Transent receptor potential(TRP)channels are non-selective cation channels.In recent years,a large number of studies have found that TRP channels participate in a variety of cardiovascular diseases.How mitochondrial function is regulated by TRP channels and the relationship to cardiovascular diseases have become research hotspots.Up to now,related studies have mainly focused on TRPV,TRPM,and TRPC channels.This review focuses on the roles of the above TRP channels in the regulation of mitochondrial function and their relationships to cardiovascular diseases.

Objective:To investigate the epidemiological characteristics and prognosis of critically ill patients with sepsis combined with acute kidney injury (AKI) in intensive care unit (ICU) in Beijing, and to analyze the risk factors associated with in-hospital mortality among these critically ill patients.Methods:Data were collected from the Beijing AKI Trial (BAKIT) database, including 9 049 patients consecutively admitted to 30 ICUs in 28 tertiary hospitals in Beijing from March 1 to August 31, 2012. Patients were divided into non-AKI and non-sepsis group, AKI and non-sepsis group, non-AKI and sepsis group, AKI and sepsis group. Clinical data recorded included demographic characteristics, primary reasons for ICU admission, comorbidities, sequential organ failure assessment (SOFA), acute physiology and chronic health evaluation Ⅱ(APACHEⅡ) within 24 hours of ICU admission, physiological and laboratory indexes, treatment in the ICU, AKI staging based on the Kidney Disease: Improving Global Outcomes (KDIGO), as well as the prognostic indicators including length of stay in ICU, length of stay in hospital, ICU and in-hospital mortality. The primary endpoint was discharge or in-hospital death. Multivariate Logistic regression analysis was used to investigate the risk factors for hospital death in ICU patients. Kaplan-Meier survival curve was drawn to analyze the cumulative survival of ICU patients during hospitalization.Results:A total of 3 107 critically ill patients were ultimately enrolled, including 1 259 cases in the non-AKI and non-sepsis group, 931 cases in the AKI and non-sepsis group, 264 cases in the non-AKI and sepsis groups, and 653 cases in the AKI and sepsis group. Compared with the other three group, patients in the AKI and sepsis group were the oldest, had the lowest mean arterial pressure (MAP), and the highest APACHEⅡscore, SOFA score, blood urea nitrogen (BUN), and serum creatinine (SCr) levels, and they also had the highest proportion of receiving mechanical ventilation, requiring vasopressor support, and undergoing renal replacement therapy (RRT), all P < 0.01. Of these 3 107 patients, 1 584 (51.0%) were diagnosed with AKI, and the incidence of AKI in patients with sepsis was significantly higher than in those without sepsis [71.2% (653/917) vs. 42.5% (931/2 190), P < 0.01]. The highest proportion of KDIGO 0 stage was observed in the non-sepsis group (57.5%), while the highest proportion of KDIGO 3 stage was observed in the sepsis group (32.2%). Within the same KDIGO stage, the mortality of patients with sepsis was significantly higher than that of non-sepsis patients (0 stage: 17.8% vs. 3.1%, 1 stage: 36.3% vs. 7.4%, 2 stage: 42.7% vs. 17.1%, 3 stage: 54.6% vs. 28.6%, AKI: 46.1% vs. 14.2%). The ICU mortality (38.7%) and in-hospital mortality (46.1%) in the AKI and sepsis group were significantly higher than those in the other three groups. Kaplan-Meier survival curves further showed that the cumulative survival rate of patients with AKI and sepsis during hospitalization was significantly lower than that of the other three groups (53.9% vs. 96.9%, 85.8%, 82.2%, Log-Rank: χ2 = 379.901, P < 0.001). Subgroup analysis showed that among surviving patients, length of ICU stay and total length of hospital stay were significantly longer in the AKI and sepsis group than those in the other three groups (both P < 0.01). Multivariate regression analysis showed that age, APACHEⅡscore and SOFA score within 24 hours of ICU admission, coronary heart disease, AKI, sepsis, and AKI combined with sepsis were independent risk factors for ICU mortality in patients (all P < 0.05). After adjusting for covariates, AKI, sepsis, and sepsis combined with AKI were significantly associated with higher ICU and in-hospital mortality, with the highest ICU mortality [adjusted odds ratio ( OR) = 14.82, 95% confidence interval (95% CI) was 8.10-27.12; Hosmer-Lemeshow test: P = 0.816] and in-hospital mortality (adjusted OR = 7.40, 95% CI was 4.94-11.08; Hosmer-Lemeshow test: P = 0.708) observed in patients with sepsis combined with AKI. Conclusions:The incidence of AKI is high in sepsis patients, and those with both AKI and sepsis have a higher disease burden, more abnormalities in physiological and laboratory indicators, and significantly increased ICU and in-hospital mortality. Among surviving patients, the length of ICU stay and total length of hospital stay are also longer in the AKI and sepsis group. Age, APACHEⅡscore and SOFA score within 24 hours of ICU admission, coronary heart disease, AKI, and sepsis are independent risk factors for in-hospital mortality in ICU patients.

  Objective  To summarize the clinical characteristics and RNF216 gene mutation of a patient with Gordon Holmes syndrome (GHS), and to improve the understanding of the genetic and clinica characteristics of this disease through literature review.  Methods  We collected the clinical data of the patient with GHS, extracted the DNA from 2 mL peripheral venous blood of the patient and his parents for whole exome gene detection, and then we analyzed the clinical and genetic characteristics of all previously reported patients with RNF216 gene mutation.  Results  The young male patient was short in stature at sixyearsold and was diagnosed growth hormone deficiency.He had no secondary sexual characteristics by the age of 15 and was diagnosed hypogonadal hypogonadism.After the age of 22, he gradually developed abnormal gait and had progressive decline in speech, motor, and cognitive functions.Whole exome sequencing revealed a homozygous, nonsense mutation c.1549C>T (p.R517*) in the RNF216 gene.His parents were consanguineous and were heterozygous carriers of the mutations with phenotypic normality.Combined with literature review and this case report results showed that a total of 21 patients of the disease in the world and among them 15 had pathogenic variants of RNF216 gene mutation.7 of the 15 had truncated mutations, 5 had missense mutations, and 1 synonym mutation, 1 splice mutation, and 1 deletion mutation respectively.RNF216 gene mutation can be seen in neurodegenerative diseases with multiple overlapping symptoms of GHS, Huntington-like disease, and 4H syndrome.The main clinical manifestations are hypogonadotropic hypogonadism and early-onset progressive neurological dysfunction in adolescence or early adulthood.The median age of onset of neurological symptoms is 28 years old, featuring cerebellar ataxia, dysarthria, and cognitive impairment, as well as imaging manifestations of extensive white matter lesions and cerebellar atrophy.  Conclusions  The mutation of RNF216 gene can cause GHS.Genetic testing is helpful to the diagnosis and treatment of rare diseases.

Objective:To report the clinical characteristics, diagnosis, and treatment of 2 cases of X-linked acrogigantism(X-LAG).Methods:The clinical information of two patients were retrospectively reported, and peripheral blood DNA was collected for copy number variations detection.Results:Both patients had onset at age of two, with common clinical characteristics including linear growth acceleration, mild facial coarsening, enlargement of hands and feet, increased appetite, and snoring, etc. The heights Z scores of the two patients before treatment were + 6.86 and + 6.53, respectively. Growth hormone(GH) glucose inhibition test showed that GH nadir values were over 1 ng/mL and insulin-like growth factor-Ⅰ(IGF-Ⅰ) were 586.0 ng/mL and 1 042.0 ng/mL, respectively. Patient 1 received three cycles of octreotide microspheres therapy followed by surgery, and achieved clinical and biochemical remission. Patient 2 had lanreotide for 5.5 years but failed biochemical remission. Microduplication of Xq26.3, which contained pathogenic gene G-protein coupled receptor 101(GPR101), was found in germline DNA of two patients through copy number variation detection, leading to the diagnosis of X-LAG.Conclusion:It should be cautious of X-LAG when children below 2 years old presents symptoms such as overgrowth and so on. Medication combined with surgery is effective.

Objective:To explore the value of octreotide suppression test(OST) in predicting the efficacy of somatostatin receptor ligands(SRLs) in the treatment of active acromegaly.Methods:The clinical data of 76 patients with active acromegaly from 2011 to 2020 was retrospectively analyzed. OST was carried out as follows: After an overnight fasting and baseline sampling of growth hormone(GH), 100 μg octreotide was subcutaneously injected, and sampling for GH was obtained every 2 hours for 8 hours. All patients were treated with SRLs for at least 3 months. A good GH response is defined as a post-treatment random GH<1 μg/L or >80% fall compared with the baseline GH. A good insulin-like growth factor Ⅰ(IGF-Ⅰ) response is defined as IGF-Ⅰ<1.3 upper limit of normal(ULN) or >50% reduction compared with the baseline. If both GH and IGF-Ⅰ fulfill the criteria of a good response, it is defined as a good GH and IGF-Ⅰ response.Results:The baseline level of GH during OST was 15.00(6.38, 34.20) μg/L, the median time to reach the nadir GH was(3.65±1.65) hours, and the nadir GH level was 1.47(0.50, 4.19) μg/L. The median GH suppression rate was 89.12%(72.71%, 95.09%). When the cutoff value of GH suppression rate in predicting a good GH response was 89.32%, the area under the curve(AUC) was 0.74, with a sensitivity of 81.80% and specificity of 66.00%. When the cutoff value of GH suppression rate in predicting a good IGF-Ⅰ response was 93.14%, the AUC was 0.64, with a sensitivity of 50.00% and specificity of 75.60%. When the GH suppression rate was 90.71%, the AUC was 0.78, with the sensitivity of 83.30% and specificity of 70.00% in predicting a good GH and IGF-Ⅰ response. Compared with GH/IGF-Ⅰ non-responders, GH/IGF-Ⅰ responders displayed lower nadir GH during OST, higher GH suppression rate and IGF-Ⅰ reduction rate, and lower ratio of IGF-1 to ULN( P<0.05). Conclusion:GH suppression rate during the OST is a valuable predictor to evaluate the efficacy of SRLs in patients with acromegaly, with the highest sensitivity and specificity when the cutoff value is 90.71%.

The current document is based on a consensus reached by a panel of experts from the Chinese Society of Allergy and the Chinese Society of Otorhinolaryngology-Head and Neck Surgery, Rhinology Group. Chronic rhinosinusitis (CRS) affects approximately 8% of Chinese adults. The inflammatory and remodeling mechanisms of CRS in the Chinese population differ from those observed in the populations of European descent. Recently, precision medicine has been used to treat inflammation by targeting key biomarkers that are involved in the process. However, there are no CRS guidelines or a consensus available from China that can be shared with the international academia. The guidelines presented in this paper cover the epidemiology, economic burden, genetics and epigenetics, mechanisms, phenotypes and endotypes, diagnosis and differential diagnosis, management, and the current status of CRS in China. These guidelines—with a focus on China—will improve the abilities of clinical and medical staff during the treatment of CRS. Additionally, they will help international agencies in improving the verification of CRS endotypes, mapping of eosinophilic shifts, the identification of suitable biomarkers for endotyping, and predicting responses to therapies. In conclusion, these guidelines will help select therapies, such as pharmacotherapy, surgical approaches and innovative biotherapeutics, which are tailored to each of the individual CRS endotypes.
Adult ; Asian Continental Ancestry Group ; Biomarkers ; China ; Consensus ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Eosinophils ; Epidemiology ; Epigenomics ; Genetics ; Humans ; Hypersensitivity ; Inflammation ; International Agencies ; Medical Staff ; Neck ; Phenotype ; Precision Medicine

OBJECTIVE: To evaluate the application of three-in-one intelligent screening in outpatient pre-inspection in children's hospital. METHODS: We randomly enrolled 100 children pre-screened by traditional method in the outpatient department of Children's Hospital of Zhejiang University from February 6th to 16th, 2020, and another 100 children by the intelligent three-in-one mode from February 17th to 27th, 2020. The traditional triage was conducted by nurses based on face-to-face, one-by-one interview of the epidemiological history and consultation department, and the temperature was measured before manual triage. The intelligent three-in-one model combined online rapid pre-inspection and triage, on-site manual confirmation, as well as synchronized online health education system. For on-line registered patients, the system automatically sent the COVID-19 epidemiological pre-screening triage questionnaire one hour before the appointment, requiring parents to complete and submit online before arriving at the hospital. The on-site registered patients were controlled at 100 m away from the hospital entrance. The nurses guided the parents to scan the QR code and fill in the COVID-19 epidemiological pre-examination triage questionnaire. At the entrance of the hospital, the nurse checked the guidance sheet and took the temperature again. The children with red guidance sheet were checked again and confirmed by pre-examination nurses, and accompanied to the isolation clinic through COVID-19 patients-only entrance. The children with yellow guidance sheet were guided to fever clinic. The children with green guidance sheet could go with their parents to the designated area, and then went to the corresponding consultation area. Health education was carried out throughout the treatment, and the system automatically posted the corresponding outpatient instructions and education courses. Parents would read the courses on their mobile phones and counsel online. The time of pre-examination and the coincidence rate of triage were compared between the two groups. RESULTS: The three-in-one intelligent pre-inspection mode took an average of (25.6±8.0) s for each child, which was significantly shorter than the traditional pre-inspection mode (74.8±36.4) s ( CONCLUSIONS The three-in-one intelligent pre-inspection model can effectively shorten the patient pre-check time, with similar triage coincidence rate to traditional model.
Adult ; Betacoronavirus ; COVID-19 ; Child ; Coronavirus Infections/diagnosis* ; Humans ; Internet ; Outpatient Clinics, Hospital ; Pandemics ; Pneumonia, Viral/diagnosis* ; SARS-CoV-2 ; Surveys and Questionnaires ; Time ; Triage/standards*

X-linked acrogigantism (X-LAG) syndrome is a newly described early-onset giant disease caused by a duplication of the X chromosome q26.3 and a mutation in the G protein-coupled receptor 101(GPR101) gene. Unlike other causes of gigantism, X-LAG syndrome is characterized by early onset, and has special histopathological manifestations and severe clinical phenotype. Early identification, diagnosis, and clinical management are particularly important and challenging. This article reviews the current research progress of X-LAG syndrome, in order to provide theoretical basis for further research and clinical diagnosis and treatment of the disease.

ObjectiveA preliminary study on pediatrics nursing-sensitive qualityindicators was carried out to construct a systematic, scientific and sensitive quality system. Methods Delphi method was used to conduct two rounds of questionnaires for 55 pediatric nursing experts, used SPSS20.0 statistical software for data analysis. Analytic hierarchy process method was used to determine the weight of the indicators. Results The final determination agreed rate of experts equal to 75 percent, the importance of assignment mean was>3.5 and the variation coefficient<0.25, which including 5 structure indicators, 17 process indicators and 18 outcome indicators. Conclusion The evaluation system of pediatrics nursing-sensitive quality indicators was constructed in preliminary which could provide the reference and basis for pediatric nursing managers.

Objective To analyze the effects of dexmedetomidine(Dex) on levels of inflammatory cytokines in patients with acute exacerbation of chronic obstructive pulmonary disease (COPD). Methods From April 2015 to April 2017,94 patients with acute exacerbations of COPD who received mechanical ventilation in Wenling Hospital Affiliated to Wenzhou Medical College were randomly divided into two groups according to the digital table,with 47 cases in each group. Group A was given Dex,and group B was given midazolam. The levels of TNF - α,IL - 1, hs - CRP between the two groups were compared. Results There were no statistically significant differences in TNF - α,IL - 1,hs - CRP between the two group before treatment (all P > 0. 05). One day after treatment,the levels of TNF - α,IL -1,hs - CRP in group A were (92. 99 ± 15. 25)ng/ L,(68. 63 ± 14. 53)ng/ L,(15. 44 ± 4. 92)mg/ L, respectively,which in group B were (150. 63 ± 20. 78)ng/ L,(91. 13 ± 12. 58)ng/ L,(19. 35 ± 5. 17)mg/ L,respec-tively,there were statistically significant differences between the two groups(t = 2. 736,2. 503,2. 421,P = 0. 008, 0. 012,0. 015). Three days after treatment,the levels of TNF - α,IL - 1,hs - CRP in group A were (73. 37 ± 11. 01)ng/ L,(51. 52 ± 9. 16) ng/ L, (14. 82 ± 3. 29) mg/ L, respectively, which in group B were (123. 80 ± 19. 62)ng/ L,(73. 46 ± 17. 36) ng/ L, (18. 77 ± 4. 02 ) mg/ L, respectively, there were statistically significant differences between the two groups(t = 2. 184,2. 354,2. 098,P = 0. 031,0. 022,0. 039). Five days after treatment, the levels of TNF - α,IL - 1,hs - CRP in group A were (63. 54 ± 13. 06)ng/ L,(34. 04 ± 8. 13) ng/ L,(13. 84 ± 4. 13)mg/ L,respectively,which in group B were (76. 63 ±17. 39)ng/ L,(50. 66 ± 9. 17)ng/ L,(14. 27 ± 3. 95)mg/ L, respectively,there were statistically significant differences between the two groups( t = 1. 906,2. 082,1. 834,P =0. 067,0. 041,0. 081). Compared with group B,the ICU time in group A was significantly decreased[(9. 27 ± 1. 63) d vs. (7. 16 ± 1. 21)d,t = 2. 215,P = 0. 031](t = 2. 215,P = 0. 031). There was no statistically significant difference in the incidence rate of multiple organ dysfunction syndrome between the two groups at 5 days after treatment (χ2 =2. 286,P = 0. 132). Conclusion Dex can effectively inhibit inflammation cytokines of the mechanical ventilation COPD patients in acute exacerbation,and can reduce the ICU time.