Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease

Vichinsky, Elliott; Neumayr, Lynne; Earles, Ann N.; Williams, Roger A.; Lennette, Evelyne T.; Dean, Deborah; Nickerson, Bruce G.; Orringer, Eugene P.; McKie, Virgil; Bellevue, Rita; Daeschner, Charles W.; Manci, Elizabeth A.

doi:10.1056/nejm200006223422502

Cited by 1,109 publications

(1,229 citation statements)

References 40 publications

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“…The specific definition varies among studies [1,2]. It is a cause of frequent hospitalization and death and a common indication for transfusion and treatment with hydroxyurea [3,4].…”

Section: Introductionmentioning

confidence: 99%

“…The incidence is highest in young children with homozygous sickle cell (HbSS), with rates of 3-25 per 100 person-years in the Cooperative Study of SCD (CSSCD) [3]. Several studies suggest that the case fatality rate is lower in children (1.1-1.5%) than adults (4.3-9%), but ACS accounts for a significant proportion of mortality in both groups [2,3,5]. Certain characteristics at presentation (hemoptysis, productive cough, dyspnea, tachypnea, age >20 years, a prior vasoocclusive event, or a platelet count <200,000/µl) and during hospitalization (fever, extensive radiographic abnormalities, pleural effusion, transfusion, or mechanical ventilation) were associated with prolonged admission in the National Acute Chest Syndrome Study and the CSSCD [2,4].…”

Section: Introductionmentioning

confidence: 99%

“…Several studies suggest that the case fatality rate is lower in children (1.1-1.5%) than adults (4.3-9%), but ACS accounts for a significant proportion of mortality in both groups [2,3,5]. Certain characteristics at presentation (hemoptysis, productive cough, dyspnea, tachypnea, age >20 years, a prior vasoocclusive event, or a platelet count <200,000/µl) and during hospitalization (fever, extensive radiographic abnormalities, pleural effusion, transfusion, or mechanical ventilation) were associated with prolonged admission in the National Acute Chest Syndrome Study and the CSSCD [2,4]. However, neither study assessed predictors of readmission nor the impact of more recently proposed therapies for ACS, including high-dose dexamethasone and preemptive transfusion [1,6].…”

Section: Introductionmentioning

confidence: 99%

“…However, neither study assessed predictors of readmission nor the impact of more recently proposed therapies for ACS, including high-dose dexamethasone and preemptive transfusion [1,6]. Currently, the use of simple or exchange transfusion, antibiotics, oxygen, and bronchodilators for wheezing is the standard of care for severe (and perhaps moderate) ACS, based on improvement in oxygenation and other clinical parameters [2,7,8]. However, transfusion has not been evaluated in a clinical trial and was associated with a new red cell antibody in 2.4% of patients in a prospective study of ACS [2].…”

Section: Introductionmentioning

confidence: 99%

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Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease

Strouse¹,

Takemoto²,

Keefer³

et al. 2007

Pediatric Blood & Cancer

106

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Background-Acute chest syndrome (ACS) is a frequent cause of hospitalization and mortality in children with sickle cell disease. Transfusion is often required to prevent respiratory failure and treatment with dexamethasone may reduce the length of admission and the need for transfusions. We performed a retrospective cohort study to evaluate risk factors for readmission and prolonged hospitalization after different treatments for ACS.

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“…The specific definition varies among studies [1,2]. It is a cause of frequent hospitalization and death and a common indication for transfusion and treatment with hydroxyurea [3,4].…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease

Strouse¹,

Takemoto²,

Keefer³

et al. 2007

Pediatric Blood & Cancer

106

View full text Add to dashboard Cite

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“…Acute chest syndrome: Presence of a new pulmonary infiltrate on chest X-ray film and/or a defect on radionuclide imaging of the chest or abnormalities on spiral CT-scan, combined with acute respiratory illness [8,11].…”

Section: Methodsmentioning

confidence: 99%

Development and validation of a pediatric severity index for sickle cell patients

et al. 2010

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There is no instrument to measure severity of sickle cell disease (SCD) in pediatric patients that is generally accepted. The aim of this study was to develop and validate a severity index for SCD in children. We developed an index consisting of 12 items and tested its validity of the index using data from 92 children. We tested whether different scores were obtained for patients classified by severity both subjectively and objectively by a partially validated existing index. Furthermore, we tested whether the index could differentiate patients classified according to genotype or the number of a-gene deletions and evaluated whether the score on the index was correlated with the average number and days of hospitalizations/year, age and a risk of death score. We explored the effect of three different weighting systems (Score A, B, and C) to summarize these items. All weightings demonstrated a significant difference between the scores of mild, moderate, and severely affected patients, as classified by a subjective rating or with an existing index (P < 0.01). The index clearly differentiated patients by genotype (P < 0.01) or a-gene deletions (P < 0.01). The correlation with hospitalization was moderate. Age and the risk of death score were weakly associated with the pediatric severity index for SCD. This is the first pediatric SCD severity index that was developed and validated using modern clinimetric methodology. The validity and reliability of this index should be further evaluated in a prospective study including a larger cohort, preferably diagnosed at birth. Am. J. Hematol. 85:746-751, 2010. V

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Nitric Oxide for Inhalation in the Acute Treatment of Sickle Cell Pain Crisis

Gladwin¹

2011

JAMA

207

139

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Context Inhaled nitric oxide (NO) has shown evidence of efficacy in mouse models of sickle cell disease (SCD), case series of patients with acute chest syndrome, and 2 small placebo-controlled trials for treatment of vaso-occlusive pain crisis (VOC). Objective To determine whether inhaled NO gas reduces the duration of painful crisis in patients with SCD who present to the emergency room or hospital for care. Design, Setting and Participants Prospective, multicenter, double-blind, randomized, placebo-controlled clinical trial for up to 72 hours of inhaled NO gas versus inhaled nitrogen placebo in 150 participants presenting with VOC of SCD at 11 centers between October 5, 2004 and December 22, 2008. The primary endpoint was the time to resolution of painful crisis, defined by: 1) freedom from parenteral opioid use for 5 hours; 2) pain relief as assessed by visual analog pain scale scores ≤ 6 cm; 3) ability to walk; and 4) patient and family’s decision, with physician consensus, that the remaining pain could be managed at home. Intervention Inhaled NO gas versus inhaled nitrogen placebo. Results There was no significant change in the primary endpoint between the NO and the placebo groups, with a median time to resolution of crisis of 73.0 hours (95% CI: 46.0–91.0) and 65.5 hours (95% CI: 48.1–84.0), respectively (P=.87). There were no significant differences in secondary outcome measures, including length of hospitalization, VAS scores, cumulative opioid usage and the rate of acute chest syndrome. Inhaled NO was well tolerated with no increase in serious adverse events. Increases in venous methemoglobin concentration confirmed compliance and randomization, but did not exceed 5% in any study participant. Significant increases in plasma nitrate occurred in the treatment group, but there were no observed increases in plasma or whole blood nitrite. Conclusions Among patients with SCD hospitalized with VOC, the use of inhaled NO compared with placebo did not improve time to crisis resolution.

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Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease

Cited by 1,109 publications

References 40 publications

Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease

Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease

Development and validation of a pediatric severity index for sickle cell patients

Nitric Oxide for Inhalation in the Acute Treatment of Sickle Cell Pain Crisis

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