Google
Trusted Resources: Evidence & Education
Scientific literature and patient education texts
Crises in Sickle Cell Disease
source: CHEST
year: 2016
authors: Novelli EM, Gladwin MT
summary/abstract:In spite of significant strides in the treatment of sickle cell disease (SCD), SCD crises are still responsible for high morbidity and early mortality. While most patients initially seek care in the acute setting for a seemingly uncomplicated pain episode (pain crisis or vaso-occlusive crisis), this initial event is the primary risk factor for potentially life-threatening complications. The pathophysiological basis of these illnesses is end-organ ischemia and infarction combined with the downstream effects of hemolysis that results from red blood cell sickling. These pathological changes can occur acutely and lead to a dramatic clinical presentation, but are frequently superimposed over a milieu of chronic vasculopathy, immune dysregulation, and decreased functional reserve. In the lungs, acute chest syndrome is a particularly ominous lung injury syndrome with a complex pathogenesis and potentially devastating sequelae, but all organ systems can be affected. It is, therefore, critical to understand the SCD patients’ susceptibility to acute complications and their risk factors so that they can be recognized promptly and managed effectively. Blood transfusions remain the mainstay of therapy for all severe acute crises. Recommendations and indications for the safest and most efficient implementation of transfusion strategies in the critical care setting are therefore presented and discussed, together with their pitfalls and potential future therapeutic alternatives. In particular, the importance of extended phenotypic red blood cell matching cannot be overemphasized, due to the high prevalence of severe complications from red cell alloimmunization in SCD.
organization: University of Pittsburgh; University of Pittsburgh School of MedicineDOI: 10.1016/j.chest.2015.12.016
read more full text
+myBinderRelated Content
-
Sickle Cell Disease and Cold Weather: Dos and Don’tsIn the United States today, one of every...
-
The first two years of life in sickle cell anemia infants: A results of a comprehensive longitudinal studyInfancy is a critical time during which ...
-
Progressive Loss of Brain Volume in Children with Sickle Cell Anemia: A Report from the Silent Cerebral Infarct Tran...Introduction: Neurological injury is a c...
-
Sickle Cell Treatments can Destroy Germ Cells in Boys, Affecting Fertility in Adulthood, Study SuggestsSome treatments for sickle cell disease ...
-
Sickle Cell Disease Management & Complications: Sophie Lanzkron MD of Johns HopkinsStuck on sickle cell disease? We hammer ...
-
Black Americans With Sickle Cell Trait At Increased Risk Of Kidney DiseaseAfrican Americans with sickle cell trait...
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.