Background: Sodium taurocholate cotransporting polypeptide (NTCP) deficiency (NTCPD) is a rare hereditary bile acid metabolism disorder caused by mutations in the SLC10A1 gene. Patients exhibit distinct clinical phenotypes across developmental stages, including neonatal cholestatic jaundice and hyperbilirubinemia, toddler-onset pruritus, school-age vitamin D insufficiency, and adult asymptomatic hypercholanemia or intrahepatic cholestasis of pregnancy. High symptom heterogeneity, frequent diagnostic delays, and the absence of standardized age-specific nursing protocols present significant challenges in clinical management. Purpose: This review aims to describe the clinical characteristics and nursing needs of NTCPD patients across the lifespan and to propose a practical, age-specific nursing management framework. Methods: A systematic literature search was conducted across PubMed, CINAHL, Embase, and CNKI in 2025 with no restriction on publication start date; 38 references were ultimately included from 312 initial records, supplemented by evidence from analogous cholestatic conditions where NTCPD-specific data were unavailable. Conclusions: An age-specific nursing management framework covering four developmental stages, with core components encompassing dynamic monitoring of bile acids and fat-soluble vitamins, individualized nutritional support, structured family health education, and caregiver psychological screening. This framework provides a theoretical basis for improving family caregiving capacity and optimizing patient outcomes, while highlighting the urgent need for prospective, multi-center research and the development of NTCPD-specific nursing guideline.
Zhu et al. (2026) studied this question.