Exchange Blood Transfusion Research Articles

Direct-Push Blood Transfusion Practice in the Neonatal Unit of a Tertiary Hospital in South-South, Nigeria

Introduction: Transfusion of blood is a life-saving intervention in the care of ill neonates. Donated blood is a scarce national resource and must be used in the most efficient way. Exchange blood transfusion using the blood bag is the commonest mode of blood delivery employed. Other modalities of safe and sustainable blood delivery should also be explored, especially where paucity of funds predominates. This study aims to assess the usefulness of the direct push method where applicable, as an alternative to blood bag delivery in neonatal units of resource poor settings.
 Methods: A two year retrospective study of newborns admitted in the neonatal wards of the University of Uyo Teaching Hospital. Data obtained were the age, gender, indication for admission, packed cell volume (PCV) before and after transfusion. Blood transfusion was done in aliquots over 24 hours under aseptic conditions, via a peripheral vein. The push and pull method was employed, with no anticoagulant in the syringe. Post-transfusion PCV was done at least 24 hours after the procedure.
 Results: Of the one thousand and seventy-seven (1077) admitted neonates, two hundred and thirty-nine (22.2%), received blood products. Of these, twenty-one (8.8%), received a direct whole blood transfusion. Age (days) of the neonates transfused ranged from 1 to 26 days, with a mean of 10.4 ± 8.13. The Packed Cell Volume (PCV) pre-transfusion ranged between 20% - 44%, with a mean of 30.05 ± 6.39 while post-transfusion PCV ranged between 31% to 51%, with a mean of 38.17 ± 5.52(Fig. 1). The commonest indication for transfusion was prematurity, 9(42.8%) and neonatal sepsis 5 (23.8%).
 Conclusion: The direct transfusion of blood occasionally used, seems a relatively safe practice to correct mild/moderate anaemia. It also provides sufficient blood, with the advantage of usage when the umbilical cord access is no longer feasible and where cost of blood would otherwise, hinder quick intervention. This practice may need further evaluation by other centers.

Trial in Progress: The Randomized, Double-Blind, Placebo-Controlled Phase Ib CROSSWALK-a Trial Evaluating the Safety of Crovalimab for the Management of Acute Uncomplicated Vaso-Occlusive Episodes (VOEs) in Patients with Sickle Cell Disease (SCD)

BackgroundSCD is a group of autosomal recessive red blood cell (RBC) disorders caused by a single point mutation in the β-globin gene, resulting in the production of hemoglobin S. Hemoglobin S polymerizes within RBCs under certain conditions, leading to the distortion of the RBC membrane and generation of dense and sickle RBCs. These pathologic RBCs contribute to microvascular occlusions in patients with SCD, which present as acute painful episodes called VOEs. Despite the majority of VOEs being managed at home, they remain the most common reason for emergency department (ED) visits and hospitalization among patients with SCD, with > 70% of ED visits and > 90% of hospital admissions for SCD being related to VOEs (Ballas et al. Am J Hematol 2005; Lanzkron et al. Am J Hematol 2010). One of the most severe complications of VOEs is acute chest syndrome, which is a leading cause of mortality among patients with VOEs (Vichinsky et al. N Engl J Med 2000; Bartolucci et al. eBioMedicine 2016). In the absence of targeted therapies for the management of VOEs in patients with SCD, treatment is currently limited to pain management, blood exchange transfusion (with the risk of complications), and other supportive care, representing a significant unmet medical need. Activation of the complement pathway has been described in patients with SCD at baseline, in acute pain crises, and in patients with delayed hemolytic transfusion reaction. Accumulating nonclinical data have suggested a multimodal role for complement dysregulation in the pathophysiology of SCD including vaso-occlusion, hemolysis, inflammation, thrombogenicity, endothelial activation, and end-organ damage (Roumenina et al. Am J Hematol 2020). Crovalimab is a novel, engineered, anti-complement C5 monoclonal antibody. In a Phase I/II study (Röth et al. Blood 2020) in patients with paroxysmal nocturnal hemoglobinuria, a complement-mediated disorder, crovalimab demonstrated rapid and sustained complement inhibition with promising efficacy and safety.Study Design and MethodsCROSSWALK-a (NCT04912869) is a randomized, double-blind, placebo-controlled, Phase Ib study evaluating the safety of crovalimab for the management of acute uncomplicated VOEs in patients with SCD. Patients aged ≥ 12 years to ≤ 55 years, weighing ≥ 40 kg, and with a confirmed diagnosis of SCD homozygous hemoglobin S (HbSS) or sickle cell β 0 thalassemia (HbSβ 0) are eligible for the study (Figure). Patients must present with an acute uncomplicated VOE, requiring hospitalization and treatment with parenteral opioid analgesics. Vaccinations against Neisseria meningitidis, Hemophilus influenzae type B, and Streptococcus pneumoniae must be up to date. Eligible patients will be randomized 2:1 to receive either a single intravenous weight-based tiered dose of crovalimab or placebo. Patients in both study arms will continue to undergo pain management and receive other supportive care for their VOE and may also continue to receive ongoing concurrent SCD-directed therapies. Patients will be followed during the hospitalization until discharge and will continue to be followed post-discharge during an observational period. The maximum total study duration for an individual patient will be 12 weeks, which includes the hospitalization and observational periods. The primary objective is to evaluate the incidence and severity of adverse events according to National Cancer Institute Common Terminology Criteria for Adverse Events, Version 5.0, incidence and severity of infusion-related reactions and hypersensitivity, and change from baseline in targeted vital signs and clinical laboratory test results. Efficacy, pharmacokinetics, pharmacodynamics, immunogenicity, and exploratory biomarker endpoints will also be evaluated. [Display omitted] DisclosuresBartolucci: F. Hoffmann-La Roche Ltd: Consultancy; Bluebird: Consultancy, Research Funding; Emmaus: Consultancy; Hemanext: Consultancy; Jazz Pharma: Other: Lecture fees; AGIOS: Consultancy; GBT: Consultancy; Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees, Other: Lecture fees, Steering committee, Research Funding; INNOVHEM: Other: Co-founder; Fabre Foundation: Research Funding; Addmedica: Consultancy, Other: Lecture fees, Research Funding. Ataga: Forma Therapeutics: Membership on an entity's Board of Directors or advisory committees; Novo Nordisk: Membership on an entity's Board of Directors or advisory committees; F. Hoffmann-La Roche Ltd: Consultancy; Novartis: Membership on an entity's Board of Directors or advisory committees; Agios Pharmaceuticals: Consultancy; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees. Callaghan: Alnylum: Current equity holder in publicly-traded company; Roche/Genentech: Consultancy, Speakers Bureau; Takeda: Consultancy, Speakers Bureau; Sanofi: Consultancy; Pfizer: Consultancy; Global Blood Therapeutics: Consultancy, Speakers Bureau; uniQure: Consultancy; Spark: Consultancy; Biomarin: Consultancy; Kedrion: Consultancy; Hema Biologics: Consultancy; Forma: Consultancy; Chesei: Consultancy; Agios Pharmaceuticals: Current Employment. De Franceschi: F. Hoffmann-La Roche Ltd: Consultancy. Minniti: F. Hoffmann-La Roche: Consultancy; Bluebird Bio: Other: Endpoint adjudicator; Forma: Consultancy; GBT: Consultancy; Novo Nordisk: Consultancy; CSL Behring: Other: Endpoint adjudicator; Novartis: Consultancy; Chiesi: Consultancy. Alexandrou: F. Hoffmann-La Roche Ltd: Consultancy, Current equity holder in publicly-traded company; Pfizer: Current equity holder in publicly-traded company. Imbs: F. Hoffmann-La Roche Ltd: Consultancy; Certara Inc.: Current Employment. Fox: Parexel International: Current Employment; Genentech, Inc.: Current Employment. Patel: Genentech, Inc.: Current Employment. Sostelly: F. Hoffmann-La Roche Ltd: Current Employment.

S2621 When Do You Use an Aggressive Exchange Blood Transfusion Strategy to Treat Acute Hepatic Sequestration in Sickle Cell Disease?: A Case Report

S2621 When Do You Use an Aggressive Exchange Blood Transfusion Strategy to Treat Acute Hepatic Sequestration in Sickle Cell Disease?: A Case Report

Коклюш: мета терапії та можливості профілактики

Статтю присвячено питанням раціональної терапії та імунопрофілактики коклюшу. Показано, що антибіотики можуть придушити кашель тільки в інкубаційному періоді та на стадії продроми за рахунок ерадикації B.pertussis. У спазматичній стадії коклюшу головною метою є боротьба з гіпоксією, а не придушення кашлю або ерадикація збудника. Зроблено акцент на значенні гіперлейкоцитозу в периферичній крові як однієї з цілей терапії тяжкого коклюшу в дітей раннього віку за допомогою лейкоферезу або замінного переливання крові. Одним із способів зниження захворюваності на коклюш є введення в календар імунопрофілактики обов’язкової ревакцинації старших дітей і дорослих.

Low‐cost alternatives for the management of Guillain‐Barré syndrome in low‐ and middle‐income countries

AbstractGuillain‐Barré syndrome (GBS) is the most common cause of acute flaccid paralysis. It can cause rapidly progressing ascending paralysis leading to respiratory failure. There is no definite treatment for GBS, but early administration of intravenous immunoglobulin or therapeutic plasma exchange accelerates motor recovery and decreases time on the ventilator support and are considered the standard of care in GBS. However, these treatments are often unaffordable for many patients in low‐ and middle‐income countries, with limited availability and specialized expertise required in prescription. Cheaper alternatives such as modified therapeutic plasma exchange, exchange blood transfusion, rituximab, and pulse steroid therapy have been practiced in different parts of the world and have been shown to be effective, albeit with limited evidence. These cost‐effective therapies warrant further research and should be strongly considered by relevant health authorities and policymakers as a potential primary treatment for GBS in resource‐limited settings.

Late Onset Neonatal Sepsis Due to Hafnia alvei in Three Neonates at the University of Nigeria Teaching Hospital, Enugu, Nigeria

Background: Hafnia alvei is a Gram-negative motile bacillus, belonging to the Enterobacteriaceae family. It is commonly found in stool of humans and animals and soil. It is very rare in neonates. This organism is resistant to most commonly used antibiotics, and causes nosocomial outbreaks with significant mortality. Therefore, awareness regarding this organism is important to improve outcome.
 Presentation of Cases: We report three cases of late onset neonatal sepsis caused by Hafnia alvei. Of these, two were preterm while one was term. The term neonate was referred on account of perinatal asphyxia and developed fever, reduced activity, tachycardia, and tachypnoea 5 days into admission. The two preterm neonates were delivered at the index hospital and developed hypothermia, and other symptoms on the 4th day of life. Two sets of blood cultures were done for each case after collecting blood from two different sites. Blood culture was done using BACT/ALERT 3D (BioMerieux, Marcy I’Etoile, France) which uses a colorimetric sensor and reflects light to monitor the presence of carbon dioxide produced by micro-organisms. This yielded Hafnia alvei in all three samples after 72 hours of incubation. Gram stain done showed presence of Gram negative bacilli and subculture was done on MacConkey and 5% sheep agar incubated at 37 0 C for 18-24 hours. Identification of isolates was done with standard biochemical test and confirmed with API 20 E identification system (BioMerieux, Marcy I’Etoile, France). Antibiotic sensitivity was done using the modified Kirby Bauer disc diffusion method (Oxoid, Cambridge, UK). At most five antibiotic discs were used for each isolate and these were incubated at 370C for 24 hours. Isolates were sensitive to fluoroquinolones, cefepime and meropenem. Patients received intravenous antibiotics for two weeks, phototherapy and exchange blood transfusion. They were subsequently discharged and are currently on follow up.
 Discussion and Conclusion: Though rare, three infections with H. alvei were reported in this study. The isolates were sensitive to fluoroquinolones, cefepime and meropenem and resistant to amoxicillin/clavulanic acid, piperacillin/tazobactam. The outcomes were improved by a high index of suspicion, early diagnosis, prompt institution of appropriate antibiotics and supportive care.

Pattern of Blood Transfusion in the Special Care Baby Unit of Benue State University Teaching Hospital in Makurdi North-Central Nigeria

Blood transfusion is a life-saving procedure in which whole blood or parts of blood are introduced into a patient's bloodstream intravenously. The need maybe due to physiological or pathological causes. The objective of this study was to document the pattern and indications for blood transfusion at the Special Care Baby Unit of the Benue State University Teaching Hospital (BSUTH), Makurdi. This study was a retrospective review of 126 neonates who received blood transfusion at the Special care baby unit of BSUTH from 1st January 2015 to 31st December 2019. Data was retrieved from patient's medical records and analyzed using IBM SPSS version 23. A total of 1142 neonates were admitted over the study period and 126 had blood transfusion giving a prevalence of 11.0%. Male/Female ratio of 1.57:1.Mean (±SD) Birth weight and gestational age (GA) were 2.23±0.82Kg and 35.48 ±3.95 weeks respectively. Exchange blood transfusion was the most frequent 53(42.1%) type of blood transfusion. The most common indications for blood transfusion were anaemia of prematurity, sepsis and severe neonatal jaundice. A hundred and twelve (88.9%) who received transfusion were discharged while 4(3.2%) died. The rate of blood transfusion was low, while severe neonatal jaundice and anaemia were the main indications for blood transfusion in this study. Exchange blood transfusion was the main type of transfusion.

Pegylated Carboxyhemoglobin (SANGUINATE®) Restores Blood Flow and Tissue Oxygenation in a Novel Rat Model of Acute Vaso-Occlusive Crisis

INTRODUCTION: Sickle Cell Disease (SCD) hemoglobin S (HbS) promotes hemoglobin polymerization and red blood cell (RBC) sickling during hypoxia. PEGylated carboxyhemoglobin bovine (PEG-COHb; SANGUINATE®) has been previously shown to reverse sickling of SCD RBC in vitro by directed gas transfer to hypoxic RBC. A rat model was used to evaluate the capability of SANGUINATE® to restore cardiovascular rheology and tissue oxygenation (PISFO2) at sites distal to acute Vaso-Occlusions (VOC).OBJECTIVES: Evaluate the in vivo capability of PEG-COHb to improve rheology in rats placed in active VOC through transfusion of human SCD RBC while monitoring treatment effects on PISFO2 and systemic functions.METHODS: Human homozygous (HbSS) whole blood was obtained from consented SCD volunteers. The SCD RBC were isolated by centrifugation, triple-washed and resuspended in lactated Ringer's saline (LRS). Male Sprague Dawley rats were anesthetized, cannulated for fluid transfer, and surgically prepared for microscopic study of the spinotrapezius microcirculation. Isovolemic exchange transfusion of rat blood with suspended SC RBCs (27.5 ± 3.5% total blood volume) was conducted until PISFO2 at the distal portion of the spinotrapezius muscle was < 1 mmHg. VOC was visually confirmed as wide-spread precapillary arteriolar flow-arrest. Animals in VOC were subsequently treated 15 minutes later with PEG-COHb or LRS administered as a bolus of 8.0 ml/kg over 5 minutes. Animals were observed for up to 4 hours post-treatment and then euthanized. Measurements included systemic cardiovascular parameters, survival, blood chemistry, and PISFO2 via phosphorescence quenching microscopy and were obtained across treatment groups and the no treatment [Sham] control group.RESULTS: Exchange transfusion successfully produced a reduction in PISFO2 in the distal portion of the spinotrapezius muscle for all animals to <1 mmHg presumably through Vaso-Occlusions. VOC was visually apparent as flow arrest was initially observed in capillaries and most small venules and arterioles, and then progressed proximally into sites with larger feed vessels and higher intravascular pressures with subsequent declines in local PISFO2. Intervention with PEG-COHb and LRS both showed a visual improvement in microvascular flow, but yielded differential effects on PISFO2 in all sites (average return to 96 ± 6% and 64 ± 20% of baseline, respectively). PEG-COHb -treated animals survived the entire 4 h observation phase with a final global spinotrapezius PISFO2 level within 10% of baseline values. In contrast, global spinotrapezius PISFO2 declined to 0 mmHg for LRS treated animals by 3 h with rapid decline in mean arterial pressure and death occurring prior to 4 h. Serum lactate levels were monitored to provide an assessment of systemic ischemia from time 0 after exchange transfusion to the last collection point near the end of the experiment. Lactate increased for untreated Sham (34 ± 4.5 to 124 ± 43.0 mg/dl) and LRS groups (48 ± 15.0 to 110 ± 43.0 mg/dl), but remained stable for SANGUINATE®-treated animals (42 ± 1.5 to 40 ± 4.5 mg/dl). Total microcirculatory collapse occurred in Sham animals by 3h along with a null-reading of PISFO2, which was followed by a rapid decline in mean arterial pressure and death at 3.5 h similar to the LRS group.SUMMARY: This study was conducted in a novel rat model providing the ability to monitor real-time microcirculatory events and systemic parameters, which is difficult in murine models due to their smaller stature. PEG-COHb treatment promoted VOC resolution as evidenced through improved blood flow and concomitant effects on PISFO2. Importantly, the gas transfer properties of SANGUINATE® not only acutely resolved VOC, but provided the added benefit of restoring PISFO2 to near baseline values. In contrast, LRS animals showed transient rheology and minimal PISFO2 improvements, but levels declined over time and outcomes were similar to sham controls. Restoring blood flow and PISFO2 are key events to limiting VOC pain and complications and the treatment with PEG-COHb demonstrated the ability to achieve these critical functions suggesting it may provide improvements in treating acute VOC in human SCD individuals. DisclosuresSong:Prolong Pharmaceuticals: Consultancy, Research Funding. Nugent:Prolong Pharmaceuticals: Consultancy, Research Funding.

Stroke in Pediatric Sickle Cell Disease in Sub-Saharan Africa: Systematic Review and Estimated Prevalence

Background: Most people with sickle cell disease (SCD) live in Sub-Saharan Africa (SSA); many do not survive to adulthood (Grosse et al. Am J Prev Med 2011). Stroke is a common complication of SCD in the region (Ohene-Frempong et al.Blood 1998), yet the region's burden of pediatric SCD stroke is unknown. This systematic review aims to describe what is known, and to estimate the number of children affected by SCD stroke in SSA.Methods: Systematic reviews using Pubmed, Embase, and Web of Science were performed for all studies reporting pediatric SCD stroke prevalence data. Keyword search strategy included these or related terms: “stroke” or “brain” and “sickle cell” and “Africa” or each country in SSA available in English or French. Publication dates were selected between 1980 and December 13, 2016. Using pre-selected inclusion criteria, titles and abstracts were screened and full-text articles were reviewed. For publications meeting inclusion criteria, extracted data included: study and participant characteristics, number of patients with stroke, diagnostic criteria, imaging, management, recurrence. The number of children affected by SCD stroke in SSA was estimated based on current estimates of national population and S allele frequency (Piel et al. Lancet 2013) and the stroke prevalence in the extracted studies. The stroke prevalence in SSA was estimated based on both fixed-effect analysis and random-effect analysis.Results: After duplicates were removed, 133 individual articles remained. Ten full text articles met review selection criteria, including one in French. In all, papers reported data from seven countries in the region, including four reports from Nigeria. Cross-sectional SCD clinic-based data, with a sample size of 6,289 patients, reported pediatric stroke prevalence of 2.9-16.9%. Diagnostic criteria of stroke varied by site and were limited by access to brain imaging, which was reported in only half of the included studies. Treatment for stroke included packed red blood cell transfusion, exchange transfusion, or hyperhydration. Secondary stroke prevention with chronic blood transfusion or hydroxyurea was offered at two of 10 sites. Recurrence data was limited by variable study follow-up and limited reporting times. Using available sickle gene frequencies by country, estimated births affected by SCD per country in SSA, and a linear- or random-effects model, pediatric stroke prevalence in SSA was projected as 279,908 (95% CI: 240,335-319,481) and 561,746 (347,472-775,056), respectively. Estimates do not include stroke- or disease-associated mortality.Conclusion: This meta-analysis estimates prevalence of pediatric SCD stroke in SSA, underscoring the region's high stroke burden. Multiple clinic-based cross-sectional studies reported stroke prevalence comparable to data from high income countries prior to implementation of standardized prevention strategies. Accuracy is limited by different approaches to stroke diagnosis, paucity of population-based data on disease prevalence and mortality, regional variation in disease prevalence and severity, and overall high rates of early death in children with SCD. Available data would skew towards surviving patients, leading to possible underreporting of people at risk for SCD stroke. Improved diagnostic specificity with standardized criteria and imaging would be useful for identification of strokes. Understanding the large numbers affected by SCD stroke is critical for focusing public health efforts to introduce preventive measures to reduce stroke risk and to implement rehabilitative strategies. Adopting standardized primary and secondary stroke prevention measures in SSA may decrease the burden of SCD-associated stroke.This work was supported by funding from the National Institutes of Health (5R21HD089791, PIs Idro, Green). DisclosuresNo relevant conflicts of interest to declare.

PHENOMENON OF DEMIKHOV. In the Sklifosovsky Institute (1960–1986). Approaches to solving the problem of organ and tissue transplantation in the USSR and abroad (1967)

Analysis of the materials of the 2nd All-Union conference on the problem of tissue incompatibility, conservation and transplantation of tissues and organs (Odessa, 1967) showed that Soviet and foreign scientists had similar approaches to solving the problem of organ and tissue transplantation. Soviet scientists spoke about overcoming tissue incompatibility by hybridization of plants and chimerization of animals, about the effect of drug sleep on transplant immunity, about neurohumoral immunological shifts and the role of the central and peripheral nervous systems in the engraftment of grafts, about the influence of external factors on immunity. They also discussed the characterization of the antigenic structure of grafts, the role of DNA in immunity, the genetic transformation of homomaterial, the use of pharmacological agents to suppress immunogenesis, the cryopreservation of auto- and homo-organs and tissues with perfusion of their vascular bed, and the study of immunogenesis at the molecular level. A year earlier, the Americans discussed immunological paralysis, the effect on the recipient's immunity of the donor's blood transfused to him and its components, and biochemical studies of immunity. At the same time, without any ethical doubts, American scientists conducted experiments, including clinical ones, with multiple passages of homosexual skin, with exchange transfusion of blood to newborns and subsequent transplantation of homosexual donors to them, with irradiation of recipients with powerful doses of X-rays. It is shown that most of the trends that had been developed by V.P. Demikhov, were approved by the 2nd All-Union Conference. But what he lacked was close and comprehensive integration with morphologists, physiologists, immunologists, biochemists, pharmacologists and, sadly, with clinical surgeons. Based on the research conducted, an unambiguous conclusion can be drawn: Soviet scientists should not have criticized V.P. Demikhov for his "misunderstanding" of immunology, and to help him in every possible way, directing his energy in the right direction.

To study the outcome of exchange transfusion in severe neonatal sepsis in neonates admitted in NICU at Dr. Bhim Rao Ambedkar memorial hospital, Raipur, Chhattisgarh, India

Background: Sepsis is one of the most common causes of neonatal mortality and morbidity.Immaturity of the immune system, newborn infants are highly susceptible to systemic infection.Blood exchange transfusion in severe neonatal sepsis remove bacteria, bacterial toxins, andcirculating pro-inflammatory cytokines, improve perfusion and tissue oxygenation, correct theplasma coagulation system and enhance immunological defence mechanisms. Material andmethods: This is a hospital-based, time-bound, analytical observational study conducted fromJanuary 2019 to December 2019 in the NICU of Dr. B.R.A.M. Hospital &amp; Pt. J. N. M. Medical College,Raipur, Chhattisgarh, India. The data was collected in pre-designed proforma, entered in MicrosoftExcel and analysis was done using SSPS v 22.0. Result: About 42 neonates were diagnosed withsevere neonatal severe. Of which 23 (54.76%) were preterm, 42.24% were term neonates.Maximum 22 (52.38%) were VLBW, 4.76% were LBW and 19.05% were with normal birth weight. Inthe study two-third of 28 (66.67%) were outborn and one third were inborn. In the present studymajority of 30 (71.43%) had EOS and 12 (28.57%) had LOS. In our study out of 42 study subjects24 (57.14%) died and 18 (42.86%) were discharged after blood exchange transfusion. Of those whodied 15 (62.5%) were preterm and of those discharged 10 (55.6%) were term neonates (p=0.349).Outborn neonates more died as compare to inborn though this was also not significant (p=0.133).Conclusion: significant reduction of mortality in patients who underwent exchange transfusion,together with the no adverse effects observed, suggest that this procedure should be considered forthe treatment of neonates with severe sepsis.

Blood groups

On June 14, 1868, Karl Landsteiner, an outstanding scientist, known for his works in the field of immunohematology and immunochemistry, who received the Nobel Prize for the discovery of blood group systems in 1930, was born in a Viennese family. In 1900, Karl Landsteiner published a work in which he described in detail the process of agglutination that occurs when the blood plasma of one person is mixed with the red blood cells of another one. At that time, the scientist came to the conclusion that this phenomenon was of an immunological nature. In 1901, Landsteiner decided to divide human blood into three subgroups: A, B, and C; a little later, the AB group was added to them, while the C group was renamed as O. In addition, it was Landsteiner who invented a fairly simple scheme that allows developing and introducing the basic principles of blood transfusion into wide practice, and the world got a wonderful opportunity to save hundreds and thousands of human lives. Thanks to this discovery, made more than 100 years ago, more than 100 million donations are made every year around the world, more than half of which are in developed countries with high living standards and incomes. Here people come to blood donation deliberately, and not for the sake of receiving financial or any other benefit. Thanks to blood transfusion, it became possible to successfully carry out many surgical interventions accompanied by the loss of a large amount of blood, exchange blood transfusion for hemolytic disease of newborns, and substitution therapy for many pathological conditions. Karl Landsteiner’s work was highly appreciated: in 1930, due to the discovery of blood groups, he became the Nobel Prize laureate in the field of medicine.

Non-spherocytic hemolytic anemia caused by erythrocyte pyruvate kinase defiiency: the analysis of genetic defects in pediatric patients, living in Russian Federation

The article presents retrospective data analysis of a cohort of patients with PKD (n = 41 patients, aged 4 months – 26,5 years, median of age – 5 years 1 month) who were examined at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology for unspecifid hereditary hemolytic anemia during the period 2013–2020. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. In all patients, the diagnosis was confimed by Next Generation sequencing (NGS). The homozygous mutations in the PKLR gene were found in 10 patients (24.39%), compound heterozygous mutations in 31 patients (75.61%), 77.78% of them were missense mutations. Gender distribution (male:female) was 1:1.73. At least once transfusion of erythrocyte suspension was required to 40 (97.56%) patients. The minimum age at the time of the debut of transfusion dependence was the fist day of life, the maximum was 4 years. Exchange blood transfusion was performed in 13 children, severe normocytic hyperregenerative anemia with transfusion of red blood cells in the fist days of life was noted in 12 children, at the 1st month of life – in 9 children, at the 2nd month of life – in 8 children, at the 3rd month – in 6 children, at the 5th month – in 2 children, at the 1st year – in 1 child, and 2 children underwent single transfusions on the background of infectious episodes at 3 and 4 years respectively. Splenectomy due to high transfusion dependence was performed in 10 patients: transfusion independence was achieved in 5 patients, in 5 – an increase in the interval between blood transfusions. Median of surgical intervention (9 patients): 7 years 4 months, minimum age – 1 year 4 months, maximum – 14 years 4 months. In total, 36 genotypes were described in 41 patients, among them were: c.1529G&gt;A in 3 patients, c.1137_1139del / c.1456C&gt;T – in 2 patients, c.1079G&gt;A/c.1529G&gt;A in 2 patients, c.1130T&gt;C/c.1456C&gt;T in 2 patients, other genotypes occurred once. Two mutations were the most frequent: c.1456C&gt;T (16.67%) and c.1529G&gt;A (16.67%). 19 (46,34%) of patients had previously not described mutations.

Effect of exchange blood transfusion on oxygen saturation of neonates with severe neonatal jaundice by pulse oximetry

Objective: To determine if there was any difference in SpO2 readings during exchange blood transfusion (EBT). Methods: A prospective cross-sectional study of neonates with severe neonatal jaundice requiring EBT was conducted. Oxygen saturation was recorded before, immediately and 15 minutes after EBT by using a pulse oximeter. Results: This study included 30 neonates with 20 males and 10 females. The age ranged from 1 to 12 days with a mean of (5.4 ±2.9) days. Pre-EBT SpO2 ranged from 90% to 98% with a mean value of (94.3 ± 2.2)%; SpO2 in the end of EBT ranged from 85% to 99% with a mean value of (94.1 ± 3.2)%; SpO2 at 15 minutes after EBT ranged from 77% to 99% with a mean value of (94.8 ± 4.1)%. There was no significant difference between SpO2 values at onset of EBT and either immediately or 15 minutes after EBT (P=0.770 and 0.422, respectively). SpO2 showed no significant difference between neonates who were infused with blood of different storage times (&lt;24 h or ≥24 h) at the onset of EBT (P=0.584), immediately (P&gt;0.999) and 15 minutes after EBT (P=0.887). Besides, SpO2 values were compariable in neonates with hematocrit &lt;45% or ≥45% at the onset of EBT (P=0.284), immediately (P=0.118) and 15 minutes after EBT (P=0.868). Conclusions: EBT does not affect SpO2 in neonates.

Scalp Swelling and Headache in a 12-year-old Boy

1. Kathryn Westphal, MD* 2. Respicius Bakalemwa, MD† 3. Elizabeth Groothuis, MD, MPH* 1. *Department of Pediatrics, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL 2. †Department of Pediatrics, Bugando Medical Centre, Mwanza, Tanzania A 12-year-old boy with sickle cell disease (SCD) is admitted to a hospital in Mwanza, Tanzania, with acute onset of periorbital and scalp swelling. Associated symptoms include fever, headache, difficulty breathing, and back pain. Because resources in sub-Saharan Africa are limited, access to comprehensive care for patients with SCD is variable. The general principles of management focus on early diagnosis, parental education, prevention of infection with vaccinations and prophylactic medications, prompt treatment of infection and pain, the use of hydroxyurea for stroke prevention, and blood transfusions for life-threatening anemia. This patient has a known diagnosis of SCD but has infrequent pain episodes and no previous transfusions. He is not receiving hydroxyurea or prophylaxis against bacterial infections or malaria. He has no history of stroke and no known head trauma. On presentation he is afebrile and his heart rate is 120 beats/min, respiratory rate is 40 breaths/min, and blood pressure is 105/75 mm Hg. His oxygen saturation is 92% in room air. Physical examination is significant for pallor, periorbital edema, scalp edema, dyspnea without adventitious lung sounds, and splenomegaly with diffuse abdominal tenderness. His scalp edema is fluctuant and extends posteriorly from the periorbital region, encompassing the entire scalp. Swelling is most prominent in the frontal and parietal areas. The overlying skin is nonerythematous and nontender but is warm to the touch. His neurologic examination has no focal findings, although generally he appears tired. Initial laboratory results demonstrate a white blood cell count of 84,000/μL (84×109/L) with a lymphocytic predominance (57%), a hemoglobin level of …

Management strategy and novel ophthalmological findings in neonatal severe hypertriglyceridemia: a case report and literature review

BackgroundNeonatal severe hypertriglyceridemia is rarely reported in the literature and there is no consensus for hypertriglyceridemia management at this age group.MethodsThe index case is a 4-week-old male infant with severe hypertriglyceridemia accidentally discovered during a circumcision surgery. His clinical and genetic characteristics and his successful management strategy are described. Furthermore, a detailed ophthalmological examination of the proband was conducted at 3 and 6 months of age using Fourier-domain-optical coherence tomography.ResultsTriglycerides level at presentation was extremely high 33,727 mg/dL (380.8 mmol/L). Two sessions of exchange blood transfusion on two consecutive days successfully reduced triglycerides to 382 mg/dL (4.3 mmol/L) with no adverse effects. The infant was discharged 3 days later. At discharge, the mother was advised to continue breastfeeding together with a medium-chain triglycerides formula. Satisfactory growth parameters and lipid profile values were obtained for a follow-up duration of 5 months with no reported attacks of acute pancreatitis. Lipoprotein lipase deficiency was confirmed by the detection of the LPL homozygous pathogenic variant c.805G > A; p.(Glu269Lys). Early corneal and macular lesions were detected and persisted on follow-up despite relatively good lipemic control.ConclusionThis case highlights the importance of the early discovery of severe hypertriglyceridemia during the neonatal period, which is needed for prompt management and prevention of severe complications. Rationalized breastfeeding can be tolerated within the diet plan of the disease with satisfactory outcomes. To our knowledge, it is the first study reporting early corneal and macular affection by severe hypertriglyceridemia in a neonate. Prolonged follow-up is needed to determine the extent of ophthalmological lesions.

Severe Perinatal Asphyxia and Risk of Neonatal Jaundice at a Tertiary Hospital in Northern Nigeria

Background: The risk factors of neonatal jaundice are largely known, yet there is little agreement on the association between it and perinatal asphyxia.&#x0D; Aim: To investigate the association between severe perinatal asphyxia (SPA) and the risk of clinical jaundice (NNJ) among neonates managed at the Federal Medical Centre, Azare, Nigeria.&#x0D; Methodology: Case control design was employed. Medical records of 315 babies managed at the special care baby unit from 1st January, 2011 to 31st December, 2018 were analysed. The exposure of interest was SPA and the outcome was jaundice. Logistic regression was applied to demonstrate the relationship between neonatal jaundice and SPA. Relative risk was provided as odds ratio and 95% confidence interval.&#x0D; Results: Sixty-three cases and 252 controls were enrolled in the study. The mean age of the cases (4.39) and that of the controls (4.95 ) did not differ significantly (t= -0.52, P= 0.30). One hundred and fifty-six (61.9%) of the controls were males while 34 (54.0%) of the cases were females. For 59 (93.7%) of the cases treatment for jaundice was done with phototherapy and 1.6% required exchange blood transfusion. SPA significantly reduced the risk of developing NNJ (adjusted OR = 0.27, P-value ˂ 0.01). &#x0D; Conclusion: We demonstrated a significantly reduced risk of developing neonatal jaundice with prior exposure to severe perinatal asphyxia. Prospective multicenter and community based studies correlated with serum bilirubin levels are recommended.

Post-Exchange Transfusion Sodium Change Within a Laboratory Reference Range of Serum in Hospitalized Severe Neonatal Jaundice Cases

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 &lt; 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 preterm-born 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 &lt; 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 &gt; 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.

Pattern of serum bilirubin changes following double volume exchange blood transfusion in neonates at a tertiary health facility in Nigeria.

Introductionexchange blood transfusion (EBT) is a form of massive transfusion useful in rapidly reducing serum bilirubin levels, but serum bilirubin levels frequently rebound within hours of completing the procedure, due to equilibration of extravascular bilirubin as well as on-going hemolysis. The study was carried out to determine the pattern of reduction in serum bilirubin levels following EBT among neonates with severe hyperbilirubinemia, as well as the factors contributing to this pattern, so as to establish evidence-based expectations following EBT.Methodsa retrospective descriptive study covering a two-year period in a Nigerian tertiary hospital. Details of the EBT procedures, including serial serum bilirubin levels, were obtained from the hospital records of all newborn babies who had double volume EBT done for severe hyperbilirubinaemia during the study period. Data was analyzed using the statistical software SPSS version 21.0.Resultsthe mean total serum bilirubin (TSB) before EBT in the 36 babies was 17.9 ± 6.3 mg/dl. The mean percentage decrease in TSB immediately following EBT was 44.3 ± 10.2%. Six hours after EBT, TSB levels had increased from the immediate post-EBT values by an average of 57.5 ± 32.2%. Twenty-four hours after the procedure, TSB values in most (87.1%) cases were still higher than the immediate post-EBT values, but lower than the pre-EBT values. Post-EBT anemia was recorded among 33.3% of the babies.ConclusionEBT is effective in rapidly reducing serum bilirubin levels and preventing acute bilirubin encephalopathy in neonates with severe hyperbilirubinemia, despite the rebound increase that occurs in TSB values after the procedure.

Klebsiella Pneumoniae sepsis, clinical features and early outcome among Newborns in neonatal intensive care unit Zawia teaching hospital

Background: Klebsiella pneumonia is one of the most common infections that cause neonatal sepsis globally. It is responsible for a significant proportion of hospital-acquired infections including septicemias, urinary tract infections, pneumonia, and soft tissue infections especially in the immunocompromised hosts such as the neonate. Aim of study: to identify, clinical features and the outcomes associated with K. pneumonia, in all newborn at NICU at Zawia teaching Hospital – Libya. Materials and Methods: Retrospective case series study conducted at neonatal intensive care unit in Zawia teaching hospital /Libya. during one year period (1st of January -30st December 2012). Four hundred and eleven neonates who were admitted to Intensive Care Unit (ICU) in pediatric department were selected. The following data was obtained from the patient’s medical records sex, birth weight, gestational age, mode of delivery, time of admission (Season), blood cultures strain of the isolates, maternal history, clinical diagnosis and features association problems mechanical ventilator, blood exchange, umbilical vein catheterization, respiratory distress syndrome, birth asphyxia, CRP for risk patient, CRP on deterioration, platelets concentrate transfusion and outcome. Result: The study results reported that 13 neonates had positive klebsiella pneumonia.9 (69.2%) preterm,9(69.2%)were males 4(30.8%)were females with 2:1 male to female ratio ,10 (76.9%) had low birth weight ,8(61.5%) were delivered by caesarean section , regarding maternal age 53.8% were between 31-40 years with mean maternal age 32.15 years and 5.5 standard deviation ,61.5% were infected at the second half of the year p value, History of PROM in 6( 46%), pre eclampsia (30.8 %) maternal fever in 1(7.7%),maternal infection (7.7%). 9(69.5%) had respiratory distress,53.8%,7 (53.8%) developed hyperbilirubenmia,4(30%) had necrotizing enterocolities ,1(7%) had acute myocarditis,1patient presented with fever and proved to have UTI with sepsis, mechanical ventilation was needed in8 (61.5%).one patient developed Pnemothorax (7.7%)and drained under water seal by insertion of chest tube. Umbilical vein catheterization inserted only in one patient7.7% Exchange blood transfusion was done in one baby 7.7%. all had negative CRP test on admission 13 (100%) and CRP was positive in all patients (100%) with positive klebsiella pneumoniae (p value 0.0001) when sepsis was suspected.10 (76.9%) of the neonates required platelets transfusion which indicate the higher rate of thrombocytopenia. Regarding neonatal outcome, the result reported that 10(76.9%) of neonates with positive klebsiella pneumonia were alive and only 3 (23.1%) died, Two due to respiratory failure and the 3rd one died due acute myocarditis complicated by heart failure. The mortality rate about 23%. 66.6%were preterm low birth weight .100% were males and due to ESBL producing strains. Conclusion: The main risk factors for the infection was preterm babies, males gender, low birth weight, caesarean section delivery, prolonged pre labor rupture of membrane (PR0M). mostly infected at the second half of the year and hospital stay, The clinical presentations are non specific, Acute myocarditis is a rare presentation with fatal prognosis. CRP is valuable for early detection of klebsiella pneumoniae infection. Thrombocytopenia is very common The mortality rate was relatively high and all deaths were male, due to ESBL producing K.pnemoniae strains which was similar to many studies in other countries.