Acute chest syndrome in children with sickle cell disease: Data from a national AIEOP cohort identify priority areas of intervention in a hub-and-spoke system.
V MunarettoPaola CortiE BertoniS I TripodiM E GuerzoniS CesaroF ArcioniC PiccoloT MinaMarco ZeccaD CuzzubboMaddalena CasaleG PalazziL D NotarangeloN MaseraP SamperiS PerrottaGiovanna RussoL SainatiRaffaella ColombattiPublished in: British journal of haematology (2023)
Acute chest syndrome (ACS) is a frequent cause of hospitalization in sickle cell disease (SCD). Despite advances in acute care, many settings still lack knowledge about ACS best practices. After the AIEOP Guidelines were published in 2012, suggesting standardized management in Italy, a retrospective study was performed to assess the diagnostic and therapeutic pathways of ACS in children. From 2013 to 2018, 208 ACS episodes were presented by 122/583 kids in 11 centres. 73 were male, mean age 10.9 years, 85% African, 92% HbSS or Sβ°. In our hub-and-spoke system, a good adherence to Guidelines was documented, but discrepancies between reference centres and general hospitals were noted. Improvement is needed for timely transfer to reference centres, use of incentive spirometry, oxygen therapy and pain management.
Keyphrases
- acute coronary syndrome
- pain management
- sickle cell disease
- acute care
- liver failure
- healthcare
- young adults
- respiratory failure
- randomized controlled trial
- clinical practice
- chronic pain
- primary care
- aortic dissection
- case report
- network analysis
- quality improvement
- electronic health record
- lung function
- hepatitis b virus
- type diabetes
- systematic review
- metabolic syndrome
- bioinformatics analysis
- machine learning
- adipose tissue
- intensive care unit
- extracorporeal membrane oxygenation
- glycemic control
- skeletal muscle
- mechanical ventilation
- acute respiratory distress syndrome
- weight loss