Abstract
INTRODUCTION: Alagille syndrome (ALGS) is a complex and rare autosomal dominant disorder that affects multiple systemic organs, including the liver, heart, kidneys, blood vessels, and eyes. However, as a rare disease, adult ALGS is still underrecognized clinically. The aim of this article is to review the research status and trends of adult ALGS worldwide in the existing literature. METHODS: The authors utilized the Web of Science Core Collection database to identify articles on adult ALGS published from the database inception to December 31, 2023. The authors used bibliometric methods to analyze authors, institutions, countries, journals, and references of 156 included articles. RESULTS: The number of publications in this field has shown a volatile growth trend. The United States published the most articles and University of Pennsylvania is the leading institution in this area. The research on adult ALGS mainly focuses on molecular biology, genetics, health, nursing and clinical medicine. The keywords were "human jagged1," mutations" and "children". The keyword with the strongest citation burst is "children". CONCLUSIONS: Our study systematically summarizes the results of adult ALGS researches, describes and predicts research hotspots and trends on a global scale, which may be helpful for clinicians and researchers to improve their clinical understanding of this disease, and provide a valuable reference for future intensive investigation.