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Fetal Hemoglobin

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https://www.readbyqxmd.com/read/29159826/pharmacological-and-molecular-approaches-for-the-treatment-of-%C3%AE-hemoglobin-disorders
#1
REVIEW
Neelam Lohani, Nupur Bhargava, Anjana Munshi, Sivaprakash Ramalingam
β-hemoglobin disorders, such as β-thalassemia and sickle cell anemia are among the most prevalent inherited genetic disorders worldwide. These disorders are caused by mutations in the gene encoding hemoglobin-β (HBB), a vital protein found in red blood cells (RBCs) that carries oxygen from lungs to all parts of the human body. As a consequence, there has been an enduring interest in this field in formulating therapeutic strategies for the treatment of these diseases. Currently, there is no cure available for hemoglobin disorders, although, some patients have been treated with bone marrow transplantation, whose scope is limited because of the difficulty in finding a histocompatible donor and also due to transplant-associated clinical complications that can arise during the treatment...
November 20, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29156994/experimental-diabetes-impairs-maternal-reproductive-performance-in-pregnant-wistar-rats-and-their-offspring
#2
Leticia Bequer, Tahiry Gómez, José L Molina, Alain Álvarez, Claudia Chaviano, Sonia Clapés
The aim of this study was to determine the effect of mild hyperglycemia on metabolism during pregnancy, the maternal reproductive performance, and the characteristics of the offspring in neonatal mild diabetic-induced Wistar rats. The experimental diabetes model was generated by neonatal streptozotocin administration (100 mg of streptozotocin/Kg bw/sc) in female Wistar rats. At adulthood, the control and diabetic group were mated. At the 20th day of gestation, a maternal and fetal blood sample were collected for biochemical measurement...
November 20, 2017: Systems Biology in Reproductive Medicine
https://www.readbyqxmd.com/read/29138844/the-%C3%A2-%C3%AE-3-7-deletion-in-%C3%AE-%C3%A2-globin-genes-increases-the-concentration-of-fetal-hemoglobin-and-hemoglobin-a2-in-a-saudi-arabian-population
#3
J Francis Borgio, Sayed Abdulazeez, Noor B Almandil, Zaki A Naserullah, Sana Al-Jarrash, Ahmed M Al-Suliman, Huda Ismail Elfakharay, Fuad S Qaw, Fatimah I Alabdrabalnabi, Mohammed A Alkhalifah, Mohammed Shakil Akhtar, Hatem Qutub, Amein K Al-Ali
The regions of Al‑Qatif and Al‑Ahssa in the Eastern Province of Saudi Arabia are known for their high prevalence of hemoglobinopathies, including β‑thalassemia and sickle cell anemia. Previously, the α‑gene deletion has been demonstrated as highly prevalent among populations residing in these two regions. The present study was conducted in order to investigate the implications of the α‑globin gene deletion on fetal hemoglobin (HbF) and hemoglobin α2 (HbA2) concentrations in patients with transfusion‑dependent β‑thalassemia...
November 13, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29127681/reactivation-of-fetal-hemoglobin-for-treating-%C3%AE-thalassemia-and-sickle-cell-disease
#4
Shuaiying Cui, James Douglas Engel
Reactivation of fetal hemoglobin (HbF) in adult hematopoietic cells has the potential for great clinical benefit in patients bearing deleterious mutations in the β-globin gene, such as β-thalassemia and sickle cell disease (SCD), since increasing the production of HbF can compensate for underproduction of β-globin chains (in β-thalassemia) and it can also disrupt sickle hemoglobin polymerization (in SCD). Thus for the past few decades, concerted efforts have been made to identify an effective way to induce the synthesis of HbF in adult erythroid cells for potential therapeutic relief from the effects of these β-globinopathies...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29127676/genetic-basis-and-genetic-modifiers-of-%C3%AE-thalassemia-and-sickle-cell-disease
#5
Swee Lay Thein
β-thalassemia and sickle cell disease (SCD) are prototypical Mendelian single gene disorders, both caused by mutations affecting the adult β-globin gene. Despite the apparent genetic simplicity, both disorders display a remarkable spectrum of phenotypic severity and share two major genetic modifiers-α-globin genotype and innate ability to produce fetal hemoglobin (HbF, α2γ2).This article provides an overview of the genetic basis for SCD and β-thalassemia, and genetic modifiers identified through phenotype correlation studies...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29124822/advanced-cell-culture-models-for-diamond-blackfan-anemia-and-other-erythroid-disorders
#6
REVIEW
Anna Rita Migliaccio, Lilian Varricchio
In vitro surrogate models of human erythropoiesis made many contributions to our understanding of the extrinsic and intrinsic regulation of this process in vivo and how they are altered in erythroid disorders. In the past, variability among the levels of hemoglobin F produced by adult erythroblasts generated in vitro by different laboratories identified stage of maturation, fetal bovine serum and accessory cells as "confounding factors", i.e. parameters intrinsically wired in the experimental approach that bias the results observed...
November 10, 2017: Stem Cells
https://www.readbyqxmd.com/read/29108926/a-thalidomide-hydroxyurea-hybrid-increases-hbf-production-in-sickle-cell-mice-and-reduces-the-release-of-proinflammatory-cytokines-in-cultured-monocytes
#7
Carolina Lanaro, Carla Franco-Penteado, Fabio H Silva, Kleber Y Fertrin, Jean Leandro Dos Santos, Marlene Wade, Shobha Yerigenahally, Thais R de Melo, Chung Man Chin, Abdullah Kutlar, Steffen E Meiler, Fernando Ferreira Costa
Fetal hemoglobin induction by hydroxyurea therapy is associated with decreased morbidity and mortality in SCA patients, but not all respond to or tolerate hydroxyurea, which provides a rationale for the development of novel HbF inducers as treatment options for SCA. Thalidomide analogs have a combined ability to induce HbF production and to inhibit the release of tumor necrosis factor α. Molecular hybridization of hydroxyurea and thalidomide was used to synthesize 3-(1,3-dioxoisoindolin-2-yl) benzyl nitrate (compound 4C)...
November 3, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/29107441/real-life-experience-with-hydroxyurea-in-sickle-cell-disease-a-multicenter-study-in-a-cohort-of-patients-with-heterogeneous-descent
#8
Paolo Rigano, Lucia De Franceschi, Laura Sainati, Antonio Piga, Frédéric B Piel, Maria Domenica Cappellini, Carmelo Fidone, Nicoletta Masera, Giovanni Palazzi, Barbara Gianesin, Gian Luca Forni
We conducted the first nation-wide cohort study of sickle cell disease (SCD) in Italy, a Southern European country exposed to intense recent flux migration from endemic areas for SCD. We evaluate the impact of hydroxyurea on a total of 652 pediatric and adult patients from 33 Reference Centers for SCD (mean age 24.5±15years, 51.4% males). Hydroxyurea median treatment duration was 7years (range: <1year to 29years) at a mean therapeutic dose of 18±4.7mg/kg/day. Hydroxyurea was associated with a significant increase in mean total and fetal hemoglobin and a significant decrease in mean hemoglobin S, white blood and platelet counts, and lactate dehydrogenase levels...
October 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29080249/concise-review-epigenetic-regulation-of-hematopoiesis-biological-insights-and-therapeutic-applications
#9
REVIEW
Chiara Antoniani, Oriana Romano, Annarita Miccio
Hematopoiesis is the process of blood cell formation starting from hematopoietic stem/progenitor cells (HSPCs). The understanding of regulatory networks involved in hematopoiesis and their impact on gene expression is crucial to decipher the molecular mechanisms that control hematopoietic development in physiological and pathological conditions, and to develop novel therapeutic strategies. An increasing number of epigenetic studies aim at defining, on a genome-wide scale, the cis-regulatory sequences (e.g., promoters and enhancers) used by human HSPCs and their lineage-restricted progeny at different stages of development...
October 28, 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/29079125/hereditary-persistence-of-hemoglobin-f-is-protective-against-red-cell-sickling-a-case-report-and-brief-review
#10
Alexandra Sokolova, Anton Mararenko, Alexander Rozin, Alida Podrumar, Vladimir Gotlieb
Fetal hemoglobin (HbF) is a physiologic protein tetramer that is crucial for a developing fetus to survive in utero. Maternal hemoglobin has a relatively lower affinity for oxygen, and thus allows for an efficient transfer of oxygen from maternal to fetal blood. In addition to fulfilling a critical physiologic role, HbF is also known to alleviate symptoms of sickle-cell disease (SCD). The concentration of HbF depends on several factors. HbF is elevated in inherited conditions, such as hereditary persistence of HbF, hereditary spherocytosis, and thalassemia...
October 16, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/29076087/functional-analysis-of-erythroid-progenitors-by-colony-forming-assays
#11
James Palis, Anne Koniski
The capacity of erythroid-lineage progenitors to form colonies of maturing red blood cells in semisolid media has provided a functional assay for these progenitors and has greatly contributed to our understanding of erythropoiesis. Studies since the 1970s have led to the development of a model of the erythron, whereby the earliest erythroid-committed progenitor, the immature burst-forming unit erythroid (BFU-E), gives rise sequentially to late-stage BFU-E and to colony-forming units erythroid (CFU-E). CFU-E give rise, in turn, to maturing erythroblast precursors that hemoglobinize...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29076081/an-introduction-to-erythropoiesis-approaches
#12
Joyce A Lloyd
Many experimental models have been used to study erythropoiesis. Even prior to the advent of the genetic manipulation of animal models, erythropoiesis was examined in the mouse, chicken, sheep, goat, and rabbit, among other vertebrates. Erythroid cell lines derived from human blood cancers were also very useful, as they could be genetically manipulated more easily than whole animals. Genetic models in the mouse, zebrafish, and frog have provided a plethora of information advancing our understanding of erythropoiesis, and remain gold standards in the field for studies of hemoglobin switching, and experiments to study authentic blood cell development...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29070547/emerging-understanding-and-measurement-of-plasma-volume-expansion-in-pregnancy
#13
REVIEW
Laura K Vricella
Plasma volume expansion is an important component of a successful pregnancy. The failure of maternal plasma volume expansion has been implicated in adverse obstetric outcomes such as pre-eclampsia, fetal growth restriction, and preterm birth. Altered iron homeostasis and elevated maternal hemoglobin concentrations have also been associated with adverse pregnancy outcomes; limited data have suggested that these effects may be mediated by inadequate plasma volume expansion. In addition, it has been noted that pregnant, obese women, compared with lean subjects, have decreased plasma volume expansion along with impaired iron homeostasis and increased inflammation...
October 25, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29067594/genetic-variation-of-kr%C3%A3-ppel-like-factor-1-klf1-and-fetal-hemoglobin-hbf-levels-in-%C3%AE-0-thalassemia-hbe-disease
#14
Pinyaphat Khamphikham, Orapan Sripichai, Thongperm Munkongdee, Suthat Fucharoen, Sissades Tongsima, Duncan R Smith
Heterogeneity of HbF levels in β(0)-thalassemia/HbE disease has been reported to be associated with variations in clinical manifestations of the disease, and several genetic-modifying factors beyond the β-globin gene cluster have been identified as HbF regulators. Down-regulation or heterozygous mutations of Krüppel-like factor 1 (KLF1) is associated with elevated HbF levels in non-thalassemia subjects. This study confirms that experimental down-regulation of KLF1 in β(0)-thalassemia/HbE-derived erythroblasts significantly increases HbF production (up to 52...
October 24, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29065736/investigation-of-possible-maternal-and-fetal-factors-which-affect-umbilical-coiling-index
#15
Muberra Namli Kalem, Ziya Kalem, Nilufer Akgun, Ebru Yuce, Hatice Aktas
AIM: The aim of this study was to investigate the possible maternal and fetal factors which affect the Umbilical Coiling Index (UCI). METHODS: This prospective, observational, analytic study was conducted using the data of 380 women with term pregnancy and newborns who presented at a University Hospital. Hemoglobin (Hb), ferritin, iron, and the total iron binding capacity (TIBC) of the maternal blood were measured, and transferrin saturation was estimated based on the ratio between serum iron and TIBC...
October 24, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29061162/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-%C3%AE-type-hemoglobinopathy-patients
#16
Vasiliki Chondrou, Petros Kolovos, Argyro Sgourou, Alexandra Kourakli, Alexia Pavlidaki, Vlasia Kastrinou, Anne John, Argiris Symeonidis, Bassam R Ali, Adamantia Papachatzopoulou, Theodora Katsila, George P Patrinos
BACKGROUND: Human 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...
October 23, 2017: Human Genomics
https://www.readbyqxmd.com/read/29054042/spontaneous-hemoperitoneum-in-pregnancy-ship-and-endometriosis-a-systematic-review-of-the-recent-literature
#17
REVIEW
Marit C I Lier, Romana F Malik, Johannes C F Ket, Cornelis B Lambalk, Ivo A Brosens, Velja Mijatovic
Spontaneous Hemoperitoneum in Pregnancy (SHiP), an unprovoked (nontraumatic) intraperitoneal bleeding in pregnancy (up to 42days postpartum), is associated with serious adverse pregnancy outcomes. To evaluate the clinical consequences of SHiP and its association with endometriosis, a systematic review was conducted according to the PRISMA guidelines. PubMed, Embase.com and Thomson Reuters/Web of Science were searched for articles published since the latest review (August 2008) until September 2016. After assessment for eligibility, forty-four articles were included in this systematic review, describing 59 cases of SHiP...
October 12, 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/29051184/novel-use-of-hydroxyurea-in-an-african-region-with-malaria-noharm-a-trial-for-children-with-sickle-cell-anemia
#18
Robert O Opoka, Christopher M Ndugwa, Teresa S Latham, Adam Lane, Heather A Hume, Phillip Kasirye, James S Hodges, Russell E Ware, Chandy C John
Hydroxyurea treatment is recommended for children with sickle cell anemia (SCA) living in high-resource malaria-free regions, but its safety and efficacy in malaria-endemic sub-Saharan Africa, where the greatest sickle cell burden exists, remain unknown. In vitro studies suggest hydroxyurea could increase malaria severity, and hydroxyurea-associated neutropenia could worsen infections. NOHARM was a randomized, double-blinded, placebo-controlled trial conducted in malaria-endemic Uganda, comparing hydroxyurea to placebo at 20 ± 2...
October 19, 2017: Blood
https://www.readbyqxmd.com/read/29049312/impact-of-annotation-error-in-%C3%AE-globin-genes-on-molecular-diagnosis
#19
J Francis Borgio
BACKGROUND: Recent studies on the variants in duplicated human alpha globin genes (HBA2 and HBA1) actively target the α-globin gene as molecular modulators for the treatment of β-thalassemia major. Identification of the exact position of variant in HBA1, HBA2 or its patchworks is mandatory to support the therapeutic aims in β-thalassemia major, by identifying specific modulators for the reactivation of fetal hemoglobin production. Hence, accurate identification of the variants in α-globin genes is crucial for the proper diagnosis, treatment and genetic counseling...
2017: PloS One
https://www.readbyqxmd.com/read/29046485/dimethyl-fumarate-increases-fetal-hemoglobin-provides-heme-detoxification-and-corrects-anemia-in-sickle-cell-disease
#20
Sriram Krishnamoorthy, Betty Pace, Dipti Gupta, Sarah Sturtevant, Biaoru Li, Levi Makala, Julia Brittain, Nancy Moore, Benjamin F Vieira, Timothy Thullen, Ivan Stone, Huo Li, William E Hobbs, David R Light
Sickle cell disease (SCD) results from a point mutation in the β-globin gene forming hemoglobin S (HbS), which polymerizes in deoxygenated erythrocytes, triggering recurrent painful vaso-occlusive crises and chronic hemolytic anemia. Reactivation of fetal Hb (HbF) expression ameliorates these symptoms of SCD. Nuclear factor (erythroid derived-2)-like 2 (Nrf2) is a transcription factor that triggers cytoprotective and antioxidant pathways to limit oxidative damage and inflammation and increases HbF synthesis in CD34+ stem cell-derived erythroid progenitors...
October 19, 2017: JCI Insight
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