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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). [ABSTRACT FROM AUTHOR]

Extreme levels of bilirubin in newborn is a major cause of lifelong neurodevelopmental impairment, which places a financial burden on healthcare resources and caregivers. To determine the incidence, aetiology and short-term outcomes of extreme hyperbilirubinaemia in term infants born in a resource-limited setting. This is a retrospective observational study looking at term neonates with a birth weight ≥2500 g, born in the Western health subdistrict of Cape Town, South Africa, between 1 January 2019 and 31 December 2020, who were exposed to a serum bilirubin level of ≥430 μmol/L in the first week of life and received care in the public health system. Extreme hyperbilirubinaemia occurred in 59 term infants. The incidence was 74 cases per 100 000 (<0.01%) live births equating to 1 case in every 1345 live births. The cause of hyperbilirubinaemia was identified in 51 of the cases (86%), the most common being ABO incompatibility (31/51, 61%), followed by glucose-6-phosphate dehydrogenase deficiency (11/51, 22%). Twelve infants (20 %) underwent an exchange transfusion. Six infants were encephalopathic. Forty-seven infants (80%) were readmitted after initial post-natal discharge, with a mean age of readmission of 113 h old (SD 31 h). The incidence of extreme hyperbilirubinaemia in the Western health subdistrict of Cape Town is higher than in high-income settings. Further work should focus on training of healthcare workers and education of caregivers, for the early detection of significant hyperbilirubinaemia to prevent neurological complications caused by bilirubin toxicity. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

Abstract 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 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).

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.

background: Neonatal jaundice may be caused by physiological events and/or as specific disease conditions, while the clinical course may be related to the aetiology. Objective: To determine the spectrum of identifiable causes as well as the clinical course of jaundice among hospitalized babies. Methods: Babies hospitalized with jaundice requiring therapy in a tertiary hospital were prospectively studied for the aetiology and the pattern of total serum bilirubin (TSB). Babies with unexplained jaundice were compared with babies in whom the aetiology was known. Results: Jaundice was unexplained in 51 (20.6 percent) of 248 babies aged 0 to 240 hours, while 50 (20.2 percent) had various combinations of prematurity, blood group incompatibilities, Glucose-6-phosphate dehydrogenase (G6PD) deficiency and septicaemia. Babies with G6PD deficiency had the highest mean peak TSB (26.3 + 9.0mg/dl), those with Rhesus incompatibility had their peak TSB earliest (5.7 + 0.8 days), while the mean duration of phototherapy was longest in babies whose jaundice was due to prematurity (10.4 + 0.9 days). Exchange blood transfusion (EBT) rate was highest among babies with Rhesus incompatibility (100.0 percent) and G6PD deficiency (70.3 percent). Preterm infants tended to have multiple EBT sessions compared to term infants (p < 0.001). Bilirubin encephalopathy was most frequent among babies with G6PD deficiency (62.1 percent). Conclusion: There appears to be a pattern of clinical events which are peculiar to the specific aetiology of newborn jaundice.

Background: Neonatal hyperbilirubinemia maybe complicated by severe neurologic disability including hearing impairment (HI). There is no agreement on the precise bilirubin level that may cause auditory affection. Aim of the work: to study the frequency of HI in neonates with indirect hyperbilirubinemia threshold for exchange transfusion (ET). Patients and Methods: This cross-sectional analytical study enrolled full-term neonates whose bilirubin levels were at the ET threshold managed by intensive phototherapy (IP) or ET. Automated auditory brainstem response (AABR) was performed within 24 hours of admission using audiometer among both groups. Results: This study included 98 full-term neonates. Forty-eight (49 %) were males and 50 (51%) were females. Their mean gestational age was 37.78±0.74 weeks. Forty-six (46.9%) neonates underwent ET while 52 (53%) improved on IP with the rapid decline of their bilirubin levels and did not undergo ET. The mean IP group bilirubin-induced neurologic dysfunction (BIND) score was 0, and reticulocyte count was 7.12±6.49% compared to 1.89±2.16 and 11.47±9.82% of ET group (p=0.001) and (p=0.004) respectively. Abnormal AABR was detected in 23 (23.5%) neonates; 8 had unilateral hearing impairment while 15 had bilateral hearing impairment. IP was associated with less HI in both ears in 3 (5.7%) compared to 12 (26.1%) who underwent ET (p=0.005).Neonates with any HI had higher total serum bilirubin (TSB) levels (p= 0.002) and BIND scores (p= 0.002). IP was associated with less (one or both ears) HI in 8 (15.3%) neonates compared to 15 (32.6%) who underwent ET (p=0.045). A TSB cut-off value of 24.5 mg/dl showed a sensitivity of 60.9% and a specificity of 81.3% while a BIND score cut-off value of ≥ 2 showed a sensitivity of 47.8% and a specificity of 86.7% for detecting any hearing impairment. Males had a higher risk for bilateral hearing impairment (p= 0.040). Conclusion: Neonates with higher bilirubin levels and BIND scores are at risk for HI. TSB and BIND score have modest sensitivity and specificity in predicting HI. IP is mandatory in all those with ET threshold bilirubin until procurement of blood to prevent HI. HI may occur despite normal BIND scores in the absence of bilirubin encephalopathy. Those who responded to IP for ET level of TSB had less frequency of HI. Males were more at risk for bilateral HI. Screening for HI among neonates who sustain hyperbilirubinemia is a necessity.

Abstract 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.

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. [ABSTRACT FROM AUTHOR]

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. See editorial on page 1385–1387, in this issue [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

IntroductionThere 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 presentationA 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.ConclusionFor 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.

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.

Peri-operative management of cyanotic congenital heart disease in a patient of sickle cell disease (SCD) can be challenging. We report a case of Tetralogy of Fallot and homozygous SCD with history of multiple blood transfusions and sickle cell crises who underwent intracardiac repair. Hemoglobin S level was reduced from 75% pre-operative to 21.8% postoperative with a combination of pre-operative blood transfusion, intraoperative exchange transfusion, and normothermic cardiopulmonary bypass (CPB). Pre-operative optimization and safe intraoperative conduct were essential to avoid sickling crises.

Methemoglobinemia is a potentially life-threatening, rare condition in which the oxygen-carrying capacity of hemoglobin is diminished. We present the case of a 3-year-old boy treated for T-cell acute lymphoblastic leukemia (T-ALL) who developed methemoglobinemia (MetHb 57.1%) as a side effect of ifosfamide administration. Due to his critical condition, the patient was transferred to the intensive care unit (ICU). The therapy included methylene blue administration, an exchange transfusion, catecholamine infusion, and steroids. Improving the general condition allowed for continuing chemotherapy without ifosfamide and completion of the HR2 block. Vigilance for methemoglobinemia as a very rare side effect should be widespread when using ifosfamide in the treatment protocols. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: To assess the prevalence of exchange transfusion requirement and identify risk factors associated with exchange transfusion in neonates with unconjugated hyperbilirubinemia in a tertiary care hospital. Study Design: Comparative Cross-sectional. Setting: Department of Neonatology, Holy Family Hospital Rawalpindi. Period: January 2022 to December 2022. Methods: A total of 136 neonates, from 28 weeks of gestation to 28 days of life after birth, with severe pathological hyperbilirubinemia, unconjugated hyperbilirubinemia and or who had signs of kernicterus were included through non-probability consecutive sampling. Patients were then divided in to two groups based on exchange transfusion received or not. SPSS version 26 was used for data entry and analysis, and descriptive and inferential statistics were applied. Results: A total of 136 neonates were recruited, out of which 57% (n=78) were male and 43% (n=58) were female. The average gestational age was 36.6 ± 1.9 weeks. 111 (82%) neonates were term, while 25 (18%) were preterm. The average age of the study population was 6.0±3.3 days, with a mean weight of 2.7 ± 0.7 kg. The average STB level was 20.8 ± 5.5 mg/dl. 64.7% (n=88) required exchange transfusion were belonged to group-A, while 35.3 %(n=48) without exchange transfusion were in group-B. The burden of disease was calculated to be 64.7%. Polycythemia, ABO and Rh incompatibility were identified as significant risk factors for ET (p<0.05). Conclusion: The burden of disease was 64.7%. Exchange transfusion was required in the majority of neonates with unconjugated hyperbilirubinemia. The identified risk factors for the requirement of exchange transfusion in neonates were polycythemia, ABO and Rh incompatibility. [ABSTRACT FROM AUTHOR]

Peri-operative management of cyanotic congenital heart disease in a patient of sickle cell disease (SCD) can be challenging. We report a case of Tetralogy of Fallot and homozygous SCD with history of multiple blood transfusions and sickle cell crises who underwent intracardiac repair. Hemoglobin S level was reduced from 75% pre-operative to 21.8% postoperative with a combination of pre-operative blood transfusion, intraoperative exchange transfusion, and normothermic cardiopulmonary bypass (CPB). Pre-operative optimization and safe intraoperative conduct were essential to avoid sickling crises. [ABSTRACT FROM AUTHOR]

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Objectives Critical hyperbilirubinemia in preterm neonates, a condition requiring greater attention, is treated with phototherapy or exchange transfusion when bilirubin results exceed gestational age and age-specific medical decision levels (MDLs) to prevent bilirubin-induced neurologic damage. Conventional evaluation involves multiple manual steps and is poised to inconsistencies and delays. Methods We designed and implemented an electronic clinical decision support (CDS) tool to identify and alert neonatal intensive care unit clinicians of critical hyperbilirubinemia with a SmartZone alert. We evaluated the performance of our manual evaluation workflow, the accuracy of the electronic CDS tool, and the outcome of the electronic CDS tool to reduce the time to place orders for interventions. Results Among the 22 patients who met the criteria to have phototherapy ordered before implementing the electronic CDS tool, 20 (90%) had phototherapy ordered. Fourteen (70%) phototherapy orders were placed less than 24 hours, 4 phototherapy orders were placed 24 to 72 hours, and 2 orders were placed more than 72 hours after bilirubin results exceeded the corresponding MDLs. Among the 15 patients who met the criteria to have phototherapy ordered after implementing the electronic CDS tool, all (100%) received phototherapy orders, with 14 (93%) placed less than 24 hours and 1 order placed less than 48 hours. The electronic CDS tool identified all eligible patients correctly. The proportion of phototherapy ordered less than 24 hours increased from 70% to 93% after the implementation of the electronic CDS tool. Conclusions The electronic CDS tool promoted more appropriate and timely intervention orders to manage critical hyperbilirubinemia in preterm neonates. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background Acute lymphoblastic leukemia (ALL) is the most common childhood cancer. Emergency complications such as hyperleukocytosis can arise as the disease develops. Exchange transfusion is one of many cytoreductive modalities to resolve the condition. Objective To analyze the clinical and laboratory effects of exchange transfusion in childhood acute lymphoblastic leukemia patients with hyperleukocytosis. Methods This analytical retrospective cohort study with a pre- and post-test design used secondary medical record data. The obtained characteristics were the incidences of dyspnea, tachypnea, headache, intracranial bleeding, hepatomegaly, splenomegaly, lymphadenopathy, abdominal pain, fever, pallor, nausea/vomiting, and skin or mucosal bleeding; hemoglobin, white blood cell, and platelet counts; and serum potassium, sodium, calcium, phosphate, blood urea nitrogen, and creatinine levels. Results A total of 16 patients underwent exchange transfusion; they had significant reductions of the incidence of dyspnea, tachypnea, hepatomegaly, and pallor and significant improvement in the form of elevation of hemoglobin level and decrease in white blood cell counts (P

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.

Background and objectivesManagement 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 methodsBAT 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.Results192 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.ConclusionIn 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.

Background Acute lymphoblastic leukemia (ALL) is the most common childhood cancer. Emergency complications such as hyperleukocytosis can arise as the disease develops. Exchange transfusion is one of many cytoreductive modalities to resolve the condition. Objective To analyze the clinical and laboratory effects of exchange transfusion in childhood acute lymphoblastic leukemia patients with hyperleukocytosis. Methods This analytical retrospective cohort study with a preand post-test design used secondary medical record data. The obtained characteristics were the incidences of dyspnea, tachypnea, headache, intracranial bleeding, hepatomegaly, splenomegaly, lymphadenopathy, abdominal pain, fever, pallor, nausea/vomiting, and skin or mucosal bleeding; hemoglobin, white blood cell, and platelet counts; and serum potassium, sodium, calcium, phosphate, blood urea nitrogen, and creatinine levels. Results A total of 16 patients underwent exchange transfusion; they had significant reductions of the incidence of dyspnea, tachypnea, hepatomegaly, and pallor and significant improvement in the form of elevation of hemoglobin level and decrease in white blood cell counts (P<0.05 for all) compared to pre-treatment. The remainder of the variables were not significantly different between pre- and post-treatment, but a general decrease in all clinical manifestations except intracranial bleeding was observed. Conclusion Exchange transfusion has the beneficial effect of resolving hyperleukocytosis and its manifestations, although correction in laboratory outcomes that remained abnormal are still needed. [ABSTRACT FROM AUTHOR]

Transfusion-related acute lung injury (TRALI), a life-threatening condition, remains an under reported complication, especially among neonates postexchange transfusion for unconjugated hyperbilirubinemia. Appropriate recognition and prompt treatment change the prognosis for good. The present case emphasizes that TRALI must be kept as a differential diagnosis in previously well neonates presenting with sudden onset hypoxia within or during 6 h of transfusion with evidence of bilateral infiltrates on a frontal chest radiograph, and no evidence of circulatory overload, left atrial hypertension, or pre-existing respiratory distress before transfusion. We report the rare occurrence of TRALI and its successful management with supportive hemodynamic management and corrective ventilator strategies.

Methemoglobinemia is a potentially life-threatening, rare condition in which the oxygen-carrying capacity of hemoglobin is diminished. We present the case of a 3-year-old boy treated for T-cell acute lymphoblastic leukemia (T-ALL) who developed methemoglobinemia (MetHb 57.1%) as a side effect of ifosfamide administration. Due to his critical condition, the patient was transferred to the intensive care unit (ICU). The therapy included methylene blue administration, an exchange transfusion, catecholamine infusion, and steroids. Improving the general condition allowed for continuing chemotherapy without ifosfamide and completion of the HR2 block. Vigilance for methemoglobinemia as a very rare side effect should be widespread when using ifosfamide in the treatment protocols.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.

Abstract 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.

Transfusion-related acute lung injury (TRALI), a life-threatening condition, remains an under reported complication, especially among neonates postexchange transfusion for unconjugated hyperbilirubinemia. Appropriate recognition and prompt treatment change the prognosis for good. The present case emphasizes that TRALI must be kept as a differential diagnosis in previously well neonates presenting with sudden onset hypoxia within or during 6 h of transfusion with evidence of bilateral infiltrates on a frontal chest radiograph, and no evidence of circulatory overload, left atrial hypertension, or pre-existing respiratory distress before transfusion. We report the rare occurrence of TRALI and its successful management with supportive hemodynamic management and corrective ventilator strategies. [ABSTRACT FROM AUTHOR]

Polycythemia is defined as a central venous hematocrit level of more than 65%. Polycythemia occurs because of increased red cell mass, with decreased, normal, or increased plasma volume. On the other hand, hyperviscosity of blood results in increased resistance to blood flow and decreased oxygen delivery. Both polycythemia and hyperviscosity can cause central nervous system dysfunction, hypoglycemia, impaired renal function, and cardiorespiratory distress. Hyperviscosity has also been reported to be associated with long-term neurodevelopmental disorders in childhood. Polycythemia and hyperviscosity are related to alterations in organ perfusion. There is a decrease in end-organ blood perfusion due to changes in red cell mass, arterial oxygen content, and/or viscosity. There are two main treatment approaches to neonatal polycythemia. The first approach is restrictive management, whereas the second approach is partial exchange transfusion, a more controversial one. Partial exchange transfusion is a procedure in which the blood of the infant is diluted. Various studies have had outcomes suggesting no clinically significant short and long-term benefits of the partial exchange transfusion, especially in asymptomatic infants and infants with minor symptoms. However, it is crucial to note that there are no long-term follow-up studies to evaluate the neurodevelopmental status of infants with neonatal polycythemia. In contrast to partial exchange transfusion, restrictive management has been confronted with several difficulties. Therefore, further controlled studies with new methods are needed to observe the long-term effects. In this review, it is aimed to evaluate the efficacy of current and recently retrieved treatment approaches in neonatal polycythemia. [ABSTRACT FROM AUTHOR]

Introduction: The aim of this study is to evaluate the long-term neurodevelopmental outcomes of newborns who had exchange transfusion due to hyperbilirubinemia and presented with bilirubin values at the border of exchange transfusion and did not need exchange transfusion during the preparation period. Methods: The study included 18 patients (n=18) who had exchange transfusion due to indirect hyperbilirubinemia (Group 1) and 10 patients (n=10) who applied with a bilirubin value at the border of exchange transfusion and decreased bilirubin during the preparation period (Group 2). Neurological examination, cranial magnetic resonance imaging (MRI), and auditory brainstem response test (BERA) results of the cases were evaluated through cross-sectional analysis. Ankara Developmental Inventory for children under the age of six and Wechsler intelligence scale-Revised for children over the age of six were performed. Results: Sixty-seven percentage (n=12) of the patients in Group 1 were male, mean gestational week was 38.4±1.8 (34-42) and mean age was 8.8±2.1 years. 40% (n=4) of the patients in Group 2 were male, mean gestational week was 37.9±1.7 (36-40) and mean age was 8.2±2.2 years. The mean venous serum total bilirubin level of group 1 was 29.5±8.2 mg/dL and Group 2 was 22.8±3.9 mg/dL, which was statistically significant (p=0.024). Neurological examination was abnormal in four patients in Group 1 and one patient in Group 2. Two of the cranial MRIs (n=8) in Group 2 were pathological. There was no significant difference between the groups in terms of neurological examination, cranial MRI, BERA, and developmental tests. Discussion and Conclusion: In this study, the fact that there was no neurodevelopmental difference between the cases who underwent exchange transfusion or whose bilirubin levels were lowered by phototherapy without the need for exchange transfusion, and the cases with neurodevelopmental abnormalities were very few, once again demonstrated the importance of intervening before bilirubin encephalopathy occurs. [ABSTRACT FROM AUTHOR]

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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 [ABSTRACT FROM AUTHOR]

Abstract 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

IntroductionLeukapheresis 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.MethodsForty-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).ResultsThe 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

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction: Exchange transfusion (ET) is the first successful treatment introduced for severe neonatal jaundice considered to be a safe procedure but not risk free. Present study aimed to determine the complications related to exchange transfusion and to compare the incidence of severe complications between healthy and ill newborns. Methods: This cross sectional study was done in Neonatal Intensive Care Unit (NICU), Dhaka Shishu Hospital and Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, over 6 months from January to June 2010, included 50 newborns who underwent exchange transfusion due to hyperbilirubinaemia. Newborns with hyperbilirubinemia alone were classified as healthy, while hyperbilirubinaemia associated with other medical problems were classified as ill. Adverse events were analyzed and compared between two groups. Results: Out of 50 patients who underwent ET, 27 were healthy had jaundice only and 23 were ill. Most of the newborns with comorbidities (87%) developed at least one complication following exchange transfusion (p<0.001). Newborns with co-morbidities developed hypoglycemia (13%), hypocalcaemia (17.4%), thrombocytopenia (26.1%), sepsis (26.1%), apnoea (4.3%) and in newborns without co-morbidities, hypoglycemia, hypocalcaemia, thrombocytopenia each was found in 7.4% cases and septicemia (14.8%). Thrombocytopenia was significantly higher in ill newborns (p = 0.039). About 9% of newborns died in ill group compared to 3.7% in healthy group (p = 0.439). Conclusions: Complications and mortality were significantly higher in newborns having hyperbilirubinemia with comorbidities. Thrombocytopenia was the most frequent complication. Majority of the hyperbilirubinemic neonates with comorbidities develop at least one complication after exchange transfusion. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]