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Fetal Hemoglobin

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https://www.readbyqxmd.com/read/28643377/randomized-feasibility-trial-to-improve-hydroxyurea-adherence-in-youth-ages-10-18-years-through-community-health-workers-the-habit-study
#1
Nancy S Green, Deepa Manwani, Sergio Matos, April Hicks, Luisa Soto, Yina Castillo, Karen Ireland, Yvonne Stennett, Sally Findley, Haomiao Jia, Arlene Smaldone
INTRODUCTION: The main therapeutic intervention for sickle cell disease (SCD) is hydroxyurea (HU). The effect of HU is largely through dose-dependent induction of fetal hemoglobin (HbF). Poor HU adherence is common among adolescents. METHODS: Our 6-month, two-site pilot intervention trial, "HABIT," was led by culturally aligned community health workers (CHWs). CHWs performed support primarily through home visits, augmented by tailored text message reminders. Dyads of youth with SCD ages 10-18 years and a parent were enrolled...
June 23, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28641125/an-altered-redox-environment-assisted-by-over-expression-of-fetal-hemoglobins-protects-from-inflammatory-colitis-and-reduces-inflammatory-cytokine-expression
#2
R M Gorczynski, C Alexander, K Brandenburg, Z Chen, A Heini, D Neumann, J P Mach, E T Rietschel, A Tersikh, A J Ulmer, Kai Yu, U Zahringer, I Khatri
C5BL/6 female mice receiving dextran sodium sulfate in their drinking water develop an acute inflammatory colitis within 7d, with weight loss, histopathologic signs of inflammation, and colonic expression of inflammatory cytokines. In previous studies we have reported that increased inflammatory cytokine expression in aged mice can be attenuated by oral gavage of a crude fetal extract containing glutathione (GSH), MPLA and fetal hemoglobin, or more specifically by injection of a combination of these purified reagents...
June 19, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28633140/early-intrauterine-transfusion-in-fetuses-with-severe-anemia-caused-by-parvovirus-b19-infection
#3
Astrid Hellmund, Annegret Geipel, Christoph Berg, Rainer Bald, Ulrich Gembruch
OBJECTIVE: To describe procedure-related complications and perinatal survival after intrauterine transfusion (IUT) before 20 weeks of gestation in fetuses with severe anemia due to human parvovirus B19 infection. MATERIALS AND METHODS: A retrospective study was conducted of all fetuses requiring IUT before 20 weeks of gestation in two tertiary referral centers between January 2002 and July 2015. Gestational age (GA) at first IUT, fetal blood sampling results, and presence of hydrops were related to procedure-related complications, fetal death (FD), and perinatal outcome...
June 21, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28628087/chromatin-enriched-lncrnas-can-act-as-cell-type-specific-activators-of-proximal-gene-transcription
#4
Michael S Werner, Matthew A Sullivan, Rohan N Shah, Rangarajan D Nadadur, Adrian T Grzybowski, Vasiliy Galat, Ivan P Moskowitz, Alexander J Ruthenburg
We recently described a new class of long noncoding RNAs (lncRNAs) that are distinguished by especially tight chromatin association and whose presence is strongly correlated to expression of nearby genes. Here, we examine the cis-enhancer mechanism of this class of chromatin-enriched RNA (cheRNA) across multiple human cell lines. cheRNAs are largely cell type specific and provide the most reliable chromatin signature to predict cis-gene transcription in every human cell type examined. Targeted depletion of three cheRNAs decreases expression of their neighboring genes, indicating potential co-activator function, and single-molecule fluorescence in situ hybridization (smFISH) of one cheRNA-distal target gene pair suggests a spatial overlap consistent with a role in chromosome looping...
June 19, 2017: Nature Structural & Molecular Biology
https://www.readbyqxmd.com/read/28627837/natural-antioxidant-vitamins-status-in-pregnancies-complicated-with-intrauterine-growth-restriction
#5
Amita K Gadhok, Tarun K Sharma, Maheep Sinha, Rakesh Khunteta, Satish K Vardey, Poonam Sahni, Manisha Sankhla
BACKGROUND: The weight of the infant at birth is a powerful predictor of infant growth and survival and is dependent on maternal health and nutrition during pregnancy. Pregnant women have a defense mechanism against increased oxidative stress composed of antioxidant enzymes and natural antioxidant vitamins like vitamin C and E. Therefore, we evaluated whether differences exist in serum levels of vitamin C and E in pregnant women complicated with intrauterine growth restriction (IUGR)...
May 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28627190/-relationships-between-umbilical-vein-and-mother-iron-status
#6
Afaf Mezdoud, Abdel-Nacer Agli, Hayet Oulamara
INTRODUCTION: Iron is an essential micronutrient in the growing fetus. OBJECTIVE: The purpose of this study is to find the possible correlations that may exist between maternal and fetal iron status and newborn weight. MATERIAL AND METHODS: The study included 97 mothers scheduled to give birth by elective caesarean section in the central maternity of Tébessa (east of Algeria) between January and August 2014. The blood collection was sampled from the antecubital vein of the mother and the umbilical vein...
June 5, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/28624809/genome-wide-analysis-of-aberrantly-expressed-lncrnas-and-mirnas-with-associated-co-expression-and-cerna-networks-in-%C3%AE-thalassemia-and-hereditary-persistence-of-fetal-hemoglobin
#7
Ketong Lai, Siyuan Jia, Shanjuan Yu, Jianming Luo, Yunyan He
The implications of lncRNAs regarding fetal hemoglobin (HbF) induction in hemoglobin disorders remain poorly understood. In this study, microarray analysis was performed to profile lncRNAs, miRNAs and mRNAs in individuals with hereditary persistence of fetal hemoglobin (HPFH), β-thalassemia carriers with high HbF levels and healthy controls. The results show aberrant expression of 862 lncRNAs, 568 mRNAs and 63 miRNAs in the high-HbF group compared with the control group. Altered NR_001589, NR_120526, T315543, miR-486-3p, miR-19b-1-5p and miR-20a-3p expression was confirmed by quantitative reverse transcription-polymerase chain reaction, and Spearman correlation coefficients revealed significant positive correlations with HbF...
May 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28611934/etiology-and-perinatal-outcome-of-nonimmune-hydrops-fetalis-in-southern-china
#8
Sheng He, Linlin Wang, Pingshan Pan, Hongwei Wei, Dahua Meng, Juan Du, Xiaoxian Tian, Chenguang Zheng, Xiaoxia Qiu, Guoyang Luo
Objective  This study aims to analyze the etiology and perinatal outcome of nonimmune hydrops fetalis (NIHF) in Southern China. Methods  All cases with NIHF diagnosed antenatally from January 1, 2007 to December 31, 2014 were identified and analyzed. Results  Total 482 cases of NIHF were identified during the study period. The most common cause of NIHF was hemoglobin (Hb) Bart's disease (61.8%), followed by chromosomal abnormalities (13.5%), idiopathic etiology (13.1%), cardiac abnormalities (6.4%), and others (5...
April 2017: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/28599394/pulmo-uterinus-a-history-of-ideas-on-fetal-respiration
#9
REVIEW
Michael Obladen
Theories about fetal respiration began in antiquity. Aristotle characterized pneuma as warm air, but also as the enabler of vital functions and instrument of the soul. In Galen's system of physiology, the vital spirit was carried by the umbilical arteries, the nutrients by the umbilical vein from the placenta to the fetus. In 1569 Aranzio postulated that the maternal and fetal vasculatures are distinct. From 1670 to 1690, a century before the discovery of oxygen, researchers understood that during respiration some form of exchange with the air must occur, naming the substance biolychnium, phlogiston, sal-nitro, or nitro-aerial particles...
May 24, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28589569/identification-of-novel-bcl11a-variants-in-patients-with-epileptic-encephalopathy-expanding-the-phenotypic-spectrum
#10
Michiko Yoshida, Mitsuko Nakashima, Tohru Okanishi, Sotaro Kanai, Ayataka Fujimoto, Kazuya Itomi, Masafumi Morimoto, Hirotomo Saitsu, Mitsuhiro Kato, Naomichi Matsumoto, Tomohiro Chiyonobu
BCL11A encodes a zinc finger protein that is highly expressed in hematopoietic tissues and the brain, and that is known to function as a transcriptional repressor of fetal hemoglobin (HbF). Recently, de novo variants in BCL11A have been reported in individuals with intellectual disability syndrome without epilepsy. In this study, we performed whole-exome sequencing of 302 patients with epileptic encephalopathies (EEs), and identified two novel BCL11A variants, c.577delC (p.His193Metfs*3) and c.2351A>C (p...
June 6, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28567595/do-alpha-thalassemia-fetal-hemoglobin-and-the-ugt1a1-polymorphism-have-an-influence-on-serum-bilirubin-levels-and-cholelithiasis-in-patients-with-sickle-cell-disease
#11
Laura Alencastro de Azevedo, Joyce Bonazzoni, Sandrine Comparsi Wagner, Mariela Granero Farias, Christina M Bittar, Liane Daudt, Simone Martins de Castro
BACKGROUND: Increased destruction of erythrocytes in patients with sickle cell disease results in chronic hyperbilirubinemia and leads to the formation of gallstones. OBJECTIVES: The objective of this study was to determine the combined influence of alpha thalassemia, fetal hemoglobin, and the UGT1A1 polymorphism on serum bilirubin levels and cholelithiasis in patients with sickle cell disease. METHODS: We analyzed 72 patients treated in the outpatient hematology unit of the Clinical Hospital of Porto Alegre...
May 31, 2017: Molecular Diagnosis & Therapy
https://www.readbyqxmd.com/read/28540888/assessment-of-renal-function-in-indian-patients-with-sickle-cell-disease
#12
Bhaskar V K S Lakkakula, Henu Kumar Verma, Mona Choubey, Suneeta Patra, Prafukka Kumar Khodiar, Pradeep Kumar Patra
Sickle cell disease (SCD) and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin. Renal disease is one of the most frequent complications, and kidney damage starts very early and progresses throughout life causing severe complications. The present study is aimed to analyze creatinine-based estimated glomerular filtration rate (eGFR) in 616 SCD patients (507 HbSS and 109 HbSB+), receiving medical care at outpatient wing of Sickle Cell Institute, Chhattisgarh. Glomerular filtration rate (GFR) estimated using the Modification of Diet in Renal Disease (MDRD), Cockcroft-Gault, chronic kidney disease epidemiology collaboration (CKD-EPI) (<17 years analyzed with Schwartz), and SCD specific Jamaica Sickle Cell Cohort Study (JSCCS)-GFR equations were compared...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28508434/maternal-arterial-stiffness-and-fetal-cardiovascular-physiology-in-diabetic-pregnancies
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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...
June 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
#20
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
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