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"Sickle Cell Disease"

Samarth P Shah, Jennifer D Twilla, Leonette Kemp, Gregory L Phelps, Anne Reaves
Sickle cell disease (SCD) is a chronic condition characterized by multiple vaso-occlusive complications, including acute pain crisis. The mainstay of treatment for patients presenting with vaso-occlusive crisis (VOC) is pain control and adequate hydration. Currently, there are no studies to determine an optimal pain control regimen in adult SCD patients. The main objective of this study is to evaluate whether outcomes differ in patients with VOC based on pain management treatment modality. A retrospective review of admissions with a primary diagnosis of VOC admitted to our facility was conducted...
March 21, 2019: Journal of Pain & Palliative Care Pharmacotherapy
Young Lu, Charles C Lin, Alex Doermann, Nilay Patel, Nitin N Bhatia, Phillip Douglas Kiester, Charles D Rosen, Yu-Po Lee
STUDY DESIGN: A retrospective study. OBJECTIVE: To determine the impact of sickle cell anemia on perioperative outcomes and resource utilization in elective spinal fusion surgery. SUMMARY OF BACKGROUND DATA: Sickle cell anemia has been identified as an important surgical risk factor in otolaryngology, cardiothoracic surgery, general surgery, and total joint arthroplasty. However, the impact of sickle cell anemia on elective spine surgery is unknown...
March 19, 2019: Clinical Spine Surgery
Anupam Aich, Michael K Jones, Kalpna Gupta
PURPOSE OF REVIEW: Pain is a major comorbidity of sickle cell disease (SCD). Opioids are the mainstay for pain treatment but remain suboptimal. We discuss mechanism-based treatable targets devoid of opioids to prevent and/or treat SCD pain. RECENT FINDINGS: Understanding the pathogenesis of pain is critical to develop targeted therapies. Nevertheless, acute and chronic pain can have independent and/or overlapping mechanisms. The origin of pain involves neurovascular and neuroimmune interactions from the periphery and/or central nervous system...
March 18, 2019: Current Opinion in Hematology
Enrico M Novelli, Lynda Little-Ihrig, Heather E Knupp, Natasha M Rogers, Mingyi Yao, Jeffrey J Baust, Daniel Meijles, Claudette M St Croix, Mark A Ross, Patrick J Pagano, Evan R DeVallance, George Miles, Karin P Potoka, Jeffrey S Isenberg, Mark T Gladwin
Pulmonary hypertension (PH) is a leading cause of death in sickle cell disease (SCD). Hemolysis and oxidative stress are contributing factors to SCD-associated PH. We have reported that the protein thrombospondin-1 (TSP1) is elevated in the plasma of patients with SCD and by interacting with its receptor CD47 limits vasodilation of distal pulmonary arteries ex vivo. We hypothesized that the TSP1-CD47 interaction may promote PH in SCD. We found that TSP1 and CD47 are upregulated in the lungs of BERK sickle mice and patients with SCD-associated PH...
March 20, 2019: American Journal of Physiology. Lung Cellular and Molecular Physiology
Yuefan Chen, Robert S White, Virginia Tangel, Selaiman A Noori, Licia K Gaber-Baylis, Neel D Mehta, Kane O Pryor
AIM: To compare readmission rates between patients with sickle cell disease (SCD) and non-sickle cell disease undergoing total hip and knee arthroplasty (THA and TKA). METHODS: Identified adult patients who underwent THA or TKA from 2007 to 2014 in California, Florida, New York, Maryland and Kentucky using a multistate database. Outcomes were 30- and 90-day readmission rates, mortality, complications, length of stay and hospital charges. Logistic regression models were used for analysis...
March 20, 2019: Journal of Comparative Effectiveness Research
Athena Starlard-Davenport, Alana Smith, Luan Vu, Biaoru Li, Betty S Pace
Sickle cell disease (SCD) affects over 2 million people worldwide with high morbidity and mortality in underdeveloped countries. Therapeutic interventions aimed at reactivating fetal haemoglobin (HbF) is an effective approach for improving survival and ameliorating the clinical severity of SCD. A class of agents that inhibit DNA methyltransferase (DNMT) activity show promise as HbF inducers because off-target effects are not observed at low concentrations. However, these compounds are rapidly degraded by cytidine deaminase when taken by oral administration, creating a critical barrier to clinical development for SCD...
March 19, 2019: British Journal of Haematology
Brian T Castle, David J Odde, David K Wood
In sickle cell disease, the aberrant assembly of hemoglobin fibers induces changes in red blood cell morphology and stiffness, which leads to downstream symptoms of the disease. Therefore, understanding of this assembly process will be important for the treatment of sickle cell disease. By performing the highest spatiotemporal resolution measurements (55 nm at 1 Hz) of single sickle hemoglobin fiber assembly to date and combining them with a model that accounts for the multistranded structure of the fibers, we show that the rates of sickle hemoglobin addition and loss have been underestimated in the literature by at least an order of magnitude...
March 2019: Science Advances
Joseph Cusano, Emily J Curry, Kiera A Kingston, Elizabeth Klings, Xinning Li
INTRODUCTION: Surgical management of patients with sickle cell disease (SCD) poses a unique challenge to orthopaedic surgeons due to increased operative and perioperative risk. Studies evaluating perioperative complications among patients with SCD undergoing orthopaedic surgery have been limited. We sought to review the clinical characteristics and perioperative complications in our patients with SCD who required orthopaedic surgery. METHODS: Our institution has one of the largest sickle cell centers in the Northeastern United States...
March 11, 2019: Journal of the American Academy of Orthopaedic Surgeons
Huy Tran, Varun Sagi, Waogwende Leonce Song-Naba, Ying Wang, Aditya Mittal, Yann Lamarre, Lei Zhang, Kalpna Gupta
No abstract text is available yet for this article.
March 26, 2019: Blood Advances
V Thonier
Sickle cell disease (SCD) is the most prevalent genetic disorder in France. Many other countries are also affected. Transfusion is still a key treatment for patients suffering from this condition. As a result, SCD patients are much more exposed to transfusions and their risks than the general population. The most feared situation is delayed hemolytic transfusion reaction (DHTR). In certain situations, defined as hyperhemolysis, autologous red blood cells (RBCs) are also targeted and destroyed. This can put the patient in a life-threating situation...
February 22, 2019: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
Javier Bolaños-Meade, Kenneth R Cooke, Christopher J Gamper, Syed Abbas Ali, Richard F Ambinder, Ivan M Borrello, Ephraim J Fuchs, Douglas E Gladstone, Christian B Gocke, Carol Ann Huff, Leo Luznik, Lode J Swinnen, Heather J Symons, Stephanie A Terezakis, Nina Wagner-Johnston, Richard J Jones, Robert A Brodsky
BACKGROUND: Although severe haemoglobinopathies can be cured with allogeneic blood or bone marrow transplantation, availability of matched donors and toxic effects can be problematic. We previously found that non-myeloablative haploidentical related bone marrow transplantation with post-transplantation cyclophosphamide expanded the donor pool while limiting graft-versus-host disease (GVHD). However, graft failure-albeit with full host haemopoietic recovery-occurred in 50% of patients...
March 13, 2019: Lancet Haematology
John Tisdale
No abstract text is available yet for this article.
March 13, 2019: Lancet Haematology
Balu K Chacko, Matthew R Smith, Michelle S Johnson, Gloria Benavides, Matilda L Culp, Jyotsna Pilli, Sruti Shiva, Karan Uppal, Young-Mi Go, Dean P Jones, Victor M Darley-Usmar
Mitochondria possess reserve bioenergetic capacity, supporting protection and resilience in the face of disease. Approaches are limited to understand factors that impact mitochondrial functional reserve in humans. We applied the mitochondrial stress test (MST) to platelets from healthy subjects and found correlations between energetic parameters and mitochondrial function. These parameters were not correlated with mitochondrial complex I-IV activities, however, suggesting that other factors affect mitochondrial bioenergetics and metabolism...
March 10, 2019: Redox Biology
Ali Alsinan, Moslem Alelq, Fozia Alsugair, Ali Hassan, Abrar Waheed, Saeed Alshomimi
INTRODUCTION: Hemoglobinopathies such as Thalassemia and Sickle Cell Disease are risk factors for splenic abscess formation. Gastro-splenic fistula is a rare complication of splenic abscess. In the literature, there are no reported cases of gastro-splenic fistula in patients with hemoglobinopathies. PRESENTATION OF CASE: We are presenting a rare case of a 15-year-old boy with undiagnosed sickle cell trait with splenic sequestration crisis as first presentation of his disease...
March 5, 2019: International Journal of Surgery Case Reports
Seyi Aderotoye-Oni, Ijeoma N Diaku-Akinwumi, Adeyinka Adeniran, Bode Falase
Worldwide, sickle cell disease (SCD) poses a significant public health concern. It causes recurrent morbidity, and premature death is a distinct possibility, especially in Nigeria, which bears half the world's burden of SCD patients. Nigeria has yet to establish a newborn screening program; consequently, most affected children are diagnosed between one and three years of age when a health problem arises. Parents are unprepared to identify SCD and seek comprehensive management early enough for the best outcome...
December 31, 2018: Curēus
Guillaume Bayer, Florent von Tokarski, Benjamin Thoreau, Adeline Bauvois, Christelle Barbet, Sylvie Cloarec, Elodie Mérieau, Sébastien Lachot, Denis Garot, Louis Bernard, Emmanuel Gyan, Franck Perrotin, Claire Pouplard, François Maillot, Philippe Gatault, Bénédicte Sautenet, Emmanuel Rusch, Matthias Buchler, Cécile Vigneau, Fadi Fakhouri, Jean-Michel Halimi
BACKGROUND AND OBJECTIVES: Thrombotic microangiopathies constitute a diagnostic and therapeutic challenge. Secondary thrombotic microangiopathies are less characterized than primary thrombotic microangiopathies (thrombotic thrombocytopenic purpura and atypical hemolytic and uremic syndrome). The relative frequencies and outcomes of secondary and primary thrombotic microangiopathies are unknown. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We conducted a retrospective study in a four-hospital institution in 564 consecutive patients with adjudicated thrombotic microangiopathies during the 2009-2016 period...
March 12, 2019: Clinical Journal of the American Society of Nephrology: CJASN
E A Hod
Red blood cells (RBCs) can be cleared from the circulation either intravascularly or extravascularly. In the setting of an IgG-mediated delayed hemolytic transfusion reaction (HTR), most RBC clearance is typically extravascular, presumably by phagocytosis by liver and splenic macrophages. Animal models of HTRs suggest that this RBC clearance is associated with a pro-inflammatory cytokine response. Although IgG-mediated HTRs are typically benign, they can lead to vaso-occlusive crises and further complications, such as hyperhemolysis, in patients with sickle cell disease (SCD)...
February 22, 2019: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
Landry Erik Mombo, Gaël Mabioko-Mbembo, Cyrille Bisseye, Kevin Mbacky, Fatoumata Thiam, Apollinaire Edou
Background: In Gabon, universal neonatal screening of sickle cell disease is not carried out in rural areas, often leading to late detection of the disease. However, complete blood counts are available in rural areas. Materials and Methods: We evaluated the haematological parameters of 45 homozygous steady-state sickle cell anaemia (SCA) patients and compared them with 45 sex- and age-matched Haemoglobin AA controls in Koula-Moutou, a rural area in Eastern Gabon...
January 2019: Nigerian Postgraduate Medical Journal
Adewole Adesoji Adebiyi, Olaniyi Mabayomije Oyebowale, Ayodele John Olaniyi, Ayodele Olawale Falase
Context: Autonomic nervous system (ANS) dysfunction assessed by abnormalities in heart rate variability (HRV) is thought to play a role in the pathophysiology of sickle cell disease (SCD). There is suggestion that changes in ANS may occur in SCD subjects during episodes of vaso-occlusive crises (VOC). Aims: The aim of this study was to evaluate the ANS by determining the HRV in patients with SCD during VOC. Settings and Design: This was a cross-sectional observational study...
January 2019: Nigerian Postgraduate Medical Journal
Ochuwa Adiketu Babah, Monsurat Bolanle Aderolu, Ayodeji A Oluwole, Bosede B Afolabi
Introduction: Sickle cell disease in pregnancy carries increased risk of maternal and perinatal morbidity and mortality. Past studies on pregnancy complications in sickle cell disease women were limited by relatively small sample sizes, and use of retrospective and hospital discharge data. Study Design: This prospective case-control study compared booked pregnant Haemoglobin (Hb) SS women with AA controls from two tertiary centres in Lagos, in order to precisely identify their complication and mortality rates and identify associated factors...
January 2019: Nigerian Postgraduate Medical Journal
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