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Fetal Hemoglobin

Zbynĕk Straňák, Simona Feyereislová, Peter Korček, Eugene Dempsey
Postnatal adaptation in preterm newborn comprises complex physiological processes that involve significant changes in the circulatory and respiratory system. Increasing hemoglobin level and blood volume following placental transfusion may be of importance in enhancing arterial oxygen content, increasing cardiac output, and improving oxygen delivery. The European consensus on resuscitation of preterm infants recommends delayed cord clamping (DCC) for at least 60 s to promote placenta-fetal transfusion in uncompromised neonates...
2018: Frontiers in Pediatrics
Ulrik Dolberg Anderson, Maya Jälmby, Marijke M Faas, Stefan R Hansson
OBJECTIVE: The aim of this study was to investigate how maternal cell-free fetal hemoglobin and heme impact the scavenger enzyme systems Hemopexin and Heme Oxygenase-1 in patients with preeclampsia (PE). The secondary aims were to evaluate these proteins as biomarkers for severity of the clinical manifestation i.e. hypertension, in early- and late onset PE. MATERIAL AND METHODS: Plasma samples taken within the last 24 h before delivery from 135 patients were analyzed, 89 PE and 46 normal pregnancies...
February 15, 2018: Pregnancy Hypertension
Andrea Tesija Kuna, Kristina Dukic, Nora Nikolac Gabaj, Marijana Miler, Ines Vukasovic, Sanja Langer, Ana-Maria Simundic, Nada Vrkic
Objective: To compare the analytical performances of the enzymatic method (EM) and capillary electrophoresis (CE) for hemoglobin A1c (HbA1c) measurement. Methods: Imprecision, carryover, stability, linearity, method comparison, and interferences were evaluated for HbA1c via EM (Abbott Laboratories, Inc) and CE (Sebia). Results: Both methods have shown overall within-laboratory imprecision of less than 3% for International Federation of Clinical Chemistry and Laboratory Medicine (IFCC) units (<2% National Glycohemoglobin Standardization Program [NGSP] units)...
March 8, 2018: Laboratory Medicine
Olga Ulyanova, Abay Baigenzhin, Zhaksylyk Doskaliyev, Temirlan Karibekov, Larissa Kozina, Samat Saparbayev, Rakhat Trimova
OBJECTIVES: Our objective was to determine transforming growth factor β1 levels in patients with type 2 diabetes mellitus after fetal pancreatic stem cell transplant. MATERIALS AND METHODS: We examined 10 patients (age range, 41-65 y) with type 2 diabetes mellitus, which we subsequently divided into 2 groups. Group 1 comprised 5 patients who received fetal pancreatic stem cell transplant (cells were 16-18 wk gestation) performed by intravenous infusion. Group 2 comprised 5 patients (control group) who were on hypoglycemic tablet therapy or insulin therapy...
March 2018: Experimental and Clinical Transplantation
Chiara Antoniani, Vasco Meneghini, Annalisa Lattanzi, Tristan Felix, Oriana Romano, Elisa Magrin, Leslie Weber, Giulia Pavani, Sara El Hoss, Ryo Kurita, Yukio Nakamura, Thomas J Cradick, Ante S Lundberg, Matthew Porteus, Mario Amendola, Wassim El Nemer, Marina Cavazzana, Fulvio Mavilio, Annarita Miccio
Naturally occurring, large deletions in the β-globin locus result in hereditary persistence of fetal hemoglobin, a condition that mitigates the clinical severity of sickle-cell disease (SCD) and β-thalassemia. We designed a CRISPR/Cas9 strategy to disrupt a 13.6-kb genomic region encompassing the δ- and β-globin genes and a putative γ-δ intergenic fetal hemoglobin (HbF) silencer. Disruption of just the putative HbF silencer results in a mild increase in γ-globin expression, whereas deletion or inversion of a 13...
March 8, 2018: Blood
Vyacheslav Buko, Ilya Zavodnik, Olena Kanuka, Elena Belonovskaya, Elena Naruta, Oxana Lukivskaya, Siargej Kirko, Grazyna Budryn, Danuta Żyżelewicz, Joanna Oracz, Natalia Sybirna
The protective effect of red cabbage extract (RCE) was evaluated in rats with streptozotocin-induced diabetes, assessing a probable role of this extract in the prevention of erythrocyte impairments associated with a high risk of vascular complications in diabetes. RCE was analyzed by ultrahigh performance liquid chromatography and mass spectrometry, and 11 anthocyanins, 3 hydroxybenzoic acids and 9 hydroxycinnamic acids were identified. Type 1 diabetes was induced by streptozotocin (60 mg kg-1 ) in Wistar male rats (n = 8 per group)...
March 8, 2018: Food & Function
Thanh-Vy Phung, Véronique Houfflin-Debarge, Nassima Ramdane, Louise Ghesquière, Anne Delsalle, Capucine Coulon, Damien Subtil, Pascal Vaast, Charles Garabedian
BACKGROUND: The antibody primarily responsible for fetal anemia may influence treatment and prognosis. The primary objective was to compare ante- and postnatal management and the outcomes of maternal red blood cell (RBC) alloimmunizations according to the antibody involved. The secondary objective was to compare anti-D alloimmunizations according to associated number of antibodies. STUDY DESIGN AND METHODS: A single-center study from 1999 to 2015 including maternal RBC alloimmunizations requiring intrauterine transfusion (IUT) was conducted...
March 6, 2018: Transfusion
Lan Liu, Wenjuan Jia, Ruike Liu, Jie Shen
BACKGROUND: Studies reported that fulminant type 1 diabetes (fT1DM) can occurred during pregnancy or within 2 weeks after delivery, and was defined as pregnancy-associated fulminant type 1 diabetes (PF). In PF patients, plasma glucose (PG) levels have an abrupt rise while glycated hemoglobin (HbA1C ) levels are not markedly elevated, resulting in a sharply increased PG/HbA1C ratio. METHODS: We studied 30 PF patients, 21 non-pregnant fulminant type 1 diabetes (NPF) patients, and 26 female patients of child-bearing age (13-49 years) with diabetic ketoacidosis (DKA), all from China...
March 2, 2018: Endocrine
Rosario Di Maggio, Matthew M Hsieh, Xiongce Zhao, Giuseppina Calvaruso, Paolo Rigano, Disma Renda, John F Tisdale, Aurelio Maggio
In sickle cell disease (SCD), hydroxyurea (HU) treatment decreases the number of vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) by increasing fetal hemoglobin (HbF). Data are lacking regarding the frequency of HU dose modification or whether sub-therapeutic doses (<15 mg/kg/day) are beneficial. We reviewed the medical records of 140 patients from 2010 to 2014. The laboratory parameters and SCD complications were compared between the first and last visits based on HU use. Fifty patients (36%) never took HU or suspended HU ("no HU" group)...
February 28, 2018: International Journal of Molecular Sciences
Sheng He, Jihui Li, Peng Huang, Shujie Zhang, Li Lin, Yangjin Zuo, Xiaoxian Tian, Chenguang Zheng, Xiaoxia Qiu, Biyan Chen
Hb Bart's hydrops fetalis is the most severe and generally fatal clinical phenotype of α-thalassemia (α-thal), which is due to the deletion of all four functional α-globin genes of hemoglobin (Hb), resulting in no α-globin chain production (- -/- -). Homozygosity for the - -SEA (Southeast Asian) α-globin gene deletion is the main cause of the Hb Bart's hydrops fetalis in Asia, especially South China. Occasionally, other α0 -thal deletions can also be found. In this study, we report a case with an atypical form of Hb Bart's hydrops fetalis that was caused by - -SEA and a large novel α0 -thal deletion (- -GX ) (Guangxi)...
March 1, 2018: Hemoglobin
O Kos, C Alexander, K Brandenburg, Z Chen, A Heini, D Heumann, I Khatri, J P Mach, E T Rietschel, A Tersikh, A J Ulmer, T Waelli, K Yu, U Zähringer, R M Gorczynski
We have shown that an altered tissue redox environment in mice lacking either murine beta Hemoglobin major (Hgbβma KO) or minor (Hgbβmi KO) regulates inflammation. The REDOX environment in marrow stem cell niches also control differentiation pathways. We investigated osteoclastogenesis (OC)/osteoblastogenesis (OB), in bone cultures derived from untreated or FSLE-treated WT, Hgbβma KO or Hgbβmi KO mice. Marrow mesenchymal cells from 10d pre-cultures were incubated on an osteogenic matrix for 21d prior to analysis of inflammatory cytokine release into culture supernatants, and relative OC:OB using (TRAP:BSP, RANKL:OPG) mRNA expression ratios and TRAP or Von Kossa staining...
February 22, 2018: International Immunopharmacology
Derek M Culnan, Beretta Craft-Coffman, Genevieve H Bitz, Karel D Capek, Yiji Tu, William C Lineaweaver, Maggie J Kuhlmann-Capek
Carbon monoxide (CO) is a small molecule poison released as a product of incomplete combustion. Carbon monoxide binds hemoglobin, reducing oxygen delivery. This effect is exacerbated in the burned pregnant patient by fetal hemoglobin that binds CO 2.5- to 3-fold stronger than maternal hemoglobin. With no signature clinical symptom, diagnosis depends on patient injury history, elevated carboxyhemoglobin levels, and alterations in mental status. The standard of care for treatment of CO intoxication is 100% normobaric oxygen, which decreases the half-life of CO in the bloodstream from 5 hours to 1 hour...
March 2018: Annals of Plastic Surgery
Aumpika Kesornsit, Nutjaree Jeenduang, Dararat Horpet, Thunyaluk Plyduang, Manit Nuinoon
Variation of fetal hemoglobin (Hb F) expression in heterozygous Hb E (HBB: c.79G>A) individuals is associated with several genetic modifiers and not well understood. This study was undertaken in order to determine the effect of single nucleotide polymorphisms (SNPs), including XmnIG γ (rs7482144), rs766432 on the BCL11A gene and rs9376074 on the HBS1L gene, on Hb F levels in Southern Thai heterozygous Hb E individuals. A total of 97 Southern Thai subjects carrying heterozygous Hb E were selected for the hematological study...
February 19, 2018: Hemoglobin
Giordana Feriotto, Nicola Marchetti, Valentina Costa, Piera Torricelli, Simone Beninati, Federico Tagliati, Carlo Mischiati
Re-expression of fetal hemoglobin (HbF) was proposed as a possible therapeutic strategy for β-haemoglobinopathies. Although several inducers of HbF were tested in clinical trials, only hydroxyurea (HU) received FDA approval. Despite it produced adequate HbF levels only in half of HU-treated SCD patients, and was ineffective at all in β-thalassemia patients, beneficial effects of this approach suggested to continue in this direction identifying further molecules capable of inducing HbF. We tested the potential of essential oil isolated from Ocimum basilicum L...
February 13, 2018: Fitoterapia
Samuel Lessard, Mélissa Beaudoin, Stuart H Orkin, Daniel E Bauer, Guillaume Lettre
In humans, fetal erythropoiesis takes place in the liver whereas adult erythropoiesis occurs in the bone marrow. Fetal and adult erythroid cells are not only produced at different sites, but are also distinguished by their respective transcriptional program. In particular, whereas fetal erythroid cells express γ-globin chains to produce fetal hemoglobin (HbF), adult cells express β-globin chains to generate adult hemoglobin. Understanding the transcriptional regulation of the fetal-to-adult hemoglobin switch is clinically important as re-activation of HbF production in adult erythroid cells would represent a promising therapy for the hemoglobin disorders sickle cell disease and β-thalassemia...
February 8, 2018: Human Molecular Genetics
Jia-Wei Liu, Tao Hong, Xin Qin, Ying-Min Liang, Ping Zhang
β-hemoglobinopathies are one of six groups of common illnesses affecting human health. Although the genetic mechanisms have been elucidated for several decades, curable treatment options, other than allogeneic bone marrow transplantation, are still lacking. In recent years, rapid development in genome editing technologies and their clinical applications have opened up new directions for treatment of β-hemoglobinopathies. Genome editing technologies, as applied in autologous CD34+ hematopoietic stem and progenitor cells, represents a promising remedial means for the β-globin disorders...
February 20, 2018: Yi Chuan, Hereditas
Amihai Rottenstreich, Uriel Elchalal, Geffen Kleinstern, Nahum Beglaibter, Abed Khalaileh, Ram Elazary
OBJECTIVE: To examine associations of laparoscopic sleeve gastrectomy with maternal and perinatal outcomes. METHODS: We conducted a retrospective case-control study of deliveries that occurred from 2006 to 2016 at two university hospitals. The study group comprised all women who had undergone laparoscopic sleeve gastrectomy and who delivered during the study period. If a woman had more than one pregnancy during the study period, data from only her first pregnancy were used...
February 5, 2018: Obstetrics and Gynecology
Mohammad Hamid, Sanaz Ershadi Oskouei, Gholamreza Shariati, Esmaeil Babaei, Hamid Galehdari, Alihossein Saberi, Alireza Sedaghat
BACKGROUND: Any mutation in the Krüppel-like factor 1 (KLF1) gene may interfere with its proper related function in the erythropoiesis process and lead to alterations in proper activation of its downstream protein through globin switching, which results in an increase in fetal hemoglobin (HbF). This study aimed to investigate whether KLF1 mutation can associate with high level of HbF in individuals with increased fetal hemoglobin referred for screening of hemoglobinopathies in south of Iran...
February 7, 2018: Journal of Pediatric Hematology/oncology
Theera Tongsong, Fuanglada Tongprasert, Kasemsri Srisupundit, Suchaya Luewan, Kuntharee Traisrisilp, Phudit Jatavan
OBJECTIVE:  To assess fetal cardiac remodeling in response to anemia, by comparing the fetal cardiac dimensions and global sphericity index (GSI) of normal fetuses and fetuses with anemia using fetal Hb Bart's disease as a study model. METHODS:  Fetuses at risk for Hb Bart's disease undergoing cordocentesis at 18 to 22 weeks of gestation were recruited. Fetal cardiac dimensions including GSI (cardiac length to cardiac width ratio), interventricular septum thickness (IVST), left ventricular wall thickness (LVWT) and right ventricular wall thickness (RVWT) were measured...
February 7, 2018: Ultraschall in der Medizin
Muhsin Aydin, Eyyup Rencuzogullari, Ayse Dalyan, Suleyman Bayram, Ahmet Genc
In the present study, sixty-two samples that have 1.5% and upper level of fetal hemoglobin (HbF), were examined to investigate the relationship between HbF level and non-deletional mutations in both Gγ (G gamma) globin (HBG2) and Aγ (A gamma) globin (HBG1) genes. Four variations were observed in the promotor of Gγ gene, which are -158C/T, -309A/G, -369C/G, and -567T/G. Also, four variations were observed in the 5'-UTR (untranslated regions) and promotor of Aγ gene, which are +25G/A, -369G/C, -499T/A, and -588G/A...
January 31, 2018: Cellular and Molecular Biology
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