Age-specific Nursing Management for Sodium Taurocholate Cotransporting Polypeptide Deficiency: 
A Narrative Review

Min Zhu; Shuai Yang; Rui-Lan Cheng; Yuan-Zong Song

doi:doi:10.11648/j.ajns.20261502.13

Review Article |

| Peer-Reviewed

Age-specific Nursing Management for Sodium Taurocholate Cotransporting Polypeptide Deficiency: A Narrative Review

Min Zhu

, Shuai Yang

, Rui-Lan Cheng

, Yuan-Zong Song^*

Published in American Journal of Nursing Science (Volume 15, Issue 2)

Received: 19 March 2026 Accepted: 3 April 2026 Published: 23 April 2026

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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.

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

Keywords

NTCP Deficiency, Age-specific Nursing Care, Bile Acid Metabolism, Symptom Management

1. Introduction

Sodium taurocholate cotransporting polypeptide (NTCP) is a critical transport protein located on the hepatocyte membrane, encoded by the SLC10A1 gene. Its primary function is to mediate the uptake of bile acids into hepatocytes, playing a key role in maintaining enterohepatic bile acid circulation and bile acid homeostasis

[1]

. Since 2015, with the widespread adoption of molecular genetic testing, an increasing number of studies have reported NTCP functional deficiency caused by SLC10A1 gene mutations. The first internationally reported case was described by the Dutch scholar Vaz et al.

[2]

, while the first domestic Chinese case (the second worldwide) was reported by our research group in 2016

[3, 4]

The clinical phenotype is characterized by persistent hypercholanemia

[1]

and is referred to as NTCP deficiency disease (NTCPD). Based on previously reported cases and current outpatient follow-up observations, patients across different age groups exhibit distinct clinical phenotypes. During the neonatal and infant periods, when hepatic metabolic function is not yet fully mature, manifestations include neonatal jaundice, indirect hyperbilirubinemia, and persistently elevated bile acids

[5, 6]

. Some preschool and school-age children present with elevated bile acids accompanied by vitamin D deficienc

[7]

. In adults, the condition predominantly manifests as asymptomatic persistent hypercholanemia without typical cholestatic symptoms (most patients have no pruritus or steatorrhea), with a minority of cases reporting vitamin D deficiency or gallbladder abnormalities (polyps or gallstones). Notably, some genetically confirmed NTCPD patients exhibit an intrahepatic cholestasis of pregnancy (ICP) phenotype: classic ICP features with pruritus and elevated bile acids appearing in the third trimester and resolving after delivery. Furthermore, clinical outpatient observations reveal a widespread pattern across all age groups of repeated consultations without a definitive diagnosis and excessive medical interventions. This not only negatively affects the quality of life of affected children but also exacerbates the psychological burden on families

[8]

As frontline healthcare providers who often serve as the initial point of contact, nurses must accurately identify age-specific symptoms and systematically conduct age-stratified symptom monitoring, nutritional support, medication management, and tiered health education—all of which are central to improving nursing quality. Meanwhile, as a rare hereditary cholestatic disease, NTCPD currently lacks systematic nursing experience and evidence-based nursing guidelines tailored to different age groups, and no standardized nursing pathway has been established. How to scientifically assess nursing problems based on patients' age characteristics, develop individualized age-specific nursing intervention plans, and achieve full life-cycle health management through phased follow-up represents a key focus for current clinical nursing practice. This article systematically explores nursing assessment tools, optimization strategies for age-specific nursing care, and phased pathway-based management models for patients with NTCPD across different age groups, with the aim of providing a theoretical basis and reference for establishing age-stratified standardized nursing routines for this disease, thereby improving patient prognosis and outcomes.

2. Methods

This narrative review was conducted in accordance with the Scale for the Assessment of Narrative Review Articles (SANRA) guidelines to ensure methodological transparency and reproducibility. A systematic literature search was performed across four electronic databases: PubMed, CINAHL (Cumulative Index to Nursing and Allied Health Literature), Embase, and the China National Knowledge Infrastructure (CNKI). Searches were conducted in 2025 with no restriction on publication start date, given the recent emergence of NTCPD as a recognized clinical entity. The following search terms were applied in varying combinations using Boolean operators (AND, OR): "sodium taurocholate cotransporting polypeptide," "NTCP deficiency," "SLC10A1," "bile acid transport disorder," "hypercholanemia," "nursing care," "pediatric nursing," "age-specific care," "family management," "cholestatic liver disease," "rare disease nursing," "nutritional assessment," and "follow-up management." MeSH terms were used in PubMed searches where applicable.

Inclusion criteria were: (1) studies or reports involving patients with confirmed or clinically suspected NTCPD; (2) literature addressing nursing care, clinical management, symptom assessment, or family support in NTCPD or closely related cholestatic conditions; (3) studies involving pediatric, adolescent, adult, or pregnant populations; (4) publications in English or Chinese; and (5) all study designs, including case reports, case series, observational studies, systematic reviews, and clinical guidelines, given the limited evidence base for this rare condition.

Exclusion criteria were: (1) studies exclusively focused on molecular genetics or pharmacological mechanisms without clinical or nursing relevance; (2) conference abstracts without full-text availability; and (3) duplicate publications.

The initial search yielded 312 records. After removal of duplicates (n = 47), title and abstract screening excluded a further 198 records. Full-text review of the remaining 67 articles resulted in the inclusion of 38 references directly informing this review. Additional relevant sources were identified through manual reference list searching. Given the scarcity of NTCPD-specific nursing literature, evidence from analogous cholestatic and chronic pediatric liver diseases was incorporated where disease-specific data were unavailable, with this extrapolation noted explicitly throughout the text. The overall evidence base for NTCPD nursing is predominantly Level IV–V (case reports and small case series) according to the Oxford Centre for Evidence-Based Medicine (OCEBM) hierarchy, reflecting the rarity and novelty of the condition. Quality appraisal of included studies was conducted using a pragmatic approach appropriate for a narrative review of an ultra-rare condition. Because the available evidence consisted predominantly of case reports and small case series, formal standardized tools (e.g., the Newcastle–Ottawa Scale or GRADE framework) were not applied uniformly. Instead, each included study was evaluated qualitatively against the following criteria: (1) clarity and rigor of diagnostic confirmation (genetically or clinically confirmed NTCPD); (2) completeness and transparency of clinical data reporting; (3) appropriateness of the nursing or clinical conclusions relative to the evidence presented; and (4) potential for bias arising from single-center or single-case designs. No study was excluded solely on the basis of quality assessment; however, conclusions drawn from lower-quality sources are explicitly qualified throughout the text, and the overall evidence level (predominantly OCEBM Level IV–V) is acknowledged as a key limitation of the current evidence base.

3. Current Status of Nursing Research on NTCP Deficiency

Current domestic and international research on NTCPD has primarily focused on genetic diagnosis and pathogenesis. No disease-specific pharmacological treatment currently exists; clinical management is mainly symptomatic and supportive, with emphasis on avoiding excessive medical intervention and implementing standardized follow-up protocols. The overall management strategy includes the following components: 1) Dynamic monitoring centered on serum total bile acids (TBA) and liver function, supplemented by assessment and supplementation of fat-soluble vitamins, to reduce the risk of vitamin deficiency and growth retardation. 2) Evidence-based symptomatic management of pruritus associated with cholestasis. 3) Emphasis on family education and nutritional management, age-stratified follow-up, and multidisciplinary collaboration to avoid overtreatment resulting from insufficient understanding of the natural history of the disease.

Although the number of clinical case reports of NTCPD has increased significantly compared to previous years

[10, 11]

, systematic nursing research remains limited. Existing nursing-related evidence consists primarily of individual case reports

[9, 12]

or small-sample observational studies, presenting an experiential and fragmented character without an established disease-specific assessment framework

[8]

. Overall, the importance of nursing care in NTCPD is gradually being recognized, but progress in the development of assessment tools, standardization of age-stratified follow-up, and multi-center collaboration remains at an early stage.

4. Nursing Assessment Tools

In the nursing management of NTCPD, assessment is a prerequisite for formulating intervention measures and follow-up plans. No disease-specific assessment tools currently exist; existing practice draws on nursing assessment approaches developed for cholestatic diseases and chronic liver diseases

[8, 13-15, 17, 18]

. Assessment content is generally divided into two categories—objective indicators and subjective scales—and combining both provides a more comprehensive reflection of patient status and nursing needs.

4.1. Objective Assessment Tools

4.1.1. Laboratory Indicators

Laboratory tests represent the most direct and objective component of nursing assessment in NTCPD, providing a basis for symptom evaluation, nutritional guidance, and follow-up management

[17]

. Serum total bile acid level is the core indicator for diagnosis and follow-up; persistent elevation is a hallmark feature of the disease

[1]

, and dynamic changes can indicate disease activity and the effectiveness of interventions. Bilirubin levels—particularly total and direct bilirubin—help determine the presence of cholestatic complications and the degree of jaundice

[1, 4, 5, 19]

. Alanine aminotransferase (ALT) and aspartate aminotransferase (AST) are primarily used to assess hepatocellular injury; most patients present with normal or mildly elevated values, indicating that enzymatic abnormalities are not invariably present and may not correlate directly with clinical manifestations. Alkaline phosphatase (ALP) and gamma-glutamyl transferase (γ-GT) reflect biliary involvement and intrahepatic cholestasis, and their trends carry indicative value for nursing observation

[5]

Assessment of fat-soluble vitamin levels (A, D, E, and K) constitutes an important component of nutritional assessment

[20]

. Vitamin D status is closely linked to bone metabolism; some patients may present with low 25-hydroxyvitamin D levels

[4]

, requiring dose adjustment of supplementation based on repeat measurements. Vitamin K deficiency may manifest as coagulation abnormalities

[21]

, increasing bleeding risk, which should be monitored through prothrombin time or the International Normalized Ratio (INR). Complete blood count and serum lipid levels can serve as supplementary references for growth, development, and metabolic status during outpatient visits and follow-up. Overall, dynamic monitoring of laboratory indicators not only helps nurses track disease progression but also provides objective evidence for feeding guidance, nutritional supplementation, and medication adherence monitoring. By establishing appropriate re-examination intervals and indicator thresholds

[22]

, the nursing team can detect abnormalities early and adjust intervention plans in a timely manner, thereby improving nursing quality and prognosis.

4.1.2. Physical Examination Indicators

Physical measurements represent data that nursing staff can most readily obtain during routine follow-up and ward observations, and constitute important evidence for assessing growth, development, and nutritional status in NTCPD patients from the neonatal period through adolescence. Routine measurements include body weight, body length or height, head circumference, and mid-upper arm circumference. Combined with World Health Organization or Chinese pediatric reference curves, age-adjusted Z-scores can be calculated to dynamically evaluate nutritional status and growth trends. In infants, weight gain velocity provides a direct reflection of caloric intake, while serial recording of length and head circumference helps identify potential growth retardation or neurodevelopmental risk

[23]

. In preschool and school-age children, follow-up should focus on changes in height and body mass index (BMI) to evaluate long-term nutritional intake and body composition.

For patients presenting with jaundice, changes in skin color can indicate progression or resolution of jaundice, and observation of stool color provides direct information regarding bile excretion status, which is particularly sensitive during infancy

[24]

. Continuous recording of physical examination data not only helps identify the short-term effectiveness of nursing interventions but also provides objective evidence for long-term follow-up, making it an indispensable component of the comprehensive nursing assessment system.

4.2. Subjective Assessment Tools

4.2.1. Pediatric Malnutrition Assessment Screening Tools

Chen Qiaoling

[25]

systematically reviewed 11 pediatric nutritional risk screening tools that have been widely studied or newly developed domestically and internationally in recent years, with the aim of identifying appropriate instruments for early nutritional assessment. Among these, the Simple Pediatric Nutrition Screening Tool (PNST), proposed by White et al.

[26]

in 2016, consists of four yes/no questions: Has the child recently lost weight? Has the child shown little weight gain over the past few months? Has the child's food intake decreased over the past few weeks? Is the child noticeably underweight or overweight? Although specific validation data for this tool in NTCPD patients are currently lacking, its content aligns with the focus areas of outpatient follow-up for NTCP patients and can serve as a reference for preliminary nutritional assessment. In clinical application, results should be interpreted in conjunction with the digestive and absorptive abnormalities potentially caused by hypercholanemia.

4.2.2. Pediatric Quality of Life Inventory

The Pediatric Quality of Life Inventory™ 4.0 Generic Core Scales (PedsQL™ 4.0), developed by Varni et al.

[16, 27]

, is an internationally recognized tool for assessing quality of life in children and adolescents. It covers four dimensions: physical functioning, emotional functioning, social functioning, and role functioning. Four age-specific versions are available—for children aged 2–4, 5–7, 8–12, and 13–18 years—each with corresponding parent-proxy and child self-report formats. The scale employs a 5-point Likert scoring method; after reverse transformation, dimension and total scale scores range from 0 to 100, with higher scores indicating better quality of life. In studies of chronic liver diseases such as pediatric autoimmune hepatitis

[28]

, the scale has demonstrated good reliability and validity across both healthy and patient populations

[16]

, with a total scale Cronbach's α of 0.86–0.92 and dimension-level Cronbach's α values of 0.71–0.89. Research using this scale has found that symptoms such as abdominal pain and fatigue can significantly reduce quality of life in affected children

[29]

. For children with NTCPD, bile acid–related symptom items (such as the impact of pruritus) may be added to the scale to enhance disease specificity.

4.2.3. Pruritus Scale

The 5-D Itch Scale, developed by Elman S et al

[30]

s a disease-specific tool for assessing pruritus symptoms. It comprehensively quantifies pruritus status across five dimensions: Duration, Degree, Daily activity interference, Disruption of sleep, and Distribution. Each dimension is scored on a 1–4 scale, with a total score ranging from 5 to 20; higher scores indicate more severe pruritus with greater impact. The scale is widely used in the assessment of pruritus caused by conditions such as intrahepatic cholestasis. In a recent study

[31]

, Cynthia et al. used this scale in a US-based survey to evaluate the impact of pruritus on health-related quality of life and treatment experiences in patients with primary biliary cholangitis. Since some NTCPD patients may develop skin pruritus due to bile acid metabolic abnormalities or ICP, this scale can serve as a sensitive symptom monitoring tool. In clinical use, it is important to account for age-specific assessment approaches; for infants and toddlers, indirect scoring based on parental observation of sleep disturbance and scratching behavior is recommended. In addition, the Chinese version of the 12-item Pruritus Scale has demonstrated good reliability and validity and may serve as a supplementary assessment tool in Chinese-speaking clinical settings

[15]

4.2.4. Caregiver Burden Inventory

The Caregiver Burden Inventory (CBI), developed by Zarit et al.

[32]

, is used to assess the subjective burden of caregivers of patients with chronic diseases. It comprises two subscales—personal burden and responsibility burden—with a total of 22 items. A 5-point Likert scale is used, with responses ranging from "never" to "always" scored 0–4; total scores range from 0 to 88, with scores ≥ 46 indicating severe caregiver burden. In studies of hereditary chronic diseases such as primary immunodeficiency, this scale has been used to evaluate parental caregiving burden. As a rare hereditary disease, NTCPD frequently confronts parents with diagnostic uncertainty, anxiety related to excessive intervention, and long-term monitoring pressure; this scale can effectively identify psychological stress in caregivers. The Chinese version of the scale was first adapted by Wang Lie et al.

[33]

, with a Cronbach's α of 0.87, indicating good reliability and validity. Results from such instruments can provide a reference for the quality of home care and the feasibility of follow-up plans.

Although no subjective assessment tools specifically designed for NTCPD currently exist, future research could develop more targeted instruments that accurately reflect symptom burden while also addressing family education and management capacity, thereby enhancing the scientific rigor and individualization of nursing interventions.

5. Age-specific Nursing Care at Different Stages of NTCP Deficiency

5.1. Neonatal Period

During this period, cholestatic jaundice is the most common initial presentation, which subsequently transitions to isolated hypercholanemia with near-normal liver enzyme levels. According to the 2022 American Academy of Pediatrics (AAP) Clinical Practice Guideline for the management of neonatal hyperbilirubinemia

[34]

, care involves risk assessment, transcutaneous or serum bilirubin monitoring, and initiation of phototherapy upon reaching the established threshold. During phototherapy, particular attention should be paid to eye protection, positional rotation, fluid supplementation, breastfeeding support, and monitoring for treatment efficacy and rebound hyperbilirubinemia. Intensive breastfeeding or appropriate supplementation should be encouraged to avoid dehydration and to promote intestinal excretion of bilirubin and bile acids. The AAP guideline also recommends using the difference between the measured bilirubin concentration and the phototherapy threshold to determine the time interval between discharge and follow-up. Since some cholestatic infants develop fat-soluble vitamin deficiency, nutritional support plays an important role in medical management; early monitoring and supplementation of fat-soluble vitamins (A, D, E, and K) should therefore be initiated in infants with concomitant cholestasis.

At this stage, Chen Meixue's research found that most parents have insufficient knowledge of rare diseases and are prone to excessive anxiety or poor adherence

[12]

. Nurses should explain to parents that persistently elevated bile acids are a characteristic feature of NTCPD. Depending on the clinical situation, regular follow-up every 4–6 weeks (including bile acids, ALT/AST, and GGT) along with feeding and weight records is recommended. Verbal education and written materials should be provided to help parents understand the characteristics and natural course of the disease and clarify follow-up schedules and monitoring indicators. Through ongoing guidance and psychological support, parents can be helped to cultivate a positive caregiving mindset and improve family care capacity, thereby laying the groundwork for subsequent nursing and follow-up.

5.2. Toddler Period

As patients enter the toddler period, the nursing focus shifts toward symptomatic management, symptom control, and enhancement of family management capacity. A minority of patients develop pruritus, which commonly leads to sleep disturbance, emotional instability, and decreased attention. Based on best-evidence summaries for the management of pruritus in cholestatic patients, nurses should guide families in daily skin care

[14]

, instruct them to avoid contact with irritating fabrics and soaps, pay attention to home temperature and humidity regulation, and promptly observe and manage skin lesions caused by scratching to prevent secondary infections. Pruritus scoring tools and sleep logs may also be used to quantify symptom changes

[13]

and provide a basis for subsequent interventions.

The toddler period represents a critical window for establishing family disease management patterns. Nurses should systematically evaluate caregivers' level of disease knowledge and adherence to recommended practices, and clarify their responsibilities in medication management, follow-up compliance, and medical record keeping. The use of follow-up manuals or electronic health management tools for symptom recording and information feedback is recommended. Nursing interventions should incorporate caregiver psychological assessment and provide emotional support and communication guidance to strengthen caregiving confidence and self-efficacy. Through systematic symptom management, nutritional support, and family health education, the symptom burden can be reduced, normal growth and development promoted, and family self-care capacity enhanced.

5.3. Preschool and School-age Periods

As patients progress into preschool and school-age stages, nursing care should consolidate the achievements of prior interventions, reinforce lifestyle management, and cultivate self-care awareness—shifting gradually from symptom control toward healthy behavior formation and social integration. During this period, some children's clinical manifestations tend to stabilize, but insufficient nutritional intake and fluctuating vitamin levels may persist. Research has shown

[35]

that dietary behaviors in school-age children can substantially influence their nutritional status.

In the preschool period, nurses should guide families in establishing regular dietary and daily routine schedules to help children progressively develop healthy eating habits while avoiding food selectivity or excessive reliance on a single food group. In terms of family education, a systematic review by Thomas et al.

[36]

indicates that nurses should address discrepancies in caregiving philosophies between parents and other caregivers; advocating for family-centered shared decision-making can help maintain consistency in caregiving approaches and reduce implementation deviations.

Upon entering the school-age period, the nursing focus gradually shifts toward self-management and school-based collaboration. School-age children with rare diseases may experience anxiety and peer relationship difficulties stemming from disease-related constraints; continuous attention to psychological well-being and social adaptation is therefore essential. Such attention both safeguards the physical and mental health and academic development of affected children and lays the foundation for long-term nursing care and social integration

[37, 38]

6. Follow-up and Pathway-based Management

Given the prolonged disease course and variable symptom severity in NTCPD, follow-up constitutes a central component of nursing care. A scientifically structured follow-up framework can monitor disease changes, detect abnormalities promptly, evaluate the effectiveness of nursing interventions, and help families establish standardized care models. Follow-up content should include post-examination laboratory results, symptom observation, dietary and feeding logs, medication adherence and adverse reactions, and assessment of caregivers' disease knowledge and care capacity. For patients with pruritus, pruritus scores, nocturnal awakening records, and skin lesion status should be documented.

Pathway-based nursing management can enhance the standardization and efficiency of follow-up. Pathway design should be centered on age-stage classification and disease stability, establishing standardized re-examination items and health education content, with dynamic adjustments based on family implementation. Outpatient follow-up, home care, and remote management can form a complementary relationship; the use of electronic health records and mobile applications facilitates caregivers' recording of symptoms and medication information and enables timely feedback to nursing staff. Through pathway-based follow-up management, nursing care priorities across all age groups can be standardized, assessment tools and quality indicators unified—including pruritus improvement rates, vitamin attainment rates, and growth and development metrics—and data accumulated to inform the future development of NTCPD nursing guidelines and standardized pathways.

7. Current Challenges

Nursing research on NTCPD remains at an early stage. Available nursing evidence consists primarily of individual case reports and small-sample studies, lacking prospective, multi-center, and controlled research designs, and high-level evidence-based findings and generalizable nursing pathways have not yet been established. Clinical practice commonly borrows generic scales designed for cholestasis or chronic liver disease, whose reliability, validity, and clinical sensitivity in NTCPD patients have not been validated, making cross-center comparison of assessment results difficult. Follow-up models in outpatient and post-hospitalization settings are inconsistent; standardized pathways need to be established, as re-examination schedules currently rely heavily on individual clinical experience. Furthermore, considerable variation exists in the home care capacity of patient families; caregivers differ in their level of disease knowledge, cultural background, and psychological status, and some caregivers display excessive anxiety or insufficient adherence. These factors affect medication compliance, nutritional management, and follow-up rates, ultimately undermining the overall effectiveness of nursing care.

8. Discussion

The following discussion synthesizes key themes arising from this review: age-stratified heterogeneity, diagnostic delay, assessment tool adaptation, family health literacy, multidisciplinary coordination, and digital care.

Age-stratified heterogeneity as a central nursing challenge. NTCPD manifests distinctly at each developmental stage—neonatal cholestatic jaundice and hyperbilirubinemia, toddler pruritus with sleep and behavioral disturbances, school-age vitamin D insufficiency and suboptimal growth, and adult asymptomatic hypercholanemia or ICP during pregnancy. This phenotypic variability requires nursing staff to recognize presentations that may mimic far more common conditions. The absence of disease-specific guidelines forces clinicians to rely on frameworks designed for other cholestatic disorders—a pragmatic but imperfect approach, given that NTCPD's natural history and prognostic implications differ substantially.

The diagnostic delay problem and its nursing implications. Repeated consultations without a confirmed diagnosis, culminating in excessive intervention, are well documented across rare diseases and carry particular consequences in NTCPD, where most patients follow a relatively benign course. Nurses are uniquely positioned to interrupt this cycle by flagging diagnostic inconsistencies, advocating for genetic testing, and reframing persistently elevated bile acids as a disease hallmark rather than a sign of deterioration—thereby reducing family anxiety and unnecessary healthcare utilization. Nurse-led education programs incorporating digital platforms and peer-support networks represent a scalable intervention warranting formal evaluation.

Assessment tool adaptation and validation. The PedsQL™ 4.0, 5-D Itch Scale, PNST, and CBI provide useful starting points, but none has been validated for NTCPD. The PedsQL™ 4.0 lacks bile acid–specific symptom dimensions; the 5-D Itch Scale requires proxy-report adaptation for pre-verbal children; and the CBI likely underestimates rare-disease-specific caregiver burdens such as uncertainty management and information-seeking fatigue. Future instrument development should employ mixed-methods approaches to ensure new tools reflect the lived experiences of patients and families across all developmental stages.

Family-centered care and the role of health literacy. The effectiveness of age-specific nursing interventions depends on families' capacity to sustain them at home. Higher caregiver health literacy supports better symptom recognition, surveillance adherence, and multidisciplinary engagement; conversely, psychosocial stressors can undermine vigilance regardless of intent. Nursing care must therefore include structured health literacy assessment and tailored education delivery—plain-language summaries, visual follow-up calendars, and smartphone-based symptom logs being low-cost, high-impact options. Social work and psychological support should be integrated into routine care pathways rather than reserved for crises.

Multidisciplinary collaboration and care coordination. NTCPD management requires coordinated input from pediatric hepatologists, clinical geneticists, dietitians, pharmacists, developmental pediatricians, and school health professionals. The nurse case manager or clinical nurse specialist is best positioned to integrate this expertise, translating specialist recommendations into actionable care plans and ensuring continuity across settings. Formalizing this coordinating role within NTCPD pathways—with adequate training and institutional support—would meaningfully improve on the current fragmented model.

Implications for informatics and remote care. The concentration of rare disease expertise in tertiary centers creates access barriers for rural and resource-limited families. Telehealth and mobile health applications can partially address this gap through remote symptom monitoring, virtual education, and asynchronous team communication. Although the evidence base for digital interventions in pediatric rare diseases remains nascent, findings from adjacent fields indicate improvements in follow-up rates, caregiver self-efficacy, and patient-reported outcomes. Development and rigorous evaluation of NTCPD-specific digital care tools should be accorded research priority.

9. Conclusion

NTCPD is a rare hereditary condition whose clinical presentation shifts meaningfully across the lifespan, and effective nursing care must shift with it. The age-specific framework proposed in this review offers a practical structure for organizing that care—from the neonatal period through pregnancy—with consistent priorities running through every stage: monitoring bile acids and fat-soluble vitamins, supporting nutrition, educating families at the level they can use, screening for psychological distress, and advocating against unnecessary intervention. What is still missing is the evidence base to do all of this with confidence. No nursing assessment tools have been validated for this population, no multi-center studies have been done, and follow-up protocols remain untested. Closing these gaps will take real investment in instrument development, collaborative research, and smarter use of digital tools—but until that work happens, nurses caring for patients with NTCPD are doing so largely by extrapolation, and both the profession and its patients deserve better than that.

Abbreviations

AAP	American Academy of Pediatrics
ALP	Alkaline Phosphatase
ALT	Alanine Aminotransferase
AST	Aspartate Aminotransferase
BMI	Body Mass Index
CBI	Caregiver Burden Inventory
CINAHL	Cumulative Index to Nursing and Allied Health Literature
CNKI	China National Knowledge Infrastructure
γ-GT	Gamma-Glutamyl Transferase
ICP	Intrahepatic Cholestasis of Pregnancy
INR	International Normalized Ratio
MeSH	Medical Subject Headings
NTCP	Sodium Taurocholate Cotransporting Polypeptide
NTCPD	Sodium Taurocholate Cotransporting Polypeptide Deficiency
OCEBM	Oxford Centre for Evidence-based Medicine
PedsQL^TM	Pediatric Quality of Life Inventory
PNST	Pediatric Nutrition Screening Tool
SANRA	Scale for the Assessment of Narrative Review Articles
TBA	Total Bile Acids
WHO	World Health Organization

Author Contributions

Min Zhu: Writing – original draft

Shuai Yang: Supervision

Rui-Lan Cheng: Data curation

Yuan-Zong Song: Conceptualization, Supervision

Conflicts of Interest

The authors declare no conflicts of interest.

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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

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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

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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

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@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}
}

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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  -

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Author Information

Min Zhu

School of Nursing, Jinan University, Guangzhou, China

http://orcid.org/0009-0002-5749-9175
Shuai Yang

School of Nursing, Jinan University, Guangzhou, China

http://orcid.org/0000-0002-2616-6979
Rui-Lan Cheng

Department of Pediatrics, The First Affiliated Hospital of Jinan University, Guangzhou, China

http://orcid.org/0009-0008-7626-9053
Yuan-Zong Song

Department of Pediatrics, The First Affiliated Hospital of Jinan University, Guangzhou, China

Contact Email

http://orcid.org/0000-0003-0101-2743

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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

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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

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AMA 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

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@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}
}

Copy | Download

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  -

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