Pediatric Hematology Oncology Unit, Department of Woman's and Child's Health, Azienda Ospedale-Università di Padova, Padova, Italy.
Clinica si Onco-Ematologia Pediatrica, Fondazione MBBM Azienda Ospedaliera S. Gerardo, Monza, Italy.
Br J Haematol. 2024 Mar;204(3):1061-1066. doi: 10.1111/bjh.19084. Epub 2023 Sep 6.
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.
急性胸部综合征（ACS）是镰状细胞病（SCD）患者频繁住院的主要原因。尽管急性护理取得了进展，但许多地区仍然缺乏 ACS 最佳实践的相关知识。2012 年 AIEOP 指南发布后，建议在意大利采用标准化管理，此后进行了一项回顾性研究，以评估意大利儿童 ACS 的诊断和治疗途径。2013 年至 2018 年，11 个中心的 583 名儿童中有 122 名出现了 208 次 ACS 发作，其中 73 名男性，平均年龄 10.9 岁，85%为非洲裔，92%为 HbSS 或 Sβ°。在我们的轮辐式系统中，记录到对指南的良好遵循，但参考中心和综合医院之间存在差异。需要改进及时转至参考中心、使用激励式肺活量计、氧疗和疼痛管理。
