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Sickle Cell Crisis

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https://www.readbyqxmd.com/read/28097791/alpha-thalassaemia-promotes-frequent-vaso-occlusive-crises-in-children-with-sickle-cell-anaemia-through-haemorheological-changes
#1
Céline Renoux, Philippe Connes, Elie Nader, Sarah Skinner, Camille Faes, Marie Petras, Yves Bertrand, Nathalie Garnier, Daniela Cuzzubbo, Lydia Divialle-Doumdo, Kamila Kebaïli, Cécile Renard, Alexandra Gauthier, Maryse Etienne-Julan, Giovanna Cannas, Cyril Martin, Marie-Dominique Hardy-Dessources, Vincent Pialoux, Marc Romana, Philippe Joly
BACKGROUND: Sickle cell anaemia (SCA) is a severe hereditary haemoglobinopathy characterised by haemorheological abnormalities, which play a role in the occurrence of several acute and chronic clinical complications. While β(S) -haplotypes and alpha-thalassaemia modulate SCA clinical severity, their effects on blood rheology have been incompletely described. The aim of this study was to test the effects of these genetic modifiers on the haemorheological properties and clinical complication of children with SCA...
January 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28097236/lung-vaso-occlusion-in-sickle-cell-disease-mediated-by-arteriolar-neutrophil-platelet-microemboli
#2
Margaret F Bennewitz, Maritza A Jimenez, Ravi Vats, Egemen Tutuncuoglu, Jude Jonassaint, Gregory J Kato, Mark T Gladwin, Prithu Sundd
In patients with sickle cell disease (SCD), the polymerization of intraerythrocytic hemoglobin S promotes downstream vaso-occlusive events in the microvasculature. While vaso-occlusion is known to occur in the lung, often in the context of systemic vaso-occlusive crisis and the acute chest syndrome, the pathophysiological mechanisms that incite lung injury are unknown. We used intravital microscopy of the lung in transgenic humanized SCD mice to monitor acute vaso-occlusive events following an acute dose of systemic lipopolysaccharide sufficient to trigger events in SCD but not control mice...
January 12, 2017: JCI Insight
https://www.readbyqxmd.com/read/28050373/histopathologic-findings-in-autopsies-with-emphasis-on-interesting-and-incidental-findings-a-pathologist-s-perspective
#3
Sapna Patel, B R Rajalakshmi, G V Manjunath
INTRODUCTION: Autopsy aids to the knowledge of pathology by unveiling the rare lesions which are a source of learning from a pathologist's perspective Some of them are only diagnosed at autopsy as they do not cause any functional derangement. This study emphasizes the various incidental lesions which otherwise would have been unnoticed during a person's life. AIM: The aim of this study was to determine the spectrum of histopathological findings including neoplastic lesions related or unrelated to the cause of death...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28027139/acute-vaso-occlusive-crisis-in-patients-with-sickle-cell-disease
#4
Megan B Lentz, Donald D Kautz
No abstract text is available yet for this article.
January 2017: Nursing
https://www.readbyqxmd.com/read/28005272/prophylactic-versus-selective-blood-transfusion-for-sickle-cell-disease-in-pregnancy
#5
REVIEW
Babasola O Okusanya, Olufemi T Oladapo
BACKGROUND: Pregnant women with sickle cell disease (HbSS, HbSC and HbSβThal) may require blood transfusion to prevent severe anaemia or to manage potential medical complications. Preventive blood transfusion in the absence of complications starting from the early weeks of pregnancy or blood transfusion only for medical or obstetric indications have been used as management policies. There is currently no consensus on the blood transfusion policy that guarantees optimal clinical benefits with minimal risks for such women and their babies...
22, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28003896/laparoscopic-splenectomy-alone-for-sickle-cell-disease-account-of-50-paediatric-cases
#6
Zainab N Al-Balushi, Khalid M Bhatti, Muhammad T Ehsan, Yousuf Al-Shaqsi, Nawal A R Al-Sharji, Hatem A A Mady, Mahmoud H Sherif
OBJECTIVES: In Oman, the most frequent indication for a splenectomy in children is sickle cell disease (SCD), which is one of the most common haematological disorders in the Gulf region. This study aimed to describe paediatric laparoscopic splenectomies alone for SCD at a tertiary hospital in Oman. METHODS: This study was conducted between February 2010 and October 2015 at the Sultan Qaboos University Hospital, Muscat, Oman. The medical records of all children aged ≤15 years old undergoing splenectomies during the study period were reviewed...
November 2016: Sultan Qaboos University Medical Journal
https://www.readbyqxmd.com/read/27994894/ischemic-monomeric-neuropathy-in-a-woman-with-sickle-cell-anaemia
#7
Alexandra Agapidou, Laura Aiken, Lisa Linpower, Dimitris A Tsitsikas
Sickle cell disease is an inherited haemoglobinopathy that can affect multiple organs and systems. The most common neurological complication in sickle cell disease is stroke and silent cerebral infarcts. Peripheral nervous system involvement has been described but is exceedingly rare. Herein, we describe the case of a young woman who presented with acute flaccid paralysis and sensory loss of the left lower extremity in the context of a painful vasoocclusive crisis which resolved rapidly after receiving an emergency automated red cell exchange transfusion...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27984639/exacerbation-of-oxidative-stress-during-sickle-vaso-occlusive-crisis-is-associated-with-decreased-anti-band-3-autoantibodies-rate-and-increased-red-blood-cell-derived-microparticle-level-a-prospective-study
#8
Régine Hierso, Nathalie Lemonne, Rinaldo Villaescusa, Marie-Laure Lalanne-Mistrih, Keyne Charlot, Maryse Etienne-Julan, Benoit Tressières, Yann Lamarre, Vanessa Tarer, Yohann Garnier, Ada Arce Hernandez, Serge Ferracci, Philippe Connes, Marc Romana, Marie-Dominique Hardy-Dessources
Painful vaso-occlusive crisis, a hallmark of sickle cell anaemia, results from complex, incompletely understood mechanisms. Red blood cell (RBC) damage caused by continuous endogenous and exogenous oxidative stress may precipitate the occurrence of vaso-occlusive crises. In order to gain insight into the relevance of oxidative stress in vaso-occlusive crisis occurrence, we prospectively compared the expression levels of various oxidative markers in 32 adults with sickle cell anaemia during vaso-occlusive crisis and steady-state conditions...
December 16, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27982320/allogenic-bone-narrow-transplantation-in-sickle-cell-diseases
#9
Belinda Pinto Simões, Fabiano Pieroni, Thalita Costa, George Navarro Barros, Guilherme Darrigo, Carlos Settani Grecco, Juliana Elias Bernardes, Daniela Moraes, Ana Beatriz Lima Stracieri, Renato Cunha, Maria Carolina Rodrigues
Sickle-cell diseases are the most common inherited hemoglobinopathies worldwide. Improvement in survival has been seen in the last decades with the introduction of careful screening and prevention of complications and the introduction of hydroxyurea. Stem-cell transplantation is currently the only curative option for these patients and has been indicated for patients with neurological events, repeated vaso-occlusive crisis, any organ damage or presence of red blood cell antibodies. Related bone-marrow or cord-blood transplant has shown an overall survival of more than 90% with a disease-free survival of 90% in 1,000 patients transplanted in the last decades...
October 2016: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/27959701/crizanlizumab-for-the-prevention-of-pain-crises-in-sickle-cell-disease
#10
Kenneth I Ataga, Abdullah Kutlar, Julie Kanter, Darla Liles, Rodolfo Cancado, João Friedrisch, Troy H Guthrie, Jennifer Knight-Madden, Ofelia A Alvarez, Victor R Gordeuk, Sandra Gualandro, Marina P Colella, Wally R Smith, Scott A Rollins, Jonathan W Stocker, Russell P Rother
Background The up-regulation of P-selectin in endothelial cells and platelets contributes to the cell-cell interactions that are involved in the pathogenesis of vaso-occlusion and sickle cell-related pain crises. The safety and efficacy of crizanlizumab, an antibody against the adhesion molecule P-selectin, were evaluated in patients with sickle cell disease. Methods In this double-blind, randomized, placebo-controlled, phase 2 trial, we assigned patients to receive low-dose crizanlizumab (2.5 mg per kilogram of body weight), high-dose crizanlizumab (5...
December 3, 2016: New England Journal of Medicine
https://www.readbyqxmd.com/read/27943026/evaluating-the-safety-and-effectiveness-of-venous-thromboembolism-prophylaxis-in-patients-with-sickle-cell-disease
#11
Denise Kelley, Lauren Thornton Jones, Jun Wu, Nicole Bohm
Nearly every component of hemostasis is altered in sickle cell disease (SCD), yet little evidence exists to guide utilization of venous thromboembolism prophylaxis (VTEP) in this population. This retrospective cohort study included 135 adult patients admitted with a diagnosis of SCD vaso-occlusive crisis to the general medicine service at a tertiary care academic medical center from August 1, 2011 to August 1, 2013. If VTEP was discontinued, the medical record was reviewed for suspicion of VTE, hemorrhage, heparin-induced thrombocytopenia (HIT), or other adverse events...
December 10, 2016: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/27934541/association-between-endothelial-dysfunction-and-otoneurological-symptoms-in-children-with-sickle-cell-disease
#12
Mara Renata Rissatto-Lago, Cristina Salles, Fernando Gesteira Campos de Pinho, Isa Menezes Lyra, Regina Terse-Ramos, Rozana Teixeira, Ana Marice Ladeia
OBJECTIVE: To evaluate the association between endothelial dysfunction and otoneurological symptoms and vaso-occlusive phenomena in children with sickle cell disease (SCD). METHODS: Cross-sectional study with 54 children, aged between 6 and19 years of age, of whom 28 had genotype SS and 26 apparently healthy (AA genotype) whose parents or guardians, or the children themselves, filled out a questionnaire designed to assess their otoneurological symptoms. All the individuals were submitted assessment of endothelial function by flow-mediated dilation (FMD) percentage with reactive hyperemia of brachial artery Doppler...
December 9, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/27906010/sickle-cell-vaso-occlusive-crisis-it-s-a-gut-feeling
#13
Seah H Lim, Loren Fast, Alison Morris
Insights in the pathogenesis of vaso-occlusive crisis in patients with sickle cell disease have changed significantly in the last decade. Various laboratory and clinical evidence have provided support to the pivotal role of activated neutrophils in this process. A recent study in murine sickle cell disease indicated that the intestinal microbiota is responsible for regulating the number of aged neutrophils, a subset of neutrophils that are overly activated. Reduction of these neutrophils in vivo protected the mice from fatal TNFα-induced vaso-occlusive crisis...
December 1, 2016: Journal of Translational Medicine
https://www.readbyqxmd.com/read/27891273/orbital-infarction-due-to-sickle-cell-disease-without-orbital-pain
#14
Cameron L McBride, Kim-Binh T Mai, Kartik S Kumar
Sickle cell disease is a hemoglobinopathy that results in paroxysmal arteriolar occlusion and tissue infarction that can manifest in a plurality of tissues. Rarely, these infarcted crises manifest in the bony orbit. Orbital infarction usually presents with acute onset of periorbital tenderness, swelling, erythema, and pain. Soft tissue swelling can result in proptosis and attenuation of extraocular movements. Expedient diagnosis of sickle cell orbital infarction is crucial because this is a potentially sight-threatening entity...
2016: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/27880985/daily-pain-in-adults-with-sickle-cell-disease-a-different-perspective
#15
Charlotte F J van Tuijn, Joep W R Sins, Karin Fijnvandraat, Bart J Biemond
Previous reports demonstrated that patients with sickle cell disease (SCD) experience pain on more than half of the observed days. Yet, these high incidences do not seem to match observations in our population. In this prospective cohort study we aimed to assess the frequency and characteristics of daily, self-reported pain among adult SCD patients in the Netherlands. Consecutive patients were enrolled during routine outpatient visits and followed up to 6 months. A total of 55 patients completed 5 982 diary observation days...
November 23, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27876368/biomechanics-and-biorheology-of-red-blood-cells-in-sickle-cell-anemia
#16
Xuejin Li, Ming Dao, George Lykotrafitis, George Em Karniadakis
Sickle cell anemia (SCA) is an inherited blood disorder that causes painful crises due to vaso-occlusion of small blood vessels. The primary cause of the clinical phenotype of SCA is the intracellular polymerization of sickle hemoglobin resulting in sickling of red blood cells (RBCs) in deoxygenated conditions. In this review, we discuss the biomechanical and biorheological characteristics of sickle RBCs and sickle blood as well as their implications toward a better understanding of the pathophysiology and pathogenesis of SCA...
January 4, 2017: Journal of Biomechanics
https://www.readbyqxmd.com/read/27869039/the-role-of-rs1984112_g-at-cd36-gene-in-increasing-reticulocyte-level-among-sickle-cell-disease-patients
#17
Miniar Kalai, Marwa Dridi, Leila Chaouch, Imen Moumni, Houyem Ouragini, Imen Darragi, Imen Boudrigua, Dorra Chaouachi, Fethi Mellouli, Mohamed Bejaoui, Salem Abbes
AIMS AND BACKGROUND: Mediators of adhesion become a potential new target for pharmacological therapy to struggle the complications of sickle cell disease (SCD). Several mechanisms for increased adherence have been postulated and the well-studied are CD36 and VLA4 which encoded by ITGA4. Herein, we sought to determine whether one polymorphism of CD36 namely: rs1984112 and three exons of ITGA4 (4, 5, and 6) are implicated in hemolytic status and clinical events among SCD Tunisian patients...
November 20, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/27866856/free-tissue-transfer-in-patients-with-sickle-cell-disease-considerations-for-multi-disciplinary-peri-operative-management
#18
REVIEW
Lilli Cooper, Rohit Seth, Elizabeth Rhodes, Mohammed Alousi, Bran Sivakumar
INTRODUCTION AND AIMS: Sickle cell disease (SCD) is an increasingly common condition in the UK. The safety of free tissue transfer in these patients is controversial, and no specific guidelines exist. The aim of this paper is to create recommendations for the plastic surgical multidisciplinary team for use in the assessment and management of SCD patients undergoing free tissue transfer and reconstruction. METHOD: A literature review was performed in PubMed of 'sickle [TiAb] AND plast* adj3 surg*...
January 2017: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
https://www.readbyqxmd.com/read/27862905/intranasal-fentanyl-for-initial-treatment-of-vaso-occlusive-crisis-in-sickle-cell-disease
#19
Daniel M Fein, Jeffrey R Avner, Kathryn Scharbach, Deepa Manwani, Hnin Khine
BACKGROUND: Analgesia administration for children with vaso-occlusive crises is often delayed in the emergency department. Intranasal fentanyl (INF) has been shown to be safe and effective in providing rapid analgesia for other painful conditions. Our objective was to determine if children with a vaso-occlusive crisis (VOC) who received initial treatment with INF compared to placebo achieved a greater decrease in pain score after 20 min. PROCEDURE: This was a randomized, double-blind, placebo-controlled trial...
November 10, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27851493/1858-intravenous-ketamine-for-refractory-sickle-cell-pain-crisis-management-in-the-medical-icu
#20
Aesha Jobanputra, Deepali Dixit, Sabiha Hussain
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
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