Research On The Attention Of Rare Diseases In China Based On Bibliometric Analysis And Baidu Index

Objective:To understand the attention of rare diseases in China from different insights based on bibliometrics and Baidu index,so as to provide the basis for future research and work in the field of rare diseases.Methods:1.Taking “rare disease” or “orphan disease” as keywords,the literatures on CNKI database were searched from 2012 to 2021.The number of publications,institutions,authors,keywords,keyword breakout analysis and time graph were analyzed by using bibliometrics method and data visualization software.Excel was used to make statistical analysis of the annual number of published papers,the number of published papers of authors,journals and institutions,and the literature with high citation rate.2.With Baidu Index as the data source and “rare disease” as the keyword,“Search index” data were collected from January 1,2012 to December 31,2021,and summarized by year and month for secondary analysis.The “population portrait” were collected from September 1 to September 30,2022.The top 10 regions in terms of online attention of rare diseases in each year from 2013 to 2021 were collected.SPSS26.0 was used for statistical analysis,and P<0.05 indicated statistically significant difference.3.Through the questionnaire designed and published by “Questionnaire Star”,the personnel participated in the International Rare Diseases Day publicity activities in the First Hospital of Shanxi Medical University were investigated about their knowledge of rare diseases,including their basic information,the focus of rare diseases,rare diseases knowledge response,avenues of concern and the advice of helping the patients,etc.SPSS26.0 was used for statistical analysis,and P<0.05 indicated statistically significant difference.Results:1.A total of 512 literatures were included,with 352 authors from 173 institutions,and published in 225 journals.The number of publications in the field of rare diseases in China showed an increasing trend year by year.The author with the most papers was Zhang Shuyang,with a total of 18.Among the institutions,the Peking Union Medical College Hospital of the Chinese Academy of Medical Sciences ranked the first,followed by China Pharmaceutical University.International Journal of Pharmaceutical Research had the largest number of publications,followed by Chinese Journal of New Drugs.The most frequently cited literature was Liang Tukun’s paper published in 2014.In addition to the keyword “rare disease”,the most frequent keyword was “orphan drug”.There were nine main Keyword clusters,respectively “# 0 orphan drug”,“# 1 drug policy”,“# 2health care”,“# 3 China”,“# 4 rare disease”,“# 5 accessibility”,“# 6 clinical trials”,“# 7 America” and“# 8 treatment”.Between 2016 and 2017,“precision medicine” had the highest intensity of emergence,with an intensity of 2.67.The emergence of “gene” took the longest period form 2018 to 2021.2.From the perspective of time,the search index of rare disease showed an increasing trend from 2012 to 2021,among which the International Rare Disease Day had the highest search index.From the perspective of population distribution,the middle-aged women between 20 and 40 years old were the main concern group,and the proportion of female search volume was higher than that of male,63.76% and 36.24% respectively.From the perspective of region,the province with the highest attention of rare disease was Guangdong,and the top 3 cities were Beijing,Shanghai and Guangzhou.3.A total of 524 valid questionnaires were collected.24.4% of respondents had never paid attention to rare diseases and only 5.2% paid attention to rare diseases frequently.For rare disease related knowledge,the overall correct rate of answering questions was less than 60%,and the proportion of “don’t know” was about 10%-78%.The answer’s accuracy rate of medical workers for rare diseases related knowledge was higher than that of non-medical workers.Except for some questions,the answers of other questions had statistical significance(P<0.05).There was no significant difference in the accuracy of answering the question of “definition,heredity and incidence” of rare diseases among different professional and technical positions medical workers(P>0.05),but the higher the education level,the higher the answer rate,and the difference was statistically significant(P<0.05).74.8% of respondents expected to obtain knowledge of rare diseases through network platform such as Baidu,Zhihu,wechat and Weibo,etc.76.0% of respondents thought that “knowledge publicity and education” could help patients with rare diseases get better treatment,and 66.2% suggested to “set up clinics for rare diseases”.Conclusion:From 2012 to 2021,the attention of rare diseases in China showed an increasing trend year by year,but it still needs to be further increased.In the field of academic literature,the subjects of rare disease research in China were mostly focused on the accessibility analysis of orphan drugs,medical insurance,drug research and development,orphan drug related systems,laws and policy recommendations,etc.There were few studies on basic research and epidemiology,which requires more research efforts.From the aspect of mass netizens,time distribution,gender,age and regional differences affected the attention of rare diseases,among which policy release and news media publicity could increase attention.It is suggested that local governments should actively respond to national policies to establish a complete local system.The media should increase the coverage of rare diseases,and carry out publicity and education on rare diseases to the public.From the aspect of the current investigation,the respondents’ general concern and recognition of rare diseases were low.In order to increase attention,online push and video teaching of doctors can be added.Knowledge education should be strengthened for patients.There are some ways for hospitals to help patients with rare diseases by setting up clinics for rare diseases,free diagnosis and other work.