简介：AbstractTuberous sclerosis complex (TSC) is a rare disease that involves multiple organs, including the brain; approximately 80%-90% of TSC patients exhibit TSC-associated epilepsy. Independent temporal lobe epilepsy (TLE), TSC-unrelated epilepsy, is particularly rare in patients with TSC. Here, we describe three patients with TSC with independent TLEs that were confirmed by stereo-electroencephalography (EEG), postoperative pathological findings, and seizure outcome at follow-up. The patients were retrospectively enrolled at two centers; their ictal epileptiform discharge onsets were determined using electrode contacts in the hippocampus during stereo-EEG. The three patients underwent anterior temporal lobectomies and remained seizure-free at 1-5 years after surgery. Postoperative pathological examinations confirmed hippocampal sclerosis in all three patients. Furthermore, postoperative intelligence quotient improvement was evident in one patient, while the quality of life was improved in two patients at 12 months after surgery.

简介：无

简介：AbstractBackgrounds:Physical activity (PA) and sedentary behavior (SB) have been associated with mortality, while the joint association with mortality is rarely reported among Chinese population. We aimed to examine the independent and joint association of PA and SB with all-cause mortality in southern China.Methods:A cohort of 12,608 China Hypertension Survey participants aged ≥35 years were enrolled in 2013 to 2014, with a followup period of 5.4 years. Baseline self-reported PA and SB were collected via the questionnaire. Kaplan-Meier curves (log-rank test) and Cox proportional hazards regression were performed to evaluate the associations of PA and SB on all-cause mortality.Results:A total of 11,744 eligible participants were included in the analysis. Over an average of 5.4 years of follow-up, 796 deaths occurred. The risk of all-cause mortality was lower among participants with high PA than those with low to moderate level (5.2% vs. 8.9%; hazards ratio [HR]: 0.75, 95% confidence interval [CI]: 0.61-0.87). Participants with SB ≥ 6 h had a higher risk of all-cause mortality than those with SB <6 h (7.8% vs. 6.0%; HR: 1.37, 95% CI: 1.17-1.61). Participants with prolonged SB (≥6 h) and inadequate PA (low to moderate) had a higher risk of all-cause mortality compared to those with SB < 6 h and high PA (11.2% vs. 4.9%; HR: 1.67, 95% CI: 1.35-2.06). Even in the participants with high PA, prolonged SB (≥6 h) was still associated with the higher risk of all-cause mortality compared with SB < 6 h (7.0% vs. 4.9%; HR: 1.33, 95% CI: 1.12-1.56).Conclusions:Among Chinese population, PA and SB have a joint association with the risk of all-cause mortality. Participants with inadequate PA and prolonged SB had the highest risk of all-cause mortality compared with others.

简介：AbstractBackground:The most common etiologies of Cushing's syndrome (CS) are adrenocorticotropic hormone (ACTH)-producing pituitary adenoma (pitCS) and primary adrenal gland disease (adrCS), both of which burden patients with metabolic disturbance. The aim of this study was to compare the metabolic features of pitCS and adrCS patients.Methods:A retrospective review including 114 patients (64 adrCS and 50 pitCS) diagnosed with CS in 2009-2019 was performed. Metabolic factors were then compared between pitCS and adrCS groups.Results:Regarding sex, females suffered both adrCs (92.2%) and pitCS (88.0%) more frequently than males. Regarding age, patients with pitCS were diagnosed at a younger age (35.40 ± 11.94 vs. 39.65 ± 11.37 years, p = 0.056) than those with adrCS, although the difference was not statistically significant. Moreover, pitCS patients had much higher ACTH levels and more serious occurrences of hypercortisolemia at all time points (8 AM, 4 PM, 12 AM) than that in adrCS patients. Conversely, indexes, including body weight, BMI, blood pressure, serum total cholesterol, low density lipoprotein cholesterol (LDL-C), high density lipoprotein cholesterol (HDL-C), triglycerides, fasting plasma glucose, and uric acid, showed no differences between adrCS and pitCS patients. Furthermore, diabetes prevalence was higher in pitCS patients than in adrCS patients; however, there were no significant differences in hypertension or dyslipidemia prevalence between the two.Conclusions:Although adrCS and pitCS had different pathogenetic mechanisms, different severities of hypercortisolemia, and different diabetes prevalences, both etiologies had similar metabolic characteristics.

简介：无

简介：AbstractObjective:This study aimed to determine: (1) whether recurrent deliveries of a small for gestational age (SGA) neonate are associated with increased obstetrical or neonatal complications; (2) whether the risk factors that can predict small for gestational age (SGA) recurrence.Methods:This study was based on Soroka Medical Center' s Obstetrics electronic database. The database consisted of 109 022 women who had 320 932 deliveries between the year 1988-2014.The study cohort included 6.8% (7 368/109 022) of these patients who gave birth to a singleton SGA neonate on their first delivery and had more than one delivery. The study population was divided into two groups according to the outcome of the subsequent delivery: (1) women with sporadic SGA who delivered a non-SGA neonate (n= 5 416); (2) women with recurrent SGA (n = 1 952). SGA defined as birthweight <10th percentile. Maternal and neonatal complications were compared between the two groups. Logistic regression was used to determine independent risk factors for SGA recurrence.Results:The prevalence of birthweight <5th percentile was higher among the recurrent SGA group in the first delivery (P < 0.001). Bedouin ethnicity was more prevalent in the recurrent SGA group (P < 0.001). The rate of preterm delivery was higher in the first delivery of the recurrent SGA group (P = 0.015). The sporadic SGA group had a higher rate of perinatal mortality during the first pregnancy (P= 0.017). The rate of severe hypertension (P= 0.005), polyhydramnios, meconium-stained amniotic fluid, nonreassuring fetal heart rate and total perinatal mortality (P < 0.001) were higher in the second delivery of the recurrent SGA group. In a logistic regression model, preterm delivery and birthweight <5th percentile at the first delivery was found to be independent risk factors for recurrence of an SGA neonate in the subsequent birth (relative risks:1.530, confidence interval: 1.249-1.875; relative risks:1.826, confidence interval: 1.641-2.030, respectively).Conclusion:Women with recurrent SGA neonates have specific clinical characteristics. Among women who deliver an SGA neonate, preterm delivery, and birthweight <5th percentile are independent predictors for its recurrence.

简介：AbstractBackground:Acute heart failure (AHF) is the most common disease in emergency departments (EDs). However, clinical data exploring the outcomes of patients presenting AHF in EDs are limited, especially the long-term outcomes. The purposes of this study were to describe the long-term outcomes of patients with AHF in the EDs and further analyze their prognostic factors.Methods:This prospective, multicenter, cohort study consecutively enrolled 3335 patients with AHF who were admitted to EDs of 14 hospitals from Beijing between January 1, 2011 and September 23, 2012. Kaplan-Meier and Cox regression analysis were adopted to evaluate 5-year outcomes and associated predictors.Results:The 5-year mortality and cardiovascular death rates were 55.4% and 49.6%, respectively. The median overall survival was 34 months. Independent predictors of 5-year mortality were patient age (hazard ratio [HR]: 1.027, 95 confidence interval [CI]: 1.023-1.030), body mass index (BMI) (HR: 0.971, 95% CI: 0.958-0.983), fatigue (HR: 1.127, 95% CI: 1.009-1.258), ascites (HR: 1.190, 95% CI: 1.057-1.340), hepatic jugular reflux (HR: 1.339, 95% CI: 1.140-1.572), New York Heart Association (NYHA) class III to IV (HR: 1.511, 95% CI: 1.291-1.769), heart rate (HR: 1.003, 95% CI: 1.001-1.005), diastolic blood pressure (DBP) (HR: 0.996, 95% CI: 0.993-0.999), blood urea nitrogen (BUN) (HR: 1.014, 95% CI: 1.008-1.020), B-type natriuretic peptide (BNP)/N-terminal pro-B-type natriuretic peptide (NT-proBNP) level in the third (HR: 1.426, 95% CI: 1.220-1.668) or fourth quartile (HR: 1.437, 95% CI: 1.223-1.690), serum sodium (HR: 0.980, 95% CI: 0.972-0.988), serum albumin (HR: 0.981, 95% CI: 0.971-0.992), ischemic heart diseases (HR: 1.195, 95% CI: 1.073-1.331), primary cardiomyopathy (HR: 1.382, 95% CI: 1.183-1.614), diabetes (HR: 1.118, 95% CI: 1.010-1.237), stroke (HR: 1.252, 95% CI: 1.121-1.397), and the use of diuretics (HR: 0.714, 95% CI: 0.626-0.814), β-blockers (HR: 0.673, 95% CI: 0.588-0.769), angiotensin-converting enzyme inhibitors (ACEIs) (HR: 0.714, 95% CI: 0.604-0.845), angiotensin-II receptor blockers (ARBs) (HR: 0.790, 95% CI: 0.646-0.965), spironolactone (HR: 0.814, 95% CI: 0.663-0.999), calcium antagonists (HR: 0.624, 95% CI: 0.531-0.733), nitrates (HR: 0.715, 95% CI: 0.631-0.811), and digoxin (HR: 0.579, 95% CI: 0.465-0.721).Conclusions:The results of our study demonstrate poor 5-year outcomes of patients presenting to EDs with AHF. Age, BMI, fatigue, ascites, hepatic jugular reflux, NYHA class III to IV, heart rate, DBP, BUN, BNP/NT-proBNP level in the third or fourth quartile, serum sodium, serum albumin, ischemic heart diseases, primary cardiomyopathy, diabetes, stroke, and the use of diuretics, β-blockers, ACEIs, ARBs, spironolactone, calcium antagonists, nitrates, and digoxin were independently associated with 5-year all-cause mortality.