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Sickle cell anemia

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https://www.readbyqxmd.com/read/28913922/a-clinically-meaningful-fetal-hemoglobin-threshold-for-children-with-sickle-cell-anemia-during-hydroxyurea-therapy
#1
Jeremie H Estepp, Matthew P Smeltzer, Guolian Kang, Chen Li, Winfred C Wang, Christina Abrams, Banu Aygun, Russell E Ware, Kerri Nottage, Jane S Hankins
Hydroxyurea has proven clinical benefits and is recommended to be offered to all children with sickle cell anemia (SCA), but the optimal dosing regimen remains controversial. Induction of red blood cell fetal hemoglobin (HbF) by hydroxyurea appears to be dose-dependent. However, it is unknown whether maximizing HbF% improves clinical outcomes. HUSTLE (NCT00305175) is a prospective observational study with a primary goal of describing the long-term clinical effects of hydroxyurea escalated to maximal tolerated dose (MTD) in children with SCA...
September 14, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28911832/association-of-silent-infarcts-in-sickle-cell-anemia-with-decreased-annexin-a5-resistance
#2
Kerry A Morrone, Lydia H Pecker, Jacob Rand, Jennifer Davila, Suzette Oyeku, Jane A Little, Xue Xiaonan, Deepa Manwani
BACKGROUND: Sickle cell anemia (SCA) is characterized by abnormally shaped, adhesive RBCs that interact with white blood cells and the endothelium, leading to chronic hemolysis, vasculopathy and a prothrombotic state. About 10% of subjects with a thrombotic event in the general population will have an associated antiphospholipid (aPL) antibody. One proposed mechanism for the thrombophilic nature of aPL antibodies is the disruption of the potent anticoagulant annexin A5 or Annexin A5 resistance (A5R)...
September 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28904678/-epidemiological-profile-of-hemoglobinopathies-a-cross-sectional-and-descriptive-index-case-study
#3
Fatima Dahmani, Souad Benkirane, Jaafar Kouzih, Aziz Woumki, Hassan Mamad, Azlarab Masrar
Hemoglobinopathies are congenital disorders resultimg from hemoglobin abnormalities. Major forms are often severe, their management is difficult and associated with a great psychosocial impact on patients and their families. They are classified as rare diseases and are still insufficiently known by health professionals. This lack of knowledge is at the origin of diagnostic errors, delay in their management and therefore high morbidity and mortality rate for these patients. In 2008, the World Health Organization (WHO) has published data on hemoglobinopathies epidemiology: more than 330...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28891545/protein-c-and-antithrombin-levels-in-patients-with-sickle-cell-anemia-in-ahmadu-bello-university-teaching-hospital-zaria-nigeria
#4
I U Kusfa, A I Mamman, S M Aminu, A Hassan, H M Muktar
BACKGROUND: Alterations in the components of hemostasis, namely platelet function, the procoagulant, anticoagulant, and the fibrinolytic systems, are observed in sickle cell anemia (SCA) and are in favor of a procoagulant phenotype. Therefore, study of protein C and antithrombin (AT) levels in patients with SCA in steady state may be used in the treatment and/or prevention of SCA-related thrombotic complications. We studied the changes of these naturally occurring anticoagulants in patients with SCA attending the sickle cell clinic in Ahmadu Bello University Teaching Hospital, Zaria...
August 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28884840/hydroxycarbamide-in-children-with-sickle-cell-anemia-after-first-dose-vs-chronic-therapy-pharmacokinetics-and-predictive-models-for-drug-exposure
#5
Jeremie H Estepp, Paweł Wiczling, Joseph Moen, Guolian Kang, Joana Marie Mack, Robert Liem, Julie A Panepinto, Uttam Garg, Gregory Kearns, Kathleen A Neville
AIMS: The purpose of this work was to (1) compare pharmacokinetic (PK) parameters for hydroxycarbamide in children receiving their first dose (HCnew ) versus those receiving chronic therapy (HCchronic ), (2) assess the external validity of a published PK dosing strategy, and (3) explore the accuracy of dosing strategies based on a limited number of HC measurements. METHODS: Utilizing data from two prospective, multicenter trials of hydroxycarbamide (Pharmacokinetics of Liquid Hydroxyurea in Pediatric Patients with Sickle Cell Anemia; NCT01506544 and Single-Dose (SD) and Steady-State (SS) Pharmacokinetics of Hydroxyurea in Children and Adolescents with Sickle Cell Disease), plasma drug concentration versus time profiles were evaluated with a model independent approach in the HCnew and HCchronic groups...
September 8, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28880867/oral-tetrahydrouridine-and-decitabine-for-non-cytotoxic-epigenetic-gene-regulation-in-sickle-cell-disease-a-randomized-phase-1-study
#6
RANDOMIZED CONTROLLED TRIAL
Robert Molokie, Donald Lavelle, Michel Gowhari, Michael Pacini, Lani Krauz, Johara Hassan, Vinzon Ibanez, Maria A Ruiz, Kwok Peng Ng, Philip Woost, Tomas Radivoyevitch, Daisy Pacelli, Sherry Fada, Matthew Rump, Matthew Hsieh, John F Tisdale, James Jacobberger, Mitch Phelps, James Douglas Engel, Santhosh Saraf, Lewis L Hsu, Victor Gordeuk, Joseph DeSimone, Yogen Saunthararajah
BACKGROUND: Sickle cell disease (SCD), a congenital hemolytic anemia that exacts terrible global morbidity and mortality, is driven by polymerization of mutated sickle hemoglobin (HbS) in red blood cells (RBCs). Fetal hemoglobin (HbF) interferes with this polymerization, but HbF is epigenetically silenced from infancy onward by DNA methyltransferase 1 (DNMT1). METHODS AND FINDINGS: To pharmacologically re-induce HbF by DNMT1 inhibition, this first-in-human clinical trial (NCT01685515) combined 2 small molecules-decitabine to deplete DNMT1 and tetrahydrouridine (THU) to inhibit cytidine deaminase (CDA), the enzyme that otherwise rapidly deaminates/inactivates decitabine, severely limiting its half-life, tissue distribution, and oral bioavailability...
September 2017: PLoS Medicine
https://www.readbyqxmd.com/read/28868627/hydroxyurea-prescription-availability-and-use-for-children-with-sickle-cell-disease-in-italy-results-of-a-national-multicenter-survey
#7
Raffaella Colombatti, Giovanni Palazzi, Nicoletta Masera, Lucia Dora Notarangelo, Elisa Bonetti, Piera Samperi, Angelica Barone, Silverio Perrotta, Elena Facchini, Maurizio Miano, Giovanni Carlo Del Vecchio, Maria Elena Guerzoni, Paola Corti, Federica Menzato, Simone Cesaro, Maddalena Casale, Paolo Rigano, Gian Luca Forni, Giovanna Russo, Laura Sainati
BACKGROUND: The number of patients with sickle cell disease (SCD) has increased in Italy in the past decade due to immigration. In spite of the established efficacy of hydroxyurea (HU) in childhood, population-based data regarding its prescription and effectiveness come mainly from studies performed in adults or outside Europe. POPULATION AND METHODS: The Hydroxyurea in SCD: A Large Nation-wide Cohort Study from Italy was a retrospective cohort study of adult and pediatric patients with SCD attending 32 centers...
September 4, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28868518/associations-of-%C3%AE-thalassemia-and-bcl11a-with-stroke-in-nigerian-united-states-and-united-kingdom-sickle-cell-anemia-cohorts
#8
Santosh L Saraf, Titilola S Akingbola, Binal N Shah, Chinedu A Ezekekwu, Omowunmi Sonubi, Xu Zhang, Lewis L Hsu, Mark T Gladwin, Roberto F Machado, Richard S Cooper, Victor R Gordeuk, Bamidele O Tayo
Alpha-thalassemia and the BCL11A rs1427407 T allele are commonly observed in sickle cell anemia (SCA) patients and are associated with reduced hemolysis and higher hemoglobin F levels, respectively. We investigated whether a high-risk genetic profile, defined as SCA patients who did not inherit either α-thalassemia or the BCL11A rs1427407 T allele, had stronger associations with clinical and laboratory variables than the individual genetic components in the University of Ibadan cohort (n=249). We then replicated our findings in SCA cohorts from the University of Illinois at Chicago (UIC)(n=260) and Walk-Treatment of Pulmonary Hypertension and Sickle cell disease with Sildenafil Therapy (Walk-PHaSST)(n=387)...
April 25, 2017: Blood Advances
https://www.readbyqxmd.com/read/28867123/comparison-of-outcomes-in-patients-having-acute-myocardial-infarction-with-versus-without-sickle-cell-anemia
#9
Gbolahan O Ogunbayo, Naoki Misumida, Odunayo Olorunfemi, Ayman Elbadawi, Deola Saheed, Adrian Messerli, Claude S Elayi, Susan S Smyth
Sickle-cell disease (SCD) affects millions worldwide. Sickle-cell anemia (SCA), the most severe form of this disease, is the most common inherited blood disorder in the United States. There are limited data on the incidence, clinical characteristics, and outcomes of acute myocardial infarction (AMI) in these patients. Using data from the National Inpatient Sample database, we matched cases (AMI with SCA) with controls (AMI without SCA) in a 1:1 ratio for age, gender, race, and year of admission. We compared both groups in terms of clinical characteristics and inpatient outcomes and performed a logistic regression with mortality as the primary outcome...
August 8, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28863145/associations-between-endothelial-dysfunction-and-clinical-and-laboratory-parameters-in-children-and-adolescents-with-sickle-cell-anemia
#10
Rozana Santos Teixeira, Regina Terse-Ramos, Tatiane Anunciação Ferreira, Vinícius Ramos Machado, Marya Izadora Perdiz, Isa Menezes Lyra, Valma Lopes Nascimento, Ney Boa-Sorte, Bruno B Andrade, Ana Marice Ladeia
BACKGROUND: Hematological changes can drive damage of endothelial cells, which potentially lead to an early endothelial dysfunction in patients with sickle cell anemia (SCA). An association may exist between endothelial dysfunction and several clinical manifestations of SCA. The present study aims to evaluate the links between changes in endothelial function and clinical and laboratory parameters in children and adolescents with SCA. METHODS: This study included 40 children and adolescents with stable SCA as well as 25 healthy children; aged 6-18 years...
2017: PloS One
https://www.readbyqxmd.com/read/28852485/prevalence-and-determinants-of-microalbuminuria-in-children-suffering-from-sickle-cell-anemia-in-steady-state
#11
Michel N Aloni, Jean-Louis L Mabidi, René M Ngiyulu, Pépé M Ekulu, Fiston I Mbutiwi, Jean Robert Makulo, Ernest K Sumaili, Jean Lambert Gini-Ehungu, Célestin N Nsibu, Nazaire M Nseka, François B Lepira
BACKGROUND: Sickle cell anemia (SCA) is considered a major risk factor for renal complications. The main goal of this study was to determine the frequency of macroalbuminuria and microalbuminuria in Congolese children <18 years of age suffering from Sickle cell anemia and to identify associated factors. METHODS: The cross-sectional study was completed in 150 hemoglobin-SS children (77 boys and 73 girls). Microalbuminuria was defined by a urine albumin:creatinine ratio of 30-299 mg/g...
August 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28845411/leech-therapy-for-treating-priapism-case-report
#12
Sayed Aladdin Asgari, Sadeq Rostami, Mojtaba Teimoori
Priapism is well-defined by persistent, painful penile erection which happens without sexual stimulation. Currently, the hirudotherapy is practiced to treat venous congestion and subsequent compartment syndrome. Here we will report a case of a male with priapism treated by leeches. The case was a 26 yr old young single male referred to the Razi Hospital Emergency Department, Guilan University of Medical Sciences, Rasht, Iran due to long-time spontaneous erections. The patient had no history of mental disorders, trauma or sickle cell anemia...
July 2017: Iranian Journal of Public Health
https://www.readbyqxmd.com/read/28840600/red-blood-cell-minor-antigen-mismatches-during-chronic-transfusion-therapy-for-sickle-cell-anemia
#13
Marianne E M Yee, Cassandra D Josephson, Anne M Winkler, Jennifer Webb, Naomi L C Luban, Traci Leong, Sean R Stowell, Ross M Fasano
BACKGROUND: Red blood cell (RBC) alloimmunization occurs at a high frequency in sickle cell anemia (SCA) despite serologic matching for Rh (C/c, E/e) and K antigens. RBC minor antigen genotyping allows for prediction of antigens and RH variants that may lead to alloimmunization. STUDY DESIGN AND METHODS: RBC antigen genotyping was performed on chronically transfused pediatric SCA patients, using PreciseType human erythrocyte antigen (HEA), RHCE, and RHD BeadChip arrays...
August 24, 2017: Transfusion
https://www.readbyqxmd.com/read/28837214/combined-genotypes-of-the-mbl2-gene-related-to-low-mannose-binding-lectin-levels-are-associated-with-vaso-occlusive-events-in-children-with-sickle-cell-anemia
#14
Fernanda Silva Medeiros, Taciana Furtado de Mendonça, Katiuscia Araújo de Miranda Lopes, Laís Medeiros da Câmara França, Andreia Soares da Silva, Luydson Richardson Silva Vasconcelos, Maria do Carmo Valgueiro Costa de Oliveira, Ana Cláudia Mendonça Dos Anjos, Betânia Lucena Domingues Hatzlhofer, Marcos André Cavalcanti Bezerra, Aderson da Silva Araújo, Patrícia Moura, Maria do Socorro de Mendonça Cavalcanti
Sickle cell anemia (SCA) presents heterogenous clinical manifestations that cannot be explained solely by alterations to hemoglobin (Hb); other components such as endothelial adhesion, thrombosis and inflammation may be involved. The mannose-binding lectin (MBL) has an important role in innate immunity and inflammatory diseases. In this report, we describe an association between MBL2 polymorphism related to low production of serum MBL and the frequency of vasoocclusive events (FVOE) in children ≤ 5 years old with SCA (p = 0...
July 2017: Genetics and Molecular Biology
https://www.readbyqxmd.com/read/28834316/history-of-parvovirus-b19-infection-is-associated-with-silent-cerebral-infarcts
#15
Foluso J Ogunsile, Kelli L Currie, Mark Rodeghier, Adetola Kassim, Michael R DeBaun, Deva Sharma
BACKGROUND: The relationship between silent cerebral infarcts (SCIs) and history of parvovirus B19 (B19V) has not been systematically evaluated. As an ancillary study from the Silent Cerebral Infarct Trial (SIT) (NCT00072761), we tested the hypothesis that a history of B19V infection is associated with an increased prevalence of SCIs in children with sickle cell anemia. PROCEDURE: We used a retrospective cross-sectional cohort study design; each participant underwent a brain magnetic resonance imaging (MRI) scan and medical record review for prior B19V infection (n = 958)...
August 17, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28834130/lack-of-mortality-in-22-children-with-sickle-cell-anemia-and-severe-malarial-anemia
#16
Robert O Opoka, Paul Bangirana, Richard Idro, Estela Shabani, Ruth Namazzi, Chandy C John
Retrospective studies suggest that there is high mortality in children with sickle cell anemia (SCA) and severe malaria. We assessed mortality in Ugandan children with severe malarial anemia (SMA, n = 232) or cerebral malaria (CM, n = 267) by sickle cell hemoglobin genotype. Admission and 2-year follow-up mortality did not differ among children with SMA who had homozygous form of sickle cell hemoglobin (HbSS) versus normal form of adult hemoglobin (admission, 0/22, 0%, vs. 1/208, 0.5%; follow-up, 1/22, 4.5%; 7/207, 3...
August 17, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28827079/application-of-genomics-for-transfusion-therapy-in-sickle-cell-anemia
#17
Stella T Chou, Connie M Westhoff
The application of genomic approaches is impacting all areas of laboratory testing including transfusion medicine. Use of DNA-based test methods is particularly applicable for red cell and platelet antigen typing as the majority of genes encoding the carrier proteins and carbohydrates are now known and were cloned in the 1990's. Many of the antigenic polymorphisms are due to single nucleotide changes (SNP's) in the respective genes and DNA-arrays that target these changes have been validated by comparison with conventional serologic typing...
August 8, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28826581/uncommon-causes-of-cerebral-microbleeds
#18
REVIEW
Nariman Noorbakhsh-Sabet, Varun Chandi Pulakanti, Ramin Zand
BACKGROUND: Cerebral microbleeds (CMBs) are small and round perivascular hemosiderin depositions detectable by gradient echo sequences or susceptibility-weighted imaging. Cerebral microbleeds are common among patients with hypertension, cerebral ischemia, or cerebral amyloid angiopathy. In this article, we describe uncommon causes of CMBs. METHODS: We searched Pubmed with the keyword CMBs for relevant studies and looked for different uncommon causes of CMBs. RESULTS: CMBs have several uncommon etiologies including posterior reversible encephalopathy syndrome, infective endocarditis, brain radiation therapy, cocaine abuse, thrombotic thrombocytopenic purpura, traumatic brain injury, intravascular lymphomatosis or proliferating angio-endotheliomatosis, moyamoya disease, sickle cell anemia/β-thalassemia, cerebral autosomal dominant arteriopathy subcortical infarcts, and leukoencephalopathy (CADASIL), genetic syndromes, or obstructive sleep apnea...
August 17, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28821470/design-of-the-drepagreffe-trial-a-prospective-controlled-multicenter-study-evaluating-the-benefit-of-genoidentical-hematopoietic-stem-cell-transplantation-over-chronic-transfusion-in-sickle-cell-anemia-children-detected-to-be-at-risk-of-stroke-by-transcranial
#19
Sylvie Chevret, Suzanne Verlhac, Elisabeth Ducros-Miralles, Jean-Hugues Dalle, Regis Peffault de Latour, Mariane de Montalembert, Malika Benkerrou, Corinne Pondarré, Isabelle Thuret, Corinne Guitton, Emmanuelle Lesprit, Maryse Etienne-Julan, Gisèle Elana, Jean-Pierre Vannier, Patrick Lutz, Bénédicte Neven, Claire Galambrun, Catherine Paillard, Camille Runel, Charlotte Jubert, Cécile Arnaud, Annie Kamdem, Valentine Brousse, Florence Missud, Marie Petras, Lydia Doumdo-Divialle, Claire Berger, Françoise Fréard, Olivier Taieb, Elise Drain, Monique Elmaleh, Manuela Vasile, Yacine Khelif, Myriam Bernaudin, Philippe Chadebech, France Pirenne, Gérard Socié, Françoise Bernaudin
BACKGROUND: Children with sickle cell anemia (SCA) have an 11% risk of stroke by the age of 18. Chronic transfusion applied in patients detected to be at risk by transcranial Doppler allows a significant reduction of stroke risk. However, chronic transfusion exposes to several adverse events, including alloimmunization and iron overload, and is not curative. Hematopoietic stem cell transplantation allows termination of the transfusion program, but its benefit has not been demonstrated...
August 15, 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/28819305/cardiorespiratory-pathogenesis-of-sickle-cell-disease-in-a-mouse-model
#20
Jun Ren, Xiuqing Ding, Marie Trudel, John J Greer, Joanna E MacLean
The nature and development of cardiorespiratory impairments associated with sickle cell disease are poorly understood. Given that the mechanisms of these impairments cannot be addressed adequately in clinical studies, we characterized cardiorespiratory pathophysiology from birth to maturity in the sickle cell disease SAD mouse model. We identified two critical phases of respiratory dysfunction in SAD mice; the first prior to weaning and the second in adulthood. At postnatal day 3, 43% of SAD mice showed marked apneas, anemia, and pulmonary vascular congestion typical of acute chest syndrome; none of these mice survived to maturity...
August 17, 2017: Scientific Reports
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