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Acute chest syndrome in sickle cell disease

Jennifer M Knight-Madden, Ian R Hambleton
BACKGROUND: Bronchodilators are used to treat bronchial hyper-responsiveness in asthma. Bronchial hyper-responsiveness may be a component of acute chest syndrome in people with sickle cell disease. Therefore, bronchodilators may be useful in the treatment of acute chest syndrome. This is an update of a previously published Cochrane Review. OBJECTIVES: To assess the benefits and risks associated with the use of bronchodilators in people with acute chest syndrome...
September 27, 2016: Cochrane Database of Systematic Reviews
T Pincez, L Calamy, Z Germont, A Lemoine, A-A Lopes, A Massiot, J Tencer, C Thivent, A Hadchouel
Acute and chronic pulmonary complications are frequent in sickle cell disease (SCD), with different spectrum and characteristics in children and adults. Chronic hypoxia is frequent and plays a role in several respiratory complications in SCD. Furthermore, hypoxia has been associated with a higher risk of cerebral ischemia. Despite differing oxygen affinity between hemoglobin A and S, standard pulse oximetry was shown to be accurate in diagnosing hypoxia in SCD patients. Whereas acute hypoxia management is similar to non-SCD patients, chronic hypoxia treatment is mainly based on a transfusion program rather than long-term oxygen therapy...
October 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Shalini Shenoy, Mary Eapen, Julie A Panepinto, Brent R Logan, Juan Wu, Allistair Abraham, Joel Brochstein, Sonali Chaudhury, Kamar Godder, Ann E Haight, Kimberly A Kasow, Kathryn Leung, Martin Andreansky, Monica Bhatia, Jignesh Dalal, Hilary Haines, Jennifer Jaroscak, Hillard M Lazarus, John E Levine, Lakshmanan Krishnamurti, David Margolis, Gail C Megason, Lolie C Yu, Michael A Pulsipher, Iris Gersten, Nancy DiFronzo, Mary M Horowitz, Mark C Walters, Naynesh Kamani
Children with sickle cell disease (SCD) experience organ damage, impaired quality of life, and premature mortality. Allogeneic bone marrow transplantation from an HLA-matched sibling can halt disease progression but is limited by donor availability. A multicenter phase II trial conducted from 2008-2014 enrolled 30 children aged 4-19 years; 29 were eligible for evaluation. The primary objective was 1-year event-free survival (EFS) after HLA allele-matched (at HLA-A, -B, -C and -DRB1 loci) unrelated donor transplantation...
September 13, 2016: Blood
Tiffany Yu, Timothy Campbell, Isabella Ciuffetelli, Carlton Haywood, Christopher Patrick Carroll, Linda Resar, John J Strouse, Sophie Lanzkron
OBJECTIVES: Sickle cell disease (SCD) is associated with high healthcare utilization rates and poor outcomes in a subset of patients, although the underlying factors that predict this phenotype are poorly understood. Prior studies suggest that comorbid avascular necrosis (AVN) contributes to high healthcare utilization. We sought to clarify whether AVN independently predicts acute care utilization in adults with SCD and to identify characteristics of those with AVN that predict higher utilization...
September 2016: Southern Medical Journal
Saeed Dastgiri, Roya Dolatkhah
BACKGROUND: Sickle cell disease is an inherited autosomal recessive blood condition and is one of the most prevalent genetic blood diseases worldwide. Acute chest syndrome is a frequent complication of sickle cell disease, as well as a major cause of morbidity and the greatest single cause of mortality in children with sickle cell disease. Standard treatment may include intravenous hydration, oxygen as treatment for hypoxia, antibiotics to treat the infectious cause and blood transfusions may be given...
2016: Cochrane Database of Systematic Reviews
Erica Simon, Brit Long, Alex Koyfman
BACKGROUND: Sickle cell disease (SCD) affects approximately 100,000 individuals in the United States. Due to alterations in the structural conformation of hemoglobin molecules under deoxygenated conditions, patients with SCD are predisposed to numerous sequelae, many of which require acute intervention. OBJECTIVE: Our aim was to provide emergency physicians with an evidence-based update regarding the diagnosis and management of SCD complications. DISCUSSION: SCD patients experience significant morbidity and mortality secondary to cerebrovascular accident, acute chest syndrome, acute vaso-occlusive pain crises, SCD-related multi-organ failure, cholecystitis, acute intrahepatic cholestasis, acute sickle hepatic crisis, acute hepatic sequestration, priapism, and renal disease...
October 2016: Journal of Emergency Medicine
Lidiane S Torres, Jéssika V Okumura, Édis Belini-Júnior, Renan G Oliveira, Patrícia P Nascimento, Danilo G H Silva, Clarisse L C Lobo, Sonia M Oliani, Claudia R Bonini-Domingos
Phenotypic heterogeneity for sickle cell disease is associated to several genetic factors such as genotype for sickle cell disease, β-globin gene cluster haplotypes and Hb F levels. The coinheritance of Hb S (HBB: c.20A > T) and Hb D-Punjab (HBB: c.364G > C) results in a double heterozygosity, which constitutes one of the genotypic causes of sickle cell disease. This study aimed to assess the phenotypic diversity of sickle cell disease presented by carriers of the Hb S/Hb D-Punjab genotype and the Bantu [- + - - - -] haplotype...
October 3, 2016: Hemoglobin
Anastassios C Koumbourlis
No abstract text is available yet for this article.
August 2016: Annals of the American Thoracic Society
Arturo J Martí-Carvajal, Ivan Solà, Luis H Agreda-Pérez
BACKGROUND: Avascular necrosis of bone is a frequent and severe complication of sickle cell disease and its treatment is not standardised. This is an update of a previously published Cochrane Review. OBJECTIVES: To determine the impact of any surgical procedure compared with other surgical interventions or non-surgical procedures, on avascular necrosis of bone in people with sickle cell disease in terms of efficacy and safety. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings...
2016: Cochrane Database of Systematic Reviews
T Wun
Acute and chronic vascular occlusion underlies much of the morbidity and mortality in sickle disease. Abnormal polymerization of deoxygenated hemoglobin S (HbS) resulting in stiff, non-deformable erythrocytes is central to sickle cell pathogenesis. However, a complex interplay of many factors determines the balance between adequate blood flow and vessel obstruction. Serum markers of inflammation have provided evidence for a state of chronic inflammation in sickle cell disease (SCD). Inflammation promotes endothelial adherence to sickle erythrocytes...
2000: Hematology (Amsterdam, Netherlands)
Pablo Bartolucci, Anoosha Habibi, Mehdi Khellaf, Françoise Roudot-Thoraval, Giovanna Melica, Anne-Sophie Lascaux, Stéphane Moutereau, Sylvain Loric, Orianne Wagner-Ballon, Jugurtha Berkenou, Aline Santin, Marc Michel, Bertrand Renaud, Yves Lévy, Frédéric Galactéros, Bertrand Godeau
BACKGROUND: Vaso-occlusive crisis (VOC), hallmark of sickle-cell disease (SCD), is the first cause of patients' Emergency-Room admissions and hospitalizations. Acute chest syndrome (ACS), a life-threatening complication, can occur during VOC, be fatal and prolong hospitalization. No predictive factor identifies VOC patients who will develop secondary ACS. METHODS: This prospective, monocenter, observational study on SS/S-β0thalassemia SCD adults aimed to identify parameters predicting ACS at Emergency-Department arrival...
August 2016: EBioMedicine
Asral Wirda Ahmad Asnawi, Jameela Sathar, Rashidah Mohamed, Rohayu Deraman, Sri Kumaran, Shahada Sobah Abd Hamid, Muhd Zanapiah Zakaria
Clinical manifestations of sickle cell disease (SCD) arise from the tendency of the sickle haemoglobin to polymerize and deform red blood cells into the characteristic sickle shape. Sickle cell crisis is a devastating complication that may occur in patients with SCD. If not managed properly permanent organ damage and even death may be the final outcome. A case of a 32-year-old Nigerian lady, Gravida 1 Para 0 in her first trimester, with SCD who developed signs and symptoms of delayed haemolytic transfusion reaction after receiving packed red cell transfusion is demonstrated...
June 2016: Indian Journal of Hematology & Blood Transfusion
Katherine Eisenbrown, Mark Nimmer, Angela M Ellison, Pippa Simpson, David C Brousseau
OBJECTIVE: Controversy exists regarding which febrile children with sickle cell disease (SCD) should receive a chest x-ray (CXR). Our goal is to provide data informing the decision of which febrile children with SCD presenting to the emergency department (ED) require a CXR to evaluate for acute chest syndrome (ACS). METHODS: Retrospective chart review of children ages 3 months to 21 years with SCD presenting to the ED at one of two academic children's hospitals with fever ≥ 38...
July 12, 2016: Academic Emergency Medicine: Official Journal of the Society for Academic Emergency Medicine
John C Aneke, Nancy Huntley, John Porter, Perla Eleftheriou
BACKGROUND: Red cell exchanges (RCEs) lead to improvement in tissue oxygenation and reduction in inflammatory markers in sickle cell disease (SCD) patients who present with acute chest syndrome (ACS). The aim of this study is to evaluate the effects of automated-RCE (auto-RCE) on oxygen saturation (SpO2) on-air, blood counts, the time to correct the parameters and length of hospitalization after the exchange in SCD patients presenting with ACS. SUBJECTS AND METHODS: This was 2 years study involving five SCD patients; the time for SpO2 on air to increase to ≥95% and chest symptoms to resolve, postprocedure, as well as the length of in-patient hospitalization was recorded...
May 2016: Nigerian Medical Journal: Journal of the Nigeria Medical Association
Michael R DeBaun, Robert C Strunk
Acute chest syndrome is a frequent cause of acute lung disease in children with sickle-cell disease. Asthma is common in children with sickle-cell disease and is associated with increased incidence of vaso-occlusive pain events, acute chest syndrome episodes, and earlier death. Risk factors for asthma exacerbation and an acute chest syndrome episode are similar, and both can present with shortness of breath, chest pain, cough, and wheezing. Despite overlapping risk factors and symptoms, an acute exacerbation of asthma or an episode of acute chest syndrome are two distinct entities that need disease-specific management strategies...
June 18, 2016: Lancet
Anoosha Habibi, Armand Mekontso-Dessap, Constance Guillaud, Marc Michel, Keyvan Razazi, Mehdi Khellaf, Btissam Chami, Dora Bachir, Claire Rieux, Giovanna Melica, Bertrand Godeau, Frédéric Galacteros, Pablo Bartolucci, France Pirenne
Delayed hemolytic transfusion reaction (DHTR) is one of the most feared complications of sickle-cell disease (SCD). We retrospectively analyzed the clinical and biological features, treatments and outcomes of 99 DHTRs occurring in 69 referral center patients over 12 years. The first clinical signs appeared a median of 9.4 [IQR, 3-22] days after the triggering transfusion (TT). The most frequent DHTR-related clinical manifestation was dark urine/hemoglobinuria (94%). Most patients (89%) had a painful vaso-occlusive crisis and 50% developed a secondary acute chest syndrome (ACS)...
October 2016: American Journal of Hematology
Dipty Jain, Aishwarya Arjunan, Vijaya Sarathi, Harshwardhan Jain, Amol Bhandarwar, Marike Vuga, Lakshmanan Krishnamurti
BACKGROUND: The clinical phenotype of sickle cell disease (SCD) has been reported to be milder in India than in the United States. The objective of this large single-center study was to examine the rate of complications to define the phenotype of SCD in India. METHODS: The rate of complications per 100 person-years in 833 pediatric SCD patients for 1954 person-years in Nagpur, India including those diagnosed on newborn screen (NBS) and those presenting later in childhood (non-NBS) was compared to those reported in the cooperative study of sickle cell disease (CSSCD)...
October 2016: Pediatric Blood & Cancer
Asmaa Ghmaird, Mohammad Mohammad Alnoaiji, Sawsan Al-Blewi, Shaimaa Zaki, Ahmad El-Lewi, Nehal Ahmad
BACKGROUND: Sickle cell disease is a common genetic disease in Saudi Arabia; it is an autosomal recessive disorder characterized by production of abnormal hemoglobin S and is associated with high morbidity and mortality. Acute splenic sequestration is a life-threatening complication for this disease. Prophylactic splenectomy is the only effective strategy for preventing future life-threatening episodes. AIM: The aim of this study was to study hospital records for all children aged 2 to 12 year old with Sickle cell disease who underwent splenectomy in Tabuk in Saudi Arabia...
March 15, 2016: Open Access Macedonian Journal of Medical Sciences
Jérôme Cecchini, Florence Boissier, Aude Gibelin, Nicolas de Prost, Keyvan Razazi, Guillaume Carteaux, Frederic Galacteros, Bernard Maitre, Christian Brun-Buisson, Armand Mekontso Dessap
BACKGROUND: Acute chest syndrome (ACS) is the most common cause of death among sickle cell disease (SCD) adult patients. Pulmonary vascular dysfunction (PVD) and acute cor pulmonale (ACP) are common during acute respiratory distress syndrome (ARDS) and their prevalence may be even more important during ARDS related to ACS (ACS-ARDS). The objective of this study was to evaluate the prevalence and prognosis of PVD and ACP during ACS-ARDS. PATIENTS AND METHODS: This was a retrospective analysis over a 10-year period of patients with moderate-to-severe ARDS...
October 2016: Shock
Dina D Daswani, Vaishali P Shah, Jeffrey R Avner, Deepa G Manwani, Jessica Kurian, Joni E Rabiner
OBJECTIVES: The objective was to determine the test performance characteristics for point-of-care lung ultrasonography (LUS) performed by pediatric emergency medicine (PEM) physicians compared with radiographic diagnosis of acute chest syndrome (ACS) in patients with sickle cell disease (SCD) and fever. METHODS: This was a prospective study of patients up to 21 years with SCD and fever requiring chest X-ray (CXR) evaluation for ACS. Before obtaining CXR, a blinded PEM physician performed LUS using a standardized scanning protocol...
August 2016: Academic Emergency Medicine: Official Journal of the Society for Academic Emergency Medicine
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