keyword
https://read.qxmd.com/read/38394231/sickle-cell-disease-in-an-older-adult-population-a-retrospective-review-of-health-care-resource-utilization
#21
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/38388223/the-role-of-preoperative-transfusion-in-sickle-cell-disease-a-systematic-review-and-meta-analysis
#22
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
#23
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/38374470/perceptions-and-preferences-for-genetic-testing-for-sickle-cell-disease-or-trait-a-qualitative-study-in-cameroon-ghana-and-tanzania
#24
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/38335412/implications-of-climatic-change-on-sickle-cell-anemia-a-review
#25
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
#26
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
#27
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/38302123/management-of-acute-complications-of-sickle-cell-disease
#28
REVIEW
Sabina Shamayeva, Perry Morocco
Sickle cell disease (SCD) is a group of inherited autosomal recessive disorders that affect hemoglobin structure. The presence of this mutated form of hemoglobin, hemoglobin S, results in the abnormally ("sickle") shaped cells. These sickle-shaped red blood cells lead to the disruption of blood flow in small vessels and result in a myriad of complications. Pain, excruciating and unpredictable, is the hallmark of the disease. In addition, many organs are affected, including but not limited to brain, kidneys, bones, and lungs...
February 2024: Pediatric Annals
https://read.qxmd.com/read/38290537/individualized-homeopathic-and-organopathic-supportive-management-of-sickle-cell-disorder-a-case-series-of-six-patients-from-a-particularly-vulnerable-tribal-group-in-india
#29
JOURNAL ARTICLE
Nisanth K M Nambison, Narendra Sharma, Abhishek D Dwivedi, Nisha Chakravarty
BACKGROUND:  Sickle cell disorder (SCD) is a hereditary blood disease characterized by an abnormality in the oxygen-carrying protein hemoglobin present in red blood cells. Genetic abnormality causes these cells to become sickle-shaped, with shorter lifespan. Vaso-occlusive crisis is a major symptom of SCD: it is a sudden and severe episode of pain, and occurs when sickle-shaped cells block blood flow. This blockage can lead to tissue damage, inflammation and pain. OBJECTIVES:  This case series aims to observe the clinical outcomes from prescribing individualized homeopathic medicines along with organopathic supportive medicine in the management of SCD through the analysis of case studies of six patients from a particularly vulnerable tribal group (PVTG) in India that manifests genetic predisposition for the disease...
January 30, 2024: Homeopathy: the Journal of the Faculty of Homeopathy
https://read.qxmd.com/read/38283498/wilson-s-disease-in-a-17-year-old-male-with-sickle-cell-trait-a-report-of-a-rare-case
#30
Aditya Jain, Revat J Meshram, Sham Lohiya, Dinesh V Hinge, Shailesh Wandile
This case report describes the atypical presentation of Wilson's disease in a 17-year-old male with sickle cell trait AS pattern. The patient initially presented with fever, generalized weakness, and joint pain, leading to an inconclusive diagnosis and unsuccessful initial treatment. A comprehensive re-evaluation revealed vitamin-A deficiency, adenoid hypertrophy, splenomegaly, and acalculous cholecystitis. Elevated copper levels in the 24-hour urine test confirmed the diagnosis of Wilson's disease. Treatment was modified to include amikacin, prednisolone, and Zinconia®, with analgesics for joint pain management...
December 2023: Curēus
https://read.qxmd.com/read/38174872/sleep-experiences-of-parents-of-children-18-years-or-younger-with-sickle-cell-disease-during-the-covid-19-pandemic-a-qualitative-study
#31
JOURNAL ARTICLE
Jennel C Osborne, Zainab Toteh Osakwe, Michelle Odlum, Aziza Sedrak
STUDY OBJECTIVES: The COVID-19 Pandemic has highlighted disparities in healthcare. Parents assist in sickle cell disease management in children. Understanding sleep in parents of children who are diagnosed with sickle cell disease is an important facet of disease management. Our objectives were to identify sleep characteristics of parents of children with sickle cell disease aged 18 years or younger during the COVID-19 pandemic, to investigate measures used by parents to achieve restful sleep, and to discuss how sleep may be improved in parents of children diagnosed with sickle cell disease...
January 4, 2024: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://read.qxmd.com/read/38162896/palliative-care-needs-and-barriers-in-an-urban-ugandan-emergency-department-a-mixed-methods-survey-of-emergency-healthcare-workers-and-patients
#32
JOURNAL ARTICLE
Eleanor Reid, Michael Lukoma, Dao Ho, Peace Bagasha, Mhoira Leng, Liz Namukwaya
BACKGROUND: Palliative Care offers patient-centered, symptom-focused relief for patients with incurable disease, and early integration of palliative care ensures quality of life and death while reducing medical impoverishment. The Emergency Department is an ideal yet understudied, under-utilized location to initiate palliative care. OBJECTIVE: To evaluate the palliative care needs of patients with incurable disease and perceived barriers amongst healthcare providers in the Emergency Department of Kiruddu National Referral Hospital, Kampala, Uganda...
December 2023: African Journal of Emergency Medicine Revue
https://read.qxmd.com/read/38161830/elective-laparoscopic-cholecystectomy-complicated-by-hemorrhagic-crisis-in-a-patient-with-sickle-cell-disease
#33
Abdulaziz Alshehri, Anwar Alfadhel, Abdullah AlZahrani, Yousif Alqahtani, Abdulaziz Al Qahtani
Sickle cell disease (SCD) is a common genetic disorder associated with complications such as cholelithiasis. Cholecystectomy is often performed in SCD patients, but they have a higher risk of postoperative complications. Blood transfusion, while beneficial, can also have adverse effects. The optimal approach to perioperative transfusion in SCD patients is still debated. This case report presents a rare surgical presentation of gallbladder stones in an SCD patient complicated by a hemolysis crisis and bleeding after laparoscopic cholecystectomy...
November 2023: Curēus
https://read.qxmd.com/read/38161155/management-of-acute-sickle-cell-disease-pain
#34
JOURNAL ARTICLE
Jason N Payne, Beatrice E Gee
Pain is a common complication of sickle cell disease. Sickle cell pain can often be effectively managed by pediatricians in outpatient and hospital settings. Acute pain management should be initiated quickly. Patients need to be evaluated for sickle cell complications and other causes of pain. Nonsteroidal anti-inflammatory drugs and opioids are the mainstay of pain treatment, but additional therapies include hydration, local pain control, muscle relaxants, and nonpharmacologic approaches. Healthy lifestyle habits and good behavioral and mental health are important for preventing and coping with sickle cell disease pain...
January 1, 2024: Pediatrics in Review
https://read.qxmd.com/read/38138299/pain-control-for-sickle-cell-crisis-a-novel-approach-a-retrospective-study
#35
JOURNAL ARTICLE
Amélie Rollé, Elsa Vidal, Pierre Laguette, Yohann Garnier, Delphine Delta, Frédéric Martino, Patrick Portecop, Maryse Etienne-Julan, Pascale Piednoir, Audrey De Jong, Marc Romana, Emmanuelle Bernit
Background and Objectives : Pain management poses a significant challenge for patients experiencing vaso-occlusive crisis (VOC) in sickle cell disease (SCD). While opioid therapy is highly effective, its efficacy can be impeded by undesirable side effects. Local regional anesthesia (LRA), involving the deposition of a perineural anesthetic, provides a nociceptive blockade, local vasodilation and reduces the inflammatory response. However, the effectiveness of this therapeutic approach for VOC in SCD patients has been rarely reported up to now...
December 18, 2023: Medicina
https://read.qxmd.com/read/38131942/formulation-of-entandrophragma-utile-into-an-herbal-emulgel-for-the-management-of-inflammation
#36
JOURNAL ARTICLE
Adeola Tawakalitu Kola-Mustapha, Haneefat Folashade Ibraheem, Suleiman Taiwo, Ismail O Ishola, Sukurat Olasumbo Usman, Yusuf Oluwagbenga Ghazali
Introduction: Globally, the incidence of inflammation and inflammatory disorders has continued to rise at an alarming rate. Entandrophragma utile is a species of flowering plant widely distributed in Africa and has been used for the management of sickle cell disease, rheumatism, ocular inflammation, duodenal and stomach ulcers. This research aims to formulate and evaluate an anti-inflammatory herbal emulgel using an extract from Entandrophragma utile stem bark (EUB). Method: Using a carrageenan-induced paw oedema model, the anti-inflammatory efficacy of EUB the extract was assessed...
December 6, 2023: Gels
https://read.qxmd.com/read/38107341/presentation-and-challenges-in-management-of-covid-19-in-sickle-cell-disease-case-report
#37
Sheliza Parvez Thaver, Saliha Shafik Dawood, Nahida Zahir Walli
Sickle cell has predominantly been a disease in the sub-Saharan Africa. There is scanty data in Africa and none in Tanzania regarding patients with sickle cell disease infected with COVID-19 especially in the paediatric group. This is concerning because a large population of children living with sickle cell disease are found in this part of the world where scarcity of resources also prevail. This is the first paediatric case of sickle cell disease and COVID-19 reported in Tanzania; highlighting the presentation and challenges faced in management...
2023: Pan African Medical Journal
https://read.qxmd.com/read/38071276/time-sensitive-healthcare-guidelines-for-youth-with-chronic-diseases-in-custody-gaps-in-care
#38
JOURNAL ARTICLE
Colin Dickens, Ahalya Ramesh, Temiloluwa Adanlawo, Michael R DeBaun
On May 9th, 2023, a U.S. Border Patrol detained a family of five near Brownsville, TX. During processing, one of the family members, an eight-year-old girl, ADRA, was noted to have sickle cell anemia and a heart disease condition. Five days after they arrived at the Donna Facility, on May 14th, ADRA displayed symptoms, including abdominal pain and fever, and tested positive for Influenza A. She was administered medication and transferred to a designated isolation unit at the Harlingen Border Patrol Station...
December 9, 2023: Pediatric Research
https://read.qxmd.com/read/38064543/sickle-cell-disease-in-australia-a-snapshot-from-the-australian-haemoglobinopathy-registry
#39
JOURNAL ARTICLE
Anna Nelson, P Joy Ho, Helen Haysom, Neil Waters, Cameron Wellard, Melissa Chee, Juliana Teo, Anthea Greenway, Kylie Mason, Giselle Kidson-Gerber, Zane Kaplan, Tina Carter, Merrole F Cole-Sinclair, Pasquale Barbaro, Erica M Wood
BACKGROUND: Sickle cell disease (SCD) is the most common monogenic disorder worldwide. In deoxygenated conditions, the altered beta chain (haemoglobin S [HbS]) polymerises and distorts the erythrocyte, resulting in pain crises, vasculopathy and end-organ damage. Clinical complications of SCD cause substantial morbidity, and therapy demands expertise and resources. Optimising care for patients and planning resource allocation for the future requires an understanding of the disease in the Australian population...
December 8, 2023: Internal Medicine Journal
https://read.qxmd.com/read/38058524/long-term-opiate-therapy-induced-secondary-adrenal-insufficiency-a-distinct-differential-diagnosis-that-should-be-considered
#40
Amal Mohamed Khair
Pain management with opioid medication is associated with several side effects. Opioid-induced adrenal insufficiency by suppression of the hypothalamic-pituitary-adrenal (HPA) axis is one of them that needs to be considered. The possible effects of opioid use on adrenal function are addressed in this case report. This is a case of a 21-year-old female patient with sickle cell disease who started, for the last year, on extended-release morphine sulfate 45mg daily in an attempt to control the severity of her pain and frequent admission with the vaso-occlusive crisis...
December 2023: Curēus
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