THERAPEUTIC GENE EDITING FOR HEMOGLOBINOPATHIES by Ugo Testa, Giuseppe Leone, Maria Domenica Cappellini

“… In the last ten years, a consistent number of clinical studies have evaluated different gene approaches for the treatment of patients with sickle cell disease (SCD) and transfusion-dependent b-thalassemia (TDT). …”
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Changes in Blood Profile from Steady State in Patients with Sickle Cell Anemia Admitted for Vaso-occlusive Crisis and Acute Chest Syndrome by Timothy Klouda, Deepti Raybagkar, Bruce Bernstein, Nataly Apollonsky

“…Close to half of all patients with sickle cell disease (SCD) will have at least one episode of acute chest syndrome (ACS) during their lifetime. …”
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HbSC Disease and Spontaneous Epidural Hematoma with Kernohan’s Notch Phenomena by Meera Yogarajah, Chidozie Charles Agu, Bhradeev Sivasambu, Mark A. Mittler

“…Spontaneous (nontraumatic) acute epidural hematoma is a rare and poorly understood complication of sickle cell disease. A 19-year-old African American male with hemoglobin SC disease (HbSC) presented with generalized body aches and was managed for acute painful crisis. …”
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Cellular Reprogramming toward the Erythroid Lineage by Laura J. Norton, Alister P. W. Funnell, Richard C. M. Pearson, Merlin Crossley

“…Haemoglobinopathies such as thalassaemia and sickle cell disease present a major health burden. Currently, the main forms of treatment for these diseases are packed red blood cell transfusions and the administration of drugs which act to nonspecifically reactivate the production of foetal haemoglobin. …”
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Unlocking the potential of serious games for rehabilitation in low and middle-income countries: addressing potential and current limitations by Diriba Dereje, Diriba Dereje, Dheeraj Lamba, Teklu Gemechu Abessa, Teklu Gemechu Abessa, Chala Kenea, Chala Kenea, Cintia Ramari, Cintia Ramari, Muhammad Osama, Muhammad Osama, Oyéné Kossi, Oyéné Kossi, Paul Muteb Boma, Paul Muteb Boma, Jules Panda, Jules Panda, Jules Panda, Anna Kushnir, Anna Kushnir, Joanna Mourad, Joanna Mourad, Jean Mapinduzi, Jean Mapinduzi, Maryam Fourtassi, Kim Daniels, Kim Daniels, Judith Deutsch, Bruno Bonnechère, Bruno Bonnechère, Bruno Bonnechère

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Safety of Pegfilgrastim (Neulasta) in Patients with Sickle Cell Trait/Anemia by Pashtoon Murtaza Kasi, Mrinal M. Patnaik, Prema P. Peethambaram

“…Caution is advised when using it in patients with sickle cell disease. Here we present a case of a Caucasian female with known sickle cell trait (SCT) with no prior complications who developed a presumed sickle cell crisis after getting Neulasta, as a part of the chemotherapy regimen used to treat her breast cancer. …”
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Sickle Cell Trait Presenting as Unilateral Proliferative Retinopathy and Macular Thinning in a Pregnant Woman by Sepideh Ghods, Elias Khalili Pour, Hooshang Faghihi, Golnaz Gharehbaghi, Ahmad Mirshahi, Fariba Ghassemi, Bahman Inanloo, Hamid Riazi-Esfahani

“…Even though the sickle cell trait is generally considered as a milder form of sickle cell disease without severe retinal manifestations, pregnancy should be considered as a trigger that can induce proliferative changes and foveal splaying in this group of patients.…”
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Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia—A Case Report and Review of the Literature by Sameera A. Alsafwani, Abdulwahed Al-Saeed, Rehab Bukhamsin

“…Bone marrow necrosis (BMN) is a rare clinical entity that was first described in an autopsy of a sickle cell disease (SCD) patient and is defined as ill-defined necrotic cells in an amorphous eosinophilic background with preservation of cortical bone. …”
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FK228 Analogues Induce Fetal Hemoglobin in Human Erythroid Progenitors by Levi Makala, Salvatore Di Maro, Tzu-Fang Lou, Sharanya Sivanand, Jung-Mo Ahn, Betty S. Pace

“…Fetal hemoglobin (HbF) improves the clinical severity of sickle cell disease (SCD), therefore, research to identify HbF-inducing agents for treatment purposes is desirable. …”
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Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest Syndrome by Hazel Villanueva, Sandeepkumar Kuril, Jennifer Krajewski, Aziza Sedrak

“…Sickle cell hemoglobin D disease (HbSD) is a rare variant of sickle cell disease (SCD). Incidence of pulmonary thromboembolism (PE) and deep venous thrombosis (DVT) in children with HbSD is unknown. …”
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Pyruvate Kinase Deficiency Causing Priapism by Vinay Hanyalu Shankar, Bharadwaj Adithya-Sateesh, Nicole Gousy, Girma Ayele, Freyr Petursson, Rediet Atalay, Miriam Michael

“…This differs greatly as priapism has been frequently reported in patients with other chronic hemoglobinopathies such as sickle cell disease, thalassemia, and G6PD with and without splenectomy. …”
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Unbinding of alpha chain of hemoglobin in sickle and normal structures by Jhulan Powrel, Rajendra Prasad Koirala, Narayan Prasad Adhikari

“…Sickle cell disease, a genetic disorder, is caused by a mutation of glutamic acid into valine in β chain of hemoglobin at the sixth residue, resulting in structural change of the entire hemoglobin molecule into a sickle shape. …”
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Hyperhemolysis Syndrome without Underlying Hematologic Disease by Lauren Anne Eberly, Diaa Osman, Nathaniel Perryman Collins

“…This phenomenon, commonly described in sickle cell disease, is a rare occurrence in patients without hemoglobinopathies. …”
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Association of Oxidative Stress Markers with Atherogenic Index of Plasma in Adult Sickle Cell Nephropathy by M. A. Emokpae, P. O. Uadia

“…Antioxidant enzymes, malondialdehyde(MDA), urea, creatinine, and glomerular filtration rate were evaluated in 110 confirmed sickle cell disease patients: 65 males in steady state, aged 21.1±6.0 years, 30 males with macroalbuminuria, aged 24.5±7.0, years and 15 with chronic kidney disease (CKD), aged 31.8±2.0 years. …”
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Hemoglobin Variants as Targets for Stabilizing Drugs by Miroslava Žoldáková, Michal Novotný, Krishna P. Khakurel, Gabriel Žoldák

“…Genetic variations in hemoglobin chains, such as those underlying sickle cell disease and thalassemias, present substantial clinical challenges. …”
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Hepatic Iron Quantification on 3 Tesla (3 T) Magnetic Resonance (MR): Technical Challenges and Solutions by Muhammad Anwar, John Wood, Deepa Manwani, Benjamin Taragin, Suzette O. Oyeku, Qi Peng

“…Our study showed that the quantification of hepatic iron on 3 T MRI in sickle cell disease patients correlates well with clinical blood test results and biopsy results. 3 T MR liver iron quantification based on MFGRE can be used for hepatic iron quantification in transfused patients.…”
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Qualitative and Quantitative Microbiological Studies of Paediatric Artemether-Lumefantrine Dry Powders and Paracetamol Syrups Obtained from Selected Drug Stores in Accra, Ghana by Solomon Opoku, Isaac Nyanor

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Cardiovascular Characterization of Children and Adolescents with Sickle Cell Anemia by Nancy González Vales, Ximena Laura Graña, Lucía Díaz Morejón, Tamara Sánchez Estrada, Belkis Rodríguez Jorge, Ifraín Machín Caride

“…<br /> <strong>Objective:</strong> to determine the cardiovascular disturbances in patients with sickle cell disease. <br /><strong>Methods:</strong> a descriptive and cross-sectional study was conducted in the hematology and cardiology consultations of the Paquito González Cueto de Cienfuegos University Pediatric Hospital from July 1, 2011 to July 31, 2012. …”
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Sickled Erythrocytes Reversal and Membrane Stabilizing Compounds in Telfairia occidentalis by Samuel Atabo, Ismaila Alhaji Umar, Dorcas Bolanle James, Aisha Indo Mamman

“…Traditional management of sickle cell disease (SCD) is ubiquitous in Africa. In south-eastern Nigeria, Telfairia occidentalis (T. occidentalis) is strongly recommended for consumption by SCD patients, owing to its presumed therapeutic effect. …”
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Ecological System Influences in the Treatment of Pediatric Chronic Pain by Deirdre E Logan, Lisa Engle, Amanda B Feinstein, Christine B Sieberg, Penny Sparling, Lindsey L Cohen, Caitlin Conroy, Dana Driesman, Akihiko Masuda

“…Finally, an acceptance and commitment therapy-focused group intervention for children with sickle cell disease and their parents demonstrates the benefits of peer contact as an element of the therapeutic intervention.…”
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