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Fetal Hemoglobin

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https://www.readbyqxmd.com/read/27923585/prenatal-lead-exposure-and-fetal-growth-smaller-infants-have-heightened-susceptibility
#1
Rodosthenis S Rodosthenous, Heather H Burris, Katherine Svensson, Chitra J Amarasiriwardena, Alejandra Cantoral, Lourdes Schnaas, Adriana Mercado-García, Brent A Coull, Robert O Wright, Martha M Téllez-Rojo, Andrea A Baccarelli
BACKGROUND: As population lead levels decrease, the toxic effects of lead may be distributed to more sensitive populations, such as infants with poor fetal growth. OBJECTIVES: To determine the association of prenatal lead exposure and fetal growth; and to evaluate whether infants with poor fetal growth are more susceptible to lead toxicity than those with normal fetal growth. METHODS: We examined the association of second trimester maternal blood lead levels (BLL) with birthweight-for-gestational age (BWGA) z-score in 944 mother-infant participants of the PROGRESS cohort...
December 3, 2016: Environment International
https://www.readbyqxmd.com/read/27909215/cure-for-thalassemia-major-from-allogeneic-hematopoietic-stem-cell-transplantation-to-gene-therapy
#2
Alok Srivastava, Ramachandran V Shaji
Allogeneic hematopoietic stem cell transplantation has been established for several decades as a gene replacement therapy for patients with thalassemia major and now offers very high rates of cure to those who are able to access this therapy. Outcomes have improved tremendously over the last decade even in high-risk patients. The limited data available suggests that the long-term outcome is also excellent with >90% survival but for best results, hematopoietic stem cell transplantation should be offered early before any end organ damage occurs...
December 1, 2016: Haematologica
https://www.readbyqxmd.com/read/27896936/health-related-quality-of-life-and-adherence-to-hydroxyurea-in-adolescents-and-young-adults-with-sickle-cell-disease
#3
Sherif M Badawy, Alexis A Thompson, Jin-Shei Lai, Frank J Penedo, Karen Rychlik, Robert I Liem
BACKGROUND: Complications related to sickle cell disease (SCD) result in significant declines in health-related quality of life (HRQOL). While hydroxyurea reduces SCD complications, adherence remains suboptimal. The study's objectives were to assess the feasibility of Internet-based electronic assessment of HRQOL in SCD clinic and to examine the relationship between HRQOL and hydroxyurea adherence in adolescents and young adults (AYAs) with SCD. PROCEDURE: A cross-sectional survey was administered on tablets to 34 AYAs (12-22 years old) in a SCD clinic from January through December 2015...
November 28, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27872735/fetal-hemoglobin-modifies-the-disease-manifestation-of-severe-plasmodium-falciparum-malaria-in-adult-patients-with-sickle-cell-anemia
#4
Prasanta Purohit, Siris Patel, Pradeep Kumar Mohanty, Padmalaya Das, Jogeswar Panigrahi
No abstract text is available yet for this article.
2016: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/27872702/gamma-reactivation-using-the-spongy-effect-of-klf1-binding-site-sequence-an-approach-in-gene-therapy-for-beta-thalassemia
#5
Nasrin Heydari, Laleh Shariati, Hossein Khanahmad, Zahra Hejazi, Mansoureh Shahbazi, Mansoor Salehi
OBJECTIVES: β-thalassemia is one of the most common genetic disorders in the world. As one of the promising treatment strategies, fetal hemoglobin (Hb F) can be induced. The present study was an attempt to reactivate the γ-globin gene by introducing a gene construct containing KLF1 binding sites to the K562 cell line. MATERIALS AND METHODS: A plasmid containing a 192 bp sequence with two repeats of KLF1 binding sites on β-globin and BCL11A promoters was constructed and used to transfect the K562 cell line...
October 2016: Iranian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/27861570/hmga2-moderately-increases-fetal-hemoglobin-expression-in-human-adult-erythroblasts
#6
Jaira F de Vasconcellos, Y Terry Lee, Colleen Byrnes, Laxminath Tumburu, Antoinette Rabel, Jeffery L Miller
Induction of fetal hemoglobin (HbF) has therapeutic importance for patients with beta-hemoglobin disorders. Previous studies showed that let-7 microRNAs (miRNAs) are highly regulated in erythroid cells during the fetal-to-adult developmental transition, and that targeting let-7 mediated the up-regulation of HbF to greater than 30% of the total globin levels in human adult cultured erythroblasts. HMGA2 is a member of the high-mobility group A family of proteins and a validated target of the let-7 family of miRNAs...
2016: PloS One
https://www.readbyqxmd.com/read/27855647/albuminuria-serum-antioxidant-enzyme-levels-and-markers-of-hemolysis-and-inflammation-in-steady-state-children-with-sickle-cell-anemia
#7
Karen E Itokua, Jean Robert Makulo, François B Lepira, Michel N Aloni, Pépé M Ekulu, Ernest K Sumaili, Justine B Bukabau, Vieux M Mokoli, Augustin L Longo, François M Kajingulu, Chantal V Zinga, Yannick M Nlandu, Yannick M Engole, Pierre Z Akilimali, René M Ngiyulu, Jean Lambert Gini, Nazaire M Nseka
BACKGROUND: Oxidative stress is thought to be involved in the pathogenesis of microalbuminuria in Sickle cell anemia (SCA). Antioxidant enzymes such as glutathione peroxidase (GPx) and Cu-Zn superoxide dismutase (SOD) may play an important protective role. This study aimed to evaluate the association between albuminuria and these two antioxidant enzymes. METHODS: We consecutively recruited Steady state children aged between 2 and 18 years old with established diagnosis of homozygous SCA in two hospitals of Kinshasa/DR Congo...
November 17, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27855048/-relationship-between-the-level-of-maternal-glycated-hemoglobin-and-fetal-hypertrophic-cardiomyopathy
#8
Karla Lucía Sánchez-Martínez, Luis Fernando Oseguera-Torres, Joel Ávalos-Nuño
BACKGROUND: Diabetes mellitus is a metabolic disorder commonly associated with pregnancy. It is shown that as the management of diabetes during pregnancy is optimized, decreases the frequency and severity of fetal and neonatal complications. The aim of this paper is to identify the relation between the level of maternal glycated hemoglobin and fetal hypertrophic cardiomyopathy. METHODS: An analitytic cross-sectional study was conducted. It included patients with single pregnancy of 28-37 weeks diagnosed with gestational or preconception diabetes mellitus...
2016: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/27826131/preconception-care-education-for-women-with-diabetes-a-systematic-review-of-conventional-and-digital-health-interventions
#9
REVIEW
Chidiebere Hope Nwolise, Nicola Carey, Jill Shawe
BACKGROUND: Worldwide, 199.5 million women have diabetes mellitus (DM). Preconception care (PCC) education starting from adolescence has been recommended as an effective strategy for safeguarding maternal and child health. However, traditional preconception care advice provided by health care professionals (HCPs) within clinic settings is hindered by inadequate resources, suboptimal coverage, and busy clinics. Electronic health (eHealth), which is instrumental in solving problems around scarce health resources, could be of value in overcoming these limitations and be used to improve preconception care and pregnancy outcomes for women with DM...
November 8, 2016: Journal of Medical Internet Research
https://www.readbyqxmd.com/read/27821015/mutation-in-a-highly-conserved-cooh-terminal-residue-of-kr%C3%A3-ppel-like-factor-1-associated-with-elevated-hb-f-in-a-compound-heterozygous-%C3%AE-thalassemia-patient-with-a-nontransfusion-dependent-thalassemia-phenotype
#10
Patrick G Gallagher, Yelena Maksimova, Vincent P Schulz, Bernard G Forget
We present a patient with a compound heterozygosity codon 39 (C > T) (β(0)) [or β39(C5)Gln→Stop (G39X); CAG > TAG; HBB: c.118C > T] and -87 (C > T) (β(+)) (HBB: c.-137C > T) β-globin mutations, a non transfusion-dependent thalassemia phenotype and 97.0% fetal hemoglobin. A novel heterozygous mutation was identified in a highly conserved residue in the COOH-terminus of the Krüppel-like factor 1, R360H, that likely altered DNA-binding and impaired transactivation.
September 2016: Hemoglobin
https://www.readbyqxmd.com/read/27812519/in-depth-medical-nutrition-therapy-for-a-woman-with-diabetes-from-pregnancy-to-delivery
#11
Miyoung Jang, Dal Lae Ju, MeeRa Kweon, Misun Park
Diabetes in pregnancy is associated with higher rates of miscarriage, pre-eclampsia, preterm labor, and fetal malformation. To prevent these obstetric and perinatal complications, women with diabetes have to control levels of blood sugar, both prior to and during pregnancy. Thus, individualized medical nutrition therapy for each stage of pregnancy is essential. We provided in-depth medical nutrition therapy to a 38-year-old pregnant woman with diabetes at all stages of pregnancy up to delivery. She underwent radiation therapy after surgery for breast cancer and was diagnosed with diabetes...
October 2016: Clinical Nutrition Research
https://www.readbyqxmd.com/read/27806557/the-effect-of-hemoglobin-correction-by-blood-transfusion-on-pregnancy-outcomes-in-minor-and-intermedia-thalassemia-patients-a-single-blind-controlled-randomized-clinical-trial
#12
Mahin Najafian, Ahmad Ahmadzadeh, Mojgan Barati, Mahin Bahadori, Zeinab Shajirat
To the best of our knowledge, there is no prospective trial study assessing the management of β-thalassemia by blood transfusion in pregnancy. The aim of this study was to investigate the effect of blood transfusion on maternal and neonatal outcomes in pregnant patients with beta thalassemia minor and intermedia. We did this randomized, single -blind, controlled clinical trial on 36 pregnant women with β thalassemia minor and intermedia at two tertiary hospitals of Imam Khomeini and Shafa in Ahvaz, Iran from January 2016 to July 2016...
August 26, 2016: Global Journal of Health Science
https://www.readbyqxmd.com/read/27801605/fetal-hemoglobin-regulation-in-%C3%AE-thalassemia-heterogeneity-modifiers-and-therapeutic-approaches
#13
Orapan Sripichai, Suthat Fucharoen
Stress erythropoiesis induces fetal hemoglobin (HbF) expression in β-thalassemias, however the level of expression is highly variable. The last decade has seen dramatic advances in our understanding of the molecular regulators of HbF production and the genetic factors associated with HbF levels, leading to the promise of new methods of the clinical induction of HbF. Areas covered: This article will review the heterogeneity and genetic modifiers of HbF and HbF induction therapy in β-thalassemia. Expert commentary: One promising curative β-thalassemia therapy is to induce HbF synthesis in β-thalassemic erythrocytes to therapeutic levels before clinical symptom occurs...
December 2016: Expert Review of Hematology
https://www.readbyqxmd.com/read/27789175/-juvenile-myelomonocytic-leukemia-a-three-case-series
#14
I Ghariani, N Jmili-Braham, H Regaieg, B Achour, Y Ben Youssef, H Sendi, L Bakir, M Kortas
Juvenile myelomonocytic leukemia (JMML), previously known as juvenile chronic myeloid leukemia (JCML), is a rare, myelodysplastic-myeloproliferative disease typically presenting in early childhood. This disorder is difficult to distinguish from other myeloproliferative syndromes such as chronic myeloid leukemia (CML) because of the similarities in their clinical and bone marrow findings. However, because of its unique biological characteristics such as absolute monocytosis with dysplasia, absence of Philadelphia chromosome or BCR-ABL fusion protein, hypergammaglobulinemia, and raised fetal hemoglobin level, this disorder does not satisfy the criteria for inclusion in the CML or chronic myelomonocytic leukemia (CMML) group, as seen in adult patients...
October 24, 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/27767389/correlation-of-sin3a-genomic-variants-with-%C3%AE-hemoglobinopathies-disease-severity-and-hydroxyurea-treatment-efficacy
#15
Aikaterini Gravia, Vasiliki Chondrou, Alexandra Kolliopoulou, Alexandra Kourakli, Anne John, Argyris Symeonidis, Bassam R Ali, Argyro Sgourou, Adamantia Papachatzopoulou, Theodora Katsila, George P Patrinos
AIMS: Hemoglobinopathies, particularly β-thalassemia and sickle cell disease, are characterized by great phenotypic variability in terms of disease severity, while notable differences have been observed in hydroxyurea treatment efficacy. In both cases, the observed phenotypic diversity is mostly dependent on the elevated fetal hemoglobin levels, resulting from the persistent fetal globin gene expression in the adult erythroid stage orchestrated by intricate mechanisms that still remain only partly understood...
October 21, 2016: Pharmacogenomics
https://www.readbyqxmd.com/read/27761979/maternal-systemic-or-cord-blood-inflammation-is-associated-with-birth-anthropometry-in-a-tanzanian-prospective-cohort
#16
A L Wilkinson, S H Pedersen, M Urassa, D Michael, A Andreasen, J Todd, S M Kinung'hi, J Changalucha, J M McDermid
OBJECTIVES: HIV infection is associated with chronic systemic inflammation, with or without antiretroviral therapy. Consequences for fetal growth are not understood, particularly in settings where multiple maternal infections and malnutrition are common. The study was designed to examine maternal systemic circulating and umbilical cord blood cytokine concentrations in relation to birth anthropometry in a Tanzanian prospective cohort. METHODS: A 9-plex panel of maternal plasma cytokines in HIV-positive (n=44) and HIV-negative (n=70) mothers and the same cytokines in umbilical cord blood collected at delivery was assayed...
October 20, 2016: Tropical Medicine & International Health: TM & IH
https://www.readbyqxmd.com/read/27754490/activation-of-fetal-%C3%AE-globin-gene-expression-via-direct-protein-delivery-of-synthetic-zinc-finger-dna-binding-domains
#17
Mir A Hossain, Yong Shen, Isaac Knudson, Shaleen Thakur, Jared R Stees, Yi Qiu, Betty S Pace, Kenneth R Peterson, Jörg Bungert
Reactivation of γ-globin expression has been shown to ameliorate disease phenotypes associated with mutations in the adult β-globin gene, including sickle cell disease. Specific mutations in the promoter of the γ-globin genes are known to prevent repression of the genes in the adult and thus lead to hereditary persistence of fetal hemoglobin. One such hereditary persistence of fetal hemoglobin is associated with a sequence located 567 bp upstream of the Gγ-globin gene which assembles a GATA-containing repressor complex...
October 18, 2016: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/27746004/the-use-of-semi-quantitative-tests-at-cesarean-section-delivery-for-the-differentiation-of-canine-fetal-fluids-from-maternal-urine-on-the-basis-of-biochemical-characteristics
#18
Orsolya Balogh, Marie Roch, Stefanie Keller, Erika Michel, Iris M Reichler
In dogs, there is no diagnostic test to identify and differentiate fetal fluids from maternal urine in the event that a clear-yellowish vulvar discharge is observed pre-whelping. The objective of this study was to find a test that could easily and accurately identify rupture of the fetal membranes preceding parturition. Maternal urine, and amniotic fluid (AMF) and allantoic fluid (ALF) from only one fetus per bitch, were collected intraoperatively during Cesarean section. Specific gravity (SG) was analyzed with a refractometer, whereas the presence of leukocytes, protein, glucose, ketones, bilirubin, urobilinogen, nitrite, erythrocyte/hemoglobin (Hb), and the pH were assessed using a urine dipstick (Combur-Test(®))...
January 15, 2017: Theriogenology
https://www.readbyqxmd.com/read/27724923/effects-of-probiotics-vivomixx%C3%A2-in-obese-pregnant-women-and-their-newborn-study-protocol-for-a-randomized-controlled-trial
#19
Sofie Ingdam Halkjaer, Lisbeth Nilas, Emma Malchau Carlsen, Dina Cortes, Thórhallur Ingi Halldórsson, Sjúrdur Frodi Olsen, Anders Elm Pedersen, Karen Angeliki Krogfelt, Andreas Munk Petersen
BACKGROUND: Maternal obesity is associated with increased risks of adverse pregnancy-related complications and outcomes for both mothers and infants. Overweight and obese women have an increased risk of pregnancy-induced hypertension, preeclampsia and gestational diabetes mellitus (GDM). Infant Body Mass index (BMI) and the risk of obesity in adulthood are related to maternal gestational weight gain (GWG). Preventive lifestyle and dietary interventions are time-consuming and do not always reduce GWG or the risk of maternal pregnancy complications...
October 11, 2016: Trials
https://www.readbyqxmd.com/read/27708211/can-mutations-in-the-gene-encoding-transcription-factor-eklf-erythroid-kr%C3%A3-ppel-like-factor-protect-us-against-infectious-and-parasitic-diseases
#20
Krzysztof Mikołajczyk, Radosław Kaczmarek, Marcin Czerwiński
Transcription factor EKLF (Erythroid Krüppel-Like Factor) belongs to the group of Krüppellike factors, which regulate proliferation, differentiation, development and apoptosis of mammalian cells. EKLF factor is present in erythroid cells, where it participates in regulation of hematopoiesis, expression of genes encoding transmembrane proteins (including blood group antigens), and heme biosynthesis enzymes. It is also a key factor in downregulation of γ-globins and activation of β-globin gene expression...
October 6, 2016: Postȩpy Higieny i Medycyny Doświadczalnej
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