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Acute chest syndrome in sickle cell disease

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https://www.readbyqxmd.com/read/29666710/acute-chest-syndrome-progressing-to-ards-in-a-patient-of-25-week-gestation
#1
Jefferson Chambers, Nichole Smith, Matthew Sehring, Subramanyam Chittivelu
Acute chest syndrome is a complication of sickle cell disease and represents the highest cause of mortality in those afflicted with the disorder. Pregnancy represents an increased risk for complications of sickle cell disease in both the mother and fetus. We present a case of a 20-year-old patient with known sickle cell disease who was at 25-week gestation and developed acute chest syndrome refractory to conventional therapies and requiring emergency cesarean section. Following delivery, the patient developed acute respiratory distress syndrome (ARDS) requiring extracorporeal membrane oxygenation (ECMO)...
2018: Case Reports in Critical Care
https://www.readbyqxmd.com/read/29666400/the-corticosteroid-compounds-prednisolone-and-vamorolone-do-not-alter-the-nociception-phenotype-and-exacerbate-liver-injury-in-sickle-cell-mice
#2
Luis E F Almeida, Jesse M Damsker, Sarah Albani, Nina Afsar, Sayuri Kamimura, Drew Pratt, David E Kleiner, Martha Quezado, Heather Gordish-Dressman, Zenaide M N Quezado
Clinicians often hesitate prescribing corticosteroids to treat corticosteroid-responsive conditions in sickle cell disease (SCD) patients because their use can be associated with complications (increased hospital readmission, rebound pain, strokes, avascular necrosis, acute chest syndrome). Consequently, SCD patients may receive suboptimal treatment for corticosteroid-responsive conditions. We conducted a preclinical trial of dissociative (vamorolone) and conventional (prednisolone) corticosteroid compounds to evaluate their effects on nociception phenotype, inflammation, and organ dysfunction in SCD mice...
April 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29648482/acute-chest-syndrome-in-sickle-cell-disease
#3
Sajid Farooq, Mohannad Abu Omar, Gary A Salzman
Acute chest syndrome (ACS) is a leading complication of sickle cell disease (SCD) with significant morbidity and mortality. ACS is the most common cause of death and the second most common cause of hospitalization in patients with SCD. Delineating the specific cause of ACS is often difficult, and multiple risk factors that precipitate ACS frequently coexist. The prominent risk factors include infection, hypoxia, bronchial hyperresponsiveness, the SCD genotype, and opioid use. The key to the successful treatment of ACS is early recognition and initiation of treatment without delay...
April 12, 2018: Hospital Practice (Minneapolis)
https://www.readbyqxmd.com/read/29614637/inflammation-in-sickle-cell-disease
#4
Nicola Conran, John D Belcher
The primary β-globin gene mutation that causes sickle cell disease (SCD) has significant pathophysiological consequences that result in hemolytic events and the induction of the inflammatory processes that ultimately lead to vaso-occlusion. In addition to their role in the initiation of the acute painful vaso-occlusive episodes that are characteristic of SCD, inflammatory processes are also key components of many of the complications of the disease including autosplenectomy, acute chest syndrome, pulmonary hypertension, leg ulcers, nephropathy and stroke...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29614629/genetic-modifiers-of-severity-in-sickle-cell-disease
#5
Alicia K Chang, Carly C Ginter Summarell, Parendi T Birdie, Vivien A Sheehan
Sickle cell disease (SCD) is one of the most common single disease disorders world-wide. It is remarkable for its clinical heterogeneity, even among individuals with identical genotypes. Some individuals experience morbidity and mortality in early childhood, while others have a relatively mild course, and normal or near normal life expectancy. Many clinical complications are associated with SCD; most notably frequent pain episodes, stroke, acute chest syndrome, avascular necrosis, nephropathy, retinopathy and pulmonary hypertension...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29614628/comorbidities-in-aging-patients-with-sickle-cell-disease
#6
Samir K Ballas
Sickle cell disease (SCD) in general and sickle cell anemia in particular is a highly complex disorder both at the molecular and clinical levels. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of the disease. Moreover, despite the fact that SCD is a chronic malady, its manifestations are both acute and chronic. The former include, among other things, the recurrent vaso-occlusive crises (its hallmark) and acute chest syndrome...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29550053/early-initiation-of-inhaled-corticosteroids-does-not-decrease-acute-chest-syndrome-morbidity-in-pediatric-patients-with-sickle-cell-disease
#7
Alexis Leonard, Nihal Godiwala, Nicole Herrera, Robert McCarter, Matthew Sharron, Emily Riehm Meier
Acute chest syndrome (ACS) is a leading cause of mortality in patients with sickle cell disease (SCD). Systemic corticosteroids decrease ACS severity, but the risk of readmission for vaso-occlusive crises (VOC) has limited their use. The efficacy of inhaled corticosteroids (ICS) as a safer alternative is currently unknown. An observational, historic cohort study compared patients with SCD with ACS who received ICS at admission (ICS) to those who did not (non-ICS). Outcome measures included rates of transfusion, oxygen requirement, BiPAP initiation, PICU transfer, intubation, readmission, hospital cost, and length of stay...
March 7, 2018: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29542687/sickle-cell-disease
#8
REVIEW
Gregory J Kato, Frédéric B Piel, Clarice D Reid, Marilyn H Gaston, Kwaku Ohene-Frempong, Lakshmanan Krishnamurti, Wally R Smith, Julie A Panepinto, David J Weatherall, Fernando F Costa, Elliott P Vichinsky
Sickle cell disease (SCD) is a group of inherited disorders caused by mutations in HBB, which encodes haemoglobin subunit β. The incidence is estimated to be between 300,000 and 400,000 neonates globally each year, the majority in sub-Saharan Africa. Haemoglobin molecules that include mutant sickle β-globin subunits can polymerize; erythrocytes that contain mostly haemoglobin polymers assume a sickled form and are prone to haemolysis. Other pathophysiological mechanisms that contribute to the SCD phenotype are vaso-occlusion and activation of the immune system...
March 15, 2018: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/29506549/lipid-profiles-in-french-west-indies-sickle-cell-disease-cohorts-and-their-general-population
#9
Marie-Laure Lalanne-Mistrih, Philippe Connes, Yann Lamarre, Nathalie Lemonne, Marie-Dominique Hardy-Dessources, Vanessa Tarer, Maryse Etienne-Julan, Dominique Mougenel, Benoît Tressières, Marc Romana
BACKGROUND: The pathophysiology of sickle cell disease (SCD) and the variability of its clinical expression remain not fully understood, whether within or between different SCD genotypes. Recent studies have reported associations between lipid levels and several SCD complications. If lipid levels have been previously described as low in sickle cell anemia (SCA), few data have been provided for sickle cell SC disease (SCC). We designed our epidemiological study to isolate lipid levels and profiles by genotype in Guadeloupian cohorts of SCA and SCC adult patients, at steady state...
March 5, 2018: Lipids in Health and Disease
https://www.readbyqxmd.com/read/29498079/the-mcconnell-sign-is-seen-in-patients-with-acute-chest-syndrome
#10
John Bates McCutcheon, Pascha Schaffer, Matthew Lyon, Richard Gordon
Bedside ultrasound is often used as a part of the evaluation of patients who are critically ill. The McConnell sign is an important echocardiographic finding in some critically ill patients with pulmonary embolism and an acute right ventricular infarct. We present 3 critically ill patients with confirmed acute chest syndrome who showed the McConnell sign on echocardiography. In patients with sickle cell disease presenting with chest pain and shortness of breath, the presence of the McConnell sign does not narrow the differential diagnosis between pulmonary embolism, an acute right ventricular infarct, and acute chest syndrome...
March 2, 2018: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/29495591/chronic-administration-of-hydroxyurea-hu-benefits-caucasian-patients-with-sickle-beta-thalassemia
#11
Rosario Di Maggio, Matthew M Hsieh, Xiongce Zhao, Giuseppina Calvaruso, Paolo Rigano, Disma Renda, John F Tisdale, Aurelio Maggio
In sickle cell disease (SCD), hydroxyurea (HU) treatment decreases the number of vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) by increasing fetal hemoglobin (HbF). Data are lacking regarding the frequency of HU dose modification or whether sub-therapeutic doses (<15 mg/kg/day) are beneficial. We reviewed the medical records of 140 patients from 2010 to 2014. The laboratory parameters and SCD complications were compared between the first and last visits based on HU use. Fifty patients (36%) never took HU or suspended HU ("no HU" group)...
February 28, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29494636/causes-of-death-and-early-life-determinants-of-survival-in-homozygous-sickle-cell-disease-the-jamaican-cohort-study-from-birth
#12
Graham R Serjeant, Nicki Chin, Monika R Asnani, Beryl E Serjeant, Karlene P Mason, Ian R Hambleton, Jennifer M Knight-Madden
Globally, the majority of persons born with sickle cell disease do not have access to hydroxyurea or more expensive interventions. The objectives were to estimate the survival in homozygous sickle cell disease, unbiased by symptomatic selection and to ascertain the causes of death in a pre-hydroxyurea population. The utility of early life biomarkers and genetically determined phenotypes to predict survival was assessed. A cohort study based on neonatal diagnosis was undertaken at the Sickle Cell Unit, a specialist clinic delivering care to persons with sickle cell disease in Jamaica...
2018: PloS One
https://www.readbyqxmd.com/read/29465435/clinical-manifestations-of-sickle-cell-disease-in-india-misconceptions-and-reality
#13
Dipty Jain, Dipika Mohanty
PURPOSE OF REVIEW: In the past, milder clinical manifestations of sickle cell disease (SCD) have been described from India. However, recent data from some parts of India suggest that the severity of the disease can be compared to that of African phenotypes. This review therefore describes the varied clinical manifestation of SCD, the success of newborn screening programme, prenatal diagnosis and low dose hydroxyurea therapy in India. RECENT FINDINGS: The varied clinical manifestations such as anemia, vaso-occlusive crisis, acute chest syndrome, renal involvement, stroke and so on vary from one part of the country to the other and also among different communities of India...
February 19, 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29450676/comorbid-obstructive-sleep-apnea-and-increased-risk-for-sickle-cell-disease-morbidity
#14
Tal Katz, Jeffrey Schatz, Carla W Roberts
PURPOSE: Sickle cell disease (SCD) imparts an increased risk for obstructive sleep apnea (OSA) in childhood. Studies of pediatric SCD have identified an increased risk for pain and neurologic complications with comorbid OSA. We determined the rate of a broad range of SCD-related medical complications to better characterize the spectrum of SCD complications related to OSA. METHODS: Retrospective chart review at a single hematology clinic identified 641 youth with SCD who received consistent screenings for OSA as part of routine hematological health maintenance visits over an 11-year period...
February 15, 2018: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/29378711/population-pharmacokinetics-of-cefotaxime-and-dosage-recommendations-in-children-with-sickle-cell-disease
#15
Elsa Maksoud, Berengere Koehl, Aude Facchin, Phuong Ha, Wei Zhao, Florentia Kaguelidou, Malika Benkerrou, Patricia Mariani, Albert Faye, Mathie Lorrot, Evelyne Jacqz-Aigrain
The pharmacokinetic profile of most drugs is dependent on patient's covariates and may be influenced by the disease. Cefotaxime is frequently prescribed in pediatric patients with sickle-cell disease (SCD), characterized by vaso-occlusive complications, chronic haemolytic anaemia and defective immunological function predisposing to severe infection. Data on the impact of the disease on cefotaxime disposition are missing. In the present study, our aims were to determine cefotaxime pharmacokinetics when prescribed in SCD children for suspected or proven bacterial infection, identify significant covariates and perform Monte-Carlo simulations to optimize drug dosage...
January 29, 2018: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/29377071/how-i-manage-red-cell-transfusions-in-patients-with-sickle-cell-disease
#16
David C Rees, Susan Robinson, Jo Howard
Sickle cell disease is one of the commonest serious inherited diseases in the world, and red cell transfusion is still one of the few effective treatments for acute and chronic complications. Transfusion corrects anaemia and dilutes out the number of red cells able to cause vaso-occlusion and vascular damage. Urgent red cell transfusions are used to correct acute anaemia, treat acute chest syndrome and patients with acute neurological symptoms. We use elective transfusions preoperatively for moderate risk surgery, and in some pregnant women...
January 29, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29356722/early-noninvasive-ventilation-and-nonroutine-transfusion-for-acute-chest-syndrome-in-sickle-cell-disease-in-children-a-descriptive-study
#17
Claire Heilbronner, Audrey Merckx, Valentine Brousse, Slimane Allali, Philippe Hubert, Mariane de Montalembert, Fabrice Lesage
OBJECTIVES: To describe the need for transfusion and short- and long-term evolutions of pediatric sickle cell disease patients with acute chest syndrome for whom early continuous noninvasive ventilation represented first-line treatment. DESIGN: Single-center retrospective chart study in PICU. SETTING: A tertiary and quaternary referral PICU. PATIENTS: All sickle cell disease patients 5-20 years old admitted with confirmed acute chest syndrome and not transfused in the previous month were included...
January 19, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29314737/airway-inflammation-in-sickle-cell-disease-a-translational-perspective
#18
REVIEW
Aliva De, Deepa Manwani, Deepa Rastogi
Asthma and sickle cell disease (SCD) are common chronic conditions in children of African ancestry that are characterized by cough, wheeze, and obstructive patterns on pulmonary function. Pulmonary function testing in children with SCD has estimated a prevalence of obstructive lung disease ranging from 13% to 57%, and airway hyper-responsiveness of up to 77%, independent of a diagnosis of asthma. Asthma co-existing with SCD is associated with increased risk of acute chest syndrome (ACS), respiratory symptoms, pain episodes, and death...
April 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29313505/effectiveness-safety-and-cost-of-partial-exchange-transfusions-in-patients-with-sickle-cell-anemia-at-a-sickle-cell-disease-center-in-sub-saharan-africa
#19
P Boma Muteb, J F J Kaluila Mamba, P Muhau Pfutila, V Bilo, J D Panda Mulefu, D A Diallo
The partial exchange transfusions necessary for management of some sickle-cell complications raise the issue of effectiveness in the context of limited resources and inadequate blood safety. This study evaluated the effectiveness, safety, and cost of partial exchange transfusions in 39 patients with sickle-cell anemia in Lubumbashi, looking at the patients' age and gender and the tolerability and direct cost of the transfusions. Excel and SPSS 18 were used for data entry and analysis. Chi2 and Fisher exact tests were used for comparisons...
November 1, 2017: Médecine et Santé Tropicales
https://www.readbyqxmd.com/read/29296770/osteonecrosis-of-the-femoral-head-in-sickle-cell-disease-prevalence-comorbidities-and-surgical-outcomes-in-california
#20
Oyebimpe Adesina, Ann Brunson, Theresa H M Keegan, Ted Wun
Osteonecrosis of the femoral head (ONFH) is a prevalent complication of sickle cell disease (SCD) that has not been well described in population-based cohort studies. Using California's Office of Statewide Planning and Development discharge databases (1991-2013), we estimated the cumulative incidence of ONFH after accounting for the competing risk of death and used a multivariable Cox proportional hazards regression to identify factors associated with ONFH diagnosis. We also calculated rates of readmissions to the hospital or emergency department within 30 to 90 days of hip replacement surgery...
July 11, 2017: Blood Advances
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