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Hepatolenticular degeneration, also known as Wilson disease(WD), is an autosomal recessive disease with copper excretion disorder caused by ATP7B gene mutation.At present, the global incidence of hepatolenticular degeneration is about 1/30 000, but the frequency of ATP7B gene mutation is about 1/90.WD mainly manifests clinically as acute and chronic liver disease, neurological damage and renal impairment, etc.It is a genetic disease that can be well controlled by early diagnosis and treatment, so early diagnosis and treatment are important.However, because of its complex genotype and phenotype, it is easy to be misdiagnosed and delayed treatment.There is no single diagnostic standard for diagnosis of WD at the moment.The Leipzig diagnostic scoring system is often used for diagnosis.In recent years, there has been a new supplement to the diagnostic methods of WD.Some studies have confirmed that measuring the concentration of ATP7B protein by using ATP7B peptide can be used as a new diagnostic method for hepatolenticular degeneration, which is helpful for early accurate diagnosis.
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Objective:To study and analyze the application effect of intervention plan based on unplanned readmission risk model (LACE) in the rehabilitation of patients with acute myocardial infarction (AMI) complicated with cardiac shock (CS) after percutaneous coronary intervention (PCI).Methods:Ninety-three patients with AMI complicated with CS who received PCI in Tianjin Union Medical Center from January 2019 to December 2020 were enrolled. The patients were divided into LACE intervention group ( n = 46) and routine intervention group ( n = 47) according to the different nursing intervention methods. The patients in the routine intervention group received routine interventions, including drug care, diet care, psychological care, health education and telephone follow-up, while those in the LACE intervention group were assessed for the risk of LACE, and then intervention measures were formulated according to the score of LACE index, including strengthening risk awareness, life behavior, daily life ability, self-care ability, health recovery and health needs. The follow-up period in both groups was 3 months. The changes of cardiac function, incidence of adverse cardiac events, readmission rate, self-management ability after intervention and quality of life level before and after intervention were compared between the two groups. Results:There was no significant difference in cardiac function or quality of life before intervention between the two groups. After intervention for 3 months, the cardiac function and quality of life in the two groups were improved as compared with those before intervention. The left ventricular ejection fraction (LVEF) in the LACE intervention group was significantly higher than that in the routine intervention group (0.533±0.076 vs. 0.492±0.072, P < 0.05), the left ventricular end diastolic diameter (LVEDD) was significantly lower than that in the routine intervention group (mm: 47.09±7.01 vs. 53.23±7.15, P < 0.01), and the World Health Organization Quality of Life-brief (WHOQOL-BREF) score was also significantly higher than that in the routine intervention group (66.32±6.19 vs. 55.79±7.26, P < 0.01). The scores of self-management ability in the coronary heart disease self-management scale (CSMS) after intervention in the LACE intervention group were significantly higher than those in the routine intervention group (adverse hobbies score: 17.37±3.24 vs. 14.21±2.73, symptoms score: 14.82±3.11 vs. 10.56±2.65, emotional cognition score: 16.17±2.83 vs. 12.95±2.41, first aid score: 11.85±1.94 vs. 10.62±1.56, disease knowledge score: 15.58±2.73 vs. 12.68±2.61, daily life score: 17.80±2.61 vs. 14.33±2.36, treatment compliance score: 11.47±1.84 vs. 8.56±1.23, all P < 0.01). The incidence of adverse cardiac events and readmission rate in the LACE intervention group were significantly lower than those in the routine intervention group (10.87% vs. 29.79%, 4.35% vs. 17.02%, both P < 0.05). Conclusion:The intervention plan based on LACE risk model can effectively promote postoperative rehabilitation of patients with AMI complicated with CS after PCI, and also help to improve patients' self-management ability and quality of life, which is worthy of clinical promotion and application.
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Objective:To investigate the status and influencing factors of enteral nutrition support in late preterm infants (34-36 +6 gestational weeks) treated in different grades of hospitals in Beijing. Methods:This was a prospective study involving late preterm infants treated in 25 hospitals in Beijing from October 2015 to October 2017. Data about nutritional management and nutrition-related complications were recorded. Exclusive breastfeeding status of the infants by gestational age(GA) and hospital levels was analyzed. The achievement of full enteral feeding and the potential influencing factors were also analyzed. t-test, Mann-Whitney U test, analysis of variance (ANOVA), Kruskal-Wallis test and Chi-square test were used for statistical analysis. Logistic regression and Cox regression analysis were used in multivariate analysis. Results:(1) A total of 1 463 late preterm infants with GA of 35.6±0.8 (ranging from 34.9 to 36.1) weeks was enrolled in this study. Compared with the infants with GA of 35-35 +6 and 36-36 +6 weeks, those born at 34-34 +6 gestational weeks had longer hospital stay [10 (8-13) vs 8 (7-10) and 7 (6-9) d, both P<0.05], greater loss of minimum weight [4.3% (2.6%-6.3%) vs 3.8% (2.0%-5.6%) and 3.3% (1.9%-5.5%), both P<0.05], higher incidence of apnea [5.3% (20/369) vs 2.1% (12/566) and 1.3% (7/528), both P<0.05] and respiratory distress syndrome (RDS) [7.1% (28/369) vs 3.0% (17/566) and 3.2% (17/528), both P<0.05], and lower percentage of failure to regain birth weight at discharge [32.5% (120/369) vs 38.7% (219/566) and 47.9% (253/528), both P<0.05]. Only the incidence of premature rupture of membranes among all maternal complications during pregnancy had statistical difference between 34-34 +6, 35-35 +6 and 36-36 +6 GA groups [6.2% (23/369) vs 12.7% (72/566) and 11.9% (63/528), χ2=10.244, P=0.007]. (2) The rate of enteral feeding increment in hospital was 13.7 (10.5-17.3) ml/(kg·d) and 46.0% (673/1 463) of the infants were fed formula. The exclusive breastfeeding rate increased from 4.5% (66/1 463) during hospitalization to 14.4% (211/1 463) at discharge. The breastfeeding rate at discharge varied widely among the 25 hospitals ( χ2=327.893, P<0.001) ranging from 32% to 0. (3) Logistic regression analysis demonstrated that gestational diabetes mellitus ( OR=2.426, 95% CI: 1.075-5.437, P=0.033) and premature rupture of membranes ( OR=8.726, 95% CI: 1.193-63.802, P=0.033) were the prenatal risk factors influencing the exclusive breastfeeding in late preterm infants. Enteral nutrition achieving 150 ml/(kg·d) and 120 kcal/(kg·d) (1 kcal=4.184 kJ) were noted for 28.4% (416/1 463) and 19.2% (281/1 463) of the late preterm infants at discharge, respectively. Cox regression analysis showed that hospital grades ( HR=1.470, 95% CI: 1.030-2.098, P=0.034), the length of hospital stay ( HR=1.162, 95% CI: 1.097-1.231, P<0.001), birth weight ( HR=0.946, 95% CI: 0.898-0.995, P=0.003), exclusive breastfeeding ( HR=2.354, 95% CI:1.031-5.374, P=0.042), feeding intolerance ( HR=3.677, 95% CI: 1.201-11.253, P=0.023), parenteral nutrition ( HR=1.900, 95% CI: 1.379-2.616, P<0.001), and the rate of enteral feeding advancement ( HR=1.426, 95% CI: 1.369-1.484, P<0.001) were independent factors associated with full enteral feeding at discharge. Conclusions:Exclusive breastfeeding rate in late preterm infants is low and enteral nutrition support varies greatly in different hospitals. The rate of enteral feeding increment is slow for hospitalized late preterm infants and most fail to achieve full enteral feeding at discharge. Gestational diabetes mellitus and premature rupture of membranes are prenatal risk factors affecting breastfeeding of late preterm infants. Those with low birth weight, exclusive breastfeeding in hospital, feeding intolerance, parenteral nutrition support, longer hospital stay or rapid enteral feeding advancement are more likely to achieve full enteral feeding at discharge.
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Objective:To investigate the current nutrition support status of hospitalized small for gestational age infants born late preterm in hospitals of Beijing, and analyze the influencing factors.Methods:Clinical data of late preterm infants from 25 medical units in Beijing between October 2015 and October 2017 was collected and analyzed. Infants were assigned into two groups according to the relationship between their gestational age and birth body weight as small for gestational age(SGA) group and not small for gestational age(non-SGA) group, to compare their nutritional status and explore the related influential factors.Results:Totally, 1 347 late preterm infants were enrolled, including 730 males and 617 females, 151 in SGA group and 1 196 in non-SGA group. The data showed that the rate of exclusive breast-feeding was higher (5.3% vs 4.5%, P<0.01), and the increasing of milk volume was slower [11.0 vs 12.1 ml/(kg·d), P=0.003] in SGA group. More parenteral nutrition was used (77.5% vs 53.1%, P<0.01), and the duration of parenteral nutrition was longer (5.0 vs 2.0 days, P<0.01) in SGA group. The birth weight(1 940 vs 2 490 g, P<0.01), the lowest body weight(1 890 vs 2 400 g, P<0.01) and the discharged body weight(2 135 vs 2 530 g, P<0.01)were lower in SGA group. The SGA group showed lower body weight loss(3.1% vs 8.0%, P=0.015), slower weight growth(13.3 vs 33.0 g/d, P<0.01), and longer length of hospital stay (11.0 vs 8.0 days, P<0.01). In SGA group, the milk volume at discharge [145.6 vs 122.2 ml/(kg·d), P<0.01] and the caloric of enteral feeding at discharge [443.9 vs 384.1 kJ/(kg·d), P<0.01] were higher, the rate of infants who regained their birth weight during hospitalization(78.8% vs 57.9%, P<0.01) was higher, and the rate of ones who achieve full enteral feeding (31.8% vs 16.6%, P<0.01) was higher. A Cox regression analysis in which we set infants can achieve full enteral feeding as goal showed that independent factors associated with full enteral feeding at discharge in SGA group included the increasing of enteral feeding, the duration of parenteral nutrition, whether the length of hospital stay longer than 7 days or not whether exclusive breastfeeding and whether the mothers of enrolled infants were diagnosed gestational diabetes mellitus or placental abruption during pregnancy ( P<0.05). Conclusions:Infants in SGA group show slower increasing of milk volume and lower caloric amount of enteral feeding. More parenteral nutrition is used, and the duration of parenteral nutrition is longer in SGA group. Due to the longer length of hospital stay in SGA group, the milk volume and the caloric of enteral feeding at discharge are higher, more infants regain their birth weight during hospitalization, and more infants achieve full enteral feeding at discharge. Despite of higher portion of parenteral nutrition, infants in SGA group show slower weight growth and lower body weight at discharge.
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Objective@#To analyze the clinical value of serum tumor markers CA19-9, CA242, CEA, CA724 and CA125 in the diagnosis and prognosis of gallbladder carcinoma patients.@*Methods@#A retrospective analysis of the preoperative serum levels of CA19-9, CA242, CEA, CA724 and CA125 in 132 patients with gallbladder cancer admitted to Eastern Hepatobiliary Surgery Hospital from March 2009 to December 2013 for sensitivity comparison, Kaplan-Meier survival table was used for univariate survival analysis, and the log-rank method was compared for differences. The Cox regression model was used for multivariate survival analysis.@*Results@#The sensitivities of CA19-9, CA242, CA125, CEA and CA724 were 67.4%, 63.6%, 42.4%, 24.2% and 22.7%, respectively. There were no significant differences of the sensitivity between CA19-9 and CA242 (P>0.05). However, whether CA19-9 or CA242, there were significant differences of diagnostic sensitivity compared to CEA or CA724 or CA125 (all P<0.05). When CA19-9 was combined with CA125 or CEA or CA724, respectively, the sensitivity was improved, but there was no significant difference compared with CA19-9 alone (all P>0.05). Similarly, CA242 also has such a situation when compared with CA242 alone (all P>0.05). Univariate survival analysis showed there were statistically significant differences in CA19-9, CA242, and CEA (all P<0.05). Cox regression suggested that CA242 is an independent prognostic factor for gallbladder carcinoma. CA242 is closely related to histological grade of gallbladder carcinoma, lymph node metastasis and TNM staging.@*Conclusions@#CA19-9 and CA242 have definite value in the diagnosis of gallbladder carcinoma. CA242 is an independent prognostic factor for gallbladder carcinoma.
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Objective To analyze the clinical value of serum tumor markers CA19-9, CA242, CEA, CA724 and CA125 in the diagnosis and prognosis of gallbladder carcinoma patients. Methods A retrospective analysis of the preoperative serum levels of CA19-9, CA242, CEA, CA724 and CA125 in 132 patients with gallbladder cancer admitted to Eastern Hepatobiliary Surgery Hospital from March 2009 to December 2013 for sensitivity comparison, Kaplan-Meier survival table was used for univariate survival analysis, and the log-rank method was compared for differences. The Cox regression model was used for multivariate survival analysis. Results The sensitivities of CA19-9, CA242, CA125, CEA and CA724 were 67. 4％, 63. 6％, 42. 4％, 24. 2％ and 22. 7％, respectively. There were no significant differences of the sensitivity between CA19-9 and CA242 (P >0. 05). However, whether CA19-9 or CA242, there were significant differences of diagnostic sensitivity compared to CEA or CA724 or CA125 (all P<0. 05). When CA19-9 was combined with CA125 or CEA or CA724, respectively, the sensitivity was improved, but there was no significant difference compared with CA19-9 alone (all P>0. 05). Similarly, CA242 also has such a situa-tion when compared with CA242 alone (all P>0. 05). Univariate survival analysis showed there were statis-tically significant differences in CA19-9, CA242, and CEA (all P<0. 05). Cox regression suggested that CA242 is an independent prognostic factor for gallbladder carcinoma. CA242 is closely related to histological grade of gallbladder carcinoma, lymph node metastasis and TNM staging. Conclusions CA19-9 and CA242 have definite value in the diagnosis of gallbladder carcinoma. CA242 is an independent prognostic factor for gallbladder carcinoma.
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Objective@#To discuss the rationality of stage pT3 in the AJCC 8th TNM criteria of gallbladder carcinoma.@*Methods@#A retrospective study was performed to analyze the clinical and pathological data of 88 patients with pT3 gallbladder carcinoma admitted to Department of Second Biliary Surgery of Eastern Hepatobiliary Surgery Hospital, affiliated to Naval Medical University from May 2013 to September 2018.pT3 stage tumors were divided into two groups: (1) pT3a stage: tumors had penetrated serosa but not directly invaded liver and/or an adjacent organ or structure; (2) pT3b stage: tumor penetrating serosa and directly invaded liver and/or an adjacent organ or structure. There were 45 patients with pT3a stage, including 15 males and 30 females, aged 36 to 80 years, with a median age of 59 years; 43 patients with pT3b, including 24 males and 19 females, aged 41 to 78 years old, median aged 63 years old.Patients with pT3a and pT3b were further divided into two groups respectively: radical resection group and extended radical resection group according to surgical radicalization. Independent sample t-test was used for comparison between two groups with normal distribution measurement data. Wilcoxon rank sum test was used between groups of non-normally distributed measurement data.The comparison of the count data was performed by χ2 test or Fisher exact probability method. Survival analysis was performed using Kaplan-Meier method, and survival rate was compared using Log-rank test.@*Results@#(1)Serum total bilirubin(15.6(90.3)mmol/L), albumin(40.2(4.8)mmol/L), and CA19-9(132.90(455.78)U/ml) levels in pT3b patients were higher than that in pT3a patients(10.2(6.8)mmol/L, 41.8(4.9)mmol/L, 14.35(36.27)U/ml), respectively(Z=-3.816, -1.966, -3.739, all P<0.05),postoperative complication rate in pT3b patients(24.4%) was higher than that in pT3a patients(8.9%)(P<0.05),postoperative hospital stay(12(7)days) and overall hospital stay((26±17)days) of pT3b patients were longer than that of pT3a patients((10±5) days and (19±7)days) (P<0.05). (2) The 1-, 3-, 5-year survival rates of pT3b and pT3a patients were 53%,22%,22% and 69%, 46%,38%,and the median survival time was 13 months and 26 months, respectively. The difference in survival rates between the two groups was statistically significant(χ2=5.117, P=0.024). (3)The 1-, 3-year survival rates of extended radical resection group(n=19) and radical resection group(n=24) in the pT3b stage were 73%, 36% and 28%, 7%, respectively.The survival time was 20 months and 9 months,respectively,and the difference in survival rates between the two groups was statistically significant(χ2=4.976, P=0.026).@*Conclusions@#pT3 gallbladder carcinoma could be further subdivided into pT3a stage and pT3b stage based on the TNM criteria of AJCC 8th gallbladder carcinoma. Extended radical resection for pT3b gallbladder carcinoma should be further considered after comprehensive assessment of the patient′s basic condition and surgical tolerance.
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@#Objective To explore the relationship between the test of drug-induced electroencephalography(EEG) and epilepsy.Methods324 patients with epilepsy were randomly divided into 2 groups, the benadrly group(n=162) and chloral hydrate group(n=162) as control. EEG was examined by evoking during sleep to observe epileptiform discharge and which stage of non-rapid eye movements (NREM) epileptiform discharge occurred more frequently.ResultsThe epileptiform discharge was 31.61% in benadrly group, and 18.52% in chloral hydrate group. There was significant difference between the two groups (P<0.01). The detection rate of epileptiform discharge at the stage I～II of NREM was significantly higher in benadrly group (83.7%) than in chloral hydrate group(63.3%)(P<0.05), which suggested that epileptiform discharge frequently occurred at the stage I～II of NREM.ConclusionBenadrly is superior to chloral hydrate in the test of drug-induced EEG of epilepsy.