Trusted Resources: Evidence & Education
Scientific literature and patient education texts
Guideline on the management of acute chest syndrome in sickle cell disease
source: British Journal of Haematology
year: 2015
authors: Jo Howard, Nicholas Hart, Marilyn Roberts-Harewood, Michelle Cummins, Moji Awogbade, Bernard Davis
summary/abstract:Sickle cell disease (SCD) affects 12 000–15 000 individuals in the UK. Whilst homozygous SCD (sickle cell anaemia — HbSS) is the most common and severe genotype, and is where most of the evidence exists, this guidance should be used for all genotypes of SCD.
Acute chest syndrome (ACS) is defined as an acute illness characterized by fever and/or respiratory symptoms, accompanied by a new pulmonary infiltrate on chest X-ray (Charache et al, 1979; Ballas et al, 2010). This definition encompasses cases both where an infective organism is isolated and where no infective cause is identified. It is unique to SCD but in some cases ACS may appear to be similar to bacterial pneumonia in a patient without SCD. ACS may have a severe clinical course and can progress rapidly from mild hypoxia to respiratory failure and death. The presence of hypoxia is not included in the definition, but in clinical practice, hypoxia is a useful predictor of severity and outcome (Vichinsky et al, 1997, 2000).
Historically, ACS is one of the most common causes of death in patients with SCD (Platt et al, 1994; Lucas et al, 2008), although mortality is improving with improved medical management (Fitzhugh et al, 2010). ACS can also be associated with significant morbidity, including long-term parenchymal lung damage, pulmonary vascular abnormality and neurological sequelae. Patients may present to hospital acutely unwell with ACS or ACS may develop during a hospital admission following a painful crisis or post-operatively.
organization: Guy's and St Thomas' NHS Foundation Trust, London; St Thomas' Hospital, London; North Middlesex Hospital, London; Bristol Royal Hospital for Children, London; King's College Hospital, London; Whittington Hospital, London.DOI: 10.1111/bjh.13348
read more full text
+myBinderRelated Content
-
Survival in Sickle Cell Disease: Data from a Well-Resourced, National Health System SettingBackground: In well-resourced countries,...
-
Sickle cell disease and albuminuria: recent advances in our understanding of sickle cell nephropathyAlbuminuria is considered to be a releva...
-
Roland B. Scott Memorial Symposium: I Want Somebody to LoveRoland B. Scott Symposium on Sickle Cell...
-
Sickle Cell Patients are Innocent Victims of the Opioid Epidemic: Samir K. Ballas, MDhttps://www.youtube.com/watch?v=BOhY_gJo...
-
Sickle Cell Retinopathyhttps://www.youtube.com/watch?v=tCgAHWvV...
-
SUSTAIN Clinical Trial Results Show Crizanlizumab Reduced Sickle Cell–Related Pain CrisesResults from the Phase 2 SUSTAIN clinica...
-
Smartphone App May Help Assess Anemia Using Eyelid PicturesA smartphone app that measures hemoglobi...
To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More Information
The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.
Powered by
Support for this site is provided by
This platform is made possible through a partnership with the Sickle Cell Disease Association of America, Inc. (SCDAA) and its member organizations. SCDAA's mission is to advocate for people affected by sickle cell conditions and empower community-based organizations to maximize quality of life and raise public consciousness while advancing the search for a universal cure.