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Aims: To define patterns of brain injury and associated neurodevelopmental outcomes in infants with severe neonatal anaemia.Methods: We studied 20 infants with severe anaemia at birth (haemoglobin<7g/dL). Clinical details were analysed for causes of anaemia and co-morbidities. All had early brain magnetic resonance imaging (MRI) scans, which were reviewed for injury pattern. Neurodevelopmental outcomes were assessed at a median age of 24months.Results: The aetiology of the anaemia was feto-maternal haemorrhage in 17 and antepartum haemorrhage in 3 infants. The predominant site of injury was the white matter, which was affected in all infants, with differing grades of severity and with cystic evolution in 45%. Only one infant showed an injury pattern typical of an acute severe hypoxic-ischaemic insult. Outcomes correlated closely to the severity of MRI findings. Cerebral palsy was seen only with the most severe neuroimaging patterns (n=6). Global developmental delay, learning or behavioural problems and seizures were common with moderate injury. Visual impairment occurred, particularly with posterior injury. Microcephaly developed in 45%.Interpretation: Severe neonatal anaemia at birth was associated with a white matter predominant pattern of injury, the severity of which was related to neurodevelopmental outcomes. Early MRI and long-term follow-up are advisable following severe neonatal anaemia. [ABSTRACT FROM AUTHOR]

Objectives: To analyze the role of middle cerebral artery (MCA) peak systolic velocity (PSV) in the prediction of severe fetomaternal hemorrhage (FMH) and to compare it with standard biophysical assessment. Data Sources: Retrospective review of cases of FMH seen in our unit and systematic review of the literature. Results: We followed the MOOSE guidelines to review the literature. From 838 articles, 16 were selected. In total, 35 women, including 3 cases from our center and 32 obtained from the literature search were included. Diagnosis of FMH was always confirmed by laboratory tests. Patients were seen at 31 ± 5 weeks' gestation (range 16-39) and the most frequent indication for referral was decreased perception of fetal movements. Cardiotocography (CTG) upon admission was sinusoidal in 18 cases, nonreactive in 6, decelerative in 2 and tachycardic in one. MCA-PSV was abnormal in all cases but one. There were 2 perinatal deaths. The mean hemoglobin concentration at birth or at intrauterine transfusion was 4.8 ± 1.9 g/dl. Discussion: The most accurate predictor of FMH was fetal MCA-PSV. CTG was always abnormal but the pattern was frequently nonspecific. We suggest including fetal cerebral Doppler in the evaluation of patients with decreased fetal movements, particularly in those cases with ambiguous results of biophysical testing. [ABSTRACT FROM AUTHOR]

Transcription factor control of cell-specific downstream targets can be significantly altered when the controlling factor is mutated. We show that the semi-dominant neonatal anemia (Nan) mutation in the EKLF/KLF1 transcription factor leads to ectopic expression of proteins that are not normally expressed in the red blood cell, leading to systemic effects that exacerbate the intrinsic anemia in the adult and alter correct development in the early embryo. Even when expressed as a heterozygote, the Nan-EKLF protein accomplishes this by direct binding and aberrant activation of genes encoding secreted factors that exert a negative effect on erythropoiesis and iron use. Our data form the basis for a novel mechanism of physiological deficiency that is relevant to human dyserythropoietic anemia and likely other disease states. [ABSTRACT FROM AUTHOR]

Preterm birth is defined as all births before 37 completed weeks of gestation. Preterm birth can be further sub-divided based on gestational age: extremely preterm (<28 weeks), very preterm (28 to <32 weeks) and moderate preterm (32 to <37 weeks). Retrospective observational case–control study review (1 year i.e., 2015) is to assess pregnancy outcome among women with gestational diabetes mellitus (GDM) delivering very preterm (28 + 0/7 to 31 + 6/7 weeks of gestation). The study group included all women diagnosed with GDM and were compared to a control group of women delivering at the same gestational age period but without GDM. In all, 30 women were included in the study, of whom 15 were diagnosed with GDM and 15 were not. All women had a cesarean delivery. Neonates of mothers with GDM had higher ponderal index. Birth weight had the positive associations with blood glucose of newborn (r = 0.37,p = 0.047) and term of births (r = 0.52,p = 0.003). Birth weight had the negative associations with maternal systolic pressure (r = −0.7,p < 0.05), pre-eclampsia (r = −0.6,p < 0.05) and maternal pre-pregnancy body mass index (r = −0.5,p < 0.05). There were no differences in mortality or other parameters for neonatal morbidity, including bronchopulmonary dysplasia, prematurity retinopathy, neonatal anemia. According to our data, very preterm delivery occurring in women with GDM does not confer an increased risk for neonatal complications. [ABSTRACT FROM AUTHOR]

Based on small studies and not on statistically valid clinical trials, guidelines for neonatal transfusions remain controversial and practices vary greatly. Premature infants and critically ill neonates in the neonatal intensive care unit (NICU) often require blood transfusions and extremely preterm neonates receive at least one red blood cell transfusion during their hospital stay. Transfusions to neonates convey both benefits and risks and conseguently it is imperative to establish specific guidelines to improve practice and avoid unnecessary transfusions. Appropriate and lifesaving platelet transfusion in thrombocytopenic bleeding neonates pertains to 2% of all neonates in NICUs. Inversely, 98% of platelet transfusions are given prophylactically, in the absence of bleeding, with the assumption that this reduces the risk of a serious hemorrhage. To date, no evidence base is available for assigning a platelet transfusion trigger to NICU patients. Each NICU should approve specific guidelines that best suit its local clinical practice. Therefore, whatever guidelines are chosen in deciding when to transfuse, what is most important is to adhere strictly to the guidelines adopted, thus limiting unnecessary transfusions that convey no benefits and carry both known and unknown risks. [ABSTRACT FROM AUTHOR]

Objective: We investigated the effects of delayed and early clamping of the cord on the hematologic status of the baby at birth and at the end of second month.Methods: Umbilical cord of 74 babies were clamped in the first 30 s (Group 1) and 76 were clamped at 90-120 s (Group 2). Levels of hemoglobin, hematocrit, iron and ferritin were analyzed from the umbilical cord blood at birth and from the venous samples at the end of second month.Results: Hemoglobin, hematocrit, iron and ferritin levels of cord blood were similar in both groups. However, their levels other than ferritin were higher in Group 2 at the end of second month. Two babies had respiratory distress and twelve neonates received phototherapy in Group 2 whereas only five neonates received phototherapy in Group 1.Conclusion: Term babies to whom delayed cord clamping was performed had improved hematological parameters at the end of second month. Therefore, delaying cord clamping in these babies may be a favorible approach in preventing anemia. [ABSTRACT FROM AUTHOR]

Introduction In various countries, standard doses of anti-D IgG used for postpartum immunoprophylaxis of hemolytic disease of fetus and newborn ( HDFN) vary from 100 μg to 300 μg. There are also different regulations concerning FMH assessment, and opinions about applicable tests are inconclusive. Methods Three flow cytometry tests ( FCTs) with anti-D, anti-HbF, anti-HbF+ CA antibodies, and two modifications of microscopic Kleihauer-Betke test ( KBT) were used. Results In all artificial mixtures with known concentrations, FCTs and KBT with counting 10 000 RBCs had similar satisfying sensitivity and specificity. KBT with counting 2000 RBCs had to be disqualified because of significant discrepancies between expected and measured values of FMH. The test procedure with anti-D was easier and shorter than the remaining tests, but it can be only used for FMH assessment in RhD-negative mothers with RhD-positive newborns. In one clinical sample, it was impossible to distinguish fetal RBCs from maternal F cells in KBT and FC with anti-HbF but other tests were useful. Conclusion In the four tests, correlation between expected and obtained results was appropriate ( CCC ̴1). Each test had some advantage and limitation in any clinical situation. Therefore, it is best to have opportunity to perform two or three assays in the laboratory. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study is to investigate the effect of second trimester anemia on maternal and perinatal outcomes in twin pregnancies. Methods: A retrospective population-based study was conducted, comparing maternal and neonatal outcomes in women carrying twins, with second trimester anemia (defined as hemoglobin < 10 g/dl) to those without anemia (defined as hemoglobin > or equal to 10 g/dl). Deliveries occurred in a tertiary medical center in 2013. Results: During the study period, there were 307 twin deliveries. Hemoglobin levels were available for 247 (80.4%) twins; 66 (26.7%) of these had anemia (<10 g/dl) during the second trimester. Women with second trimester anemia had a higher parity (p= 0.03), and needed more blood transfusions than those with hemoglobin level > or equal to 10 g/dl (OR = 1.6; 95% CI 1.11–2.43,p < 0.001). No significant differences were noted between the groups regarding other obstetrical outcomes or regarding perinatal outcomes. Conclusion: Second trimester anemia in women carrying twins is associated with a high parity and increases the risk for blood transfusions. However, in our population, maternal anemia in twin gestations does not increase the risk for adverse perinatal outcome. [ABSTRACT FROM PUBLISHER]

Unlabelled: Maternal combined antiretroviral therapy (cART) successfully prevents HIV mother-to-child transmission but also causes hematological toxicity in the HIV-exposed uninfected (HEU) infant. We performed a single-center prospective observational study. Hematological toxicity during the first year of life (at 3 and 6 weeks, and 3, 6, and 12 months) was compared between HEU infants born in two different time periods: P1 (2000-2001) and P2 (2007-2013). Mother-infant pairs in P1 (n = 55) and P2 (n = 48) mainly differed in maternal ethnic origin, HIV route of transmission, and cART regimens. Anemia and neutropenia were both less common in P2 than P1, albeit not significantly. Earlier normalization of red blood cell mean corpuscular volume levels in P2 infants suggests that current cART maternal regimens and shorter neonatal prophylaxis are less toxic. Leukocyte, lymphocyte, and platelet counts remained within normal values during follow-up, without differences between groups.Conclusion: New cART regimens have had very little impact on the hematological toxicity in HEU infants.What Is Known: • Antiretroviral drugs during pregnancy and the neonatal period very effectively prevent mother-to-child transmission of HIV infection. • Hematological toxicity has been widely reported among HIV-exposed uninfected children. What is New: • In HIV-exposed uninfected children, hematological toxicity is still mainly caused by exposure to zidovudine. • New antiretroviral drugs have very little impact on hematological toxicity among HIV-exposed uninfected children. [ABSTRACT FROM AUTHOR]

Purpose: The study was conducted to evaluate clinical and microbiological profile of neonates with septic arthritis and also to assess changing epidemiology in the microbial etiology. Methods: Twenty-nine neonates (1-28 days of life) presenting to the Department of Orthopaedics with acute septic arthritis were included in the study. This was a descriptive study, and the data were collected during the time of hospital admission. History and clinical examination of the neonates were taken, and diagnosis was made based on clinical and laboratory parameters. Emergency arthrotomy was performed to prevent catastrophic sequelae in all the cases given antibiotics as per the culture results. Results: Female children predominated in our study. The children were brought to the OPD with an average of 2.7 days of fever. Thirty-four joints were involved in 29 neonates, out of whom five had more than one joint involvement. Joint effusion or subperiosteal abscess was found in 22 patients by USG. All children had leukocytosis with neutrophilic predominance. Twenty-one of twenty-nine patients had hip-joint involvement followed by knee in seven patients. Gram-negative organisms had grown more commonly, among which Klebsiella pneumonia was grown in nine patients. Conclusion: Prematurity and anemia still appear to be important risk factors for neonatal septic arthritis. As there is changing trend toward gram-negative infections, attention has to be given toward preventing nosocomial and community-acquired infections. This is very important in premature infants who are susceptible for infection when they are kept in resuscitative units in hospitals. [ABSTRACT FROM AUTHOR]

Background and Objective: Severe neonatal anaemia can impair cerebral oxygen supply. Data on long-term outcomes following severe neonatal anaemia are scarce. Methods: Clinical data and neurodevelopmental outcome of 49 (near) term infants with haemoglobin concentration after birth <6.0 mmol/l were retrospectively collected and analysed. In a subgroup of 28 patients, amplitude-integrated EEG was available and in 25 infants cerebral MRI was obtained. Infants were followed up at 14-35 months of age and assessed with the Griffiths Scale of Mental Development or Bayley Scale of Infant Development. Results: Eighteen patients (37%) died during the neonatal period. In 25 patients MRI was performed. A predominant pattern of injury on MRI was seen in the basal ganglia and thalami in 7 patients (28%), whereas some form of white matter injury was present in 16 (64%) and a combination in 3 (12%). Follow-up data were available for 26 patients (84% of survivors). Formal assessment of neurodevelopmental outcome was performed in 20 of 31 (65%) infants who survived (median age: 19 months, range: 14-35). Sixteen infants (80%) had a developmental quotient appropriate for age in the first 2 years after birth. On motor outcome, 1 patient (5%) scored below average (Z-score -1.10). One patient developed cerebral palsy. Conclusion: Early neurodevelopmental outcome in surviving patients with severe neonatal anaemia was within the normal range in the majority of the survivors. MRI showed mild-to-moderate white matter injury in two thirds of the infants. Prospectively collected data with a longer follow-up period are needed. [ABSTRACT FROM AUTHOR]

Background: Fetomaternal hemorrhage (FMH) signifies failure of the placental barrier with whole blood transfer. Fetal anemia following FMH is associated with significant morbidity and mortality. If FMH is identified early, fetal anemia can be treated to minimize adverse outcomes. Risk factors for FMH are not known, limiting efforts to provide targeted screening for FMH. Objective: To identify maternal and/or pregnancy characteristics associated with FMH that are recognizable prior to fetal morbidity. Methods: This is the first published case-control study of FMH. Cases were identified from a prospectively maintained database of all hospital births between 1988 and 2010. Each case was matched to 4 controls by date and time of birth, allowing for assessment of a wide range of clinical and demographic data. Logistic regression modeling was used to assess the association between demographic and clinical characteristics and the diagnosis of FMH. Results: A total of 23 mother-baby pairs impacted by FMH and 92 matched controls were evaluated. Compared to controls, case mothers were more likely to have private insurance and to work outside the home and at night during pregnancy. Cases were more likely to be delivered preterm, but preterm labor was not more common among cases. There was no difference in race/ethnicity of cases compared to controls. Conclusions: Severe FMH is associated with significant morbidity and mortality of the affected neonate. Women with FMH were more likely to work outside the home during pregnancy than women with normal pregnancies. This finding has implications for third-trimester screening of pregnant women who work in strenuous fields. [ABSTRACT FROM AUTHOR]

Background and Objectives: Many observational studies reporting a temporal association between red cell transfusions (RBCTs) and necrotizing enterocolitis (NEC) in preterm infants fail to take into account RBCTs in infants without NEC. The objective of this study was to investigate the association between RBCTs and NEC in an analytical retrospective cohort study with minimization of selection and measurement bias and controlling for clinical covariates.Methods: Inborn preterm infants [23-32 weeks gestational age (GA)] without major congenital anomalies were eligible. Association of RBCT and modified Bell's Stage ≥2A NEC was explored using bivariate analyses and verified using multivariable Cox regression.Results: Of 627 eligible infants, 305 neither received RBCT nor developed NEC and 12 developed NEC prior to RBCT. Of 310 infants with RBCT, 27 developed NEC. Compared to infants without NEC, infants with NEC received significantly lower number of RBCTs before diagnosis of NEC (p = 0.000). On multivariable Cox regression controlling for clinical covariates, dichotomous RBCT exposure was associated with 60% reduced hazard for NEC.Conclusions: RBCT exposure was associated with decreased hazards for NEC in preterm infants in this study; factors previously reported to be associated with NEC remained statistically significant predictors. [ABSTRACT FROM AUTHOR]

Our evidence-based review set out to answer the clinical question 'In a preterm infant (patient) with anaemia of prematurity, do feeding practices (intervention) during blood transfusion reduce the risk of developing transfusion-associated necrotising enterocolitis (outcome)'? We found limited evidence that withholding feeding during red blood cell transfusion in preterm infants may reduce the risk of development of transfusion-associated necrotising enterocolitis. As clinical equipoise seemingly exists, it seems reasonable for individual units to make their own decisions regarding whether to withhold or continue enteral feeds during red blood cell transfusion until further evidence is available. The UK-based Withholding Enteral Feeds Around Transfusion (WHEAT) trial, a nation-wide multi-centre 'opt-out' randomised controlled study, hopefully will definitively answer our clinical question. Further research in other areas of neonatal care, using this innovative study design, is needed and it is exciting to see such a study underway. [ABSTRACT FROM AUTHOR]

Background: The detection of sideroblastic anemia in a newborn may suggest developing Pearson syndrome. The prognosis of these patients is severe and death occurs in the first 3 years of life, so it is important to find new ways of diagnosis. Case Presentation: In the case of our patient the diagnosis was supported only at the age of 5 months, highlighting the difficulties of diagnosis at this age. Conclusions: The diagnosis of Pearson syndrome with neonatal onset is difficult to sustain or even impossible at that age. This diagnosis can be confirmed and supported during disease progression. [ABSTRACT FROM AUTHOR]

Introduction: Anemia is a very common hematological problem particularly among pregnant women, more important it also affects the neonate’s health in various aspects. This study was conducted to assess mothers’ anemia and its effects on the growth and hematological status of neonates. Objective: The objective of this study is to assess the hematological profile and physical growth of neonates born to anemic mothers at birth and 3&1/2 months of age. Methods: In present study 130 neonates born at tertiary care institute during 2009 were divided into two group according to their mothers’ hemoglobin level (less than 10 gm % and 10 or more gm %). Both groups were compared for maturity, birth weight, hematological profile and physical growth at birth and 3&1/2 months of age. Results: During this study period, total 6799 deliveries were conducted in tertiary care institute. Prevalence of anemia among mothers was 64.05%. Out of 130 neonates, 34 (26.15%) were preterm neonates delivered at birth, while 63 (48.46%) were low birth weight neonates. Preterm deliveries and low birth weight neonates were higher in anemic mothers (p

Twin anemia-polycythemia sequence (TAPS) is recognized increasingly antenatally by the demonstration of an anemic twin and a polycythemic cotwin using the middle cerebral artery peak systolic velocity (MCA-PSV). While the MCA-PSV has been shown to correlate well with anemia in singleton fetuses, the evidence to support its use to diagnose fetal polycythemia appears to be less clear-cut. We aimed to evaluate fetal, neonatal and adult literature used to support the use of MCA-PSV for the diagnosis of polycythemia. Comprehensive literature searches were performed for ultrasound evidence of polycythemia in the human fetus, neonate and adult using key search terms. Only manuscripts in the English language with an abstract were considered for the review, performed in June 2014. Fifteen manuscripts were found for the human fetus, including 38 cases of TAPS. Nine of these defined fetal polycythemia as MCA-PSV < 0.8 multiples of the median (MoM), five used < 1.0 MoM and one used 0.8-1.0 MoM. Only two studies, involving a total of 15 cases, proposed a diagnostic level, acknowledging false-positive and -negative cases, though neither reported sensitivities or specificities. Six neonatal studies (96 neonates) demonstrated evidence of decreased cerebral velocities in polycythemia and a consequent increase with hemodilution. In the adult, five studies (57 polycythemic adults) demonstrated increased flow or velocity with hemodilution. Neither neonatal nor adult studies conclusively defined levels for screening for polycythemia. Despite widespread adoption of a cut-off of < 0.8 MoM in the published literature for the polycythemic fetus in TAPS, this is based upon minimal evidence, with unknown sensitivity and specificity. We recommend caution in excluding TAPS based purely upon the absence of a reduced MCA-PSV. [ABSTRACT FROM AUTHOR]

Introduction: Fatal antepartum fetomaternal hemorrhage is a relatively uncommon clinical presentation, though one that appears quickly and without warning. The pathophysiology of this disease is unclear, and the incidence does not appear to be decreasing in line with overall antepartum mortality. This study was undertaken to analyse trends in antepartum fetal death from fetomaternal hemorrhage over a 25-year period in a single maternity hospital in Dublin, Ireland.Material and Methods: A cross-sectional study of 192 132 nonanomalous infants weighing 500 g or more, delivered in a single tertiary-referral university institution between 1987 and 2011. Data was compared using Fisher's exact test, univariate analysis, and Cuzick's test for trend.Results: There was no decrease in the rate of fatal fetomaternal hemorrhage over the past 25 years (p = 0.29), despite a decline in overall antepartum deaths (p = 0.0049). Fetomaternal hemorrhage accounted for 4.1% (34/828) of antepartum stillbirths. A higher proportion of these stillbirths occurred at term gestations (74%; 25/34) compared with other causes (40%; 321/794; p = 0.0003). Female infants were statistically more likely to be involved than males [odds ratio (OR) 2.33, 95% confidence interval (CI) 1.08-5.47, p = 0.02). Multiple gestations were up to six times as likely to be affected as singleton pregnancies (OR 6.52, 95% CI 1.67-18.50, p = 0.005).Conclusions: Over the past 25 years there has been no reduction in rates of fatal fetomaternal hemorrhage. Female infants and multiple gestations remain at higher risk of antepartum death from fatal fetomaternal hemorrhage. [ABSTRACT FROM AUTHOR]

Introduction: nutritional deficiencies are still a common problem during pregnancy causing anemia. Gestational anemia is still considered a public health problem in Brazil, because it is hazardous to both mother and fetus, and is associated with increased risk of maternal-fetal morbidity, as well as the nutritional status of child.Objective: to evaluate the frequency of maternal gestational anemia in newborns and its relation to the nutritional status of the child at birth.Methods: anthropometric data of pregnant women and their newborns were obtained. Blood was collected from pregnant women and the umbilical cord of newborns for analysis of hemoglobin, hematocrit, RDW, iron, ferritin and transferrin saturation index in automatic devices. The results are presented such as the arithmetic mean and the standard deviation. GraphPadinStat Software version 3.0 was used, with a maximum significance level of 5%.Results: the frequency of maternal anemia was 53.7%, and 32.6% in newborns. Half the newborns were anemic children of anemic mothers. 79.3% of the anemic pregnant women had mild anemia and in 20.7% moderate. The average concentration of hemoglobin and hematocrit was lower in anemic pregnant women (9.7 ± 0.9 g/dL and 29.8 ± 3.2%) compared with non-anemic (11.9 ± 0.7 g/ dL and 36.5 ± 2.7%). The maternal iron was positively correlated with ferritin (r = 0.3889, p = 0.01) from umbilical cord blood. The newborns' weight, length and head circumference of anemic mothers were 3 375.9 ± 506,9 g, 51.2 ± 1.7 cm and 34.5 ± 1.5 cm, respectively, while of nonanemic mothers were 3 300.2 ± 458,4 g, 50.3 ± 2.0 cm and 34.2 ± 2.0 cm, respectively. There were no significant correlations between maternal hemoglobin, iron and ferritin with weight, length and head circumference of newborns.Conclusion: the results of this study show that maternal iron deficiency anemia (mild to moderate) can affect the blood profile and iron concentrations in umbilical cord blood of newborns, but without interfering with the child's anthropometric parameters. [ABSTRACT FROM AUTHOR]

Background: Noninvasive prenatal testing can be used to accurately detect chromosomal aneuploidies in circulating fetal DNA; however, the necessity of parental haplotype construction is a primary drawback to noninvasive prenatal diagnosis (NIPD) of monogenic disease. Family-specific haplotype assembly is essential for accurate diagnosis of minuscule amounts of circulating cell-free fetal DNA; however, current haplotyping techniques are too time-consuming and laborious to be carried out within the limited time constraints of prenatal testing, hampering practical application of NIPD in the clinic. Here, we have addressed this pitfall and devised a universal strategy for rapid NIPD of a prevalent mutation in the Ashkenazi Jewish (AJ) population.Methods: Pregnant AJ couples, carrying mutation(s) in GBA, which encodes acid β-glucosidase, were recruited at the SZMC Gaucher Clinic. Targeted next-generation sequencing of GBA-flanking SNPs was performed on peripheral blood samples from each couple, relevant mutation carrier family members, and unrelated individuals who are homozygotes for an AJ founder mutation. Allele-specific haplotypes were constructed based on linkage, and a consensus Gaucher disease-associated founder mutation-flanking haplotype was fine mapped. Together, these haplotypes were used for NIPD. All test results were validated by conventional prenatal or postnatal diagnostic methods.Results: Ten parental alleles in eight unrelated fetuses were diagnosed successfully based on the noninvasive method developed in this study. The consensus mutation-flanking haplotype aided diagnosis for 6 of 9 founder mutation alleles.Conclusions: The founder NIPD method developed and described here is rapid, economical, and readily adaptable for prenatal testing of prevalent autosomal recessive disease-causing mutations in an assortment of worldwide populations.Funding: SZMC, Protalix Biotherapeutics Inc., and Centogene AG. [ABSTRACT FROM AUTHOR]

Background: Blood trafficking from fetus to mother and vice versa is a well-known physiological event that occurs at any stage in pregnancy. If the fetus looses high blood quantities to the maternal blood stream it becomes symptomatic. These symptoms can vary from cardiovascular distress to fetal death. Materials and methods: We give a review of current literature on Fetomaternal hemorrhage (FMH). Conclusion: This article highlights the importance of physician's awareness on detecting this rare but life threatening entity with both severe consequences for mother and neonate. The traditional measurement of FMH and the co-usage of alpha-fetoprotein are debated. To conclude we describe and discuss an illustrative case of FMH. This article gives an applicatory overview of symptoms, diagnostics and treatment of FMH to facilitate physicians to detect this disease precociously. [ABSTRACT FROM AUTHOR]

Several abstracts on blood transfusion and blood related diseases is presented including pre-operative Anemia in Neonates, transfusion of red blood cells in patients with acute leukemia and blood loss in Posterior Spinal Fusion Surgery.

Clinicians observed that the treatment of premature human newborns for anemia with erythropoietin (Epo) also improved their respiratory autonomy. This observation is in line with our previous in vitro studies showing that acute and chronic Epo stimulation enhances fictive breathing of brainstem-spinal cord preparations of postnatal day 3–4 mice during hypoxia. Furthermore, we recently reported that the antagonization of the cerebral Epo (by using the soluble Epo receptor; sEpoR) significantly reduced the basal ventilation and the hypoxic ventilatory response of 10 days old mice. In this study, we used transgenic (Tg21) mice to investigate the effect of the chronic cerebral Epo overexpression on the modulation of the normoxic and hypoxic ventilatory drive during the post-natal development. Ventilation was evaluated by whole body plethysmography at postnatal ages 3 (P3), 7 (P7), 15 (P15) and 21 (P21). In addition Epo quantification was performed by RIA and mRNA EpoR was evaluated by qRT-PCR. Our results showed that compared to control animals the chronic Epo overexpression stimulates the hypoxic (but not the normoxic) ventilation assessed as V ˙ E / V ˙ O 2 at the ages of P3 and P21. More interestingly, we observed that at P7 and P15 the chronic Epo stimulation of ventilation was attenuated by the down regulation of the Epo receptor in brainstem areas. We conclude that Epo, by stimulating ventilation in brainstem areas crucially helps tolerating physiological ( e.g ., high altitude) and/or pathological ( e.g ., respiratory disorders, prematurity, etc .) oxygen deprivation at postnatal ages. [ABSTRACT FROM AUTHOR]

We describe a case of severe neonatal anemia with kernicterus caused by compound heterozygosity for null mutations in KLF1, each inherited from asymptomatic parents. One of the mutations is novel. This is the first described case of a KLF1-null human. The phenotype of severe nonspherocytic hemolytic anemia, jaundice, hepatosplenomegaly, and marked erythroblastosis is more severe than that present in congenital dyserythropoietic anemia type IV as a result of dominant mutations in the second zinc-finger of KLF1. There was a very high level of HbF expression into childhood (>70%), consistent with a key role for KLF1 in human hemoglobin switching. We performed RNA-seq on circulating erythroblasts and found that human KLF1 acts like mouse Klf1 to coordinate expression of many genes required to build a red cell including those encoding globins, cytoskeletal components, AHSP, heme synthesis enzymes, cell-cycle regulators, and blood group antigens. We identify novel KLF1 target genes including KIF23 and KIF11 which are required for proper cytokinesis. We also identify new roles for KLF1 in autophagy, global transcriptional control, and RNA splicing. We suggest loss of KLF1 should be considered in otherwise unexplained cases of severe neonatal NSHA or hydrops fetalis. [ABSTRACT FROM AUTHOR]

Background: Little evidence about the effects of antenatal iron supplementation on infant anaemia is available. The aim was to compare effects on six-month-old infants’ Haemoglobin (Hb) concentration and anaemia of daily iron–folic acid (IFA), twice-weekly IFA with or without other micronutrients (MMN) and usual antenatal care in rural Vietnam. Methods and Findings: Secondary data analysis from: a prospective population-based observational study (OS) which examined effects of antenatal psychosocial factors, anaemia and iron deficiency on infant development and health; and a three-arm cluster randomised trial (CRT) of different antenatal iron supplementation regimens. In the OS 497 women (<20 weeks gestation) from 50 randomly-selected communes participated, and in the CRT 1,258 pregnant women (<16 weeks gestation) in 104 communes were allocated randomly to trial arms. The main outcome was six-month-old infant Hb concentration. Baseline data included women’s socio-demographic characteristics, reproductive health, Hb and serum ferritin. Mean differences in infant Hb and odds ratios of infant anaemia between CRT arms and OS were calculated by multivariable regression models, controlling for baseline differences and clustering, using robust standard errors. Infant anaemia prevalence was 68.6% in the OS, 47.2% daily IFA, 53.5% weekly IFA, and 50.3% MMN conditions. After adjustment, mean infant haemoglobin levels in daily IFA (mean difference = 0.95 g/dL; 95%CI 0.7-11.18); weekly IFA (0.91; 95%CI 0.69-1.12) and MMN (1.04; 95%CI 0.8-1.27) were higher than in the OS. After adjustment there were lower odds ratios of anaemia among infants in the daily IFA (OR = 0.31; 95% CI 0.22-0.43), weekly IFA (0.38; 95%CI 0.26-0.54) and MMN (0.33; 95%CI 0.23-0.48) groups than in the OS. Conclusions: Infant anaemia is a public health problem in Vietnam and other resource-constrained countries. All supplementation regimens could have clinically significant benefits for Hb and reduce anaemia risk among six-month-old infants. Universal provision of free intermittent iron supplements is warranted. [ABSTRACT FROM AUTHOR]

Background: Rd (SC4) is a low-frequency antigen of the Scianna blood group system. Only very few reports on anti-Rd in pregnancy exist. Mild to moderate hemolytic disease of the newborn caused by anti-Rd has been reported. This report may add further information on the clinical significance of anti-Rd for the fetus.Case Report: In a case of severe fetal anemia (hemoglobin concentration, 3.0 g/dL) repeated intrauterine transfusions were required. The strongly positive direct antiglobulin test (DAT) of the fetal red blood cells led to the diagnosis of hemolytic disease. The routine antibody screen was negative, extended testing revealed a maternal anti-Rd with a titer of 256. Both the newborn and her father were confirmed to carry the SC*01.04 allele.Conclusion: Anti-Rd can cause fetal anemia. Low-frequency antigens including Rd are normally not present on screening cells. Antibodies directed against low-frequency antigens will usually not be detected by routine antibody screening in pregnancy. Thus, in cases of fetal anemia the DAT should always be included in the diagnostic workup. [ABSTRACT FROM AUTHOR]

Background: Preterm infants often receive blood transfusions early in life. In this setting, umbilical cord blood (UCB) might be safer than adult blood (A) with respect to infectious and immunologic threats. Objectives: To evaluate, as a first objective, the feasibility of fulfilling transfusion needs of preterm infants with allogeneic UCB red blood cell (RBC) concentrates and, as a secondary objective, to assess the safety of allogeneic cord blood transfusions. Methods: At the Neonatal Intensive Care Unit and the UNICATT Cord Blood Bank of ‘A. Gemelli' Hospital in Rome, a prospective study was carried out over a 1-year period, enrolling newborns with gestational age ≤30 weeks and/or birth weight ≤1,500 g requiring RBC transfusions within the first 28 days of life. At first transfusion, patients were assigned to receive UCB-RBCs or A-RBCs depending on the availability of ABO-Rh(D)-matched UCB-RBC units. The same regimen (UCB-RBC or A-RBC units) was thereafter maintained, unless ABO-Rh(D)-matched UCB-RBC units were not available. Results: Overall, 23 UCB-RBC units were transfused to 9 patients; the requests for UCB-RBC units were met in 45% of patients at the first transfusion and in 78% at the subsequent transfusions. At a median follow-up of 57 days (range 6-219), no acute or delayed transfusion-related adverse events occurred. Hematocrit gain after transfusion and time intervals between transfusions were similar in the UCB-RBC and A-RBC group, as well. Conclusions: Transfusing allogeneic UCB-RBC units in preterm infants appears a feasible and safe approach, although the transfusion needs of our study population were not completely covered. More data are necessary to validate this novel transfusion practice. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

A newborn boy was referred to our hospital because of hemolytic anemia and severe hyperbilirubinemia. Extensive investigations aimed at determining the cause of hemolysis was initiated at the time of admission and 3 months after blood transfusion. Notably, no intrinsic erythrocyte abnormalities could be detected. The only possible cause explaining the progressive anemia and unconjugated hyperbilirubinemia was the finding of pyknocytes, severely distorted erythrocytes, on the blood film at hospital admission. We propose a role for an increased free fraction of plasma unconjugated bilirubin in the formation of pyknocytes through bilirubin membrane toxicity with subsequent anemia and progressive hyperbilirubinemia. Conclusion: Pyknocytosis is a transitory erythrocyte-related condition which can result in severe anemia and hyperbilirubinemia. Recognition of pyknocytes by microscopic analysis of a blood film is essential for a correct diagnosis. Treatment consists of correction of the anemia by top-up blood transfusion and light therapy to prevent toxic bilirubin buildup. High levels of free unconjugated bilirubin could be the underlying cause for the formation of pyknocytes. [ABSTRACT FROM AUTHOR]

5q31.3 microdeletion syndrome is characterized by neonatal hypotonia, encephalopathy with or without epilepsy, and severe developmental delay, and the minimal critical deletion interval harbors three genes. We describe 11 individuals with clinical features of 5q31.3 microdeletion syndrome and de novo mutations in PURA , encoding transcriptional activator protein Pur-α, within the critical region. These data implicate causative PURA mutations responsible for the severe neurological phenotypes observed in this syndrome. [ABSTRACT FROM AUTHOR]

Anemia during the neonatal period is a disorder for diagnosis is dependent on the gestational age of newborn and their hours and days after birth because during this period and subsequent months produced significant change in red cell mass. The etiology of neonatal anemia can be divided into three groups: a) for loos of blood, b) hemolysis, and c) decreased production of red blood cells. We report a case of neonatal anemia blood loss secondary to chronic maternal fetal transfusion, strengthened by the Kleihauer-Betke stain for fetal cells in maternal blood and extension. [ABSTRACT FROM AUTHOR]

Mutations in genes encoding proteins that are involved in mitochondrial heme synthesis, iron-sulfur cluster biogenesis, and mitochondrial protein synthesis have previously been implicated in the pathogenesis of the congenital sideroblastic anemias (CSAs). We recently described a syndromic form of CSA associated with B-cell immunodeficiency, periodic fevers, and developmental delay (SIFD). Here we demonstrate that SIFD is caused by biallelic mutations in TRNT1, the gene encoding the CCA-adding enzyme essential for maturation of both nuclear and mitochondrial transfer RNAs. Using budding yeast lacking the TRNT1 homolog, CCA1, we confirm that the patient-associated TRNT1 mutations result in partial loss of function of TRNT1 and lead to metabolic defects in both the mitochondria and cytosol, which can account for the phenotypic pleiotropy. [ABSTRACT FROM AUTHOR]

Background: Fetomaternal hemorrhage (FMH) is a poorly understood condition in which the placenta allows transmission of fetal whole blood to the mother. FMH can cause fetal anemia resulting in critical illness, death or lifelong disability. Ascertainment of the incidence of FMH is limited by reliance on retrospective studies that are dependent on a diagnosis of FMH being made at the time of patient presentation. Objective: To determine whether the diagnosis of FMH is made more frequently after an educational intervention to increase physician awareness of the condition. Methods: This is a retrospective cohort study of all neonates born at our institution from 1988 through 2010. The medical records of all neonates diagnosed with anemia in the first 24 h of life were reviewed. The incidence of FMH as a documented etiology of anemia was compared between infants born before and after our educational intervention. Results: Of 124,738 births during the study period, 572 neonates with neonatal anemia were identified. A total of 23 cases of FMH demonstrated by positive Kleihauer-Betke testing occurred in our cohort. The incidence of diagnosed FMH prior to our intervention was 22 per 1,000 anemic neonates compared to 182 per 1,000 afterwards (p < 0.001), while the incidence of neonatal anemia remained unchanged (p = 0.377). Conclusions: FMH may be a significant cause of neonatal anemia. Diagnosis of FMH is highly dependent on physician awareness of the condition. Incorrect or absent diagnosis of the etiology of neonatal anemia has significant implications for our understanding of the epidemiology of FMH. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Epidemiological observations report an association between intrauterine growth restriction (IUGR) and cardiovascular diseases. Systemic maternal inflammation is the most common stress during pregnancy, leading to IUGR. We hypothesized that perinatal inflammation and hyperoxygenation induce discernible alterations in cardiomyocyte contractility and calcium signaling, causing early cardiac dysfunction. Pregnant C3H/HeN mice were injected with LPS or saline on embryonic day 16. Newborn mice were placed in 85% O2 or room air (RA) for 14 days. Pups born to LPS-injected dams had reduced birth weight. Echocardiographic measurements revealed that in vivo LV function was compromised in LPS/O2 mice as early as 3 days of life. Isolated cardiomyocytes from LPS/O2 mice at day 14 exhibited decreased sarcomere fractional shortening, along with decreased timeto- 90% peak shortening. Calcium transient amplitude was greatest in LPS/O2 mice. SERCA2a mRNA and protein levels were increased and phospholamban mRNA levels were decreased in LPS/O2 mice. Phosphorylation of phospholamban was increased, along with Sorcin mRNA levels in LPS/O2 mice. Combined exposure to perinatal inflammation and hyperoxia resulted in growth restriction, in vivo and in vitro cardiac dysfunction, coinciding with humans and animal models of cardiac dysfunction. Expression of calcium handling proteins during the neonatal period was similar to that observed during fetal stages of development. Our data suggest that perinatal inflammation and hyperoxia exposure alter fetal development, resulting in early cardiac dysfunction. [ABSTRACT FROM AUTHOR]

Background Twins, compared to singletons, have an increased risk of perinatal mortality and morbidity, due mainly to a higher prevalence of preterm birth and low birthweight. Intrauterine growth restriction (IUGR) is also common and can affect one or both fetuses. In some cases, however, one twin is much smaller than the other (growth discordance). Usually, high birthweight discordance is associated with increased perinatal morbidity. The aim of this study is to describe the epidemiological features of a population of twins at birth, with particular reference to the interpretation and clinical effects of birthweight discordance. Methods We evaluated retrospectively the clinical features of 70 infants born from twin pregnancies and assessed birthweight discordance in 31 pregnancies where both twins were followed at our institution. Discordance was treated both as a continuous and a categorical variable, using a cutoff of 18%. Possible relationships between birthweight discordance and other variables, such as maternal age, gestational age, birthweight percentile, number of SGA newborns in the pair, Hematocrit (Ht) discordance and neonatal anemia, prevalence of malformations, neonatal morbidity and death, were analyzed. Results In our cohort birthweight percentile decreased slightly with increasing gestational age. Birthweight discordance, on the contrary, increased slightly with the increase of gestational age. A high discordance is associated to the presence of one SGA twin, with the other AGA or LGA. In our population, all 6 pregnancies in which discordance exceeded 18% belonged to this category (one SGA twin). Ht discordance at birth is associated to the presence of neonatal anemia in a twin, but it is not significantly related to weight discordance. Finally, in our case history, weight discordance is not associated in any way with the prevalence of malformations, morbidity and mortality Conclusions Birthweight discordance is an important indicator of complications that act asymmetrically on the two fetuses, affecting intrauterine growth in one of them, and usually determining the birth of a SGA infant. Our case history shows a significant statistical association between pair discordance and IUGR in one of the twins, but we could not demonstrate any relationship between discordance and the prevalence of malformations, morbidity and mortality. [ABSTRACT FROM AUTHOR]

Objective: The primary objective was to explore perinatal and neonatal outcomes amongst infants who received intrauterine transfusion (IUT) for the management of hemolytic disease of the fetus and newborn (HDFN). The secondary objective was to evaluate the role of key investigations in the fetus at risk of HDFN and assess the relationship with neonatal outcomes. We hypothesized that middle cerebral artery peak systolic velocity (MCA-PSV) and corresponding multiples of the median (MoM) would be predictive of neonatal course., Methods: This was a retrospective observational study conducted at a tertiary center in the United Kingdom between January 2000 and August 2020. Trust approval was obtained to conduct this service review. Pregnancies requiring IUT for HDFN were identified using the fetal medicine department database. Inclusion criteria were infants who received IUT for HDFN. 67 pregnancies were eligible for inclusion in the study with 156 IUT events. Data were extracted using healthcare records. Statistical analysis was performed using SPSS version 28.0, data were assessed for normality and Spearman's correlation analysis was performed with p values < .05 considered significant., Results: 67 pregnancies were included in the study which led to the live birth of 68 infants (one twin pregnancy). There were no fetal deaths following IUT. There was one neonatal death due to extreme prematurity following spontaneous vaginal delivery at 23 + 4 weeks gestation, occurring three days following IUT. 97% of infants required admission to the neonatal intensive care unit and 88% required phototherapy. 25% of infants required readmission for red blood cell transfusion due to anemia. There was a significant correlation between maternal anti-D antibody levels and length of neonatal admission r  = 0.477, p  = .014. MCA-PSV and MoM measured prior to the last IUT had a significant positive correlation with the duration of phototherapy: r  = 0.527 ( p  < .001) and r  = 0.313 ( p  < .05) respectively. Linear regression analysis demonstrated a significant positive relationship between MCA-PSV and corresponding MoM recorded prior to the last IUT with r 2 = 0.177 ( p  = .003) and r 2 = 0.101 ( p  = .029)., Conclusion: HDFN is an important cause of fetal anemia associated with significant neonatal morbidity. MCA-PSV and MoM may be predictive of neonatal phototherapy requirements. The predictive value of MCA-PSV appears to be dependent on the timing of measurement during the antenatal period and more research is needed. Multicentre collaboration is required to generate a reliable large-scale database to further delineate the value of MCA-PSV and MoM and predict neonatal outcomes in cases of HDFN requiring IUT. This data would assist clinicians in antenatal planning and enable more informed counseling of parents in the antenatal period.

Objective: Placenta previa is one of the causes of neonatal anemia. This condition is mainly explained by antenatal hemorrhage and incision of the anteriorly located placenta during cesarean section. However, the mechanism of neonatal anemia in placenta previa has not been extensively studied or well elucidated. This study investigates whether placenta previa is associated with lower hematocrit levels in newborns with no antenatal hemorrhage and placental incision., Key Findings: This prospective study investigated 47 patients with previa and 43 control patients who gave birth with a cesarean section at 34-38 weeks of gestation. Blood samples were obtained from the fetal end of the umbilical vein. The mean umbilical cord hematocrit value was 49.3% in the control patients and 46.7% in the patients with previa, and the difference was statistically significant ( p  = .029). No significant association was observed between hematocrit value and birth weight, gestational age, newborn gender, placenta position, or preoperative maternal hemoglobin level., Conclusion: The study findings reveal that even if not complicated by antepartum or intrapartum hemorrhage, placenta previa may be associated with lower hematocrit values in newborns. Although in none of the cases, the umbilical cord hematocrit value was not as low as to be defined as anemia, this effect of previa on newborns should be considered because of the importance of iron status.

Fetomaternal hemorrhage (FMH) refers to the entry of fetal blood into the maternal bloodstream before or during delivery. FMH of more than 30 mL occurs with the frequency of about 1/300. Fetal outcomes may be compromised by still births, hydrops fetalis, cardiac complications, and increased rates of postpartum infant death. In most cases, the cause is not identified. Clinical manifestations of FMH depend on the volume of blood lost and the rate that it occurred. We report a case of chronic massive FMH in a newborn of an immigrant mother with a favorable outcome. Medical visits and tests during pregnancy, including ultrasound scans, were not performed. The baby was hemodynamically stable after birth, manifesting only pallor. The complete blood count revealed severe hypochromic anemia (hemoglobin 3,8 g/dl, hematocrit 14,4%) and reticulocytosis (reticulocyte 25,2%). There was no ABO blood type incompatibility and the result of direct Coomb’s test was negative. The Kleihauer-Betke test revealed 5 % of fetal erythrocytes in the maternal bloodstream equivalent to 180 mL. The fact that FMH can occur without prior risk factors, and the diagnosis is often postnatal, underscores the importance of heightened of medical suspicion particularly in infants born to immigrants where there is often the lack of prenatal visits.

Parvovirus B19 is a well-established cause of fetal anemia and nonimmune fetal hydrops in pregnancy. Fetal parvovirus infection can cause severe destruction of erythroid progenitor cells, resulting in fetal anemia, hydrops, and intrauterine death. However, viral myocarditis with subsequent heart failure is another possible mechanism for hydrops formation as viral infection of fetal myocardial cells has been reported in postmortem examinations. We herein report a case of fetal cardiomegaly and massive pericardial effusion secondary to myocarditis as a result of parvovirus B19 infection. The case developed hydrops as consequence of severe anemia and experienced terminal heart failure, which led to the fetus dying an intrauterine death at 22 weeks of gestation. This case demonstrates that there may be an association between myocarditis caused by intrauterine parvovirus B19 infection and a poor outcome. The presence of viral myocarditis may be the determining prognostic factor in that situation. [ABSTRACT FROM AUTHOR]

Introduction. Fetomaternal hemorrhage represents a transfer of fetal blood to the maternal circulation. Although many etiologies have been described, most causes of fetomaternal hemorrhage remain unidentified. The differentiation between acute and chronic fetomaternal hemorrhage may be accomplished antenatally and may influence perinatal management. Case. A 36-year-old gravida 6 para 3 presented at 37 and 5/7 completed gestational weeks with ultrasound findings suggestive of chronic fetal anemia such as right ventricular enlargement, diminished cerebral vascular resistance, and elevated middle cerebral artery end-diastolic velocity. On the other hand, signs of acute fetal decompensation such as deterioration of the fetal heart tracing, diminished biophysical score, decreased cord pH, and increased cord base deficit were noted. Following delivery, the neonate's initial hemoglobin was 4.0 g/dL and the maternal KB ratio was 0.015 indicative of a significant fetomaternal hemorrhage. Discussion. One should consider FMH as part of the differential diagnosis for fetal or immediate neonatal anemia. We describe a unique case of FMH that demonstrated both acute and chronic clinical features. It is our hope that this case will assist practitioners in differentiating acute FMH that may require emergent delivery from chronic FMH which may be able to be expectantly managed. [ABSTRACT FROM AUTHOR]

Background Packed Red Blood Cell (PRBC) transfusions are often administered to patients in the neonatal intensive care unit. Aims The purpose of this study was to determine whether current blood transfusion clinical practice guidelines are as useful as clinical judgement in identifying patients in need of a PRBC transfusion. Methods The study is a post-transfusion survey on premature newborns less than 32 weeks old that received a PRBC transfusion. These patients were divided into three groups, based on the criteria used for transfusion: (a) clinical practice guidelines; (b) clinical judgement/symptoms of need for PRBC transfusion; or (c) both. These three groups were further subdivided based on clinical response to transfusion. Demographic data and clinical variables were compared among the groups. 35 preterm infants who received transfusions were identified. Thirteen patients (37%) were transfused based on guidelines, 4 (11%) based on clinical judgement, and 18 (52%) based on both. Results Neonates transfused based on guidelines alone were more likely to have received the transfusion in the first week of life, had a lower pre-transfusion hematocrit, were less symptomatic and had a higher likelihood of requiring mechanical ventilation. Neonates transfused based on clinical judgement were more likely to be on noninvasive ventilatory support and were more symptomatic. Neonates who improved after a transfusion had a lower pretransfusion hematocrit (p=0.03), were more symptomatic (p=0.01) and were more likely to be on non-invasive ventilatory support (p=0.02) when compared to the group without clinical improvement. The group without improvement had an increase in oxygen requirement (+3.8±2.4) after the transfusion (p=0.0004). Conclusion Guidelines on when to transfuse stable growing premature newborns with PRBC should be reevaluated to include more clinical judgement and perhaps be more restrictive for critically ill neonates. [ABSTRACT FROM AUTHOR]

Anemia in the newborn can result from bleeding, hemolysis and deficiency in red blood cell production. Anemia at birth is mainly due to bleeding and immune hemolysis. Anemia blood loss can occur through obstetrical accidents, occult blood loss and internal bleeding. A retrospective study conducted in 236 anemic infants in the early neonatal period, hospitalized for neonatal anemia between January 1, 2010 and December 31, 2012 in "Bega" Clinic of Neonatology in Timisoara, identified an accumulation of significant risk factors influencing prognosis and treatment. Among infants with neonatal anemia, a total of 147 were premature. The most common cause of hemolytic anemia was the blood group incompatibility between mother and fetus, the prevalence being 9.55%. [ABSTRACT FROM AUTHOR]

BACKGROUND: Very preterm infants commonly develop anemia requiring multiple red blood cell transfusions (RBCTx). This is in part attributable to heavy laboratory phlebotomy loss. Quantification of the extent to which laboratory blood loss contributes to anemia sufficient to prompt RBCTx has not been examined. STUDY DESIGN AND METHODS: Twenty-six preterm infants weighing less than 1500 g at birth requiring ventilator support who received one or more RBCTx were intensively studied during the first month of life. Hemoglobin (Hb) loss via laboratory blood loss and RBC senescence and Hb gain from RBCTx were precisely accounted for in a Hb mass balance mathematical model developed to assess the impact of phlebotomy on RBCTx when restrictive RBCTx criteria were applied. RESULTS: Study subjects had a birth weight of 880 ± 240 g (mean ± SD) and a Hb level of 14.4 ± 2.4 g/dL at birth and received 3.81 ± 2.15 RBCTx during the study period. Modeling indicated that even with the total elimination of laboratory phlebotomy loss, a reduction of 41% to 48% in RBCTx was achievable. CONCLUSION: The present modeling results indicate that while phlebotomy reduction can significantly decrease the number of RBCTx administered to preterm infants, total elimination of all RBCTx will likely require other approaches, for example, stimulation of erythropoiesis with erythropoiesis-stimulating agents. [ABSTRACT FROM AUTHOR]

Objective:The incidence of fetomaternal hemorrhage that is severe enough to cause neonatal anemia is not known. Owing to its relative rarity, much of the literature describing this condition is in the form of case reports and small case series. We performed a large, muiticentered, sequential, case series to determine the incidence, antecedents and outcomes.Study Design:From the multicentered databases of Intermountain Healthcare, we obtained records of all neonates with hematocrit (Hct) <30% or hemoglobin (Hgb) <10 g dl−1 on the day of birth, who had Kleihauer-Betke staining or flow cytometric evidence of fetomaternal hemorrhage.Result:Among 219 853 live births, 24 had anemia with evidence of fetomaternal hemorrhage (incidence estimate, 1 per 9160 live births). The initial Hgb ranged from 1.4 to 10.2 g dl−1 (Hct 29.8%). The initial Hgb was <7 g dl−1 in 18 (67%), <5 g dl−1 in 12 (50%) and was <3 g dl−1 in 7 (29%). All 7 mothers in whom neonatal Hgb was <3 g dl−1 had reported absent fetal movement, as did 13 of 18 mothers when the initial Hgb was <7 g dl−1. Outcomes were poorer in those with the lowest initial Hgb; in the two lowest, one died on day 1, and the other developed a grade 4 intraventricular hemorrhage (IVH). The adverse outcomes of death, IVH, periventricular leukomalacia, bronchopulmonary dysplasia or hypoxic-ischemic encephalopathy were common; occurring in 71% (17 of the 24), including all with an initial Hgb <5 g dl−1 and all born at 35 weeks of gestation.Conclusion:Fetomaternal hemorrhage is a rare but sometimes devastating condition. Those with fetomaternal hemorrhage and an initial Hgb of <5 g dl−1 are expected to need resuscitation at birth, to receive emergent transfusion support and to be at risk for death and major morbidities. Antenatal suspicion of this diagnosis should occur when absent fetal movement is reported. Improvements in rapid diagnosis are needed to prepare first responders and transfusion services. [ABSTRACT FROM AUTHOR]

Objective The epsilon gamma delta beta (εγδβ)-thalassemias are rare sporadic disorders caused by deletion of the β-globin gene cluster. The main clinical feature is marked prenatal and neonatal anemia that resolves spontaneously within a few months. Reports originating mainly from Europe have so far identified 30 such deletions The aim of the present work was to describe a novel 1.78-Mb deletion, the longest ever reported, and to detail the clinical features in 12 members of an extended Bedouin family. Methods The deletion was identified by globin gene multiplex ligation-dependent probe amplification ( MLPA) of the β-globin cluster and further characterized by comparative genomic hybridization. Past and present clinical and laboratory data of ten symptomatic and two asymptomatic patients were collected. Results A 1.78-Mb εγδβ-deletion, the largest ever described, was identified in all patients. Although other genes were included in the deletion, no other symptoms were observed. Of the ten symptomatic fetuses and neonates, three died of the disease. The remainder required packed cell transfusions during the first months of life. Pregnancy complications included intrauterine growth restriction and oligohydramnios, as well as additional neonatal complications including prematurity and persistent pulmonary hypertension of the neonate. Conclusions We suggest that εγδβ-thalassemia be added to the list of hemoglobinopathies that can cause neonatal anemia and that MLPA of the β-globin cluster be used to confirm its diagnosis. Careful surveillance during pregnancy is important to reduce neonatal mortality and morbidity, especially given the dramatic improvement that occurs later. [ABSTRACT FROM AUTHOR]

Anemia in neonatal period is rare, with the common causes being Rh and ABO blood group incompatibility, hemorrhagic disease of newborn, congenital hemolytic anemia, hemoglobinopathies, and TORCH (toxoplasmosis, rubella, cytomegalovirus, herpes virus) infections. Congenital leukemia and infantile osteopetrosis (OP) are among the rare causes of neonatal anemia. A review of the literature shows approximately 200 reported cases of congenital leukemia. Articles describing the imaging features of congenital leukemia are still rarer. Infantile OP, another rare disorder with a reported incidence of 1 in 250,000 has characteristic imaging features, which are diagnostic of the disease. We report a case each, of two rare diseases: Congenital leukemia and infantile osteopetrosis. Additionally, our report highlights the radiological and imaging features of congenital leukemia and infantile OP and their crucial role in arriving at an early diagnosis. [ABSTRACT FROM AUTHOR]

The development of anemia after birth in very premature, critically ill newborn infants is a universal well-described phenomenon. Although preventing anemia in this population, along with efforts to establish optimal red blood cell (RBC) transfusion and pharmacologic therapy continue to be actively investigated, the present review focuses exclusively on nonpharmacological approaches to the prevention and treatment of neonatal anemia. We begin with an overview of topics relevant to nonpharmacological techniques. These topics include neonatal and fetoplacental hemoglobin levels and blood volumes, clinical and laboratory practices applied in critically ill neonates, and current RBC transfusion practice guidelines. This is followed by a discussion of the most effective and promising nonpharmacological blood conservation strategies and techniques. Fortunately, many of these techniques are feasible in most neonatal intensive care units. When applied together, these techniques are more effective than existing pharmacotherapies in significantly decreasing neonatal RBC transfusions. They include increasing hemoglobin endowment and circulating blood volume at birth; removing less blood for laboratory testing; and optimizing nutrition. [Copyright &y& Elsevier]

Abstract: Treatment of severe anemia with intrauterine red cell transfusions in fetuses with red cell alloimmunization has led to a dramatic increase in perinatal survival. Due to this increased survival focus is nowadays shifting towards improving postnatal treatment options. Phototherapy, exchange transfusions and intravenous immunoglobulin are used to treat hyperbilirubinemia and prevent kernicterus. Postnatal treatment of anemia consists of top-up transfusions, supplements to support erythropoiesis such as folic acid and iron, and occasionally erythropoietin treatment. In addition to anemia, other hematological complications such as thrombocytopenia, coagulation disturbances, leucopenia and iron overload have been reported. This review focuses on the hematological morbidity in neonates with red cell alloimmunization and summarizes the current evidence on management options. [Copyright &y& Elsevier]

Objective: To assess the accuracy of the combined use of the cardiofemoral index (CFI) and the middle cerebral artery peak systolic velocity (MCA-PSV), converted to multiples of the median (MoM), as noninvasive means to detect severe fetal anemia. Method: We measured CFI and MCA-PSV MoM in 37 fetuses just before their first (n=37), second (n=22), and third (n=14) cordocenteses and transfusions. Then, using 2 different criteria for severe fetal anemia detection (Hb deficit ≥7 g/dL and hemoglobin level ≤0.55 of MoM), we assessed their hemoglobin status during cordocentesis and the accuracy of CFI and MCA-PVS was determined. Results: At the first cordocentesis the mean hemoglobin level was 8.5±3.6 g/dL and 15 fetuses (40.5%) had hydrops. In a total of 81 fetal evaluations, 58 (71.6%) of the CFIs and 34 (42.0%) of the MCA-PSV MoM measurements were abnormal. The result of one of these tests was abnormal in 65 evaluations (80.3%) and the results of both tests were abnormal in 27 evaluations (33.3%). All fetuses diagnosed as being severely anemic by at least one of the hemoglobin criteria during cordocentesis had an abnormal result by at least one of the noninvasive tests. Before the second and third transfusions, the combined use of the CFI and MCA-PSV MoM predicted severe fetal anemia with 100% sensitivity. When the CFI and MCA-PSV MoM measurements were normal, the negative likelihood ratio was zero. Conclusion: When associated, CFI and MCA-PSV MoM were accurate predictors of severe fetal anemia. [ABSTRACT FROM AUTHOR]