keyword
https://read.qxmd.com/read/38418238/sickle-eye-project-a-cross-sectional-non-interventional-study-of-the-prevalence-of-visual-impairment-due-to-sickle-cell-retinopathy-and-maculopathy-in-the-uk
#21
JOURNAL ARTICLE
Christiana Dinah, Konstantinos Balaskas, Brigit Greystoke, Rossby Awadzi, Peter Beke, Roger Ahern, James Talks
INTRODUCTION: Sickle cell disease (SCD) is one of the most common genetic disorders in the UK, with over 15 000 people affected. Proliferative sickle cell retinopathy (SCR) is a well-described complication of SCD and can result in significant sight loss, although the prevalence in the UK is not currently known. There are currently no national screening guidelines for SCR, with wide variations in the management of the condition across the UK. METHODS AND ANALYSIS: The Sickle Eye Project is an epidemiological, cross-sectional, non-interventional study to determine the prevalence of visual impairment due to SCR and/or maculopathy in the UK...
February 28, 2024: BMJ Open
https://read.qxmd.com/read/38412482/development-of-a-hematology-general-medicine-hybrid-team-to-improve-care-of-patients-with-sickle-cell-disease
#22
JOURNAL ARTICLE
Lauren E Merz, Miriam A Osei, Aliza Green, Charlotte M Story, Kelly M Schuering, Maureen Okam Achebe
PROBLEM: Approximately 100,000 individuals in the United States have sickle cell disease (SCD). These individuals face multiple barriers to equitable care. At Brigham and Women's Hospital, existing health inequities for these patients were compounded by admitting, rounding, and team structures that assigned patients with SCD to multiple medicine teams with a hematologist attending, leading to delays in patient care and gaps in residents' hematology knowledge. APPROACH: A hematology-general medicine hybrid team was created in September 2021 to enhance trainee knowledge, skill, and confidence in managing hematology conditions and improve the quality of care delivered to individuals with SCD...
February 27, 2024: Academic Medicine
https://read.qxmd.com/read/38408160/recurrent-nontraumatic-subgaleal-hematomas-in-a-pediatric-patient-with-sickle-cell-disease
#23
JOURNAL ARTICLE
Irtiza N Sheikh, Olayinka Okeleji, Rabya Afzal, Eliana Bonfante, Monica Kodakandla, Neethu M Menon
Spontaneous subgaleal hematoma in pediatric patients with sickle cell disease (SCD) is a rare occurrence that can present with symptoms mimicking ischemic stroke, a known complication of SCD. However, unlike ischemic stroke, subgaleal hematoma is nonlethal and can be managed conservatively without major sequelae. Here, we present the case of an adolescent with SCD who presented with 2 episodes of subgaleal and epidural hematomas, 2 years apart. The latter episode occurred while on crizanlizumab, an anti-P-selectin antibody, approved for use in SCD in 2019 to reduce the number of acute pain crises...
February 19, 2024: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/38406507/patient-and-clinician-beliefs-about-potential-barriers-to-treatment-of-neuropathic-pain-for-adolescents-with-sickle-cell-disease
#24
JOURNAL ARTICLE
Matthew Rees, Holly Spraker-Perlman, Raechyl Moore, Paul Lavoie, Linda Schiff, Jennifer M Allen, Parul Rai, Doralina L Anghelescu
Pain is the hallmark symptom causing morbidity for people with sickle cell disease (SCD) and may present as nociceptive, neuropathic, or mixed type pain. Neuropathic pain (NP) is underrecognized and undertreated in patients with SCD and is associated with decreased patient-reported quality of life. Surveys were completed by clinicians caring for adolescents with SCD in the outpatient setting. SCD patients ages 1418 at increased risk of NP completed a patient-facing survey at a scheduled clinic visit. Ninety-four percent of responding clinicians agreed that NP significantly contributes to reported pain in SCD...
February 2024: EJHaem
https://read.qxmd.com/read/38394231/sickle-cell-disease-in-an-older-adult-population-a-retrospective-review-of-health-care-resource-utilization
#25
JOURNAL ARTICLE
Jessica L Ryan, Jeremiah S Rastegar, Jessica M Dobbins, Deborah N Peikes, Anna Theodorou, Brian Garcia, Bryan Loy, Ebony Bell, J Nwando Olayiwola
Sickle cell disease (SCD) has a history of health inequity, as patients with SCD are primarily Black and often marginalized from the health care system. Although recent health care and treatment advancements have prolonged life expectancy, it may be insufficient to support the complex needs of the growing population of older adults with SCD. This retrospective study used a cohort ( N  = 812) of Medicare Advantage beneficiaries 45 years and older (ages: 45-54, 55-64, 65-74, 75-89) with SCD to identify associations of SCD-related complications and comorbidities with emergency department (ED) visits, potentially avoidable ED visits, all-cause hospitalization, and potentially avoidable hospitalizations, 2018-2020...
February 23, 2024: Population Health Management
https://read.qxmd.com/read/38391334/glucose-6-phosphate-dehydrogenase-deficiency-with-coinherited-gaucher-disease-a-rare-association
#26
JOURNAL ARTICLE
Nupur Parakh, Kusha Sharma, Sunita Sharma, Priti Chatterjee, Varinder Singh
Anemia coexisting with Gaucher disease (GD) is often associated with non-hemolytic processes. Few cases of GD with autoimmune hemolytic anemia have been reported. However, literature on GD with concomitant nonimmune hemolytic anemia is scarce. A 1-year 6-month-old male child presented in 2018 with complaints of palpable mass in left upper abdomen, fever, cough, and vomiting. On examination, he had pallor, hepatosplenomegaly of 2 cm and 8 cm below costal margin, respectively. A clinical diagnosis of hemolytic anemia was suspected...
July 6, 2023: Indian Journal of Pathology & Microbiology
https://read.qxmd.com/read/38388223/the-role-of-preoperative-transfusion-in-sickle-cell-disease-a-systematic-review-and-meta-analysis
#27
REVIEW
Yasamin Abdu, Alaa Rahhal, Khalid Ahmed, Nada Adli, Mariam Abdou, Elrazi Awadelkarim Hamid Ali, Salam Al-Kindi, Mona Al Rasheed, Jaffer Altooq, Iheb Bougmiza, Mohamed A Yassin
This systematic review and meta-analysis aimed to provide guidance on preoperative blood transfusion strategies for patients with sickle cell disease (SCD). We included all randomized controlled and observational studies exploring the clinical outcomes of preoperative blood transfusion among patients with SCD compared to the conservative transfusion strategy until 14/09/2022. Sixteen studies involving 3486 participants were analysed. The findings revealed a significantly higher bleeding rate in patients who received preoperative transfusion than those who followed a conservative strategy (RR = 4...
February 18, 2024: Blood Reviews
https://read.qxmd.com/read/38384294/soluble-guanylate-cyclase-stimulators-and-activators-new-horizons-in-the-treatment-of-priapism-associated-with-sickle-cell-disease
#28
REVIEW
Dalila Andrade Pereira, Tammyris Helena Rebecchi Silveira, Fabiano Beraldi Calmasini, Fábio Henrique Silva
Priapism, defined as a prolonged and often painful penile erection occurring without sexual stimulation or desire, is a common complication in sickle cell disease (SCD), affecting up to 48% of male patients. This condition presents significant clinical challenges and can lead to erectile dysfunction if not properly managed. Current pharmacological treatments for SCD-related priapism are primarily reactive rather than preventative, highlighting a gap in effective medical intervention strategies. A critical factor in developing priapism is the reduced basal bioavailability of nitric oxide (NO) and cyclic guanosine monophosphate (cGMP) in erectile tissues...
2024: Frontiers in Pharmacology
https://read.qxmd.com/read/38374865/knowledge-of-sickle-cell-disease-among-medical-students-at-al-baha-university-saudi-arabia-a-cross-sectional-study
#29
JOURNAL ARTICLE
Saleh Saad J Alzahrani, Nawaf Saleh A Alghamdi, Eyad Awad M Alzahrani, Fadi Ahmed M Alzahrani, Mohammed Ahmed A Alghamdi, Zaher Musleh S Hafiz, Amr A Fouad
BACKGROUND: Sickle cell disease (SCD) presents a major health challenge in Saudi Arabia due to its high prevalence. The important role of medical students as future healthcare leaders necessitates high awareness and knowledge about the disease. AIM: To assess SCD awareness among Al-Baha University medical students, and to evaluate its relation to gender and academic level. METHODS: A cross-sectional study was conducted, including 105 medical students from the first to sixth year at Al-Baha University...
January 2024: Curēus
https://read.qxmd.com/read/38374470/perceptions-and-preferences-for-genetic-testing-for-sickle-cell-disease-or-trait-a-qualitative-study-in-cameroon-ghana-and-tanzania
#30
JOURNAL ARTICLE
Nchangwi Syntia Munung, Karen Kengne Kamga, Marsha J Treadwell, Jemima Dennis-Antwi, Kofi A Anie, Daima Bukini, Julie Makani, Ambroise Wonkam
Sickle cell disease (SCD) is a single gene blood disorder characterised by frequent episodes of pain, chronic anaemic, acute chest syndrome, severe disease complications and lifelong debilitating multi-system organ damage. Genetic testing and screening programs for SCD and the sickle cell trait (SCT) are valuable for early diagnosis and management of children living with SCD, and in the identification of carriers of SCT. People with SCT are for the most part asymptomatic and mainly identified as through genetic testing or when they have a child with SCD...
February 19, 2024: European Journal of Human Genetics: EJHG
https://read.qxmd.com/read/38358434/ovarian-tissue-cryopreservation-for-fertility-preservation-before-hematopoietic-stem-cell-transplantation-in-patients-with-sickle-cell-disease-safety-ovarian-function-follow-up-and-results-of-ovarian-tissue-transplantation
#31
JOURNAL ARTICLE
Mitawa Millin Missontsa, Françoise Bernaudin, Anne Fortin, Nathalie Dhédin, Corinne Pondarré, Karima Yakouben, Bénédicte Neven, Martin Castelle, Marina Cavazzana, Harry Lezeau, Matthieu Peycelon, Annabel Paye-Jaouen, Jeremy Sroussi, Tamara Diesch-Furlanetto, Virginie Barraud-Lange, Sabine Sarnacki, Mony Fahd, Isis Marchand, Clémence Delcour, Dominique Vexiau, Jean-Benoît Arlet, Annie Kamdem, Cécile Arnaud, Jean-Hugues Dalle, Catherine Poirot
PURPOSE: To describe the experience of performing ovarian tissue cryopreservation (OTC) before hematopoietic stem cell transplantation (HSCT), among girls/women with severe sickle cell disease (SCD)(SS or S/β0 -thalassemia) who are, besides the usual surgical risk, at risk of SCD-related complications during the fertility preservation procedure for improving their counseling and management. METHODS: This retrospective study included 75 patients (girls/women) with SCD who have had OTC before myeloablative conditioning regimen (MAC) for HSCT...
February 15, 2024: Journal of Assisted Reproduction and Genetics
https://read.qxmd.com/read/38353144/maldi-ms-in-first-line-screening-of-newborns-for-sickle-cell-disease-results-from-a-prospective-study-in-comparison-to-hplc
#32
JOURNAL ARTICLE
Marven El Osta, Jean-François Benoist, Pierre Naubourg, Stéphane Bonacorsi, Reine Messine, Patrick Ducoroy, Bichr Allaf
OBJECTIVES: Newborn screening (NBS) for sickle cell disease (SCD) requires a robust, high-throughput method to detect hemoglobin S (HbS). Screening for SCD is performed by qualitative methods, such as isoelectric focusing (IEF), and both qualitative and quantitative methods such as high performance liquid chromatography (HPLC), capillary electrophoresis (CE), and tandem mass spectrometry (MS/MS). All these methods detect HbS, as well as low-level or absent HbA, and also other variants of hemoglobin...
February 5, 2024: Clinical Chemistry and Laboratory Medicine: CCLM
https://read.qxmd.com/read/38352919/psychosis-secondary-to-covid-19-in-pediatric-sickle-cell-disease
#33
Ashish Khanchandani, Chinonso Onuoha, Beng Fuh
KEY CLINICAL MESSAGE: COVID-19 psychosis is a potential long-term sequela of COVID-19. Vulnerable populations, such as individuals with sickle cell disease, are at high risk for psychosis. Given the limited number of cases, more investigations in the etiopathology and management of this new disease is needed. ABSTRACT: We report a case of a 15-year-old female with a past medical history of depression who developed psychosis post-SARS-CoV-2 infection (COVID-19). After an initial moderate COVID-19 infection, the patient appeared to recover and was discharged home...
February 2024: Clinical Case Reports
https://read.qxmd.com/read/38345709/proliferative-sickle-cell-retinopathy-a-patient-and-a-physician-s-perspective-on-quality-of-life-and-quality-of-eye-care
#34
JOURNAL ARTICLE
Chanel Taylor, Rossby Awadzi, Jamie Enoch, Christiana Dinah
The impact of visual impairment in the context of sickle cell disease is poorly understood. Despite the significant advancements over the past three decades in retinal imaging and in the understanding of molecular mechanisms that drive retinal neovascularization, there has been little improvement in the management of proliferative sickle cell retinopathy. This article is co-authored by a patient impacted by proliferative sickle cell retinopathy. She highlights her personal experience of sight loss from proliferative sickle cell retinopathy and the impact on her daily life and mental health...
February 12, 2024: Ophthalmology and Therapy
https://read.qxmd.com/read/38335412/implications-of-climatic-change-on-sickle-cell-anemia-a-review
#35
JOURNAL ARTICLE
Emmanuel Ifeanyi Obeagu, Getrude Uzoma Obeagu
Sickle cell anemia (SCA) is a hereditary blood disorder characterized by abnormal hemoglobin, causing red blood cells to assume a sickle shape, leading to various complications. Climate change has emerged as a significant global challenge, influencing environmental conditions worldwide. This paper explores the implications of climatic variations on the prevalence, management, and outcomes of SCA. Climate change affects weather patterns, leading to altered temperatures, increased frequency of extreme weather events, and variations in humidity levels...
February 9, 2024: Medicine (Baltimore)
https://read.qxmd.com/read/38330698/chronic-humeral-osteomyelitis-in-an-adult-with-sickle-cell-disease
#36
Pablo Viñuales, Paola Andrea Hortua, Jordi Zafra, Ramón Clos, Jordi Villalba
INTRODUCTION AND IMPORTANCE: The rising incidence of sickle-cell disease in European countries has led to an increase in associated complications. Osteomyelitis, a rare complication in non-traumatic adult cases, poses diagnostic challenges and presents treatment difficulties due to limited cases and studies. CASE PRESENTATION: A 23-year-old woman diagnosed with sickle-cell disease presented with a six-day fever and painful swelling in the left upper extremity persisting for a fortnight...
January 26, 2024: International Journal of Surgery Case Reports
https://read.qxmd.com/read/38326179/consensus-of-the-brazilian-association-of-hematology-hemotherapy-and-cellular-therapy-abhh-and-the-brazilian-ministry-of-health-general-management-of-blood-and-blood-products-on-the-tests-necessary-for-the-release-of-exceptional-medicines-for-sickle-cell-disease
#37
JOURNAL ARTICLE
Clarisse Lobo, Aderson Araújo, Alexandre de Albuquerque Antunes, Ana Cristina Silva Pinto, Ariadne Carvalho Godinho, Cassia Silvestre Mariano Pires, Cinthia Cristina Matheus, Xerez de Albuquerque, Daniele Campos Fontes Neves, Fábio de Lima Moreno, Giorgio Baldanzi, Grazziella Curado Siufi, Heloisa Helena Pereira Miranda, Jane Hankins, Joice Aragão, Josefina Aparecida Pellegrini Braga, Juliana Touguinha Neves Martins, Luciana Campos Costa Machado de Souza, Maria Stella Figueiredo, Mirella Rodrigues Oliveira, Patricia Santos Resende Cardoso, Patricia Costa Alves Pinto, Patricia Gomes Moura, Rodolfo Delfino Cançado, Paulo Ivo Cortez de Araujo, Sara Olalla Saad, Sandra Regina Loggetto, Teresa Cristina Cardoso Fonseca
To date, hydroxyurea is the only effective and safe drug that significantly reduces morbidity and mortality of individuals with Sickle cell disease. Twenty years of real-life experience has demonstrated that hydroxyurea reduces pain attacks, vaso-occlusive events, including acute chest syndrome, the number and duration of hospitalizations and the need for transfusion. The therapeutic success of hydroxyurea is directly linked to access to the drug, the dose used and adherence to treatment which, in part, is correlated to the availability of hydroxyurea...
February 1, 2024: Hematology, Transfusion and Cell Therapy
https://read.qxmd.com/read/38316197/nitric-oxide-a-potential-etiological-agent-for-vaso-occlusive-crises-in-sickle-cell-disease
#38
REVIEW
Parul Gupta, Ravindra Kumar
Nitric oxide (NO), a vasodilator contributes to the vaso-occlusive crisis associated with the sickle cell disease (SCD). Vascular nitric oxide helps in vasodilation, controlled platelet aggregation, and preventing adhesion of sickled red blood cells to the endothelium. It decreases the expression of pro-inflammatory genes responsible for atherogenesis associated with SCD. Haemolysis and activated endothelium in sickle cell patients reduce the bioavailability of NO which promotes the severity of sickle cell disease mainly causes vaso-occlusive crises...
February 3, 2024: Nitric Oxide: Biology and Chemistry
https://read.qxmd.com/read/38310045/bloodless-management-of-significantly-elevated-transcranial-doppler-velocity-value-in-a-jehovah-s-witness-child-with-sickle-cell-disease-a-tertiary-centre-experience-a-case-report
#39
Chisom Adaobi Nri-Ezedi, Thomas Ulasi, Chilota Chibuife Efobi, John Chinawaeze Aneke, Nwanneka Ugwu, Chinekwu Nwosu
BACKGROUND: Effective management of complications in sickle cell disease (SCD), such as stroke prevention, often necessitates the use of blood transfusions. However, individuals who adhere to the religious tenets of Jehovah's Witnesses strictly abstain from accepting blood transfusions, thereby presenting a formidable challenge in clinical decision-making. CASE REPORT: This is a case of a 3 year old child Jehovah's Witness who was found to have significantly elevated transcranial Doppler (TCD) velocity values between 193 and 203 cm/s, following routine screening...
February 2, 2024: Journal of the National Medical Association
https://read.qxmd.com/read/38310012/dermatology-in-black-skin
#40
JOURNAL ARTICLE
Maurício Mota de Avelar Alchorne, Katleen da Cruz Conceição, Leonardo Lora Barraza, Marilda Aparecida Milanez Morgado de Abreu
The vast majority of publications in dermatology refer to lightly pigmented skin, with few addressing the peculiarities of black skin. In addition there is no consensus on what it means to be black in different regions of the world. The lack of knowledge on the subject makes it difficult to recognize and manage dermatoses in this type of skin. This article aims to review the literature on intrinsic characteristics, as well as epidemiological and clinical aspects of the cutaneous manifestations of different dermatoses in black skin...
February 2, 2024: Anais Brasileiros de Dermatologia
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