Guidelines for the management of emergencies and critical illness in pediatric and adult patients with sickle cell disease.

Summary

GRADE-based guidelines on the emergency and critical care management of sickle cell disease in both children and adults, covering 42 questions and 45 recommendations plus three therapeutic algorithms. Domains include referral and ICU admission criteria, vaso-occlusive crisis (analgesia, hydration, incentive spirometry, oxygenation targets), acute chest syndrome (imaging, corticosteroids, non-invasive ventilation, anticoagulation, antibiotics), transfusion indications and delayed haemolytic transfusion reactions, and priapism. Most recommendations rest on low-quality evidence or expert opinion, with some questions left unanswered. Relevant to PICU teams managing children with sickle cell crises and acute chest syndrome.

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