scholarly journals Mortality and Causes of Death in Inherited Antithrombin Deficiency

1997 ◽  
Vol 77 (03) ◽  
pp. 452-455 ◽  
Author(s):  
H H van Boven ◽  
J P Vandenbroucke ◽  
R G J Westendorp ◽  
F R Rosendaal

SummaryTo assess the contribution of inherited antithrombin deficiency to mortality, we investigated the causes of death in 14 families with inherited antithrombin deficiency. Between 1830 and 1994, 86 of 266 family members who had a probability of 0.5 or more for heterozygosity died. The causes of death were obtained for 58 of 66 deaths occurring between 1940 and 1994. Standardized mortality ratios (SMR) were calculated using mortality rates from the general population adjusted for age, sex and calendar period.The overall SMR was 0.90 from 1830 to 1994 (95% C.1.0.72-1.11). From 1940 until 1994 44 men and 22 women died (SMR = 1.09, 95% C.I. 0.84-1.39; SMR men = 1.20, 95% C.I. 0.87-1.61; SMR women = 0.92,95% C.I. 0.58-1.39). No excess mortality compared to the general population was found for cancer (14 deaths) or circulatory diseases (28 deaths). A slightly increased mortality caused by respiratory diseases (7 deaths, SMR = 1.68,95% C.I. 0.68-3.47) seemed due to pneumonia (4 deaths, SMR = 2.86, 95% C.I. 0.78-7.32). Venous thromboembolic complications were listed once in association with a risk situation, and one other death could be attributed to fatal pulmonary embolism. Cerebral hemorrhages were listed three times. It could not be verified whether these hemorrhages were related to anticoagulant therapy; the frequency was slightly higher than the expected population figure (SMR = 1.49,95% C.I. 0.31-4.36). The mean age of death for all causes was 64 years; the two fatal thromboembolic episodes occurred at age 20 and 30 years.The data show that antithrombin deficiency is associated with a normal survival and a low risk of fatal thromboembolic events. The use of long-term anticoagulant treatment in asymptomatic individuals should be considered carefully in view of the greater risk of fatal bleeding associated with long-term anticoagulant prophylaxis.

Author(s):  
Natalie Glaser ◽  
Michael Persson ◽  
Anders Franco‐Cereceda ◽  
Ulrik Sartipy

Background Prior studies showed that life expectancy in patients who underwent surgical aortic valve replacement (AVR) was lower than in the general population. Explanations for this shorter life expectancy are unknown. The aim of this nationwide, observational cohort study was to investigate the cause‐specific death following surgical AVR. Methods and Results We included 33 018 patients who underwent primary surgical AVR in Sweden between 1997 and 2018, with or without coronary artery bypass grafting. The SWEDEHEART (Swedish Web‐System for Enhancement and Development of Evidence‐Based Care in Heart Disease Evaluated According to Recommended Therapies) register and other national health‐data registers were used to obtain and characterize the study cohort and to identify causes of death, categorized as cardiovascular mortality, cancer mortality, or other causes of death. The relative risks for cause‐specific mortality in patients who underwent AVR compared with the general population are presented as standardized mortality ratios. During a mean follow‐up period of 7.3 years (maximum 22.0 years), 14 237 (43%) patients died. The cumulative incidence of death from cardiovascular, cancer‐related, or other causes was 23.5%, 8.3%, and 11.6%, respectively, at 10 years, and 42.8%, 12.8%, and 23.8%, respectively, at 20 years. Standardized mortality ratios for cardiovascular, cancer‐related, and other causes of death were 1.79 (95% CI, 1.75–1.83), 1.00 (95% CI, 0.97–1.04), and 1.08 (95% CI, 1.05–1.12), respectively. Conclusions We found that life expectancy following AVR was lower than in the general population. Lower survival after AVR was explained by an increased relative risk of cardiovascular death. Future studies should focus on the role of earlier surgery in patients with asymptomatic aortic stenosis and on optimizing treatment and follow‐up after AVR. Registration URL: https://www.clinicaltrials.gov ; Unique identifier: NCT02276950.


2020 ◽  
Vol 4 (9) ◽  
pp. 2084-2094
Author(s):  
Justine M. Kahn ◽  
Ruta Brazauskas ◽  
Heather R. Tecca ◽  
Stephanie Bo-Subait ◽  
David Buchbinder ◽  
...  

Abstract We examined the risk of subsequent neoplasms (SNs) and late mortality in children and adolescents undergoing allogeneic hematopoietic cell transplantation (HCT) for nonmalignant diseases (NMDs). We included 6028 patients (median age, 6 years; interquartile range, 1-11; range, <1 to 20) from the Center for International Blood and Marrow Transplant Research (1995-2012) registry. Standardized mortality ratios (SMRs) in 2-year survivors and standardized incidence ratios (SIRs) were calculated to compare mortality and SN rates with expected rates in the general population. Median follow-up of survivors was 7.8 years. Diagnoses included severe aplastic anemia (SAA; 24%), Fanconi anemia (FA; 10%), other marrow failure (6%), hemoglobinopathy (15%), immunodeficiency (23%), and metabolic/leukodystrophy syndrome (22%). Ten-year survival was 93% (95% confidence interval [95% CI], 92% to 94%; SMR, 4.2; 95% CI, 3.7-4.8). Seventy-one patients developed SNs (1.2%). Incidence was highest in FA (5.5%), SAA (1.1%), and other marrow failure syndromes (1.7%); for other NMDs, incidence was <1%. Hematologic (27%), oropharyngeal (25%), and skin cancers (13%) were most common. Leukemia risk was highest in the first 5 years posttransplantation; oropharyngeal, skin, liver, and thyroid tumors primarily occurred after 5 years. Despite a low number of SNs, patients had an 11-fold increased SN risk (SIR, 11; 95% CI, 8.9-13.9) compared with the general population. We report excellent long-term survival and low SN incidence in an international cohort of children undergoing HCT for NMDs. The risk of SN development was highest in patients with FA and marrow failure syndromes, highlighting the need for long-term posttransplantation surveillance in this population.


2017 ◽  
Vol 29 (3) ◽  
pp. 1001-1010 ◽  
Author(s):  
Samuel A. Silver ◽  
Ziv Harel ◽  
Eric McArthur ◽  
Danielle M. Nash ◽  
Rey Acedillo ◽  
...  

Mortality after AKI is high, but the causes of death are not well described. To better understand causes of death in patients after a hospitalization with AKI and to determine patient and hospital factors associated with mortality, we conducted a population-based study of residents in Ontario, Canada, who survived a hospitalization with AKI from 2003 to 2013. Using linked administrative databases, we categorized cause of death in the year after hospital discharge as cardiovascular, cancer, infection-related, or other. We calculated standardized mortality ratios to compare the causes of death in survivors of AKI with those in the general adult population and used Cox proportional hazards modeling to estimate determinants of death. Of the 156,690 patients included, 43,422 (28%) died in the subsequent year. The most common causes of death were cardiovascular disease (28%) and cancer (28%), with respective standardized mortality ratios nearly six-fold (5.81; 95% confidence interval [95% CI], 5.70 to 5.92) and eight-fold (7.87; 95% CI, 7.72 to 8.02) higher than those in the general population. The highest standardized mortality ratios were for bladder cancer (18.24; 95% CI, 17.10 to 19.41), gynecologic cancer (16.83; 95% CI, 15.63 to 18.07), and leukemia (14.99; 95% CI, 14.16 to 15.85). Along with older age and nursing home residence, cancer and chemotherapy strongly associated with 1-year mortality. In conclusion, cancer-related death was as common as cardiovascular death in these patients; moreover, cancer-related deaths occurred at substantially higher rates than in the general population. Strategies are needed to care for and counsel patients with cancer who experience AKI.


2019 ◽  
Vol 111 (9) ◽  
pp. 961-969 ◽  
Author(s):  
Ruth A Kleinerman ◽  
Margaret A Tucker ◽  
Byron S Sigel ◽  
David H Abramson ◽  
Johanna M Seddon ◽  
...  

Abstract Background Previous studies of hereditary retinoblastoma survivors have reported elevated mortality, particularly for sarcomas, compared with the general population. However, cause-specific mortality patterns for long-term hereditary and nonhereditary retinoblastoma survivors are poorly understood. Methods Among 2053 retinoblastoma patients diagnosed during 1914–2006 at two major US treatment centers and followed to 2016, we estimated cumulative mortality, standardized mortality ratios (SMRs), and absolute excess risks (AERs) compared with the US general population. Results Most deaths occurred in 1129 hereditary retinoblastoma patients (n = 518 deaths, cumulative mortality 70 years after retinoblastoma = 75.8%, 95% CI = 69.0% to 82.6%; SMR = 8.5, 95% CI = 7.7 to 9.2). Of these, 267 were due to subsequent cancers (SMR = 27.4, 95% CI = 24.2 to 30.9; AER = 72.3 deaths/10 000 person-years), for which SMRs were highest 15–29 years after diagnosis (n = 69, SMR = 89.9, 95% CI = 70.0 to 113.8) but remained statistically significantly elevated at 60 and more years (n = 14, SMR = 6.7, 95% CI = 3.6 to 11.2), whereas AERs increased with time (AER<15years = 38.0; AER60+years = 327.5). Increased risk of death due to cancers of pancreas, large intestines, and kidney were noted for the first time. Overall risk of subsequent cancers was greater for those treated with radiotherapy and chemotherapy compared to radiotherapy alone, although patterns varied by organ site. For 924 patients with nonhereditary retinoblastoma, we noted a modestly increased risk of death for subsequent cancers (n = 27, SMR = 1.8, 95% CI = 1.2 to 2.6) possibly due to treatment or misclassification of hereditary status. Risks of noncancer causes of death were not elevated for hereditary or nonhereditary patients. Conclusion Hereditary retinoblastoma survivors died mainly from an excess risk of subsequent cancers up to six decades later, highlighting the need to develop long-term clinical management guidelines for hereditary retinoblastoma survivors treated in the past.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 5006-5006
Author(s):  
Ragnhild Hellesnes ◽  
Tor Åge Myklebust ◽  
Sophie D. Fossa ◽  
Roy M. Bremnes ◽  
Asa Karlsdottir ◽  
...  

5006 Background: Previous studies have reported an increased risk of premature mortality in testicular cancer (TC) survivors, probably associated with previous platinum-based chemotherapy (PBCT) or radiotherapy (RT). However, complete data regarding PBCT cycles are lacking in available literature. Using complete TC treatment data, this population-based cohort study aimed to investigate non-TC mortality in relation to TC treatment. Methods: Overall, 5,707 men diagnosed with TC 1980-2009 were included, identified from the Cancer Registry of Norway. Clinical parameters and treatment data were abstracted from medical records and linked with the Norwegian Cause of Death Registry. Causes of death were classified by the European Shortlist. Standardized mortality ratios (SMRs) were calculated to compare the cause-specific mortality in the cohort to an age-matched general population. Age-adjusted hazard ratios (HRs) were estimated to evaluate the impact of number of PBCT cycles on non-TC mortality. Results: During a median follow-up of 18.7 years, 665 (12%) men were registered with non-TC death. The overall excess non-TC mortality was 23% (SMR 1.23, 95% CI 1.14-1.33) compared with the general population, with increased risks after PBCT (SMR 1.23, 95% CI 1.06-1.42) and RT (SMR 1.28, 95% CI 1.15-1.43), but not after surgery (SMR 0.95, 95% CI 0.79-1.14). SMRs increased significantly with increasing follow-up time ≥10 years, and the overall risk of non-TC death reached a maximum after ≥30 years follow-up (SMR 1.64, 95% CI 1.31-2.06). The most important cause of death was non-TC second cancer with an overall SMR of 1.53 (95% CI 1.35-1.73). Increased risks appeared after PBCT (SMR 1.43, 95% CI 1.12-1.83) and RT (SMR 1.59, 95% CI 1.34-1.89). Treatment with PBCT was associated with significantly 1.69-6.78-fold increased SMRs for cancers of the oral cavity/pharynx, esophagus, lung, bladder, and leukemia. After RT, significantly 3.02- 4.91-fold increased SMRs emerged for cancers of the oral cavity/pharynx, stomach, liver, pancreas and bladder. Non-cancer mortality was also increased by 15% (SMR 1.15, 95% CI 1.04-1.27), and excesses appeared after PBCT (1.23, 95% CI 1.03-1.47) and RT (SMR 1.17, 95% CI 1.01-1.34). Importantly, we report excess suicides after PBCT (SMR 1.65, 95% CI 1.01-2.69). Long-term overall cardiovascular mortality was not increased in the study cohort nor according to treatment modality. Compared with surgery, the overall non-TC mortality was increased after 4 (HR 1.41, 95% CI 1.00-1.98) and >4 (HR 2.03, 95% CI 1.24-3.33) PBCT cycles after >10 years of follow-up. Conclusions: TC treatment with PBCT or RT is associated with significantly increased long-term non-TC mortality, with non-TC second cancer being the most important cause of death. Significantly elevated risks for non-TC mortality emerged after ≥4 PBCT cycles after >10 years of follow-up.


2010 ◽  
Vol 16 (12) ◽  
pp. 1437-1442 ◽  
Author(s):  
Marja-Liisa Sumelahti ◽  
Matti Hakama ◽  
Irina Elovaara ◽  
Eero Pukkala

Background: Several studies show a high mortality risk among patients with multiple sclerosis (MS). Objectives: In this study, mortality and underlying causes of death were analysed among patients with MS diagnosed between 1964—1993 in Finland ( n = 1595). Methods: Standardized mortality ratios (SMRs) were calculated for both genders. The follow-up was based on linkage to the national computerized Cause-of-Death Register of Statistics Finland. Results: Altogether, 464 deaths were recorded by the end of 2006. The SMR as compared with the general population among females was 3.4 (95% confidence interval 3.0—3.9) and among males 2.2 (1.9—2.6). In total, 270 patients (58%) died from MS; only one of these deaths occurred during the first 2 years after the MS diagnosis. Mortality was also increased for other natural causes of death ( n = 160) in patients followed for more than 10 years (SMR 1.4, 1.2—1.7), with a significant increase in deaths from influenza (29, 6.0—85), pneumonia (4.7, 2.5—8.0) and gastrointestinal causes (4.4, 2.3—7.7). The SMR for violent causes was 1.2 (0.7—1.9) and for alcohol-related deaths 0.2 (0.02—0.7). The SMR for suicides was 1.7 (0.9—2.7). Conclusions: The MS population has an increased disease mortality, while the increase in the risk of accidents and suicides is not significantly increased among patients with MS in Finland.


Healthcare ◽  
2021 ◽  
Vol 9 (11) ◽  
pp. 1402
Author(s):  
Po-Feng Lee ◽  
Chung-Yi Li ◽  
Yen-Chin Liu ◽  
Chang-Ta Chiu ◽  
Wen-Hsuan Hou

(1) Background: The prevalence of opioid use in Taiwan increased by 41% between 2002 and 2014. However, little is known regarding the risk of mortality among long-term opioid analgesics users who do not have cancer. This study investigated this mortality risk with an emphasis on the calendar year and patients’ age and sex. (2) Methods: This retrospective cohort study included 12,990 adult individuals without cancer who were long-term users of opioid analgesics and were randomly selected from the data set of Taiwan’s National Health Insurance program from 2000 to 2012. They were then followed up through 2013. Information on the underlying causes of death was retrieved from the Taiwan Death Registry. Age, sex, and calendar year-standardized mortality ratios (SMRs) of all-cause and cause-specific mortality were calculated with reference to those of the general population. (3) Results: With up to 14 years of follow-up, 558 individuals had all-cause mortality in 48,020 person-years (cumulative mortality: 4.3%, mortality rate: 11.62 per 1000 person-years). Compared with the general population, the all-cause SMR of 4.30 (95% confidence interval (95% CI): 3.95–4.66) was significantly higher: it was higher in men than in women, declined with calendar year and age, and was significantly higher for both natural (4.15, 95% CI: 3.78–4.53) and unnatural (5.04, 95% CI: 3.88–6.45) causes. (4) Conclusions: Long-term opioid analgesics use among individuals without cancer in Taiwan was associated with a significantly increased risk of mortality. The notably increased mortality in younger adults warrants attention. Strategies to reduce long-term opioid analgesics use, especially their overuse or misuse, are in an urgent need.


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 3320-3320
Author(s):  
Mary Eapen ◽  
Kwang Woo Ahn ◽  
Paul Orchard ◽  
Morton Jerome Cowan ◽  
Stella M. Davies ◽  
...  

Abstract Abstract 3320 Poster Board III-208 Late mortality in children who have undergone hematopoietic stem cell transplantation (HCT) for severe combine immunodeficiency (SCID), non-SCID immune diseases and inborn errors of metabolism (IEM) has not been studied. The goals of the current analyses were to: 1) determine the probability of long-term survival after HCT in patients who survive the first 2 years after HCT; 2) identify risk factors for late deaths; and 3) determine excess mortality relative to rates in an age and sex-matched general population. Nine hundred and sixty patients with >95% donor chimerism or recovery of T-cell function who survived at least 2 years after their transplantation were included in the study. Two hundred and one patients had SCID, 407, non-SCID immune diseases and 352, IEM. Seventy percent of SCID transplant recipients received grafts from an HLA-matched sibling or mismatched relative. Fifty six percent of non-SCID and IEM transplant recipients received grafts from unrelated donors, 32% from a matched sibling and 12% from a mismatched relative. All transplantations occurred in 1980 – 2003; the median follow-up of surviving patients was 7 years. Median ages of long-term survivors were 7, 9 and 10 years for SCID, non-SCID immune diseases and IEM, respectively. Because of differences in biologic features and transplant strategies for SCID, non-SCID immune diseases and IEM, the disease groups were analyzed separately. The 7-year probabilities of overall survival were 93%, 96% and 90% for SCID, non-SCID immune diseases and IEM, respectively. No patient, disease or transplant characteristic was associated with late deaths in patients with SCID. For non-SCID immune diseases, late deaths were higher in recipients of T-cell depleted grafts (RR 4.63, p=0.003). For IEM, late deaths were higher after unrelated donor (RR 2.75, p=0.018) and mismatched related donor (RR 2.77, p=0.042) transplants compared to matched sibling donor transplant. There were 69 late deaths with 52 occurring 2 – 6 years after transplantation and 17 after 6 years. Causes of death in patients who died between 2 – 6 years included: chronic graft-versus-host disease [CGVHD] (n=12), infection including encephalitis (n=11), organ failure (n=12), post-transplant lymphoproliferative disease (n=4), primary disease (n=5), acute abdomen (n=1), status epilepticus (n=1), acute myeloid leukemia (n=1), accidental death (n=1) and not reported (n=3). Causes of death beyond 6 years included CGVHD (n=1), infection including encephalitis (n=3), organ failure (n=4), primary disease (n=2), acute abdomen (n=1), brain stem glioma (n=1) and not reported (n=5). The table below shows the estimated excess deaths per 1000 compared to an age- and sex-matched general population at 2 – 6 years and beyond 6 – 10 years after HCT. Though the risk of late deaths in this population is in excess of that for the general population for several years after transplantation, with extended follow up, the risk appears to decrease towards normal rates. Beyond 6 years after HCT, among patients transplanted for SCID and non-SCID immune diseases, the risk of mortality does not differ significantly from the general population whereas for patients with IEM, mortality rates continue to be higher than that in the general population. Screening programs aimed at identifying late complications together with planned intervention may improve long-term survival in these patients. Excess deaths per 1000 (95% confidence interval) SCID Non-SCID IEM 2 – 6 years after HSCT 54 (28, 79)* 41 (28, 53)* 95 (82, 109)* 6 – 10 years after HSCT 25 (0, 51) 16 (0, 39) 45 (27, 62)* * Significant differences in mortality risks compared to the general population Disclosures No relevant conflicts of interest to declare.


2020 ◽  
Vol 54 (5) ◽  
pp. 375-382
Author(s):  
Esther Cubo ◽  
Carla Collazo Riobo ◽  
Cesar Gallego-Nieto ◽  
Miren Elizari-Roncal ◽  
Teresa Barroso-Pérez ◽  
...  

<b><i>Background:</i></b> A growing body of evidence relates restless legs syndrome (RLS) to an increased risk of mortality attributable to both cerebrovascular and cardiovascular events. The aim was to investigate survival in patients with RLS. <b><i>Methods:</i></b> This was an observational, retrospective longitudinal study of a cohort of patients followed up for 11 years. RLS was diagnosed by a physician using the International RLS Study Group criteria. Mortality was analyzed using age-standardized mortality ratios (SMR: observed/expected deaths) and Cox regression analysis. <b><i>Results:</i></b> Vital status was studied in a cohort of 232 patients: 181 women (78%), 96 with RLS (41.4%) with a mean age at baseline of 49.8 ± 15.0 years and a mean RLS duration of 14.1 ± 1.9 years, and 136 non-RLS (58.6%) with a mean age of 51.3 ± 14.9 years. This RLS cohort was followed up for a period of 10.4 ± 2.0 years. As of September 2019, 17 (7.3%) patients died (6 with RLS, 6.3%), and the most frequent cause was oncological (66.7%). A total of 944 person-years of observations were available for survival analysis. RLS was not associated with increased mortality in adjusted Cox regression analysis (HR = 1.12, 95% CI: 0.40–3.15), and survival was similar to that expected for the general population (SMR = 0.61, 95% CI: 0.27–1.36). <b><i>Conclusions:</i></b> RLS seems not to be associated with increased mortality compared to the general population. Still, studies with prospective data collection with large samples are needed to study the long-term mortality risk factors in RLS cohorts.


2021 ◽  
Author(s):  
Peri Harish Kumar ◽  
Sai Sharan Dwarka ◽  
Talal Zahid ◽  
Habib Ur Rehman ◽  
Tajbinder Singh Bains ◽  
...  

Abstract Objective: To identify cancer and non-cancer causes of death in hepatocellular carcinoma (HCC) patients over different time periods after diagnosis and to compare the mortality risk of each cause in HCC patients with the general population.Methods: In this retrospective cohort study, data of 67,637 HCC patients from 1975 to 2016 were collected from the Surveillance, Epidemiology, and End Results (SEER) database. We investigated the association between different causes of death and the following variables: age, race, tumor stage at diagnosis, and treatment (surgery, chemotherapy, and radiotherapy); each according to the periods of < 1 year, 1–5 years, 5–10 years, and > 10 years following the diagnosis. Standardized mortality ratios (SMRs) and their 95% confidence intervals (CIs) were calculated for cancer and non-cancer deaths in each of the mentioned periods following diagnosis.Results: Data of 67,637 patients, of whom 50,571 patients died during the follow-up period, were analyzed. Most deaths were due to HCC itself (35,535, 70.3%), followed by other cancers (3,983, 7.9%). Common causes of non-cancer mortality included infectious and parasitic diseases including HIV (2,823 patients, SMR = 105.68, 95% CI: 101.82-109.65), chronic liver disease (2,719 patients, SMR = 76.56, 95% CI: 73.71,79.5), and heart diseases (1,265 patients, SMR = 2.26, 95% CI: 2.14–2.39), with higher mortality risk in HCC patients than in the general population.Conclusion: Cancers stand for most deaths in patients with HCC. Besides, infectious, and parasitic diseases including HIV represent the commonest non-cancer cause of mortality.


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