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Sickle Cell Crisis

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https://www.readbyqxmd.com/read/28443234/plastic-bronchitis-an-unusual-complication-of-acute-chest-syndrome-in-adult
#1
Sarah Feray, Pierre Mora, Maxens Decavele, Tài Pham, El Mahdi Hafiani, Muriel Fartoukh
Plastic bronchitis is used to designate endobronchial plugs of rubber-like consistency that form into bronchial trees. It has been described in several diseases like asthma, cystic fibrosis, pulmonary infection, cyanotic congenital heart disease and in few young children with homozygous sickle cell disease. We report the first sickle cell adult case of plastic bronchitis during acute chest syndrome. He developed severe acute respiratory distress syndrome. This unusual presentation related to obstruction by voluminous casts may alert physicians to focus more on the bronchi in sickle cell patients...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28439419/chronic-complications-and-quality-of-life-of-patients-living-with-sickle-cell-disease-and-receiving-care-in-three-hospitals-in-cameroon-a-cross-sectional-study
#2
Anne M Andong, Eveline D T Ngouadjeu, Cavin E Bekolo, Vincent S Verla, Daniel Nebongo, Yannick Mboue-Djieka, Simeon-Pierre Choukem
BACKGROUND: Sickle Cell Disease (SCD) is associated with chronic multisystem complications that significantly influence the quality of life (QOL) of patients early in their life. Although sub-Saharan Africa bears 75% of the global burden of SCD, there is a paucity of data on these complications and their effects on the QOL. We aimed to record these chronic complications, to estimate the QOL, and to identify the corresponding risk factors in patients with SCD receiving care in three hospitals in Cameroon...
2017: BMC Hematology
https://www.readbyqxmd.com/read/28439418/prevention-practices-influencing-frequency-of-occurrence-of-vaso-occlusive-crisis-among-sickle-cell-patients-in-abeokuta-south-local-government-area-of-ogun-state-nigeria
#3
Olorunfemi Emmanuel Amoran, Ahmed Babatunde Jimoh, Omotola Ojo, Temitope Kuponiyi
BACKGROUND: Africa is the most affected continent with 200,000 new born affected by sickle cell anemia annually with of 5% of under five deaths. Nigeria has the largest sickle cell gene pool in the world with about 2% of all babies born to Nigerian parents. This study therefore sets out to assess the prevention practices influencing the frequency of occurrence of vaso-occlusive crisis among patients in Ogun State. METHODS: This study is a descriptive cross-sectional study conducted in Abeokuta South Local Government Area Ogun State...
2017: BMC Hematology
https://www.readbyqxmd.com/read/28426137/hydroxyurea-hydroxycarbamide-for-sickle-cell-disease
#4
REVIEW
Sarah J Nevitt, Ashley P Jones, Jo Howard
BACKGROUND: Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and a reduced life expectancy. Hydroxyurea (hydroxycarbamide), an oral chemotherapeutic drug, ameliorates some of the clinical problems of SCD, in particular that of pain, by raising fetal haemoglobin. This is an update of a previously published Cochrane Review. OBJECTIVES: To assess the effects of hydroxyurea therapy in people with SCD (all genotypes), of any age, regardless of setting...
April 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28413532/spontaneous-extradural-and-subgaleal-hematoma-a-rare-neurosurgical-crisis-of-sickle-cell-disease
#5
Sudhansu S Mishra, Satya B Senapati, Amiya K Gouda, Sanjay K Behera, Ashis Patnaik
Extradural hematoma (EDH) in absence of trauma is a rare entity with only few cases reported in literature. The various causes reported include: Vascular malformation of dura, coagulopathies, sinus infection, middle ear or orbital infection, and tumor. Occurrence of spontaneous EDH as a complication of sickle cell disease is even much rarer. We report a case with sickle cell disease who presented with spontaneous extradural and subgaleal hematomas following an episode of vaso-oclusive crisis. He was managed successfully with surgery...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28409830/magnesium-for-treating-sickle-cell-disease
#6
REVIEW
Nan Nitra Than, Htoo Htoo Kyaw Soe, Senthil K Palaniappan, Adinegara Bl Abas, Lucia De Franceschi
BACKGROUND: Sickle cell disease is an autosomal recessive inherited haemoglobinopathy which causes painful vaso-occlusive crises due to sickle red blood cell dehydration. Vaso-occlusive crises are common painful events responsible for a variety of clinical complications; overall mortality is increased and life expectancy decreased compared to the general population. Experimental studies suggest that intravenous magnesium has proven to be well-tolerated in individuals hospitalised for the immediate relief of acute (sudden onset) painful crisis and has the potential to decrease the length of hospital stay...
April 14, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28397407/sanguinate-pegylated-carboxyhemoglobin-bovine-mechanism-of-action-and-clinical-update
#7
Abraham Abuchowski
Historically, blood substitutes were under development that would provide oxygen carrying capacity as well as fluid replacement for both trauma and surgical indications. Their development was halted by the inability of the products to deliver therapeutic amounts of oxygen targeted to hypoxic tissue as well as from the inherent toxicity of the molecules. This led to the concept of an oxygen therapeutic that would be targeted for indications caused by anemia/ischemia/hypoxia but would not exhibit the toxicity that plagued earlier products...
April 2017: Artificial Organs
https://www.readbyqxmd.com/read/28361592/lactate-dehydrogenase-a-marker-of-the-severity-of-vaso-occlusive-crisis-in-children-with-sickle-cell-disease-presenting-at-the-emergency-department
#8
Marina García-Morin, Carolina López-Sangüos, Paula Vázquez, Teresa Alvárez, Rafael Marañón, Jorge Huerta, Elena Cela
The aim of this study was to describe the characteristics of vaso-occlusive crises (VOC) in children with sickle cell disease and to identify factors associated with greater severity. We performed a prospective observational study from August 2012 to January 2014. The study population comprised patients with sickle cell disease who consulted at the emergency department (ED) for VOC. We recorded demographic variables, history of complications related to the disease, and data on usual treatment. We also assessed pain, analgesia at home, need for admission, length of stay, and analgesia during admission...
November 2016: Hemoglobin
https://www.readbyqxmd.com/read/28356267/hemoglobin-inhibits-albumin-uptake-by-proximal-tubule-cells-implications-for-sickle-cell-disease
#9
Megan L Eshbach, Amandeep Kaur, Youssef Rbaibi, Jesus Tejero, Ora A Weisz
Proximal tubule (PT) dysfunction, including tubular proteinuria, is a significant complication in young sickle cell disease (SCD) that can eventually lead to chronic kidney disease. Hemoglobin (Hb) dimers released from red blood cells upon hemolysis are filtered into the kidney and internalized by megalin/cubilin receptors into PT cells. The PT is especially sensitive to heme toxicity, and tubular dysfunction in SCD is thought to result from prolonged exposure to filtered Hb. Here we show that concentrations of Hb predicted to enter the tubule lumen during hemolytic crisis competitively inhibit the uptake of another megalin/cubilin ligand (albumin) by proximal tubule (PT) cells...
March 29, 2017: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/28334590/a-prospective-emergency-department-quality-improvement-project-to-improve-the-treatment-of-vaso-occlusive-crisis-in-sickle-cell-disease-lessons-learned
#10
Paula Tanabe, Caroline E Freiermuth, David M Cline, Susan Silva
BACKGROUND: Guidelines recommend rapid, aggressive management of vaso-occlusive crisis (VOC) for patients with sickle cell disease (SCD). A large prospective research and quality improvement (QI) project was conducted to measure changes in clinical outcomes in two EDs-academic medical centers with emergency medicine residency programs and Level 1 trauma centers-during a 2.5-year time period (October 2011-March 2014). METHODS: A QI team used a Plan-Do-Study-Act approach to modify and implement changes to opioid analgesic protocols for the emergency department (ED) treatment of VOC...
March 2017: Joint Commission Journal on Quality and Patient Safety
https://www.readbyqxmd.com/read/28323673/clinical-pearls-of-maternal-critical-care-part-2-sickle-cell-disease-in-pregnancy
#11
Vinod Patil, Gamunu Ratnayake, Galina Fastovets
PURPOSE OF REVIEW: The current review outlines the challenges in managing pregnant women with sickle-cell anemia, who are at risk of becoming critically ill during pregnancy. RECENT FINDINGS: Sickle obstetric patients pose unique challenges to the anesthetist and intensivist. We discuss the role of prophylactic transfusions for specific indications like acute anemia and twin pregnancies. The management and prevention of vaso-occlusive crises and chest crisis are also outlined...
March 18, 2017: Current Opinion in Anaesthesiology
https://www.readbyqxmd.com/read/28270345/clinical-and-laboratory-profile-of-patients-with-sickle-cell-anemia
#12
Phelipe Gabriel Dos Santos Sant'Ana, Ariane Moreira Araujo, Cynthia Teixeira Pimenta, Mário Lúcio Pacheco Ker Bezerra, Sílvio Pereira Borges Junior, Viviana Martins Neto, Janaina Sousa Dias, Aline de Freitas Lopes, Danyelle Romana Alves Rios, Melina de Barros Pinheiro
OBJECTIVE: This study aimed to describe and analyze clinical and laboratory characteristics of patients with sickle cell anemia treated at the Hemominas Foundation, in Divinópolis, Brazil. Furthermore, this study aimed to compare the clinical and laboratory outcomes of the group of patients treated with hydroxyurea with those patients that were not treated with hydroxyurea. METHODS: Clinical and laboratorial data were obtained by analyzing medical records of patients with sickle cell anemia...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28267033/factors-influencing-utilization-of-hospital-services-by-adult-sickle-cell-disease-patients-a-systematic-review
#13
Irina Benenson, Yuri Jadotte, Mercedes Echevarria
BACKGROUND: Painful vaso-occlusive crisis is a hallmark of sickle cell disease (SCD) that commonly results in utilization of hospital services. Recurrent use of hospital services by SCD patients is associated with high healthcare costs and adverse clinical outcomes. Understanding the factors influencing the pattern of utilization is a first step in improving medical care of this patient population while reducing healthcare expenditures. OBJECTIVES: The primary objective of this systematic review was to determine what modifiable and non-modifiable factors influence utilization of hospital services by adult SCD patients...
March 2017: JBI Database of Systematic Reviews and Implementation Reports
https://www.readbyqxmd.com/read/28238158/acute-kidney-injury-during-a-pediatric-sickle-cell-vaso-occlusive-pain-crisis
#14
Sujatha Baddam, Inmaculada Aban, Lee Hilliard, Thomas Howard, David Askenazi, Jeffrey D Lebensburger
BACKGROUND: Patients who develop sickle cell disease (SCD) nephropathy are at a high risk for mortality. The pathophysiology of vaso-occlusive pain crisis may contribute to acute kidney injury (AKI). Non-steroidal anti-inflammatory drugs, known inducers of AKI, are used to treat pain crises. Multiple gaps exist in the knowledge about the impact of AKI in SCD. METHODS: We conducted a 2-year retrospective review of AKI events in patients admitted for vaso-occlusive crisis...
February 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28221266/body-mass-index-and-the-association-with-vaso-occlusive-crises-in-pediatric-sickle-cell-disease
#15
Andrea Zivot, Nataly Apollonsky, Edward Gracely, Deepti Raybagkar
BACKGROUND: Children with sickle cell disease (SCD) historically have been underweight and have poor overall growth. Recent studies have demonstrated a trend toward obesity in pediatric SCD populations. MATERIALS AND METHODS: Through retrospective chart review of patients with SCD followed at our center, we collected patient's data, including body mass index (BMI), weight percentiles, sickle cell genotype, baseline hemoglobin, medical and psychiatric comorbidities, 25-hydroxy vitamin D level, treatment with hydroxyurea, and chronic transfusions...
May 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28192225/a-randomized-trial-of-yoga-for-children-hospitalized-with-sickle-cell-vaso-occlusive-crisis
#16
Karen Moody, Bess Abrahams, Rebecca Baker, Ruth Santizo, Deepa Manwani, Veronica Carullo, Doris Eugenio, Aaron Carroll
CONTEXT: Sickle cell disease (SCD) vaso-occlusive crisis (VOC) remains an important cause of acute pain in pediatrics and the most common SCD complication. Pain management recommendations in SCD include nonpharmacological interventions. Yoga is one nonpharmacological intervention that has been shown to reduce pain in some populations, however, evidence is lacking in children with VOC. OBJECTIVES: The primary objective of this study was to compare the effect of yoga versus an attention control on pain in children with VOC...
February 9, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/28185829/a-systematic-review-of-the-literature-for-severity-predictors-in-children-with-sickle-cell-anemia
#17
Emily Riehm Meier, Ross M Fasano, Paul R Levett
All patients with HbSS (SCA) share the same genetic mutation but the clinical phenotype is variable and difficult to predict early in life. A reliable severity predictor would be invaluable toward directing therapeutic decisions in those patients at highest risk of SCA complications. A search of PubMed, Cochrane Clinical Trials Register, and Scopus was performed to determine which SCA severity predictors have been validated in pediatric patients. The full text of 94 of the 590 references identified was reviewed based on the title/abstract...
February 2, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28162515/-280-interleukin-1alpha-rs1800587-associates-with-chronic-non-crisis-pain-in-sickle-cell-disease
#18
X Hu, E Jhun, Y Yao, Y He, R Molokie, D Wilkie, Z Wang
No abstract text is available yet for this article.
April 2016: Journal of Pain: Official Journal of the American Pain Society
https://www.readbyqxmd.com/read/28159390/sickle-cell-disease
#19
REVIEW
Russell E Ware, Mariane de Montalembert, Léon Tshilolo, Miguel R Abboud
Sickle cell disease is a common and life-threatening haematological disorder that affects millions of people worldwide. Abnormal sickle-shaped erythrocytes disrupt blood flow in small vessels, and this vaso-occlusion leads to distal tissue ischaemia and inflammation, with symptoms defining the acute painful sickle-cell crisis. Repeated sickling and ongoing haemolytic anaemia, even when subclinical, lead to parenchymal injury and chronic organ damage, causing substantial morbidity and early mortality. Currently available treatments are limited to transfusions and hydroxycarbamide, although stem cell transplantation might be a potentially curative therapy...
January 31, 2017: Lancet
https://www.readbyqxmd.com/read/28133359/myonecrosis-in-sickle-cell-anemia-case-study
#20
Lalita Prabha Turaga, Prajwal Boddu, Steve Kipferl, Anupam Basu, Martin Yorath
BACKGROUND Myonecrosis is one of the more poorly studied, painful manifestations of sickle cell crisis. Medical literature is sparse detailing the manifestations and management of such symptoms. In myonecrosis, red cells containing sickle hemoglobin become rigid, resulting in reduced blood flow and myonecrosis. CASE REPORT We present a case study of a patient in sickle cell crisis with an episode of acute pain and swelling to the intrinsic muscles of the foot as a prominent feature of the crises. Although muscle biopsy is considered the gold standard for the diagnosis of myositis or myonecrosis, a low intensity signal on T1 and high intensity signal on T2 at the affected muscle belly can be as conclusive as imaging studies...
January 30, 2017: American Journal of Case Reports
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