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Fetal Hemoglobin

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https://www.readbyqxmd.com/read/28508434/maternal-arterial-stiffness-and-fetal-cardiovascular-physiology-in-diabetic-pregnancies
#1
Shreya Moodley, Alisa Arunamata, Katie Jo Stauffer, Susan E Nourse, Angela Chen, Amy Quirin, Elif Seda Selamet Tierney
OBJECTIVES: In mothers with pre-gestational or gestational diabetes, abnormal arterial stiffness (stiffer arteries) has been reported. The impact of abnormal maternal arterial stiffness on fetal placental and cardiovascular physiology is unknown. The purpose of the study was to determine the impact of maternal diabetes on maternal arterial stiffness and the association with fetal cardiovascular physiology as measured by fetal echocardiography. METHODS: Between December 2013 and January 2017 we conducted a prospective study on diabetic (otherwise healthy) and non-diabetic, healthy pregnant mothers (at 20-28 gestational weeks and 18-40 years of age) who had a normal fetal cardiac echocardiogram and obstetric ultrasound...
May 15, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28500485/hydroxyurea-lactose-interaction-study-in-silico-and-in-vitro-evaluation
#2
Kunal B Bachchhao, R R Patil, C R Patil, Dipak D Patil
The Maillard reaction between hydroxyurea (a primary amine-containing drug) and lactose (used as an excipient) was explored. The adduct of these compounds was synthesized by heating hydroxyurea with lactose monohydrate at 60 °C in borate buffer (pH 9.2) for 12 h. Synthesis of the adduct was confirmed using UV-visible spectroscopy and Fourier transform infrared, differential scanning calorimetry, high-pressure liquid chromatography, and liquid chromatography-mass spectrometry studies. An in silico investigation of how the adduct formation affected the interactions of hydroxyurea with its biological target oxyhemoglobin, to which it binds to generate nitric oxide and regulates fetal hemoglobin synthesis, was carried out...
May 12, 2017: AAPS PharmSciTech
https://www.readbyqxmd.com/read/28499394/sar1a-promoter-polymorphisms-are-not-associated-with-fetal-hemoglobin-in-patients-with-sickle-cell-disease-from-cameroon
#3
Gift Dineo Pule, Valentina Josiane Ngo Bitoungui, Bernard Chetcha Chemegni, Andre Pascal Kengne, Ambroise Wonkam
BACKGROUND: Reactivation of adult hemoglobin (HbF) is currently a dominant therapeutic approach to sickle cell disease (SCD). In this study, we have investigated among SCD patients from Cameroon, the association of HbF level and variants in the HU-inducible small guanosine triphosphate-binding protein, secretion-associated and RAS-related (SAR1a) protein, previously shown to be associated with HbF after HU treatment in African American SCD patients. RESULTS: Only patients >5 years old were included; hemoglobin electrophoresis and a full blood count were conducted upon arrival at the hospital...
May 12, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28495915/extracellular-glycine-is-necessary-for-optimal-hemoglobinization-of-erythroid-cells
#4
Daniel Garcia-Santos, Matthias Schranzhofer, Richard Bergeron, Alex D Sheftel, Prem Ponka
Vertebrate heme synthesis requires three substrates: succinyl-CoA, which regenerates in the tricarboxylic acid cycle, iron and glycine. For each heme molecule synthesized, one atom of iron and eight molecules of glycine are needed. Inadequate delivery of iron to immature erythroid cells leads to a decreased production of heme, but virtually nothing is known about the consequence of an insufficient supply of extracellular glycine on the process of hemoglobinization. To address this issue, we exploited mice in which the gene encoding glycine transporter 1 (GlyT1) was disrupted...
May 11, 2017: Haematologica
https://www.readbyqxmd.com/read/28478827/mild-anemia-during-pregnancy-upregulates-placental-vascularity-development
#5
A Stangret, M Skoda, A Wnuk, M Pyzlak, D Szukiewicz
The connection between maternal hematological status and pregnancy outcome has been shown by many independent researchers. Attention was initially focused on the adverse effects of moderate and severe anemia. Interestingly, some studies revealed that mild anemia was associated with optimal fetal development and was not affecting pregnancy outcome. The explanation for this phenomenon became a target for scientists. Hemodilution, physiologic anemia and relative decrease in hemoglobin concentration are the changes observed during pregnancy but they do not explain the reasons for the positive influence of mild anemia on a fetomaternal unit...
May 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28473619/nrf2-mediates-%C3%AE-globin-gene-regulation-and-fetal-hemoglobin-induction-in-human-erythroid-progenitors
#6
Xingguo Zhu, Biaoru Li, Betty S Pace
No abstract text is available yet for this article.
May 4, 2017: Haematologica
https://www.readbyqxmd.com/read/28472705/measurements-of-red-cell-deformability-and-hydration-reflect-hbf-and-hba2-in-blood-from-patients-with-sickle-cell-anemia
#7
Nermi L Parrow, Hongbin Tu, James Nichols, Pierre-Christian Violet, Corinne A Pittman, Courtney Fitzhugh, Robert E Fleming, Narla Mohandas, John F Tisdale, Mark Levine
Decreased erythrocyte deformability, as measured by ektacytometry, may be associated with disease severity in sickle cell anemia (SCA). Heterogeneous populations of rigid and deformable cells in SCA blood result in distortions of diffraction pattern measurements that correlate with the concentration of hemoglobin S (HbS) and the percentage of irreversibly sickled cells. We hypothesize that red cell heterogeneity, as well as deformability, will also be influenced by the concentration of alternative hemoglobins such as fetal hemoglobin (HbF) and the adult variant, HbA2...
April 18, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28453928/the-clinical-severity-of-hemoglobin-s-black-a-%C3%AE-%C3%AE-%C3%AE-0-thalassemia
#8
Maria I Cancio, Banu Aygun, David H K Chui, Jennifer A Rothman, J Paul Scott, Jeremie H Estepp, Jane S Hankins
Hemoglobin S/Black ((A) γδβ)(0) -thalassemia is a rare sickle cell disease (SCD) variant. On the basis of limited descriptions in the literature, the disease is reported as a mild microcytic anemia with an uncomplicated course. We report the clinical and laboratory data of nine patients whose diagnoses were confirmed by DNA-based techniques. Despite having mild anemia and high fetal hemoglobin level postinfancy, these patients developed many of the classic complications of SCD, including vaso-occlusive crisis, acute chest syndrome, avascular necrosis, and cholelithiasis...
April 28, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28453539/fetal-hemoglobin-in-umbilical-cord-blood-in-preeclamptic-and-normotensive-pregnancies-a-cross-sectional-comparative-study
#9
Zahra Masoumi, Mary Familari, Karin Källén, Jonas Ranstam, Per Olofsson, Stefan R Hansson
Preeclampsia (PE) is associated with increased fetal hemoglobin (HbF) in the maternal circulation but its source is unknown. To investigate whether excessive HbF is produced in the placenta or the fetus, the concentration of HbF (cHbF) in the arterial and venous umbilical cord blood (UCB) was compared in 15825 normotensive and 444 PE pregnancies. The effect of fetal gender on cHbF was also evaluated in both groups. Arterial and venous UCB sampled immediately after birth at 36-42 weeks of gestation were analyzed for total Hb concentration (ctHb) (g/L) and HbF% using a Radiometer blood gas analyzer...
2017: PloS One
https://www.readbyqxmd.com/read/28435973/acclimatization-to-middle-altitude-hypoxia-protects-against-developmental-and-cognitive-deficits-caused-by-acute-fetal-hypoxia-in-mice
#10
Hui-Lang Liu, Yong-Mei Sun, Chuan-Yu Li, Hai-Chen Niu, Min Su, Jing-Kun Wang
Acute fetal hypoxia (AFH) can elicit postnatal motor deficits and cognitive impairments. To test whether lifelong acclimatization to middle altitude (MA) hypoxia has protective effects on the impairments caused by AFH, ICR mice bred at 1 900 m altitude for 6-7 generations were evaluated under AFH. On gestation day 9 (GD 9), 13 (GD 13) or 17 (GD 17), pregnant mice received a single exposure to acute hypoxia (7% O2, 6 h). Physiological and neurodevelopmental behaviors, motor function (open field), spatial learning and memory (Morris water maze), and anxiety level (elevated plus maze) were examined in the offspring from neonate to adulthood...
April 25, 2017: Sheng Li Xue Bao: [Acta Physiologica Sinica]
https://www.readbyqxmd.com/read/28435652/stroke-in-a-child-with-hemoglobin-sc-disease-a-case-report-describing-use-of-hydroxyurea-after-transfusion-therapy
#11
Diana Fridlyand, Caroline Wilder, E Leila Jerome Clay, Bruce Gilbert, Betty S Pace
Children with hemoglobin SC (HbSC) disease suffer a significant incidence of silent cerebral infarcts but stroke is rare. A 2-year-old African American boy with HbSC disease presented with focal neurologic deficits associated with magnetic resonance imaging evidence of cerebral infarction with vascular abnormalities. After the acute episode he was treated with monthly transfusions and subsequently transitioned to hydroxyurea therapy. The benefits of hydroxyurea as a fetal hemoglobin inducer in HbSC disease, to ameliorate clinical symptoms are supported by retrospective studies...
March 22, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/28424258/serum-hepcidin-concentrations-decline-during-pregnancy-and-may-identify-iron-deficiency-analysis-of-a-longitudinal-pregnancy-cohort-in-the-gambia
#12
Amat Bah, Sant-Rayn Pasricha, Momodou W Jallow, Ebrima A Sise, Rita Wegmuller, Andrew E Armitage, Hal Drakesmith, Sophie E Moore, Andrew M Prentice
Background: Antenatal anemia is a risk factor for adverse maternal and fetal outcomes and is prevalent in sub-Saharan Africa. Less than half of antenatal anemia is considered responsive to iron; identifying women in need of iron may help target interventions. Iron absorption is governed by the iron-regulatory hormone hepcidin.Objective: We sought to characterize changes in hepcidin and its associations with indexes of iron stores, erythropoiesis, and inflammation at weeks 14, 20, and 30 of gestation and to assess hepcidin's diagnostic potential as an index of iron deficiency...
April 19, 2017: Journal of Nutrition
https://www.readbyqxmd.com/read/28420619/analysis-of-variants-in-upstream-open-reading-frames-of-human-globin-related-genes
#13
Ye Yuhua, Zhang Qianqian, Zhong Jianmei, Li Yihong, Zhang Li, Yu Qiuxia, Xu Xiangmin
β-thalassemia is an autosomal recessive monogenic disease that is caused by defects in the production of β-like globin chains. Activation of γ-globin gene and the increase in fetal hemoglobin expression have been demonstrated as one of the most important factors to ameliorate the clinical outcome of β-thalassemia patients. In this study, 202 genes or miRNAs associated with human hemoglobin gene expression from 1802 β-thalassemia patients were analyzed with target capture and next generation sequencing strategies in terms of functional variants that might affect hemoglobin gene expression...
March 20, 2017: Yi Chuan, Hereditas
https://www.readbyqxmd.com/read/28399852/association-of-classical-markers-and-establishment-of-the-dyslipidemic-sub-phenotype-of-sickle-cell-anemia
#14
Milena Magalhães Aleluia, Caroline Conceição da Guarda, Rayra Pereira Santiago, Teresa Cristina Cardoso Fonseca, Fábia Idalina Neves, Regiana Quinto de Souza, Larissa Alves Farias, Felipe Araújo Pimenta, Luciana Magalhães Fiuza, Thassila Nogueira Pitanga, Júnia Raquel Dutra Ferreira, Elisângela Vitória Adorno, Bruno Antônio Veloso Cerqueira, Marilda de Souza Gonçalves
BACKGROUND: Sickle cell anemia (SCA) patients exhibit sub-phenotypes associated to hemolysis and vaso-occlusion. The disease has a chronic inflammatory nature that has been also associated to alterations in the lipid profile. This study aims to analyze hematological and biochemical parameters to provide knowledge about the SCA sub-phenotypes previously described and suggest a dyslipidemic sub-phenotype. METHODS: A cross-sectional study was conducted from 2013 to 2014, and 99 SCA patients in steady state were enrolled...
April 11, 2017: Lipids in Health and Disease
https://www.readbyqxmd.com/read/28369821/does-the-novel-klf1-gene-mutation-lead-to-a-delay-in-fetal-hemoglobin-switch
#15
Priya Hariharan, Manju Gorivale, Roshan Colah, Kanjaksha Ghosh, Anita Nadkarni
The Kruppel-like factor 1 (KLF1) gene is an essential transcription factor that is required for the proper maturation of the erythroid cells. Recent studies have reported that KLF1 variations are associated with increased fetal hemoglobin (HbF) levels. Here we report a novel KLF1 gene variation codon 211 A→G (c.632 A>G) in a family who was referred for hemoglobinopathy screening. Both parents were classical β-thalassemia trait (mother: HbA2 4.1%, HbF 8.6%; father: HbA2 5.5%, HbF 0.6%) codon 15 G→A heterozygous, and the child was β-thalassemia homozygous...
May 2017: Annals of Human Genetics
https://www.readbyqxmd.com/read/28361594/an-unusual-hydrops-fetalis-associated-with-compound-heterozygosity-for-kr%C3%A3-ppel-like-factor-1-mutations
#16
Helena H L Lee, Annisa S L Mak, K O Kou, C F Poon, W S Wong, K H Chiu, Patrick K C Au, Kelvin Y K Chan, Anita S Y Kan, Mary H Y Tang, K Y Leung
Hydrops fetalis is commonly due to Hb Bart's (γ4) disease in South East Asia. Here, we report an unusual case of hydrops fetalis due to congenital dyserythropoietic anemia (CDA) associated with compound heterozygosity for Krüppel-like factor 1 (KLF1) gene mutations. Fetal cardiomegaly was first detected on routine mid-trimester scan in a pregnant woman with normal mean corpuscular volume (MCV) and Rhesus positive status. The fetus subsequently developed hydrops fetalis, and cordocentesis showed severe fetal anemia with a hemoglobin (Hb) level of 3...
November 2016: Hemoglobin
https://www.readbyqxmd.com/read/28355685/-clinical-outcome-and-placenta-characteristics-of-spontaneous-twin-anemia-polycythemia-sequence
#17
X J Wang, L Y Li, Y Wei, Y Y Zhao, P B Yuan
Objective: To investigate the clinical outcome and placental characteristics of spontaneous twin anemia-polycythemia sequence (sTAPS). Methods: Twelve cases with sTAPS delivered in Peking University Third Hospital from May 2013 to August 2016. The data of ultrasound characteristics, gestational age at delivery, and 1 minute Apgar score were analyzed, retrospectively. Placental superficial vascular anastomoses, placental territory discordance and the ratio of umbilical cords insertion distance to the longest placental diameter were also analyzed...
March 25, 2017: Zhonghua Fu Chan Ke za Zhi
https://www.readbyqxmd.com/read/28346212/stability-of-extemporaneously-prepared-hydroxycarbamide-oral-suspensions
#18
Djamila Kabiche, Issa-Bella Balde, Elyes Majoul, Sofiane Kabiche, Elodie Bourguignon, Jean-Eudes Fontan, Salvatore Cisternino, Jöel Schlatter
Hydroxycarbamide, available as tablets, is a pharmacological agent for fetal hemoglobin induction such as sickle cell anemia. The need for alternative dosage form options for patients unable to take tablets led hospital pharmacies to prepare solutions and suspensions. The objective of this study was to determine the stability of hydroxycarbamide in Ora-Plus in combination with either Ora-Sweet or Ora-Sweet SF, Ora-Blend, or Ora-Blend SF suspending agents. The studied samples were compounded into 100-mg/mL suspensions and stored in 60-mL amber glass bottles at room (22°C to 25°C) or refrigerated (4°C to 8°C) temperature...
March 2017: International Journal of Pharmaceutical Compounding
https://www.readbyqxmd.com/read/28344999/long-term-engraftment-and-fetal-globin-induction-upon-bcl11a-gene-editing-in-bone-marrow-derived-cd34-hematopoietic-stem-and-progenitor-cells
#19
Kai-Hsin Chang, Sarah E Smith, Timothy Sullivan, Kai Chen, Qianhe Zhou, Jason A West, Mei Liu, Yingchun Liu, Benjamin F Vieira, Chao Sun, Vu P Hong, Mingxuan Zhang, Xiao Yang, Andreas Reik, Fyodor D Urnov, Edward J Rebar, Michael C Holmes, Olivier Danos, Haiyan Jiang, Siyuan Tan
To develop an effective and sustainable cell therapy for sickle cell disease (SCD), we investigated the feasibility of targeted disruption of the BCL11A gene, either within exon 2 or at the GATAA motif in the intronic erythroid-specific enhancer, using zinc finger nucleases in human bone marrow (BM) CD34(+) hematopoietic stem and progenitor cells (HSPCs). Both targeting strategies upregulated fetal globin expression in erythroid cells to levels predicted to inhibit hemoglobin S polymerization. However, complete inactivation of BCL11A resulting from bi-allelic frameshift mutations in BCL11A exon 2 adversely affected erythroid enucleation...
March 17, 2017: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/28341055/obstetric-care-for-women-with-thalassemia
#20
REVIEW
Terence T Lao
Thalassemia is the commonest monogenic disease and manifests as severe anemia. It is increasingly encountered outside the Mediterranean region, Africa, Middle East, and Southeast Asia because of immigration. Pregnancy, previously uncommon in patients with homozygous β-thalassemia, is encountered increasingly because of improved management and assisted reproduction technology; however, preconceptional problems that include anemia, iron overload, cardiac dysfunction, thromboembolism, alloimmunization, infections, and endocrine and bone disorders, could influence maternal and obstetric outcome...
February 2017: Best Practice & Research. Clinical Obstetrics & Gynaecology
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