Trends in Congenital Insensitivity to Pain with Anhidrosis: A Bibliometric Analysis from 2000 to 2021

Abstract

Background Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare inherited autosomal recessive disease that has multiple clinical manifestations. Since its symptoms are related to different systems, this disorder has been investigated on a variety of topics. To better understand publications about CIPA, we conducted a bibliometric study to evaluate research publications on CIPA from 2000 to 2021, and delineate the key contributions in terms of countries, authors and sources. Methods Quantitative analysis of publications on CIPA from 2000 to 2021 was interpreted and graphed through the Science Citation Index Expanded (SCIE) of Web of Science (WOS) Core Collection. The bibliometric package in R 4.1.1, VOSviewer 1.6.18, and GraphPad Prism 8.4 were used to conduct the bibliometric analysis. Results From 2000 to 2021, a total of 163 publications were retrieved. China had the largest number of publications (n = 31), while Japan had the highest number of citations (621 citations). Levy J and Indo Y were perhaps the most impactful researchers in the field of CIPA. The co-authorship of authors and institutions indicated little cooperation on CIPA research between different countries. Annals of Neurology (n=5) and Nature Genetics (120 citations) were the most productive and cited journals, respectively, and the top 10 local cited references clarified the theoretical basis of the CIPA research area. Furthermore, the important topics on CIPA mainly include NTRK1 mutations and nerve growth factor (NGF). Conclusion Based on the bibliometric analysis, we have a comprehensive view of the global status of CIPA research, and the results indicate that CIPA needs more attention and cooperation to facilitate the study of its pathological mechanisms.

A Systematic Review of Congenital Insensitivity to Pain, a Rare Disease

“…Ahmed et al posit that recurrent febrile episodes and self-mutilation incidents may serve as conspicuous red flags for early detection [ 3 ]. Furthermore, the imperative of further research into this enigmatic malady, as underscored by Qiu et al, is advocated to deepen our comprehension of its pathological underpinnings [ 21 ]. Additionally, Svec et al, Khurshid et al, and Yu et al accentuate the indispensable nature of preventive measures to avert potential complications [ 2 , 6 , 13 ].…”

“…Activating mutations in SCN11A cause persistent depolarization of the neuronal membrane, preventing the effective generation and transmission of pain signals. Together, mutations in SCN9A and SCN11A disrupt the proper transmission of pain signals, albeit by different mechanisms, leading to the pain insensitivity characteristic of CIPA [ 21 ].…”

“…It is pertinent to highlight that while various authors advocate for different approaches to address bone and anesthetic issues, as detailed in the Section 3 , there is a consensus in the literature regarding the diagnosis and treatment of this condition [ 15 , 17 , 21 ].…”

“…Zhao et al [21] Trends in congenital insensitivity to pain with anhidrosis: a bibliometric analysis from 2000 to 2021 7…”