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.
| Published in | American Journal of Nursing Science (Volume 15, Issue 2) |
| DOI | 10.11648/j.ajns.20261502.13 |
| Page(s) | 35-43 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2026. Published by Science Publishing Group |
NTCP Deficiency, Age-specific Nursing Care, Bile Acid Metabolism, Symptom Management
| [1] | Song Yuanzong. Pathogenesis, clinical manifestations and advances in diagnosis and treatment of sodium taurocholate cotransporting polypeptide deficiency [J]. Journal of Clinical Hepatology, 2019, 35(8): 1690–1692. |
| [2] |
Vaz FM, Huidekoper HH, Paulusma CC. Extended Abstract: Deficiency of Sodium Taurocholate Cotransporting Polypeptide (SLC10A1): A New Inborn Error of Metabolism with an Attenuated Phenotype. Dig Dis. 2017; 35(3): 259-260.
https://doi.org/10.1159/000450984 Epub 2017 Mar 1. |
| [3] |
Deng M, Mao M, Guo L, Chen FP, Wen WR, Song YZ. Clinical and molecular study of a pediatric patient with sodium taurocholate cotransporting polypeptide deficiency. Exp Ther Med. 2016 Nov; 12(5): 3294-3300.
https://doi.org/10.3892/etm.2016.3752 Epub 2016 Sep 27. |
| [4] | Jiang Qi. Clinical analysis and literature review of 8 cases of sodium taurocholate cotransporting polypeptide deficiency [D]. Chengdu Medical College, 2023. |
| [5] | Yang Fengxia, Zeng Fansen, Tan Limei, et al. Clinical features and SLC10A1 gene mutation analysis of sodium taurocholate cotransporting polypeptide deficiency [J]. Journal of Clinical Hepatology, 2022, 38(3): 613–616. |
| [6] | Li Hua, Qiu Jianwu, Lin Guizhi, et al. Clinical and genetic analysis of a child with sodium taurocholate cotransporting polypeptide deficiency [J]. Chinese Journal of Contemporary Pediatrics, 2018, 20(4): 279–284. |
| [7] | Erlinger S. NTCP deficiency: A new inherited disease of bile acid transport [J]. Clinics and Research in Hepatology and Gastroenterology, 2015, 39(1): 7–8. |
| [8] | Chen Xiao, Zhang Yuxia, Gu Ying. A qualitative study on family management approaches in children with hereditary cholestatic liver disease [J]. Journal of Nursing Science, 2018, 33(11): 90–93. |
| [9] | Zhang N, Song Y, Chen W, et al. Nursing care of an extremely preterm infant with sodium taurocholate cotransporting polypeptide deficiency: A case report [J]. American Journal of Nursing Science, 2020, 9(4). |
| [10] | Huang Fangli, Deng Yinye, Lai Wuchao, et al. Clinical features and gene analysis of 14 children with sodium taurocholate cotransporting polypeptide deficiency [J]. Chinese Journal of New Clinical Medicine, 2025, 18(3): 263–267. |
| [11] | Song Yuanzong, Deng Mei. A difficult case: sodium taurocholate cotransporting polypeptide deficiency presenting as cholestatic jaundice in early infancy [J]. Chinese Journal of Contemporary Pediatrics, 2017, 19(3): 350–354. |
| [12] | Chen Meixue. Correlation study of illness uncertainty, parenting stress, and family management approaches in parents of children with NTCPD [D]. Jinan University. |
| [13] | Liu Yufeng, Luo Yongqi, Zhou Fanren, et al. Systematic management of skin pruritus in children [J]. Contemporary Nurses (First Edition), 2018, 25(11): 124–126. |
| [14] | Cao Chunju, Wang Wen, Huang Meiying, et al. Best evidence summary for the management of pruritus in cholestatic patients [J]. Journal of Nursing (China), 2024, 31(18): 39–44. |
| [15] | Xu Lin, Xu Huiwen, Wang Suhang. Translation and reliability/validity evaluation of the 12-item Pruritus Scale (Chinese version) [J]. Journal of Nursing Science, 2019, 34(16): 26–29. |
| [16] |
Varni JW, Burwinkle TM, Seid M, Skarr D. The PedsQL 4.0 as a pediatric population health measure: feasibility, reliability, and validity. Ambul Pediatr. 2003 Nov-Dec; 3(6): 329-41.
https://doi.org/10.1367/1539-4409(2003)003<0329:tpaapp>2.0.co;2 |
| [17] | Dong Chen. Basic and clinical research on cholestatic liver disease in children [D]. Huazhong University of Science and Technology, 2019. |
| [18] | Depping MK, Uhlenbusch N, Härter M, Schramm C, Löwe B. Efficacy of a Brief, Peer-Delivered Self-management Intervention for Patients With Rare Chronic Diseases: A Randomized Clinical Trial. JAMA Psychiatry. 2021 Jun 1; 78(6): 607-615. |
| [19] | Ding Haiyan, Chen Qi, Zhang Lianhong. Two cases of neonatal sodium taurocholate cotransporting polypeptide deficiency and literature review [J]. Laboratory Medicine and Clinics, 2023, 20(11): 1658–1660. |
| [20] | Expert Consensus on the Clinical Application of Vitamin A and Vitamin D in Chinese Children [Z]. |
| [21] | Tang Aiqun, Tang Yuxuan, Zhang Dexiang, et al. Analysis of fat-soluble vitamin levels in children with cholestatic liver disease [J]. Chinese Journal of Liver Disease (Electronic Edition), 2021, 13(3): 48–55. |
| [22] | Li Jing, Xia Jing, Wu Jie. Correlation analysis between fat-soluble vitamin levels and biochemical indicators in children with cholestatic liver disease [J]. Journal of Clinical Hepatology, 2021, 37(10): 2376–2379. |
| [23] | Yin Chunlan, Zhao Ruiqin, Fu Haiyan, et al. Analysis of growth and developmental status and influencing factors in infants with intrahepatic cholestasis [J]. Clinical Medicine Research and Practice, 2023, 8(14): 1–4. |
| [24] | Li Hua, Xiong Qiulan, Zhang Lu, et al. Effectiveness of evidence-based nursing combined with health education in phototherapy for neonatal jaundice [J]. Journal of Rare and Uncommon Diseases, 2025, 32(9): 158–160. |
| [25] | Chen Qiaoling, Lu Wei. Research progress on nutritional risk screening in hospitalized children [J]. Chinese Journal of Child Health Care, 2024, 32(6): 653–660. |
| [26] |
White M, Lawson K, Ramsey R, Dennis N, Hutchinson Z, Soh XY, Matsuyama M, Doolan A, Todd A, Elliott A, Bell K, Littlewood R. Simple Nutrition Screening Tool for Pediatric Inpatients. JPEN J Parenter Enteral Nutr. 2016 Mar; 40(3): 392-8.
https://doi.org/10.1177/0148607114544321 Epub 2014 Aug 5. |
| [27] | Varni JW, Seid M, Kurtin PS. PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care. 2001 Aug; 39(8): 800-12. |
| [28] |
Bozzini AB, Neder L, Silva CA, Porta G. Decreased health-related quality of life in children and adolescents with autoimmune hepatitis. J Pediatr (Rio J). 2019 Jan-Feb; 95(1): 87-93.
https://doi.org/10.1016/j.jped.2017.10.013 Epub 2018 Jan 10. |
| [29] | Gulati R, Radhakrishnan KR, Hupertz V, Wyllie R, Alkhouri N, Worley S, Feldstein AE. Health-related quality of life in children with autoimmune liver disease. JPediatr Gastroenterol Nutr. 2013 Oct; 57(4): 444-50. |
| [30] |
Elman S, Hynan LS, Gabriel V, Mayo MJ. The 5-D itch scale: a new measure of pruritus. Br J Dermatol. 2010 Mar; 162(3): 587-93.
https://doi.org/10.1111/j.1365-2133.2009.09586.x Epub 2009 Dec 1. |
| [31] |
Levy C, Younossi ZM, Mayo MJ, Kremer AE, Pate J, Ieyoub J, Zincavage R, Padilla B, Ito D, Perez CFM. Assessment of PBC pruritus from the ITCH-E Study: quality of life, productivity, and treatment experiences. Liver Int. 2025 Sep; 45(9): e70276.
https://doi.org/10.1111/liv.70276 Digital Object Identifier. |
| [32] | Zarit SH, Reever KE, Bach-Peterson J. Relatives of the impaired elderly: correlates of feelings of burden [J]. Gerontologist, 1980, 20(6): 649–655. |
| [33] | Wang Lie, Yang Xiaoshi, Hou Zhe, et al. Application and evaluation of the Chinese version of the Caregiver Burden Inventory [J]. Chinese Journal of Public Health, 2006(8): 970–972. |
| [34] | Kemper AR, Newman TB, Slaughter JL, Maisels MJ, Watchko JF, Downs SM, Grout RW, Bundy DG, Stark AR, Bogen DL, Holmes AV, Feldman-Winter LB, Bhutani VK, Brown SR, Maradiaga Panayotti GM, Okechukwu K, Rappo PD, Russell TL. Clinical Practice Guideline Revision: Management of Hyperbilirubinemia in the Newborn Infant 35 or More Weeks of Gestation. Pediatrics. 2022 Sep 1; 150(3): e2022058859. |
| [35] | Wu Hongjing. Relationship between family food environment and dietary behavior in school-age children: the mediating role of nutrition literacy [D]. Hebei Medical University, 2024. |
| [36] |
Thomas S, Ryan NP, Byrne LK, Hendrieckx C, White V. Unmet supportive care needs of families of children with chronic illness: A systematic review. J Clin Nurs. 2023 Oct; 32(19-20): 7101-7124.
https://doi.org/10.1111/jocn.16806 Epub 2023 Jun 23. |
| [37] | Burns S, Doering K, Koller D, Stratton C. School reintegration following hospitalisation for children with medical complexity and chronic disease diagnoses: a scoping review protocol. BMJ Open. 2021 Oct 25; 11(10): e052493. |
| [38] | Huang Yun, Liu Yanhong, Huang Li, et al. Association between nutrition literacy and unhealthy dietary behaviors in school-age children [J]. Practical Preventive Medicine, 2025, 32(10): 1199–1204+1208. |
APA Style
Zhu, M., Yang, S., Cheng, R., Song, Y. (2026). Age-specific Nursing Management for Sodium Taurocholate Cotransporting Polypeptide Deficiency: A Narrative Review. American Journal of Nursing Science, 15(2), 35-43. https://doi.org/10.11648/j.ajns.20261502.13
ACS Style
Zhu, M.; Yang, S.; Cheng, R.; Song, Y. Age-specific Nursing Management for Sodium Taurocholate Cotransporting Polypeptide Deficiency: A Narrative Review. Am. J. Nurs. Sci. 2026, 15(2), 35-43. doi: 10.11648/j.ajns.20261502.13
@article{10.11648/j.ajns.20261502.13,
author = {Min Zhu and Shuai Yang and Rui-Lan Cheng and Yuan-Zong Song},
title = {Age-specific Nursing Management for Sodium Taurocholate Cotransporting Polypeptide Deficiency:
A Narrative Review},
journal = {American Journal of Nursing Science},
volume = {15},
number = {2},
pages = {35-43},
doi = {10.11648/j.ajns.20261502.13},
url = {https://doi.org/10.11648/j.ajns.20261502.13},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajns.20261502.13},
abstract = {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.},
year = {2026}
}
TY - JOUR T1 - Age-specific Nursing Management for Sodium Taurocholate Cotransporting Polypeptide Deficiency: A Narrative Review AU - Min Zhu AU - Shuai Yang AU - Rui-Lan Cheng AU - Yuan-Zong Song Y1 - 2026/04/23 PY - 2026 N1 - https://doi.org/10.11648/j.ajns.20261502.13 DO - 10.11648/j.ajns.20261502.13 T2 - American Journal of Nursing Science JF - American Journal of Nursing Science JO - American Journal of Nursing Science SP - 35 EP - 43 PB - Science Publishing Group SN - 2328-5753 UR - https://doi.org/10.11648/j.ajns.20261502.13 AB - 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. VL - 15 IS - 2 ER -