| Background
Sickle cell disease is an inherited autosomal recessive blood condition and is one of themost prevalent genetic blood diseases worldwide.
Acute chest syndrome is a frequent complication of sickle cell disease, as well as a major cause of morbidity and the greatest single
cause of mortality in children with sickle cell disease. Standard treatment may include intravenous hydration, oxygen as treatment for
hypoxia, antibiotics to treat the infectious cause and blood transfusions may be given. This is an update of a Cochrane review first
published in 2010.
Objectives
To assess the effectiveness of blood transfusions, simple and exchange, for treating acute chest syndrome by comparing improvement
in symptoms and clinical outcomes against standard care.
Search methods
We searched The Cochrane Cystic Fibrosis and Genetic Disorders Group’s Haemoglobinopathies Trials Register, which comprises
references identified from comprehensive electronic database searches and handsearching of relevant journals and abstract books of
conference proceedings.
Date of the most recent search: 25 April 2016.
Selection criteria
Randomised controlled trials and quasi-randomised controlled trials comparing either simple or exchange transfusion versus standard
care (no transfusion) in people with sickle cell disease suffering from acute chest syndrome.
Data collection and analysis
Both authors independently selected trials and assessed the risk of bias, no data could be extracted.
Main results
One trial was eligible for inclusion in the review. While in the multicentre trial 237 people were enrolled (169 SCC, 42 SC, 15 S -
thalassemia, 11S+-thalassemia); themajority were recruited to an observational armand only ten participants met the inclusion criteria
for randomisation. Of these, four were randomised to the transfusion arm and received a single transfusion of 7 to 13 ml/kg packed
red blood cells, and six were randomised to standard care. None of the four participants who received packed red blood cells developed
acute chest syndrome, while 33% (two participants) developed acute chest syndrome in standard care arm. No data for any pre-defined
outcomes were available. |