scholarly journals Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections

2020 ◽  
Vol 105 (6) ◽  
pp. e2207-e2213 ◽  
Author(s):  
Joonatan Borchers ◽  
Eero Pukkala ◽  
Outi Mäkitie ◽  
Saila Laakso

Abstract Context Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an autoimmune endocrinopathy with severe and unpredictable course. The impact of APECED on mortality has not been determined. Objective To assess overall and cause-specific mortality of patients with APECED. Design and Setting A follow-up study of Finnish patients with APECED from 1971 to 2018. Causes and dates of death were collected from Finnish registries. Patients Ninety-one patients with APECED. Main Outcome Measure Overall and cause-specific standardized mortality ratios (SMRs) determined by comparing the observed numbers of death and those expected on the basis of respective population death rates in Finland. Results The overall disease mortality was significantly increased (29 deaths, SMR 11; 95% confidence interval [CI] 7.2-16; P < 0.001). The relative risk (SMR) was highest in the youngest age groups but the absolute excess risk was similar (about 10 per 10 000 person-years) in all age categories. The highest SMRs were seen for endocrine and metabolic diseases (SMR 570; 95% CI, 270-1000; P < 0.001) and for oral and esophageal malignancies (SMR 170; 95% CI, 68-360; P < 0.001). Mortality was also increased for infections, diseases of digestive system, alcohol-related deaths, and for accidents. Due to the small number of cases we were unable to evaluate whether mortality was affected by disease severity. Conclusions Patients with APECED have significantly increased mortality in all age groups. Highest SMRs are found for causes that are directly related to APECED but also for infections. Increased alcohol- and accident-related deaths may be influenced by psychosocial factors.

Author(s):  
Shaun Purkiss ◽  
Tessa Keegel ◽  
Hassan Vally ◽  
Dennis Wollersheim

BackgroundQuantifying the mortality risk for people with diabetes is challenging because of associated comorbidities. The recording of cause specific mortality from accompanying cardiovascular disease in death certificate notifications has been considered to underestimate the overall mortality risk in persons with diabetes. Main AimDevelop a technique to quantify mortality risk from pharmaceutical administrative data and apply it to persons diagnosed with diabetes, and associated cardiovascular disease and dyslipidaemia before death. MethodsPersons with diabetes, cardiovascular disease and dyslipidaemia were identified in a publicly available Australian Pharmaceutical data set using World Health Organization anatomic therapeutic codes assigned to medications received. Diabetes associated multi-morbidity cohorts were constructed and a proxy mortality (PM) event determined from medication and service discontinuation. Estimates of mortality rates were calculated from 2004 for 10 years and compared persons with diabetes alone and associated cardiovascular disease and dyslipidemia. ResultsThis study identified 346,201 individuals within the 2004 calendar year as having received treatments for diabetes (n=51,422), dyslipidaemia (n=169,323) and cardiovascular disease including hypertension (n=280,105). Follow up was 3.3 x 106 person-years. Overall crude PM was 26.1 per 1000 person-years. PM rates were highest in persons with cardiovascular disease and diabetes in combination (47.5 per 100 person years). Statin treatments significantly improved the mortality rates in all persons with diabetes and cardiovascular disease alone and in combination over age groups >44 years (p<.001). Age specific diabetes PM rates using pharmaceutical data correlated well with Australian data from the National Diabetes Service Scheme (r=0.82) ConclusionProxy mortality events calculated from medication discontinuation in persons with chronic conditions can provide an alternative method to estimate disease mortality rates. The technique also allows the assessment of mortality risk in persons with chronic disease multi-morbidity.


Author(s):  
I.V. Bukhtiyarov ◽  
◽  
E.V. Zibarev ◽  
K.V. Betts

Abstract. Introduction. The work of civilian aviation pilots is characterized by heavy psychological and emotional stress in combination with other occupational factors. Such complex of adverse working conditions appears to be a risk for functional and somatic disorders, which may subsequently be reflected in the causes and rates of mortality in the distant period. The aim of this work is to study the mortality of retired civilian aviation pilots. Methods. A prospective cohort epidemiological study of civilian aviation pilots’ mortality. The cohort included 4513 male civilian aviation pilots of Russia who completed their employment and received employment pension. The follow-up period was 10 years (01.01.2010-31.12.2019), with 22156.9 person-years obtained. The age-specific mortality rates were calculated for 5-year age groups, the mortality risk was assessed using standardized mortality ratio (SMR) with 95% confidence interval (95% CI). The comparison group was the male Russian population. Results. As of 31.12.2019, out of 4513 civilian aviation pilots, 150 people deceased (3.3%). The age-specific mortality rates in the retired pilots’ cohort were lower in all age groups compared to the male Russian population, except for the 35-39 age group. The all-cause mortality risk for civilian aviation pilots was significantly lower compared to the male Russian population, SMR=0.31 (95%CL 0.26-0.36). Conclusion. Further research is required to determine the long-term effects of working conditions on civilian aviation pilots’ health. The follow-up period for the pilots’ cohort should be increases to 20 years and more.


2001 ◽  
Vol 179 (6) ◽  
pp. 498-502 ◽  
Author(s):  
Matti Joukamaa ◽  
Markku HeliöVaara ◽  
Paul Knekt ◽  
Arpo Aromaa ◽  
Raimo Raitasalo ◽  
...  

BackgroundThe impact of clinically diagnosed mental disorders on mortality in the general population has not been established.AimsTo examine mental disorders for their prediction of cause-specific mortality.MethodMental disorders were determined using the 36-item version of the General Health Questionnaire and the Present State Examination in a nationally representative sample of 8000 adult Finns.ResultsDuring the 17-year follow-up period 1597 deaths occurred. The presence of a mental disorder detected at baseline was associated with an elevated mortality rate. The relative risk in men was 1.6 (95% confidence interval 1.3–1.8) and in women, 1.4 (95% Cl 1.2–1.6). In men and women with schizophrenia the relative risks of death during the follow-up period were 3.3 (95% Cl 2.3–4.9) and 2.3 (95% Cl 1.3–3.8) respectively, compared with the rest of the sample. In both men and women with schizophrenia the risk of dying of respiratory disease was increased, but the risk of dying of cardiovascular disease was increased only in men with neurotic depression.ConclusionsSchizophrenia and depression are associated with an elevated risk of natural and unnatural deaths.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 840-840
Author(s):  
Rachael E. Hough ◽  
Clare Rowntree ◽  
Rachel Wade ◽  
Nicholas Goulden ◽  
Chris Mitchell ◽  
...  

Abstract Despite the substantial improvements made in the outcomes of paediatric ALL, with ‘cure' rates now in excess of 90%, survival in teenage and young adult (TYA) patients has remained inferior. The reasons for this are likely multifactorial, including tumour biology, toxicity, compliance, access to clinical trials and protocol (adult or paediatric) used. We report the toxicity profiles observed in children, teenagers and young adults treated on the UK intensive, minimal residual disease (MRD) directed ALL protocol, UKALL2003. Of a total of 3126 patients treated, 1520 patients were under 5 years old, 767 were aged 5-9 years, 610 aged 10-15 years and 229 aged 16-24 years, with a median overall follow-up of 4 year and 10 months. The risk of serious adverse events (SAEs) was higher in patients older than 10 years (56% in 10-15 year olds, 53% in 16-24 year olds) compared to those aged 9 or younger (30% in under 5 years and 31% in 5-9 years)(p<0.0001), with no difference in the those aged 16-24 compared to younger teenagers (p=0.5). The incidence (per number of patients in each group) and distribution of toxicities according to age group is summarised in the table.Table 1Age in years<55-910-1516-24AllTotal number of patients1520767610229 NB: 56 pts≥20 years3126Infection n (%)328 (21.6%)130 (17.0%)145 (23.8%)72 (31.4%)675 (21.6%)Asaparaginase n (%)57 (3.8%)57 (7.4%)64 (10.5%)31 (13.5%)209 (6.7%)Methotrexate n (%)100 (6.6%)74 (9.6%)123 (20.2%)33 (14.4%)330 (10.6%)Steroid n (%)54 (3.6%)37 (4.8%)141 (23.1%)52 (22.7%)284 (9.0%)Vincristine n (%)34 (2.2%)11 (1.4%)22 (3.6%)7 (3.0%)74 (2.4%)Other SAEs94 (6.2%)42 (5.5%)90 (14.8%)25 (10.9%)251 (8.0%) The incidence of certain toxicities including viral infection (5.3%), asparaginase hypersensitivity (1.9%) and vincristine neurotoxicity (2.1%) appeared equivalent across all age groups. Avacular necrosis was seen predominantly in adolescents (83% of 147 events in 10-19 year olds) and was rare in those younger than 10 years (n=18) or older than 20 years (n=7). Asparaginase thrombotic events increased in frequency with increasing age (1.5% in under 5 years, 3.3% in 5-9 years, 4.4% in 10-15 years and 8.3% in 16-24 year olds)(p<0.0001). All other toxicities were more frequently observed in over 10 year olds compared to patients aged 9 or younger, with no difference between 16-24 year olds and 10-15 year olds. The impact of age on SAEs associated with intensive ALL chemotherapy varies according to specific toxicities. In general, toxicity is higher in those over 10 years compared to younger patients, with no excess toxicity in those aged 16-24 compared to 10-15 years. However, specific toxicities may increase with increasing age (thrombosis), be restricted to adolescence (AVN) or be unrelated to age (vincristine neurotoxicity, asparaginase hypersensitivity). Disclosures: No relevant conflicts of interest to declare.


1985 ◽  
Vol 5 (6) ◽  
pp. 957-974 ◽  
Author(s):  
Robin S. Roberts ◽  
James A. Julian ◽  
Daniel Sweezey ◽  
David C.F. Muir ◽  
Harry S. Shannon ◽  
...  

Following the publication of the NIOSH nickel criteria document in 1977, the Joint Occupational Health Committee of the International Nickel Company (INCO) commissioned a mortality study of the company's Ontario workforce. This paper describes the detailed methodology and primary mortality results of the ensuing study; subsequent papers will describe more detailed findings of cause-specific mortality. An historical prospective mortality study of approximately 54,000 INCO workers has been conducted. Men with six months or more of service were followed for mortality during a 35-year period by computerized record linkage to the Canadian National Mortality Data Base. From a company-provided list of men known to have died and through independent follow-up of a random sample of 1,000 subjects of unknown status, we estimate a mortality ascertainment rate of 95%. Cause-specific standardized mortality ratios calculated with respect to Ontario provincial mortality rates indicate an excess of accidental deaths in men working in the Sudbury area and an excess of cancer deaths at the company's Port Colborne nickel refinery. A strong healthy worker effect was found for both all-disease mortality ad cancer mortality. The lower than expected mortality persisted for about 15 years beyond initial hiring.


1995 ◽  
Vol 2 (1) ◽  
pp. 61-66 ◽  
Author(s):  
Robert S Hogg ◽  
Martin T Schechter ◽  
Julio SG Montaner ◽  
James C Hogg

OBJECTIVE: To assess the impact of asthma on Canadian mortality rates over a 45-year period.DESIGN: A descriptive, population-based study.SETTING: Canada.SUBJECTS: All persons who died from asthma in Canada from 1946 to 1990 as reported to Statistics Canada in Ottawa.MAIN OUTCOME MEASURES: Standardized mortality ratios, age-specific patterns of death, potential years of life lost (PYLL) and life expectancy lost.RESULTS: A total of 12,010 male and 8486 female asthma deaths were recorded in Canada from 1946 to 1990. Mortality rates for both sexes declined from a high of between three to six deaths in 1951 to 1955 to approximately two deaths per 100,000 in 1986 to 1990, with the decline in rates being greater for males than females. Age-specific mortality rates were highest al all ages in 1951 to 1955, except for 15 to 24 years when deaths rates for the 1981 to 1985 period were greater. PYLL exhibit the same pattern as mortality, peaking in 1951 to 1955 and subsequently declining with each period. Loss in life expectancy due to asthma was about one month (not significant) in all time periods.CONCLUSIONS: Asthma mortality rates have declined significantly over the study period. This decline appears to be linked with the convergence of sex-specific rates and with changes in the patterning or age-specific mortality. The impact of asthma on the life expectancy of Canadians is small.


2020 ◽  
Vol 3 (2) ◽  
pp. 01-07
Author(s):  
Güven Serçin ◽  
Öztarhan Kazım

Aim: The aim of our study is to be able to predict the prognosis of patients with isolated pulmonary valvular stenosis on the basis of age and degree of stenosis. Identification of the course of pulmonary stenosis of different age groups will significantly contribute both to the physicians and the relatives of the patient. Material and Methods: 105 pediatric patients diagnosed with isolated pulmonary valvular stenosis were included in our study. We investigated the impact of the gradient of stenosis and the age at the time of diagnosis on the natural course of pulmonary stenosis. Mean follow-up time of the children was 19 months, 25.45±22.48 months. The patients were divided into four groups over their trans-valvular gradient degrees and<20 mmHg was defined as transient, 20-39 mmHg mild, 40-59 mmHg moderate, 60 mmHg and over as severe pulmonary stenosis. Results: Between two to five months, none of the moderate stenosis cases progressed unlike other patient groups. The decline in the final gradient versus initial gradient was significant in children between two to five months and six months to two years in our study, and yet there was no significant change of initial and final gradients in patients under one month, and at two years and over. Conclusion: It would be reasonable to conclude that the progression of pulmonary valvular stenosis is benign in patients with pulmonary valvular stenosis under 40 mmHg of systolic gradient diagnosed after 6th month of life.


PLoS ONE ◽  
2021 ◽  
Vol 16 (9) ◽  
pp. e0243804
Author(s):  
Anne Sverdrup Efjestad ◽  
Hege Ihle-Hansen ◽  
Vidar Hjellvik ◽  
Knut Engedal ◽  
Hege Salvesen Blix

Background/aims The aim was to explore the impact of sex on prevalence, patterns and trends in the prescription of psychotropics and analgesics in users of acetylcholinesterase inhibitors (AChEIs), before and after AChEI initiation, compared to the general population. Methods A prospective study applying data from the Norwegian Prescription Database (NorPD) in the period 2004–2016. Prescription of antidepressants, antipsychotics, analgesics including opioids, benzodiazepines and z-hypnotics in persistent AChEI users was studied in a follow-up period from four years before to two years after AChEI initiation in men and women of four age groups: 37–64, 65–72, 73–80 and 81–88 years. Results Use of antidepressants, antipsychotics and weaker analgesics increased in both sexes during the follow-up period in 11.764 persistent AChEI users. Women with pre-dementia and dementia stages of AD showed a prescription pattern with more use of psychotropics and opioids than men, except for antipsychotics. Conclusion Female sex showed to have a significant influence on the prescriptions of psychotropics and analgesics in AD patients in a pre-dementia and dementia stage. The exception is for antipsychotics, that men used more than women. The prescription pattern showed a higher extent of polypharmacy of psychotropics and/or opioids in women than in men. The total prescription pattern of analgesics could indicate an undertreatment of pain in pre-dementia and dementia stages, most pronounced in men.


2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e19056-e19056
Author(s):  
Nicole H. Dalal ◽  
Graca Dores ◽  
Rochelle E. Curtis ◽  
Martha S. Linet ◽  
Lindsay M. Morton

e19056 Background: LPL and WM are rare, indolent mature B-cell lymphomas. While recent studies reveal improving survival after LPL/WM, cause-specific mortality has not been comprehensively studied. Methods: We identified 6659 adults with first primary LPL (n = 2866) or WM (n = 3793) within 17 US population-based cancer registries from 2000 to 2015. Patients were followed for vital status (mean follow-up = 5.07 years), and causes of death were determined from death certificates. Standardized mortality ratios (SMRs) estimated relative risk of death compared to the general population. We estimated cumulative mortality and absolute excess risk (AER) per 10,000 person-years. Results: We observed 2826 deaths overall, of which 43%, 13%, and 42% were due to lymphoma, cancers excluding lymphoma, and non-malignant causes, respectively. There was a 20% higher risk of death due to non-malignant causes compared to the general population (n = 1194, SMR = 1.2, 95% confidence interval [CI] = 1.1 to 1.2). The most common non-malignant causes included infectious (n = 162, SMR = 1.8, 95% CI = 1.5 to 2.1, AER = 21.0), respiratory (n = 131, SMR = 1.2, 95% CI = 1.0 to 1.5, AER = 7.4), and digestive (n = 76, SMR = 1.9, 95% CI = 1.5 to 2.4, AER = 10.7) diseases. Cause-specific mortality varied by time since and age at LPL/WM diagnosis. Risks were highest in the first year after LPL/WM for non-malignant causes (SMR = 1.4, AER = 34.3), particularly infections (SMR = 2.4, AER = 34.4) and non-neoplastic hematologic diseases (SMR = 17.3, AER = 20.7). In contrast, risk of death due to cancers excluding lymphoma increased with time since diagnosis (SMR< 1y = 1.2, SMR≥5y = 1.7; AER< 1y = 15.1, AER≥5y = 60.0). Analyses by age, focused on AERs, showed generally similar risks across age groups (cancers excluding lymphoma: AER< 65= 26, AER65-75= 28, AER≥75= 31; non-malignant causes: AER< 65= 52, AER65-75= 66, AER≥75= 23). Cumulative mortality from non-malignant causes (23.7%) exceeded that from lymphoma (22.9%) beginning 9 years after LPL/WM diagnosis. Conclusions: Using population data, we identified areas to improve survivorship care of LPL/WM patients, particularly for non-malignant causes of death.


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