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sickle cell disease managment

David M Cline, Susan Silva, Caroline E Freiermuth, Victoria Thornton, Paula Tanabe
Introduction: Use of alternative venues to manage uncomplicated vaso-occlusive crisis (VOC), such as a day hospital (DH) or ED observation unit, for patients with sickle cell anemia, may significantly reduce admission rates, which may subsequently reduce 30-day readmission rates. Methods: In the context of a two-institution quality improvement project to implement best practices for management of patients with sickle cell disease (SCD) VOC, we prospectively compared acute care encounters for utilization of 1) emergency department (ED); 2) ED observation unit; 3) DH, and 4) hospital admission, of two different patient cohorts with SCD presenting to our two study sites...
March 2018: Western Journal of Emergency Medicine
Brianna Marie Lutz, Shaogen Wu, Xiyao Gu, Fidelis E Atianjoh, Zhen Li, Brandon M Fox, David M Pollock, Yuan-Xiang Tao
Sickle cell disease is associated with acute painful episodes and chronic intractable pain. Endothelin-1, a known pain inducer, is elevated in the blood plasma of both sickle cell patients and mouse models of sickle cell disease. We show here that the levels of endothelin-1 and its endothelin type A receptor are increased in the dorsal root ganglia of a mouse model of sickle cell disease. Pharmacologic inhibition or neuron-specific knockdown of endothelin type A receptors in primary sensory neurons of dorsal root ganglion alleviated basal and post-hypoxia evoked pain hypersensitivities in sickle cell mice...
March 15, 2018: Haematologica
Sangeeta Chattoo
This paper engages critically with the global assemblage framing sickle cell and thalassaemia disorders as a 'global health crisis'; and the promise of genomics, largely DNA-based carrier/pre-conceptual screening, prenatal diagnosis with a view to terminations, deployed in framing a solution to these historically racialised spectrum of diseases as essentially preventable. Sickle cell and thalassaemia are recessively inherited, potentially life-threatening haemoglobin disorders with significant variation of severity, often needing life-long treatment...
April 2018: Anthropology & Medicine
M Colinart-Thomas, V Noël, G Roques, S Gordes-Grosjean, M Abely, C Pluchart
Sickle cell disease, a hemoglobin disorder with autosomal recessive transmission, is one of the most common genetic diseases screened in France. Thanks to early management, 95% of sickle cell patients reach adulthood and require transition from pediatric care to adult care. Through a retrospective study of records from serious sickle cell patients over 17 years old, followed in the hematology-oncology pediatric unit of Reims University Hospital Center in France, we analyzed transition conditions, compared pediatric and adult management, and proposed a plan for transition care...
March 9, 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Benjamin Zielonka, Alan R Cohen, Kim Smith-Whitley, Bhavya S Doshi
Although musculoskeletal pain in patients with sickle cell disease (SCD) is most frequently the result of vaso-occlusive episodes, clinicians often consider other etiologies including osteomyelitis, avascular necrosis, and trauma. In this study, we report the case of a young female with SCD with hip and back pain secondary to a nontraumatic iliopsoas periosteal hematoma with evidence of adjacent bone infarction. The pathophysiology, diagnostic considerations, and management of periosteal hematomas in SCD are reviewed...
March 12, 2018: Pediatric Blood & Cancer
Ilknur Kozanoglu, Hakan Ozdogu
Sickle cell disease (SCD) is a life-threatening chronic condition primarily caused by genetic mutation. The disease is characterized by intermittent vaso-occlusive events and chronic hemolytic anemia. Acute complications in patients with SCD are difficult to manage due to the pathophysiological nature of the disease. Transfusion therapy is the cornerstone of management of acute complications and significantly reduces SCD morbidity and mortality. Red cell exchange (RCE), which is characterized by low iron accumulation and volume overload, has been widely used for transfusion therapy in recent years...
February 21, 2018: Transfusion and Apheresis Science
Alana Goldstein-Leever, Lindsey L Cohen, Carlton Dampier, Soumitri Sil
BACKGROUND: Youth with sickle cell disease (SCD) are at risk for recurrent pain and depressive symptoms, both of which contribute to poorer health outcomes. Furthermore, youth and family coping with child pain, including pain catastrophizing, is known to be associated with poorer psychosocial adjustment and greater functional disability among youth with SCD. In particular, child catastrophizing about pain and parent catastrophizing about their child's pain have been linked to increased pain and depressive symptoms in youth with chronic pain conditions...
March 7, 2018: Pediatric Blood & Cancer
Marina Viegas Moura Rezende Ribeiro, João Vitor de Omena Jucá, Anna Luyza Correia Dos Santos Alves, Caio Victor Oliveira Ferreira, Fabiano Timbó Barbosa, Êurica Adélia Nogueira Ribeiro
Hemoglobinopathies are a group of hereditary diseases that cause quantitative or qualitative changes in the shape, function or synthesis of hemoglobin. One of the most common is sickle cell anemia, which, due to sickling of erythrocytes, causes vaso-occlusive phenomena. Among the possible ocular manifestations, the most representative is retinopathy, which can lead to blindness if left untreated. Therefore, periodic ophthalmologic monitoring of these patients is important for early diagnosis and adequate therapeutic management, which can be done localy by treating the lesions in the eyes, or systemically...
December 2017: Revista da Associação Médica Brasileira
Michael N Levas, Marlene Melzer-Lange, Sergey Tarima, Lauren M Beverung, Julie Panepinto
OBJECTIVES: Measuring health-related quality of life (HRQOL) provides the patient's perspective of his/her well-being and offers a unique outcome measure to demonstrate the impact of violence on the victim. To date, no study has described HRQOL in youth victims of violence in the United States. The purpose of this study was to describe HRQOL in youth victims of violence as compared with healthy youth and youth with chronic disease. METHODS: We conducted an exploratory cross-sectional study of the HRQOL of victims of violence aged 8 to 18 years...
February 28, 2018: Pediatric Emergency Care
William J Sanders
Background: Sickle cell trait is usually an asymptomatic presentation of a patient with slightly different hemoglobin molecule makeup than normal. It is similar to a more serious disease, sickle cell disease, in which a person's hemoglobin is mutated in such a way that causes their red blood cells to easily change shape in certain environmental and internal states; this causes red blood cells to adhere to the walls and occlude the lumen of the arteries in which they travel, leading to downstream effects secondary to ischemia...
2018: BMC Hematology
P Brandicourt, L Bonnet, Y Béjot, C Drouet, T Moulin, L Thines
INTRODUCTION: Moya-Moya angiopathy is a neurovascular disease that predisposes to ischemic or hemorrhagic strokes. It is generated by a steno-occlusion of the terminal portion of the internal carotid arteries, which induces the development of abnormal neovessels in the deep regions of the brain. Some pathologies such as sickle cell disease, Down syndrome or Graves' disease may be associated with Moya-Moya angiopathy. These syndromic forms harbor several differences compared with idiopathic Moya-Moya disease...
February 20, 2018: Neuro-Chirurgie
Nadia Kawar, Sahar Alrayyes, Bin Yang, Haider Aljewari
Sickle cell disease (SCD) can affect many systems in the body including the oral cavity. This necessitates modifications in oral health care management of these patients. The purpose of this paper is to make recommendations for management of SCD patients according to their oral health care needs based on the known findings.
February 19, 2018: Disease-a-month: DM
Dipty Jain, Dipika Mohanty
PURPOSE OF REVIEW: In the past, milder clinical manifestations of sickle cell disease (SCD) have been described from India. However, recent data from some parts of India suggest that the severity of the disease can be compared to that of African phenotypes. This review therefore describes the varied clinical manifestation of SCD, the success of newborn screening programme, prenatal diagnosis and low dose hydroxyurea therapy in India. RECENT FINDINGS: The varied clinical manifestations such as anemia, vaso-occlusive crisis, acute chest syndrome, renal involvement, stroke and so on vary from one part of the country to the other and also among different communities of India...
February 19, 2018: Current Opinion in Hematology
C Mallet, A Abitan, C Vidal, L Holvoet, K Mazda, A-L Simon, B Ilharreborde
Purpose: Sickle cell disease (SCD) is the most common cause of femoral head osteonecrosis (ONFH) during childhood with an overall prevalence of 10%. In children, spontaneous revascularization can occur, as in Legg-Calve-Perthes disease. Consequently, the aim of treatment is to restore proper hip containment to prevent joint arthritis. This is the first study reporting long-term results at skeletal maturity of non-operative and surgical treatments for ONFH in SCD children. Methods: All children with ONFH due to SCD were retrospectively reviewed...
February 1, 2018: Journal of Children's Orthopaedics
F Martino, R Di Mauro, K Paciaroni, J Gaziev, C Alfieri, L Greco, R Floris, S Di Girolamo, M Di Girolamo
OBJECTIVES: Sickle cell anemia (SCA) and β -thalassemia major are well-recognized beta-globin gene disorders of red blood cells associated to mortality and morbidity included bone morbidities due to ineffective erythropoiesis and bone marrow expansion, which affect every part of the skeleton. While there are an abundance of described disease manifestations of the head and neck, the manner of paranasal sinuses involvement and its relations to β-thalassemia and SCA process was not studied yet...
March 2018: International Journal of Pediatric Otorhinolaryngology
Oluseyi Oniyangi, Damian H Cohall
BACKGROUND: Sickle cell disease, a common recessively inherited haemoglobin disorder, affects people from sub-Saharan Africa, the Middle East, Mediterranean basin, Indian subcontinent, Caribbean and South America. It is associated with complications and a reduced life expectancy. Phytomedicines (medicine derived from plants in their original state) encompass many of the plant remedies from traditional healers which the populations most affected would encounter. Laboratory research and limited clinical trials have suggested positive effects of phytomedicines both in vivo and in vitro...
February 15, 2018: Cochrane Database of Systematic Reviews
Affette M McCaw-Binns, Leroy V Campbell, Simone S Spence
OBJECTIVE: Describe trends in indirect cause-specific pregnancy-related mortality from 1998-2015. DESIGN: Secondary analysis of annual, national cross-sectional database of maternal and late maternal deaths, identified through active surveillance of deaths among women 10-50 years. SETTING: Jamaica, a middle-income Caribbean country. POPULATION: Maternal and late maternal deaths. METHODS: Descriptive trend analyses of demographic and cause-specific maternal and pregnancy-related mortality ratios undertaken comparing the periods 1998-2003, 2004-9 and 2010-15...
February 8, 2018: BJOG: An International Journal of Obstetrics and Gynaecology
Hakan Ozdogu, Can Boga, Suheyl Asma, Ilknur Kozanoglu, Cigdem Gereklioglu, Mahmut Yeral, Nurhilal Turgut Buyukkurt, Soner Solmaz, Aslı Korur, Pelin Aytan, Erkan Maytalman, Mutlu Kasar
The Eastern Mediterranean is among the regions where sickle cell disease (SCD) is common. The morbidity and mortality of this disease can be postponed to adulthood through therapies implemented in childhood. The present study focuses on the organ damage-reducing effects of the Baskent Sickle Cell Medical Care Development Program (BASCARE), which was developed by a team who lives in this region and has approximately 25 years of experience. The deliverables of the program included the development of an electronic health recording system (PRANA) and electronic vaccination system; the use of low citrate infusion in routine prophylactic automatic erythrocyte exchange (ARCE) programs including pregnant women; the use of leukocyte-filtered and irradiated blood for transfusion; the use of magnetic resonance imaging methods (T2) for the management of transfusion-related hemosiderosis; and the implementation of an allogeneic hematopoietic stem cell transplantation protocol for adult patients...
February 2018: Medicine (Baltimore)
Ian C Han, Alice Y Zhang, Tin Y A Liu, Marguerite O Linz, Adrienne W Scott
PURPOSE: To determine whether ultra-widefield (UWF) retinal imaging changes the staging or management of sickle cell retinopathy compared with clinical examination. METHODS: Prospective, observational study including patients with sickle cell disease. All patients underwent dilated fundus examination by a fellowship-trained retina specialist, as well as UWF fundus photography (FF) and fluorescein angiography (FA). Sickle retinopathy stage and treatment recommendation per eye were determined after clinical examination, UWF-FF, and UWF-FA, respectively, and differences in retinopathy stage and treatment recommendation were compared...
January 30, 2018: Retina
Pradeep Padmanabhan, Chikelue Oragwu, Bibhuti Das, John A Myers, Ashok Raj
Pain crisis in children with sickle cell disease (SCD) is typically managed with intravenous fluids and parenteral opioids in the pediatric emergency department. Electrical cardiometry (EC) can be utilized to measure cardiac output (CO) and cardiac index (CI) non-invasively. Near-infrared spectroscopy (NIRS) measuring cerebral (rCO₂) and splanchnic regional (rSO₂) mixed venous oxygenation non-invasively has been utilized for monitoring children with SCD. We studied the value and correlation of NIRS and EC in monitoring hemodynamic status in children with SCD during pain crisis...
January 29, 2018: Children
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