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Vascular occlusive crisis with a concurrent vision loss on both eyes is one of the most devastating disability for sickle cell disease patients. Reportedly occlusive crisis in the eyes is usually temporary whereas if not appropriately managed can result in permanent vision loss. A carefully managed sickle cell crisis could prevent multiple disabilities including blindness and stroke. We report a case of a 24-year-old female with a history of sickle cell disease who had acute bilateral vision loss during a sickle crisis and recovered significantly with a timely emergent erythrocytapheresis. [ABSTRACT FROM AUTHOR]

Little is reported in the literature regarding hereditary spherocytosis (HS) and cardiopulmonary bypass (CPB). We present a case of a 19-month-old girl child who was referred for an atrioventricular septal defect (AVSD) and HS. The patient underwent surgical repair, and an exchange transfusion was performed at the initiation of CPB. No significant hemolysis or events attributed to HS were observed during or after CPB. The surgical repair of an AVSD in a pediatric patient with HS and total volume exchange transfusion is herein reported. [ABSTRACT FROM AUTHOR]

Sickle cell disease patients are commonly treated at transfusion medicine services, and understanding of the hepatic manifestations of the disease is key for optimal management, specifically, in individuals presenting with sickle cell intrahepatic cholestasis (SCIC). SCIC represents a rare, severe hepatic crisis wherein sinusoidal red cell sickling leads to massive hepatocyte dysfunction and cholestatic laboratory findings. Acute SCIC is defined by abdominal pain with progressive hepatic injury associated with hyperbilirubinemia, renal failure, encephalopathy, and coagulopathy. Patients are generally managed with red blood cell exchange transfusion (RBCEx), when available, as this is a potentially fatal condition. Simple transfusion may be utilized in resource-poor environment or when patients refuse RBCEx. As less than 50 adult cases have been described in the literature, many of them with limited follow-up, randomized clinical trials comparing RBCEx with other treatments are currently unfeasible. Likewise, a chronic form exists, but is less well characterized, and is associated with persistent bilirubinemia and a variable course in terms of progressive hepatic disease. We undertake a brief review of the literature and discuss two cases of SCIC managed with RBCEx at our institution.

Background and Objectives: Management of severe allergic transfusion reactions (ATR) is challenging. In this study, we investigate the usefulness of skin tests and basophil activation tests (BAT) in chronically transfused patients for the prevention of future ATR., Materials and Methods: BAT and skin tests were carried with the supernatant of red blood cell (RBC) units for a sickle-cell disease patient under chronic exchange transfusion who has presented a severe ATR, in order to prevent potential future ATR. If the results for both BAT and skin tests were negative, the RBC units could be transfused to the patient. If either one of the results was positive, the tested RBC unit was discarded for the patient., Results: 192 RBC units were tested with both tests. The level of results concordance between the two tests was 95%. Out of the 169 negative units with both tests, 118 units were transfused to the patient for which he presented no ATR., Conclusion: In our study, combining both BAT and skin tests was associated with a good negative predictive value since we were able to safely transfuse our patient. Further studies are still necessary to confirm this result but this pilot study indicates that skin tests and BAT might help prevent ATR. When BAT is not available, skin tests may also be useful in preventing ATR., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (© 2024 Akiki, Dedeken, Ferster, Doyen, Dupire, Nagant, Smet, Ghorra, Ruth, Lauwers, Daubie, Corazza and El Kenz.)

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

Background: Pregnancy in women with sickle cell disease (SCD) is associated with a number of fetal complications such as intra-uterine death, intra-uterine growth restriction (IUGR), preterm birth, low birth weight and an increased perinatal mortality and morbidity. Hyperbilirubinaemia necessitating exchange transfusion in an infant of a mother with SCD, to the best of our knowledge, has not been previously described. Case presentation: An infant was delivered at 33 weeks and 5 days of gestation due to a maternal sickle cell crisis. The infant had an unconjugated bilirubin level of 153 μmol/L on admission to the neonatal intensive care unit at 30 min of age. Phototherapy was immediately commenced, intravenous immunoglobulin administered and then a double-volume exchange transfusion was performed. There was, however, no evidence of haemolysis in the infant and the infant's haemoglobin level remained stable following the exchange. No further exchange transfusions were required. The mother had a high unconjugated bilirubin level (151 μmol/L) prior to delivery. Conclusion: High neonatal unconjugated bilirubin levels necessitating exchange transfusion can occur due to haemolysis in the maternal circulation, in this case due to SCD. [ABSTRACT FROM AUTHOR]

The article discusses a 2019 study on fat embolism syndrome (FES) in sickle cell disease (SCD) and its diagnosis and emergency therapies. Topics covered include the findings of predilection for non-homozygous patients and those with previous mild disease (PMD) and of infection with human parvovirus B19 (HPV B19), and the use of red cell exchange transfusion (RCE) with varying outcomes. Also noted is the pre-emptive treatment with RCE +- therapeutic plasma exchange (TPE) before organ failure.

To explore the effects of exchange transfusion on auditory neuropathy spectrum disorder (ANSD) in neonates with severe hyperbilirubinemia (SH). The clinical data of 2216 SH neonates who met the standard of exchange transfusion and 732 non severe-hyperbilirubinemia (NSH) neonates in the same period who did not require exchange transfusion in the neonatology department of Childrens' Hospital of Chongqing Medical University between January 2010 and December 2015 were retrospectively analyzed. In addition, the SH neonates were further divided into the exchange transfusion group and photography group. Hearing screening was conducted on all neonates using transiently evoked otoacoustic emission (TEOAE) and auto auditory brainstem response (AABR), and neonates who failed the above screening were performed diagnostic hearing test. And then neonates diagnosed with hearing disorder were followed up for 2–5 years. The pass rates of hearing screening were 80.58%, 79.71% and 87.84% in the phototherapy group, exchange transfusion group and NSH group respectively, with a significant difference(P < 0.05). Hearing loss was diagnosed in 10.15%, 12.39% and 8.54% of neonates in the phototherapy group, exchange transfusion group and NSH group. After follow-up, ultimate incidence rates of ANSD were 11.96%, 11.57% and 2.4% respectively in the 3 groups, with a significant difference (P < 0.05). SH is one of risk factors for ANSD. SH neonates have a lower incidence of ANSD in the exchange transfusion group than in the phototherapy group. Neonates who meet the standards of exchange transfusion adopt this therapy in early stage, which can quickly decrease bilirubin level and ultimately reduce incidence of ANSD. [ABSTRACT FROM AUTHOR]

• Neonatal BET humoral immune response to diphtheria and tetanus vaccine. • Neonatal BET affects occurrence of autoimmune antibodies. • Neonatal BET does not affect morbidity and the occurrence of autoimmune diseases. • Children after neonatal BET should follow official vaccination schedules against diphtheria and tetanus. A blood exchange transfusion (BET) is most commonly performed to treat severe neonatal haemolytic disease. A distinct form of blood transfusion adverse reaction is transfusion-related immunomodulation. The purpose of our retrospective single-centre case-control cohort study was to investigate whether a blood exchange transfusion in the neonatal period provokes immunomodulation and affects humoral immune response to vaccination, morbidity and occurrence of autoantibodies. Study subjects were 74 apparently healthy children, who were born at term as appropriate for gestational age and received four doses of diphtheria and tetanus toxoid vaccine. Forty-one received BET due to neonatal hemolytic disease and no other blood product afterwards, while 33 did not receive any blood products. Analysis of diphtheria, tetanus and autoimmune antibodies was performed and their medical records were analyzed for infectious, allergic, cancerous and autoimmune diseases. A clearly exaggerated immune response to diphtheria (1.016 IU/mL, 95% confidence interval (CI) 0.662–1.369 IU/mL vs. 0.515 IU/mL, 95% CI 0.363 to 0.626 IU/mL, P = 0.011) and slightly exaggerated immune response to tetanus vaccine (1.798 IU/mL, 95% CI 1.180–2.416 IU/mL vs. 1.036 IU/mL, 95% CI 0.398–1.673 IU/mL, P = non-specific) were observed in BET subjects. A propensity towards autoimmunity (25.8% vs. 12.5%, P = non-specific) was observed in BET subjects. However, BET in the neonatal period did not influence the occurrence of bacterial, childhood viral diseases with exception of varicella (43.9% vs. 21.2%, P = 0.040), autoimmune and cancer diseases. BET impacted humoral immune response to diphtheria and tetanus vaccine and occurrence of autoimmune antibodies, but did not affect morbidity and the occurrence of autoimmune diseases. These effects could be related to massive antigenic load of BET and an accelerated priming of immune cells and consequent immunomodulation. [ABSTRACT FROM AUTHOR]

Background: Exchange transfusion (ET) for hyperbilirubinemia is associated with many complications. The complications are underreported as most of the published studies are retrospective, used varying definitions of adverse events (AEs) and variable follow-up periods. Aim: We evaluated the incidence of clinical, biochemical, hematological, and radiological AEs, including serious AEs, within 2 weeks of ET for hyperbilirubinemia in neonates, using standard definitions. Materials and Methods: This prospective observational study was conducted in level III newborn unit of north India from February 2008 to February 2009. We enrolled consecutive inborn and outborn neonates admitted with hyperbilirubinemia and required ET. Babies requiring partial exchange for anemia/polycythemia or ET for indications other than hyperbilirubinemia were excluded. They were prospectively monitored for clinical, biochemical, hematological, and radiological AEs up to 2 weeks following the procedure. We calculated the incidence/AE rate (AER) as the rate of events per 100 ET and compared them among various groups using the Chi-square test. The SPSS v20 was used for the analysis, and value of P < 0.05 was considered as statistically significant. Results: A total of 202 neonates were screened and 141 neonates (182 ET) were enrolled. The overall AER was 112/100 ETs. The most common AE was biochemical (45.6/100 ET), followed by hematological (44.5/100 ETs), clinical (15.9/100 ETs), and radiological (8.9/100 ETs). Severe AER was 12.6/100 ETs. The AER was significantly more in lower gestation and birth weight groups. Conclusion: ET is a high-risk procedure and should be performed only when the benefit of the procedure offsets the risks. [ABSTRACT FROM AUTHOR]

Background: Infant jaundice is one the most common causes of hospitalization in infant in the first month of birth, which is defined an abnormal increase in blood bilirubin levels. Exchange transfusion is the recommended treatment for neonatal jaundice who do not respond to phototherapy and experience dangerous complication of jaundice and signs of kernicterus. However, this treatment may lead to complications such as thrombocytopenia. This study aimed to investigate the severity and duration of thrombocytopenia following the exchange transfusion in neonatal jaundice. Material and Methods: This cross section study was performed on 217 infants. Infants with a gestational age of 35 to 42 weeks and bilirubin levels of above 17 mg/dl, who were undergoing treated with exchange transfusion, entered in this study. This study was conducted from 2012 to 2018 in the Ghaem Hospital (Mashhad, Iran). The samples were selected by convenience sampling. The platelet count was measured before exchange transfusion, right after exchange transfusion, 6 hours after exchange transfusion, and platelet count continued until platelet level was normal. At the time of discharge, platelet levels were re-measured. Results: Among the samples, 104(53.8%) were males and 89 (46.2%) females. Of the infants who were transfused, 15% had thrombocytopenia. After the exchange transfusion, 80% of infants had thrombocytopenia. The mean platelet count before the exchange transfusion was 299,180 per mm3 of blood, and it was 105.140 per mm3 of blood after the exchange transfusion. With respect to severity of this complication, 86% of the thrombocytopenia after exchange transfusion was mild to moderate. Conclusion: In this study, nearly one-sixth of the infants who needed exchange transfusions had thrombocytopenia that most of them had platelet of more than 100000. Thrombocytopenia is associated with jaundice and can be exacerbated by phototherapy. [ABSTRACT FROM AUTHOR]

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

Abstract Babesia divergens is an intraerythrocytic parasite, which is the major cause of babesiosis in Europe. For years, clinicians have been publishing stunning case reports that describe how some – but not all – conventional red blood cell (RBC) exchange transfusions have saved the lives of severely ill babesiosis patients. Due to markedly different patient outcomes, clinicians agree that new treatments and additional studies are needed. Here we argue that we should evaluate "therapeutically-rational exchange" (T-REX) in which the RBCs used to replace Babesia -parasitized RBCs are special disease-resistant RBC genetic variants (instead of the nondescript, "standard issue" RBCs used in conventional exchanges). T-REX seems prudent because with conventional exchange only some units of "standard issue" RBCs may be disease-resistant, while other units may not protect or may even promote disease. The random selection of RBCs for conventional RBC exchange may explain why clinical outcomes can vary dramatically. Fortunately, researchers have found that M antigen-negative (M−) and S antigen-negative (S−) RBCs resist invasion by B. divergens. Thus, we recommend evaluating T-REX of RBC variants that are B. divergens invasion-resistant: RBCs that are (1) M−, (2) S−, or (3) both M− and S−. By using only Babesia -resistant RBCs, T-REX eliminates the risk of unintentionally infusing Babesia -susceptible RBCs that might increase the severity of babesiosis. Because the T-REX variation of the conventional RBC exchange procedure is feasible, safe, and biologically plausible, we feel T-REX of Babesia -resistant RBCs should now be evaluated. [ABSTRACT FROM AUTHOR]

Background: Jaundice is a common neonatal problem. This may be due to the limited ability of a neonate to metabolize indirect bilirubin, which predisposes to the risk of encephalopathy and long-term sequelae if not managed promptly. Sufficient hydration and good urine output improve the efficacy of intensive phototherapy The aim of this study: was to evaluate the role of intravenous extra fluid supplementation in accelerating the reduction of serum bilirubin levels in neonates and to find out whether intravenous fluid supplementation decrease the total duration of phototherapy and the need for exchange transfusion Methods: This is a prospective clinical trial study that was conducted at phototherapy ward at Karbala teaching hospital for children, Iraq from January 2017 to December 2017. Fifty-two term neonates with non-hemolytic hyperbilirubinemia [total serum bilirubin >18 mg/dl (308 pmol /L) and <25 mg/dl (428 pmol /L)] were randomly divided into 2 equal groups; (non-supplemented group) received breast milk and or formula, and (supplemented group) given intravenous fluid besides breast milk or formula. Both groups exposed to intensive phototherapy. The rate of bilirubin decrement, duration of phototherapy, and rate of exchange transfusion were compared Results: Rate of bilirubin decrement at 4 hours, 8 hours, 24 hours of study were significantly higher in supplemented group as compared to non-supplemented group (p- value <0.001). Duration of phototherapy required in non-supplemented and supplemented groups was 56.54 vs. 41.54 hours respectively (p-value =0.0001). No statistically significant effect of intravenous extra fluid supplementation on exchange transfusion rate between these two groups. Conclusions: Intravenous extra fluid supplementation in non-hemolytic jaundiced term neonates can accelerate decrement of serum bilirubin levels and decreases the duration of phototherapy [ABSTRACT FROM AUTHOR]

Acute pancreatitis is one of the dreaded complications of hypertriglyceridemia. According to the literature, 7% of cases of acute pancreatitis are due to hypertriglyceridemia or chylomicronemia associated with genetic defects in the lipid metabolism. Here, we report a 17-day-old baby, who presented as a case of necrotizing enterocolitis. Incidental phlebotomy showed pink creamy serum. This highly suggestive of hypertriglyceridemia, lipid profile study was done that confirmed elevated serum triglyceride (TG) and cholesterol levels with decreased high-density lipoprotein. To minimize the risk of pancreatitis, exchange transfusion, a procedure widely performed in neonatology was sought to, that dramatically reduced the serum TG and cholesterol levels. Baby was then started on skimmed milk, fenofibrate, and medium-chain TGs oil to maintain the plasma TG levels low. Genetic analysis confirmed the diagnosis of type I hyperlipoproteinemia/familial combined hyperlipidemia-3. This report emphasizes the importance of suspecting hypertriglyceridemia on witnessing milky serum so that early intervention with novel modalities such as exchange transfusion can prevent complications such as acute pancreatitis. [ABSTRACT FROM AUTHOR]

Strengthened recommendation on neonatal platelet transfusion thresholds New additional wording is shown in italics: B For preterm neonates with very severe thrombocytopenia (platelet count below 25 × 10 SP 9 sp /l) platelet transfusions should be administered in addition to treating the underlying cause of the thrombocytopenia (2C). Apart from the change in plasma component specifications, other information on plasma transfusion in the guidelines, e.g. indications or typical transfusion volumes and rates, is essentially unchanged. Platelet components for neonates, infants and older children Neonatal platelets - should be used for neonates and infants (see I New neonatal/infant specification platelet component in plasma/platelet additive solution i above) For children from 1 year of age, either standard apheresis or pooled platelets may be used. [Extracted from the article]

Neonatal liver failure (NLF) is a rare diagnosis but carries with it significant risks of mortality and morbidity. Common etiologies for NLF include metabolic causes, gestational alloimmune liver disease (GALD or neonatal hemochromatosis), and viral infections. We report a case of liver failure in a premature infant with abnormal iron profile within 48 hours of birth. Lack of accepted guidelines for the initial management of severe jaundice with a high direct component in the first week after birth made treatment challenging. The infant underwent intensive phototherapy along with four exchange transfusions (ET) and two courses of intravenous immunoglobulins (IVIG). The clinical goals were to keep total bilirubin values ≤ 20 mg/dL in this premature neonate and to minimize the risk of bilirubin-induced neurologic dysfunction and decompensated liver failure. Abnormal iron studies and later magnetic resonance imaging were suggestive of GALD. Liver functions improved over time with normal neurodevelopmental assessment at 3 years of age. To conclude, in infants with NLF soon after birth, earlier consideration of IVIG/ET in the first few days may be beneficial. Larger multicenter data analyses are required to formulate treatment guidelines and indications for phototherapy, ET, and IVIG in sick neonates with NLF.

Introduction: Haemolytic disease of foetus and newborn due to Rh-D alloimmunisation was one of the grave complications of pregnancy years back, which contributed for a number of perinatal deaths and disabilities. Yet, advancement in technologies used for early detection and treatment of haemolytic disease of newborn as well as better neonatal care has further contributed to bring down the magnitude. Our study attempts to follow-up Rh-D alloimmunised pregnancies and collect data regarding the perinatal and postnatal plight of affected neonates. Aim: To follow-up Rh-D alloimmunised pregnancies and describe the perinatal and postnatal characteristics of affected neonates. Materials and Methods: This two year prospective study was done on Rh-D alloimmmunised women. Diagnosis of haemolytic disease of newborn due to Rh-D alloimmunisation was confirmed if a positive Direct Coomb’s Test (DCT) was found in an Rh-D positive baby. Severity of disease in terms of haemoglobin and bilirubin levels and presence of hydrops were assessed. Newborns were followed-up till discharge. Results: Out of 2496 Rh-D alloimmunised women, 78 antenatal cases were found positive for anti D-antibodies. Frequency of haemolytic disease of foetus and newborn was 57 out of 64 cases which were followed, four newborns were DCT negative although, Rh-D positive and three newborns were Rh-D negative. Mean cord Hb in unaffected newborns were significantly higher. Twenty nine out of 58 live born newborns needed no treatment. Four cases (6.25%) needed exchange transfusion. Out of 60 live born infants, 59 survived. Overall survival rate of newborns in 64 alloimmunised pregnancies was 92.18%. The survival rate in live borns was 98.33%. Conclusion: Severe cases of haemolytic disease of newborn in Rh-D alloimmunisation is limited to

Neonatal hemochromatosis (NH) has been defined as neonatal liver disorder accompanied by extrahepatic siderosis, and gestational alloimmune liver disease (GALD) is the main cause of NH. We report an atypical case of NH that may have gone underdiagnosed. A male infant was born at term after an uneventful antenatal period. At 7 h of life, he was noted to be tachypneic. Chest X-ray was normal and capillary blood gas analysis showed severe lactic acidosis. An extended blood test showed elevated levels of tyrosine and methionine that, after excluding an inborn error of metabolism, led to the diagnosis of acute liver failure. Hyperferritinemia and elevated transferrin saturation were suggestive of NH. Extrahepatic siderosis on MRI confirmed the diagnosis of NH, so even though the infant was in good general condition, a dose of intravenous immunoglobulin was administered and double volume exchange transfusion was performed. Treatment of a suspected case of GALD and prevention in subsequent gestations is imperative. [ABSTRACT FROM AUTHOR]