Elevated Hemoglobin Levels Research Articles

Relation of Preprocedural Hemoglobin Level to Outcomes After Percutaneous Coronary Intervention

Adverse effects have been reported in patients with preprocedural anemia after percutaneous coronary intervention (PCI), but data regarding the relation between elevated hemoglobin (Hb) level and post-PCI prognosis remain limited. This study assessed the impact of elevated Hb on major adverse cardiac and cerebrovascular event (MACCE) at 12 months, a composite of all-cause mortality, nonfatal myocardial infarction, and ischemic stroke after PCI. We pooled patient-level data from four Korean multicenter drug-eluting stent registries from 2010 to 2016. In total, 5,107 patients were divided into 5 categories according to the baseline Hb level (<10, 10 to 12.9, 13 to 14.9, 15 to 16.9 and ≥17 g/dl). Patients with higher Hb levels were significantly younger, predominantly male, current smokers with higher body mass index, and more frequent dyslipidemia. Hypertension, diabetes, chronic kidney disease, and cerebrovascular accident were more prevalent in lower Hb groups. Categorically, a U-shaped curvilinear relation was observed between baseline Hb and clinical outcomes showing significantly higher MACCE rate in <10g/dl (hazard ratio [HR], 4.62 [2.81 to 7.68]) and ≥17 g/dl (HR, 4.06 [1.57 to 10.5]) groups compared with the reference group (13 to 14.9 g/dl), especially in men. In nonanemic patients (Hb ≥13 g/dl), adjusted HRs of MACCE, mortality, and stroke were significantly higher in ≥17 g/dl group than in the reference group. Furthermore, ≥17 g/dl was an independent predictor for MACCE and all-cause mortality after PCI. In conclusion, not only low Hb but also elevated Hb of ≥17 g/dl was significantly associated with higher MACCE rates and all-cause mortality after PCI. An appropriate treatment strategy for patients with high Hb level should be identified through future studies.

1621-P: Nonlinear Relationship between Elevated Hemoglobin Level and the Risk of Glucose Intolerance in Tibet Residents

Introduction: The aim of this study is to explore the relationship between hemoglobin level and the risk of glucose intolerance in Tibet residents. Method: Eligible participants were aged from 20 to 80 years old, living in Tibet plateau for more than 3 months. The exclusion criteria were diagnosed diabetes, anemia or other hematological diseases, having been received transfusion in the past month. OGTT test was performed. Logistic regression and generalize additive models was used to explore the relationship between hemoglobin level and the risk of impaired glucose intolerance. Result: A total of 818 people was enrolled in our research, including 586 (71.6%) male and 232 (28.4%) female. Participants were divided into normal group (Hb&amp;lt;210, n=747), and polycythemia group (Hb&amp;gt;210, n=71). There were 229 (30.66%) and 33 (46.48%) people were glucose intolerance in two groups separately (P&amp;lt;0.001). The odds ratio for male and female patients were 1.821 (95% CI 1.026-3.232) and 0.603 (95% CI 0.940-2.824), respectively. After stratified by sex, we find a “J” shaped relationship in male participants (Figure), and the turning point was 196g/L. Conclusion: Elevated hemoglobin level is associated with increasing risk of glucose intolerance in male participants. When hemoglobin level is over 198g/L, the risk raised obviously as the hemoglobin level raising in male Tibet residents. Disclosure W. Suyuan: None. W. Yunhong: None. Z. Chenghui: None. L. Mingxia: None.

A time-to-event analysis describing virologic response in patients with chronic hepatitis C infection

The objective of this project was to describe longitudinal change in chronic hepatitis C virologic reponse using time-to-event (TTE) analysis and to identify patient characteristics that determine the dynamics of this change. We compiled demographic, clinical, and genetic data from 715 chronic hepatitis C virus (HCV) patients treated with pegylated interferon (PEG-IFN) alfa-2a and ribavirin. TTE modelling described the time between antiviral treatment initiation and the first observation of undetectable HCV RNA. A lognormal TTE model was selected to describe time to first undetectable HCV RNA. The identified predictors of prolonged time to achieve undetectable HCV RNA include HCV genotype 1, low pre-treatment ALT level, older age, or with elevated baseline haemoglobin level. In conclusion, a cohort of patients with low probability of achieving SVR can be identified. This project identifies patients with a low risk of responding to PEG-IFN alfa-2a and ribavirin combination.

A Case Report on Post-Transplant Erythrocytosis

Post-transplant erythrocytosis is defined as persistently elevated haemoglobin and haematocrit levels that occur following renal transplantation and persist for more than six months in the absence of thrombocytosis, leukocytosis or other potential causes of erythrocytosis. Here, we report the case history of a 35-year-old male, who underwent live related kidney transplantation ten months ago, presented with high haemoglobin level and high haematocrit. It is an uncommon complication of kidney transplant recipient,whichprompted us to report the case&#x0D; Birdem Med J 2019; 9(2): 170-173

Domain-Focused CRISPR-Cas9 Screen Identifies the E3 Ubiquitin Ligase Substrate Adaptor Protein SPOP as a Novel Repressor of Fetal Hemoglobin

Elevated levels of fetal hemoglobin (HbF) are beneficial to patients with sickle cell disease (SCD) and b-thalassemia. The identification of pharmacologically controllable pathways that regulate HbF levels remains an important goal in the treatment of these disorders. BCL11A and LRF (ZBTB7A) are the two major transcriptional repressors of g-globin (fetal beta-type globin). However, since transcription factors are challenging to inhibit with small molecules, we aimed to identify novel HbF regulators that might be amenable to control by pharmacologic means.Using an improved protein-domain based CRISPR-Cas9 screen recently developed in our laboratory (Grevet et al., Science 361:6399, 285-290), we screened for new HbF regulators using a gRNAs library targeting chromatin associated proteins including BTB domain containing factors. We identified SPOP, a substrate adaptor of the CUL3 ubiquitin ligase complex, as a novel inhibitor of g-globin expression. Depletion of SPOP strongly elevated the mRNA and protein levels of g-globin in both HUDEP-2 and primary human CD34+ derived erythroid cells. The effects of SPOP loss were remarkably selective as evidenced by RNA-seq and whole cell mass spectrometry. Importantly, SPOP depletion did not appear to impair cell growth or differentiation. Mechanistically, we demonstrated that the repressive role of SPOP on g-globin levels is dependent on the CUL3 ubiquitin ligase complex. Additionally, forced expression of a dominant negative form of SPOP (Y87N) unable to bind to its substrates strongly induced HbF production. We also found that SPOP depletion did not measurably alter LRF levels and modestly reduced BCL11A levels. Overexpression of BCL11A did not restore g-globin silencing in SPOP depleted cells, indicating that SPOP works largely independently of these two factors. Finally, we tested whether the effects of SPOP depletion can be augmented through the use of experimental pharmacologic HbF inducers, such as pomalidomide. In proof of principle experiments we found that SPOP depletion and pomalidomide treatment induced HbF production to levels higher than either treatment alone.In sum, our work uncovered SPOP as a new regulator of HbF and suggests targeting SPOP alone or in combination with other HbF inducers may provide a novel therapeutic strategy for the treatment of hemoglobinopathies such as sickle cell disease and b-thalassemia. DisclosuresNo relevant conflicts of interest to declare.

Elevated fetal haemoglobin levels are associated with decreased incidence of retinopathy in adults with sickle cell disease

Among the many vascular complications of sickle cell disease (SCD), retinopathy is the most prevalent and represents a leading cause of blindness. Hydroxycarbamide therapy ameliorates many symptoms of SCD, and high fetal haemoglobin (HbF) levels have been shown to protect against the development of retinopathy in children with HbSS. Its effect on adults with SCD, who are at a much higher risk of developing retinopathy, has not been studied. We aimed to investigate the effect of hydroxycarbamide use and HbF level on sickle cell retinopathy development in adults. We performed a retrospective cross-sectional study and collected demographics, comorbidities, and ocular and haematological data from 300 adult sickle cell subjects examined at the Henkind Eye Institute at Montefiore Medical Center during a 5-year period, from October 2012 to November 2017. The cohort was comprised mainly of Black and Hispanic subjects with all SCD genotypes, aged 18-71years. Results show that in HbSS patients treated with hydroxycarbamide, those with retinopathy had significantly lower HbF levels compared to patients without retinopathy (P=0·018). Our study identified the optimal HbF cut-off point for retinopathy protection to be 14·87%. Thus, a HbF level of 15% appears to be the threshold above which the odds for developing retinopathy in SS patients are reduced by 50%.

Polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin

BackgroundHemangioblastomas are associated with elevated hemoglobin (Hb) levels (polyglobulia), which is associated with a higher risk for cerebral stroke, cardiac infarction and pulmonary embolism. The pathomechanism of polyglobulia remains unclear and different theories have been postulated. Among those are elevated serum erythropoietin (EPO) levels caused by secretion of the tumor or associated tumor cyst.MethodsTo elucidate the pathomechanism, we systematically investigated the relation between polyglobulia, serum EPO level, size of the solid tumor and associated cyst in hemangioblastomas. We prospectively evaluated hemoglobin and EPO levels in a series of 33 consecutive patients operated on hemangioblastomas in our center. We measured the size of the solid tumor and associated cyst in magnetic resonance imaging. Statistical evaluations were performed using the Fisher’s exact test and student’s t-test.ResultsAs a result five patients had elevated hemoglobin levels. Only one of these had an elevated serum EPO level. Of 26 patients with normal hemoglobin levels, 4 patients had elevated EPO levels.Patients with low or normal hemoglobin levels (84%) had an average tumor size of 0.8 cm3, which differed significantly from patients with elevated hemoglobin levels (16%), who had an average solid tumor size of 8.0 cm3 (p < 0.05). We did not observe a significant correlation between EPO levels or polyglobulia and associated cysts.ConclusionsWe therefore conclude that in contrast to previous case reports and interpretations, our data show no correlation between polyglobulia and EPO levels or associated cysts in patients with hemangioblastomas. In fact, it is the size of the solid tumor that correlates with polyglobulia.The study was retrospectively registered in the German Clinical Trial Registry on 10 July 2014; Trial registration: DRKS00006310.

Effect of Endoplasmic Reticular Stress on Free Hemoglobin Metabolism and Liver Injury.

Elevated soluble (s) CD163 and free hemoglobin (Hb) levels predict fatty liver progression; however, the molecular mechanisms underlying Hb metabolism and liver injury remain undefined. We investigated the effects of endoplasmic reticular (ER) stress on red blood cell (RBC) rheology and free Hb recycling pathways. ER stress was induced in Sprague-Dawley rats by an intraperitoneal injection of tunicamycin (TM) (50, 100, and 200 μg/100 g body weight (BW)) or an intravenous injection of Hb (5 mg/100 g BW). A TM injection increased sCD163 levels, attenuated free Hb uptake, and maintained RBC aggregability. An Hb injection increased serum LVV-hemorphin-7 and total bilirubin levels, but this effect was suppressed by TM. A Western blot analysis showed that ER stress suppressed Hb degradation in the liver through downregulation of globin degradation proteins cathepsin D and glyoxalase-1, as well as heme degradation protein heme oxyganase-1 and keap-1 expression. An ER stress activator also increased the translocation of nuclear factor (NF)-κB (p65) and nuclear factor-erythroid 2-related factor 2 (Nrf2) to nuclei. In conclusion, ER stress triggers ineffective Hb metabolism via altering globin and heme iron degradation pathways. Inability to recycle and metabolize free Hb may underlie the association between iron dysfunction and liver injury.

Serum of sickle cell disease patients contains fetal hemoglobin silencing factors secreted from leukocytes.

BackgroundThe mechanisms that regulate fetal hemoglobin (HbF) expression in sickle cell disease (SCD) remain elusive. We previously showed that steady-state SCD patients with high HbF levels due to a γ-globin gene mutation demonstrate strong inverse correlations between HbF levels and leukocyte counts, suggesting that leukocytes play a role in regulating HbF in SCD.Materials and methodsTo further investigate the role of leukocytes in HbF expression in SCD, we examined the presence of HbF silencing factors in the serum of 82 SCD patients who received hydroxyurea (HU) therapy.ResultsHU-mediated HbF induction was associated with elevated total hemoglobin levels and improved red blood cell parameters, but there was no correlation with reticulocyte or platelet counts. Importantly, we again found that HU-induced HbF levels correlated with reductions in both neutrophils and lymphocytes/monocytes, indicating that these cell lineages may have a role in regulating HU-mediated HbF expression. Our in vitro studies using CD34+-derived primary erythroblasts found that patient serum preparations include HbF silencing factors that are distinct from granulocyte-macrophage colony-stimulating factor, and the activity of such factors decreases upon HU therapy.ConclusionTogether, these results demonstrate the importance of leukocyte numbers in the regulation of HbF levels for SCD patients both in steady state and under HU therapy, and that leukocytes secrete HbF silencing factors that negatively affect HbF expression in erythroid-lineage cells in SCD.

Genetic basis of congenital erythrocytosis.

Congenital Erythrocytosis (CE) represents a rare and heterogeneous clinical entity. It is caused by deregulated erythropoiesis where red blood cell overproduction results in elevated hemoglobin and hematocrit levels. CE may either be primary or secondary to elevated erythropoietin concentrations. The only known form of primary CE is caused by mutations in the EPOR gene. Secondary CE can be a consequence of tissue hypoxia, being caused by congenital defects such as hemoglobin variants with increased oxygen affinity, due to mutations in the α- or β-globin genes (HBB, HBA2, HBA1), or due to mutations in the BPGM gene. Secondary CE can also result from defects in the components of the oxygen-sensing pathway (PHD2, HIF2α and VHL). The family history and the quantification of serum EPO are mandatory to define the best diagnostic strategy regarding molecular studies. Based upon the serum EPO level, P50 and familial data, it is possible to establish a diagnostic algorithm. Despite recent important discoveries in the molecular pathogenesis of CE, in about 70% of the patients the genetic causes remain to be identified. Clinical data on patients suffering from CE are sparse. This fact is conditional upon the effective way to predict the disease evolution, the establishment of the best management and the genetic counselling. The number and phenotypic variability of patients that remain without an identified etiology suggest that other genes have to be implicated. Studies by next generation sequencing methodologies are already being performed and it is expected the identification of other genes involved in the pathophysiology of the CE.

30-Year-Old Man With Outside-of-Hospital Cardiac Arrest

30-Year-Old Man With Outside-of-Hospital Cardiac Arrest

Congenital methemoglobinemia misdiagnosed as polycythemia vera: Case report and review of literature.

Methemoglobinemia is a rare overlooked differential diagnosis in patients presented with cyanosis and dyspnea unrelated to cardiopulmonary causes. Our patient is 29 year old Indian non-smoker male, his story started 6 months prior to presentation to our center when he had generalized fatigue and discoloration of hands. He presented with persistent polycythemia with elevated hemoglobin level. The patient was misdiagnosed in another center as polycythemia and treated with Imatinib. The diagnosis of PV was revisited and ruled out in view of negative JAK2, normal erythropoietin level and absence of features of panmyelosis. Clinical cyanosis and lowoxygen saturation in the presence of normal arterial oxygen tension was highly suggestive of methemoglobinemia. Arterial blood gas revealed a methemoglobin level of 38% (normal: 0-1.5%). Cytochrome B5 reductase (Methemoglobin reductase B) was deficient at level of <2.6 U/g Hb) (normal: 6.6-13.3), consistent with methemoglobin reductase (cytochrome b5) deficiency and hence the diagnosis of congenital methemoglobinemia was established. The role of Imatinib in provoking methemoglobinemia is questionable and association between Imatinib and methemoglobinemia never described before. In our case, there were no other offending drugs in aggravating the patients’ symptoms and cyanosis. The patient started on Vitamin C 500 mg once daily for which he responded well with less cyanosis and significant reduction of methemoglobin level. Congenital methemoglobinemia is a rare underreported hemoglobin disease and often clinically missed. Upon extensive review of English literature for cases of congenital methemoglobinemia due to deficiency of cytochrome b5 reductase, we found 23 cases diagnosed as type I (including the case reported here). 17 cases (~74%) of type I and 6 cases (27%) of type II. There is male predominance 73% versus 26% in females. Almost half of reported cases 12 cases (52%) are Indian, 2 Japanese, 3 English, 2 Arabic, one case Spanish and one case Italian. For type I, the median calculated age is 31 years with cyanosis and shortness of breath being the most common sign and symptoms. For type II: Six cases were reported in English literature, all in pediatric age group with median calculated age at presentation is 6 years with neurologic manifestations and mental retardation are the most common type II associated symptoms. Due to lack of systematic epidemiological studies, congenital methemoglobinemia is under diagnosed as it is under investigated and usually overlooked especially when presenting in adulthood and in absence of obvious acquired agents.

Are elevated serum haemolysis markers a harbinger of adverse events in HeartMate II patients?

Haemolysis during left ventricular assist device support is associated with thrombosis. In this retrospective study, we analysed whether low-level haemolysis (LLH) as defined by simultaneously elevated lactate dehydrogenase (LDH) and free haemoglobin (fHb) levels had an impact on thromboembolic and bleeding events and on von Willebrand factor levels in HeartMate II patients. After exclusion of patients with LDH >700 U/l and fHb >40 mg/dl at hospital discharge, 79 HeartMate II patients were included. LDH and fHb levels were measured at discharge and in 3 months interval. von Willebrand factor activity and antigen activity were measured 3 months postoperatively. Outcomes regarding ischaemic stroke (IS), pump thrombosis (PT) and gastrointestinal bleeding were recorded. Patients with LLH (400 < LDH ≤ 700 U/l and 30 < fHb ≤ 40 mg/dl) at discharge (pre-Hemolyzers) were compared with the rest of the cohort (non-Hemolyzers). Competing risk analysis and Cox regression were applied for the comparison between groups. In all, 20% of the patients were identified as pre-Hemolyzers. Of these, 5 patients had PT and 3 patients had IS compared with 2 PT and 2 IS in the non-Hemolyzers group (P = 0.003 and P = 0.053, respectively). Fifty percent of the pre-Hemolyzers suffered gastrointestinal bleeding compared with 42% of the non-Hemolyzers (P = 0.399). The cumulative risk of thromboembolic events (IS or PT) in the pre-Hemolyzers group was significantly higher compared with the non-Hemolyzers group (hazard ratio 11.8, 95% confidence interval 3.7-37.7; P = 0.005). LLH did not have an impact on von Willebrand factor and the incidence of gastrointestinal bleeding. LLH as assessed by elevated fHb and LDH values at discharge during HeartMate II support is associated with thromboembolic events.

PENYULUHAN GIZI DAN PEMBERIAN TABLET BESI TERHADAP PENGETAHUAN DAN KADAR HEMOGLOBIN SISWI SEKOLAH MENENGAH ATAS NEGERI DI MAMUJU

The prevalence of anemia in adolescent girls junior and senior high school in Mamuju is 27%. This study to determine the effect of nutritional counseling and suplementation iron tablets to knowledge and hemoglobin ( Hb ) status of senior high school student in West Sulawesi. This type of research is a quasi-experimental design using non- randomized control group pretest-posttest. Samples of the intervention group were 30 students and a control group of 30 students. Student knowledge was measured using questionnaires and hemoglobin concentration using hemoglobinometer. Analysis using Wilcoxon Test, Paired Sample T-Test and Independent T Test. The results showed the results of this study showed no difference in the increase in knowledge after counseling, while the suplementation of iron tablets contained elevated levels of hemoglobin

Deep Ocean Mineral Supplementation Enhances the Cerebral Hemodynamic Response during Exercise and Decreases Inflammation Postexercise in Men at Two Age Levels.

Background: Previous studies have consistently shown that oral supplementation of deep ocean minerals (DOM) improves vascular function in animals and enhances muscle power output in exercising humans.Purpose: To examine the effects of DOM supplementation on the cerebral hemodynamic response during physical exertion in young and middle-aged men.Design: Double-blind placebo-controlled crossover studies were conducted in young (N = 12, aged 21.2 ± 0.4 years) and middle-aged men (N = 9, aged 46.8 ± 1.4 years). The counter-balanced trials of DOM and Placebo were separated by a 2-week washout period. DOM and Placebo were orally supplemented in drinks before, during, and after cycling exercise. DOM comprises desalinated minerals and trace elements from seawater collected ~618 m below the earth's surface.Methods: Cerebral hemodynamic response (tissue hemoglobin) was measured during cycling at 75% VO2max using near infrared spectroscopy (NIRS).Results: Cycling time to exhaustion at 75% VO2max and the associated plasma lactate response were similar between the Placebo and DOM trials for both age groups. In contrast, DOM significantly elevated cerebral hemoglobin levels in young men and, to a greater extent, in middle-aged men compared with Placebo. An increased neutrophil to lymphocyte ratio (NLR) was observed in middle-aged men, 2 h after exhaustive cycling, but was attenuated by DOM.Conclusion: Our data suggest that minerals and trace elements from deep oceans possess great promise in developing supplements to increase the cerebral hemodynamic response against a physical challenge and during post-exercise recovery for middle-aged men.

Whole transcriptome analysis of human erythropoietic cells during ontogenesis suggests a role of VEGFA gene as modulator of fetal hemoglobin and pharmacogenomic biomarker of treatment response to hydroxyurea in \u03b2-type hemoglobinopathy patients

BackgroundHuman erythropoiesis is characterized by distinct gene expression profiles at various developmental stages. Previous studies suggest that fetal-to-adult hemoglobin switch is regulated by a complex mechanism, in which many key players still remain unknown. Here, we report our findings from whole transcriptome analysis of erythroid cells, isolated from erythroid tissues at various developmental stages in an effort to identify distinct molecular signatures of each erythroid tissue.ResultsFrom our in-depth data analysis, pathway analysis, and text mining, we opted to focus on the VEGFA gene, given its gene expression characteristics. Selected VEGFA genomic variants, identified through linkage disequilibrium analysis, were explored further for their association with elevated fetal hemoglobin levels in β-type hemoglobinopathy patients. Our downstream analysis of non-transfusion-dependent β-thalassemia patients, β-thalassemia major patients, compound heterozygous sickle cell disease/β-thalassemia patients receiving hydroxyurea as fetal hemoglobin augmentation treatment, and non-thalassemic individuals indicated that VEGFA genomic variants were associated with disease severity in β-thalassemia patients and hydroxyurea treatment efficacy in SCD/β-thalassemia compound heterozygous patients.ConclusionsOur findings suggest that VEGFA may act as a modifier gene of human globin gene expression and, at the same time, serve as a genomic biomarker in β-type hemoglobinopathy disease severity and hydroxyurea treatment efficacy.

Significantly elevated foetal haemoglobin levels in individuals with glucose 6-phosphate dehydrogenase disease and/or sickle cell trait: a cross-sectional study in Cape Coast, Ghana

BackgroundPreviously published data have demonstrated that sickle red blood cells produce twice as much reactive oxygen species (ROS) suggesting that co-inheritance of sickle cell disease (SCD) and glucose 6-phosphate dehydrogenase (G6PD) enzymopathy could lead to more severe anaemia during sickling crises. Elevated foetal haemoglobin (Hb F) levels have been shown to have positive modulatory effects on sickling crises and disease outcomes. This study sought to assess how inheritance of G6PD enzymopathy affects the level of Hb F and haemoglobin concentration in adults in steady state.MethodsThis cross-sectional study selected 100 out-patients (41 males and 59 females) visiting the University of Cape Coast hospital, between January, 2016 and May, 2016. Cellulose acetate electrophoresis (pH 8.2–8.6), methaemoglobin reductase test, modified Betke alkaline denaturation methods were used to investigate haemoglobin variants, qualitative G6PD status, and %Hb F levels in venous blood samples drawn from these participants. Data was analysed with GraphPad Prism 6 and SPSS and significance set at p < 0.05.ResultsForty one percent of the participants demonstrated qualitative G6PD enzymopathy whereas only 10% demonstrated Hb AS type (Sickle cell trait, SCT). 5% of the participants co-inherited SCT and G6PD enzymopathy. %Hb F levels in G6PD deficient males was significantly higher than in G6PD deficient females [(p = 0.0003, 2.696% (males) vs 1.975% (females)], although the %Hb F levels was comparable in non-G6PD deficient individuals. %Hb F levels were significantly elevated in males with SCT only (p < 0.05), or G6PD enzymopathy only (p < 0.0001), or SCT + G6PD enzymopathy (p < 0.0001) compared to males with none of these pathologies even though their respective haemoglobin levels were comparable. Male participants with G6PD enzymopathy + SCT co-inheritance had significantly elevated %Hb F when compared to their counterparts with only G6PD enzymopathy (p < 0.001). Male gender [(p = 0.001, OR: 6.912 (2.277–20.984)] partial defective G6PD enzyme [(p = 0.00, OR: 7.567E8 (8.443E7–6.782E9)] SCT [(p = 0.026, OR: 4.625 (1.196–17.881)] were factors associated with raised %Hb F levels ≥2.5.ConclusionThe inheritance of G6PD defect and/or SCT significantly elevate %Hb F levels in the steady state even though haemoglobin levels are not affected.

Elevated Hemoglobin Level Is Associated With Advanced Fibrosis in Pediatric Nonalcoholic Fatty Liver Disease.

Hemoglobin (Hb) and red blood cell distribution width (RDW) have been reported to be a risk marker of metabolic syndrome and nonalcoholic fatty liver disease (NAFLD). No study exists on pediatric populations. We aimed to determine the association between hematological parameters, and the severity of disease in children with biopsy-proven NAFLD. A total of 117 children (85 boys, mean age 12 years) with ultrasound evidence of NAFLD undergoing liver biopsy for diagnosis of nonalcoholic steatohepatitis (NASH), were prospectively enrolled between January 2011 and May 2013 in the setting of a tertiary care center. Children were screened for routine hematological and metabolic parameters, and causes of liver steatosis other than nonalcoholic were excluded, before liver biopsy was performed. A total of 41 NAFLD (boys 29, mean age 11.2 years) and 76 NASH (boys 56, mean age 12.8 years) children were studied. Alanine transaminase levels were significantly higher in NASH group compared with NAFLD group (P = 0.05), and homeostatic model assessment of insulin resistance and triglycerides levels (P = 0.03 and 0.02, respectively). Regarding hematological components: red cell count, Hb, hematocrit, and RDW values were all significantly higher in NASH group compared with NAFLD group (P < 0.05 for each parameter). Children with NASH were more likely to have high levels of RDW compared to those with steatosis only. Moreover, NASH was associated with higher red cell count, Hb, and hematocrit. If confirmed in future follow-up studies, hematological parameters may be introduced in algorithms for NASH risk prediction.

Prevalence of hemolysis and metabolic acidosis in patients with circulatory failure supported with extracorporeal life support: a marker for survival?

Elevated levels of plasma free hemoglobin (fHb) indicate red blood cell (RBC) damage. The aim of this study was to analyze the prevalence of hemolysis and metabolic acidosis in patients on extracorporeal life support (ECLS) and to investigate whether it is a marker for outcome. This retrospective analysis included 215 adult patients with cardiac failure treated with ECLS. The cohort was divided into three groups: ECLS (1) during ongoing cardiopulmonary resuscitation (CPR, n = 110); (2) after CPR with return of spontaneous circulation and sustained cardiogenic shock (n = 45); (3) in severe cardiogenic shock without previous CPR (n = 60). Lactate, arterial pH value and fHb were measured daily before (pre-fHb) and during ECLS. CPR caused a pronounced increase in pre-fHb (group1, 318 (138/586) mg/L; group2, 212 (107/439) mg/L; group3, 79 (53/232) mg/L; p < 0.001). Within 24 hours on ECLS, fHb declined significantly. Compared to group 3 without CPR, group1 and 2 had a lower pH value (group1, 7.10 (6.93/7.20); group2, 7.21 (7.16/7.27); group3, 7.28 (7.20/7.35); p < 0.001), and an increased lactate level (group1, 88 (55/129) mg/dL; group2, 76 (36/111) mg/dL; group3, 52 (25/83) mg/dL; p < 0.0001). Multivariante analysis showed that pre-fHb had no prognostic value for survival. Only a low pre-lactate was a surrogate marker for successful weaning (p < 0.0001) and discharge from hospital (p = 0.0028). CPR was associated with a strongly increased fHb irrespective of ECLS. Implantation of ECLS did not aggravate hemolysis but instead decreased it within 24 hours. In this study low pre-fHb had no predictive value for survival.

Macular hemorrhages associated with neonatal polycythemia and thrombocytopenia: A case report

Thrombocytopenia occurs in 51% of neonates with polycythemia and is independently associated with growth restriction. Increased hematocrit is associated with decreased platelet count. The possibility of a hemorrhage should be noted. A Chinese male newborn presented with elevated hemoglobin and hematocrit levels. The platelet count decreased to 10×109/L during the 1st week after birth and remained abnormal at day 12. Vitreous turbidity of the right eye was detected 2days later and was suspected of stemming from endophthalmitis or ocular inflammation. Two weeks later, vitreous turbidity decreased and a macular hemorrhage became visible. Optical coherence tomography confirmed the diagnosis of a retinal hemorrhage. Thrombocytopenia associated with polycythemia can induce a vitreous hemorrhage, which may be confused with ocular infection or inflammation.