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. 2024 Mar;204(3):1061-1066.
doi: 10.1111/bjh.19084. Epub 2023 Sep 6.

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 Munaretto  1 , P Corti  2 , E Bertoni  3 , S I Tripodi  4 , M E Guerzoni  5 , S Cesaro  6 , F Arcioni  7 , C Piccolo  8 , T Mina  4 , M Zecca  4 , D Cuzzubbo  9 , M Casale  10 , G Palazzi  5 , L D Notarangelo  3 , N Masera  2 , P Samperi  11 , S Perrotta  10 , G Russo  11 , L Sainati  1 , R Colombatti  1
Affiliations

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 Munaretto et al. Br J Haematol. 2024 Mar.

Abstract

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.

Keywords: Italy; acute chest syndrome; children; guideline adherence; sickle cell disease.

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Conflict of interest statement

No conflict of interests for this study.

References

REFERENCES

    1. Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000;342:1855-1865.
    1. Desselas E, Thuret I, Kaguelidou F, Benkerrou M, de Montalembert M, Odièvre MH, et al. Mortality in children with sickle cell disease in mainland France from 2000 to 2015. Haematologica. 2020;105(9):e440-e443.
    1. Lobo CLC, Nascimento EMD, Jesus LJC, Freitas TG, Lugon JR, Ballas SK. Mortality in children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil. Rev Bras Hematol Hemoter. 2018;40(1):37-42.
    1. Klings ES, Steinberg MH. Acute chest syndrome of sickle cell disease: genetics, risk factors, prognosis, and management. Expert Rev Hematol. 2022;15(2):117-125.
    1. Howard J, Hart N, Roberts-Harewood M, Cummins M, Awogbade M, Davis B, et al. Guideline on the management of acute chest syndrome in sickle cell disease. Br J Haematol. 2015;169(4):492-505.

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