies are still necessary to confirm this result but this pilot study indicates that skin tests and BAT might help prevent ATR. When BAT is not available, skin tests may also be useful in preventing ATR., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (© 2024 Akiki, Dedeken, Ferster, Doyen, Dupire, Nagant, Smet, Ghorra, Ruth, Lauwers, Daubie, Corazza and El Kenz.)

Background: Neonates affected by isoimmune hemolytic disease (HDN) are at risk of developing severe hyperbilirubinemia. Studies show that increasing levels of bilirubin impact neonatal neurodevelopment. To avoid complications associated with exchange transfusion, intravenous immunoglobulin G (IVIG) is used to treat hyperbilirubinemia. We included all infants who received more than two doses of IVIG treatment for isoimmune hemolytic disease. We analyzed the incidence of side effects associated with IVIG treatment and the rate of exchange transfusion., Methods: A retrospective chart review performed between October 2011-October 2022 at East Carolina University Health identified neonates who received more than two doses IVIG for HDN. Neonates of postmenstrual age greater than 28 days old, receiving less than three doses of IVIG or received IVIG for other indications were excluded. The occurrences of adverse events, demographics and use of other medical therapies were reviewed., Results: Eleven neonates were included in the case series. Most common cause of severe hyperbilirubinemia was attributed to ABO incompatibility. Six patients (54%) received three doses of IVIG, and five patients (45%) received four doses of IVIG with bilirubin levels decreasing below exchange transfusion. No treatment exceeding four doses of IVIG was reported, nor adverse events during treatment., Conclusions: In this cohort of neonates with HDN, bilirubin levels decreased after treatment with multiple doses of IVIG. Future research on recommendations of optimal total number doses of IVIG to reduce the risk for exchange transfusion.

Background: Red blood cell (RBC) transfusion remains a major treatment for sickle cell disease (SCD). Patients with SCD have a high prevalence of renal impairment and cardiorespiratory disease, conferring risk of transfusion-associated circulatory overload (TACO)., Study Design and Methods: We describe an approach, titled euvolemic automated transfusion (EAT), to transfuse SCD patients with severe anemia who are at risk of TACO. In EAT, plasmapheresis is performed using donor RBCs, rather than albumin or plasma, as replacement fluid. Euvolemia is maintained. A retrospective analysis was conducted of patients with SCD who underwent EAT at our institution over a 10-year period, to evaluate the efficacy and safety of EAT., Results: Eleven SCD patients underwent 109 EAT procedures (1-59 procedures per patient). The median age was 42 years (IQR = [30-49]) and 82% (n = 9) were female. Most (82%; n = 9) patients had severe chronic kidney disease and 55% (n = 6) had heart failure. One (9%) patient had a history of life-threatening TACO. Mean pre- and post-procedure Hct values were 19.8% (SD ± 1.6%) and 29.1% (SD ± 1.4%), respectively. The average Hct increment was 3.2% per RBC unit. Only two EAT-related complications were recorded during the 109 procedures: central line-associated infection and citrate toxicity (muscle cramping). EAT used an average of two RBC units less than that projected for standard automated RBC exchange., Conclusion: Our findings suggest that EAT is safe and effective to treat patients with SCD and severe anemia, who are at risk for TACO. EAT requires fewer RBC units compared to automated RBC exchange., (© 2023 AABB.)

Sickle cell disease (SCD) is one of the most common hematological diseases, which results in variable complications. The treatment of SCD is evolving but limited options are available for now. Acute chest syndrome (ACS) is one of the serious complications observed in SCD and a challenging one in prevention. Crizanlizumab is a monoclonal antibody that binds to P-selectin and improves blood flow by preventing sickle cell adhesion to endothelium, resulting in improvement of vaso-oclusive crises (VOC). It is not well evaluated in terms of ACS prevention. Here we report a 23-year-old patient with SCD and recurrent ACS; she was started on Crizanlizumab and she had no more ACS, but once she was off Crizanlizumab she developed ACS again, later Crizanlizumab was re-started, and the patient has improved significantly.

Pediatric chronic myeloid leukemia (CML) is a very rare malignancy (age-related incidence 0.1/100,000) typically presenting with leucocyte counts >100,000/µL. However, clinical signs of leukostasis are observed at diagnosis in only approximately 10% of all cases and among these, priapism is infrequent. Here, we analyze data from pediatric CML registries on the occurrence of priapism heralding diagnosis of CML in 16/491 (3.2%) boys (median age 13.5 years, range 4-18) with pediatric CML. In the cohort investigated, duration of priapism resulting in a diagnosis of CML was not reported in 5 patients, and in the remaining 11 patients, occurred as stuttering priapism over 3 months ( n = 1), over 6 weeks ( n = 1), over 1-2 weeks ( n = 2), over several days ( n = 2), or 24 h ( n = 1), while the remaining 4 boys reported continuous erection lasting over 11-12 h. All patients exhibited splenomegaly and massive leukocytosis (median WBC 470,000/µL, range 236,700-899,000). Interventions to treat priapism were unknown in 5 patients, and in the remaining cohort, comprised intravenous fluids ± heparin ( n = 2), penile puncture ( n = 5) ± injection of sympathomimetics ( n = 4) ± intracavernous shunt operation ( n = 1) paralleled by leukocyte-reductive measures. Management without penile puncture by leukapheresis or exchange transfusion was performed in 3 boys. In total, 7 out 15 (47%) long-term survivors (median age 20 years, range 19-25) responded to a questionnaire. All had maintained full erectile function; however, 5/7 had presented with stuttering priapism while in the remaining 2 patients priapism had lasted <12 h until intervention. At its extreme, low-flow priapism lasting for longer than 24 h may result in partial or total impotence by erectile dysfunction. This physical disability can exert a large psychological impact on patients' lives. In a narrative review fashion, we analyzed the literature on priapism in boys with CML which is by categorization stuttering or persisting as mostly painful, ischemic (low-flow) priapism. Details on the pathophysiology are discussed on the background of the different blood rheology of hyperleukocytosis in acute and chronic leukemias. In addition to the data collected, instructive case vignettes demonstrate the diagnostic and treatment approaches and the outcome of boys presenting with priapism. An algorithm for management of priapism in a stepwise fashion is presented. All approaches must be performed in parallel with cytoreductive treatment of leukostasis in CML which comprises leukapheresis and exchange transfusions ± cytotoxic chemotherapy.

Hemolytic disease of the newborn is commonly diagnosed and managed by pediatric and newborn hospitalists. Severe cases, however, pose unique challenges for community hospitals without higher level neonatal intensive care units. This case highlights the challenges faced by pediatric hospitalists in the community and suggests a focused approach to management.

Introduction: Leukapheresis reduces hyperleukocytosis in children with acute leukemia. Although the usefulness of this procedure is under debate, a repeated small-volume exchange transfusion along with leukapheresis yielded satisfactory results., Methods: Forty-seven patients with acute leukemia [32 acute lymphocytic leukemia (ALL) and 15 acute myeloblastic leukemia (AML)] were enrolled between January 2017 and June 2022 and underwent repeated small-volume exchange transfusion. The following were measured: demographic and clinical characteristics, time of the procedure, PWBC (peripheral white blood cell) count, hemoglobin, platelet count, blood biochemistry, electrolytes, coagulation, leukostasis, TLS (tumor lysis syndrome), DIC (disseminated intravascular coagulopathy), adverse events (AEs), and serious AEs (SAEs)., Results: The demographic and clinical characteristics were not significantly different between ALL and AML patients, but differences were observed in PWBC counts (424.2 ± 135.6 vs. 223.8 ± 58.0 × 109/L). The procedures needed 3-8 processes, and the average procedure time was not significantly different between ALL and AML. The PWBC count gradually reduced to <100 × 109/L; hemoglobin, platelet count, K+, Na+, and Ca2+ were unchanged. Alanine aminotransferase, aspartate aminotransferase, total bilirubin, blood urea nitrogen, creatinine, troponin-I, creatine kinase-MB, prothrombin time, and activated partial thromboplastin time maintained normal or recovered from abnormal ranges. The manifestations of leukostasis, TLS, and DIC improved or disappeared. No AEs and SAEs occurred. The required total blood volume was based on initial PWBC count, manifestations of leukostasis, and age., Conclusions: Our finding suggests that repeated small-volume exchange transfusion is effective and safe for treating hyperleukocytosis in children with acute leukemia., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Zhang, Lin and Huang.)

Background: Neonatal jaundice is one of the most common physiologic problems requiring medical attention in newborns. It is benign in most cases; however, high levels of bilirubin are neurotoxic and can lead to serious brain damage., Objectives: This study aimed at assessment of magnitude of neonatal jaundice in cases of neonatal hyperbilirubinemia admitted into neonatal intensive care unit (NICU), Cairo University Pediatric Hospital and to detect possible etiologies, management and outcome., Methods: The present work is a retrospective study, included 789 neonates suffered from hyperbilirubinemia over a two-year period., Results: Intensive phototherapy and exchange transfusion were used together in 6 cases. Two hundreds and twenty-two cases (28.1%) had exchange transfusion once, 44 cases had it twice, 6 cases had it 3 times and one case had it 4 times. Number of exchange transfusion significantly affects mortality among cases (P= 0.02)., Conclusion: Neonatal hyperbilirubinemia is an existing problem in our NICU. Intensive phototherapy is an excellent substitute for exchange transfusion. Respiratory distress and sepsis are significantly higher among dead cases. Screening for risk factors is needed to avoid critical hyperbilirubenemia., Competing Interests: The authors have no conflicts of interest relevant to this article., (© 2023 Ghobrial EE et al.)

Red blood cells circulating through the brain are briefly but closely apposed to the capillary endothelium. We hypothesized that this contact provides a nearly direct pathway for metabolic substrate transfer to neural cells that complements the better characterized plasma to endothelium transfer. While brain function is considered independent of normal fluctuations in blood glucose concentration, this is not borne out by persons with glucose transporter I (GLUT1) deficiency (G1D). In them, encephalopathy is often ameliorated by meal or carbohydrate administration, and this enabled us to test our hypothesis: Since red blood cells contain glucose, and since the red cells of G1D individuals are also deficient in GLUT1, replacing them with normal donor cells via exchange transfusion could augment erythrocyte to neural cell glucose transport via mass action in the setting of unaltered erythrocyte count or plasma glucose abundance. This motivated us to perform red blood cell exchange in 3 G1D persons. There were rapid, favorable and unprecedented changes in cognitive, electroencephalographic and quality-of-life measures. The hypothesized transfer mechanism was further substantiated by in vitro measurement of direct erythrocyte to endothelial cell glucose flux. The results also indicate that the adult intellect is capable of significant enhancement without deliberate practice.      ClinicalTrials.gov registration: NCT04137692 https://clinicaltrials.gov/ct2/show/NCT04137692.

Since the discovery of the Rh blood group system in 1940, a greater understanding of hemolytic disease of the fetus and newborn (HDFN) was gained. In the years thereafter, researchers and clinicians came to the current understanding that fetal and neonatal red blood cells (RBC) are hemolyzed by maternal alloantibodies directed against RBC antigens potentially leading to severe disease. Preventative measures, such as Rhesus(D) immunoprophylaxis (RhIG), have greatly decreased the prevalence of Rh(D)-mediated HDFN, although a gap between high-income countries and middle- to low-income countries was created largely due to a lack in availability and high costs of RhIG. Other important developments in the past decades have improved the identification, monitoring, and care of pregnancies, fetuses, and neonates with HDFN. Prenatally, fetal anemia may occur and intrauterine transfusions may be needed. Postnatally, pediatricians should be aware of the (antenatally determined) risk of hemolysis in RBC alloimmunization and should provide treatment for hyperbilirubinemia in the early phase and monitor for anemia in the late phase of the disease. Through this review, we aim to provide an overview of important historic events and to provide hands-on guidelines for the delivery and postnatal management of neonates with HDFN. Secondarily, we aim to describe recent scientific findings and evidence gaps., Conclusion:  Multiple developments have improved the identification, monitoring, and care of pregnancies and neonates with HDFN throughout the centuries. Pediatricians should be aware of the (antenatally determined) risk of hemolysis in RBC alloimmunization and should provide treatment for hyperbilirubinemia in the early phase and monitor for late anemia in the late phase of the disease. Future studies should be set in an international setting and ultimately aim to eradicate HDFN on a global scale., What Is Known: • Developments have led to a greater understanding of the pathophysiology, an improved serological identification and monitoring of at-risk cases and the current pre- and postnatal treatment., What Is New: • This review provides the pediatrician with hands-on guidelines for the delivery and postnatal management of neonates with HDFN. • Future studies should be set in an international setting with the ultimate aim of eradicating HDFN., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Intravenous immunoglobulin G (IVIG) is used to treat blood-type incompatibility hemolytic disease of newborns (BTHDN). Although IVIG's efficacy for treating BTHDN has been challenged, as an updated systematic review suggests, IVIG could significantly reduce exchange transfusions. We conducted a mail-in questionnaire survey to ascertain actual use of IVIG for BTHDN in Japan., Methods: The survey, conducted in 2014, included infants born between January 1, 2009, and December 31, 2013. Questionnaires were sent to the heads of neonatal intensive care units (NICUs) at perinatal centers of the Japan Neonatologist Association., Results: A total of 195 centers (64.6%) responded to the questionnaire. During the study period, 170 centers (87.2%) reported incidences of BTHDN. Among these centers, there were 1726 diagnosed cases of BTHDN in neonates. Of these cases, 419 infants were treated with IVIG in 127 centers, representing approximately 74.7% of all centers. After the exclusion of cases with missing data and those where consent for data usage was not obtained, a total 916 infants were included in this study. Of these, 219 (23.9%) were treated with IVIG after phototherapy, and 187 (20.4%) of these infants did not require further blood exchange transfusion. The IVIG dosages ranged from 40 to 1200 mg/kg/dose, but the majority were between 500 and 1000 mg/kg/dose, with a median of 800 mg/kg/dose. About 20% of the infants treated with IVIG showed late-onset anemia and required treatment. Adverse events were reported in less than 1% of infants., Conclusions: For the treatment of BTHDN, IVIG administration was widely used in NICUs in Japan without severe adverse events., (© 2023 Japan Pediatric Society.)

Background: Red cell transfusion remains the gold standard in managing sickle cell disease (SCD) with severe complications. Offering red blood cell exchange (RBCX) either manual exchange transfusion (MET) or automated RBCX (aRBCX) can reduce the complications of chronic transfusion and maintain target Hb thresholds. This study audits the hospital experience of overseeing adult SCD patients treated with RBCX, both automated and manual, and compares the safety and efficacy., Materials and Methods: This retrospective observational study was conducted as an audit for chronic RBCX for adult patients with SCD in 2015-2019 at King Saud University Medical City, Riyadh, Saudi Arabia., Results: A total of 344 RBCX for 20 adult SCD patients who were enrolled in regular RBCX, (11/20) patients had regular aRBCX with a total of (157) sessions, and (9/20) patients had MET with a total of (187) sessions. The median level of HbS% post-aRBCX was significantly lower than MET (24.5.9% vs. 47.3%, P < 0.010). Patients on aRBCX had fewer sessions (5 vs. 7.5, P < 0.067) with better disease control. Although the median yearly pRBC units per patient for aRBCX was more than the double needed for MET (28.64 vs. 13.39, P < 0.010), the median ferritin level was 42 μg/L in aRBCX versus 983.7 μg/L in MET, P < 0.012., Conclusion: Compared to MET, aRBCX was more effective in reducing HbS, with fewer hospital visits and better disease control. Although more pRBCs were transfused, the ferritin level was better controlled in the aRBCX group without increasing alloimmunization risk., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Asian Journal of Transfusion Science.)

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., (© 2023 Japan Pediatric Society.)

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: The primary objective was to explore perinatal and neonatal outcomes amongst infants who received intrauterine transfusion (IUT) for the management of hemolytic disease of the fetus and newborn (HDFN). The secondary objective was to evaluate the role of key investigations in the fetus at risk of HDFN and assess the relationship with neonatal outcomes. We hypothesized that middle cerebral artery peak systolic velocity (MCA-PSV) and corresponding multiples of the median (MoM) would be predictive of neonatal course., Methods: This was a retrospective observational study conducted at a tertiary center in the United Kingdom between January 2000 and August 2020. Trust approval was obtained to conduct this service review. Pregnancies requiring IUT for HDFN were identified using the fetal medicine department database. Inclusion criteria were infants who received IUT for HDFN. 67 pregnancies were eligible for inclusion in the study with 156 IUT events. Data were extracted using healthcare records. Statistical analysis was performed using SPSS version 28.0, data were assessed for normality and Spearman's correlation analysis was performed with p values < .05 considered significant., Results: 67 pregnancies were included in the study which led to the live birth of 68 infants (one twin pregnancy). There were no fetal deaths following IUT. There was one neonatal death due to extreme prematurity following spontaneous vaginal delivery at 23 + 4 weeks gestation, occurring three days following IUT. 97% of infants required admission to the neonatal intensive care unit and 88% required phototherapy. 25% of infants required readmission for red blood cell transfusion due to anemia. There was a significant correlation between maternal anti-D antibody levels and length of neonatal admission r  = 0.477, p  = .014. MCA-PSV and MoM measured prior to the last IUT had a significant positive correlation with the duration of phototherapy: r  = 0.527 ( p  < .001) and r  = 0.313 ( p  < .05) respectively. Linear regression analysis demonstrated a significant positive relationship between MCA-PSV and corresponding MoM recorded prior to the last IUT with r 2 = 0.177 ( p  = .003) and r 2 = 0.101 ( p  = .029)., Conclusion: HDFN is an important cause of fetal anemia associated with significant neonatal morbidity. MCA-PSV and MoM may be predictive of neonatal phototherapy requirements. The predictive value of MCA-PSV appears to be dependent on the timing of measurement during the antenatal period and more research is needed. Multicentre collaboration is required to generate a reliable large-scale database to further delineate the value of MCA-PSV and MoM and predict neonatal outcomes in cases of HDFN requiring IUT. This data would assist clinicians in antenatal planning and enable more informed counseling of parents in the antenatal period.

Purpose: The study aimed to evaluate the application of Red Blood Cell Distribution Width (RDW) and Platelet Distribution Width (PDW) in the treatment of neonatal exchange transfusion (ET) with hyperbilirubinemia as well as to provide relevant reference materials for clinical diagnosis and treatment., Patients and Methods: This was a retrospective study in a single center. Between January 2011 and December 2020, a total of 198 neonates, who were admitted to Fujian Maternity and Child Health Hospital for hyperbilirubinemia and treated with ET therapy were selected. They were divided into blood group antibody negative (BGAbN) ET ( n  = 92) and blood group antibody positive (BGAbP) ET ( n  = 106) groups. We analyzed changes in serum total bilirubin (STB), serum indirect bilirubin (SIB), and platelet count(PLT) before and after ET; The clinical data of the neonates with hyperbilirubinemia were collected, and RDW and PDW were compared in the two groups before ET.., Results: The concentrations of STB, SIB, and platelet count were much higher before ET and decreased significantly after ET; the difference was statistically significant ( p <.001); There were significant differences between the two groups in RDW and PDW before ET., Conclusion: ET therapy is the most timely and effective treatment method for severe hyperbilirubinemia in neonates clinically; RDW and PDW can help determine neonatal hemolysis caused by blood group antibodies.

Background: Automated red cell exchange (RCE) is a common treatment for patients with sickle cell disease (SCD). Two key parameters are used to determine the volume of blood for RCE to reduce sickle hemoglobin (eg, HbS): fraction of cells remaining (FCR) and target hematocrit. We evaluated how the calculated FCR-using the manufacturer's algorithm-impacted blood utilization and incidence of acute care encounters., Study Design and Methods: Retrospective chart review was conducted of 15 adults with SCD who underwent chronic RCE from July 1, 2015 to August 31, 2019. Blood utilization and acute care encounters were compared across three time periods: (a) when a fixed FCR of 30% was used (12 months); (b) transition period during which physicians made ad hoc changes to the FCR (25 months); (c) algorithm phase when a procedural FCR between 30% and 50% was selected using an algorithm generated by the manufacturer's built-in software to target a HbS fraction of 8% post-procedure (12 months). Wilcoxon signed rank test was used to determine statistical significance., Results: Median blood utilization per procedure decreased from 2398 mL (interquartile range [IQR]: 2271-2759 mL) during the fixed FCR phase to 1887 mL (IQR: 1495-2241 mL) during the algorithm phase (P < 0.001). Similarly, median number of units transfused decreased from 10 (9-11) to 7 (5-9) during the respective phases (P < 0.001). Visits to the emergency department were 1 (0-4) in the fixed FCR phase and 0 (0-3) in the algorithm phase., Conclusion: Algorithm-based selection of a procedural FCR significantly reduced blood utilization (~21%) without appearing to increase acute care encounters., (© 2022 Wiley Periodicals LLC.)

Introduction: Exchange transfusion (ECT) is one of effective treatments for rapid reduction of the bilirubin serum levels. The main purpose of this study was to offer greater insights into the effects of ECT on the hematologic factors and bilirubin in neonatal hyperbilirubinemia., Methods: This cross-sectional study was performed on 380 neonates over 35 gestational weeks, and 2-14 days old with a bilirubin of above 17 mg/dl who had undergone ECT at Ghaem Hospital of Mashhad in Iran from 2011 to 2021. Blood samples were examined before, immediately after, 6 h and 60 h after ECT for complete blood cell count (CBC), platelet count and bilirubin serum level analysis., Results: In this study, the mean age of neonates was 5.21 ± 3.55 days with a mean birth weight of 2810 ± 710 gr. The mean platelet count (PLT), white blood cell (WBC) and the serum level of bilirubin were estimated at 260,000/mm 2 , 12,400/mm 2 , 23 mg/dl before ECT and 97,000/mm 2 , 7370//mm 2 and 12.6 mg/dl immediately after ECT, respectively (P-value <0.001)., Conclusion: The results indicated that the mean serum levels of bilirubin, platelets, and leukocytes dropped to 55%, 30%, and 60% of their baseline levels before ECT, respectively, but they all spiked after ECT., Competing Interests: Conflicts of interest All authors declared there is no relevant interest with whole aspects of study., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

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).)

Human babesiosis is an emerging infectious disease with a progressively rising number of cases in the Northeast over the last few decades. We report a case of fatal babesiosis in a 48-year-old male without significant risk factors for a severe presentation. Clinicians should be aware that even in patients without the classic risk factors of asplenia, advanced age, and immunocompromised status for severe presentations of babesiosis, a deadly case can present. There is a need for further research regarding optimal treatment options for severe babesiosis considering the questionable efficacy of red blood cell exchange (RCE) transfusion in patients who do not improve on the current first-line antimicrobials.

Background: Alloimmune hemolytic disease of the newborn (AIHDN) results in hemolysis, anemia, hyperbilirubinemia with the potential for brain damage. Intravenous immunoglobulin (IVIG) has been investigated as an alternative low-risk procedure for the treatment of AIHDN in addition to traditional treatment methods such as phototherapy and exchange transfusion (ET)., Aim: To evaluate the effectiveness of IVIG therapy in decreasing ET needs based on risk factors and clinical outcomes., Materials and Methods: Charts of neonates born >30 weeks of gestation who underwent phototherapy and were administered IVIG therapy due to AIHDN between January 2013 and July 2018 were retrospectively reviewed., Results: Sixty-three neonates were included in our study. Forty-three of them (68.3) % were full-term infants. ABO incompatibility (n = 33, 52.4%) was the major cause of AIHDN (n = 63). Additional risk factors for jaundice were found to coexist in 95.2% (n = 60) of the infants. Fifteen infants (23.8%) required ET, mostly due to Rh incompatibility (n = 11, 73.3%). Mortality was observed in 3.2% (n = 2) of the patients, 1.6% (n = 1) of whom were related to ET. Serum albumin value was found to be negatively correlated with the requirement for ET (r = 0.713, P < 0.001), whereas serum bilirubin albumin ratio was positively correlated (r = 0.489, &#95;P < 0.001). Nine (14.3%) infants needed a simple transfusion during the hospitalization period, whereas five (7.9%) infants had readmission for simple transfusion after discharge. Apnea was the only complication seen in one (1.6%) patient., Conclusion: IVIG treatment should be considered due to its relative benefits when compared to exchange transfusion. In addition to its safety, it is a less complicated treatment modality with low side effect rates. It may be justified for elective use in neonates suffering from AIHDN, who will require ET with a risk of mortality by decreasing the peak of total serum bilirubin levels., Competing Interests: None

To study the prevalence of direct antiglobulin test (DAT) positivity and outcomes in babies with ABO incompatibility and the role of DAT in predicting need for phototherapy. This was an analytical retrospective cohort study conducted in a tertiary care hospital in South India. DAT was performed routinely in cord blood of all neonates born to O positive mothers. The case records of neonates ≥ 35 weeks of gestation and birth weight > 2000 gm with ABO setting born between January 2019 and December 2020 were reviewed and outcomes were analyzed. Among 2484 live births, there were 426 eligible ABO incompatible births and DAT was positive in 68(15.9%). DAT positive neonates had significantly higher peak mean bilirubin [16.02 mg/dL vs. 13.68 mg/dL, p  = 0.00], need for phototherapy in first 24 h [23(33.8%) vs. 32(8.9%), p  = 0.00], need for exchange transfusion [2(2.9%) vs. 0(0%), p  = 0.025] and immunoglobulin [6(8.8%) vs. 0(0%), p  = 0.00]. Cord DAT showed 29.9% sensitivity, 96.9% specificity, 89.7% positive predictive value and 60.1% negative predictive value for predicting need for phototherapy. DAT positivity occurs in a significant proportion of ABO incompatible births and a "selective" cord blood DAT in neonates born to O positive mothers can identify those neonates at risk of significant hyperbilirubinemia with high diagnostic accuracy., Competing Interests: Conflict of interestThe authors have no conflicts of interest to disclose., (© Indian Society of Hematology and Blood Transfusion 2021.)

Introduction: Exchange transfusion is the treatment of choice for patients with severe hyperbilirubinemia who do not respond to phototherapy. This procedure is highly complex and requires substantial expertise to perform, however it´s not done frequently enough to guarantee adequate training. Traditional learning scenarios do not have a space reserved for teaching this procedure or an instrument that fully and objectively evaluates the skills that a professional must acquire.  OBJECTIVES: The purpose was to construct and evaluate the INEXTUS instrument´s validity evidence relevant to internal structure, in a simulated scenario through the performance of an objective structured clinical exam (OSCE).  MATERIALS AND METHODS: The Delphi consensus methodology was utilized to design the instrument; six experts participated through three rounds using the Google Forms platform. The categories and items previously obtained were subjected to validation by nine experts through a dichotomous survey. Prior to data collection, the evaluators were trained through a pilot test with 10 medical students. Subsequently, all residents of a paediatric programme were evaluated through the OSCE methodology in a simulated scenario, with 6 stations, of a clinical case of a new-born with an explicit need for exchange transfusion. During their participation in the scenario, the residents were first evaluated with the instrument developed. Additionally, audio and video filming of all students who participated was performed with the aim of conducting a second evaluation two weeks after the first four evaluators participated., Results: The final INEXTUS instrument consists of 46 subitems grouped into 23 items divided into 6 categories, demonstrating an inter-rater intraclass correlation coefficient of 0.96 (95% CI 0.94, 0.98 p-value < 0.001). For the Fleiss Kappa of the 23 items evaluated, concordance was evaluated for 14 items but could not be determined for the 9 remaining items because all the ratings were equal, either because the items were not performed or they were all performed adequately. Of the 14 items, 9 good scores were obtained (95% CI 0.61 to 0.8; p value < 0.001), and 5 very good scores were obtained (95% CI 0.81 to 1; p value < 0.001)., Conclusions: The INEXTUS instrument evaluates exchange transfusion skills in medical personnel in training in simulated scenarios using the OSCE methodology; it has high validity and reliability and is a high-impact educational tool., (© 2022. The Author(s).)

Gestational alloimmune liver disease (GALD) is a materno-fetal alloimmune disorder that targets the fetal liver and often causes neonatal liver failure. GALD most commonly presents as neonatal hemochromatosis (NH), which is a severe neonatal liver injury confirmed by extra-hepatic iron accumulation at various sites. With the discovery of the alloimmune mechanism of GALD, exchange transfusion and intravenous immunoglobulin (IVIG) administration are being used as novel treatments. Here, we present a rare case of an 11-day-old female infant who presented with marked hyperbilirubinemia. Laboratory findings showed significantly elevated direct and indirect bilirubin, high ferritin and alpha fetoprotein levels, high transferrin saturation, and severe coagulopathy. Abdominal magnetic resonance imaging revealed markedly reduced T2 signal intensity in the liver and pancreas compared to the spleen, suggesting iron deposition. The infant was diagnosed with NH and successfully treated with exchange transfusion and four doses of IVIG., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Introduction: Intravenous immunoglobulin (IVIG) has been widely used to treat the hemolytic disease of the newborn (HDN). Although it has been shown that IVIG treatment reduces the duration of phototherapy and hospitalization, the use of IVIG in hemolytic disease due to ABO incompatibility has been controversial in recent years. This study aimed to investigate the role of IVIG in the prevention of exchange transfusion in infants with ABO HDN who presented with bilirubin levels at or above the level of exchange transfusion., Materials and Methods: This study evaluated the data of infants with ABO HDN in the Turkish Neonatal Jaundice Online Registry. The infants with ABO HDN who met the total serum bilirubin level inclusion criteria (within 2-3 mg/dL of exchange transfusion or even above exchange transfusion level) were included in the study according to the guidelines from the American Academy of Pediatrics and the Turkish Neonatal Society. All patients were managed according to the unit protocols recommended by these guidelines and received light-emitting diode (LED) phototherapy. Infants who only received LED phototherapy, and who received one dose of IVIG with LED phototherapy were compared., Results: During the study period, 531 term infants were included in the study according to inclusion criteria. There were 408 cases in the phototherapy-only group, and 123 cases in the IVIG group. The demographic findings and the mean bilirubin and reticulocyte levels at admission were similar between the groups ( p > 0.05), whereas the mean hemoglobin level was slightly lower in the IVIG group ( p = 0.037). The mean age at admission was earlier, the need for exchange transfusion was higher, and the duration of phototherapy was longer in the IVIG group ( p < 0.001, p = 0.001, and p < 0.001, respectively). The rate of re-hospitalization and acute bilirubin encephalopathy (ABE) was higher in the IVIG group ( p < 0.001 and p = 0.01, respectively)., Conclusion: In this study, we determined that one dose of IVIG did not prevent an exchange transfusion nor decrease the duration of phototherapy in infants, who had bilirubin levels near or at exchange transfusion level, with hemolytic disease due to ABO incompatibility., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Okulu, Erdeve, Kilic, Olukman, Calkavur, Buyukkale, Cetinkaya, Ulubas, Demirel, Hanta, Ertugrul, Gultekin, Tuncer, Demir, Bilgin, Narli, Yildiz, Terek, Koroglu, Seren, Ozyazici, Ozdemir, Turgut, Narter, Akin, Ozyazici, Zenciroglu, Asker, Gokmen, Salihli, Bulbul, Zubarioglu, Atasay, Koc and Turkish Neonatal Society IVIG Study Group.)

Background: Chronic red blood cell transfusions reduce acute care utilization for sickle cell disease (SCD) pain. However, little is known about whether chronic transfusions treat or prevent the development of non-crisis pain. We investigated patient-report of pain in adults with SCD receiving chronic exchange transfusions (CET) compared to adults not on CET with similar disease characteristics., Study Method and Design: Eleven participants receiving chronic exchange transfusion (CET) for at least one year were compared to 33 participants not receiving CET. Participants completed validated patient-reported outcomes regarding pain impact and quality of life at regularly scheduled visits or before CET. One year of health care utilization and opioid prescriptions were examined., Results: After 1:1 propensity matching was performed for age, genotype, WBC and neutrophil counts, patients on CET had lower Pain Impact scores (-5.1, p = 0.03) and higher Neuropathic (7.4, p < 0.001) and Nociceptive Pain Quality (3.7, p < 0.001) scores, all indicating worse pain. However, CET was associated with a reduction in annual all cause admissions (-3.1, p < 0.001), length of stay (-2.1 days, p < 0.001) and ED visits (-2.7, p < 0.001). CET was not associated with differences in opioids dispensed., Conclusions: After adjusting for disease characteristics, CET was associated with worse pain impact and neuropathic and nociceptive pain quality, lower health care utilization and with similar levels of opioids dispensed. This data suggest that CET may reduce hospitalizations for acute pain but may not adequately treat nociceptive or neuropathic pain in SCD., (Copyright © 2021 Elsevier Ltd. All rights reserved.)