scholarly journals Predictors of Survival in a Huntington's Disease Population from Southern Italy

2012 ◽  
Vol 39 (1) ◽  
pp. 48-51 ◽  
Author(s):  
Carlo Rinaldi ◽  
Elena Salvatore ◽  
Ilaria Giordano ◽  
Sara De Matteis ◽  
Tecla Tucci ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Southern Italy ◽  
Cox Regression ◽  
Age Of Onset ◽  
Survival Rates ◽  
Cox Regression Analysis ◽  
Therapeutic Trials ◽  
Expanded Allele ◽  
History Of

Background:The primary aim of the present study was to determine the survival rates and identify predictors of disease duration in a cohort of Huntington's disease (HD) patients from Southern Italy.Methods:All medical records of HD patients followed between 1977 and 2008 at the Department of Neurological Sciences of Federico II University in Naples were retrospectively reviewed and 135 patients were enrolled in the analysis. At the time of data collection, 41 patients were deceased (19 males and 22 females) with a mean ± SD age at death of 56.6 ± 14.9 years (range 18-83).Results:The median survival time was 20 years (95% CI: 18.3-21.7). Cox regression analysis showed that the number of CAG in the expanded allele (HR 1.09 for 1 point triplet increase, p=0.002) and age of onset (HR 1.05 for 1 point year increase, p=0.022) were independent and significant predictors of lower survival rates.Conclusions:We believe that these findings are important for a better understanding of the natural history of the disease and may be relevant in designing future therapeutic trials.

scholarly journals Comparison of the Development and Prognosis in Patients of Hypertriglyceridemic Pancreatitis with and without Diabetes

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Wenjian Liao ◽  
Xiuxiu Niu ◽  
Wei Zhang ◽  
Xiaobing Liu
Keyword(s):  
Cox Regression ◽  
Age Of Onset ◽  
Previous History ◽  
Apache Ii ◽  
Apache Ii Score ◽  
Cox Regression Analysis ◽  
Glucose Levels ◽  
Heart Ischemia ◽  
Blood Glucose Levels ◽  
History Of

Aim. To investigate the clinical features and prognosis in patients of hyperlipidemic acute pancreatitis with or without diabetes. Methods. 157 patients with hypertriglyceridemic pancreatitis (HTGP) were included in this study. Patients with a previous history of diabetes were identified in the group of HTGP with diabetes (HTGPD), while patients without a history of diabetes were identified in the group of HTGP. The clinical characteristics and prognosis data of these patients in the two groups were analyzed. Results. Multivariate Cox regression analysis showed that age, body mass index, glycated serum protein (GSP), and Acute Physiology and Chronic Health Evaluation (APACHE) II score were significantly associated with mortality in patients with HTGP. The mortality was significantly higher in the HTGPD group than in the HTGP group ( p < 0.001 ). Compared to patients of HTGP, those of HTGPD had older age of onset, higher blood glucose levels, and higher GSP levels on admission. Electrocardiograms showed that patients of HTGPD had a significantly higher risk of heart ischemia than those of HTGP ( p < 0.05 ). Patients of HTGPD had higher APACHE II scores than those of HTGP ( p < 0.001 ). Single-factor analysis showed that higher triglyceride levels, GSP, LDL, and previous history of diabetes were associated with HTGP recurrence. Conclusions. Clinicians should be alert to patients of HTGP with diabetes. Diabetes is an important risk factor for HTGP and hyperglycemia may affect the development and prognosis of HTGP.

Age of Onset of Huntington's Disease in Carriers of Reduced Penetrance Alleles

2021 ◽  
Author(s):  
Erin I. McDonnell ◽  
Yuanjia Wang ◽  
Jill Goldman ◽  
Karen Marder
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Age Of Onset ◽  
Reduced Penetrance

684 Cardiac troponins and adverse outcomes in European patients with atrial fibrillation: a report from the ESC-EHRA EORP atrial fibrillation general long-term registry

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Marrco Vitolo ◽  
Vincenzo Livio Malavasi ◽  
Marco Proietti ◽  
Igor Diemberger ◽  
Laurent Fauchier ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Regression Analysis ◽  
Cox Regression ◽  
Adverse Outcomes ◽  
Cox Regression Analysis ◽  
Coronary Syndrome ◽  
Increased Risk ◽  
History Of ◽  
Cardiac Troponins

Abstract Aims Cardiac troponins (cTn) have been reported to be predictors for adverse outcomes in atrial fibrillation (AF), patients, but their actual use is still unclear. To assess the factors associated with cTn testing in routine clinical practice and to evaluate the association of elevated levels of cTn with adverse outcomes in a large contemporary cohort of European AF patients. Methods and results Patients enrolled in the ESC-EHRA EORP-AF General Long-Term Registry were stratified into three groups according to cTn levels as (i) cTn not tested, (ii) cTn in range (≤99th percentile), and (iii) cTn elevated (&gt;99th percentile). The composite outcome of any thromboembolism/any acute coronary syndrome (ACS)/cardiovascular (CV) death, defined as major adverse cardiovascular events (MACE) and all-cause death were the main endpoints. 10 445 (94.1%) AF patients were included in this analysis [median age 71 years, interquartile range (IQR): 63–77; males 59.7%]. cTn were tested in 2834 (27.1%). Overall, cTn was elevated in 904 (8.7%) and in-range in 1930 (18.5%) patients. Patients in whom cTn was tested tended to be younger (P &lt; 0.001) and more frequently presenting with first detected AF and atypical AF-related symptoms (i.e. chest pain, dyspnoea, or syncope) (P &lt; 0.001). On multivariable logistic regression analysis, female sex, in-hospital enrollment, first-detected AF, CV risk factors, history of coronary artery disease (CAD), and atypical AF symptoms were independently associated with cTn testing. After a median follow-up of 730 days (IQR: 692–749), 957 (9.7%) composite endpoints occurred while all-cause death was 9.5%. Kaplan–Meier analysis showed a higher cumulative risk for both outcomes in patients with elevated cTn levels (Figure) (Log Rank tests, P &lt; 0.001). On adjusted Cox regression analysis, elevated levels of cTn were independently associated with a higher risk for MACE [hazard ratio (HR): 1.74, 95% confidence interval (CI): 1.40–2.16] and all-cause death (HR 1.45, 95% CI: 1.21–1.74). Elevated levels of cTn were independently associated with a higher occurrence of MACE, all-cause death, any ACS, CV death and hospital readmission even after the exclusion of patients with history of CAD, diagnosis of ACS at discharge, those who underwent coronary revascularization during the admission and/or who were treated with oral anticoagulants plus antiplatelet therapy. Conclusions Elevated cTn levels were independently associated with an increased risk of all-cause mortality and adverse CV events, even after exclusion of CAD patients. Clinical factors that might enhance the need to rule out CAD were associated with cTn testing.

Natural history definition and a suggested clinical approach to Buerger's disease: a case-control study with survival analysis

Vascular ◽  
2012 ◽  
Vol 20 (4) ◽  
pp. 198-202 ◽  
Author(s):  
Bahare Fazeli ◽  
Hassan Ravari ◽  
Reza Assadi
Keyword(s):  
Multivariate Analysis ◽  
Natural History ◽  
Cox Regression ◽  
Major Amputation ◽  
Clinical Approach ◽  
Buerger’S Disease ◽  
Buerger's Disease ◽  
Cox Regression Analysis ◽  
Kaplan Meier ◽  
History Of

The aim of this study was first to describe the natural history of Buerger's disease (BD) and then to discuss a clinical approach to this disease based on multivariate analysis. One hundred eight patients who corresponded with Shionoya's criteria were selected from 2000 to 2007 for this study. Major amputation was considered the ultimate adverse event. Survival analyses were performed by Kaplan–Meier curves. Independent variables including gender, duration of smoking, number of cigarettes smoked per day, minor amputation events and type of treatments, were determined by multivariate Cox regression analysis. The recorded data demonstrated that BD may present in four forms, including relapsing-remitting (75%), secondary progressive (4.6%), primary progressive (14.2%) and benign BD (6.2%). Most of the amputations occurred due to relapses within the six years after diagnosis of BD. In multivariate analysis, duration of smoking of more than 20 years had a significant relationship with further major amputation among patients with BD. Smoking cessation programs with experienced psychotherapists are strongly recommended for those areas in which Buerger's disease is common. Patients who have smoked for more than 20 years should be encouraged to quit smoking, but should also be recommended for more advanced treatment for limb salvage.

“organic Personality Disorder” as Part of Early Neuropsychiatric Manifestations in Huntington’s Disease - a Case Report

2009 ◽  
Vol 24 (S1) ◽  
pp. 1-1
Author(s):  
J. Maia
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Psychiatric Symptoms ◽  
Age Of Onset ◽  
Neuropsychiatric Symptoms ◽  
Personality Change ◽  
Psychotic Symptoms ◽  
Autosomal Dominant Disorder ◽  
Neuropsychiatric Manifestations ◽  
Psychiatric Manifestations

Huntington's Disease (HD) is an inherited autosomal dominant disorder characterized by motor, cognitive and psychiatric symptomatology, being considered a paradigmatic neuropsychiatric disorder that includes all three components of the "Triadic Syndromes": dyskinesia, dementia and depression.Firstly described in 1872 as an "Hereditary Chorea" by George Huntington only in 1993 was its responsible gene identified. A person who inherits the HD gene will sooner or later develop the disease. the age of onset, early signs and rate of disease progression vary greatly from person to person.Neuropsychiatric symptoms are an integral part of HD and have been considered the earliest markers of the disease, presenting sometimes more than 10 years before a formal diagnosis is done. Patients may experience dysphoria, mood swings, agitation, irritability, hostile outbursts, psychotic symptoms and deep bouts of depression with suicidal ideation. Personality change is reported in 48% of the cases, with the paranoid subtype being described as the most prevalent. the clinical case presented illustrates a case of HD which started with insidious psychiatric symptoms and an important personality change.Despite a wide number of medications being prescribed to help control emotional, movement and behaviour problems, there is still no treatment to stop or reverse the course of the disease. Furthermore, psychiatric manifestations are often amenable to treatment, and relief of these symptoms may provide significant improvement in patient's and caregivers quality of life.A greater awarness of psychiatric manifestations of HD is essential to an earlier diagnosis and an optimized therapeutic approach.

scholarly journals Identification and validation of immunotypes for clear cell RCC prognosis

2021 ◽  
Author(s):  
Zhaolin Yang ◽  
Jiale Zhou ◽  
Yizheng Xue ◽  
Yu Zhang ◽  
Kaijun Zhou ◽  
...  
Keyword(s):  
Overall Survival ◽  
Cox Regression ◽  
Clear Cell ◽  
Survival Rates ◽  
Prognostic Index ◽  
Gene Expression Omnibus ◽  
The Cancer Genome Atlas ◽  
Cox Regression Analysis ◽  
Immune Genes ◽  
Metastasis Model

Abstract Purpose To develop an immunotype-based prognostic model for predicting the overall survival (OS) of patients with clear cell renal carcinoma (ccRCC). We explored novel immunotypes of patients with ccRCC, particularly those associated with overall survival. A risk-metastasis model was constructed by integrating the immunotypes with immune genes and used to test the accuracy of the immunotype model. Patients and Methods Patient cohort data were obtained from The Cancer Genome Atlas (TCGA) database, Gene Expression Omnibus (GEO) database, Renji database, and Surveillance, Epidemiology, and End Results (SEER) database. We employed the R software to select 3 immune cells and construct an immunotype-based prediction model. Immune genes selected using random Forest Algorithm were validated by immunohistochemistry (IHC). The H&L risk-metastasis model was constructed to assess the accuracy of the immunotype model through Multivariate COX regression analysis. Result Patients with ccRCC were categorized into immunotype H subgroup and immunotype L subgroup based on the overall survival rates. The immunotypes were found to be the independent prognostic index for ccRCC prognosis. As such, we constructed a new immunotypes-based SSIGN model. Three immune genes associated with difference between immunotype H and L were identified. An H&L risk-metastasis model was constructed to evaluate the accuracy of the immunotype model. Compared to the W-Risk-metastasis model which did not incorporate immunotypes, the H&L risk-metastasis model was more precise in predicting the survival of ccRCC patients. Conclusion The established immunotype model can effectively predict the survival of ccRCC patients. Except for mast cells, T cells and macrophages are positively associated with the overall survival of patients. The three immune genes identified, herein, can predict the survival rate of ccRCC patients, and expression of these immune genes is strongly linked to poor survival. The new SSIGN model provides an accurate tool for predicting the survival of ccRCC patients. H&L risk-metastasis model can effectively predict the risk of tumor metastasis.

scholarly journals Surgical Resection Significantly Promotes the Overall Survival of Patients with Hepatocellular Carcinoma: A Propensity Score Matching Analysis

2021 ◽  
Author(s):  
Pei-Min Hsieh ◽  
Hung-Yu Lin ◽  
Chao-Ming Hung ◽  
Gin-Ho Lo ◽  
I-Cheng Lu ◽  
...  
Keyword(s):  
Risk Factors ◽  
Hepatocellular Carcinoma ◽  
Overall Survival ◽  
Propensity Score ◽  
Propensity Score Matching ◽  
Surgical Resection ◽  
Cox Regression ◽  
Survival Rates ◽  
Hbv Infection ◽  
Cox Regression Analysis

Abstract Background: The benefits of surgical resection (SR) for various Barcelona Clinic Liver Cancer (BCLC) stages of hepatocellular carcinoma (HCC) remain unclear. We investigated the risk factors of overall survival (OS) and survival benefits of SR over nonsurgical treatments in patients with HCC of various BCLC stages.Methods: Overall, 2316 HCC patients were included, and their clinicopathological data and OS were recorded. OS was analyzed by the Kaplan-Meier method and Cox regression analysis. Propensity score matching (PSM) analysis was performed.Results: In total, 66 (2.8%), 865 (37.4%), 575 (24.8%) and 870 (35.0%) patients had BCLC stage 0, A, B, and C disease, respectively. Furthermore, 1302 (56.2%) of all patients, and 37 (56.9%), 472 (54.6%), 313 (54.4%) and 480 (59.3%) of patients with BCLC stage 0, A, B, and C disease, respectively, died. The median follow-up duration time was 20 (range 0-96) months for the total cohort and was subdivided into 52 (8-96), 32 (1-96), 19 (0-84), and 12 (0-79) months for BCLC stages 0, A, B, and C cohorts, respectively. The risk factors for OS were 1) SR and cirrhosis; 2) SR, cirrhosis, and Child-Pugh (C-P) class; 3) SR, hepatitis B virus (HBV) infection, and C-P class; and 4) SR, HBV infection, and C-P class for the BCLC stage 0, A, B, and C cohorts, respectively. Compared to non-SR treatment, SR resulted in significantly higher survival rates in all cohorts. The 5-year OS rates for SR vs non-SR were 44.0% vs 28.7%, 72.2% vs 42.6%, 42.6% vs 36.2, 44.6% vs 23.5%, and 41.4% vs 15.3% (all p-values<0.05) in the total and BCLC stage 0, A, B, and C cohorts, respectively. After PSM, SR resulted in significantly higher survival rates compared to non-SR treatment in various BCLC stages.Conclusion: SR conferred significant survival benefits to patients with HCC of various BCLC stages and should be considered a recommended treatment for select HCC patients, especially patients with BCLC stage B and C disease.

scholarly journals A Novel Six-mRNA Signature Predicts Survival of Patients With Glioblastoma Multiforme

2021 ◽  
Vol 12 ◽  
Author(s):  
Zhentao Liu ◽  
Hao Zhang ◽  
Hongkang Hu ◽  
Zheng Cai ◽  
Chengyin Lu ◽  
...  
Keyword(s):  
Glioblastoma Multiforme ◽  
Cox Regression ◽  
Methylation Status ◽  
Survival Rates ◽  
Gene Expression Omnibus ◽  
The Cancer Genome Atlas ◽  
Survival Prediction ◽  
Lasso Regression ◽  
Cox Regression Analysis ◽  
Score Model

Glioblastoma multiforme (GBM) is a devastating brain tumor and displays divergent clinical outcomes due to its high degree of heterogeneity. Reliable prognostic biomarkers are urgently needed for improving risk stratification and survival prediction. In this study, we analyzed genome-wide mRNA profiles in GBM patients derived from The Cancer Genome Atlas (TCGA) and Gene Expression Omnibus (GEO) databases to identify mRNA-based signatures for GBM prognosis with survival analysis. Univariate Cox regression model was used to evaluate the relationship between the expression of mRNA and the prognosis of patients with GBM. We established a risk score model that consisted of six mRNA (AACS, STEAP1, STEAP2, G6PC3, FKBP9, and LOXL1) by the LASSO regression method. The six-mRNA signature could divide patients into a high-risk and a low-risk group with significantly different survival rates in training and test sets. Multivariate Cox regression analysis confirmed that it was an independent prognostic factor in GBM patients, and it has a superior predictive power as compared with age, IDH mutation status, MGMT, and G-CIMP methylation status. By combining this signature and clinical risk factors, a nomogram can be established to predict 1-, 2-, and 3-year OS in GBM patients with relatively high accuracy.

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