scholarly journals Predictors of mortality in patients with hereditary hemorrhagic telangiectasia

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
K. P. Thompson ◽  
◽  
J. Nelson ◽  
H. Kim ◽  
L. Pawlikowska ◽  
...  

Abstract Background Retrospective questionnaire and healthcare administrative data suggest reduced life expectancy in untreated hereditary hemorrhagic telangiectasia (HHT). Prospective data suggests similar mortality, to the general population, in Denmark’s centre-treated HHT patients. However, clinical phenotypes vary widely in HHT, likely affecting mortality. We aimed to measure predictors of mortality among centre-treated HHT patients. HHT patients were recruited at 14 HHT centres of the Brain Vascular Malformation Consortium (BVMC) since 2010 and followed annually. Vital status, organ vascular malformations (VMs) and clinical symptoms data were collected at baseline and during follow-up (N = 1286). We tested whether organ VMs, HHT symptoms and HHT genes were associated with increased mortality using Cox regression analysis, adjusting for patient age, sex, and smoking status. Results 59 deaths occurred over average follow-up time of 3.4 years (max 8.6 years). A history of anemia was associated with increased mortality (HR = 2.93, 95% CI 1.37–6.26, p = 0.006), as were gastro-intestinal (GI) bleeding (HR = 2.63, 95% CI 1.46–4.74, p = 0.001), and symptomatic liver VMs (HR = 2.10, 95% CI 1.15–3.84, p = 0.015). Brain VMs and pulmonary arteriovenous malformations (AVMs) were not associated with mortality (p > 0.05). Patients with SMAD4 mutation had significantly higher mortality (HR = 18.36, 95% CI 5.60–60.20, p < 0.001) compared to patients with ACVRL1 or ENG mutation, but this estimate is imprecise given the rarity of SMAD4 patients (n = 33, 4 deaths). Conclusions Chronic GI bleeding, anemia and symptomatic liver VMs are associated with increased mortality in HHT patients, independent of age, and in keeping with the limited treatment options for these aspects of HHT. Conversely, mortality does not appear to be associated with pulmonary AVMs or brain VMs, for which patients are routinely screened and treated preventatively at HHT Centres. This demonstrates the need for development of new therapies to treat chronic anemia, GI bleeding, and symptomatic liver VMs in order to reduce mortality among HHT patients.

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
K. P. Thompson ◽  
J. Nelson ◽  
H. Kim ◽  
S. M. Weinsheimer ◽  
D. A. Marchuk ◽  
...  

Abstract Background Approximately 10% of hereditary hemorrhagic telangiectasia (HHT) patients harbour brain vascular malformations (VMs). Intracranial hemorrhage (ICH) from brain VMs can lead to death or morbidity, while treatment options for brain VMs also have associated morbidity. The modified Rankin Scale (mRS) may provide an approach to identifying HHT-brain VM patients with poor outcomes, and their predictors. We aimed to measure the relationship between mRS score and brain VM, brain VM number, as well as other aspects of HHT, at enrollment and during prospective follow-up. Methods 1637 HHT patients (342 with brain VMs) were recruited from 14 HHT centres of the Brain Vascular Malformation Consortium since 2010 and followed prospectively (mean = 3.4 years). We tested whether the presence of brain VM, other HHT organ involvement, and HHT mutation genotype were associated with worse mRS scores at baseline and during follow-up, using linear mixed models, adjusting for age, sex, and year of visit. Results Presence of brain VMs was not associated with worse mRS score at baseline and there was no significant worsening of mRS with prospective follow-up in these patients; 92% had baseline mRS of 0–2. HHT-related gastrointestinal (GI) bleeding was associated with worse mRS scores at baseline (0.37, 95% CI 0.26–0.47, p < 0.001), as were history of anemia (0.35, 95% CI 0.27–0.43, p < 0.001) and liver VMs (0.19, 95% CI 0.09–0.30, p < 0.001). Presence of pulmonary arteriovenous malformations (AVMs) was not associated with worse mRS scores at baseline. mRS score was not associated with either HHT genotype (Endoglin vs ACVRL1). Only GI bleeding was associated with a significantly worsening mRS during prospective follow-up (0.64, 95% CI 0.21–1.08, p = 0.004). Conclusion Most HHT-brain VM patients had good functional capacity (mRS scores 0–2) at baseline that did not change significantly over 3.4 mean years of follow-up, suggesting that mRS may not be useful for predicting or measuring outcomes in these patients. However, HHT patients with GI bleeding, anemia history or liver VMs had worse mRS scores, suggesting significant impact of these manifestations on functional capacity. Our study demonstrates the insensitivity of the mRS as an outcomes measure in HHT brain VM patients and reinforces the continued need to develop outcomes measures, and their predictors, in this group.


2020 ◽  
Vol 44 (10) ◽  
pp. 3564-3572
Author(s):  
Louise B. D. Banning ◽  
Linda Visser ◽  
Clark J. Zeebregts ◽  
Barbara L. van Leeuwen ◽  
Mostafa el Moumni ◽  
...  

Abstract Background Frailty in the vascular surgical ward is common and predicts poor surgical outcomes. The aim of this study was to analyze transitions in frailty state in elderly patients after vascular surgery and to evaluate influence of patient characteristics on this transition. Methods Between 2014 and 2018, 310 patients, ≥65 years and scheduled for elective vascular surgery, were included in this cohort study. Transition in frailty state between preoperative and follow-up measurement was determined using the Groningen Frailty Indicator (GFI), a validated tool to measure frailty in vascular surgery patients. Frailty is defined as a GFI score ≥4. Patient characteristics leading to a transition in frailty state were analyzed using multivariable Cox regression analysis. Results Mean age was 72.7 ± 5.2 years, and 74.5% were male. Mean follow-up time was 22.7 ± 9.5 months. At baseline measurement, 79 patients (25.5%) were considered frail. In total, 64 non-frail patients (20.6%) shifted to frail and 29 frail patients (9.4%) to non-frail. Frail patients with a high Charlson Comorbidity Index (HR = 0.329 (CI: 0.133–0.812), p = 0.016) and that underwent a major vascular intervention (HR = 0.365 (CI: 0.154–0.865), p = 0.022) had a significantly higher risk to remain frail after the intervention. Conclusions The results of this study, showing that after vascular surgery almost 21% of the non-frail patients become frail, may lead to a more effective shared decision-making process when considering treatment options, by providing more insight in the postoperative frailty course of patients.


Author(s):  
Charmaine Chan ◽  
Kristyn Kamke ◽  
Freda Assuah ◽  
Sherine El-Toukhy

Abstract Although text-messaging interventions are effective for smoking cessation, few target teens in the USA and little is known about their effectiveness. The purpose of this manuscript is to examine correlates of dropout, response to smoking status prompts, and abstinence rates among subscribers of SmokeFreeTeen, a free, publicly available text-messaging smoking cessation intervention sponsored by the National Cancer Institute’s SmokeFree.Gov initiative, on quit day through 1 month follow-up. In a sample of teens (N = 2,685), aged 13–19, we examined demographics, smoking frequency, cigarettes smoked per day, prequit intervention time (i.e., maximum of 14 days of prequit day preparation), and number of quit attempts as correlates of response and abstinence rates among program initiators (i.e., participants who reached quit day but dropped out on or before intervention end) and completers (i.e., participants who reached quit day and completed the intervention). We also conducted Cox regression analysis of time from quit day to dropout by daily and nondaily smoking status. Two-thirds (n = 1,733, 64.54%) dropped out before the intervention ended, with dropout rates peaking on quit day (n = 289, 13.10%). Response rate to smoking status prompts remained below 30% throughout the intervention. At intervention end and 1 month follow-up, abstinence was 2.63% and 2.55% among program initiators, whereas abstinence was 6.09% and 6.01% among program completers. Dropout, response, and abstinence rates did not consistently differ by subscriber characteristics. Prequit time was associated with decreased likelihood of dropping out (adjusted hazards ratio: 0.94, confidence interval [CI]: 0.93–0.95), responding to smoking status prompts (adjusted odds ratio [aOR]: 0.94, CI: 0.92–0.96), and being abstinent (aOR: 0.96, CI: 0.93–0.99) on quit day. Two or more quit attempts were associated with increased response (aOR: 1.61, CI: 1.16–2.23) and abstinence (aOR: 1.91, CI: 1.25–2.92) rates on Day 7. In a first assessment of SmokeFreeTeen outcomes, we document high dropouts and low response and abstinence rates. SmokeFreeTeen produced abstinence rates lower than comparable text-messaging interventions targeting teens and young adults. Improving SmokeFreeTeen’s reach, engagement, and effectiveness is needed.


2020 ◽  
Vol 25 (5) ◽  
pp. 427-435
Author(s):  
Damianos G Kokkinidis ◽  
Stefanos Giannopoulos ◽  
Moosa Haider ◽  
Timothy Jordan ◽  
Anita Sarkar ◽  
...  

The association between active smoking and wound healing in critical limb ischemia (CLI) is unknown. Our objective was to examine in a retrospective cohort study whether active smoking is associated with higher incomplete wound healing rates in patients with CLI undergoing endovascular interventions. Smoking status was assessed at the time of the intervention, comparing active to no active smoking, and also during follow-up visits at 6 and 9 months. Cox regression analysis was conducted to compare the incomplete wound healing rates of the two groups during follow-up. A total of 264 patients (active smokers: n = 41) were included. Active smoking was associated with higher rates of incomplete wound healing in the 6-month univariate Cox regression analysis (hazard ratio (HR) for incomplete wound healing: 4.54; 95% CI: 1.41–14.28; p = 0.012). The 6-month Kaplan–Meier (KM) estimates for incomplete wound healing were 91.1% for the active smoking group versus 66% for the non-current smoking group. Active smoking was also associated with higher rates of incomplete wound healing in the 9-month univariable (HR for incomplete wound healing: 2.32; 95% CI: 1.11–4.76; p = 0.026) and multivariable analysis (HR for incomplete wound healing: 9.09; 95% CI: 1.06–100.0; p = 0.044). The 9-month KM estimates for incomplete wound healing were 75% in the active smoking group versus 54% in the non-active smoking group. In conclusion, active smoking status at the time of intervention in patients with CLI is associated with higher rates of incomplete wound healing during both 6- and 9-month follow-up.


Diabetologia ◽  
2021 ◽  
Author(s):  
Laura J. Reid ◽  
Fraser W. Gibb ◽  
Helen Colhoun ◽  
Sarah H. Wild ◽  
Mark W. J. Strachan ◽  
...  

Abstract Aims/hypothesis We aimed to compare diabetic retinopathy outcomes in people with type 1 diabetes following introduction of continuous subcutaneous insulin infusion (CSII) therapy with outcomes in people receiving continuing therapy with multiple daily insulin injections (MDI). Methods This is a retrospective cohort study using the Scottish Care Information – Diabetes database for retinal screening outcomes and HbA1c changes in 204 adults commenced on CSII therapy between 2013 and 2016, and 211 adults eligible for CSII during the same period but who continued on MDI therapy. Diabetic retinopathy progression (time to minimum one-grade worsening in diabetic retinopathy from baseline grading) was plotted for CSII and MDI cohorts using Kaplan–Meier curves, and outcomes were compared using multivariate Cox regression analysis adjusting for age, sex, baseline HbA1c, blood pressure, cholesterol, smoking status and socioeconomic quintile. Impact of baseline HbA1c and change in HbA1c on diabetic retinopathy progression was assessed within CSII and MDI cohorts. Results CSII participants were significantly younger, were from less socially deprived areas, and had lower HbA1c and higher diastolic BP at baseline. There was a larger reduction in HbA1c at 1 year in those on CSII vs MDI (−6 mmol/mol [−0.6%] vs −2 mmol/mol [−0.2%], p < 0.01). Diabetic retinopathy progression occurred in a smaller proportion of adults following commencement of CSII vs continued MDI therapy over mean 2.3 year follow-up (26.5% vs 18.6%, p = 0.0097). High baseline HbA1c (75 mmol/mol [9%]) was associated with diabetic retinopathy progression in the MDI group (p = 0.0049) but not the CSII group (p = 0.93). Change in HbA1c at follow-up, irrespective of baseline glycaemic status, did not significantly affect diabetic retinopathy progression in either group. Conclusions/interpretation CSII was associated with reduced diabetic retinopathy progression compared with continued MDI therapy, and may be protective against diabetic retinopathy progression for those with high baseline HbA1c. Progression of diabetic retinopathy over 3 years was not associated with a change in HbA1c. Graphical abstract


2021 ◽  
Vol 12 ◽  
Author(s):  
Xianqiu Chen ◽  
Jian Guo ◽  
Dong Yu ◽  
Bing Jie ◽  
Ying Zhou

Background: Progressive fibrosing interstitial lung disease (PF-ILD) and idiopathic pulmonary fibrosis (IPF) share similar progression phenotype but with different pathophysiological mechanism. The purpose of this study was to assess clinical characteristics and outcomes of patients with PF-ILD in a single-center cohort.Methods: Patients with PF-ILD treated in Shanghai Pulmonary Hospital from Jan. 2013 to Dec. 2014 were retrospectively analyzed. Baseline characteristics and clinical outcomes were collected for survival analysis to identifying clinical predictors of mortality.Results: Among 608 patients with ILD, 132 patients met the diagnostic criteria for PF-ILD. In this single-center cohort, there were 51 (38.6%) cases with connective tissue disease-associated interstitial lung disease (CTD-ILD) and 45 (34.1%) with unclassifiable ILDs. During follow-up, 83 patients (62.9%) either died (N = 79, 59.8%) or underwent lung transplantations (N = 4, 3.0%) with a median duration follow-up time of 53.7 months. Kaplan-Meier survival curves revealed that the 1, 3 and 5-years survival of PF-ILD were 90.9, 58.8 and 48.1%, respectively. In addition, the prognosis of patients with PF-ILD was similar to those with IPF, while it was worse than non-PF-ILD ones. Multivariate Cox regression analysis demonstrated that high-resolution computed tomography (HRCT) scores (HR 1.684, 95% CI 1.017–2.788, p = 0.043) and systolic pulmonary artery pressure (SPAP) &gt; 36.5 mmHg (HR 3.619, 95%CI 1.170–11.194, p = 0.026) were independent risk factors for the mortality of PF-ILD.Conclusion: Extent of fibrotic changes on HRCT and pulmonary hypertension were predictors of mortality in patients with PF-ILD.


2021 ◽  
pp. 1-8
Author(s):  
Nina Vodošek Hojs ◽  
Robert Ekart ◽  
Sebastjan Bevc ◽  
Nejc Piko ◽  
Radovan Hojs

<b><i>Introduction:</i></b> Chronic kidney disease (CKD) is a risk factor for cardiovascular and all-cause mortality. Recognition of high-risk patients is important and could lead to a different approach and better treatment. The CHA<sub>2</sub>DS<sub>2</sub>-VASc score was originally used to predict cerebral infarction in patients with atrial fibrillation (AF), but it is also a useful predictor of outcome in other cardiovascular conditions, independent of AF. Therefore, the aim of our research was to assess the role of CHA<sub>2</sub>DS<sub>2</sub>-VASc score in predicting cardiovascular and all-cause mortality in CKD patients. <b><i>Methods:</i></b> Stable nondialysis CKD patients were included. At the time of inclusion, medical history data and standard blood results were collected and CHA<sub>2</sub>DS<sub>2</sub>-VASc score was calculated. Patients were followed till the same end date, until kidney transplantation or until their death. <b><i>Results:</i></b> Eighty-seven CKD patients were included (60.3 ± 12.8 years, 66% male). Mean follow-up time was 1,696.5 ± 564.6 days. During the follow-up, 21 patients died and 11 because of cardiovascular reasons. Univariate Cox regression analysis showed that CHA<sub>2</sub>DS<sub>2</sub>-VASc score is a significant predictor of cardiovascular and all-cause mortality. In multivariate Cox regression analysis, in which CHA<sub>2</sub>DS<sub>2</sub>-VASc score, serum creatinine, urinary albumin/creatinine, hemoglobin, high-sensitivity C-reactive protein, and intact parathyroid hormone were included, CHA<sub>2</sub>DS<sub>2</sub>-VASc score was an independent predictor of cardiovascular (HR: 2.04, CI: 1.20–3.45, <i>p</i> = 0.008) and all-cause mortality (HR: 2.06, CI: 1.43–2.97, <i>p</i> = 0.001). The same was true after adding total cholesterol, triglycerides, and smoking status to both the analyses. <b><i>Conclusion:</i></b> The CHA<sub>2</sub>DS<sub>2</sub>-VASc score is a simple, practical, and quick way to identify the risk for cardiovascular and all-cause mortality in CKD patients.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 389.1-389
Author(s):  
M. Breda ◽  
M. G. Lazzaroni ◽  
F. Franceschini ◽  
P. Airò

Background:Systemic Sclerosis (SSc) is characterized by increased mortality and organ damage accrual. A composite SSc Damage Index was recently developed by the Scleroderma Clinical Trials Consortium (SCTC-DI) and was demonstrated as a predictor of mortality both in the Australian derivation cohort and in the Canadian validation cohort (1). Several independent predictors of mortality in SSc have been reported, but only limited data are available on the role of smoking.Objectives:To evaluate smoking history and SCTC-DI as independent predictors of mortality in SSc in a single centre Italian cohort.Methods:A retrospective analysis was performed on patients prospectively followed in our centre from 1989 to 2019, with at least 2 evaluations and/or cause of death available. Organ damage was evaluated through the SCTC-DI (0-55 scale; severe damage>12), while comorbidities through the Charlson Comorbidity Index (CCI). Survival analysis was performed with Kaplan-Meier curves and with Log-rank test to compare different subsets. Cox-regression analysis was performed to identify baseline independent predictors of mortality.Results:648 SSc patients were 99% Caucasian, 90% female and had a median age at diagnosis of 55.5 years (IQR: 45.0-65.6); 19% had diffuse cutaneous involvement. ACA was positive in 52%; anti-TopoI in 22% and anti-RNA Polymerase III in 5%.Median SCTC-DI at diagnosis was 2 (0-4) (n=560); ever smokers were 27%. During the follow-up 240 patients died after a median period of 10.2 years (4.9-16.8). The cause of death was related to SSc in 41% (n=65; most frequent causes pulmonary arterial hypertension (n=35) and interstitial lung disease (n=30)) and to other diseases in 40% (n=95; most frequent cause cancer (n=40)), while was indeterminate in 19%.Overall survival at 5, 10, 15, 20 years was 87.8% (SE 1.3%), 75.6% (1.9), 63.8% (2.3), 47.7% (2.8), respectively and was higher in females vs. males (p<0.0001) and in ACA+ vs ACA- (p=0.005), but did not differ between cutaneous subsets. 111 patients were lost at follow-up (17%).In the Cox-regression analysis smoking history and severe organ damage at diagnosis (score>12) were identified as independent predictors of death, while age at diagnosis, female gender, diffuse cutaneous subsets, ACA positivity and CCI were not (Table 1).Conclusion:Smoking history and severe organ damage were independent predictors of death in a large Italian single centre SSc cohort. SCTC-DI was confirmed as a useful tool to predict mortality at every timepointReferences:[1]Ferdowsi N, et al. Ann Rheum Dis. 2019;78:807-16.Disclosure of Interests:None declared


2015 ◽  
Vol 24 (3) ◽  
pp. 287-292 ◽  
Author(s):  
Petra A. Golovics ◽  
Laszlo Lakatos ◽  
Michael D. Mandel ◽  
Barbara D. Lovasz ◽  
Zsuzsanna Vegh ◽  
...  

Background & Aims: Limited data are available on the hospitalization rates in population-based studies. Since this is a very important outcome measure, the aim of this study was to analyze prospectively if early hospitalization is associated with the later disease course as well as to determine the prevalence and predictors of hospitalization and re-hospitalization in the population-based ulcerative colitis (UC) inception cohort in the Veszprem province database between 2000 and 2012. Methods: Data of 347 incident UC patients diagnosed between January 1, 2000 and December 31, 2010 were analyzed (M/F: 200/147, median age at diagnosis: 36, IQR: 26-50 years, follow-up duration: 7, IQR 4-10 years). Both in- and outpatient records were collected and comprehensively reviewed. Results: Probabilities of first UC-related hospitalization were 28.6%, 53.7% and 66.2% and of first re-hospitalization were 23.7%, 55.8% and 74.6% after 1-, 5- and 10- years of follow-up, respectively. Main UC-related causes for first hospitalization were diagnostic procedures (26.7%), disease activity (22.4%) or UC-related surgery (4.8%), but a significant percentage was unrelated to IBD (44.8%). In Kaplan-Meier and Cox-regression analysis disease extent at diagnosis (HR extensive: 1.79, p=0.02) or at last follow-up (HR: 1.56, p=0.001), need for steroids (HR: 1.98, p<0.001), azathioprine (HR: 1.55, p=0.038) and anti-TNF (HR: 2.28, p<0.001) were associated with the risk of UC-related hospitalization. Early hospitalization was not associated with a specific disease phenotype or outcome; however, 46.2% of all colectomies were performed in the year of diagnosis. Conclusion: Hospitalization and re-hospitalization rates were relatively high in this population-based UC cohort. Early hospitalization was not predictive for the later disease course.


2020 ◽  
Vol 17 (3) ◽  
pp. 218-223
Author(s):  
Haichao Wang ◽  
Li Gong ◽  
Xiaomei Xia ◽  
Qiong Dong ◽  
Aiping Jin ◽  
...  

Background: Depression and anxiety after stroke are common conditions that are likely to be neglected. Abnormal red blood cell (RBC) indices may be associated with neuropsychiatric disorders. However, the association of RBC indices with post-stroke depression (PSD) and poststroke anxiety (PSA) has not been sufficiently investigated. Methods: We aimed to investigate the trajectory of post-stroke depression and anxiety in our follow- up stroke clinic at 1, 3, and 6 months, and the association of RBC indices with these. One hundred and sixty-two patients with a new diagnosis of ischemic stroke were followed up at 1, 3, and 6 months, and underwent Patient Health Questionnaire-9 (PHQ-9) and the general anxiety disorder 7-item (GAD-7) questionnaire for evaluation of depression and anxiety, respectively. First, we used Kaplan-Meier analysis to investigate the accumulated incidences of post-stroke depression and post-stroke anxiety. Next, to explore the association of RBC indices with psychiatric disorders after an ischemic stroke attack, we adjusted for demographic and vascular risk factors using multivariate Cox regression analysis. Results: Of the 162 patients with new-onset of ischemic stroke, we found the accumulated incidence rates of PSD (1.2%, 17.9%, and 35.8%) and PSA (1.2%, 13.6%, and 15.4%) at 1, 3, and 6 months, respectively. The incident PSD and PSA increased 3 months after a stroke attack. Multivariate Cox regression analysis indicated independent positive associations between PSD risk and higher mean corpuscular volume (MCV) (OR=1.42, 95% CI=1.16-1.76), older age (OR=2.63, 95% CI=1.16-5.93), and a negative relationship between male sex (OR=0.95, 95% CI=0.91-0.99) and PSA. Conclusion: The risks of PSD and PSA increased substantially 3 months beyond stroke onset. Of the RBC indices, higher MCV, showed an independent positive association with PSD.


Sign in / Sign up

Export Citation Format

Share Document