Required Exchange Transfusion Research Articles

Incremental eligibility criteria for the BMT CTN 1507 haploidentical trial for children with sickle cell disease

AbstractThe Blood and Marrow Transplant Clinical Trials Network (BMT CTN) 1507 leadership and the data safety monitoring board (DSMB) established incremental entry criteria for children aged 5 to 14.99 years with sickle cell disease (SCD) enrolling in a phase 2 trial of HLA-haploidentical hematopoietic stem cell transplantation. First, the enrollment was limited to overt stroke in the first 10 participants (stage 4). Subsequently, the DSMB reviewed the interim results and expanded the eligibility to include children with silent cerebral infarcts or abnormal transcranial Doppler velocities with magnetic resonance angiography–defined cerebral vasculopathy (stage 3). A third cohort was enrolled after the DSMB reviewed the clinical outcomes in these cumulative initial enrollments (n = 18). Additions to the entry criteria included nonneurologic morbidities (stage 2). Eligibility criteria included the following: (1) life-threatening acute chest syndrome requiring exchange transfusion; (2) right heart catheterization confirmed pulmonary hypertension; (3) persistent systemic hypertension despite maximum medical therapy; (4) acute pain despite maximum medical therapy in the absence of psychosocial factors and unmanaged asthma after adjudication; or (5) 2 major priapism episodes in 12 months or 3 in 24 months. Children with SCD who did not meet the criteria for stages 4, 3, and 2 were not eligible. To our knowledge, for the first time, we introduce a staged strategy for eligibility in a curative therapy trial for children with SCD concordant with 45 Code of Federal Regulations § 46.405(b). The research governance–mandated eligibility strategy used within the BMT CTN 1507 phase 2 study may apply to future pediatric SCD curative therapy trials. This trial was registered at www.ClinicalTrials.gov as #NCT032635590.

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

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

Feto-maternal outcome of Rh-Negative pregnancy presenting in a tertiary healthcare centre

Background: The term "Rhesus incompatibility" describes the mismatched Rh types of the mother and the fetus. It is linked to the onset of hemolytic disease of the newborn (HDN) ranging from haemolytic anemia to hydrops fetalis and also related to maternal Rh sensitization. The disease incidence is currently declining globally, having dropped from 1.3%–1.7% in the 1980s to 0.17% in the 1990s1. Erythroblastosis foetalis is one of the most terrible consequences of an ABO incompatible or Rh incompatible pregnancy. About 10% of all Rh-negative pregnancies result in Rh incompatibility in the Rh-negative mother carrying a Rh-positive fetus. Methods: This observational study was carried out among 60 Rh negative pregnant women attending our antenatal clinic and delivered in our institution from October 2022 to March 2024.The Rh negative women were followed up with a series of investigations such as Indirect Coombs Test (ICT), MCA-PSV and with regular antenatal care (ANC). After birth neonates were followed up with blood grouping and Rh typing, direct coombs test (DCT), duration of phototherapy, duration of NICU stay , need for immunoglobulin and exchange transfusion were recorded to evaluate the maternal and neonatal outcomes.The data was collected and tabulated in Microsoft excel sheet and the percentages were calculated. Results: This study included a total of 60 Rh negative mothers ,where 48 (80%) of them were aged less than 30 years and 12 of them (20%) were more than 30 years of age. Antenatally, 51 (85%) women were ICT negative and received RAADP, 9(15%) women were ICT positive and anti D titres were less than 1:16 followed up with MCA-PSV which was normal/less than 1.5MoM. The total preterm births were 6(10%).The total admissions to NICU were 12(20%) in our study. All the 9 babies born to ICT positive mothers turned out to be Direct Coombs test (DCT) positive and were managed with double surface phototherapy. For 2(3.3%) babies, human immunoglobulin was given. Only 1(1.6%) baby required exchange transfusion. Conclusion: We conclude that, severe hyperbilirubinemia and hydrops foetalis, which were observed previously are drastically reduced with recent advances and the use of Routine antenatal Anti D prophylaxis (RAADP). Neonatal morbidity and mortality reduced drastically with newer advances like immunoglobulin reducing the need for exchange transfusion and better NICU care. ICT positivity of 15% despite of postnatal immunisation suggest that there is chances of silent fetomaternal haemorrhage during antenatal period, health care professionals should be more knowledgeble with prenatal screening, the value of blood grouping and Rh typing, anti-D immunisation following sensitising events like MTP, abortion, ectopic pregnancy, ECV and regular implementation of RAADP in clinical practice.

A prospective study on the causes of hyperbilirubinemia and its clinical markers in term and near-term newborns admitted to a tertiary care hospital in Patna, India

This study aimed to investigate the root causes, prognostic factors, and therapeutic strategies for neonatal hyperbilirubinemia among term and near-term infants admitted to the Neonatal Intensive Care Unit (NICU) at IGIMS, Patna. Furthermore, the research aimed to analyze the clinical progression of these infants throughout their NICU stay.Conducted within the NICU at IGIMS, Patna, Bihar, this study encompassed term and late preterm infants admitted with serum bilirubin levels exceeding 12 mg/dl. The primary focus was on identifying risk factors, etiological factors, and the clinical profile of these infants during their NICU tenure.The study examined birth weight distribution and concurrent medical conditions. Parameters included the initiation of phototherapy based on bilirubin levels and treatment methodologies. Out of the 210 infants studied, 27.14% and 47.62% weighed between 2001 and 2500 grams. The most prevalent causes were physiological (129 cases), fetal prematurity (30 cases), birth asphyxia (7 cases), and prolonged labor (18 cases). Idiopathic causes were observed in 28 instances, ABO incompatibility in 7 cases, and Rh incompatibility in 7 cases. G6PD deficiency was identified once. At the commencement of phototherapy, 19.52% had bilirubin levels between 12 and 15 mg/dl, 58.57% between 15.1 and 18 mg/dl, 11.91% above 20 mg/dl, and 10% between 18.1 and 20 mg/dl.Physiological causes emerged as the most frequent contributors to neonatal hyperbilirubinemia, followed by septicemia and idiopathic factors. Infrequent causes included blood group incompatibility. Phototherapy was established as a cost-effective and efficient method for reducing bilirubin levels in neonatal jaundice. Exchange transfusion proved to be a safe therapeutic measure in cases of severe hyperbilirubinemia. Blood group incompatibility was the main determinant requiring exchange transfusion.

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

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

Environmental Exposure to Pesticides and the Risk of Child Neurodevelopmental Disorders.

Background and Objectives: Neurodevelopment is a fragile brain process necessary for learning from the beginning of childhood to adulthood. During the procedure, several risks could affect it, including environmental factors such as neurotoxic chemicals or environmental pollutants and, within them, exposure to pesticides. Materials and Methods: This ecological descriptive study attempted to assess the association between environmental exposure to pesticides and neurodevelopmental disorders. This study was conducted on 4830 children diagnosed for 11 years in a total population of 119,897 children in three areas: high, medium, and low greenhouse concentrations. Results: Chromosomal abnormalities were the most common prenatal disorder (28.6%), while intrauterine physical factors were the least common (0.5%). Among perinatal diagnoses, gestational age less than 32 weeks was the most common (25%), while hyperbilirubinemia requiring exchange transfusion and birth complications was the least common (0.4%). Brain damage was the most common problem detected in postnatal diagnosis (36.7%), while unspecified postnatal abnormalities were the least common (3.1%). Conclusions: The areas with the highest greenhouse concentration had higher incidences of neurodevelopmental disorders, particularly in boys, and lower age of referral. Chromosomal abnormalities were prevalent for prenatal diagnoses, gestational age below thirty-two weeks for perinatal diagnoses, and brain damage for postnatal diagnoses. Future studies should analyze the connection between pesticide exposure and neurodevelopmental disorders using spatial point pattern analysis.

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

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

Cord blood albumin level as a predictor of neonatal physiological jaundice in healthy term neonate

Background: Neonatal jaundice is a very common condition worldwide, occurring in up to 60% of term and 80% of pre-term newborns in the 1st week of life and the most common cause for readmission. The present study was conducted to evaluate the predictive value of umbilical cord blood albumin level for subsequent development of neonatal jaundice in healthy term neonates. The present study is conducted to find usefulness of cord blood albumin in predicting the subsequent development of significant neonatal jaundice. Methods and Methods: This is a hospital-based prospective study conducted in 100 healthy term neonates. Cord blood albumin level estimation was done at birth. Total serum bilirubin estimation was done for all babies with clinically suspected jaundice at 72–96 h of age. The neonates were divided into three Groups A, B, and C based on the cord albumin levels of &lt;2.8 g/dL, 2.8–3.3 g/dL, and &gt;3.3 g/dL, respectively. The main outcome of the study was inferred in terms of serum bilirubin ≥17 mg/dL, newborn required phototherapy, and exchange transfusion. Results: Groups A, B, and C had 21, 35, and 44 newborns, respectively. In Group A, 18 (85.7%) neonates had total bilirubin of &gt;17 mg/dL, of which 16 (76.19%) required phototherapy and 2 (9.52%) needed exchange transfusion. 23 (65.7%) neonates in Group B developed jaundice, of which 12 (34.2%) needed phototherapy and none of them required exchange transfusion. In Group C, 15 (34.09%) developed jaundice of which 1 (2.2%) required phototherapy and none of them required exchange transfusion (p&lt;0.001). Conclusion: Cord blood albumin level ≤2.8 g/dL is a significant risk factor for developing neonatal hyperbilirubinemia that requires early intervention, while cord blood albumin &gt;3.3 g/dL is probably safe for early discharge. Hence, this can help to identify the at-risk neonates. Hence, routine determination of cord blood albumin level can be advocated to keep a track on at-risk neonates.

Prevalence and risk factors of hearing loss in high-risk neonates at a tertiary care hospital in Mumbai

Background: The present study was conducted to assess the prevalence of hearing loss using measurements of otoacoustic emission (OAE) in newborns admitted to NICU and to determine the risk factors predictive of hearing impairment in these newborns. Methods: This was a Prospective observational study conducted among 220 newborns admitted to the NICU over 18 months. Ethical clearance was obtained from the IEC. The sample size was obtained by the universal sampling technique. All candidates underwent hearing assessment in a sound-treated room in the department using a distortion product OAEs (DPOAE) test at the time of discharge. Babies who did not pass the initial screening underwent another OAE test within one month after being discharged. If they failed the test again, they were referred to an otologist for BERA. Results: Of the 226 neonates included in the study, 40 cases (17.7%) failed the initial screening. 29 cases (12.8%) failed the second OAE screening and were subjected to BERA. Risk factors included asphyxia (p≤0.001), mechanical ventilation (p&lt;0.0004), history of ototoxic medication (p=0.004), neonates with hyperbilirubinemia requiring exchange transfusion (p=0.005), low Apgar scores (p≤0.001). Conclusions: A two-stage OAE hearing screening can be a suitable method for early detection of hearing impairment in newborns. This method has shown high prevalence of hearing loss among high-risk neonates but needs to be confirmed with diagnostic BERA. It is crucial to have universal hearing screening to detect the large number of hearing-impaired neonates in the newborn population of our country, including those who are not considered at risk.

Clinical profile of term neonates requiring intervention for hyperbilirubinemia

ABSTRACT Introduction: Neonatal jaundice is the imbalance between bilirubin production and conjugation, resulting in increased serum bilirubin levels. It is visible in the skin and eyes, only when the total serum bilirubin level exceeds 5–7 mg/dl. Sixty percent of the term and almost 80% of the preterm babies develop neonatal jaundice. Objectives: To identify the etiology of neonatal hyperbilirubinemia in term neonates and to determine short-term outcomes in terms of rebound hyperbilirubinemia during hospital stay and other modalities of intervention in these babies. Methods: We did a cross-sectional, descriptive, and retrospective chart-based study. The clinical profile of term neonates treated for hyperbilirubinemia was procured from the inpatient medical records and neonatal intensive care unit (NICU) discharge summaries over 5 years from January 2017 to December 2021. Short-term outcomes, such as duration of hospital stay, rebound hyperbilirubinemia, and other modalities of treatment used, were noted. Results: A total of 252 term babies who fulfilled the inclusion criteria were included in the study. Two hundred and thirty-four babies were delivered vaginally, 10 by cesarean section, and 8 extracted by instrumental delivery. Ninety-eight babies had ABO incompatibility and 19 Rh incompatibility. Twelve babies had elevated cord thyroid-stimulating hormone levels. Seventy-one babies had signs of bilirubin encephalopathy. Thirty-nine babies had sepsis. Fifty-three of them had significant weight loss suggestive of improper feeding. Thirty-three babies had cephalhematoma. Nine babies were born to mothers with gestational diabetes mellitus. All 252 babies underwent phototherapy. Thirty-two babies required a prolonged hospital stay of more than 1 week and seven had rebound hyperbilirubinemia. Nine babies required exchange transfusion. Two received intravenous immunoglobulin. Conclusions: The most common risk factor associated was ABO incompatibility, followed by inadequate breastfeeding. As only nine neonates required exchange transfusion, early and prompt initiation of phototherapy remains the best modality of treatment. Emphasis on proper feeding technique can go a long way in preventing admission to NICU, as it is the second-most common preventable cause of hyperbilirubinemia.

Universal screening for hyperbilirubinemia in term healthy newborns at discharge: A systematic review and meta-analysis.

All term healthy neonates are screened for jaundice before hospital discharge as a standard clinical practice, but methods vary from clinical screening (visual inspection and/or risk factor assessment) to transcutaneous bilirubin (TcB) or total serum bilirubin (TSB) testing, depending on the setting. This systematic review of randomized and non-randomized studies evaluated the effectiveness of universal TcB and universal TSB screening at discharge compared to clinical screening alone for term healthy neonates. The outcomes were neonatal mortality, readmission for jaundice, severe hyperbilirubinemia (>20 mg/dL), jaundice requiring exchange transfusion, and bilirubin-induced neurological dysfunction (BIND). We searched MEDLINE via Ovid, EBM reviews, Embase, CINAHL, clinical trials databases, and reference lists of retrieved articles. Two authors separately evaluated the risk of bias, extracted data, and synthesized effect estimates using relative risk (RR) for randomized and odds ratio (OR) for non-randomized studies. For universal TcB at discharge, we included one randomized trial enrolling 1858 participants and four non-randomized studies enrolling 375 956 participants. No study reported neonatal mortality. The randomized trial suggested that universal TcB at discharge may decrease readmission for jaundice (risk ratio (RR) = 0.24, 95% confidence interval (CI) = 0.13 to 0.46; low certainty evidence) and severe hyperbilirubinemia (RR = 0.27, 95% CI = 0.08 to 0.97; low certainty evidence), but the effect on jaundice requiring exchange transfusion (RR = 0.20, 95% CI = 0.01 to 41.6) and BIND (RR = 0.33, 95% CI = 0.01 to 8.17) was uncertain. Meta-analysis of non-randomized studies suggested that TcB may decrease severe hyperbilirubinemia (odds ratio (OR) = 0.25, 95% = CI 0.12 to 0.52; low certainty evidence) and jaundice requiring exchange transfusion (OR = 0.28, 95% CI = 0.19 to 0.42; low certainty evidence), but the effect on readmission for jaundice was uncertain (OR = 1.01, 95% CI = 0.38 to 2.7; very low certainty evidence). For universal TSB, we included three studies from the United States enrolling 490 426 participants. The effect on severe hyperbilirubinemia (OR = 0.37, 95% CI = 0.15 to 0.88), jaundice requiring exchange transfusion (OR = 0.53, 95% CI = 0.13 to 2.25) and readmission for jaundice (OR = 1.01, 95% CI = 0.62 to 1.67) was uncertain. Universal TcB at discharge may improve clinical outcomes for term healthy neonates. Evidence for universal TSB is uncertain. PROSPERO 2020 CRD42020187279.

Bilirubin Measurement Through a Smartphone Application in Preterm Infants.

Aim of the present study is to evaluate the feasibility and reliability of an smartphone application for monitore of bilirubin levels in preterm infants. Preterm infants hospitalized in the neonatal intensive care unit with gestational age of<35 weeks were included. Exclusion criteria were parental reluctance and requirement of phototherapy in the last 12 hours. Measurements were obtained through a smartphone application (BiliScan) along with simultaneous transcutaneous device (Dräger JM 105) and venous blood biochemistry. Mean gestational age was 30.8±2.4 weeks and birth weight was 1622±566 g. Measurements were obtained at a median of 4 (1-21) days of life. Twenty-five infants (19.4%) had ABO and/or Rh incompatibility and 39 infants (30.2%) required phototherapy. None of the cases required exchange transfusion. Mean total serum bilirubin (TSB) level was 8.16±2.60 mg/dL, mean transcutaneous bilirubin (TcB) level was 8.60±2.70 mg/dL, and the mean bilirubin level measured by BiliScan was 7.26±2.68 mg/dL. For TSB and TcB measurements, the intraclass correlation coefficient (ICC) was found to be 0.915 (95% confidence interval 0.835-0.951; p<0.001) and a strong positive correlation was found between these two measurements. When TSB and BiliScan measurements were compared, ICC was found to be significant as 0.512 (95% confidence interval 0.353-0.638; p<0.001), with a moderate correlation. In this study, we evaluated the feasibility and reliability of a smartphone application for monitoring bilirubin levels in preterm infants. Although BiliScan measurements reported to display high correlation in term infants, a moderate correlation was found in the preterm infants. It is an emerging low-cost, non-invasive alternative for neonatal jaundice monitoring, however, results should be interpreted with caution in preterm infants.

399: A CASE OF SEVERE BABESIOSIS REQUIRING EXCHANGE TRANSFUSION

Introduction: Babesiosis is an infection caused by a protozoan that infects human erythrocytes. In the United States, Babesia microti is the most commonly isolated strain transmitted by tick vector (Ixodes scapularis). The severity of infection usually depends on host immune status and Babesia species, ranging from asymptomatic to life-threatening. We describe a case of severe babesiosis requiring exchange transfusion in an elderly but otherwise healthy individual. Description: A previously healthy 90-year-old man presented to the emergency department with a 1-week history of dyspnea and dark-colored urine. Patient reported a tick bite two months prior for which he was empirically treated with doxycycline. On admission, he was afebrile, blood pressure was 130/69 mmHg, heart rate was 84 bpm, and respiratory rate was 22 breaths/min. Physical exam was unremarkable. Labs were notable for serum creatinine 2.70mg/dl, total bilirubin 3.2mg/dl, direct bilirubin 1.6mg/dl, AST 125U/L, ALT 66U/L, hemoglobin 10.1g/dl, and platelets 44k/µL. Peripheral smear showed babesia species without Anaplasma and Ehrlichia, and Lyme serology was negative. Patient was started on intravenous azithromycin and atovaquone and admitted to medical ward. Later that day he became hypotensive with elevated lactate of 3.1mmol/dl. His blood pressure was minimally fluid-responsive. Further labs revealed a haptoglobin < 10, elevated lactate dehydrogenase, and parasitemia of 31.4%. Due to the hypotension with evidence of organ damage, intravascular hemolysis, and high-grade parasitemia, he was transferred to the medical intensive care unit (MICU) for exchange transfusion. In the ICU patient received two rounds of exchange red blood cell transfusion with which parasitemia decreased to 1.4% and hemolysis parameters improved. His blood smear was negative for babesia on hospital day 5 and he was discharged home to complete a 2-week course of azithromycin and atovaquone from the day of negative smear. Discussion: Babesiosis can be life-threatening, especially in elderly, asplenic, and immunocompromised patients. Our case outlines the importance of early institution of exchange transfusion in addition to appropriate antimicrobial therapy in patients with end-organ damage, intra-vascular hemolysis, and high-grade parasitemia for better outcomes.

329: A PERFECT STORM OF SICKLE CELL HEPATOPATHY

Introduction: Intrahepatic cholestasis (SCIC) an uncommon but serious complication of vaso-occlusive crisis in sickle cell disease (SCD). Presenting with abdominal pain, hyperbilirubinemia, and transaminitis, and in severe cases with renal failure, encephalopathy, and coagulopathy. Early diagnosis is challenging but essential to improving outcomes. Herein, we report a case of a young patient with SCD who presented with SCIC requiring exchange transfusion which resulted in an eventual favorable outcome. Description: 18-year-old man with history of SCD presented for generalized abdominal pain. He complained of severe diffuse body pain. On admission, he had a total bilirubin (T.Bili) of 5.5 mg/dL, hemoglobin of 9.1 g/dL (baseline 8-9) and a clear chest x-ray. He was started on morphine PCA due to increasing pain and transfused one unit of packed red cells (pRBC). He subsequently developed a fever and had a chest x-ray concerning for acute chest syndrome. On day 2, hemoglobin dropped to 5.1 g/dL and he was transfused a further 2 units of pRBC. Over the course of day 2, he developed worsening kidney function and metabolic acidosis, so he was transferred to the medical intensive care unit. Overnight, he became increasingly encephalopathic and developed acute liver failure with AST 2317 IU/L, ALT of 1976 IU/L, INR of 4.7 and T.Bili of 13.3 mg/dL. An exchange transfusion was urgently arranged and initiated by morning of Day 3. His hospital course was complicated by ongoing encephalopathy, respiratory failure requiring mechanical ventilation and renal failure requiring hemodialysis. His total bilirubin, direct bilirubin and alkaline phosphatase peaked at 32.0 mg/dL, 30.0 mg/dL, 1024 IU/L, respectively. With further supportive care, he had recovery of liver function, was extubated, weaned off dialysis, and eventually discharged home. Discussion: This case illustrates the rapid and life threatening nature of SCIC and the importance of prompt initiation of exchange transfusion therapy. While SCIC is a challenging given its non-specific constellation of symptoms with significant overlap with several other hepatic processes, nevertheless it should be high on the differential in patients with SCD who develop hepatic abnormalities during vaso-occulsive crisis.

Frequency of Indirect Hyperbilirubinemia Requiring Exchange Transfusion, Etiology and its Immediate Outcome in Term Neonates in Neonatal ICU of National Institute of Child Health, Karachi

Objectives: The present study assessed the frequency of causes of indirect hyperbilirubinemia (IH) requiring exchange transfusion and its immediate adverse events of exchange transfusion in term neonates. Methods: A prospective, observational study was undertaken at the Neonatal ICU of National Institute of Child Health, Karachi between July 2021 and December 2021. Term neonates arriving at the newborn unit of NICH with indirect hyperbilirubinemia were included in this study. All enrolled neonates were monitored for clinical, biochemical, and hematological adverse events for 7 days following exchange transfusion, and a predesigned performa was used to document observations. Results: Among 730 neonates with indirect hyperbilirubinemia, a total of 63 term neonates underwent exchange transfusion. The most common causes of hyperbilirubinemia were sepsis in 19 (30.2%) patients and ABO incompatibility in 13 (20.6%) patients. The most common adverse events noted were hypokalemia, which occurred in 10 (15.9%) and thrombocytopenia, which occurred in 10 (15.9%) cases. In six patients, sepsis developed. In total, five neonates died while four developed bilirubin encephalopathy. The total serum bilirubin and direct bilirubin among patients were significantly lowered after exchange transfusion (p&lt;0.0001). There were no umbilical catheter-related complications. Bilirubin encephalopathy was present in 4 patients. There were five deaths, but not due to the procedure. Practical implication Conclusion: We highlighted the main causes of indirect hyperbilirubinemia as sepsis and ABO incompatibility in our setting. Furthermore, we also identified the immediate and short-term outcomes of exchange transfusion with a mortality rate of 7.9%. The overall outcome was favorable after the exchange transfusion. Keywords: Hyperbilirubinemia, kernicterus, exchange transfusion, indirect bilirubin, neonates , jaundice

Hypocalcemia in Jaundiced Term Neonates Undergoing Phototherapy

Objective: frequency of hypocalcemia in jaundiced term neonates undergoing phototherapy. Methodology: A total of 150 term neonates of either gender who develop jaundice requiring phototherapy are included in this study presenting within first week of life whereas those term jaundiced neonates who are at known risk of developing hypocalcemia, infant of diabetic mother, neonates having history of birth asphyxix, septic neonates, those requiring exchange transfusion and those with hemolytic anemias were excluded from study. The patients were examined thoroughly (complete history and physical examination).Informed consent was obtained to include the data in study. Jaundiced neonate is applied phototherapy and after 48 hrs of phototherapy session, 5ml blood sample is drawn and sent to hospital laboratory for estimation of total serum calcium level. Hypocalcemia (i.e. Calcium level &lt; 8 mg/dl after 48 hours application of phototherapy for jaundice neonatorum) was recorded and managed as per hospital protocol. Results: Out of 150 cases, 46%(n=69) were in range of 1-3 days while 54%(n=81) were between the range of 4-7 days of life. The common age was 3.59+1.60 days. There were 53.33%(n=80) male while 46.67%(n=70) were female participants. Hypocalcemia was recorded in 20.67%(n=31). Conclusion: We concluded that the frequency of phototherapy associated hypocalcemia is not very high, however, calcium levels should be monitored as it is a potential but very less studied complication in our clinical settings. Keywords: Term neonates, jaundice, phototherapy, hypocalcemia

Effects of Different Types of Phototherapy Units on Neonatal Jaundice: A Cross-sectional Study

Introduction: Phototherapy is the mainstay of treatment of neonates who develop significant jaundice. Light-emitting diode and compact florescent lamp phototherapy units are the newer devices in the management of neonatal hyperbilirubinaemia. The advantage of light-emitting diode phototherapy includes portability, energy efficiency, less heat production, and durability. Blue and white phototherapy devices are more economical but generate more heat leading to hyperthermia and dehydration more often. Aim: To determine the efficacy and side effects of three modes of phototherapy units viz conventional blue and white light phototherapy, Compact Fluorescent Lamp (CFL) phototherapy, and Light-emitting Diode (LED) phototherapy on neonates having jaundice. Materials and Methods: This cross-sectional study was conducted in Department of Paediatrics at SRM Medical college Hospital and Research Centre (tertiary care teaching hospital), Kattankulathur, Tamil Nadu, India, from February 2021 to January 2022. A total of 150 neonates with hyperbilirubinaemia in the phototherapy range, American Academy of Paediatrics (AAP), were included in this study. Study subjects were divided into three groups of 50 neonates each to receive phototherapy using one of the three phototherapy devices, i.e., Blue and white light, CFL, LED phototherapy groups. Data of serum bilirubin levels were recorded at the beginning and end of phototherapy. The number of babies who required exchange transfusion and other clinical side effects among the study groups was recorded. Descriptive statistics were reported as Mean±SD for continuous variables, frequencies (percentage) for categorical variables. The One-way Analysis of Variance (ANOVA) test was used to determine the statistically significant differences between the means of three independent groups. Results: All the three type of phototherapy units generated statistically non significant mean flux (p-value=0.754). Mean difference (from the baseline bilirubin) at end of phototherapy for blue and white light group was 5.77±1.72 mg%, for CFL group was 5.48±1.32 mg%, and for LED group was 6.34±1.48 mg%. The difference was statistically significant in all three groups (p-value=&lt;0.001). The reduction in serum bilirubin at the end of phototherapy was lesser in CFL group compared to blue and white light group and LED group. Conclusion: Light-emitting diode and blue and white light phototherapy units are more effective than compact fluorescent lamp phototherapy in treating neonatal hyperbilirubinaemia.

Toxic methemoglobinemia due to biofertilizers, herbicides

Introduction : Methemoglobinemia is an emergency condition which is the result of the oxidization of iron into ferric form. It can be congenital or acquired. Acquired methemoglobinemia due to drug toxicity, in rural areas most commonly due to intentional self-poisoning and the most common compounds, are plant growth enhancers, herbicides and indoxacarb which is a non-organophosphorus insecticide. Methods: This is a retrospective observational study of patients who are admitted in an emergency with methemoglobinemia from 2018 to the present. Results: A total of 10 cases were found in the hospital records with toxic methemoglobinemia and the sex ratio is equally distributed. Farmers were predominant with 60. Ingestion was the mode of consumption in all cases. The most common compound was seaweed extract followed by plant alkaloids all cases presented to emergency initial saturation was less than 80% with the most common presenting feature being breathlessness and cyanosis and irritability in cases presenting late. Out of the 10 cases, 30% cases required mechanical ventilation at initial presentation. Despite 100% oxygen supplementation saturation levels did not improve indicating a problem with oxygen binding and unloading. All the cases were given methylene blue as the antidote of choice. 6 patients required 1 dose. In all cases saturation levels improved after ingestion of methylene blue, but 4 patients required repeat dosing, and 3 patients required exchange transfusion. Conclusion: Methemoglobinemia is a potential complication of ingestion of herbicides, plant growth extracts and plant alkaloid compounds. The presence of breathlessness, cyanosis, and persistent hypoxemia should raise suspicion of the disorder. Early institution of easily available antidote methylene blue can save lives and avert potential complications.

Microfluidic photoreactor to treat neonatal jaundice

While in most cases, jaundice can be effectively treated using phototherapy, severe cases require exchange transfusion, a relatively risky procedure in which the neonate's bilirubin-rich blood is replaced with donor blood. Here, we examine extracorporeal blood treatment in a microfluidic photoreactor as an alternative to exchange transfusion. This new treatment approach relies on the same principle as phototherapy but leverages microfluidics to speed up bilirubin removal. Our results demonstrate that high-intensity light at 470 nm can be used to rapidly reduce bilirubin levels without causing appreciable damage to DNA in blood cells. Light at 470 nm was more effective than light at 505 nm. Studies in Gunn rats show that photoreactor treatment for 4 h significantly reduces bilirubin levels, similar to the bilirubin reduction observed for exchange transfusion and on a similar time scale. Predictions for human neonates demonstrate that this new treatment approach is expected to exceed the performance of exchange transfusion using a low blood flow rate and priming volume, which will facilitate vascular access and improve safety.

Persistence of Chronic Inflammation Despite Years of Transfusion Program in SCD Patients: Changing Red Blood Cells Is Not Sufficient to Treat Sickle Cell Disease

▪Sickle cell disease (SCD) is a severe hemoglobinopathy due to abnormal hemoglobin S (HbS). Although red blood cell dysfunction is at the core of the SCD pathophysiology, several studies have highlighted the important role of inflammatory cells like neutrophils. One of the most serious complications of SCD is cerebral vasculopathy (CV), due to the occlusion of one or more intracranial or cervical arteries. In 1998, the STOP study demonstrated that monthly blood transfusions could reduce the risk of stroke by 90% in children with CV. However, there is large heterogeneity in the evolution of CV under chronic transfusion, sometimes requiring exchange transfusion (ET) program for years without succeeding in healing the CV.The aim of the study is to investigate the impact of long-term transfusion program on neutrophil dysfunction, in order to understand if persistent inflammation could contribute to the non-healing of CV despite HbS permanently below 40%. In SCD children undergoing ET program for at least 1 year, we analysed i)the phenotype of neutrophils with 8 markers of activation/adhesion/ageing, ii)the plasmatic levels of elastase, witnessing the NETose activity of neutrophils, and iii)the ex-vivo adhesion of neutrophils on activated endothelial cells.One hundred and two SCD children with an ET transfusion program for at least 6 months because of CV were included in the study. ET session, carried out every 5 weeks and most of the time by erythrapheresis, reached their biological objectives with a mean HbS rate after ET session of 14.1%, and 35.4% before the next ET session, which means that these patients globally live at an average HbS level of 24% for at least 1 year. We managed to limit iron overload with a mean ferritinemia of 207 µg/L in the whole cohort. Despite these satisfactory results in terms of HbS reduction, the efficiency in curing the CV was modest in accordance with the previously described efficiency of ET program in SCD children: after a mean ET program duration of 4.4 years only 22% of them had an improvement of their CV since the beginning of the ET program, while 60% of them had a stagnation of their CV, and 18% of them worsened their vascular lesions. Considering inflammatory parameters, the patients had persistence of high leukocytosis and high neutrophils count (respective mean of 9810 G/L and 5742 G/L), significantly not different of neutrophils count before inclusion in the ET program. In a random subgroup of 20 patients, we analysed neutrophils phenotype, NETose and endothelial adhesion and compared them to healthy controls and SCD children without ET, treated or not with Hydroxyurea (HU). Overall, we observed as expected an activated, aged and adherent profile of neutrophils from untreated SCD children compared to healthy controls, characterized by an overexpression of CD18/CD11b (p=0,03), CD18/CD11a (p=0,02), CD162 (p=0,01), CD66a (p=0,01) and the ageing markers CD184 high/CD62Llow (p=0,04) as well as a higher plasmatic level of elastase (p=0. 01) and higher adhesion of neutrophils to endothelial cells. All these parameters were alleviated in SCD patients treated with HU. In SCD patient undergoing ET program, we found a similar profile of activated neutrophils to that of untreated SCD patients with a similar expression of activation molecules, high level of elastase and the same increase of neutrophils adhesion to endothelial cells compared to controls, witnessing a persistence of chronic inflammation despites years of ET.Overall, our study highlights that the replacement of sickle red blood cells, even for years, is not sufficient to reverse the deleterious inflammatory phenotype of neutrophils. Given the major role of inflammation in endothelial dysfunction, these could contribute to the persistence of CV in a majority of patients despite efficient ET programs. This raises the question of systematically combining ET program with anti-inflammatory treatment such as HU or P-selectin inhibitors in children with CV. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.