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Kidney Week

Abstract: PUB123

Emerging Evidence for the Evaluation of Atypical Hemolytic Uremic Syndrome: A Bibliometric Analysis

Session Information

Category: Genetic Diseases of the Kidneys

  • 1202 Genetic Diseases of the Kidneys: Non-Cystic (Complex and Non-Cystic Monogenic)

Authors

  • Stuart, Dan, Texas Tech University Health Sciences Center School of Medicine, Amarillo, Texas, United States
  • Kanneganti, Krishna Varsha, Texas Tech University Health Sciences Center School of Medicine, Amarillo, Texas, United States
  • Vasylyeva, Tetyana L., Texas Tech University Health Sciences Center School of Medicine, Amarillo, Texas, United States
  • Smith, Roger Lee, Texas Tech University Health Sciences Center, Amarillo, Texas, United States
Background

Atypical hemolytic uremic syndrome (aHUS) is a condition characterized by symptoms of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. It has been theorized to result from overactivity of the complement system or from autoantibodies directed toward complement cascade regulatory proteins. Given the role of the complement cascade in disease pathogenesis, current treatment protocols include complement C5 inhibitors such as Eculizumab, which has shown to significantly improve patient outcomes. Summarizing the current research on managing patients with this rare condition will aid in handling these unconventional clinical case presentations.

Methods

A comprehensive literature search was executed in Clarivate's Web of Science database using preferred indexing terms related to the clinical management of aHUS. The search strategy for all relevant studies was limited to treatment-and management-oriented articles, with specific criteria for emerging interventions and immunologic factors. A preliminary analysis of the condition's publication metrics was conducted using Clarivate’s Analytics suite and the database’s Citation Report assessment. Represented data visualization was supplemented using Application Programming Interface (API) tools and network group plots generated in R Bibliometrix and VOSViewer.

Results

The results showed that the top countries with publications on this topic were the United States of America, France, and the United Kingdom, with the United States and France accounting for the majority of publications through university affiliations. Most publications were concentrated in North America and Western Europe. There were also a limited number of top authors who published repeated publications. The publication trend for aHUS research has exponentially increased in the last decade or so, showing a marked rise in research output since 2013.

Conclusion

Through this bibliometric analysis, the current information available on the treatment and management of aHUS, including evolving medical interventions, was summarized in a single study. It has also highlighted impediments to research in recent years and variations in publications in resource-limited countries. Overall, the data highlighted the need for further research on this topic to build on knowledge of the diagnosis and treatment plan for the condition.