The Experts below are selected from a list of 12069 Experts worldwide ranked by ideXlab platform
Michael R. Debaun - One of the best experts on this subject based on the ideXlab platform.
-
rapidly progressive Acute Chest Syndrome in individuals with sickle cell anemia a distinct Acute Chest Syndrome phenotype
American Journal of Hematology, 2016Co-Authors: Shruti Chaturvedi, Djamila Labib Ghafuri, Jeffrey A. Glassberg, Adetola A. Kassim, Mark Rodeghier, Michael R. DebaunAbstract:Current definitions of Acute Chest Syndrome (ACS) in sickle cell anemia (SCA) do not account for the rapidity of progression of respiratory compromise. In this two-center retrospective cohort study, we tested the hypothesis that in patients with SCA, rapidly progressive ACS characterized by respiratory failure (requiring at least 3L of oxygen or intubation and mechanical ventilation to maintain oxygen saturation ≥ 90%) within 24 hours of onset of respiratory symptoms exists as a distinct phenotype and is associated with multi-organ failure. We identified 173 individuals (97 children <20 years and 76 adults ≥20 years) with SCA and at least one episode of ACS. Only one ACS episode, either the most severe or most recent, was considered per individual. Rapidly progressive ACS occurred in 21% (n=16) adults but only 2.1% (n=2) children. Compared with adults without rapidly progressive ACS, adults with rapidly progressive ACS more frequently developed Acute kidney injury (68.8% vs. 3.3%, P<0.001), hepatic dysfunction (75.0% vs. 15.0%, P<0.001), altered mental status (43.8% vs. 11.7%, P<0.001), multiorgan failure (93.8% vs. 10%, P<0.001) and death (6.3% vs. 0%, P=0.05). Clinical and laboratory covariates that were evaluable on the first day of respiratory symptoms were evaluated to identify predictors of rapidly progressive ACS. On multivariable analysis, decline in platelet count at presentation was the only predictor of rapidly progressive ACS [odds ratio 6.20 (95% CI 1.33-28.90), P= 0.020]. In conclusion, rapidly progressive ACS is a distinct phenotype that occurs more frequently in adults, is preceded by thrombocytopenia, and associated with multiorgan failure. This article is protected by copyright. All rights reserved.
-
Rapidly progressive Acute Chest Syndrome in individuals with sickle cell anemia: a distinct Acute Chest Syndrome phenotype.
American journal of hematology, 2016Co-Authors: Shruti Chaturvedi, Djamila Labib Ghafuri, Jeffrey A. Glassberg, Adetola A. Kassim, Mark Rodeghier, Michael R. DebaunAbstract:Current definitions of Acute Chest Syndrome (ACS) in sickle cell anemia (SCA) do not account for the rapidity of progression of respiratory compromise. In this two-center retrospective cohort study, we tested the hypothesis that in patients with SCA, rapidly progressive ACS characterized by respiratory failure (requiring at least 3L of oxygen or intubation and mechanical ventilation to maintain oxygen saturation ≥ 90%) within 24 hours of onset of respiratory symptoms exists as a distinct phenotype and is associated with multi-organ failure. We identified 173 individuals (97 children
-
The intersection between asthma and Acute Chest Syndrome in children with sickle-cell anaemia
Lancet (London England), 2016Co-Authors: Michael R. Debaun, Robert C. StrunkAbstract: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. Although understanding has increased about asthma as a comorbidity in sickle-cell disease and its effects on morbidity, substantial gaps remain in knowledge about best management.
Hiroshi Noto - One of the best experts on this subject based on the ideXlab platform.
-
Hemoglobin SC disease presenting as Acute Chest Syndrome with ventilation-perfusion mismatches.
Respiration; international review of thoracic diseases, 1999Co-Authors: Hiroshi NotoAbstract:Acute Chest Syndrome, characterized by fever, Chest pain and pulmonary infiltrates, is a known complication of hemoglobin SC disease. A 41-year-old African male with previously unknown hemoglobinopathy developed an Acute episode of fever, Chest pain and dyspnea right after surgery for retinal detachment. The patient was suspected of having pulmonary thromboembolism. This was further suggested by a 'high probability' ventilation-perfusion scan. However, a pulmonary angiogram revealed no evidence of thromboembolism. Subsequently, the patient was recognized to have hemoglobin SC disease, with the Acute Chest Syndrome. After appropriate treatment with antibiotics, hydration and exchange transfusion, the patient underwent another surgery without complications.
-
Hemoglobin SC disease presenting as Acute Chest Syndrome with ventilation-perfusion mismatches.
Respiration; international review of thoracic diseases, 1999Co-Authors: Hiroshi NotoAbstract:Acute Chest Syndrome, characterized by fever, Chest pain and pulmonary infiltrates, is a known complication of hemoglobin SC disease. A 41-year-old African male with previously unknown hemoglobinopath
Cédric Broquere - One of the best experts on this subject based on the ideXlab platform.
-
Hemorheological risk factors of Acute Chest Syndrome and painful vaso-occlusive crisis in children with sickle cell disease.
Haematologica, 2012Co-Authors: Yann Lamarre, Marc Romana, Xavier Waltz, Marie Laure Lalanne-mistrih, Benoit Tressières, Lydia Divialle-doumdo, Marie-dominique Hardy-dessources, Jens Vent-schmidt, Marie Petras, Cédric BroquereAbstract:BACKGROUND: Little is known about the effects of blood rheology on the occurrence of Acute Chest Syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease. DESIGN AND METHODS: To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed in 44 children with sickle cell anemia and 49 children with hemoglobin SC disease (8-16 years old) followed since birth. Clinical charts were retrospectively reviewed to determine prior Acute Chest Syndrome or vaso-occlusive episodes, and rates of these complications were calculated. RESULTS: Multivariate analysis revealed that: 1) a higher steady-state blood viscosity was associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, but not in children with hemoglobin SC disease; 2) a higher steady-state red blood cell disaggregation threshold was associated with previous history of Acute Chest Syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. CONCLUSIONS: Our results indicate for the first time that the red blood cell aggregation properties may play a role in the pathophysiology of Acute Chest Syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. In addition, whereas greater blood viscosity is associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, no association was found in children with hemoglobin SC disease, underscoring differences in the etiology of vaso-occlusive crises between sickle cell anemia and hemoglobin SC disease.
-
Hemorheological risk factors of Acute Chest Syndrome and painful vaso-occlusive crisis in children with sickle cell disease
Haematologica, 2012Co-Authors: Yann Lamarre, Marc Romana, Xavier Waltz, Marie Laure Lalanne-mistrih, Benoit Tressières, Lydia Divialle-doumdo, Marie-dominique Hardy-dessources, Jens Vent-schmidt, Marie Petras, Cédric BroquereAbstract:Little is known about the effects of blood rheology on the occurrence of Acute Chest Syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease. To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed in 44 children with sickle cell anemia and 49 children with hemoglobin SC disease (8-16 years old) followed since birth. Clinical charts were retrospectively reviewed to determine prior Acute Chest Syndrome or vaso-occlusive episodes, and rates of these complications were calculated. Multivariate analysis revealed that: 1) a higher steady-state blood viscosity was associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, but not in children with hemoglobin SC disease; 2) a higher steady-state red blood cell disaggregation threshold was associated with previous history of Acute Chest Syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. Our results indicate for the first time that the red blood cell aggregation properties may play a role in the pathophysiology of Acute Chest Syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. In addition, whereas greater blood viscosity is associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, no association was found in children with hemoglobin SC disease, underscoring differences in the etiology of vaso-occlusive crises between sickle cell anemia and hemoglobin SC disease.
Marc Romana - One of the best experts on this subject based on the ideXlab platform.
-
Hemorheological risk factors of Acute Chest Syndrome and painful vaso-occlusive crisis in children with sickle cell disease.
Haematologica, 2012Co-Authors: Yann Lamarre, Marc Romana, Xavier Waltz, Marie Laure Lalanne-mistrih, Benoit Tressières, Lydia Divialle-doumdo, Marie-dominique Hardy-dessources, Jens Vent-schmidt, Marie Petras, Cédric BroquereAbstract:BACKGROUND: Little is known about the effects of blood rheology on the occurrence of Acute Chest Syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease. DESIGN AND METHODS: To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed in 44 children with sickle cell anemia and 49 children with hemoglobin SC disease (8-16 years old) followed since birth. Clinical charts were retrospectively reviewed to determine prior Acute Chest Syndrome or vaso-occlusive episodes, and rates of these complications were calculated. RESULTS: Multivariate analysis revealed that: 1) a higher steady-state blood viscosity was associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, but not in children with hemoglobin SC disease; 2) a higher steady-state red blood cell disaggregation threshold was associated with previous history of Acute Chest Syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. CONCLUSIONS: Our results indicate for the first time that the red blood cell aggregation properties may play a role in the pathophysiology of Acute Chest Syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. In addition, whereas greater blood viscosity is associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, no association was found in children with hemoglobin SC disease, underscoring differences in the etiology of vaso-occlusive crises between sickle cell anemia and hemoglobin SC disease.
-
Hemorheological risk factors of Acute Chest Syndrome and painful vaso-occlusive crisis in children with sickle cell disease
Haematologica, 2012Co-Authors: Yann Lamarre, Marc Romana, Xavier Waltz, Marie Laure Lalanne-mistrih, Benoit Tressières, Lydia Divialle-doumdo, Marie-dominique Hardy-dessources, Jens Vent-schmidt, Marie Petras, Cédric BroquereAbstract:Little is known about the effects of blood rheology on the occurrence of Acute Chest Syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease. To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed in 44 children with sickle cell anemia and 49 children with hemoglobin SC disease (8-16 years old) followed since birth. Clinical charts were retrospectively reviewed to determine prior Acute Chest Syndrome or vaso-occlusive episodes, and rates of these complications were calculated. Multivariate analysis revealed that: 1) a higher steady-state blood viscosity was associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, but not in children with hemoglobin SC disease; 2) a higher steady-state red blood cell disaggregation threshold was associated with previous history of Acute Chest Syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. Our results indicate for the first time that the red blood cell aggregation properties may play a role in the pathophysiology of Acute Chest Syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. In addition, whereas greater blood viscosity is associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, no association was found in children with hemoglobin SC disease, underscoring differences in the etiology of vaso-occlusive crises between sickle cell anemia and hemoglobin SC disease.
-
ET-1 and ecNOS gene polymorphisms andsusceptibility to Acute Chest Syndrome and painful vaso-occlusive crises in children with sickle cell anemia.
Haematologica, 2006Co-Authors: Vicky Chaar, Vanessa Tarer, Maryse Etienne-julan, Jean Pierre Diara, Jacques Elion, Marc RomanaAbstract:The association of endothelin 1 (ET-1) and endothelial constitutive nitric oxide synthase (ecNOS) gene polymorphisms (G5665T and T8002C, VNTR and T-786C respectively) with the occurrence of Acute Chest Syndrome and painful vaso-occlusive crises was evaluated in homozygous SS children. This retrospective study reveals that ET-1 T8002 and ecNOS C-786 alleles are associated with, respectively, an increased and a decreased risk of Acute Chest Syndrome.
Muriel Fartoukh - One of the best experts on this subject based on the ideXlab platform.
-
Extracorporeal Life Support for Severe Acute Chest Syndrome in Adult Sickle Cell Disease: A Preliminary Report.
Critical care medicine, 2019Co-Authors: Florence Boissier, Muriel Fartoukh, François Bagate, Matthieu Schmidt, Vincent Labbé, Antoine Kimmoun, Armand Mekontso DessapAbstract:Objectives:Extracorporeal life support could be helpful for severe Acute Chest Syndrome in adults sickle cell disease, because of the frequent hemodynamic compromise in this setting, including Acute pulmonary vascular dysfunction and right ventricular failure. The aim of this study was to report the
-
Plastic bronchitis: An unusual complication of Acute Chest Syndrome in adult.
Respiratory medicine case reports, 2017Co-Authors: Sarah Feray, Pierre Mora, Maxens Decavèle, Tài Pham, El Mahdi Hafiani, Muriel FartoukhAbstract: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. Realization of fiberoptic bronchoscopy to diagnose endobronchial injury and preventive measures such as fluidification of sputum at the early stage of thoracic vaso-occlusive crisis are essential.
-
Early intermittent noninvasive ventilation for Acute Chest Syndrome in adults with sickle cell disease: a pilot study
Intensive care medicine, 2010Co-Authors: Muriel Fartoukh, Bernard Maitre, Anoosha Habibi, Yannick Lefort, Dora Bachir, Frédéric Galactéros, Bertrand Godeau, Laurent BrochardAbstract:Purpose Alveolar hypoxia and hypoxic vasoconstriction lead to trapping of sickle cells within the pulmonary vasculature. Improving alveolar ventilation and oxygenation may improve the outcome of Acute Chest Syndrome (ACS).
