Objective To analyze the mutations in keratin 1 (KRT1), KRT9 and KRT10 genes in a Chinese family with epidermolytic palmoplantar keratoderma (EPPK). Methods Clinical data were collected from a family with EPPK. Genomic DNA was extracted from the peripheral blood of 12 family members, including 6 patients and 6 unaffected members, as well as from 50 unrelated normal human controls. PCR was performed to amplify all the exons and flanking sequences of KRT1, KRT9 and KRT10 genes followed by DNA sequencing.Results A missense mutation C.1436T > C was found in the highly conserved helix termination motif of KRT1 gene of all the patients, resulting in a substitution of isoleucine by threonine at position 479 of the KRT1 protein. No mutation was found in the unaffected members or unrelated controls. Conclusions The missense mutation C.1436T > C in K.RT1 gene is likely to be the main cause of the phenotype of EPPK in this family.This is the first report of a pedigree with KRT1 gene mutation-induced EPPK in China.

Objective To evaluate the effect of transitional care on the quality of life in patients with maintenance hemodialysis (MHD).Methods 101 MHD patients with symptoms of kidney ＜ 80 points were assigned into the interventional group (47 of 51 cases completed the study) and the control group (45 of 50 cases completed the study) by random number table method.The patients in the interventional group received transitional care for 6 months,while control group received routine care.The effects on function index,satisfaction index and clinical index were evaluated at the 3rd month and 6th month after intervention.Results 6 months after intervention,the scores of physical composite,mental composite,burden of kidney disease,symptoms/problems list,effects of kidney disease in the interventional group were 51.78±5.91,55.32±5.36,29.79±17.64,79.39±6.73,68.02±11.38,while the scores of the control group were 38.22±9.46,42.03±8.32,19.58±17.25,69.49±10.10,52.09±16.78,and the difference between the two groups was statistically significant (t=5.308-9.063,Z=-3.07,P ＜ 0.01).The scores of self-management behavior,health locus of control in the interventional group were 71.30-±5.42,25.74±2.42,while the scores of the control group were 65.0±8.74,22.47±3.97 (t=4.132,4.759,P ＜ 0.01).The nursing satisfaction in the interventional group scored 91.06±7.29,which was significantly higher than the control group scored 86.22±10.72 (Z=-2.45,P＜ 0.05).The level of serum phosphorus (PO4),Ca × PO4 products,parathormone (PTH) in the interventional group were (1.76±0.35) mmol/L,(51.02±10.04) mg2/dl2,464.56 ng/L,which were significanlty lower than (2.01±0.54) mmol/L,(57.41±17.38) mg2/dl2,625.78 ng/L in the control group (t=-2.691,-2.167,-2.000 in tum,P ＜ 0.01 or 0.05).Conclusions Transitional care achieves good effectiveness on the quality of life in patients with MHD.

Objective To investigate the current situation of nurses' practice environment in tertiary general hospitals in Anhui Province,and to provide references for improving it.Methods The Nursing Work Index-Revised was used to investigate nurses from 35 tertiary general hospitals in Anhui Province.Results The mean score of Nursing Work Index-Revised was 64.79±26.07.The entry of hospital has a training program for newly recruited nurses,nurses in the work team are qualified for care received the highest score;the entry of the level of remuneration of nurses is appropriate in all sectors of society,nurses have the opportunity to participate in hospital internal management and nurses have the opportunity to participate in hospital management decisions got the lowest score.Whether a teaching hospital,the number of years of work,gender,education,department,title,age and whether recruited with authorized strength played important roles in nurses' practice environment.Conclusion The score for nurses practice environmental in Anhui Province was not high,and there were differences between regions.Hospital management mode and nurse salary distribution system need to be improved.Nursing managers should take appropriate measures to address existing problems and improve the nurses' practice environment according to different characteristics.

Objective To identify predictors of prescription initiation and persistence of warfarin in non-valvular atrial fibrillation ( NVAF ) patients with high risk of stroke ( CHA2 DS2-VASc≥2 ) . Methods NVAF patients consulted in our hospital from Aug , 2011 to Apr, 2015 were enrolled.Patients who underwent radiofrequency catheter ablation were excluded . Patients were divided into two groups (warfarin group and non-warfarin group).Logistic regression was used to estimate the predictors of initiation warfarin prescription.Kaplan-Meier survival and Cox proportional hazards model was performed to determine rate of warfarin persistence and its associated factors .Results A total of 622 AF patients were enrolled and 490 patients with CHA2DS2-VASc≥2.Ten patients lost follow up and 480 patients were followed up with a mean follow-up period of ( 40.0 ±11.55 ) months.Of which 245 NVAF patients ( 51%) had a warfarin prescription.Patients with ischemic stroke ( OR 2.447 , 95%CI 1.435-4.171 , P=0.001 ) , heart failure ( OR 2.009 , 95%CI 1.084-3.724 , P=0.027 ) and persistent AF ( OR 2.231 , 95%CI 1.448-3.437 , P=0.0001 ) had a higher likelihood of warfarin prescription .Anemia ( OR 0.479 , 95%CI 0.238-0.964 , P=0.039), concommitant Traditional Chinese Medicine (TCM) use (OR 0.638, 95%CI 0.456-0.891, P=0.008 ) and longer distance to hospital ( OR 0.759 , 95%CI 0.610-0.945 , P=0.014 ) decreased the likelihood of warfarin prescription . One hundred and seventy-six ( 71.8%) warfarin users continued persistent therapy and the overall proportion of warfarin persistence was 78.3% for one year , 71.0% for 3 years.Seventy-six existing warfarin users continued the warfarin therapy (80%, 76/95),one hundred new users showed persistence to therapy ( 66.7%, 100/150 ) .Warfarin use before enrollment significantly increased warfarin persistence than new prescription ( P =0.008 ) .Variables associated with higher discontinuation were new prescription ( HR 1.786 , 95% CI 1.029-3.100 , P=0.039 ) , TCM use ( HR 1.687 , 95%CI 1.201-2.37 , P=0.003 ) and longer distance to hospital ( HR 1.446 , 95% CI 1.121-1.865, P=0.005).Conclusions In anticoagulation clinic, concommitant TCM use, distance to hospital and other factors were associated with warfarin initiation prescription and persistence .Identifying factors associated with warfarin treatment could help in developing adherence of patients .

24 cases of renal failure induced by chemotherapy were treated with Wuling powder plus additives.Theresult of clinical amelioration was 87.5%.with a to-tal effective yielded marked actions of promoting ap-petite in animals,lowering serum urea nitrogen,cre-atinine,2 microglobulin.It also decreased the inhibi-tion of PDD on the enzyme activity of renal Na~+—K~+ATP.Microscopic exam revealed the pathological le-sion of kidney to be milder in the tested group.

Objective To study the expression of epidermal growth factor receptor (EGFR) in cervical car-cinoma and cervical intraepithelial neoplasia(CIN),and to elucidate its relation with the genesis, infiltration, metas-tasis and prognosis of cervical carcinoma. Methods EGFR was determined by means of S-P immunohistochemistry in tissue of 100 cases of cervical carcinoma,60 cases of CIN and 40 cases of controls. Results The overexpression rates of EGFR were 0% (0/40), 51.67% (31/60),78.00% (78/100), respectively in normal cervical epithelium, CIN and cervical tumor tissues. The overexpression rate of EGFR was significantly higher in cervical tumor tissue than in control group(P<0.01). The overexpression of EGFR didn't demonstrate significant association with clinical staging, tumor size, pathological type, differentiation, cervical invasion depth, cervical canal invasion, lymphnode me-tastasis or the prognosis of cervical neoplasm (P>0.05). Conclusion Overexpression of EGFR is worsened with the severity of cervical lesion, suggesting that overexpression of EGFR is correlated with the genesis of cervical neo-plasms,which may be a valuable biological indicator of cervical carcinoma,but is not correlated with clinical patho-logical features and prognosis of cervical carcinoma.

Aim To investigate the protective effects of total triterpenoid from Prunella vulgaris L. ( TTP) on CCl4-induced hepatic fibrosis in rats and its mecha-nism. Methods Rat liver fibrosis was induced by 50% CCl4 twice a week for 12 weeks. From the 5th week, all the therapeutic groups were treated with the TTP(25, 50, 100 mg·kg-1 ) and the colchicine (0. 1 mg· kg-1 ) respectively once a day for 8 weeks. At the end of the twelfth week, the levels of ALT, AST, HA, PCⅢ, CⅣ, MDA, SOD, GSH-Px, Hyp were measured . HE and Masson staining were used to evalu-late the degree of hepatic fibrosis. The mRNA expres-sion ofα-SMA, procollagen I, Smad2, Smad3, Smad7 in liver was detected by RT-PCR, and the p-ERK pro-tein expression was evaluated by Western blot. Results Compared with the model group, TTP(25, 50, 100 mg·kg-1 ) not only reduced serum content of ALT, AST, HA, PCⅢ, CⅣand Hyp, MDA in liver tissue, improved the morphologic changes of hepatic fibrosis, but also increased SOD and GSH-Px activity. Moreo-ver, it decreased the α-SMA, procollagen I, Smad2, Smad3 mRNA expression and increased Smad7 mRNA expression in liver tissues obviously. Furthermore, TTP reduced the protein expression of p-ERK. Conclusions TTP can protect rats from CCl4-induced liver fibro-sis. The mechanism of this process may involve inhibi-ting the expression of p-ERK and interference with TGF-β1/Smad signal transduction pathway.

Objective To observe the prevalence of hyperuricemia(HUA)among obese children,and to ex-plore the association between uric acid(UA)levels and cardiometabolic risk factors,acanthosis nigricans and non - al-cohol fatty liver disease(NAFLD). Methods By using representative sampling method,1 753 obese children aged 6 -17 years old from 18 schools in 3 districts of Beijing(Xicheng,Haidian,Miyun)were selected to participate in the clini-cal examinations,including anthropometric measurements(height,weight)and blood pressure. Serum biochemical pa-rameters were assessed,including fasting plasma glucose(FPG),total cholesterol(TC),triglyceride(TG),high - densi-ty lipoprotein cholesterol(HDL - C),low - density lipoprotein cholesterol(LDL - C)and UA. Acanthosis nigricans and B - model ultrasonography of the liver were conducted. Results The prevalence of hypertension,impaired fasting glu-cose,dyslipidemia,acanthosis nigricans,and NAFLD among these 1 753 obese children was 33. 6%(589 cases), 66. 5%(1 156 cases),54. 3%(943 cases),23. 3%(408 cases),and 17. 0%(298 cases),respectively. The preva-lence of HUA was 40. 70%(714 / 1 753 cases),with 50. 17%(581 / 1 158 cases)in boys and 22. 34%(133 / 595 ca-ses)in girls. There was a significant increase in body mass index,systolic blood pressure,diastolic blood pressure, FPG,TG and LDL - C with the increase of UA,but there was a decrease in HLD - C with the increase of UA(all P ﹤0. 05). In boys,the adjusted odds ratios( OR)and 95% CI of the highest quartile of UA for hypertension,impaired fasting glucose,dyslipidemia,acanthosis nigricans,and NAFLD were 1. 16(0. 77 - 1. 74),1. 34(0. 90 - 1. 99),1. 29 (0. 89 - 1. 87),1. 89(1. 17 - 3. 04),and 1. 71(1. 03 - 2. 84),respectively;in girls,the adjusted OR and 95% CI of the highest quartile of UA for hypertension,impaired fas-ting glucose,dyslipidemia,acanthosis nigricans,and NAFLD was 0. 70(0. 40 - 1. 24),0. 60(0. 40 - 1. 00),1. 69(1. 04 - 2. 70),1. 67(0. 80 - 3. 49),and 1. 33(0. 48 - 3. 66),re-spectively. Conclusions The prevalence of HUA is relatively high in obese children and there is a strong association between UA and some car-diovascular metabolic disorders,acanthosis nigricans and NAFLD.

Objective To determine the trend of emergency department (ED)mortality of a tertiary general hospital from 2004 to 2014 in order to find the factors that may impact on ED mortality.Methods Mortality in ED was estimated according to the ratio of ED visiting patients to ED deaths.And the data of all ED deaths in 2004,2009 and 2014 were collected.Variance analysis and chi-square test were used for data analysis.Results During the past decade,ED visiting patients was increased significantly by 38.0% in 2014,compared with those in 2004,and the mortality was also increased accordingly from 0.7% in 2004, to 0.9% in 2009,to 1.2% in 2014 (P <0.01).Finally,a total of 1,091 deaths occurred in these three years were included for further evaluation.There were no significant changes in average age and gender distribution,and the average age was 61.9 and the male /female ratio was 1.36∶1 during past decade.The number of adults under 40 years old (18 -39)increased from 7.5% in 2004,to 10.6% in 2009,to 14.4% in 2014 (P <0.05).Both the facilities were upgraded and the number of staffs in ED increased markedly.The cardiovascular illness,cerebrovascular diseases,and sudden death were the leading causes of ED death during past decade.The incidences of trauma and tumor remained unchanged.Average time consumed from onset of illness to arrival to ED didn’t vary significantly during past decade.The study showed no changes in use of ambulance,but remarkable increases in number of non-compliant patients or their family from 18.3% in 2004,to 25.6% in 2009,to 38.3% in 2014 (P <0.01).The percentage of patients in the night time was higher,but there were no significant changes in number of emergency patients in the night time and during holidays in the past decade,but the mean ED stay time increased obviously from 22.4 h in 2004 to 53.3 h in 2014 (P <0.05 ).Conclusions During the past decade,although ED facilities and number of staffs have been improved apparently,ED mortality rate still keeps on escalating. The increase in ED mortality rate may be related to the severely ill patients presenting to ED,the obvious decrease in compliance of patients and the prolonged ED stay time.

Objective: To analyze the research status, hotspots and frontier progress of hypertension in children in English and Chinese literature, so as to provide reference for the early prevention of hypertension in children. Methods: The Web of Science core collection database and CNKI database were searched to collect the literature related to the study of hypertension in children from 2000 to 2021, and the CiteSpace 5.8.R3 and VOSviewer 1.6.18 visualization tools were used to analyze the literature characteristics including publications, authors, regions, institutional cooperation, research hotspots and frontiers. Results: A total of 22 687 English studies and 4 440 Chinese studies were finally included. According to the analysis results, the number of articles published on hypertension in children was on the rise. The published English articles were mainly University of Toronto and University of Colorado. The main publishing institution of Chinese articles was the First Affiliated Hospital of Peking University. The United States and China took the lead in the number of core journals published in the field of hypertension in children, the United States ranked first in terms of the influence of publications. Keyword co occurrence analysis showed that the high frequency keywords in the English literature included prevalence, risk, obesity, risk factor, body mass index, insulin resistance, overweight, metabolic syndrome, cardiovascular disease and mortality. Chinese high frequency keywords in the literature include obesity, risk factors, adiposis, influencing factors, overweight, prevalence, diabetes, treatment, health education and body mass index. The analysis of keywords showed that 25 burst terms were obtained separately in English and Chinese literature. Conclusion In recent years, the research interest on hypertension in children continues to grow and keeps updated, with the research scope expanding significantly, regarding obesity, diabetes and cardiovascular diseases.