Guideline on the management of acute chest syndrome in sickle cell disease

Howard, Jo; Hart, Nicholas; Roberts-Harewood, M; Cummins, Michelle; Awogbade, Moji; Davis, Bernard A.

doi:10.1111/bjh.13348

Cited by 157 publications

(187 citation statements)

References 74 publications

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“…Acute thoracic syndrome (STA), a severe complication characterized by pulmonary infiltrates associated with at least one clinical sign or symptom such as chest pain, cough, wheezing, tachypnea and fever [8,[14][15][16][17]. The clinical characteristics of STA vary with age and can overlap with infectious causes and other pulmonary symptoms, preventing adequate diagnosis [1].…”

Section: Acute Thoracic Syndromementioning

confidence: 99%

“…The central focus of treatment in acute chest syndrome is to prevent or reverse acute respiratory failure, however, as the causes of the complication are relatively unknown in the scientific and medical setting, central therapy continues to be supportive and palliative [14]. Once the diagnosis is made, the treatment is directed to limit the progression of the disease in the acute phase, and later, to prevent its recurrence and sequelae that may occur in the long term.…”

Section: Therapeutic Approachesmentioning

confidence: 99%

“…Therefore all patients with the syndrome should receive broad spectrum parenteral antibiotics consisting of third generation cephalosporins such as penicillin's and macrolides such as azithromycin [34]. The antibiotic treatment regimen may further be modified depending on the culture results and the clinical condition of the patient [14,34]. For pregnant women at risk or at the beginning of untreated disease, the indicated antibiotic is vancomycin [7].…”

Section: Therapeutic Approachesmentioning

confidence: 99%

“…In addition, it may also be required in less severe degrees of hypoxemia depending on the patient's individual and clinical history or whether the patient's oxygen needs are increasing. For patients with severe disease characteristics, exchange transfusion is indicated [14,33]. Not yet investigated as a blood transfusion paper background as a clinical approach to an acute chest syndrome, therefore some randomized trials are needed [37,38].…”

Section: Transfusionmentioning

confidence: 99%

“…It is characterized by the presence of pulmonary infiltrates associated with at least one clinical sign or symptom such as chest pain, cough, wheezing, tachypnea, and fever [8][9][10]. This complication accounts for a quarter of sickle cell deaths (SCD [1,[11][12][13], and is also considered the second most common cause of hospitalization of patients with SCD [14]. Considering the clinical relevance of the disease, this article intends to carry out a small bibliographic review on the therapeutic approaches for STA.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Therapeutic Approaches of Acute Thoracic Syndrome in Patients with Falciform Disease

Ac¹,

Costa²,

Tec³

et al. 2017

J Blood Lymph

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Sickle cell disease (SCD) is an autosomal recessive inheritance disorder that affects the beta-globin gene and results in the replacement of the amino acid glutamic acid by valine in the β chain of the hemoglobin molecule, producing erythrocytes with defective forms and functions. A range of pathological conditions is associated with SCD, however, one in particular stands out by gravity. Acute thoracic syndrome (STA), characterized by the presence of pulmonary infiltrates associated with a clinical symptom such as chest pain, cough, wheezing, tachypnea and fever, is considered a leading cause of death in patients with sickle cell disease. Early diagnosis and introduction of an effective approach to complication are needed to improve outcomes and minimize associated morbidity and mortality. Risk factors associated with STA in the SCDAbdominal surgery for splenectomy and cholecystectomy [19] Hypersensitivity [20,21] Smoking [20,21] Asthma [22] Infections [9,[23][24][25] Post-Surgical Hypoventilation [9,[23][24][25] Fatty Embolism [9,[23][24][25]

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Section: Acute Thoracic Syndromementioning

confidence: 99%

Section: Therapeutic Approachesmentioning

confidence: 99%

Section: Therapeutic Approachesmentioning

confidence: 99%

Section: Transfusionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Therapeutic Approaches of Acute Thoracic Syndrome in Patients with Falciform Disease

Ac¹,

Costa²,

Tec³

et al. 2017

J Blood Lymph

View full text Add to dashboard Cite

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Incidence of Acute Chest Syndrome in Children With Sickle Cell Disease Following Implementation of the 13-Valent Pneumococcal Conjugate Vaccine in France

et al. 2022

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IMPORTANCEAcute chest syndrome (ACS) is one of the leading acute severe complications of sickle-cell disease (SCD). Although Streptococcus pneumoniae (S pneumoniae) is highly prevalent in children with SCD, its precise role in ACS is unclear. The efficacy of 13-valent pneumococcal conjugate vaccine (PCV13) implementation on ACS is still unknown. OBJECTIVE To assess the association of PCV13 implementation in the general pediatric population with the incidence of ACS in children with SCD. DESIGN, SETTING, AND PARTICIPANTS This cohort study used an interrupted time-series analysis of patient records from a national hospital-based French surveillance system. All children younger than 18 years with SCD (based on the International Statistical Classification of Diseases and Related Health Problems, Tenth Revision definition) hospitalized in France between January 2007 and December 2019 were included. EXPOSURES PCV13 implementation. MAIN OUTCOMES AND MEASURESMonthly incidence of ACS per 1000 children with SCD over time as analyzed by segmented linear regression with autoregressive error; monthly incidence of hospitalization for vaso-occlusive crisis, asthma crisis, and acute pyelonephritis per 1000 children with SCD over the same period as the control outcomes. RESULTS Among the 107 694 hospitalizations of children with SCD, 4007 episodes of ACS were included (median [IQR] age,[8][9][10][11][12] years; 2228 [55.6%] boys). PCV13 implementation in 2010 was followed by a significant decrease in the incidence of ACS (−0.9% per month; 95% CI, −1.4% to −0.4%; P < .001), with an estimated cumulative change of −41.8% (95% CI, −70.8% to −12.7%) by 2019. Sensitivity analyses yielded the same results, including the incidence of ACS adjusted for that of vaso-occlusive crisis over time. The results were similar among different age groups. By contrast, no change was found for the 3 control outcomes over the study period.CONCLUSIONS AND RELEVANCE PCV13 implementation was associated with an important reduction in the incidence of ACS in children with SCD. This vaccine benefit provides new evidence of the key role of S pneumoniae in ACS and should be considered when estimating outcomes associated with current PCVs and the potential benefit of next-generation PCVs in children.

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Association of Guideline-Adherent Antibiotic Treatment With Readmission of Children With Sickle Cell Disease Hospitalized With Acute Chest Syndrome

et al. 2017

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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. DESIGN, SETTING, AND PARTICIPANTSRetrospective cohort study including 14 480 hospitalizations in 7178 children (age 0-22 years) with a discharge diagnosis of SCD and either ACS or pneumonia. Information was obtained from 41 children's hospitals submitting data to the Pediatric Health Information System from January 1, 2010, to December 31, 2016.EXPOSURES National Heart, Lung, and Blood Institute guideline-adherent (macrolide with parenteral cephalosporin) vs non-guideline-adherent antibiotic regimens. MAIN OUTCOMES AND MEASURESAcute chest syndrome-related and all-cause 7-and 30-day readmissions. RESULTSOf the 14 480 hospitalizations, 6562 (45.3%) were in girls; median (interquartile range) age was 9 (4-14) years. Guideline-adherent antibiotics were provided in 10 654 of 14 480 hospitalizations for ACS (73.6%). Hospitalizations were most likely to include guideline-adherent antibiotics for children aged 5 to 9 years (3230 of 4047 [79.8%]) and declined to the lowest level for children 19 to 22 years (697 of 1088 [64.1%]). Between-hospital variation in antibiotic regimens was wide, with use of guideline-adherent antibiotics ranging from 24% to 90%. Children treated with guideline-adherent antibiotics had lower 30-day ACS-related (odds ratio [OR], 0.71; 95% CI, 0.50-1.00) and all-cause (OR, 0.50; 95% CI, 0.39-0.64) readmission rates vs children who received other regimens (cephalosporin and macrolide vs neither drug class).CONCLUSIONS AND RELEVANCE Current approaches to antibiotic treatment in children with ACS vary widely, but guideline-adherent therapy appears to result in fewer readmissions compared with non-guideline-adherent therapy. Efforts to increase the dissemination and implementation of SCD treatment guidelines are warranted as is comparative effectiveness research to strengthen the underlying evidence base.

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Guideline on the management of acute chest syndrome in sickle cell disease

Cited by 157 publications

References 74 publications

Therapeutic Approaches of Acute Thoracic Syndrome in Patients with Falciform Disease

Therapeutic Approaches of Acute Thoracic Syndrome in Patients with Falciform Disease

Incidence of Acute Chest Syndrome in Children With Sickle Cell Disease Following Implementation of the 13-Valent Pneumococcal Conjugate Vaccine in France

Association of Guideline-Adherent Antibiotic Treatment With Readmission of Children With Sickle Cell Disease Hospitalized With Acute Chest Syndrome

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