BACKGROUND:Sarcopenia is an age-related degenerative syndrome,and the relationship between mitochondrial autophagy and exercise in preventing and treating sarcopenia has been demonstrated.However,there is a lack of comprehensive reviews detailing the specific receptor proteins and signaling pathways involved in the role of exercise in sarcopenia prevention and treatment. OBJECTIVE:To comprehensively introduce the specific receptor proteins and signaling pathways related to mitochondrial autophagy and their role in the prevention and treatment of sarcopenia through exercise. METHODS:A literature search was conducted between February 1,2023,and April 1,2023,covering literature from database inception to April 2023.Databases included the Web of Science,PubMed,China National Knowledge Infrastructure(CNKI),WanFang Data,and VIP.Keywords used for the search included sarcopenia,muscle wasting,aging,elderly,mitochondria,mitochondrial function,proteins,pathways,and others.After strict inclusion and exclusion criteria,76 articles were ultimately included. RESULTS AND CONCLUSION:Sarcopenia is a disease characterized by a decline in muscle mass and function with age,and its pathogenesis involves neuro-muscular functional decline,chronic inflammation,acid-base imbalance,and mitochondrial dysfunction.Mitochondrial autophagy is an important process for clearing damaged mitochondria in cells,in which receptor proteins and signaling pathways are involved in the regulation of mitochondrial autophagy.Exercise can promote the occurrence of mitochondrial autophagy by regulating the activity of these receptor proteins and signaling pathways,thereby playing an important role in the prevention and treatment of sarcopenia.Exercise can induce mitochondrial autophagy in sarcopenia by upregulating AMPK,phosphorylating ULK1,and reducing mitochondrial energy,enhancing the expression of mitochondrial autophagy-related proteins associated with AMBRA1,and regulating the PINK1/Parkin pathway,to improve mitochondrial dysfunction caused by sarcopenia.In addition,exercise can activate the mTOR pathway to promote muscle growth and increase glucose uptake,thereby preventing and treating sarcopenia.Future studies are needed to further investigate the specific mechanisms and regulatory pathways of mitochondrial autophagy-related receptor proteins and signaling pathways in the prevention and treatment of sarcopenia by exercise,and to conduct more clinical trials in humans,thereby to promote further development in this field.

Objective:To explore the clinical phenotype and genetic etiology for a Chinese pedigree affected with Autosomal dominant polycystic kidney disease (ADPKD).Methods:A pedigree with ADPKD diagnosed at the Department of Gynaecology of the First Affiliated Hospital of Zhengzhou University in December 2020 was selected as the study subject. Clinical data of the pedigree was collected, and whole exome sequencing (WES) was carried out for the proband. Candidate variants were verified by Sanger sequencing of the proband and her relatives. This study was approved by the First Affiliated Hospital of Zhengzhou University (Ethics No. KS-2018-KY-36).Results:Fetal ultrasonography showed increased volume and parenchymal echogenicity in both kidneys. The fetus was found to harbor c. 11098C>T (p.R3700C) and c.11039T>C (p.F3680S) compound heterozygous variants of the PKD1 gene, which were respectively inherited from its mother and father. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), both variants were predicted to be likely pathogenic (PM1+ PM2_Supporting+ PP3). Conclusion:The c. 11098C>T (p.R3700C) and c. 11039T>C (p.F3680S) compound heterozygous variants of the PKD1 gene probably underlay the ADPKD in the fetus. Above finding has provided guidance for the genetic counseling and prenatal diagnosis for this pedigree.

Objective To investigate the effectiveness of applying the CIPP(Context,Input,Process,and Product)model in the training of general workers in a disinfection supply center.Methods From January to March 2023,a total of 24 general workers in our hospital's disinfection supply center underwent traditional training as the pre-management phase.Subse-quently,from July to September 2023,a training system centered on the CIPP model was implemented as the post-management phase.After the training,the examination results and training evaluations were analyzed using SPSS 13.0 statistical software.Results The technical training of the general workers resulted in improved learning outcomes compared to before the training.Conclusion By applying the CIPP model in the training of general workers in a disinfection supply center,their grasp of basic knowledge and professional skills can be enhanced.This can reduce technical errors during operations,improve the quality of sterile items,and reduce the risk of nosocomial infections,thereby ensuring patient safety.

Objective:To investigate the current situation and influencing factors of patients′ satisfaction with nursing humanistic care, and to provide reference for improving the quality of such care provided by hospitals.Methods:From July to August 2022, outpatients and inpatients in 30 provinces were selected by multi-stage stratified sampling as the survey objects. A cross-sectional survey was conducted on an online platform, using the general information questionnaire and Chinese version of methodist health care system nurse caring instrument revised by the research group. The latter instrument consists of 12 dimensions. namely care coordination, competence, teaching/learning, emotional support, respect for individuality, physical comfort, availability, helping/trusting relationship, patient/family engagement, physical environment, spiritual environment and outcomes. Descriptive analysis was performed on the data collected by the questionnaires, and independent sample t-test and one-way ANOVA were used to analyze the influencing factors of patient satisfaction. Results:A total of 107 hospitals were selected for questionnaire survey, including 86 tertiary hospitals and 21 secondary hospitals, and 29 108 valid questionnaires were recovered. The patient satisfaction with nursing humanistic care scored (5.40±0.86); the top three dimensions were competence (5.50±0.89), emotional support (5.47±0.88) and helping/trusting relationship (5.46±0.86); the lowest scoring dimensions were teaching/learning (5.38±1.01), spiritual environment (5.36±1.04) and patient/family engagement (5.11±1.28). Differences with gender, age, marital status, child status, educational level, occupation, place of residence, economic region, per capita monthly income of the family, type of medical insurance, medical department visited and surgery or not presented significant differences on the patient satisfaction with nursing humanistic care scores ( P<0.05). Conclusions:The satisfaction of patients with hospital′s nursing humanistic care in China was at the middle to upper level. In the future, health education for patients should be strengthened, and a mode of family-engaged nursing humanistic care should be constructed in line with the Chinese cultural background. In the process of nursing services, the particularity of patient groups should be considered to better meet their needs.

Objective:To identify the relationship between exposure to mobile phones and other electronic products and the ability development in children.Methods:Retrospective study.A total of 218 children aged 0.5-6.0 years presented to the Department of Health Care, Beijing Children′s Hospital, Capital Medical University form September 2019 to June 2020 for developmental examination were included.Those with nervous system, mental system diseases, endocrine system diseases and premature children were excluded.Their development was evaluated and the information about exposure to mobiles phones and other electronic products were collected. Chi- square test and Fisher′ s exact probability method were used to analyze the relationship between the exposure to mobile phones and other electronic products and the ability development in children.The influencing factors of children′s development in various fields were analyzed by the binary Logistic regression. Results:Abnormal fine movements of children aged 0.5-0.9 years were related to the latest time every night of exposure ( P<0.05). Abnormalities of adaptability, language and social self-care in children aged 1.0-2.9 years and abnormality of language in children aged 3.0-5.9 years were correlated with the number of hours of daily exposure (all P<0.05). Abnormalities of language and social self-care in children aged 1.0-2.9 years and abnormalities of adaptability, language and social self-care in children aged 3.0-5.9 years were correlated with the purposes of exposure (all P<0.05). Binary Logistic regression analysis indicated that the children′s gross motor was affected by the number of hours of daily exposure ( OR=1.868, P<0.05). The children′s fine motor movements were affected by mother′s educational level and the latest time every night of exposure ( OR=1.722, 2.355, all P<0.05). The children′s adaptability was affected by mother′s educational level, child caregivers, the number of hours of daily exposure and the latest time every night of exposure ( OR=1.711, 2.866, 1.895, 1.650, all P<0.05). The children′s speech was affected by the number of hours of daily exposure, the latest time every night of exposure, and the purposes of exposure (telephone or video phone, early education or study)( OR=2.348, 1.806, 0.328/0.350, all P<0.05). The children′s social interaction and self-care delay were affected by mother′s educational level, the number of hours of daily exposure and the purposes of exposure (telephone or video phone, early education or study)( OR=1.647, 2.678, 0.307/0.363, all P<0.05). Conclusions:The adverse effects of exposure to mobile phones and other electronic products on children should be well concerned.The exposure time of developing young children should be strictly controlled to prevent the adverse effects on the nervous system and development in children.For children who were already affected, relevant habits and behaviors should be timely corrected to avoid irreversible damages.

A case of ichthyosis follicularis,alopecia and photophobia syndrome caused by a novel mutation c.1165C＞T in the membrane-bound transcription factor protease site 2 (MBTPS2) gene was firstly reported.The proband presented with dry skin,congenital hairlessness,follicular keratotic papules,photophobia,epilepsy,and mental and motor retardation.Next-generation and Sanger sequencing analysis confirmed that the proband and his mother both had a c.1165C＞T (p.pro389Ser) mutation in exon 9 of the MBTPS2 gene.According to the clinical manifestations of the patient and genetic characteristics of the MBTPS2 gene mutation,the patient was diagnosed with ichthyosis follicularis,alopecia and photophobia syndrome.

Clinical features of and genetic mutations in two cases of pseudohypoparathyroidism type Ⅰ a(PHP Ⅰ a) with early-onset skin nodules were analyzed.Both of the two patients were males,and their ages at onset were 2 and 3 months respectively.They both presented with early-onset skin nodules as the main clinical manifestation,and were clinically characterized by a round face,short neck and early obesity.Histopathological examination of skin lesions showed subcutaneous ectopic osteogenesis in both patients.The first patient had low blood calcium,high blood phosphorus,high parathyroid hormone (PTH),and gene sequencing showed a heterozygous mutation c.399delT causing a T base deletion at position 399 in exon 5 of the GNAS gene.The second patient had normal blood calcium and phosphorus levels as well as normal PTH levels at early stage,and gene sequencing showed a heterozygous mutation c.939delT causing a T base deletion at position 939 in exon 9 of the GNAS gene.The blood PTH level was found to increase in the second patient after 1-year follow-up.Both the patients were confirmedly diagnosed with PHP Ⅰa.After treatment with vitamin D3,no new skin nodules occurred,and the blood calcium and phosphorus levels returned to normal.

A male patient, who was aged 3 months and 12 days, presented with well-circumscribed erythema and scales on the scrotum, perineum, buttocks and perianal region at 1 month after birth. The lesions gradually involved the perioral and axillary regions, flexor aspect of the elbow, popliteal fossa and neck. Shortness of breath, crying, dysphoria and vomiting occurred without fever and cough 3 days before hospitalization. Laboratory examinations at admission showed metabolic acidosis, hyperlactacidemia, hyperammonemia and organic aciduria. Second-generation sequencing and Sanger sequencing of the holocarboxylase synthetase gene revealed a known mutation c.1522C>T in exon 9 and a novel mutation c.1796_1814del in exon 11. According to a guideline from the American College of Medical Genetics and Genomics, this novel mutation was ranked as a pathogenic mutation. The patient was diagnosed as multiple carboxylase deficiency. His clinical symptoms were improved after oral biotin treatment, no neurological symptoms or signs were observed.

Objective To study the clinical characteristics of outpatients with hand,foot and mouth disease (HFMD) caused by different serotypes of enteroviruses.Methods This was a prospective study.From February 2017 to March 2018,563 outpatients with HFMD were enrolled by systematic sampling in the Department of Infectious Diseases,Henan Children's Hospital.Throat swabs were collected to determine the serotypes via PCR.Demographic,clinical,and laboratory data were collected by standard questionnaire.All cases were followed up twice at 2 and 9 weeks after the initial outpatient visit through telephone interview.A total of 563 cases were enrolled and 555 (98.6％) cases were positive for human enteroviruses,including 338 (60.9％) males.Analyses were stratified by enterovirus serotypes,Chi square test or Fisher's exact test,Rank sum test was used for comparison among different groups.Results The age of 555 cases was 24.2 (16.4,41.3) months.Among them 44.0％ (224 cases) were identified as coxsackievirus (CV)-A6,while 189 cases,35 cases,14 cases and 73 cases were identified as CV-A16,enterovirus (EV)-A71,CV-A10 and other serotypes,respectively.Fever (≥37.5 ℃C) was present in 51.4％(285/555) of laboratory confirmed cases.The proportions of fever in cases of CV-A6 (68.9％(168/244)) and CV-A10 (12/14) were significantly higher than those in cases of CV-A16 (31.7％(60/189),x2=57.344,14.313,both P=0.000),other serotypes (43.8％(32/73),x2=15.101 and 8.242,P=0.000 and 0.004) and EV-A71 (37.1％(13/35),x2=13.506 and 9.441,P=0.000 and 0.002) respectively.There was no significant difference between CV-A6 and CV-A10 in presentation of fever (x2=1.785,P=0.182).There were 359 cases (64.7％) with eruptions in mouth,hands,feet and buttocks.Cases infected with EV-A71 had the highest proportions (74.3％(26/35)) of rash emerging simultaneously in mouth,hands,feet,and buttocks.The proportion in cases of CV-A 16,CV-A6,CVA 10 and other serotype were 73.5％ (139/189),61.9％ (151/244),7/14 and 49.3％ (36/73),respectively.The proportion of rash on other parts of body,such as face,limbs or torso in cases infected with CV-A6 (16.8％ (41/244)) was the higherest and the proportion in cases of CV-A16,EV-A71,CV-A10 or other serotypes were 8.5％(16/189),5.7％(2/35),1/14,6.8％(5/73),respectively.None of these cases developed serious complications.Desquamation occurred in 45.5％ (179/393) cases 7.5 (5.0,9.0) days after disease onset and 13.5％ (53/393) cases showed onychomadesis 31.0 (18.0,33.5) days after disease onset.The proportion of desquamation and onychomadesis associated with CV-A6 (64.2％ (95/148) and 31.8％ (47/148)) was significantly higher than CV-A16 (31.8％ (49/154) and 1.3％ (2/154),x2=33.601 and 52.482,both P=0.000) and other serotypes (38.0％(19/50) and 6.0％(3/50),x2=10.236 and 12.988,P=0.001 and 0.000).Desquamation appeared more in cases of CV-A6 than in cases of CV-A10 (2/11,x2=9.386,P=0.002),with the proportion of onychomadesis higher in CV-A6 than in EV-A71 (3.3％ (1/30),x2=11.088,P=0.001).Conclusion Clinical manifestation such as fever,rash emerging parts,desquamation and onychomadesis are different among outpatient HFMD cases infected with CV-A16,CV-A6,EV-A71,CV-A10 and other enteroviruses.

Objective To investigate the changes of follicular regulatory T cells ( Tfr cells) and follicular T helper cells ( Tfh cells) in peripheral blood of children with myasthenia gravis ( MG) . Methods We recruited 28 MG patients and 20 healthy subjects in this study. The percentages of Tfh and Tfr cells in peripheral blood samples were measured by flow cytometry. Real-time PCR was performed to detect the ex-pression of transcription factors and regulatory factors of Bcl-6, c-MAF, Blimp-1 and PD-1 at mRNA level. ELISA was used to detect the levels of IL-2, IL-6, IL-10 and IL-21 in plasma samples and the titers of Ach-Rab and PsMab. Results Compared with the healthy subjects, the MG patients showed higher percentages of Tfh cells and lower percentages of Tfr cells before receiving treatment. The expression of Bcl-6 and c-MAF on CD4+T lymphocytes cells at transcriptional level were significantly enhanced, while the expression of Blimp-1 on CD4+T cells and the expression of PD-1 on Treg cells at transcriptional level were inhibited in the MG patients in comparison with those in healthy subjects. Moreover, decreased levels of IL-2 and increased levels of IL-21 were found in plasma samples collected from the MG patients. Conclusion The decreased percentages of Tfr cells and increased percentages of Tfh cells in patients with MG resulted in abnormal ratios of Tfr/Tfh cells, which might be involved in the immunological pathogenesis of MG. Several changes in the patients with MG might be responsible for the imbalanced ratio of Tfr/Tfh cells, which included changes of IL-2 and IL-21 in microenvironment, enhanced expression of Bcl-6 and c-MAF at mRNA level and inhibited expression of Blimp-1 at mRNA level on CD4+T cells as well as over-expression of PD-1 at mRNA level on Treg cells.