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Acute chest syndrome in sickle cell disease

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https://www.readbyqxmd.com/read/29465435/clinical-manifestations-of-sickle-cell-disease-in-india-misconceptions-and-reality
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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...
January 4, 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
#7
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
#8
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
https://www.readbyqxmd.com/read/29279787/acute-chest-syndrome-in-children-with-sickle-cell-disease
#9
REVIEW
Shilpa Jain, Nitya Bakshi, Lakshmanan Krishnamurti
Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle cell disease (SCD). Patients may present with ACS or may develop this complication during the course of a hospitalization for acute vaso-occlusive crises (VOC). ACS is associated with prolonged hospitalization, increased risk of respiratory failure, and the potential for developing chronic lung disease. ACS in SCD is defined as the presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray...
December 1, 2017: Pediatric Allergy, Immunology, and Pulmonology
https://www.readbyqxmd.com/read/29226507/age-is-the-only-predictor-of-small-decrease-in-lung-function-in-children-with-sickle-cell-anemia
#10
Shaina M Willen, Robyn Cohen, Mark Rodeghier, Fenella Kirkham, Susan S Redline, Carol Rosen, Jane Kirkby, Michael R DeBaun
The longitudinal pattern of lung function in children with sickle cell anemia (SCA) has shown a decrease in FEV1 % predicted, a risk factor for death in adults with SCA, but predictors for this decline are poorly characterized. In a prospective longitudinal multi-center cohort of children with SCA, we tested the hypotheses that: 1) FEV1 % predicted declines over time; and 2) SCA-specific characteristics and therapy predict this decline. At three clinical centers, children with SCA (HbSS or HbSβ0 thalassemia), unselected for respiratory disease, were enrolled in the Sleep and Asthma Cohort (SAC) study...
December 11, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29200151/recurrent-acute-chest-syndrome-in-pediatric-sickle-cell-disease-clinical-features-and-risk-factors
#11
Gaylen D Patterson, Hafsat Mashegu, Jordan Rutherford, Samantha Seals, David Josey, Cynthia Karlson, Melissa McNaull, Warren May, Clinton Carroll, Frederick E Barr, Suvankar Majumdar
Acute chest syndrome (ACS) is a common and serious lung complication in sickle cell disease. A retrospective medical chart review was performed over a 6-year period in all pediatric ACS patients to investigate whether factors during the initial hospitalization were associated with recurrent ACS episodes. There were 386 episodes of ACS: 149 had only 1 episode of ACS, and 76 had >1 episode of ACS; 172 (76.4%) had hemoglobin SS, and 39 (17.3%) had hemoglobin SC. The most common presenting features were fever (83%), pain (70%), and cough (61%), which changed with the number of ACS episodes...
January 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29183008/correlates-of-pulmonary-function-in-children-with-sickle-cell-disease-and-elevated-fetal-hemoglobin
#12
Adekunle D Adekile, Asmaa Azab, Abdullah Owayed, Mousa Khadadah
OBJECTIVE: The current study was carried out to compare pulmonary function tests (PFT) in pediatric Kuwaiti sickle cell disease (SCD) patients to age-matched normal controls and to investigate the association of PFTS to selected clinical and laboratory parameters. SUBJECTS AND METHODS: There were 38 patients with SCD and 36 controls in the study. The patients were recruited from the Pediatric Hematology Clinics of Mubarak Al-Kabeer and Al-Amiri Hospitals, Kuwait and were studied in steady state...
November 28, 2017: Medical Principles and Practice: International Journal of the Kuwait University, Health Science Centre
https://www.readbyqxmd.com/read/29182088/perinatal-maternal-mortality-in-sickle-cell-anemia-two-case-reports-and-review-of-the-literature
#13
Sanaa Rizk, Elizabeth D Pulte, David Axelrod, Samir K Ballas
As outcomes of patients with sickle cell anemia improve and survival into adulthood with good quality of life and expectation of long-term survival becomes more common, challenges have developed, including issues related to reproduction. Pregnancy is frequently complicated in patients with sickle cell anemia with mortality up to 4.0%. Here we report maternal perinatal mortality in two women with sickle cell anemia who died post-partum due to acute chest syndrome (ACS), caused by bone marrow fat embolism and review the literature pertinent to this subject...
November 28, 2017: Hemoglobin
https://www.readbyqxmd.com/read/29181140/foetal-haemoglobin-and-disease-severity-in-nigerian-children-with-sickle-cell-anaemia
#14
Oluwagbemiga O Adeodu, Morenike A Akinlosotu, Samuel A Adegoke, Saheed B A Oseni
Background: Foetal haemoglobin (HbF) is a major modifying factor influencing sickle cell disease (SCD) severity. Despite this, HbF estimation is not routinely done in Nigeria. The relationship between HbF and SCD severity among affected children is also poorly studied. Methods: In this descriptive cross-sectional study, we determined the relationship between steady state HbF levels and disease severity of Nigerian children aged 1 - 15 years with homozygous SCD. For each child, the socio-demographic characteristics and SCD clinical severity were determined...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29134776/acute-chest-syndrome-among-children-hospitalized-with-vaso-occlusive-crisis-a-nationwide-study-in-the-united-states
#15
Takuto Takahashi, Yusuke Okubo, Atsuhiko Handa
PURPOSE: Acute chest syndrome (ACS) is a common complication among pediatric inpatients with sickle cell disease and vaso-occlusive crisis (VOC). However, little is known about the factors associated with ACS complication. The present study assessed the epidemiological features of children hospitalized with VOC and ascertained factors associated with ACS complication. METHODS: Hospital discharge records of patients with VOC aged <20 years were obtained for the years 2003, 2006, 2009, and 2012 from the Kids' Inpatient Database...
March 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29107441/real-life-experience-with-hydroxyurea-in-sickle-cell-disease-a-multicenter-study-in-a-cohort-of-patients-with-heterogeneous-descent
#16
Paolo Rigano, Lucia De Franceschi, Laura Sainati, Antonio Piga, Frédéric B Piel, Maria Domenica Cappellini, Carmelo Fidone, Nicoletta Masera, Giovanni Palazzi, Barbara Gianesin, Gian Luca Forni
We conducted the first nation-wide cohort study of sickle cell disease (SCD) in Italy, a Southern European country exposed to intense recent flux migration from endemic areas for SCD. We evaluate the impact of hydroxyurea on a total of 652 pediatric and adult patients from 33 Reference Centers for SCD (mean age 24.5±15years, 51.4% males). Hydroxyurea median treatment duration was 7years (range: <1year to 29years) at a mean therapeutic dose of 18±4.7mg/kg/day. Hydroxyurea was associated with a significant increase in mean total and fetal hemoglobin and a significant decrease in mean hemoglobin S, white blood and platelet counts, and lactate dehydrogenase levels...
October 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29098436/low-impact-laparoscopic-cholecystectomy-is-associated-with-decreased-postoperative-morbidity-in-patients-with-sickle-cell-disease
#17
Nicola de'Angelis, Solafah Abdalla, Maria Clotilde Carra, Vincenzo Lizzi, Aleix Martínez-Pérez, Anoosha Habibi, Pablo Bartolucci, Frédéric Galactéros, Alexis Laurent, Francesco Brunetti
BACKGROUND: Laparoscopic cholecystectomy (LC) is one of the most frequent surgeries performed in patients with sickle cell disease (SCD). LC in SCD patients is associated with a particularly high postoperative morbidity. The aim of the present study is to assess the safety and feasibility of cholecystectomy performed by mini-laparoscopy with low- and stable-pressure pneumoperitoneum (MLC + LSPP) and to compare the rate of postoperative SCD-related morbidity with standard LC. METHODS: Thirty-five consecutive SCD patients admitted between November 2015 and March 2017 for cholelithiasis requiring surgery were compared with an historical cohort of 126 SCD patients who underwent LC for the same indication...
November 2, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28905364/a-clinical-risk-score-for-pulmonary-artery-thrombosis-during-acute-chest-syndrome-in-adult-patients-with-sickle-cell-disease
#18
Anaïs Winchenne, Jérôme Cecchini, Jean-François Deux, Nicolas De Prost, Keyvan Razazi, Guillaume Carteaux, Frederic Galacteros, Anoosha Habibi, Pablo Bartolucci, Giovanna Melica, Mehdi Khellaf, Marc Michel, Bernard Maitre, Armand Mekontso Dessap
Pulmonary artery thrombosis (PAT) is involved in lung vascular dysfunction during acute chest syndrome (ACS) complicating sickle cell disease (SCD). No clinical score is available to identify patients eligible for multi-detector computed tomography (MDCT) angiography during ACS. This retrospective study aimed to develop a risk score for PAT during ACS (PAT-ACS risk score). Patients with SCD were investigated by MDCT during ACS. A logistic regression was performed to determine independent risks factors for PAT and to build the PAT-ACS risk score...
September 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28892533/association-of-guideline-adherent-antibiotic-treatment-with-readmission-of-children-with-sickle-cell-disease-hospitalized-with-acute-chest-syndrome
#19
David G Bundy, Troy E Richardson, Matthew Hall, Jean L Raphael, David C Brousseau, Staci D Arnold, Ram V Kalpatthi, Angela M Ellison, Suzette O Oyeku, Samir S Shah
Importance: Acute chest syndrome (ACS) is a common, serious complication of sickle cell disease (SCD) and a leading cause of hospitalization and death in both children and adults with SCD. Little is known about the effectiveness of guideline-recommended antibiotic regimens for the care of children hospitalized with ACS. Objectives: To use a large, national database to describe patterns of antibiotic use for children with SCD hospitalized for ACS and to determine whether receipt of guideline-adherent antibiotics was associated with lower readmission rates...
September 11, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28882446/relationship-between-mixed-donor-recipient-chimerism-and-disease-recurrence-after-hematopoietic-cell-transplantation-for-sickle-cell-disease
#20
Allistair Abraham, Matthew Hsieh, Mary Eapen, Courtney Fitzhugh, Jeanette Carreras, Daniel Kessler, Gregory Guilcher, Naynesh Kamani, Mark C Walters, Jaap J Boelens, John Tisdale, Shalini Shenoy
Mixed donor chimerism after hematopoietic cell transplantation for sickle cell disease (SCD) can result in resolution of disease symptoms, but symptoms recur when donor chimerism is critically low. The relationship between chimerism, hemoglobin S (HbS) level, and symptomatic disease was correlated retrospectively in 95 patients who had chimerism reports available at day 100 and at 1 and 2 years after transplantation. Recurrent disease was defined as recurrence of vaso-occlusive crises, acute chest syndrome, stroke, and/or HbS levels > 50%...
September 4, 2017: Biology of Blood and Marrow Transplantation
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