BMC Hematology最新文献

{"title":"Pregnant mothers are more anemic than lactating mothers, a comparative cross-sectional study, Bahir Dar, Ethiopia.","authors":"Berhanu Elfu Feleke,&nbsp;Teferi Elfu Feleke","doi":"10.1186/s12878-018-0096-1","DOIUrl":"https://doi.org/10.1186/s12878-018-0096-1","url":null,"abstract":"<p><strong>Background: </strong>Information on the hemoglobin status of pregnant and lactating mothers was scarce. The objectives of this study were to determine the burden and determinants of anemia in the pregnant and lactating mother.</p><p><strong>Methods: </strong>A comparative cross-sectional study was conducted. Descriptive statistics were used to identify the prevalence of anemia. Binary logistic regression and multiple linear regressions were used to identify the predictors of anemia.</p><p><strong>Results: </strong>The prevalence of anemia in lactating and pregnant women was 43.00% (95% CI {confidence interval}, 41% - 45%) and 84% of anemia was microcytic and hypocromic anemia. Anemia in lactating and pregnant women was positively associated with malaria infection [AOR{adjusted odds ratio} 3.61 (95% CI: 2.63-4.95)], abortion [AOR 6.63 (95% CI: 3.23-13.6)], hookworm infection [AOR 3.37 (95% CI: 2.33-4.88)], tea consumption [AOR 3.63 (95% CI: 2.56-5.14)], pregnancy [AOR 2.24 (95% CI: 1.57-3.12)], and Mid-upper arm circumference [ <b><i>B</i></b> 0.36 (95% <i>CI:</i> 0.33, -0.4)]. Anemia in pregnant and lactating mother was negatively associated with urban residence [AOR 0.68, (95% CI: 0.5-0.94)], iron supplementation during pregnancy [AOR 0.03 (95% CI, 0.02-0.04)], parity [ <b><i>B</i></b> -0.18 (95% CI: -0.23, -0.14)], age [B -0.03 (95% <i>CI:</i> -0.04, -0.03)].</p><p><strong>Conclusion: </strong>The burden of anemia was higher in pregnant women than lactating women.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"2"},"PeriodicalIF":0.0,"publicationDate":"2018-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-018-0096-1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35768807","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A novel fibrinogen mutation: <i>FGA</i> g. 3057 C > T (p. Arg104 > Cys) impairs fibrinogen secretion.","authors":"R Marchi,&nbsp;M Linares,&nbsp;H Rojas,&nbsp;A Ruiz-Sáez,&nbsp;M Meyer,&nbsp;A Casini,&nbsp;S O Brennan","doi":"10.1186/s12878-017-0086-8","DOIUrl":"https://doi.org/10.1186/s12878-017-0086-8","url":null,"abstract":"<p><strong>Background: </strong>Abnormal fibrinogens can be caused by clinically silent hereditary mutations. A new case was detected accidentally in an 11-year-old girl when routine pre-operative coagulation tests were performed for nasal turbinate surgery.</p><p><strong>Methods: </strong>The fibrinogen genes FGA, FGG and FGB were sequenced using standard protocols. The kinetics of fibrin formation were followed by turbidity at 350 nm. Purified fibrinogen was incubated with plasmin, and the degradation products analyzed by SDS/PAGE. The formation of fibrinogen-albumin complexes was analyzed by immunobloting. Fibrin structure was examined in a Nikon Eclipse TE 2000-U laser microscope. Secretion of the variant protein was analyzed directly by reverse phase-electrospray time of flight-mass spectrometry (TOF-MS).</p><p><strong>Results: </strong>DNA sequencing revealed a novel heterozygous g. 3057 C > T mutation in the <i>FGA</i> that predicts a p. Arg104 > Cys substitution, in the proband and her father. Both patients were asymptomatic with low functional and antigen fibrinogen concentrations. The proband's plasma fibrinogen polymerization was almost normal, with a 12% decrease in the final turbidity, while, the father's fibrin formation had a diminished slope and final turbidity (2.5× and 40%, respectively). Aα Arg104 is located at a plasmin cleavage site in the coiled-coil region of fibrinogen. However, the father's fibrinogen plasmin degradation was normal. Although the exchanged Cys introduces an unpaired -SH, immunoblotting showed no fibrinogen-albumin complexes. Furthermore, the plasma clot structure observed by confocal microscopy appeared almost normal. TOF-MS showed that the variant Aα chain was underrepresented in plasma and made up only about 25% of the total.</p><p><strong>Conclusions: </strong>The low expression of the Aα Arg104 > Cys chain in circulation could account for the observed hypodysfibrinogenemia.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":" ","pages":"22"},"PeriodicalIF":0.0,"publicationDate":"2017-12-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-017-0086-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35707025","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A comparative cross-sectional study of some hematological parameters of hypertensive and normotensive individuals at the university of Gondar hospital, Northwest Ethiopia.","authors":"Bamlaku Enawgaw,&nbsp;Nigist Adane,&nbsp;Betelihem Terefe,&nbsp;Fikir Asrie,&nbsp;Mulugeta Melku","doi":"10.1186/s12878-017-0093-9","DOIUrl":"https://doi.org/10.1186/s12878-017-0093-9","url":null,"abstract":"<p><strong>Background: </strong>Hypertension is a major health problem worldwide. It can lead to cardiovascular disease and also leads to functional disturbances including hematological parameters. The abnormalities of haematological parameters may enhance an end-organ damage. Therefore, the aim of this study was to assess some hematological parameters of hypertensive individuals in comparison with normotensive individuals at University of Gondar hospital, northwest Ethiopia.</p><p><strong>Methods: </strong>A cross sectional comparative study was conducted from October to November 2015 on a total of 126 hypertensive and 126 normotensive individuals at University of Gondar Hospital. All participants after taking informed consent were interviewed for detailed history and 3 ml of blood was collected for hematological test analysis. Independent <i>t</i>-test and the Mann Whitney <i>u</i>-test were used to find out significant difference and Pearson's and Spearman's correlation were used for correlation test. <i>P</i> values less than 0.05 was considered the level of significance.</p><p><strong>Result: </strong>From a total of 252 study subjects, about 67.5% were females. The mean age of study subjects was 50.3 ± 11 years for hypertensive individuals and 49.8 ± 11.6 years for normotensive individuals with range of 18-65 years. In the present study, the median (IQR) value of WBC, RBC, Hgb, HCT, MCV and the mean value of MCHC, RDW, MPV and PDW were significantly higher in hypertensive group compared to apparently healthy normotensive groups. Additionally, WBC, RBC, Hgb, HCT and PLT showed statistically significant positive correlations with blood pressure indices. Platelet count and MCH did not show statistically significant difference between the two groups.</p><p><strong>Conclusion: </strong>Hypertension has impact on hematological parameters. In this study, the mean and median values of haematological parameters in hypertensive individuals were significantly different compared to apparently healthy normotensive individuals. Hence, hematological parameters can be used to monitor the prognosis of the disease and manage hypertensive related complications, and it is important to assess hematological parameters for hypertensive individuals which may help to prevent complications associated hematological disorders.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":" ","pages":"21"},"PeriodicalIF":0.0,"publicationDate":"2017-11-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-017-0093-9","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35227206","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Knowledge, attitude and practice of students towards blood donation in Arsi university and Adama science and technology university: a comparative cross sectional study.","authors":"Habtom Woldeab Gebresilase,&nbsp;Robera Olana Fite,&nbsp;Sileshi Garoma Abeya","doi":"10.1186/s12878-017-0092-x","DOIUrl":"https://doi.org/10.1186/s12878-017-0092-x","url":null,"abstract":"<p><strong>Background: </strong>Blood can save millions of lives. Even though people do not donate blood regularly, there is a constant effort to balance the supply and demand of blood. The aim of this study was, therefore, to determine the knowledge, attitude and practice of blood donation between university students.</p><p><strong>Methods: </strong>The comparative cross sectional study design was used in Adama Science and Technology University and Arsi University from April 11-May 2, 2016.360 students were selected using stratified sampling. Frequencies and proportions were computed. Chi-Square and logistic regressions were carried out and associations were considered significant at <i>p</i><0.05.</p><p><strong>Result: </strong>The study revealed that there was a significant knowledge difference (χ2 = 152.779, <i>p</i><0.001) and Attitude difference (χ2 = 4.142, <i>p</i> = 0.042) between Health Science students of Arsi University and Non-Health Science students of Adama Science and Technology University. The gender of the students (AOR = 3.150, 95% CI: 1.313, 7.554) was a significant predictor of the level of knowledge of Health Science students. The ethnicity of students (AOR = 2.085, 95% CI: 1.025, 4.243) was a significant predictor of the level of an attitude of Health Science students and gender of students (AOR = 0.343, 95% CI: 0.151, 0.779) was a significant predictor of the level of an attitude of Health Science students. Concerning Non-Health Science students, religion (AOR = 10.173, 95% CI: 1.191, 86.905) and original residence (AOR = 0.289, 95% CI: 0.094, 0.891) were a significant predictor of the level of knowledge of Non-Health Science students. Gender (AOR = 0.389, 95% CI: 0.152, 0.992) and Year of study (AOR = 0.389(0.164, 0.922) were significant predictor of level of attitude of Non-Health Science students. Year of study (AOR = 5.159, 95% CI: 1.611, 16.525) was a significant predictor of level of practice of Health Science students.</p><p><strong>Conclusion: </strong>Significant knowledge difference and attitude difference were observed between students from Arsi University and Adama Science and Technology University.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":" ","pages":"20"},"PeriodicalIF":0.0,"publicationDate":"2017-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-017-0092-x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35307867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Emergency blood transfusion practices among anaemic children presenting to an urban emergency department of a tertiary hospital in Tanzania.","authors":"Catherine R Shari,&nbsp;Hendry R Sawe,&nbsp;Brittany L Murray,&nbsp;Victor G Mwafongo,&nbsp;Juma A Mfinanga,&nbsp;Michael S Runyon","doi":"10.1186/s12878-017-0091-y","DOIUrl":"https://doi.org/10.1186/s12878-017-0091-y","url":null,"abstract":"<p><strong>Background: </strong>Severe anaemia contributes significantly to mortality, especially in children under 5 years of age. Timely blood transfusion is known to improve outcomes. We investigated the magnitude of anaemia and emergency blood transfusion practices amongst children under 5 years presenting to the Emergency Department (ED) of Muhimbili National Hospital (MNH) in Tanzania.</p><p><strong>Methods: </strong>This prospective observational study enrolled children under 5 years old with anaemia, over a 7-week period in August and September of 2015. Anaemia was defined as haemoglobin of <11 g/dL. Demographics, anaemia severity, indications for transfusion, receipt of blood, and door to transfusion time were abstracted from the charts using a standardized data entry form. Anaemia was categorized as severe (Hb <7 g/dL), moderate (Hb 7-9.9 g/dL) or mild (Hb 10-10.9 g/dL).</p><p><strong>Results: </strong>We screened 777 children, of whom 426 (55%) had haemoglobin testing. Test results were available for 388/426 (91%), 266 (69%) of whom had anaemia. Complete data were available for 257 anaemic children, including 42% (<i>n</i> = 108) with severe anaemia, 40% (<i>n</i> = 102) with moderate anaemia and 18% (<i>n</i> = 47) with mild anaemia. Forty-nine percent of children with anaemia (<i>n</i> = 125) had indications for blood transfusion, but only 23% (29/125) were transfused in the ED. Among the non-transfused, the provider did not identify anaemia in 42% (<i>n</i> = 40), blood was not ordered in 28% (<i>n</i> = 27), and blood was ordered, but not available in 30% (<i>n</i> = 29). The median time to transfusion was 7.8 (interquartile range: 1.9) hours. Mortality was higher for the children with severe anemia who were not transfused as compared with those with severe anaemia who were transfused (29% vs 10%, <i>p</i> = 0.03).</p><p><strong>Conclusion: </strong>The burden of anaemia is high among children under 5 presenting to EMD-MNH. Less than a quarter of children with indications for transfusion receive it in the EMD, the median time to transfusion is nearly 8 h, and those not transfused have nearly a 3-fold higher mortality. Future quality improvement and research efforts should focus on eliminating barriers to timely blood transfusion.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":" ","pages":"19"},"PeriodicalIF":0.0,"publicationDate":"2017-11-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-017-0091-y","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35618430","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Coagulation profile of Sudanese children with homozygous sickle cell disease and the effect of treatment with omega-3 fatty acid on the coagulation parameters.","authors":"Shiekh Awoda,&nbsp;Ahmed A Daak,&nbsp;Nazik Elmalaika Husain,&nbsp;Kebreab Ghebremeskel,&nbsp;Mustafa I Elbashir","doi":"10.1186/s12878-017-0089-5","DOIUrl":"https://doi.org/10.1186/s12878-017-0089-5","url":null,"abstract":"<p><strong>Background: </strong>It has been reported that patients with SCD do have an abnormal coagulation profile. Coagulopathy is thought to be one of the key factors that contribute to the vaso-occlusive crisis that characterises sickle cell disease (SCD). In this study, we investigated whether Sudanese sickle cell patients have an abnormal coagulation profile. In addition, the effect of treatment with either omega-3 fatty acids or hydroxyurea on coagulation profile was assessed.</p><p><strong>Methods: </strong>Homozygous SCD patients untreated (<i>n</i> = 52), omega-3 treated (<i>n</i> = 44), hydroxyurea (HU) treated (<i>n</i> = 8) and healthy (HbAA) controls (<i>n</i> = 52) matched for age (4-20 years), gender and socioeconomic status were enrolled. Patients on omega-3 fatty acids, according to age, received one to four capsules containing 277.8 mg DHA and 39.0 mg eicosapentnoic. Patients on Hydroxyurea were in on dosage more than 20 mg/kg/day. The steady state levels of the coagulation parameters and the effect of the treatments with either HU or omega-3 fatty acids on markers of coagulation were investigated.</p><p><strong>Results: </strong>Compared to the healthy controls, treated and untreated HbSS patients had lower hemoglobin, plasma Protein C, proteins S and higher white blood cell count (WBC), platelets count (PLTs) and plasma D-dimer levels,(<i>p</i> < 0.05). In comparison to untreated HbSS, treatment with neither omega-3 nor HU had effect on the WBC, plasma proteins C and S, (<i>p</i> > 0.05). HU treated group had a lower PLTs count compared to HbSS untreated group (<i>p</i> < 0.5). The prothrombin and activated partial thromboplastin times and international normalized ratio (INR) of untreated patients are significantly higher than n-3 treated, HU-treated patients and health controls, (<i>p</i> < 0.05). Patients treated with omega-3 had lowered D-dimer levels in comparison to HU-treated and untreated HbSS patients, (<i>p</i> < 0.001).</p><p><strong>Conclusion: </strong>This study provides evidence that Sudanes patients have abnormal coagulation profile and treatment with either HU or omega-3 fatty acids might partially ameliorate SCD-associated chronic coagulopathic state.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":" ","pages":"18"},"PeriodicalIF":0.0,"publicationDate":"2017-11-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-017-0089-5","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35618429","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence and determinants of anemia among pregnant women in Ethiopia; a systematic review and meta-analysis.","authors":"Getachew Mullu Kassa,&nbsp;Achenef Asmamaw Muche,&nbsp;Abadi Kidanemariam Berhe,&nbsp;Gedefaw Abeje Fekadu","doi":"10.1186/s12878-017-0090-z","DOIUrl":"https://doi.org/10.1186/s12878-017-0090-z","url":null,"abstract":"<p><strong>Background: </strong>Anemia during pregnancy is one of the most common indirect obstetric cause of maternal mortality in developing countries. It is responsible for poor maternal and fetal outcomes. A limited number of studies were conducted on anemia during pregnancy in Ethiopia, and they present inconsistent findings. Therefore, this review was undertaken to summarize the findings conducted in several parts of the country and present the national level of anemia among pregnant women in Ethiopia.</p><p><strong>Methods: </strong>Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline was followed for this systematic review and meta-analysis. The databases used were; PUBMED, Cochrane Library, Google Scholar, CINAHL, and African Journals Online. Search terms used were; anemia, pregnancy related anemia and Ethiopia. Joanna Briggs Institute Meta-Analysis of Statistics Assessment and Review Instrument (JBI-MAStARI) was used for critical appraisal of studies. The meta-analysis was conducted using STATA 14 software. The pooled Meta logistic regression was computed to present the pooled prevalence and relative risks (RRs) of the determinate factors with 95% confidence interval (CI).</p><p><strong>Results: </strong>Twenty studies were included in the meta-analysis with a total of 10, 281 pregnant women. The pooled prevalence of anemia among pregnant women in Ethiopia was 31.66% (95% CI (26.20, 37.11)). Based on the pooled prevalence of the subgroup analysis result, the lowest prevalence of anemia among pregnant women was observed in Amhara region, 15.89% (95% CI (8.82, 22.96)) and the highest prevalence was in Somali region, 56.80% (95% CI (52.76, 60.84)). Primigravid (RR: 0.61 (95% CI: 0.53, 0.71)) and urban women (RR: 0.73 (95% CI: 0.60, 0.88)) were less likely to develop anemia. On the other hand, mothers with short pregnancy interval (RR: 2.14 (95% CI: 1.67, 2.74)) and malaria infection during pregnancy (RR: 1.94 (95% CI: 1.33, 2.82)) had higher risk to develop anemia.</p><p><strong>Conclusions: </strong>Almost one-third of pregnant women in Ethiopia were anemic. Statistically significant association was observed between anemia during pregnancy and residence, gravidity, pregnancy interval, and malaria infection during pregnancy. Regions with higher anemia prevalence among pregnant women should be given due emphasis. The concerned body should intervene on the identified factors to reduce the high prevalence of anemia among pregnant women.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":" ","pages":"17"},"PeriodicalIF":0.0,"publicationDate":"2017-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-017-0090-z","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35548154","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"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.","authors":"Patrick Adu,&nbsp;Essel K M Bashirudeen,&nbsp;Florence Haruna,&nbsp;Edward Morkporkpor Adela,&nbsp;Richard K D Ephraim","doi":"10.1186/s12878-017-0088-6","DOIUrl":"https://doi.org/10.1186/s12878-017-0088-6","url":null,"abstract":"<p><strong>Background: </strong>Previously 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.</p><p><strong>Methods: </strong>This 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 <i>p</i> < 0.05.</p><p><strong>Results: </strong>Forty 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 [(<i>p</i> = 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 (<i>p</i> < 0.05), or G6PD enzymopathy only (<i>p</i> < 0.0001), or SCT + G6PD enzymopathy (<i>p</i> < 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 (<i>p</i> < 0.001). Male gender [(<i>p</i> = 0.001, OR: 6.912 (2.277-20.984)] partial defective G6PD enzyme [(p = 0.00, OR: 7.567E8 (8.443E7-6.782E9)] SCT [(<i>p</i> = 0.026, OR: 4.625 (1.196-17.881)] were factors associated with raised %Hb F levels ≥2.5.</p><p><strong>Conclusion: </strong>The inheritance of G6PD defect and/or SCT significantly elevate %Hb F levels in the steady state even though haemoglobin levels are not affected.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":" ","pages":"16"},"PeriodicalIF":0.0,"publicationDate":"2017-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-017-0088-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35500189","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparative study of sickle cell anemia and hemoglobin SC disease: clinical characterization, laboratory biomarkers and genetic profiles.","authors":"Milena Magalhães Aleluia,&nbsp;Teresa Cristina Cardoso Fonseca,&nbsp;Regiana Quinto Souza,&nbsp;Fábia Idalina Neves,&nbsp;Caroline Conceição da Guarda,&nbsp;Rayra Pereira Santiago,&nbsp;Bruna Laís Almeida Cunha,&nbsp;Camylla Villas Boas Figueiredo,&nbsp;Sânzio Silva Santana,&nbsp;Silvana Sousa da Paz,&nbsp;Júnia Raquel Dutra Ferreira,&nbsp;Bruno Antônio Veloso Cerqueira,&nbsp;Marilda de Souza Gonçalves","doi":"10.1186/s12878-017-0087-7","DOIUrl":"https://doi.org/10.1186/s12878-017-0087-7","url":null,"abstract":"<p><strong>Background: </strong>In this study, we evaluate the association of different clinical profiles, laboratory and genetic biomarkers in patients with sickle cell anemia (SCA) and hemoglobin SC disease (HbSC) in attempt to characterize the sickle cell disease (SCD) genotypes.</p><p><strong>Methods: </strong>We conducted a cross-sectional study from 2013 to 2014 in 200 SCD individuals (141 with SCA; 59 with HbSC) and analyzed demographic data to characterize the study population. In addition, we determined the association of hematological, biochemical and genetic markers including the β^S-globin gene haplotypes and the 3.7 Kb deletion of α-thalassemia (-α^3.7Kb-thal), as well as the occurrence of clinical events in both SCD genotypes.</p><p><strong>Results: </strong>Laboratory parameters showed a hemolytic profile associated with endothelial dysfunction in SCA individuals; however, the HbSC genotype was more associated with increased blood viscosity and inflammatory conditions. The BEN haplotype was the most frequently observed and was associated with elevated fetal hemoglobin (HbF) and low S hemoglobin (HbS). The -α^3.7Kb-thal prevalence was 0.09 (9%), and it was associated with elevated hemoglobin and hematocrit concentrations. Clinical events were more frequent in SCA patients.</p><p><strong>Conclusions: </strong>Our data emphasize the differences between SCA and HbSC patients based on laboratory parameters and the clinical and genetic profile of both genotypes.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":" ","pages":"15"},"PeriodicalIF":0.0,"publicationDate":"2017-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-017-0087-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35428832","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Alterations in hematologic indices during long-duration spaceflight.","authors":"Hawley Kunz, Heather Quiriarte, Richard J Simpson, Robert Ploutz-Snyder, Kathleen McMonigal, Clarence Sams, Brian Crucian","doi":"10.1186/s12878-017-0083-y","DOIUrl":"10.1186/s12878-017-0083-y","url":null,"abstract":"<p><strong>Background: </strong>Although a state of anemia is perceived to be associated with spaceflight, to date a peripheral blood hematologic assessment of red blood cell (RBC) indices has not been performed during long-duration space missions.</p><p><strong>Methods: </strong>This investigation collected whole blood samples from astronauts participating in up to 6-months orbital spaceflight, and returned those samples (ambient storage) to Earth for analysis. As samples were always collected near undock of a returning vehicle, the delay from collection to analysis never exceeded 48 h. As a subset of a larger immunologic investigation, a complete blood count was performed. A parallel stability study of the effect of a 48 h delay on these parameters assisted interpretation of the in-flight data.</p><p><strong>Results: </strong>We report that the RBC and hemoglobin were significantly elevated during flight, both parameters deemed stable through the delay of sample return. Although the stability data showed hematocrit to be mildly elevated at +48 h, there was an in-flight increase in hematocrit that was ~3-fold higher in magnitude than the anticipated increase due to the delay in processing.</p><p><strong>Conclusions: </strong>While susceptible to the possible influence of dehydration or plasma volume alterations, these results suggest astronauts do not develop persistent anemia during spaceflight.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":" ","pages":"12"},"PeriodicalIF":0.0,"publicationDate":"2017-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5590186/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35402257","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}