Objective:To analyze the associated symptoms of progressive symmetrical erythema keratosis (PSEK) and the related literature was reviewed.Methods:Two Mongolian PSEK families in the dermatology department of the People′s Hospital of Inner Mongolia Autonomous Region from 2016 to 2017 were collected, and 40 complete PSEK families and 156 sporadic cases at home and abroad published since 1980 to 2020 were searched by using the database to analyze the concomitant symptoms of PSEK.Results:A total of 40 complete PSEK families were included, including 714 cases. The incidence of PSEK was 57.38% in foreign countries and 37.42% in China; The most common concomitant symptoms abroad were palmoplantar keratosis (PPK), followed by nail changes, neurological symptoms, dysplasia, combined with variable erythema keratosis (EKV), and the concomitant symptoms were more serious; The most common concomitant symptoms in China were nail changes, followed by PPK, damp hyperhidrosis, pruritus, pain and some skin diseases, and the concurrent symptoms were mild.Conclusions:PSEK has many associated symptoms and the molecular genetic mechanism is still unclear. It is necessary to conduct a more comprehensive and in-depth study and understanding of the disease through the development of sequencing technology and the expansion of clinical cases.

Objective:To summarize the best evidence for extubation management of adult patients with mechanical ventilation in the intensive care unit (ICU), which will provide practical guidelines for medical staffs.Methods:BMJ best clinical practice, UpToDate clinical consultants, Guidelines International Network (GIN), National Guideline Clearinghouse (NGC), National Institute for Health and Care Excellence (NICE), Scottish Intercollegiate Guidelines Network (SIGN), Registered Nurses' Association of Ontario (RNAO), Medlive guide, Cochrane Library, JBI evidence-based Health Care Center Database, Web of Science, PubMed, Cumulative Index to Nursing and Allied Health Literature (CINAHL), Ebsco, SinoMed, CNKI, Wanfang Database, etc., were systematically searched. Clinical guidelines, systematic reviews, expert consensus, and randomized controlled trial (RCT) studies were searched from the above database unit August 31st, 2020. Literature quality was evaluated using Appraisal of Guidelines for Research and Evaluation Ⅱ (AGREEⅡ), JBI quality evaluation tools, and Cochrane risk bias assessment. Two researchers evaluated the quality of the included literature respectively, and then the best evidence of endotracheal intubation and extubation management in ICU adult patients with mechanical ventilation was extracted and summarized.Results:A total of 12 articles were collected, including 2 guidelines, 5 systematic reviews, 2 expert consensus, and 3 RCTs. This paper summarizes 17 best evidences on extubation management of adult patients with mechanical ventilation in ICU, including accurate pre-extubation assessment, personnel and equipment, medication, posture, oxygen therapy, airway management, and post-extubation monitoring.Conclusion:Medical staff should choose the best evidence that meets the requirements of the clinical settings and standardize the management of patients after extubation to reduce the incidence of complications and re-intubation rate to ensure the safety of patients, which will be very important for the management of ICU adult patients with mechanical ventilation after extubation.