Underwent Exchange Transfusion Research Articles

Clinical Course and Outcomes of Sickle Cell Disease Patients with COVID-19 at a New York Hospital

Introduction:The outbreak of a novel infection, COVID-19, has greatly impacted the well-being of individuals worldwide. Persons with sickle cell disease (SCD) constitute a vulnerable population, subject to health disparities, who may have worse outcomes from COVID-19. Within the United States, New York has a large population of patients with SCD. Here, we analyze the clinical course and outcomes of SCD patients with COVID-19 who were admitted to a community teaching hospital in Brooklyn, NY.Methods:We conducted a retrospective chart review of adult patients with SCD hospitalized with laboratory- confirmed COVID-19. Electronic health records were reviewed to identify patients and analyze their clinical course. Clinical characteristics, laboratory and radiology data were assessed. Rates of acute chest syndrome (ACS), acute kidney injury (AKI) and venous thromboembolism (VTE) were determined. ACS was defined by the presence of fever and/or respiratory symptoms accompanied by a new pulmonary infiltrate on chest Xray. Data on use of blood transfusion, treatments, length of stay and mortality were collected.Results:Between March 1 to June 30, 2020, 53 adults with SCD were hospitalized at our institution. Of these, 13 patients had COVID-19 infection. The mean (±SD) age of the COVID-19 patients was 34±10 years (range, 22 to 50) with 54% being female. Seven patients (54%) were Hb SS, and 6 patients (46%) were Hb SC. Comorbid conditions included Diabetes Mellitus (1 patient), SLE (1), End-stage renal disease (1), prior VTE (4) and Avascular necrosis of hip (3). Four patients were on hydroxyurea.Clinical, laboratory and radiological findings are summarized in Table 1. While all the Hb SS patients presented with vaso-occlusive crisis, 4 of the 6 patients with Hb SC did not have symptoms of pain crisis. Chest pain and cough were the most common symptoms at presentation. During the hospital stay, 12 patients (92%) had at least one febrile episode >38°C, with 77% having recurrent fevers above 38.5°C.Eleven patients (85%) met criteria for ACS. Seventy-seven percent of all patients required supplemental oxygen. Nine patients (69%) were transfused, with 4 patients undergoing exchange transfusion. Sixty-seven percent of the transfused patients were transfused within 48 hours of admission. No patients required intubation or mechanical ventilation and none were admitted to the intensive care unit (ICU). Five patients (38.5%) received hydroxychloroquine while 84.6% were treated with antibiotics. No patient received remdesivir.Three patients (23%) developed AKI: of these, one patient required acute hemodialysis, the other two cases were mild with peak creatinine less than 2.0 mg/dl.Ninety-two percent of patients received prophylactic anticoagulation with either unfractionated heparin, enoxaparin or fondaparinux. One patient who did not receive an anticoagulant due to thrombocytopenia developed an acute deep vein thrombosis which was also catheter-related. Of note, during the initial phase of the pandemic standard dosing of prophylactic anticoagulants were used but in the later months, some patients received higher prophylactic doses in keeping with hospital protocol.The median length of hospital stay was 9.4 days (interquartile range, 8.1 to 13.3). There were no deaths - all patients were discharged home.Summary:Panepinto et al (Emerg Infect Dis.) reported a mortality of 7% in 178 SCD patients with COVID-19 in the United States. Other published reports have detailed more favorable outcomes (Arlet et al, Lancet and Appiah-Kubi et al, Br J Haematol.). In this small retrospective analysis of hospitalized SCD patients, there was no mortality. Acute chest syndrome was the most common complication observed. VTE and severe AKI were infrequent. Blood transfusion was performed in the majority of patients (69%); two thirds of the patients transfused received blood within 48 hours of hospitalization. There were no ICU admissions and no use of mechanical ventilation indicative perhaps of less severe COVID-19 disease. This may have been due to the young age of the cohort. Early use of blood transfusion may have been a factor in reducing disease severity and improving outcomes. The best approach to managing these patients is unclear. We advocate for the development and dissemination of evidence-based guidelines to manage SCD patients with COVID-19 to reduce morbidity and mortality in this at-risk population.DisclosuresNo relevant conflicts of interest to declare.

Sickle Cell-Related Complications in Patients Undergoing Cardiopulmonary Bypass.

We aimed to describe our experience with patients with sickle cell trait (SCT) and undergoing surgery on cardiopulmonary bypass (CPB). Data on all patients with SCT or sickle-α thalassemia who underwent surgery on CPB were collected (1996-2017). Overall, 46 patients were included, 37 (80%) had SCT and 9 (20%) had sickle-α thalassemia. A total of 4 (9%) developed a potential sickle cell-related complication. Patients with sickle cell-related complications were significantly older (median 14 years vs 14 months, P = .037) and heavier (median 54 kg vs 9 kg, P = .041). Complications occurred, although without statistical significance, in patients who underwent longer median CPB times (249 minutes vs 137 minutes, P = .069), lower median temperature (31.7 °C vs 33.3 °C, P = .094), and a higher percentage underwent deep hypothermic circulatory arrest (50% vs 7%, P = .053). A total of 30 (65%) patients underwent exchange transfusion (ET) pre-bypass. Patients who underwent ET were significantly older (median 4 years vs 7 months, P = .003) and heavier (median 16 kg vs 6 kg, P = .015) than patients who did not undergo ET. The incidence of complications was comparable between patients who underwent ET (10%) and those who did not (6%). In this retrospective, single-center study, it has been shown that cardiac surgery requiring CPB in patients with SCT and sickle-α thalassemia had a low risk for sickle cell-associated complications. In this cohort of patients, older age, longer CPB times, lower median temperature, and the utilization of deep hypothermic circulatory arrest appear to play an important role in the development of complications.

Exchange transfusion for neonatal hyperbilirubinemia: A multicenter, prospective study of Turkish Neonatal Society.

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. 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. 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. 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.

Etiology and clinico-hematological profile of neonates with pathological unconjugated hyperbilirubinemia: a tertiary care centre experience

Background: Jaundice is visible manifestation of raised serum bilirubin. Jaundice in newborns is not an uncommon occurrence. Usually jaundice in newborn is due to elevation of unconjugated bilirubin. There are varied causes of unconjugated hyper bilirubenemia. This study was conceptualized to see the etiology and clinico-hematological profile of neonates with pathological unconjugated hyperbilirubinemia who were admitted in the specific time frame in Sharda Hospital, Greater Noida which is a tertiary care hospital in Western U.P. Methods: This is a retrospective study in which data of all neonates admitted to NICU with unconjugated hyperbilirubinemia requiring phototherapy and/or exchange transfusion in the period 1.7.2018 to 31.12.2018 was collected and analyzed. History including birth weight, mode of delivery, gestational age, mother's blood group, etc. was recorded. Complete physical examination and investigation done for diagnosis noted. Results: It was seen that out of 438 admissions in NICU, 63% had neonatal jaundice and 18.8% of this had pathological unconjugated hyper bilirubenemia. 63.5% were males and 36.5% females. Majority were term babies. Most of the babies developed jaundice on day 3 of life. In 61% cases no cause for jaundice could be ascertained despite investigations for the same. ABO and Rh incompatibility accounted for 15.30% and 5.7% cases respectively. All patients received phototherapy and 3.8% underwent exchange transfusion. Conclusions: From the study it can be concluded that although blood group incompatibility is an important cause of pathological unconjugated hyper bilirubenemia but in most of the cases no cause is usually found.

PB2306 SURGICAL PROCEDURES AND OUTCOMES AMONG CHILDREN WITH SICKLE CELL DISEASE

Background:Although children with sickle cell disease (SCD) often undergo surgery, there are limited current epidemiological data for this pediatric population. Sickle cell patients present a high risk of postoperative complications and have a higher morbidity and mortality than the general population after surgery. During the perioperative period there are certain situations that can trigger an increase in falciformation and vaso‐occlusive crises that lead to complications mainly of respiratory type. Our protocol of perioperative general care includes: transfusion of packed red blood cells to obtain a hemoglobin value of 10 g/dL, hyperhydration, avoiding hypoxia and cold, strict control of pain, early ambulation and incentivized spirometry.Aims:To study the prevalence and severity of postoperative complications in pediatric patients with SCD (HbSS/Sβ0 o SC) followed in our center in a period of 22 yearsMethods:We reviewed the medical records of patients with SCD who underwent surgery between January 1997 and January 2019. We described characteristics of patients, the type of surgery that was performed and the postoperative complications they presented.Results:From 1997 to 2019 out of 91 pediatric patients followed in our center with SCD, 38 patients underwent surgery (41.8%). A total of sixty‐one interventions were performed in these patients (1.6 surgeries per patient). The median age at the moment of surgery was 7.5 years. The most frequent procedure was otorhinolaryngological surgery (21.3%), followed by trauma surgery (18%), splenectomy (14.7%), herniorrhaphy (11.4%), cholecystectomy (8.2%), circumcision (6.6%) and others (19.6%). Postoperative complications were observed in 7 out of the 61 surgical interventions: 4 acute thoracic syndromes, 2 bronchospasm crises and 1 vaso‐occlusive crisis. In 4 of the 7 cases, complications occurred after abdominal surgeries. Most complications occurred within 24‐48 hours after the surgery (1 day median with a range of 0 to 6 days). In 4/7 of the perioperative complications patients were previously receiving hydroxyurea, 3/7 were not receiving hydroxyurea at the moment of the surgery; two because of medical indications and one patient arrived from country of origin where the medication was not administered. Three patients required admission to the intensive care unit (ICU). Two patients required exchange transfusion and respiratory support. There were no strokes and no deaths.Summary/Conclusion:We observed a high rate of surgical procedures in our patients. The burden of surgical disease in SCD children differs from the general pediatric population. In our series the most frequent indication for surgery was tonsillectomy and adenoidectomy. Despite the perioperative care described, we observed a prevalence of postsurgical complications of 11.5%. The majority of the complications were of respiratory type coinciding with the existing literature, practically half of them required admission to the ICU and a 28.5% underwent exchange transfusion and required respiratory support after the event. The incidence of death and stroke is nule in our series. There are few adequate trials to determine optimal perioperative management in these patients. Future research should determine how to predict and decrease perioperative complications.image

Neurological outcome of neonates undergoing exchange transfusion for hyperbilirubinemia: A study in tertiary care centre in Bihar

Neurological outcome of neonates undergoing exchange transfusion for hyperbilirubinemia: A study in tertiary care centre in Bihar

Comparison between Automated Erythrocytopharesis (AECP) and Manual Exchange Transfusion (M-Ex)) in Reducing Hb-S and in Recovery of Acute Chest Syndrome and Other Acute Presentations of Sickle Cell Disease Patients

Background:Sickle Cell Disease represents a national health problem in Saudi Arabia with close to 150 thousands of the population are afflicted with the disease. Patients are frequently admitted with life threatening complications like Acute chest syndrome (ACS), Hyperhemolysis (HH), pneumonia, thromboembolism including pulmonary Artery Thrombosis (PAT) or severe protracted painful vasoocclusive crises (VOC). Rapid lowering of Hemoglobin S helps in reducing sickling and in alleviating such complications and allowing rapid recovery. Exchange transfusion is the fastest way to remove pathogenetic sickling red blood cells and reducing Hb S level to a safe level. It can be done manually (Mex) or via Automated Erythrocytopharesis machine (AECP). In this study we compared manual exchange to AECP in achieving the targeted lowering of Hb S and in accelerating clinical recovery.Patients and Methods:Patients included are sickle cell disease patients (HbSS, HbSC, Hb S/thal) admitted to the ER of a central Hospital. Indications of exchange were: acute chest syndrome, acute severe painful vaso-occlusive crises refractory to standard ER protocol of analgesia, stroke, priapism, Hyperhemolysis, and acute pulmonary embolism.P value of significance was calculated using student t-test comparing between median Hb S achieved after manual exchange vs AECP.To assess the rapidity of reversal of desaturation in acute chest syndrome patients, the cumulative incidence of reversal of desaturation and normalization of Oxygen saturation on room air were plotted against time at 0 time of the start of exchange, 2 hours,4 , 12, 24, 48 and 72 hours/discharge (D/C)Results:Table 1 shows clinic-biological characteristics of patients who underwent exchange transfusion. A total of 230 patients-admissions were registered between Dec 1. 2017 to July 27, 2018 for painful VOC to ER; 51 (32%) had clinical indications for exchange (ACS 25, Stroke / fits 1, priapism 1, pulmonary artery thrombosis 1, Hyperhemolysis with VOC (n:7) , VOC with HLH (n:1), and the remaining with “refractory” painful VOC with or without infection. One patient died immediately at the time of arrival to ER before starting any standard resuscitative measures .Exchange transfusion was indicated and done for 53 (23%); 12 (22,6%) AECP and the remaining (77.4%) had Manual Exchange.The median Hb S after manual exchange was 44 % (range 31-74%) which was unsatisfactory and way higher than the targeted level while Automated ECP reached down satisfactorily to a median of 31%(range 8%-50%) ; 67% of whom achieved it with only one session. No mortalities or major procedure related complication reported with manual or automated ECP. Procedurally, 3 patients needed 2 automated sessions and 1 patient used 2 kits for one session. Manual exchange could not achieve the target Hemoglobin S level below or around 30% due to logistic and technical difficulties and sometimes patients' refusal while Automated ECP reached to a mean Hb s level of 28%( range 8%-50%) and nearly two thirds (67%) reached to as low as 31% Hb S level with only one session of Automated ECP and was associated with rapid improvement of the oxygenation within the first 2 hours of the procedure.Conclusions:Erythrocytopharesis (Automated RBC exchange) is effective, quick and safe procedure that is life saving for many patients with ACS and is associated with less difficulties and complications if compared with the manual exchange. Because SCA is a national problem in Saudi Arabia and acute chest syndrome and other acute major complications comprehensively kill SCD patients, Automated Erythocytopharesis should be available nation-wide like dialysis machines at all large hospitals in all cities and should be distributed according to the prevalence of SCA in the area or location. DisclosuresNo relevant conflicts of interest to declare.

Outcome of Age-Adapted Approach to HLA-Identical Related Hematopoietic Stem Cell Transplantation in Severe Sickle Cell Disease: Saudi Experience

▪Sickle cell disease (SCD) is one of the most common inherited disorders in Saudi Arabia with nearly 60,000 affected individuals (Alsultan et al. Ped. Transplant. 2016). Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative treatment in SCD. A recent international survey showed excellent long-term survival of SCD patients after HLA-identical sibling HSCT; however, patients aged 16 years or older had higher risk of GVHD and mortality (Gluckman et al. Blood 2017). HLA-identical related HSCT in adult SCD patients using non-myeloablative (NMA) conditioning consisting of alemtuzumab, 300 cGy total body irradiation (TBI), and sirolimus was successful in establishing long term stable donor chimerism with minimal toxicity and GVHD (Hsieh et. al. JAMA 2014). In this study, we reviewed the outcome of SCD patients who underwent transplant at our institution using standard protocols (NMA regimen in patients ≥14 years and myeloablative regimen in < 14 years) to address whether age remains a risk factor that influences HSCT outcome in SCD.Children (<14 years) with severe SCD received myeloablative conditioning using one of two regimens: first regimen was cyclophosphamide (Cy) 200mg/kg, busulfan (Bu) 16mg/kg, and thymoglobulin (ATG) 10mg/kg and recently we use thiotepa 8mg/kg, Bu 16mg/kg, and fludarabine (Flu) 160mg/m2. Bu pharmacokinetics was performed to target AUC of 900-1350 µmol/L.min. GVHD prophylaxis included cyclosporine and methotrexate 10mg/m2 on day +1, +3, and +6. Bone marrow was the source of stem cells in all pediatric patients. Cyclosporine was continued during the first-year post HSCT and tapered slowly afterward based on donor chimerism. Adult patients (≥14 years) received hydroxyurea at maximum tolerated dose and hypertransfusion for 2-3 months with iron chelation if indicated prior to HSCT followed by NMA conditioning consisting of alemtuzumab (1mg/kg divided over 5 days on day -7 to -3) and TBI 300 cGy with testicular shielding in males on day -1. Sirolimus was used as GVHD prophylaxis starting day -2 and continued for at least one-year post HSCT with subsequent taper based on donor chimerism. Stem cells source was GCSF mobilized peripheral blood stem cells in all adult patients targeting cell dose of CD34 10X106/kg based on recipient weight. All SCD patients underwent exchange transfusion prior to HSCT to achieve HbS <30%. Hemoglobin was maintained between 9-11 g/dL and platelet count > 50,000/uL during HSCT. Supportive care included penicillin V, acyclovir, antifungal, and Pneumocystis jiroveci pneumonia prophylaxis. Levetiracetam was used as seizure prophylaxis. Strict monitoring of blood pressure and magnesium was performed. Graft failure and death from any cause were considered as events.A total of 51 patients with severe SCD were transplanted at our center, 17 children and 34 adults. Patient and transplant characteristics are summarized in Table. Indications for HSCT included recurrent severe pain crisis (n=33), stroke (n=14), avascular necrosis (n=2), sickle cell hepatopathy (n=1), and priapism (n=1). All patients engrafted successfully for both neutrophil and platelets. There was no acute or chronic GVHD among adult patients. Two pediatric patient had mild grade I acute GVHD in the skin that was controlled with topical treatment and none of pediatric patients had chronic GVHD. Three adult patients developed secondary graft failure on day 61, 170, and 225 post transplant with aplastic marrow in two patients despite full donor chimerism and autologous recovery in the third patient. Among the two patients who had aplastic anemia, one died secondary to sepsis and one underwent successful second transplant using cyclophosphamide, Flu, and ATG. Thirty-one adults have stable donor chimerism. Event free survival (EFS) at 2 years was 90% in adult patients and 100% in children (P=0.3). Overall survival (OS) at 2 years was 97% in adults and 100% in children (P=0.4). Median follow up duration was 416 days (range, 61-1256) in adults and 203 (range, 30-2505) days in children.We demonstrated that age-adapted approach in guiding the choice of conditioning regimen intensity in severe SCD is associated with excellent outcome and minimal risk of graft rejection, GVHD, and transplant-related mortality. This real world data is important to encourage patients and transplant physicians to consider this curative treatment. Longer follow up duration is needed to confirm our findings. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Role of Automated Red Cell Exchange in Acute and Chronic Complications of Sickle Cell Disease

Background. Automated red cell exchange transfusion (ARCET) is commonly used in patients with sickle cell disease, but objective data on its impact on acute and chronic complications are limited.Methods. Fifty-two sickle cell disease patients at the University of Illinois at Chicago underwent exchange transfusion from January 2011 to January 2016. Six patients were excluded due to incomplete data leaving 46 patients available for analysis. We collected data from the year before, year after and the year of ARCET to study the impact of red cell exchange on clinical, biological and hematological parameters.Results. There were 435 procedures with average of 9.45 per patient (range 4-14). The mean age of our cohort was 58.2 years. There were 22 (47.8%) males and 24 (52.2%) females. Genotypes include 42 (91.3%) HbSS, 1 (2.2%) HbSC, 1 (2.2%) HbSBeta+thalassemia and 2 (4.3%) HbSBeta0thalassemia. The most common indication for ARCET in our cohort was prior stroke in 32 patients (69.6%) and prevention of stroke in 7 patients (15.2%), followed by frequent vaso-occlusive crisis (VOC) 8 patients (17.4%), multiple acute chest syndrome 6 patients (13%), pulmonary hypertension 6 patients (13%) and chronic kidney disease 5 patients (8.9%). Iron overload, sickle hepatopathy, cardiomyopathy and seizure were some of the other indications. Twenty-five patients (54.3%) had more than one indication to undergo the ARCET. Thirty-one patients (67.4%) are still continuing the treatment. Thirty patients (65.2%) were on hydroxyurea (HU) prior to ARCET and 8 patients (17.4%) were still on HU while on ARCET. The mean frequency of ARCET was every 6 weeks. The mean pre and post ARCET values for hemoglobin(Hb), hematocrit (Hct), Hemoglobin S %(HbS), white cell count (wbc) and platelets(plt) are shown in Table 1. Paired t-test and Wilcoxon signed-rank test were used to analyze the clinical and hematological parameters. Analysis shows increase in mean Hb and Hct post ARCET and decrease in mean wbc, plt and HbS % post ARCET and the difference is statistically significant. (Table 3). Post ARCET body mass index (BMI) and weight are increased and the difference is statistically significant with p value 0.002 for BMI and 0.003 for weight. (Table 3). Ten (21.7%) patients showed decrease in the ferritin level post exchange. Thirty patients (65%) had VORTEX port whereas 29 patients (63%) had central venous access for procedures prior to Vortex placement (17/29, 59%). Nine patients (20%) had peripheral access mostly power port (for access) with one peripheral vein for return (6/9 67%). There were 10 access related complications and there were 3 port replacements due to septum damage and infection. There were 10 procedure related complications and 10 transfusion reactions as described in Table 2. ED admissions were decreased in 13(28.3%) patients from mean 7.69 to 2.92 admissions. The annual inpatient admissions showed a decrease in 18(39.2%) patients from mean 4.6 days pre ARCET to 1.6 admissions post ARCET. The acute care admissions showed an increase due to program expansion of our acute care center during this study period.Discussion. Our study shows that red cell exchange is an effective treatment modality for patients with sickle cell disease. It contributes to improvement in weight, increase in Hb and Hct and decrease in wbc, plt, HbS% and iron overload. It also decreases inpatient and ED admissions. The procedure is safe and tolerable with minimal complications. Long term studies are needed study the efficacy of this treatment modality and its contribution to improvement of quality of life and life expectancy in sickle cell disease patients. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

421. Babesiosis: Retrospective Review of 38 Cases from Upper Midwest

BackgroundBabesiosis is a tick-borne illness caused by protozoal infection of the genus Babesia. Clinical presentation varies widely from asymptomatic to rapidly fatal infection and diagnosis requires a high index of clinical suspicion. It is an emerging health risk and clinicians need to be aware of its different clinical manifestations.MethodsWe retrospectively collected and analyzed data from 38 patients with babesiosis from 1990 to 2015.ResultsMean age of patients was 63 years. 68% of patients required hospitalization with 21% requiring intensive care unit (ICU) stay. Mean length of illness before diagnosis was 15.6 days and symptoms comprised of malaise (82%), subjective fever (71%), chills (55%), anorexia (29%), arthralgia (29%), and nausea (16%). Only 47% of the patients recalled tick bites. Mean hemoglobin in the outpatients was 12.4 g/dL compared with 9.8 g/dL in the hospitalized patients (P < 0.01). Among hospitalized patients, mean hemoglobin for ICU admissions was 7.5 g/dL vs. 10.9 g/dL (P < 0.01) for those without ICU stay. Mean parasitemia was 10.1% in those requiring ICU compared with 1.4% in those admitted to the medical floor (P < 0.01). 28.9% had Lyme disease, and 10.5% had anaplasma coinfection. Co-morbidities included diabetes mellitus (n = 3), asplenia (n = 5), and immunosuppression (n = 3). Diagnosis was made with PCR and peripheral smear in 50% of patient whereas 50% were diagnosed with PCR alone. In 27% of patients with positive PCR, peripheral smear was negative. All patients with asplenia required hospitalization with 3/5 requiring ICU with initial parasitemia ranging from 2.5 to 28% and duration of parasitemia ranging from 10 to 142 days. Initial treatment comprised of clindamycin plus quinine in 31% of patients whereas combination of atovaquone and azithromycin was used in 69% of patients. Median duration of treatment was 10 days. Overall three patients underwent exchange transfusion with parasitemias ranging from 12.3 to 28.5%. None of the patients died during hospitalization.ConclusionLess than half of the patients with babesiosis recall tick bites. There is usually a delay in diagnosis of up to 2 weeks due to nonspecific nature of symptoms. In more than one-fourth of patients with babesiosis peripheral smear may be falsely negative. Hemoglobin and percentage parasitemia seemed to correlate with severity of illness.Disclosures All authors: No reported disclosures.

Application of leukoreduction therapy in severe pertussis with leukocytosis and pulmonary hyperten-sion:3 infant cases reports and literatures review

Objective To investigate the efficacy and safety of leukoreduction therapy in severe pertussis in infants. Methods Therapeutic processes of 3 cases of severe pertussis in PICU of Shanghai Children′s Medical Center were retrospectively studied from October 2017 to May 2018.We reviewed the related literatures and summarized the time and effectiveness of leukoreduction therapy in severe pertussis. Results All 3 cases had leukocytosis, respiratory faliure, pulmonary hypertension and right heart failure.One case had multiple organ failure before undergoing exchange transfusion therapy and eventually died.Two cases that had pulmonary hypertension during the period of WBC′s rising accepted leukopheresis therapy before multiple organ failure, and eventually survived.We reviewed the foreign literatures which was almost case reports, leukoreduction therapy might improve the prognosis of severe pertussis in infants, but the time of using it had no conclusion. Conclusion This is the first report of leukoreduction therapy for the severe pertussis in infants in China.It provides a new method for the treatment of severe pertussis in infants.It is worth looking forward to use this method combined with continuous renal replacement therapy and extracorporeal membrane oxygenation technology.In the future, multicenter clinical research should be done to explore the effectiveness and safety of leukoreduction therapy in the severe pertussis in infants. Key words: Severe pertussis; Leukocytosis; Pulmonary hypertension; Leukoreduction; Leukopheresis; Exchange transfusion

INFLUENZA A PRECIPITATING MULTIORGAN FAILURE SYNDROME IN SICKLE CELL DISEASE

SESSION TITLE: Critical Care 1 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Management of sickle cell patients in the intensive care unit is challenging and requires multi-disciplinary care coordination. Multi-organ failure syndrome (MOFS) presents in a small portion of patients experiencing vaso-occlusive crises with or without acute chest syndrome, but can result in acute decompensation with a high mortality rate (1). We present a patient with influenza A who developed MOFS and was salvaged with an exchange transfusion. CASE PRESENTATION: 35 y/o African American male with HgSS sickle cell disease (SCD) who presented in a pain crisis with an acute kidney injury and transaminitis after experiencing flu-like symptoms, dark urine, and diffuse pain. Hb was 6 so he was transfused. Day 1 he developed acute renal failure and required hemodialysis. He required intubation and mechanical ventilation due to his low oxygenation and altered mental status. Day 2 he became hypotensive and was started on pressors. His chest radiograph had alveolar and interstitial opacities. Hg was still 6 so he received another 2U prbcs. Influenza A was positive and started on Tamiflu. HgSS was 44%, retic ct 9.7, haptoglobin 0, troponin 46, AST 1788, ALT 414, T bili 27.5. He underwent exchange transfusion promptly with 5 U prbcs with resulting HgSS of < 15%. P/F ratio improved from 183 to 260. Day 5 he developed a sick sinus node in the setting of septic shock requiring transvenous pacing. Day 12 he was extubated. Day 13 he developed bilateral subdural hematomas. He received more blood products and was transferred out of the ICU on Day 20. DISCUSSION: Among the acute crises that occur in sickle cell disease, acute chest syndrome is currently the only category which holds a strong recommendation for exchange transfusion. MOFS is rare in a sickle cell crisis, but has a high mortality and early recognition is key. It occurs in patients with significant hemolysis and is characterized by hemodynamic instability and compromise of at least two of three major organs (1). RBC transfusion is indicated (2), but the evidence for exchange transfusion in MOFS is less succinct (3). CONCLUSIONS: Influenza A can precipitate MOFS in SCD. Early recognition and timely exchange transfusion should be considered in MOFS, even in the initial absence of acute chest syndrome. Reference #1: 1. Hassell et al (1994). Acute multiorgan failure syndrome: a potentially catastrophic complication of severe sickle cell pain episodes. Am J Med. 96(2): 155-62. Reference #2: 2.Bernard et al (2016). Guidelines on red cell transfusion in sickle cell disease part II: indications for transfusion. British Journal of Haematology. 176:192-209. Reference #3: 3. Novelli, EM and MT Gladwin (2016). Crises in sickle cell disease. Contemporary Reviews in Critical Care Medicine. 149(4): 1082-1093. DISCLOSURES: No relevant relationships by Crystal Duran, source=Web Response No relevant relationships by Varsha Taskar, source=Web Response

Risk Factors of Severe Hyperbilirubinemia in Neonates Undergoing Exchange Transfusion in Imam Reza Hospital Kermanshah- Iran, during 2012 to 2016

Background Jaundice is one of the most common problems in neonatal period. Although the efficacy of exchange transfusion in the treatment of neonatal jaundice is knownand quickly reduces blood bilirubin levels, but severe neonatal hyperbilirubinemia is associated with significant morbidity and mortality. We conducted this study to investigate the risk factorsof severe hyperbilirubinemia in neonates undergoing exchange transfusion in Imam Reza Hospital Kermanshah, Iran. Materials and Methods In this retrospective study, records of newborns with hyperbilirubinemia who were undergoing exchange transfusion to Imam Reza hospital in Kermanshah from of 2012 to 2016 were studied. Information were extracted from the patient case and recorded in the data collection form. Data analyzed using SPSS software (version 24.0). Results Birth weight of 42.1% of neonates was less than 2,500 gr and 43.1% were preterm. The most common causes of severe hyperbilirubinemia including: unknown causes (42.2%, n=38), ABO incompatibility (27.8%, n=25), and sepsis (12.2%, n=11). The most common complications of exchange transfusion in this study were thrombocytopenia (33.4%), hypocalcaemia (18.7%), hyperglycemia (12.3%), and hypoglycemia (12.3%). Conclusion According to the results, major risk factors for the occurrence of severe hyperbilirubinemia in neonates were birth weight less than 2,500 gram, gestational age below 37 weeks, age less than 5 days, and weight loss more than 10% of birth weight at the time of admission.

Report about term infants with severe hyperbilirubinemia undergoing exchange transfusion in Southwestern China during an 11-year period, from 2001 to 2011.

ObjectivesThis study was intended to explore the etiology and risk factors of severe neonatal hyperbilirubinemia and to analyze the adverse events associated with ECT (Exchange Transfusion), as well as to identify the factors related to the poor prognosis.MethodsAll of the full-term neonates who had undergone ECT for hyperbilirubinemia at Children’s Hospital of Chongqing Medical University from January 2001 to December 2011 were enrolled in this study. General demographic characteristics, comorbidities, pre- and post-exchange TSB(Total Serum Bilirubin) levels, duration and frequency of ECT, and clinical outcomes were recorded and analyzed anonymously.ResultsOf 614 total infants, 368 patients (59.9%) with ABO incompatibility were identified, of whom 197 (53.5%) developed acute bilirubin encephalopathy (ABE) and 16 (4.3%) suffered a poor prognosis. The etiology was unidentified in 103 patients (16.8%), of whom 62 (60.1%) developed ABE and 9 (8.7%) had a poor prognosis. Identified adverse events secondary to ECT included thrombocytopenia (54.6%), hyperglycemia (42.8%), apnea (3.3%) and necrotizing enterocolitis (NEC) (1.3%). No ECT-related mortality was documented in this study.ConclusionsThe etiology, peak TSB level before ECT, and time of ECT had a significant impact on the outcome of severe neonatal hyperbilirubinemia. ABO incompatibility was the most common cause of extreme neonatal hyperbilirubinemia. Pathological weight loss could be involved in the development of extreme hyperbilirubinemia with an unidentified cause.

Severe Apnea in a Premature Infant after Accidental Vancomycin Overdose Responsive to Treatment with Exchange Transfusion

Background: Mostly seen toxicities following vancomycin are ototoxicity and nephrotoxicity. We here report a very low birth weight preterm neonate who developed severe episodes of apnea after accidental iatrogenic vancomycin overdose, responsive to treatment with double volume exchange transfusion. Case report: A preterm neonate weighing 1380 grams received two doses of 10-fold of the normal dose of vancomycin per kg in this age group. She developed sudden onset of frequent and severe episodes of apnea, which required noninvasive ventilation. Using fluorescence polarization immunoassay, serum vancomycin level was found to be 84 μg/mL 10 hours after the last dose. The patient underwent exchange transfusion. Apnea episodes terminated 12 hours after exchange transfusion. The blood level of vancomycin decreased from 84 μg/mL before exchange to 67 μg/mL immediately post-exchange and eventually to less than 1 μg/mL in 36th hour after exchange. Discussion: Target peak concentration of vancomycin in neonates is between 20 and 40 μg/mL and trough concentration ranges from 5 to 10 μg/mL. Peak serum concentration of our patient can be back extrapolated to be about 336 μg/mL which was higher than the target level. This high plasma levels of vancomycin might be the cause of apnea in our patient as evidenced in similar reports. Conclusion: Apnea is a potential sign of vancomycin overdose in neonates and infants treated with this antibiotic. Exchange transfusion is a potential effective treatment to rapidly resolve this unwanted complication.

Exchange Transfusion for Neonatal Hyperbilirubinemia in Johannesburg, South Africa, from 2006 to 2011.

Background. Severe hyperbilirubinaemia requiring exchange transfusion has become less common in recent years; however, kernicterus still occurs. The aim of this study was to review babies undergoing exchange transfusion for severe hyperbilirubinaemia in a Johannesburg hospital. Methodology. This was a retrospective review of babies who required exchange transfusion in both the neonatal and the paediatric wards from June 1, 2006, to December 31, 2011. Results. There were 64 patients who underwent 67 exchange transfusions. Isoimmune haemolysis (both Rh and ABO incompatibility) was the cause of jaundice in 9/64 (14%). Most babies who underwent exchange transfusion were sick or preterm and were admitted in hospital after birth (38/64; 59.5%); three of these babies died, but not during the exchange transfusion (3/38; 7.9%); all three had signs suggestive of neonatal sepsis. The remaining 26 babies (40.6%) were readmitted to the paediatric wards for exchange transfusion. Six of these babies (6/26; 23.0%) had signs of kernicterus. The most significant complication of exchange transfusion was apnoea requiring mechanical ventilation in three patients (3/64; 4.6%). Conclusion. Despite a relatively low number of babies undergoing exchange transfusion, kernicterus still occurs and must be prevented. Proper protocols for screening and management of severe hyperbilirubinaemia need to be enforced.

Acute Kidney Injury Is Prevalent Among Pediatric Admissions for Acute Chest Syndrome

Background: Growing evidence shows that acute kidney injury (AKI) leads to chronic kidney disease in pediatric and adult populations. Patients with sickle cell disease (SCD) that develop acute chest syndrome (ACS) are at risk for developing AKI due to the hypoxic, acidic environment in the renal medulla, the use of nephrotoxic agents (vancomycin, NSAIDs), hyposthenuria and potential dehydration experienced by patients prior to admission for ACS. As AKI and acute lung injury are linked by systemic organ cross-talk, AKI with or without oliguria, may exacerbate the pathologic state of ACS. For these reasons, improving our understanding of the epidemiology and pathophysiology of AKI during sickle cell crisis is paramount.Methods: We conducted a six year retrospective observational evaluation of 236 episodes of ICD-9 defined ACS among 166 admitted pediatric patients. We excluded patients admitted for one day or had normal CXR throughout hospitalization despite being coded as ACS. AKI was defined by KDIGO definition of either a ≥ 0.3 mg/dL or 50% increase in serum creatinine (SCr) from baseline. Forty eight episodes were excluded as a baseline SCr was not identified. Variables analyzed included age, gender, SCD genotype, length of stay, CBC (baseline and admission), current SCD therapy, transfusion intervention and length of stay (LOS). Per institutional standard of care, patients with ACS are treated with Vancomycin, Azithromycin, and Ceftriaxone and receive NSAIDS (ibuprofen or toradol) for pain/fevers but the type/timing of transfusion is determined by SCD provider preferences. Logistic regression analysis was performed to assess associations between clinical variables and AKI for all patients and severe phenotypes. Multiple linear regression analysis was performed to determine if AKI was independently associated with hospital length of stay.Results: The mean age of patients admitted with ACS was 9 years (range 0.8yrs-19) and 61% were male. Genotypes included SS (79%) SB0 thalassemia (4%), SB+ thalassemia (5%), SC (12%) and SD (1%). AKI was identified in 13 of 188 (7%) episodes of ACS. The majority of cases developed AKI within two days of admission and resolved 2.5 days later. Compared to children without AKI, those with AKI had a lower admission hemoglobin (6.3 vs 7.5 g/dL, p=0.02), lower decrease in hemoglobin from baseline (3.2 vs. 1.3 g/dL, p<0.001), and higher admission WBC (32.3 vs. 20.3 x103/uL, p<0.001). Finally, need for exchange transfusion was associated with AKI (χ2 = 9.8, p=0.007). Seven of 39 patients (18%) that underwent exchange transfusion, 5 of 137 (4%) simple transfusion patients, and 1 of 16 no transfusion patients (the one patient had severe RBC antibodies so was not transfused) developed AKI. The patients with and without AKI were similar for age, gender, SCD genotype, platelet count, and current SCD therapy. When evaluating whether AKI was associated with longer LOS, we found that those with AKI had longer LOS than those without (12.4 vs 5.0 days, p<0.001). Using nominal logistic regression, drop in hemoglobin from baseline (p<0.001), admission WBC (p=0.01), and length of stay (p<0.001) remained significantly associated with AKI (Χ2 =42, p<0.001). When excluding patients with milder SCD phenotypes (SC and SB+ thalassemia), similar results were observed. Among 164 ACS events with severe SCD genotypes, 11 (14%) patients developed AKI. Using nominal logistic regression to assess severe SCD genotypes, drop in hemoglobin, admission WBC, and LOS were significantly associated with the development of AKI (Χ2=36, p<0.001).Conclusion: This study demonstrates that AKI is prevalent during admissions for ACS and is highly associated with a drop in Hb and increase in WBC on admission and the need for exchange transfusion. While AKI presented and resolved early during hospitalization, it is strongly associated with a prolonged hospital course. The impact of AKI on the pathophysiology of ACS in the acute setting as well as its role in progression to SCD nephropathy should be studied prospectively. DisclosuresNo relevant conflicts of interest to declare.

Exchange transfusion in complicated pediatric malaria: A critical appraisal.

Complicated falciparum malaria is a killer disease resulting in high mortality in spite of appropriate treatment. Some workers have reported improved survival when adjunct exchange blood transfusion is included in the treatment modality while others opine against it. This review is an effort to address and critically appraise current evidence for the treatment mode for severe malaria. The literature was searched with a specified search strategy to identify reports of children who underwent exchange transfusion for severe malaria. Total 23 children who underwent exchange transfusion for severe falciparum malaria published by 9 authors were identified. Age ranged from 5 months to 16 years with a mean age of 6.4 years. The average preprocedure parasite index (PI) was 41.4% (95confidence interval [CI]; 31.2-51.4). The average blood volume exchanged was 118.6% (95% CI; 94.7-143) of the circulating blood volume. The average postexchange reduction in PI was 34.1% (95% CI; 25.4-42.8). Three out of 23 children encountered some complications. All the children survivedKeywords: Exchange blood transfusion, parasite index, pediatric Intensive Care Unit, red cell exchange, severe falciparum malaria.

Problems with using total serum bilirubin as a criterion for phototherapy in extremely low‐birthweight infants

Despite the early use of phototherapy and exchange transfusion in premature infants based on total serum bilirubin (TSB), the reemergence of kernicterus has been reported. The aim of this study was to assess the validity of using TSB as the criterion for phototherapy in extremely low-birthweight infants (ELBWI). We reviewed the medical charts of 43 ELBWI admitted to hospital between January 2009 and December 2010, and analyzed the relationship between TSB and unbound bilirubin (UB). No infant underwent exchange transfusion or developed acute bilirubin encephalopathy. There was a significant correlation between TSB and UB measured immediately before phototherapy during the first 7 days of life (r = 0.657, P < 0.001), but none thereafter (r = 0.120, P = 0.213). Thirty-seven percent of infants who underwent phototherapy during the first 7 days of life had suprathreshold USB but subthreshold TSB, whereas this rose to 97% thereafter. No correlation was observed between TSB and UB in ELBWI after the first 7 days of life, and almost all phototherapy sessions were initiated based on the UB criterion, even though TSB was below the accepted threshold. UB may be high if jaundice is evaluated solely on the basis of TSB.

Encefalopatía por Kernicterus: Serie clínica

Kernicterus (bilirubin encephalopathy): case reports Introduction: Jaundice is common in newborn babies (NB). Bilirubin levels of 20 mg/dL or higher may cause choreoathetoid cerebral palsy, sensorineural hearing loss, eye disorders and enamel dysplasia in term infants; clinical picture compatible with kernicterus. Objective: To describe five cases of kernicterus treated at a Neu - rology Unit between 2002 and 2012. Case reports: Five cases of babies with gestational ages between 35 and 39 weeks, birth-weight ranging from 2580 to 4250 grams and bilirubin levels between 24 and 47 mg/dL are presented. Two infants were at home when acute encephalopathy developed, all were treated with photothera- py and 3 of them underwent exchange transfusion. The age of diagnosis of kernicterus was between 12 days to 10 years; three patients were diagnosed in neonatal period through MRI that revealed basal ganglia impregna- tion. All patients evolved presenting movement disorders of varying severity. Three of them were diagnosed with sensorineural hearing impairments and two presented eye disorders. Psychometric tests showed cogni- tive delay in three patients and normal development in the remaining children. Conclusion: Kernicterus in a devastating disease present in the national reality. It is a preventable cause of cerebral palsy; therefore, it is necessary to educate parents, population and health care professionals about neonatal hyperbilirubinemia early detection and treatment. (Key words: Kernicterus, bilirubin, basal ganglia, cerebral palsy, hearing loss). Rev Chil Pediatr 2013; 84 (6): 659-666