To explore the role of the "Clinical Practice Guidelines" column and others in the Medical Joumnal of Peking Union Medical College Hospital (Med J PUMCH) in promoting diseiplinary development.

Fabry disease(FD)is an X-linked genetic disorder caused by mutations in the GLA gene.It leads to reduced or complete deficiency of the activity of α-galactosidase A(α-Gal A),resulting in an accumu-lation of the metabolic substrate globotriaosylceramide(Gb3)and its derivative,globotriaosylsphingosine(Lyso-Gb3),in a wide range of cells and tissues,which causes multiple organ pathologies.In the cardiovascu-lar system,FD predominantly leads to left ventricular hypertrophy and/or conduction abnormalities known as FD cardiomyopathy.Since FD cardiomyopathy is the leading cause of death in adult patients with FD,early diagnosis combining cardiac imaging,enzyme and substrate activity,genetic testing,and tissue biopsy,as well as early specific enzyme replacement therapy are important to improve patient prognosis.This consensus comprehen-sively summarizes the published evidence related to the diagnosis and treatment of FD cardiomyopa-thy at home and abroad,and provides a basis for the diagnosis and management of FD cardiomyopathy.

Autoimmune pancreatitis(AIP)is an immune-mediated,special type of chronic pancreatitis,which can involve multiple organs.The clinical manifestation of AIP is complex and varied,making the diagnosis and treatment challenging.With reference to the latest guidelines and studies from both domestic and international sources,this guideline comprises 20 recommendations regarding the diagnosis,treatment,follow-up,and prognosis of AIP.The aim of this guideline is to promote the care capability and improve the outcome of patients with AIP in China.

Providing adequate and balanced nutrition for preterm infants,especially extremely/very preterm infants,is the material basis for promoting their normal growth and development and improving long-term prognosis.Enteral nutrition is the best way to feed preterm infants.Previous systematic reviews have shown that using evidence-based standardized feeding management strategies can effectively promote the establishment of full enteral feeding,reduce the duration of parenteral nutrition,improve the nutritional outcomes of preterm infants,and not increase the risk of necrotizing enterocolitis or death.Based on relevant research in China and overseas,the consensus working group has developed 20 recommendations in 5 aspects including the goal of enteral nutrition,transitioning to enteral nutrition,stable growth period enteral nutrition,supplementation of special nutrients,and monitoring of enteral nutrition for preterm infants,using the Grading of Recommendations Assessment,Development and Evaluation.The aim is to provide recommendations for healthcare professionals involved in the management of enteral nutrition for preterm infants,in order to improve the clinical outcomes of preterm infants.

Establishing enteral nutrition after the birth of preterm infants presents numerous challenges,particularly for those in special situations.Various disease factors and medical interventions impede the establishment of enteral feeding,leading to conflicts and controversies regarding feeding goals,feeding methods,and the challenges and solutions faced by these infants.A critical issue for clinical physicians is how to safely and promptly establish enteral nutrition to achieve full enteral feeding as quickly as possible.The consensus formulation working group,based on both domestic and overseas research,adopted the Grading of Recommendations Assessment,Development and Evaluation,and formed an expert consensus on enteral nutrition management for preterm infants in special situations.This consensus provides 14 recommendations for 9 common special situations,aiming to offer guidance on enteral nutrition management for preterm infants to improve their short and long-term outcomes.

BACKGROUND: This study aimed to develop a comprehensive instrument for evaluating and ranking clinical practice guidelines, named Scientific, Transparent and Applicable Rankings tool (STAR), and test its reliability, validity, and usability. METHODS: This study set up a multidisciplinary working group including guideline methodologists, statisticians, journal editors, clinicians, and other experts. Scoping review, Delphi methods, and hierarchical analysis were used to develop the STAR tool. We evaluated the instrument's intrinsic and interrater reliability, content and criterion validity, and usability. RESULTS: STAR contained 39 items grouped into 11 domains. The mean intrinsic reliability of the domains, indicated by Cronbach's α coefficient, was 0.588 (95% confidence interval [CI]: 0.414, 0.762). Interrater reliability as assessed with Cohen's kappa coefficient was 0.774 (95% CI: 0.740, 0.807) for methodological evaluators and 0.618 (95% CI: 0.587, 0.648) for clinical evaluators. The overall content validity index was 0.905. Pearson's r correlation for criterion validity was 0.885 (95% CI: 0.804, 0.932). The mean usability score of the items was 4.6 and the median time spent to evaluate each guideline was 20 min. CONCLUSION The instrument performed well in terms of reliability, validity, and efficiency, and can be used for comprehensively evaluating and ranking guidelines.
Reproducibility of Results ; Surveys and Questionnaires ; Practice Guidelines as Topic ; Humans

Humans ; Communicable Diseases ; Research

Humans ; Communicable Diseases ; Research

Transparency Ecosystem for Research and Journals in Medicine (TERM) working group summarized the essential recommendations that should be considered to review and publish a high-quality guideline. These recommendations from editors and reviewers included 10 components of essential requirements: systematic review of existing relevant guidelines, guideline registration, guideline protocol, stakeholders, conflicts of interest, clinical questions, systematic reviews, recommendation consensus, guideline reporting and external review. TERM working group abbreviates them as PAGE (essential requirements for Publishing clinical prActice GuidelinEs), and recommends guideline authors, editors, and peer reviewers to use them for high-quality guidelines.
Humans ; Practice Guidelines as Topic

Objective: To analyze the citation of articles in Chinese Journal of Otorhinolaryngology Head and Neck Surgery, and to explore the strategy for running scientific journals. Methods: Using the Citation Database of Chinese Biomedical Journals, the citations of articles in this journal from 2009 to 2019 were statistically analyzed, and the characteristics of highly cited articles were analyzed. Results: From 2009 to 2019, Chinese Journal of Otorhinolaryngology Head and Neck Surgery published 2 814 papers. Cited papers accounted for 75.69% of all papers, and each paper was cited 7.24 times. The citation rate of published papers was consistent with the variation trend of citation frequency. Non treatises such as guide consensus and original articles for special issues were cited better. There was no obvious correlation between paper funding and citation. Conclusion: Improving the content quality and optimizing the column setting are important measures to enhance the influence of scientific journals.
China ; Humans ; Otolaryngology