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Neonatal Hemochromatosis (NH) is a rare phenotype of severe fetal/neonatal liver injury that is accompanied by extrahepatic siderosis. Current clinical evidence shows that NH is not a disease per se, but is the consequence of fetal liver injury. Gestational alloimmune liver disease is the cause of nearly all cases of NH. Affected babies may die in utero, or present postnatally with severe acute liver failure or decompensated congenital liver cirrhosis. Diagnosis depends on the demonstration of extrahepatic siderosis by Magnetic Resonance Imaging (MRI), buccal biopsy, or detection of complement C5b–9 complex on hepatocytes from liver biopsy. Prognosis is generally bad without treatment. Treatment with iron chelators and antioxidants is not helpful. The more recent treatment approach of exchange transfusion and Intravenous Immunoglobulin (IVIG) has shown favorable outcomes. In this report, we describe a case of NH that presented with liver cell failure and high serum ferritin. Diagnosis was confirmed by MRI by demonstration of siderosis in the liver and pancreas while sparing the spleen. The infant was successfully treated with a combination of exchange transfusion and IVIG, and discharged at age 30 days in good condition.

Background: Gestational alloimmune liver disease (GALD) is a rare but critical cause of neonatal liver failure. After discovering the maternal–fetal alloimmune mechanism, intravenous immunoglobulin (IVIG) with or without exchange transfusion (ET) has gradually replaced antioxidant cocktails as the first-line therapy. Whether such therapy changes the outcome of neonates with GALD is yet to be defined.Method: We reported a pair of twins with discordant presentations, mild and self-limited in the older, whereas liver failure in the younger, who was successfully rescued by ET and IVIG. To investigate the outcome after therapeutic alteration, 39 cases between 2005 and 2020 from literature research were collected.Results: Half of the collected cases (47.1%) were preterm. Common presentations were ascites, jaundice, respiratory distress, hepatomegaly, and edema. Leading laboratory abnormalities were coagulopathy, hypoalbuminemia, and elevated serum ferritin. Salivary gland biopsy and magnetic resonance imaging detected extrahepatic siderosis in 70% (14/20) and 56% (14/25), respectively. IVIG, ET, and liver transplantation were performed in 19 (48.7%), 15 (38.5%), and 8 (20.5%) patients, respectively. The overall survival (OS) rate and native liver survival (NLS) rate were 64.1% (25/39) and 43.6% (17/39), respectively. Although the compiled results did not support a significant benefit, the OS and NLS were higher in the IVIG with/without ET group compared with those treated with conventional therapy [OS (70 vs. 57.9%) and NLS (55 vs. 31.6%), respectively].Conclusion: A high index of suspicion for GALD is crucial when facing a neonate with liver failure. Despite no significant influence on the outcome over conventional therapy in such a rare and detrimental disease, IVIG with or without ET can be worth trying before resorting to liver transplantation, which is resource-demanding and technique-challenging in small infants.

Introduction: Though predicting the emerging hyperbiluribinemia is the rational approach, timely exchange transfusion in significant neonatal hyperbilirubinemia is vital. The aim of this study is to evaluate the socio-demographic and clinical characteristics of newborns with significant hyperbilirubinemia, to evaluate risk factors for exchange transfusion and long-term neurodevelopmental status. Materials and Methods: Newborns who were admitted with bilirubin levels above the excahge transfusion thresholds (American Academy of Pediatrics, guideline 2004) total 104 cases were enrolled to the study. A total of 65 cases at 12-36 months were evaluated with Guide for Monitoring Child Development. The clinical and demographic characteristics, risk factors, treatment modalities, etiology of the groups and long-term neurodevelopmental status were compared between the groups with and without exchange transfusion, Results: Exchange transfusion was performed in 19 (18.3%) patients. Main factors that increase the risk of exchange transfusion were bilirubin level and bilirubin/albumin ratio. Cut-off bilirubin level and bilirubin/albumin ratio which increase the risk for exchage transfusion were 26.43mg/dl and 7.43mg/g respectively. The underlying etiologies were hemolytic disease (29.8%), dehydration and breast milk jaundice (28.8%), prematurity (26.9%). Late comers (postnatal >96 hours) were more likely to have bilirubin level ≥25 mg/dl. Development delays were detected in 13.8% of 65 cases. Conclusions: Admission bilirubin level and the ratio of bilirubin to albumin was among the factors that increase the risk of exchange transfusion. Late admission with significant hyperbiluribinemia may be prevented by identification of neonates at risk for developing significant hyperbilirubinemia before discharge or early in follow-up. [ABSTRACT FROM AUTHOR]

Bilirubin neurotoxicity involves a spectrum of varying severity that could result in adverse long-term sequelae. To compare the neurodevelopmental outcome of full-term neonates who underwent exchange transfusion with those who did not. A retrospective cohort study. This study included a retrospective review of records of sixty neonates who were matched in admission ages and serum bilirubin levels and the comparison groups were those who received an exchange transfusion (n = 30) versus those where exchange transfusion was planned, but the bilirubin levels dropped sufficiently during the period where the exchange blood was being prepared (n = 30). History, clinical examination, and laboratory investigations were documented. Neurodevelopmental outcome, at 6 months of age, using Bayley scales of infant development was assessed. The exchange group had statistically significant lower cognitive scores (p -value 0.005). The higher the rate of bilirubin decline, the better the language and motor scores in the phototherapy group (p -values 0.020 and 0.024 respectively). Infants with longer duration to exchange transfusion had lower cognitive, language, and motor scores (p-values 0.01, 0.001, and 0.003 respectively). Slower rates of bilirubin decline and longer duration before intervention increase the chances of adverse neurodevelopmental outcomes. • A slower rate of bilirubin clearance on phototherapy has adverse outcomes on motor and language development. • Delayed exchange transfusion beyond a cutoff point is associated with cognitive delay. • Total serum bilirubin does not predict adverse neurodevelopmental outcomes. • The rate of bilirubin decline and timing of intervention should be given a more predictive value for adverse outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Today, in the developed world, virtually all deaths due to Bordetella pertussis illnesses occur in young infants. Areas Covered: Pertussis in young infants is characterized by an afebrile cough illness with coryza, apnea, seizures, cyanosis, and emesis. Severe illness is associated with high leukocyte and lymphocyte counts, rapid respiratory and cardiac rates and pneumonia. Many routine intensive care treatment procedures are detrimental: these include steroids and nitric oxide. Preventative measures include: quarantine, prophylactic antimicrobial agents and immunizations of the mother to be with Tdap between 27 and 36 weeks gestation. Expert Opinion: Infants deaths are due to the irreversible pulmonary hypertension which is caused by aggregates of leukocytes in the small vessels in the lung. The leukocytosis with lymphocytosis is due to pertussis toxin. It can be treated by exchange blood transfusions. However for this to be successful it needs to be started before shock or organ failure has occurred. To prevent pertussis in young infants, attention needs to be directed to the diagnosis and treatment of pertussis in adolescents and adults. Also important are antimicrobial prophylaxis in the infant and the immunization of mothers to be with Tdap vaccine during all pregnancies.

Sclerema neonatorum (SN) is a rare clinical condition usually seen during the 1st week of life. It presents as hardening of the skin and has a high mortality rate. It is usually associated with congenital anomalies, hypothermia, respiratory illnesses, and sepsis. Defective lipolytic enzymes, a high melting point, and resultant low solidification point of saturated fatty acids of subcutaneous fat, edema of connective tissue septae, and signs of underlying systemic diseases are the various theories proposed for the development of SN. The skin biopsy shows the thickening of trabeculae, sparse inflammatory infiltrates of lymphocytes, histiocytes and multinucleate giant cells, and X-ray diffraction of a biopsy sample of SN shows crystals in affected sites. The systemic corticosteroids have been used by various authors with variable response. Use of exchange transfusion has recently shown good results in improving the outcome of SN.

Objective  The current study initiated to address the effect of glucose-6-phosphate dehydrogenase (G6PD) deficiency on the pathogenesis and the severity of neonatal hyperbilirubinemia (NHB). Study Design  A total of 100 newborns with moderate to severe indirect hyperbilirubinemia and 50 normal neonates without hyperbilirubinemia had been enrolled in the current case–control study. All enrolled neonates had been tested for ABO and Rh(D) blood grouping, Total serum bilirubin measurement, complete blood count, morphology, reticulocyte counts, direct Coombs' test, and G6PD enzyme assay. Results  From all enrolled hyperbilirubinemic neonates, 16% were G6PD deficient and this displays a statistically significant difference in comparison to controls (only 6% were G6PD deficient). Also, significant difference was found in the level of serum indirect bilirubin among G6PD-deficient neonate in comparison to G6PD nondeficient neonates which had contributed significantly to the difference in the duration of phototherapy and hospitalization among deficient neonate. Despite this, no significant difference found in the onset of presentation, reticulocytes count, and age of neonates between the two groups (G6PD-deficient and G6PD nondeficient neonates). Conclusion  The current study augments the etiological role of G6PD in the causation and severity of NHB in the region; however, in the absence of significant difference in the reticulocytes and the hemoglobin level, the underlying mechanism cannot be backed to the excess hemolysis alone. [ABSTRACT FROM AUTHOR]

The acute changes in brain function in newborn infants undergoing ET remain unclear. This study aimed to determine whether fully automated simultaneous peripheral arteriovenous ET would influence the brain function. A retrospective analysis was conducted on the clinical data of 39 neonates with hyperbilirubinemia who received ET. Seventeen patients were in the encephalopathy group, and the other 22 patients were in the non-encephalopathy group. Changes in amplitude-integrated electroencephalogram (aEEG) during ETs were analyzed, including background activities, sleep-wake cycling (SWC), and seizures. Before and after the ET, routine blood test parameters, electrolytes, blood glucose, and blood gas parameters were measured. After ETs, there were no significant changes in the levels of pH, PaO 2 , PaCO 2 , lactate, and red blood cells, while the levels of total bilirubin, indirect bilirubin, blood potassium, blood sodium, serum calcium, while blood cells, and platelets were significantly lower and the level of blood glucose was significantly higher compared to those before therapy. There was no significant difference in the changes of electroencephalographic activities during ETs, including background activities, SWC, and seizures. However, there were significant differences in suppressions on background activities, while there were no significant statistical differences in SWC or seizures between the 2 groups. Fully automated simultaneous peripheral arteriovenous ET is safe and efficient without significant influence on the disorder of the internal environment and electroencephalographic activities after ET in neonates. However, background activities are more significantly depressed in infants of bilirubin encephalopathy than infants of non-encephalopathy during ET. [ABSTRACT FROM AUTHOR]

Background: Blood exchange transfusion (ECT) therapy is an essential part of the modern health care for high-risk icteric neonates. Objectives: Therefore, this study sought to examine the effectiveness of monitoring sodium disorders and the required interventions indicating the overall state of health of hospitalized jaundiced neonates. Methods: This prospective analytic study was performed on 49 neonates diagnosed with severe jaundice undergoing screened exchange blood transfusion from November 2018 to May 2019. Serum sodium ion concentrations testing was performed before the exchange, then 1 - 3 hours later, and again 24 hours after receiving ECT, using a sensitive ELISA kit in a laboratory. Using the newest version of SPSS 24 software program, the association between different numerical variables was calculated by a repeated-measure ANOVA test. P < 0.05 criterion was set as the threshold of significance. Results: Out of the total 49 neonates, 24 (48.5%) were girls and 25 (51.5%) were boys, of which 93.9% and 6.1% were term and pretermborn neonates, respectively. The average birth weight was 2843 ± 267/510 g and the mean period of hospital stay was 4.55 ± 2.399 days. The various causes of severe neonatal jaundice of all the hospitalized cases were 45% unknown, 43% combined ABO and Rh incompatibility, 10% breastfeeding pattern, and 2% G6PD deficiency. A continual fall in serum sodium was observed following banked blood exchange (P < 0.05). However, these reversible changes were outside the expected normal range. Despite a diminished sodium concentration, the mean sodium levels were within the laboratory reference range of serum (137 - 142 mEq/L) in all the three measured duration times. Moreover, variation in the amount of serum sodium was associated with an unknown underlying cause that led to neonatal jaundice (P = 0.04) and this difference did not reach statistical significance in terms of birth weight, gestational age, and other causes accounting for high bilirubin (P > 0.05). Conclusions: Given these facts, it was concluded that neonatal jaundice was significantly associated with post-exchange serum sodium changes within the laboratory reference range of serum. [ABSTRACT FROM AUTHOR]

Objective: The frequency of neonatal exchange transfusion has declined in recent years, but is still performed in many countries. The procedure is associated with complications. The aim of the study was to determine the clinical features and etiologies of infants with hyperbilirubinemia who underwent exchange transfusion and evaluate the adverse events and clinical outcomes. Material and Methods: We performed a secondary analysis of the multicenter Turkish Neonatal Jaundice Online Registry data. Otherwise healthy newborns born ≥35 weeks of gestation who were hospitalized for jaundice and underwent exchange transfusion were included. Results: One-hundred thirty-two patients with a mean serum bilirubin level on admission of 24.9±9.1 mg/dL were enrolled in the study. The most common cause for exchange transfusion was hemolytic jaundice (63.6%), followed by lack of proper feeding (12.9%). It was found that the infants with lack of proper feeding were discharged earlier from the maternity ward (p=0.02), but they were admitted to hospital later (p<0.001) with a higher bilirubin level (p=0.001), and geater weight loss (p=0.04). The reported rate of adverse events associated with exchange transfusion was 11.4%. The most common complication was thrombocytopenia (40%). None of the infants died during the procedure. Acute bilirubin encephalopathy was reported in 13 (9.8%) patients. Conclusion: Severe hyperbilirubinemia requiring exchange transfusion and acute bilirubin encephalopathy are still challenging problems in neonatal periodin our country. The policies including blood group analysis of pregnant women, programs informing parents about breastfeeding and jaundice, and monitoring bilirubin levels of high-risk newborns should be developed to reduce the necessitating for exchange transfusion and to avoid related complications. [ABSTRACT FROM AUTHOR]

Streptococcus agalactiae or group B streptococcus (GBS) is a pathogen that causes severe neonatal infections, resulting in sepsis, pneumonia, and meningitis. Neonatal GBS meningitis has a poor neurological prognosis and a high mortality rate. GBS disease is classified as early- and late-onset if the onset age is 0-6 and 7-89 days after birth, respectively. There is currently no effective preventive strategy against late-onset GBS (LOGBS) disease. Here, we report a case of female infant with LOGBS meningitis who recovered from the septic shock by two exchange transfusions (ExTs) but still experienced severe neurological sequela. She was born at a gestational age of 39 weeks via caesarian section due to oligohydramnios and had fever 11 days after birth. GBS was detected in her cerebrospinal fluid (CSF) and blood but not in the vaginal or breast-milk cultures of the mother. The patient was treated with intravenous antibiotic administration; however, she suddenly developed pulseless ventricular tachycardia and asystole the next day. Her heart rate was normalized via cardiopulmonary resuscitation. We also performed two ExTs, and she recovered from the septic shock. Cytokine-profile analysis revealed that the serum and CSF levels of various pro-inflammatory and anti-inflammatory cytokines were elevated before the ExTs, after which the serum levels of several of these cytokines decreased. Two ExTs were effective in saving the life of the patient but did not improve the neurological prognosis. Given that neonatal GBS meningitis has high fatality and sequela rates; thus, it is necessary to establish a preventive strategy. [ABSTRACT FROM AUTHOR]

Our descriptive study examines the clinical profile of referred neonates who underwent exchange blood transfusion (EBT) and identifies possible interventions at peripheral hospitals to decrease their severe hyperbilirubinemia. Among the 38 neonates enrolled, the following were identified as potential clinical gaps in management: early discharge within 24 h of birth (57%); non-availability of ABORh blood grouping (43%); lack of anti-D immunoprophylaxis (75%); pathological weight loss because of inadequate breastfeeding (42%); and low usage of phototherapy. Because of late recognition, the mean age at admission was 5.4 ± 3.3 days, levels of total serum bilirubin (TSB) were 516.4 ± 123.1 µmol/L, and acute bilirubin encephalopathy (ABE) was seen in 45% of neonates. Rh iso-immunisation (39.5%), ABO iso-immunisation (21%) and sepsis (8%) were major risk factors for severe hyperbilirubinaemia. Quality prenatal screening identifying at-risk newborns, preventing early discharge after birth, a bilirubin nomogram risk assignment before discharge and assuring early recognition of hyperbiliubinaemia by parents may well minimise the incidence of EBT. [ABSTRACT FROM AUTHOR]

Glucose 6 phosphate dehydrogenase enzyme deficiency, is an x linked recessive, it presented with jaundice in neonatal period as acute hemolytic anemia. Early diagnosis and prompt treatment by phototherapy or combined with exchange transfusion are important to avoid occasional complication including kernicterus. To determine the frequency of G6PD deficiency among patients with neonatal jaundice and percentage of kernicterus. A cross sectional study was conducted at Babylon teaching hospital for maternity and children, for six months period starting from 1st of January 2019 to 30th of June 2019. A total 100 neonates (70 males and 30 females) with indirect hyperbilirubinemia were admitted and tested ( using methemoglobin reduction test and spectrophotometric method) to diagnose G6PD deficiency. The frequency of G6PD deficiency is (14%), its increment among male gender, lived in rural areas, with body weight less than 3.5 Kg, presented in first 5 days and its duration in 4 days. In conclusion, G6PD deficiency is a quite common in neonates, it may cause severe hyperbilirubinemia which may result neurological damage including kernicterus. [ABSTRACT FROM AUTHOR]

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Background: Neonatal hyperbilirubinemia is a common disease in neonates especially in early days of birth that requires a good and successful treatment for reducing the severity and its complications that can produce important and irreversible effects. Objectives: To evaluate the effectiveness of conventional phototherapy, intensive phototherapy and exchange transfusion on outcomes of neonatal jaundice at Fatima Al-Zahra Hospital for maternity and child care in Baghdad. Patients & Methods: A retrospective study was carried out using medical records of neonates with diagnosis of unconjugated jaundice, admitted in the septic neonatal care unit of Fatima Al-Zahra hospital over 6 months period between 1st May till 31st October 2018. The total serum bilirubin, fractionations and blood group were done in all cases. They treated with conventional phototherapy, intensive phototherapy and exchange transfusion according to the severity of jaundice. Results: Total neonates admitted from 1st may to 31st October 2018 in septic neonatal care unit were 1254, among them 432 (35%) were diagnosed as unconjugated neonatal jaundice “indirect hyperbilirubinemia”. Male: Female ratio (1.4:1), males 256(59.3%), females 176(40.7%). Physiological jaundice was the most common cause 129(29.9%) cases. Prematurity in 104(24.1%) and ABO incompatibility 59(13.7%) while Rh incompatibility 14(3.2%), sepsis 8(1.9%) and unknown causes of jaundice were 118(27.3%) because lack of lab facilities. Conventional phototherapy was the most common kind of treatment in 237(55%) while intensive phototherapy used in 175(40.3%) cases with successful reduction in T.S.B level and the rate of improvement without need for exchange transfusion (92%) (161/175).Only 20(4.5%) cases were treated with exchange transfusion especially for ABO incompatibility 8 (42.1%) cases and Rh incompatibility 4 (21.1%) cases. Most of neonates 429 (99.3%) discharged with complete improvement and only 2 (0.5%) neonates suffered from kernicterus and one death (0.2%). Conclusion: Conventional phototherapy is still the standard treatment of mild to moderate indirect hyperbilirubinemia. Use of intensive phototherapy in the treatment of unconjugated neonatal hyperbilirubinemia is effective in reducing T.S.B level, need for exchange transfusion and hospital staying. Recommendations: provide aseptic neonatal care unit in the hospital with further number of intensive phototherapy devices as it is so effective in treating unconjugated neonatal jaundice and reduces need for exchange transfusion as it is proven in the study.

At present, effective antibiotics and comprehensive symptomatic/supportive treatment as early as possible are mainly used for the treatment of severe pertussis in clinical practice. However, some children with severe pertussis have unsatisfactory response to commonly used drugs and treatment measures in the intensive care unit and thus have a high risk of death. Studies have shown that certain treatment measures given in the early stage, such as exchange transfusion, may help reduce deaths, but there is still a lack of uniform implementation norms. How to determine the treatment regimen for severe pertussis and improve treatment ability remains a difficult issue in clinical practice. This article reviews the advances in the treatment of severe pertussis, in order to provide a reference for clinical treatment and research. [ABSTRACT FROM AUTHOR]

Background: The association between parasite burden and end‐organ dysfunction in subjects with Babesia microti infection has not been extensively studied, nor has the optimal role of red blood cell exchange (RCE) transfusion in babesiosis treatment. This retrospective chart review evaluates the associations between parasitemia, end‐organ dysfunction, and outcomes in babesiosis patients treated with antimicrobial agents and RCE compared to those treated with antimicrobial agents alone. Materials and Methods: We evaluated adults (≥18 years of age) with laboratory‐confirmed babesiosis who were admitted between 2011 and 2017 to Yale New Haven Hospital, located in a Babesia‐endemic region of the Northeastern United States. Patient demographics, parasitemia levels, clinical and laboratory indicators of end‐organ dysfunction, and outcomes were examined. Results: Ninety‐one subjects (mean age 65.1 years, 69.2% male) were studied. Subjects were stratified according to peak parasitemia: <1% (n = 34), 1‐5% (n = 24), 5‐10% (n = 15), and >10% (n = 18). Laboratory measures indicating degrees of hemolysis, coagulopathy, and pulmonary, renal and hepatic dysfunction differed significantly across peak parasitemia levels. Median length of hospital stay increased with each successive peak parasitemia level (P <.001). These results indicate a strong association between peak parasitemia level and disease severity. Nineteen subjects underwent RCE, all with peak parasitemia ≥9% and some degree of end‐organ dysfunction. Conclusions: Babesia microti parasitemia is closely associated with disease severity, though not all subjects with end‐organ dysfunction had high‐grade parasitemia. Our data suggest that the use of parasitemia >10%, coupled with clinical status, is a reasonable indicator for RCE in babesiosis patients. [ABSTRACT FROM AUTHOR]

Background: Severe hyperbilirubinemia is potentially neurotoxic and can lead to long-term complications in neonates. Exchange transfusion (ECT) is one of the most important treatments for hyperbilirubinemia. In this regard, the present study aimed to determine the characteristics of hyperbilirubinemic neonates who need ECT and their mothers. Methods: This cross-sectional study was performed on 380 infants born after 35 weeks of gestation who were 2-14 days old. The studied neonates had bilirubin levels higher than 17 mg/dl and underwent ECT in Ghaem Hospital in Mashhad, Iran during 2010-20. Moreover, it should be noted that the participants were selected using the convenience sampling method and the required data were collected using a checklist. This checklist was designed based on the neonatal examination, maternal (maternal age, parity), and neonatal status (age, gender, and weight) and serial laboratory tests before and after ECT (total bilirubin, hematocrit, and platelet). Finally, these variables were compared based on the cause of hyperbilirubinemia. Results: The mean levels of serum bilirubin were 28.5 mg/dl and 26.5 mg/dl in male and female infants (P=0.096), respectively. Furthermore, the mean levels of serum bilirubin in neonates born by cesarean section and normal vaginal delivery were 29.5 and 28.1 mg/dl, respectively (P=0.458). Based on the findings, 60% of the neonates suffered from weight loss and 22% had more than 3% daily weight loss. In the present study, the most prevalent risk factors among the studied neonates were RH incompatibility, ABO incompatibility, and G6PD deficiency, in that order. Conclusion: Overall, these findings suggest that normal vaginal delivery, repeated breastfeeding, prevention of severe weight loss, early detection of RH and ABO incompatibility, and G6PD deficiency, as well as appropriate management of hyperbilirubinemia, can reduce the need for ECT and alleviate complications of neonatal hyperbilirubinemia. [ABSTRACT FROM AUTHOR]

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

Summary: Objectives: To establish, in an unselected population of London haemoglobinopathy patients, transfusion requirements, blood antigens/alloantibodies, transfusion modalities, burden of transfusion reactions and donor exposure. Background: Haemoglobinopathy patients are among the most highly transfused patient populations, and the overall population and number of patients on long‐term transfusion programmes are increasing. To provide a safe and efficacious transfusion service for patients, it is important to understand current practice, morbidity associated with transfusion, efficacy of different transfusion modalities and geno‐/phenotype requirements. Methods: Data on 4451 transfusion episodes in 760 patients from 12 London hospitals were collected retrospectively over a 6‐month period in 2011. Results: Alloimmunisation prevalence was 17% for sickle cell disease (SCD) and 22% for thalassaemia, most commonly anti‐Rh/Kell/Kpa/Cw. Rh phenotypes differed between SCD (Ror 59.8%/R1r 15.9%/R2r 15.6%) and thalassaemia (R1R1 29.6%/R1r 28.4%/R1R2 15.4%). Recording of pheno‐/genotypes fell below recommendations. A 2‐weekly manual exchange and 3‐weekly automated exchange came closest to achieving presumptive targets. In adults with thalassaemia, the mean blood requirement was 36 units per year; for SCD, erythrocytapheresis was carried out every 7 weeks with 66 units; for manual exchange, it was 38 units every 4 weeks; and for simple transfusion, it was 30 units p.a. every 4 weeks. Conclusion: Transfusion modality choice was influenced by the resources available—children mostly received simple transfusions, and adults received erythrocytapheresis; the relationships between frequency of exchanges/transfusion modality/target HbA% were not simple, possibly reflecting the difference in recipient erythropoiesis and consequent transfusion modality selection bias; adherence to existing and current guidelines regarding geno‐/phenotyping was limited; and alloimmunisation had a low incidence and high prevalence in both disorders. [ABSTRACT FROM AUTHOR]

A 17-day-old premature baby girl had received a blood exchange transfusion because of hyperbilirubinemia and got another blood transfusion because of severe anemia on day 45. The diagnosis of transfusion vivax malaria was made when she had severe anemia again on day 78. The most predominant clinical signs were fever, anemia, hepatosplenomegaly, and thrombocytopenia. Treatment with chloroquine 25 mg base/kg BW showed resistance at RIII level on a 7 -day follow up. She was retreated. with quinine 10 mg salt/age in month divided in 3 doses/day for 7 days. lt also showed resistance at late RI level on day-30. Then she was retreated with quinine 15 mg salt/age in month divided in 3 doses/day for 7 days and still showed resistance at late Rl level on day 32. Finally she was treated with quinine 10 mg salt/kg BW /dose, tid for 7 days which was effective. During the course of treatment, no adverse reactions were found clinically. This malaria case was transfusion vivax malaria resistant to choloroquine at R III level and to quinine at late RI level. Quinine 10 mg salt/BW I dose tid for 7 days was effective and safe for infants.

Manar Al-Lawama, Eman Al-Rimawi, Rawan Al-Shibi, Eman Badran Department of Pediatrics, The University of Jordan, School of Medicine, Amman, Jordan Introduction: Severe neonatal hyperbilirubinemia can cause mortality and serious morbidities. When phototherapy fails, neonates with severe hyperbilirubinemia should undergo double volume blood exchange transfusion (BET). As this procedure carries a significant risk of mortality and morbidity, adopting guidelines for the treatment of neonatal hyperbilirubinemia is critical to avoid hyperbilirubinemia toxicity and also the complication of an unindicated procedure. Methods: This study investigated the causes, complications, and trend of BET rate in our unit over a 13-year period. The medical charts and laboratory databases of all infants who underwent BET in Jordan University Hospital between 2003 and 2015 were retrospectively reviewed. Results: The rate of exchange cases decreased significantly after adopting the guidelines of American Academy of Pediatrics (P12 months of age. Conclusions: This study showed a clear decline in the rate of BET after implementing the guidelines of American Academy of Pediatrics. In addition to improving the strategies for the identification and follow-up of at-risk newborns, we should intensify our efforts to prevent the progression of neonatal hyperbilirubinemia to the exchange level by enhancing parents’ ­awareness of this potentially harmful neonatal condition. Keywords: neonate, exchange transfusion, hyperbilirubinemia, guidelines, Jordan

Abstract Background This study was designed to evaluate the efficacy of exchange transfusion in patients with severe imported falciparum malaria. Twelve patients who met the diagnostic criteria for severe malaria were treated with exchange transfusion 14 times according to a conventional anti-malarial treatment. This study evaluated the efficacy of exchange transfusion for severe imported falciparum malaria. Methods Clinical data of severe imported falciparum malaria patients admitted to the intensive care unit (ICU) of Nantong Third People’s Hospital from January 2007 to December 2016 were investigated in this retrospective study. Patients were divided into the intervention group, which received exchange transfusion, and the control group. This study assessed parasite clearance and outcomes of the two groups, and levels of erythrocytes, haemoglobin, platelets, coagulation, liver function, lactate, C-reactive protein, and procalcitonin, before and after exchange transfusion in the intervention group. Results There was no significant difference in the severity of admitted patients. Exchange transfusion was successfully applied 14 times in the intervention group. Differences in the levels of erythrocytes, haemoglobin and platelets did not reach statistical significance. Exchange transfusion improved coagulation, liver function, lactic acid, C-reactive protein, and procalcitonin. No differences were observed in parasite clearance, ICU and hospital length of stay, in-hospital mortality, and costs of hospitalization between the two groups. Conclusion Exchange transfusion as adjunctive therapy for severe malaria was observed to be safe in this setting. Exchange transfusion can improve liver function and coagulation and reduce inflammation, but it failed to improve parasite clearance and the outcomes of severe imported falciparum malaria in this case series.

ABO incompatibility and other alloantibodies have emerged as a significant cause of hemolytic disease of fetus and newborn (HDFN), leading to neonatal morbidity and mortality. We report three cases of severe ABO-HDFN where blood exchange transfusions (ETs) were required in neonates with hyperbilirubinemia. Blood grouping (ABO/RhD) was performed using conventional tube technique. The antibody screen was done using commercial three-cell panel (Bio-Rad ID-Diacell-I-II-III, Switzerland) by gel technique. Direct antiglobulin test (DAT) on neonatal sample and compatibility testing were also done by gel technique. Elution on DAT-positive sample was performed using “heat elution” method. All the three neonates were A RhD positive and were born to O RhD-positive mothers who were negative for antibody screen. Their DAT was positive (2+) and the elution of neonatal red cells yielded a positive reaction with A cells which was suggestive of anti-A antibody. The maternal anti-A (immunoglobulin G) antibody titers were high: 512 and 1024 (in two cases). The total serum bilirubin (mg/dl) of the three neonates was 22, 27, and 25 which came down significantly after they received ETs. Severe ABO-HDFN may occur in neonates born to mother with high titer ABO antibodies which can be effectively managed with ET.

Hyperleukocytosis in pertussis has a correlation with disease severity. We describe a 5-week-old infant with severe pertussis and hyperleukocytosis requiring mechanical ventilation. Baby developed refractory hypoxemia. Two cycles of exchange transfusion were done on different occasions. Exchange transfusion resulted in significant improvement in oxygenation and hemodynamic parameters. Exchange transfusion is a viable treatment option in pertussis hyperleukocytosis complicated by refractory hypoxemia or persistent features of Systemic Inflammatory Response Syndrome.

Children with erythropoietic porphyria are generally under the care of paediatric dermatologists. When these children undergo major surgery, they are at risk of unusual complications due to their photosensitivity. Dermatologists may be consulted prior to surgery for advice. We describe a case of a child with erythropoietic porphyria undergoing open heart surgery, utilising an exchange transfusion alongside other strategies to minimise the risk of photosensitivity‐induced haemolysis. [ABSTRACT FROM AUTHOR]

The widespread use of anti-D immunoglobulin has resulted in a relative increase in the importance of non-D alloimmunization as a cause of hemolytic disease of the fetus and newborn (HDFN). Non-D alloantibodies that are capable of causing severe HDFN include anti-K, anti-E, and anti-c. Anti-c is clinically the most important Rh system antibody after anti-D. Here, we report three cases of neonates presenting with anemia and hyperbilirubinemia with strongly positive direct antiglobulin test who required phototherapy and neonatal exchange transfusion due to non-D antibody in RhD positive antenatal women. Anti-c was common in all the three cases while two cases have one additional non-D antibody. Due to faulty practices, antenatal antibody screening was not done for any case considering the mother's RhD positive status. Hence, antenatal antibody screening should be performed routinely, in all RhD positive pregnant women to reduce the delay in diagnosis and the management of HDFN occurring due to non-D antibodies. [ABSTRACT FROM AUTHOR]

Objective　To explore the difference in the incidence of complications and hospitalization time between exchange transfusion combined with intensive phototherapy and single intensive phototherapy in the treatment of neonatal hyperbilirubinemia. Methods　We retrospectively analyzed the data of 121 hyperbilirubinemia in term infants. We compared the difference in the incidence of complications and the hospitalization time between the exchange transfusion combined with intensive phototherapy and single intensive phototherapy in the treatment of neonatal hyperbilirubinemia. We also studied the incidences of anemia when we use different volume ratios of red blood cells and plasma. Results　There were more complications in the group of exchange transfusion combined with intensive phototherapy than the intensive phototherapy group， otherwise there was no difference in the hospitalization time between the two groups. The incidence of anemia of the 1:1 exchange transfusion group was higher than that of the 2:1 exchange group. Conclusions　All neonates with hyperbilirubinemia should be considered phototherapy for 4 hours or 6 hours and reassess whether they need exchange transfusion therapy to reduce the incidence of complications. If the exchange transfusion therapy is used， it is suggested that the volume ratios of red blood cells to plasma is 2:1 rather than 1:1. [ABSTRACT FROM AUTHOR]

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Background: Jaundice is the most common condition that requires medical attention in newborns. The yellow coloration of the skin and sclera in newborns with jaundice is the result of accumulation of unconjugated bilirubin. Objectives: To assess the efficacy of intensive phototherapy as a treatment modality for neonatal hyperbilirubinemia. Subjects and methods: This was A prospective, cohort, controlled study that was conducted on 100 babies with neonatal jaundice. They were admitted to Luxor General Hospital NICU at the beginning of November 2018 to the end of October 2019. Those 100 cases had been divided into 2 groups: the first group received intensive phototherapy sessions as a treatment modality for neonatal jaundice and the second group received conventional phototherapy. Results: The results of the study revealed that intensive phototherapy had succeeded in decreasing the need for exchange transfusion in 39 cases (78 %), whereas 11 cases (22%) remained for exchange transfusion (p value (0.001) was highly significant). Conclusion: The use of intensive phototherapy in the treatment of indirect pathological hyperbilirubinaemia is as effective as exchange transfusion in lowering total serum bilirubin when its level is within 2-3 mg/dl (34-51 µmol/l) of the exchange level and it is effective in reducing needs for exchange transfusion and duration of phototherapy. [ABSTRACT FROM AUTHOR]

Background: Phototherapy and exchange transfusion (ECT) are the mainstay treatments in neonatal hyperbilirubinemia. However, ECT is associated with morbidity and mortality. Hence, there is need to reduce the requirement of ECT. Objective: To compare and assess the efficacy of intensive phototherapy and conventional phototherapy in decreasing the need for ECT in neonatal hyperbilirubinemia. Methods: I n this prospective comparative study 720 term (>37 weeks) neonates diagnosed with indirect hyperbilirubinemia were selected and allocated to study group (N=366) and control group (N=354). The study group received intensive phototherapy and controls received conventional phototherapy. ECT was performed in neonates unresponsive to phototherapy. Demographic data and clinical data such as total serum bilirubin (TSB), complete blood count (CBC), blood group and reticulocyte count were collected. The data were analyzed by SPSS 20.0. Mann Whitney U test, Odds ratio and proportion test were used to test statistical significance. Results: ABO incompatibility was the most common reason for hyperbilirubinemia in the study. The odds of study group undergoing ECT is 20.23% less than the control group (P<0.05), which shows that intensive phototherapy had reduced the need for ECT. There was considerable reduction in TSB in study group (12.11 ±0.793 mg/dl) than control group (14.86± 1.53 mg/dl) (P<0.001) and also the duration of phototherapy was considerably lower in study group (2.63±0.48 days) than the controls (3.49±0.50 days). Conclusion: Hence, it can be concluded that intensive phototherapy is more effective at reducing the need for ECT than conventional phototherapy in term neonates with indirect neonatal hyperbilirubinemia. [ABSTRACT FROM AUTHOR]

Background: Bilirubin is toxic substance to the central nervous system of neonates, so when neonates suffer from sever hyperbilirubinemia they need to be treated with phototherapy and even exchange transfusion. Objectives: is to detect the effects of intravenous infusion of human albumin before double volume exchange transfusion in rapid reduction of total serum bilirubin after exchange and in decreasing the risk of second time exchange. Patients and methods: A randomized control trial study done between January and December 2017 on fifty three neonates admitted to neonatal care unit of AL-Zahraa Teaching Hospital at AL-Najaf that fulfill the inclusion criteria and exclusion criteria. So two groups enrolled in the study as control group(n=29) and albumin group (n=24), the albumin group receive albumin 1g/kg 20% one hour prior to exchange transfusion while in control group exchange transfusion only without albumin used. Results: A significantly lower mean post exchange total serum bilirubin in albumin group than that in control group at 6,12and 24 hours as p-value< 0.001,the mean of phototherapy duration significantly decreased in test group in comparison to control group (8.6±0.96hoursvs 17.9±3.8hours) respectively as p-value<0.001, also none of albumin group need second time exchange while three cases in control group underwent second time exchange with no side effects related to albumin use noticed. Conclusion: We conclude that Albumin administration before exchange blood transfusion can effectively reduces total serum bilirubin, period of phototherapy and greatly minimize risk of another exchange transfusion. [ABSTRACT FROM AUTHOR]

Despite advancement in medical care, Rh alloimmunisation remains a major cause of neonatal hyperbilirubinaemia, neuro-morbidity, and late-onset anaemia. Delayed cord clamping (DCC), a standard care now-a-days, is yet not performed in Rh-alloimmunised infants due to paucity of evidence. Hence, we randomised these infants of 28- to 41-week gestation to delayed cord clamping (N = 36) or early cord clamping (N = 34) groups. The primary outcome variable was venous packed cell volume (PCV) at 2 h of birth. The secondary outcomes were incidence of double volume exchange transfusion (DVET) and partial exchange transfusion (PET), duration of phototherapy (PT), functional echocardiography (parameters measured: superior vena cava flow, M-mode fractional shortening, left ventricular output, myocardial perfusion index, and inferior vena cava collapsibility) during hospital stay, and blood transfusion (BT) until 14 weeks of life. Neonates were managed as per unit protocol. The baseline characteristics of enrolled infants were comparable between the groups. The median (IQR) gestation and mean (SD) birth weight of enrolled infants were 35 (33-37) weeks and 2440 (542) g, respectively. The DCC group had a higher mean PCV at 2 h of life (48.4 ± 9.2 vs. 43.5 ± 8.7, mean difference 4.9% (95% CI 0.6-9.1), p = 0.03). However, incidence of DVET and PET, duration of PT, echocardiography parameters, and BT until 14 weeks of postnatal age were similar between the groups.Conclusion: DCC in Rh-alloimmunised infants improved PCV at 2 h of age without significant adverse effects.Trial registration: Clinical Trial Registry of India (CTRI), Ref/2016/11/012572 http://ctri.nic.in/Clinicaltrials, date of trial registration 19.12.2016, date of first patient enrolment 1 January 2017.What is Known:•Delayed cord clamping improves haematocrit, results in better haemodynamic stability, and decreases the need of transfusion in early infancy.•However, due to lack of evidence, potential risk of hyperbilirubinaemia, and exacerbation of anaemia (following delayed cord clamping), early cord clamping is the usual norm in Rh-alloimmunised infantsinfants.What is New:•Delayed cord clamping in Rh-alloimmunised infants improves haematocrit at 2 h of life without any increase in incidence of serious adverse effects. [ABSTRACT FROM AUTHOR]

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

Background/aim: Severe neonatal hyperbilirubinemia is an important cause of morbidity and mortality in developing countries. The aim was to assess etiologic reasons for development of severe hyperbilirubinemia and define risk factors for exchange transfusion and acute bilirubin encephalopathy (ABE) in Şanlıurfa located in the southeast region of Turkey. Materials and methods: An observational cohort study included 115 infants with ≥35 weeks of gestation admitted with diagnosis of severe hyperbilirubinemia in a period of 18 months. Potential risk factors associated with exchange transfusion and development of ABE were analyzed. Results: Among 115 infants, 67 (58.3%) received exchange transfusion and 45 (39.1%) developed ABE. Rh isoimmunization (OR: 24.6, 95% CI = 2.2-271, P = 0.009), glucose-6-phosphate dehydrogenase deficiency (G6PD) (OR: 21.1, 95% CI = 1.8-238.4, P = 0.01), early discharge (OR: 14.4, 95% CI = 4.2-48.9, P ≤ 0.001), and male sex (OR: 4.3, 95% CI = 1.3-14.1, P = 0.02) were independently associated with an increased risk for exchange transfusion. Being a refugee (OR: 6.8, 95% CI = 1.8-25.8, P = 0.005) and G6PD deficiency (OR: 9.9, 95% CI = 1.3-71.9, P = 0.02) were associated with development of ABE. Conclusion: Early discharge, Rh isoimmunization, and G6PD deficiency are significant risk factors for severe hyperbilirubinemia and exchange transfusion. Prevention of early hospital discharges, family education to increase awareness for hazardous effects of hyperbilirubinemia, and early follow-up visits after discharge would reduce the disease burden. [ABSTRACT FROM AUTHOR]

Purpose: Acute liver failure (ALF) and acute on chronic liver failure (ACLF) are associated with significant mortality and morbidity. Extracorporeal liver support (ECLS) devices have been used as a bridge to liver transplant; however, the efficacy and safety of ECLS are unclear. We conducted a systematic review and meta-analysis of randomized controlled trials (RCTs) to examine the efficacy and safety of ECLS in liver failure.Methods: We searched MEDLINE, EMBASE and Cochrane Central Register of Controlled Trials from inception through March 13, 2019. RCTs comparing ECLS to usual care in ALF or ACLF were included. We used the Grading of Recommendations Assessment, Development and Evaluation approach to assess the certainty of the evidence.Results: We identified 25 RCTs (1796 patients). ECLS use was associated with reduction in mortality (RR 0.84; 95% CI 0.74, 0.96, moderate certainty) and improvement in hepatic encephalopathy (HE) (RR 0.71; 95% CI 0.60, 0.84, low certainty) in patients with ALF or ACLF. The effect of ECLS on hypotension (RR 1.46; 95% CI 0.98, 2.2, low certainty), bleeding (RR 1.21; 95% CI 0.88, 1.66, moderate certainty), thrombocytopenia (RR 1.62; 95% CI 1.0, 2.64, very low certainty) and line infection (RR 1.92; 95% CI 0.11, 33.44, low certainty) was uncertain.Conclusions: ECLS may reduce mortality and improve HE in patients with ALF and ACLF. The effect on other outcomes is uncertain. However, the evidence is limited by risk of bias and imprecision, and larger trials are needed to better determine the effect of ECLS on patient-important outcomes. [ABSTRACT FROM AUTHOR]

Objective: To study the contribution of blood transfusion management in the improvement of maternal and perinatal outcomes in pregnant women with sickle cell disease in Ouagadougou.Methods: A cross-sectional retrospective study with data collected from February 2012 to January 2014 was used. Patients were differentiated into three groups: patients with at least one exchange transfusion, patients who received blood transfusion, and patients who did not receive any transfusion. Data were collected from patients' patient care documents.Results: One hundred and sixty-four patients were included, of whom 53 were in the first group, 32 in the second group, and 79 in the third group. Maternal complications in the last trimester of pregnancy were significantly less important (P=0.000) in the first group (58.5%) than in the second (78.5%) and third group (91.1%). The same trend was observed for postpartum maternal mortality (5.7%; 12.5%; 12.6%; P=0.009). Fetal complications such as preterm birth and early neonatal death were lower in the first group (15.1%; 1.8%) than in the second (40.6%; 23.1%) and third group (32.9%; 7.6%).Conclusion: Prophylactic blood transfusion is an important part of the management of pregnant patients with sickle cell disease. [ABSTRACT FROM AUTHOR]