Acute Chest Syndrome

Respiratory syndrome characterized by the appearance of a new pulmonary infiltrate on chest x-ray, accompanied by symptoms of fever, cough, chest pain, tachypnea, or DYSPNEA, often seen in patients with SICKLE CELL ANEMIA. Multiple factors (e.g., infection, and pulmonary FAT EMBOLISM) may contribute to the development of the syndrome.

Also Known As:

Networked: 202 relevant articles (13 outcomes, 23 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pain (Aches)
2.	Sickle Cell Anemia (Hemoglobin S Disease)
3.	Pulmonary Hypertension (Ayerza Syndrome)
4.	Necrosis
5.	Stroke (Strokes)

Experts

1.	Miller, Scott T: 9 articles (04/2013 - 10/2003)
2.	Wang, Winfred C: 7 articles (12/2014 - 10/2003)
3.	Ware, Russell E: 7 articles (12/2014 - 01/2009)
4.	Casella, James F: 7 articles (10/2013 - 11/2006)
5.	Gordeuk, Victor R: 6 articles (10/2015 - 08/2008)
6.	DeBaun, Michael R: 6 articles (09/2015 - 11/2006)
7.	Ballas, Samir K: 6 articles (03/2015 - 01/2002)
8.	Knight-Madden, Jennifer M: 6 articles (01/2015 - 01/2012)
9.	Ataga, Kenneth I: 5 articles (12/2014 - 10/2011)
10.	Kato, Gregory J: 5 articles (07/2013 - 05/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Acute Chest Syndrome:

1.	Hydroxyurea (Hydrea)FDA LinkGeneric 03/01/2013 - "Hydroxyurea has documented laboratory efficacy with increases in Hb and HbF; treatment also significantly reduces the number of painful episodes, acute chest syndrome, transfusions, and hospitalizations. " 09/01/2004 - "Results of many clinical trials point to hydroxyurea administration for patients with frequent painful crises and acute chest syndrome. " 08/01/2013 - "results: The main indications of hydroxyurea were the prevention of the recidivism of an acute chest syndrome in 8 cases, iterative painful crises, more than 3 events per year, in 53 cases and anemic forms of the disease in 4 cases. " 03/01/2012 - "Plasma thrombospondin-1 is increased during acute sickle cell vaso-occlusive events and associated with acute chest syndrome, hydroxyurea therapy, and lower hemolytic rates." 10/01/2011 - "One patient not taking hydroxyurea developed a delayed hyperhemolytic transfusion reaction, and 2 children taking hydroxyurea developed acute chest syndrome. "
2.	Nitric Oxide (Nitrogen Monoxide)FDA Link 01/01/2008 - "To assess the effectiveness of inhaled nitric oxide for treating acute chest syndrome by comparing improvement in symptoms and clinical outcomes against standard care. " 12/01/2015 - "Inhaled nitric oxide for acute chest syndrome in adult sickle cell patients: a randomized controlled study." 05/01/2010 - "We present a case of acute chest syndrome successfully treated with inhaled nitric oxide and exchange transfusion. " 03/01/2008 - "Evidence is available to support decreased nitric oxide production, increased nitric oxide consumption, and abnormal metabolism of nitric oxide in patients with acute chest syndrome. " 03/01/2008 - "To review the role of endothelial dysfunction and nitric oxide metabolism in the pathogenesis of the acute chest syndrome. "
3.	Bronchodilator Agents (Bronchodilators)IBA 01/01/2014 - "Therefore, bronchodilators may be useful in the treatment of acute chest syndrome. " 01/01/2012 - "Therefore, bronchodilators may be useful in the treatment of acute chest syndrome. " 01/01/2003 - "Therefore, bronchodilators may be useful in the treatment of acute chest syndrome. " 01/01/2014 - "To assess the benefits and risks associated with the use of bronchodilators in people with acute chest syndrome. " 01/01/2012 - "To assess the benefits and risks associated with the use of bronchodilators in people with acute chest syndrome. "
4.	Arginine (L-Arginine)FDA Link 10/01/2010 - "Effect of oral arginine supplementation on exhaled nitric oxide concentration in sickle cell anemia and acute chest syndrome." 11/01/2000 - "Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome." 07/01/2003 - "Nitric oxide therapy by inhalation has improved pulmonary hypertension associated with acute chest syndrome in sickle cell disease, and several studies demonstrate therapeutic benefits of arginine therapy for primary and secondary pulmonary hypertension. " 03/01/2008 - "A thorough literature search of PubMed for publications relevant to acute chest syndrome and nitric oxide metabolism in sickle cell disease was performed using search terms that included acute chest syndrome, sickle cell disease, nitric oxide metabolism, arginine, nitrite, nitrate, exhaled nitric oxide, nitric oxide synthase, and oxidant injury. "
5.	Heme Oxygenase-1IBA 11/01/2012 - "Heme oxygenase-1 gene promoter polymorphism is associated with reduced incidence of acute chest syndrome among children with sickle cell disease."
6.	Hemoglobins (Hemoglobin)IBA 08/01/2011 - "The vaso-occlusive phenotype of SCD, which is marked by higher hemoglobin, manifests with frequent painful crises and is associated with a higher risk for developing acute chest syndrome. " 03/01/2012 - "Among steady-state patients at UPMC, TSP1 values correlated positively with lifetime history of acute chest syndrome (r = 0.72, P < 0.0001) and hemoglobin concentration (r = 0.49, P = 0.01), and negatively with markers of hemolysis, such as LDH (r = -0.50, P = 0.009). " 06/25/2002 - "This treatment increases the total hemoglobin concentration, reduces the vaso-occlusive complications of pain and acute chest syndrome, and attenuates mortality in adults. " 10/01/2012 - "Infants with lower hemoglobin at baseline were more likely to have a higher incidence of clinical events (acute chest syndrome, pain crisis, fever) as well as higher TCD velocities and lower neuropsychological scores at study exit. " 07/01/2013 - "Odds ratio of significant developmental delay was >9 times more likely among those who had experienced vaso-occlusive pain episodes, after controlling for socioeconomic status (SES), gender, pneumonia/acute chest syndrome, and hemoglobin concentration. "
7.	Sickle HemoglobinIBA 01/01/2013 - "The present study evaluated the relationship between acute chest syndrome (ACS) and autonomic nervous system (ANS) activity in patients with hemoglobin SS disease (Hb SS). " 01/01/2013 - "Relationship between acute chest syndrome and the sympatho-vagal balance in adults with hemoglobin SS disease; a case control study." 07/01/2013 - "A patient-reported history of one or more acute pulmonary events, either acute chest syndrome (ACS) or pneumonia, was obtained in 195 hemoglobin SS patients (52%) and 51 patients with other genotypes (40%). " 05/01/1996 - "The authors present a case of a child with hemoglobin SS disease who was treated with ECMO after acute chest syndrome and acute respiratory distress syndrome developed. " 08/01/2013 - "Red blood cell exchange (RBCEx) is frequently used in the management of patients with sickle cell disease (SCD) and acute chest syndrome or stroke, or to maintain target hemoglobin S (HbS) levels. "
8.	Biological Markers (Surrogate Marker)IBA 03/15/2008 - "The aim of the present study was to evaluate changes in pulmonary pressures and cardiac biomarkers during severe acute chest syndrome and their associations with mortality. " 09/01/2005 - "To compare the accuracy of biomarkers for identifying acute chest syndrome (ACS) in patients with sickle cell disease presenting to a pediatric emergency department (ED). " 09/01/2005 - "Serum biomarkers for identifying acute chest syndrome among patients who have sickle cell disease and present to the emergency department." 10/01/2011 - "Tapered oral dexamethasone for acute chest syndrome (ACS) in sickle cell anaemia was studied using a novel ACS assessment tool and investigational biomarkers. "
9.	Poloxamer (Poloxamer 407)IBA 05/01/2004 - "Safety of purified poloxamer 188 in sickle cell disease: phase I study of a non-ionic surfactant in the management of acute chest syndrome." 04/01/2002 - "Purified poloxamer 188 (PP188) is a non-ionic, block copolymer surfactant that is currently being evaluated clinically in sickle cell disease vaso-occlusive crisis and acute chest syndrome and preclinically in spinal cord injury and muscular dystrophy. "
10.	alpha-GlobinsIBA 06/01/1984 - "To further explore this relationship, we examined the incidence of painful episodes, acute chest syndrome, aseptic bone necrosis, and leg ulcers in 3 patient groups with sickle cell disease: (1) 2,147 patients over age 2 yr, stratified according to mean corpuscular volume (MCV); (2) 183 patients selected on the basis of microcytosis and elevated HbA2, on whom globin biosynthesis studies were done; and (3) 125 patients who had alpha-globin genotype assigned by restriction endonuclease gene mapping. "

Therapies and Procedures

1.	Blood Transfusion (Blood Transfusions) 10/01/2013 - "Vasoocclusive crises, blood transfusions, acute chest syndrome, sequestration crises and hospitalizations decreased significantly. " 11/01/2011 - "There was also a significant relationship between LV dilatation and frequent blood transfusions (>5/years) and acute chest syndrome (p<0.05). " 01/01/2010 - "No significant association with blood transfusion or acute chest syndrome (ACS), was found. " 01/01/2002 - "Acute chest syndrome is the most common form of organ failure and its management should be agressive, including adequate ventilation, multiple antibacterials and simple or exchange blood transfusion depending on its severity. " 05/01/1999 - "A decrease in the frequency of vaso-occlusive crises, acute chest syndrome, hemolytic crises, blood transfusions, and days spent in the hospital was demonstrated during the HU treatment period compared to the same period before. "
2.	Splenectomy 05/01/2010 - "Thirty-seven patients (64%) underwent laparoscopic splenectomy, and acute chest syndrome (ACS) was the most common post-operative complication (6.9%). " 09/01/2009 - "There was a significant difference between the splenectomy group and the non-splenectomy group, respectively, regarding Hb levels (median 7.2 g/dL vs. 12.5 g/dL, P < 0.0001), platelet counts (median 146 x 10(6)/L vs. 275 x 10(6)/L, P = 0.031), palpable spleen rate (66% vs. 16%, P = 0.0003%), acute chest syndrome frequency (75% vs. 12%P = 0.0004) and cholecystectomy rate (66% vs. 13%, P = 0.0004). " 05/01/2008 - "The purpose of this study was to determine the incidence of acute chest syndrome (ACS) in children with sickle cell disease (SCD) undergoing laparoscopic or open splenectomy and to assess factors that may predispose to this complication. " 11/01/2014 - "Acute chest syndrome after laparoscopic splenectomy in children with sickle cell disease: operative time dependent?" 05/01/2008 - "Acute chest syndrome after splenectomy in children with sickle cell disease."
3.	Contraception (Birth Control) 01/01/2005 - "A summary of SCD management principles in the areas of health care maintenance, transfusion therapy, treatment and prevention of painful episodes, acute chest syndrome, stroke, renal disease, contraception and pregnancy, and priapism is predominantly based on the authors' interpretation of available observational studies as well as the opinions of experts in SCD. "
4.	Extracorporeal Membrane Oxygenation (ECMO) 09/01/1997 - "This case suggests that treatment with venovenous ECMO should be strongly considered for sickle cell patients with life-threatening acute chest syndrome, despite maximal conventional support." 09/01/1997 - "This is the first report of a pediatric patient with acute chest syndrome successfully managed with venovenous ECMO. " 05/01/1996 - "To date, there have been 15 pediatric patients with acute chest syndrome treated with ECMO. " 09/01/2013 - "Previous reports have described the use of extracorporeal membrane oxygenation (ECMO) for acute chest syndrome of sickle cell disease (SCD). " 10/01/2011 - "Apparently data is sparse regarding the use of ECMO for treatment of a life-threatening acute chest syndrome in sickle cell disease. "
5.	Transplantation (Transplant Recipients) 02/01/2012 - "Indications for transplantation were vaso-occlusive crisis, acute chest syndrome, avascular bone necrosis, chronic RBC transfusions, or hemorrhagic stroke. " 12/01/1997 - "Indications for transplantation included a history of stroke (n = 17), recurrent acute chest syndrome or sickle pulmonary disease (n = 10), and recurrent vaso-occlusive crises (n = 7). " 05/01/2015 - "Before transplantation, 14 patients had recurrent, painful, vaso-occlusive crisis; ten patients had recurrent painful crisis in association with acute chest syndrome; three patients experienced ischaemic stroke and recurrent vaso-occlusive crisis; two patients experienced ischaemic stroke; one patient exhibited leukocytosis; and one patient exhibited priapism. " 08/08/1996 - "The indications for transplantation included a history of stroke (n = 12), recurrent acute chest syndrome (n = 5), and recurrent painful crises (n = 5). "