An evidence-based specialist nursing protocol for children with hypoprothrombinemia-lupus anticoagulant syndrome (HLAS) and its application research.

Abstract

ObjectiveTo develop a standardized nursing protocol for pediatric hypoprothrombinemia-lupus anticoagulant syndrome (HLAS), a rare condition lacking unified clinical nursing guidelines worldwide, aiming to improve nurses' predictive, precise and whole-course care capabilities and optimize long-term clinical outcomes in affected children.MethodsEvidence-based nursing principles were integrated with clinical practice. After the admission and standardized management of a typical index case in March 2025, a multidisciplinary team composed of pediatric nephrology, rheumatology, laboratory medicine and nursing specialists conducted retrospective case analysis and collaborative protocol formulation, with its core innovation being the integration of thromboelastography (TEG) parameters to establish a quantitative bleeding risk stratification model. The systematic literature search covered all mainstream Chinese and English databases from database establishment to March 2025, consistent with the timeline of case admission and protocol development.ResultsApplication of this protocol in the index case enabled early identification of high bleeding risk, effectively avoiding severe bleeding events and nosocomial infections. The standardized health education and targeted psychological intervention significantly improved the disease cognition and family self-management ability of caregivers. It also transformed nurses from passive caregivers to proactive care managers.ConclusionThis protocol fills the gap in specialized nursing guidelines for pediatric HLAS, standardizes clinical nursing practices, and provides a replicable methodological reference for the development of standardized nursing protocols for other rare pediatric diseases.