Early mortality in EURODIAB population-based cohorts of type 1 diabetes diagnosed in childhood since 1989

AIMS/HYPOTHESIS: The aims of this study were to provide a contemporary picture of mortality and causes of death in Europe following a diagnosis of type 1 diabetes made before the 15th birthday, and to examine excess mortality by country for possible links to incidence level or national prosperity. METHODS: Thirteen population-based EURODIAB registers in 12 countries followed-up 28,887 children diagnosed since 1989, either by record linkage to population registers or through contact with doctors providing care. RESULTS: There were 141 deaths in the cohort during 219,061 person-years of follow-up compared with 69.1 deaths expected from national mortality rates, a standardised mortality ratio (SMR) of 2.0 (95% CI 1.7-2.4). The SMR varied from 0 to 4.7 between countries, but showed little relationship with the country's incidence rate or gross domestic product (US$ per capita). The SMR did not change significantly with attained age, calendar period or time since diagnosis. The female SMR (2.7; 95% CI 2.0-3.5) was greater than the male SMR (1.8; 95% CI 1.4-2.2), although absolute numbers of excess deaths were similar in the two sexes. One-third of deaths were classified as directly attributable to diabetes (many with mention of ketoacidosis) and half were unrelated to diabetes. There was a non-significant excess of accidental/violent deaths (48 observed vs 40.7 expected; SMR 1.2; 95% CI 0.9-1.6) but little excess in suicides (11 observed, 10.2 expected; SMR 1.1; 95% CI 0.5-1.9). CONCLUSIONS/INTERPRETATION: Before the onset of late complications, significant excess mortality existed following the diagnosis of type 1 diabetes in childhood, even in recent years. Variation between countries in this excess could not be explained.     

Increased cerebrovascular mortality in patients with hypopituitarism

OBJECTIVE  An increased prevalence of atherosclerosis has been shown among patients with hypopituitarism. The aim of the present study was to assess whether patients with hypopituitarism experience increased cardiovascular, in particular cerebrovascular, mortality.
DESIGN AND PATIENTS  Retrospective cohort study of mortality, 1952–1992, in 344 patients, of whom 130 were female, receiving conventional hormone replacement for hypopituitarism following neurosurgery for pituitary tumours. The general population in the catchment area of southern Sweden from which the patients were recruited constituted the reference population. Expected mortality was obtained from cause, sex, calendar year, and 5-year age-specific death rates for the area.
RESULTS  Increased mortality from cerebrovascular disease (standardized mortality ratio (SMR) 3.39; 95% CI 2.27–4.99) was the main contributor to the increased overall cardiovascular mortality (SMR 1.75; 95% CI 1.40–2.19). The increase in mortality from cardiac diseases was much smaller (SMR 1.41; 95% CI 1.04–1.88). The risk for cerebrovascular death was higher in women (SMR 4.91) than in men (SMR 2.64). The relative risk for cerebrovascular death was independent of the time interval since diagnosis of pituitary insufficiency, but was greater in subjects diagnosed at an earlier age (<55 years). No increased mortality in malignant tumours was observed (SMR 0.95; 95% CI 0.60–1.48).
CONCLUSION  The increased cerebrovascular mortality may be due to GH deficiency, or to long-term lack or inadequacy of substitution for other pituitary hormones. The observations that an early onset of pituitary insufficiency and female sex are predictors for a high risk for cerebrovascular mortality merit particular attention when treating this group of patients.     

Long‐term mortality and renal outcome in a cohort of 100 patients with Lupus Nephritis

Objective

To evaluate the long‐term mortality and renal outcome in a cohort of Danish patients with lupus nephritis (LN) and to identify outcome predictors among findings registered at the time of the first renal biopsy.

Methods

The cohort consisted of 100 patients diagnosed with LN (World Health Organization classes I–VI) between 1971 and 1995 and followed for a median duration of 14.7 years (range 0.01–36.9 years). Standardized mortality ratios (SMRs) were calculated on the basis of national age‐, sex‐, and calendar‐year period–specific death rates.

Results

Thirty‐seven deaths occurred in the cohort, corresponding to an overall SMR of 6.8 (95% confidence interval [95% CI] 4.9–9.4). Excess mortality was observed throughout followup. The SMR estimates were 9.0 (95% CI 4.7–17.1), 6.2 (95% CI 4.0–9.5), and 6.6 (95% CI 3.1–13.8) for patients diagnosed during the calendar‐year periods 1971–1979, 1980–1989, and 1990–1995, respectively. The cumulative renal survival after 5, 10, and 20 years of followup was 87%, 83%, and 73%, respectively. The risk of end‐stage renal disease (ESRD) did not decrease significantly across calendar‐year periods. Systolic blood pressure ≥180 mm Hg, focal segmental nephritis, and advanced sclerosing nephritis were identified as baseline predictors of death in multivariate regression analyses, while systolic blood pressure ≥180 mm Hg, serum creatinine level ≥140 μmoles/liter, and diagnostic delay predicted progression to ESRD.

Conclusion

LN is associated with excess long‐term mortality, and patients may progress to ESRD even after prolonged followup. Our analyses indicate that focal segmental histopathology at disease onset constitutes an important risk factor for death among LN patients. Moreover, our data underscore the importance of early intervention, blood pressure control, and long‐term followup in LN.     

Mortality among women with ductal carcinoma in situ of the breast in the population-based surveillance, epidemiology and end results program

BACKGROUND: Over 14% of breast cancers diagnosed in the United States annually are ductal carcinomas in situ (DCIS). There are no published population-based reports of the likelihood of breast cancer death among US women with DCIS. METHODS: We used data from the Surveillance, Epidemiology and End Results program to determine the likelihood of breast cancer death at 5 and 10 years among US women aged 40 and older diagnosed with DCIS from 1978 to 1983 (before screening mammography was common; n = 1525) and from 1984 to 1989 (when screening mammography became common; n = 5547). We also calculated standardized mortality ratios (SMRs) to compare observed deaths from breast cancer, cardiovascular disease, and all causes combined among women with DCIS with deaths expected based on general population mortality rates. RESULTS: Among women diagnosed with DCIS from 1978 to 1983, 1.5% died of breast cancer within 5 years and 3.4% within 10 years. Among women diagnosed from 1984 to 1989, 0.7% died of breast cancer within 5 years and 1.9% within 10 years. Relative to the general population, risk of breast cancer death was greater for women diagnosed from 1978 to 1983 (SMR, 3.4; 95% confidence interval [CI], 2.5-4.5) than for women diagnosed from 1984 to 1989 (10-year SMR, 1.9; 95% CI, 1.5-2.3). Women diagnosed from 1984 to 1989 were significantly less likely than women in the general population to have died of cardiovascular diseases (10-year SMR, 0.6; 95% CI, 0.5-0.7) or of all causes combined (SMR, 0.8; 95% CI, 0.7-0.8). CONCLUSIONS: Among women diagnosed with DCIS, risk of death from breast cancer was low, at least within the 10 years following diagnosis. This may reflect the effectiveness of treatment for DCIS, the "benign" nature of DCIS, or both. At 10 years, women diagnosed from 1984 to 1989 were less likely than women diagnosed from 1978 to 1983 to have died of breast cancer, and their risk of dying of all causes combined was lower than that in the general population.     

Crohn's disease: increased mortality 10 years after diagnosis in a Europe-wide population based cohort

BACKGROUND: No previous correlation between phenotype at diagnosis of Crohn's disease (CD) and mortality has been performed. We assessed the predictive value of phenotype at diagnosis on overall and disease related mortality in a European cohort of CD patients. METHODS: Overall and disease related mortality were recorded 10 years after diagnosis in a prospectively assembled, uniformly diagnosed European population based inception cohort of 380 CD patients diagnosed between 1991 and 1993. Standardised mortality ratios (SMRs) were calculated for geographic and phenotypic subgroups at diagnosis. RESULTS: Thirty seven deaths were observed in the entire cohort whereas 21.5 deaths were expected (SMR 1.85 (95% CI 1.30-2.55)). Mortality risk was significantly increased in both females (SMR 1.93 (95% CI 1.10-3.14)) and males (SMR 1.79 (95% CI 1.11-2.73)). Patients from northern European centres had a significant overall increased mortality risk (SMR 2.04 (95% CI 1.32-3.01)) whereas a tendency towards increased overall mortality risk was also observed in the south (SMR 1.55 (95% CI 0.80-2.70)). Mortality risk was increased in patients with colonic disease location and with inflammatory disease behaviour at diagnosis. Mortality risk was also increased in the age group above 40 years at diagnosis for both total and CD related causes. Excess mortality was mainly due to gastrointestinal causes that were related to CD. CONCLUSIONS: This European multinational population based study revealed an increased overall mortality risk in CD patients 10 years after diagnosis, and age above 40 years at diagnosis was found to be the sole factor associated with increased mortality risk.     

Survival and cause-specific mortality in ulcerative colitis: follow-up of a population-based cohort in Copenhagen County

BACKGROUND & AIMS: A population-based cohort from Copenhagen County comprising 1160 patients diagnosed with ulcerative colitis between 1962 and 1987 was followed-up until 1997 to describe survival and cause-specific mortality. METHODS: Observed vs. expected deaths were presented as standardized mortality ratio (SMR) with exact 95% confidence intervals (CI) calculated by using individually registered person-years at risk and Danish 1995 mortality rates. Cumulative survival curves were calculated. RESULTS: A total of 261 deaths occurred, not significantly different from the expected number of 249 (SMR, 1.05; 95% CI, 0.92-1.19). The median age at death among men was 70 years (range, 6-96 years) and among women 74 years (range, 25-96 years). Twenty-five deaths (9.6%) were caused by complications to ulcerative colitis, mostly infectious and cardiovascular postoperative complications. Patients older than 50 years of age at diagnosis and with extensive colitis showed an increased mortality within the first 2 years because of ulcerative colitis-associated causes. The mortality from colorectal cancer was not increased and that of cancer in general was significantly lower than expected: 50 vs. 71 (SMR, 0.70; 95% CI, 0.52-0.93). A significantly increased mortality from pulmonary embolism and pneumonia was found. Among women only, death from genitourinary tract diseases and suicide was significantly increased. CONCLUSIONS: Despite an overall normal life expectancy for patients with ulcerative colitis, patients >50 years of age and with extensive colitis at diagnosis had increased mortality within the first 2 years after diagnosis, owing to colitis-associated postoperative complications and comorbidity.     

Mortality in systemic sclerosis—a single centre study from the UK

This study aims to determine the cause and predictors of mortality in a cohort of patients with systemic sclerosis (SSc) and assess whether the mortality rate differs significantly from the general population. Patients enrolled onto the Royal National Hospital for Rheumatic Diseases Connective Tissue Disease database between 1999 and 2010 were included in this study. The NHS Strategic Tracing Service and UK Registry of Births, Marriages and Deaths were used to establish date and cause of deaths. A retrospective case note review collected information on clinical phenotype and serology. A standardised mortality ratio (SMR) was calculated and survival was determined using Kaplan–Meier estimates. Univariate and multivariate predictors of survival were assessed using proportional hazards regression modelling. Amongst this cohort of 204 patients (25 males, 40 diffuse SSc), the mean age at diagnosis was 51.6 years (SD13.7) and the mean duration of follow-up was 12.5 years (SD 8.8 years). In the deceased group (53 patients), the mean age of death was 72.0 years (SD 12.3 years). The mean disease duration at death was 14.2 years (SD 8.5 years). The overall SMR was 1.34 (95 % confidence interval (CI) 1.00–1.75). The SMR was higher in males (1.54 [95 % CI 0.67–3.04] vs. 1.30 [95 % CI 0.95–1.74]). The leading causes of death in this cohort were infection, respiratory disease and malignancy. The most common cause of SSc-related mortality was pulmonary complications. Factors adversely affecting survival were older age at diagnosis, male gender, interstitial lung disease (ILD) and anti-RNA polymerase III antibody. The mortality rate of our cohort, who had predominantly limited disease, was higher than that of the general population; although not as high as reported in previous retrospective studies.     

Ulcerative colitis: no rise in mortality in a European-wide population based cohort 10 years after diagnosis

BACKGROUND: Population based studies have revealed varying mortality for patients with ulcerative colitis but most have described patients from limited geographical areas who were diagnosed before 1990. AIMS: To assess overall mortality in a European cohort of patients with ulcerative colitis, 10 years after diagnosis, and to investigate national ulcerative colitis related mortality across Europe. METHODS: Mortality 10 years after diagnosis was recorded in a prospective European-wide population based cohort of patients with ulcerative colitis diagnosed in 1991-1993 from nine centres in seven European countries. Expected mortality was calculated from the sex, age and country specific mortality in the WHO Mortality Database for 1995-1998. Standardised mortality ratios (SMR) and 95% confidence intervals (CI) were calculated. RESULTS: At follow-up, 661 of 775 patients were alive with a median follow-up duration of 123 months (107-144). A total of 73 deaths (median follow-up time 61 months (1-133)) occurred compared with an expected 67. The overall mortality risk was no higher: SMR 1.09 (95% CI 0.86 to 1.37). Mortality by sex was SMR 0.92 (95% CI 0.65 to 1.26) for males and SMR 1.39 (95% CI 0.97 to 1.93) for females. There was a slightly higher risk in older age groups. For disease specific mortality, a higher SMR was found only for pulmonary disease. Mortality by European region was SMR 1.19 (95% CI 0.91 to 1.53) for the north and SMR 0.82 (95% CI 0.45-1.37) for the south. CONCLUSIONS: Higher mortality was not found in patients with ulcerative colitis 10 years after disease onset. However, a significant rise in SMR for pulmonary disease, and a trend towards an age related rise in SMR, was observed.     

Short-term mortality risk in children and young adults with type 1 diabetes: the population-based Registry of the Province of Turin, Italy

Short-term mortality risk in young diabetic people is an indicator of quality of care. We assessed this in the Italian incident population-based registry of Turin. The study base included 1210 incident cases (n=677 aged 0-14 years and n=533 aged 15-29 years) with diabetes, onset period 1974-2000 in the Province of Turin, Italy. The relevant timescale for analysis was the time since the onset of diabetes to death, or till 31 December 2003. Standardized mortality ratio (SMR) for all-cause mortality was computed using the Italian population as a standard, by 5 years, age group, sex, and calendar period. Mean attained age of the incident cohort was 29.7 years (range 5.2-49.7 years). During a mean follow-up period of 15.8 years (range 2.0-29.9 years), there were 19 deaths in 15,967. Nine person-years of observation (n=9.5 expected deaths), giving an all-cause mortality rate of 1.19/1000 person-years (95% CI 0.76-1.87) and an SMR of 1.96 (1.25-3.08). In no cases did death occur at the onset of diabetes or in childhood. Out of 19 deaths, 9 were diabetes related (n=6 coma and n=3 end-stage renal disease). In Cox regression analysis, the hazard ratio (HR) was higher in adult-onset than in childhood-onset diabetes (HR=3.90, 95% CI 1.14-13.39), independently of calendar period and gender. (1) Children and young adults with type 1 diabetes experienced a two-fold higher short-term mortality risk than Italian people of similar age and sex and (2) the risk was higher in adult-onset than in childhood-onset diabetes. The quality of diabetes care should be improved to prevent early deaths.     

Cardiovascular disease a hazard despite improved prognosis in patients with systemic lupus erythematosus: results from a Swedish population based study 1964-95

OBJECTIVE: Although short term prognosis has improved in patients with systemic lupus erythematosus (SLE) during the early disease course, less is known about the longterm prognosis. METHODS: A cohort of 4737 patients with a diagnostic code of SLE was identified 1964-94 in the Swedish Hospital Discharge Register and followed by linkage to the Cause of Death Register until the end of 1995. Mortality was separately analyzed in 3 different calendar periods (1964-75, 1975-84, 1985-95). The relative risk of death was estimated as standardized mortality ratio (SMR) using the Swedish population as a reference. RESULTS: In total 2314 patients were deceased. Mortality was 3-fold increased (SMR = 3.63, 95% CI 3.49, 3.78) and cardiovascular disease (CVD) was the major cause of death. Patients aged 20-39 years at the first discharge had a 16-fold increased risk of death from coronary heart disease (SMR = 15.99, 95% CI 10.4, 23.6). All-cause mortality had decreased since 1975 and the reason for this decrease was entirely due to a decrease in causes attributed to SLE, but not CVD. Patients aged 20-39 years at the first discharge had a pronounced decrease in mortality, with SMR 33.59 (95% CI 24.3, 45.3) before 1975 compared with SMR 14.23 (95% CI 8.70, 22.0) after 1984. CONCLUSION: Cardiovascular disease was the major cause of death in patients with SLE and young patients had a pronounced increased risk of death. Even if all-cause mortality had declined during the last 2 decades due to causes attributed to SLE, the risk of cardiovascular death remained unchanged.     

Mortality and causes of death in Crohn's disease: follow-up of a population-based cohort in Copenhagen County,Denmark

BACKGROUND & AIMS: A population-based cohort comprising 374 patients with Crohn's disease diagnosed in Copenhagen County between 1962 and 1987 was observed until 1997 for mortality and causes of death. METHODS: Observed deaths were compared with expected deaths calculated by using individually computed person-years at risk and 1995 rates for Copenhagen County. Cumulative survival curves were calculated. RESULTS: A total of 84 deaths occurred vs. 67 expected (standardized mortality ratio [SMR], 1.3; 95% confidence interval [CI], 1.01-1.56): 45 women vs. 31.8 expected (SMR, 1.4; 95% CI, 1.03-1.89) and 39 men vs. 35.2 expected (SMR, 1.1; 95% CI, 0.79-1.51). An excess mortality was observed among women observed for 21-25 years after diagnosis. Among women aged <50 years at diagnosis, 25 deaths were observed vs. 7.3 expected (SMR, 3.42; 95% CI, 2.21-5.04). Fourteen (31%) of the observed deaths among women and 8 (21%) among men had a certain or possible connection to Crohn's disease. Among causes of death unrelated to Crohn's disease, an overrepresentation of gastrointestinal diseases, infections, and diseases of the urinary organs was observed. CONCLUSIONS: An increased mortality was observed late in the disease course that was most pronounced among women younger than 50 years at diagnosis and was attributed to death associated with severe Crohn's disease.     

Causes of death in patients with celiac disease in a population-based Swedish cohort

BACKGROUND: Patients with celiac disease have an increased risk of death from gastrointestinal malignancies and lymphomas, but little is known about mortality from other causes and few studies have assessed long-term outcomes. METHODS: Nationwide data on 10 032 Swedish patients hospitalized from January 1, 1964, through December 31, 1993, with celiac disease and surviving at least 12 months were linked with the national mortality register. Mortality risks were computed as standardized mortality ratios (SMRs), comparing mortality rates of patients with celiac disease with rates in the general Swedish population. RESULTS: A total of 828 patients with celiac disease died during the follow-up period (1965-1994). For all causes of death combined, mortality risks were significantly elevated: 2.0-fold (95% confidence interval [CI], 1.8-2.1) among all patients with celiac disease and 1.4-fold (95% CI, 1.2-1.6) among patients with celiac disease with no other discharge diagnoses at initial hospitalization. The overall SMR did not differ by sex or calendar year of initial hospitalization, whereas mortality risk in patients hospitalized with celiac disease before the age of 2 years was significantly lower by 60% (95% CI, 0.2-0.8) compared with the same age group of the general population. Mortality risks were elevated for a wide array of diseases, including non-Hodgkin lymphoma (SMR, 11.4), cancer of the small intestine (SMR, 17.3), autoimmune diseases (including rheumatoid arthritis [SMR, 7.3] and diffuse diseases of connective tissue [SMR, 17.0]), allergic disorders (such as asthma [SMR, 2.8]), inflammatory bowel diseases (including ulcerative colitis and Crohn disease [SMR, 70.9]), diabetes mellitus (SMR, 3.0), disorders of immune deficiency (SMR, 20.9), tuberculosis (SMR, 5.9), pneumonia (SMR, 2.9), and nephritis (SMR, 5.4). CONCLUSION: The elevated mortality risk for all causes of death combined reflected, for the most part, disorders characterized by immune dysfunction.     

Survival and cause specific mortality in patients with inflammatory bowel disease: a long term outcome study in Olmsted County, Minnesota, 1940-2004

BACKGROUND AND AIMS: We followed a population based cohort of patients with inflammatory bowel disease (IBD) from Olmsted County, Minnesota, in order to analyse long term survival and cause specific mortality. Material and METHODS: A total of 692 patients were followed for a median of 14 years. Standardised mortality ratios (SMRs, observed/expected deaths) were calculated for specific causes of death. Cox proportional hazards regression was used to determine if clinical variables were independently associated with mortality. RESULTS: Fifty six of 314 Crohn's disease patients died compared with 46.0 expected (SMR 1.2 (95% confidence interval (CI) 0.9-1.6)), and 62 of 378 ulcerative colitis (UC) patients died compared with 79.2 expected (SMR 0.8 (95% CI 0.6-1.0)). Eighteen patients with Crohn's disease (32%) died from disease related complications, and 12 patients (19%) died from causes related to UC. In Crohn's disease, an increased risk of dying from non-malignant gastrointestinal causes (SMR 6.4 (95% CI 3.2-11.5)), gastrointestinal malignancies (SMR 4.7 (95% CI 1.7-10.2)), and chronic obstructive pulmonary disease (COPD) (SMR 3.5 (95% CI 1.3-7.5)) was observed. In UC, cardiovascular death was reduced (SMR 0.6 (95% CI 0.4-0.9)). Increased age at diagnosis and male sex were associated with mortality in both subtypes. In UC but not Crohn's disease, a diagnosis after 1980 was associated with decreased mortality. CONCLUSIONS: In this population based study of IBD patients from North America, overall survival was similar to that expected in the US White population. Crohn's disease patients were at increased risk of dying from gastrointestinal disease and COPD whereas UC patients had a decreased risk of cardiovascular death.     

Late mortality,secondary malignancy and hospitalisation in teenage and young adult survivors of Hodgkin lymphoma: report of the Childhood/Adolescent/Young Adult Cancer Survivors Research Program and the BC Cancer Agency Centre for Lymphoid Cancer

Late complications affecting Hodgkin lymphoma (HL) survivors are well described in paediatric and adult‐based publications. This study determined the late morbidity and mortality risk for 442 teenage and young adult (TYAs) 5‐year HL survivors, diagnosed at 15–24 years of age between 1970 and 1999, identified from the British Columbia Cancer Registry. Treatment details were abstracted from charts. Survivors and a matched comparison cohort were linked to provincial administrative health datasets until December 2006 and regression analysis was performed, providing risk ratios regarding mortality, secondary malignancy and morbidity causing hospitalisation. Sixty (13·6%) survivors experienced late mortality with excess deaths from secondary cancer [standardised mortality ratio (SMR) 18·6; 95% confidence interval (CI) 11–29·4] and non‐malignant disease (SMR 3·6; 95% CI 2·2–5·5). Excess secondary cancers (standardised incidence ratio 7·8; 95% CI 5·6–10·5) were associated with radiotherapy [Hazard ratio (HR) 2·7; 95% CI 1–7·7] and female gender (HR 1·8; 95% CI 1–3·4). Of 281 survivors treated between 1981 and 1999, 143 (51%) had morbidity resulting in hospitalisation (relative risk 1·45; 95% CI 1·22–1·73). Hospitalisation significantly increased with combined modality therapy, chemotherapy alone and recent treatment era. TYA HL survivors have excess risk of mortality and secondary malignancy continuing 30 years from diagnosis. Radiotherapy is associated with secondary malignancy and current response‐adapted protocols attempt to minimise exposure, but late morbidity causing hospitalisation remains significant.     

Mortality in Patients With Giant Cell Arteritis: A Cohort Study in UK Primary Care

Objective

To examine whether giant cell arteritis (GCA) is associated with increased all‐cause mortality and whether mortality differs according to age, sex, and calendar year of cohort entry.

Methods

Using the UK‐based Clinical Practice Research Datalink, we identified 9,778 newly diagnosed GCA patients from 1990–2014, and up to 10 nonvasculitis patients randomly matched to each case on age, sex, practice, and years of history before cohort entry. We used Cox regression to estimate adjusted hazard ratios (HRs) for mortality of GCA patients in comparison to nonvasculitis patients, then stratified by age, sex, and calendar year of cohort entry.

Results

Compared with nonvasculitis patients, GCA patients had increased mortality during the first year following diagnosis (adjusted HR 1.51, 95% confidence interval [95% CI] 1.40–1.64), and marginally increased mortality between 1 and 5 years after the diagnosis (adjusted HR 1.16, 95% CI 1.09–1.23), but not >5 years after the diagnosis (adjusted HR 1.06, 95% CI 1.00–1.12). GCA patients diagnosed before age 65 years had the highest mortality risk during the first year following diagnosis (adjusted HR 2.32, 95% CI 1.60–3.35). The mortality risk did not differ substantially by sex or calendar year of cohort entry.

Conclusion

GCA patients had an increased risk of mortality during the period shortly after the GCA diagnosis, in particular during the first year, but no increased risk after 5 years postdiagnosis. The mortality risk differed by age with an even greater increased 1‐year mortality in those age <65 years at diagnosis, but not by sex or calendar year of cohort entry.

     

Cause-specific and total mortality in the Canterbury (New Zealand) insulin-treated Diabetic Registry population: a 15-year follow-up study.

AIMS: To establish all-cause and cause-specific death rates, and risk factors for mortality in insulin-treated diabetic individuals living in the province of Canterbury, New Zealand. METHODS: Insulin-treated diabetic subjects (n = 995) on the Canterbury Diabetes Registry were followed up over 15 years and vital status determined. Death rates were standardized and hazard regression was used to model the effects of demographic covariates on relative survival time. RESULTS: There were 419 deaths in 11 226.3 person-years of follow-up with a standardized mortality ratio (SMR) of 2.0 (95% confidence interval (CI) 1.8-2.2). Relative mortality was greatest for the group aged 0-29 years (SMR 3.0 (95% CI 2.4-3.7)). After controlling for diabetes duration and gender, a 10-year increment in age of onset was associated with a 33% decrease in relative hazard (95% CI 29-36%), indicating that excess mortality due to diabetes declines with rising age of onset. After controlling for age of onset and gender, each 10-year increment in duration of diabetes is associated with a 26% decrease in relative hazard (95% CI 24-29%), indicating that with longer survival the mortality hazard approaches the general population hazard. Relative mortalities were increased for cardiovascular, renal and respiratory disease, but not malignancy. Relative mortality from acute metabolic complications was increased in the subgroup with age of onset of diabetes < 30 years and requiring insulin within 1 year of diagnosis. CONCLUSIONS: Mortality rates are high for insulin-treated diabetic individuals relative to the general population.     

Additional risk factors influence excess mortality in heterozygous familial hypercholesterolaemia

Life expectancy of patients with familial hypercholesterolaemia is decreased. Some untreated patients reach a normal life span and, therefore, additional risk factors and the type of mutation in the low-density lipoprotein (LDL) receptor gene are likely to influence the clinical outcome. We determined all cause mortality in kindreds with the disorder, who were untreated, in order to study (a) additional risk factors for coronary artery disease (CAD) and (b) the types of LDL receptor gene mutations that may contribute to a poor prognosis. The mortality in all 855 first-degree relatives of 113 unrelated patients was compared to the Dutch population after standardisation for age, gender, and calendar period. Analyses restricted to affected relatives could have underestimated the mortality risk due to lack of information about severe cases, who died prematurely. Therefore, all first-degree relatives were analysed and as a result the standardised mortality ratios (SMRs) exhibit only 50% of the excess mortality from familial hypercholesterolaemia. We observed 190 deaths in 32048 person-years leading to an overall SMR of 1.34 (95% confidence interval (CI) 1. 16-1.55, P=0.001). High excess mortality occurred in males between age 40 and 54 (SMR 2.34, 95% CI 1.60-3.31, P<0.001). The excess mortality decreased during the last decades. This change of mortality over calendar time shows that additional risk factors modulate the mortality from the disorder. The SMR of 62 families referred with premature CAD was 1.62 (95% CI 1.32-1.93, P<0.001) and the SMR was 1.10 (95% CI 0.86-1.34, P=0.4) in 51 families without premature CAD. The mortality risk of kindreds with null alleles was similar to that of kindreds with other mutations. In conclusion, the burden of the untreated disorder occurred mainly among middle-aged males and was not influenced by the type of mutation. Additional risk factors increased excess mortality significantly and are highlighted by the presence of premature CAD among first-degree relatives. This underscores the need for active identification of all hypercholesterolaemic relatives of such patients.     

Body mass index, playing position, race, and the cardiovascular mortality of retired professional football players

Concern exists about cardiovascular disease (CVD) in professional football players. We examined whether playing position and size influence CVD mortality in 3,439 National Football League players with ≥ 5 pension-credited playing seasons from 1959 to 1988. Standardized mortality ratios (SMRs) compared player mortality through 2007 to the United States population of men stratified by age, race, and calendar year. Cox proportional hazards models evaluated associations of playing-time body mass index (BMI), race, and position with CVD mortality. Overall player mortality was significantly decreased (SMR 0.53, 95% confidence interval [CI] 0.48 to 0.59) as was mortality from cancer (SMR 0.58, 95% CI 0.46 to 0.72), and CVD (SMR 0.68, 95% CI 0.56 to 0.81). CVD mortality was increased for defensive linemen (SMR 1.42, 95% CI 1.02 to 1.92) but not for offensive linemen (SMR 0.70, 95% CI 0.45 to 1.05). Defensive linemen's cardiomyopathy mortality was also increased (SMR 5.34, 95% CI 2.30 to 10.5). Internal analyses found that CVD mortality was increased for players of nonwhite race (hazard ratio 1.69, 95% CI 1.13 to 2.51). After adjusting for age, race, and calendar year, CVD mortality was increased for those with a playing-time BMI ≥ 30 kg/m2 (hazard ratio 2.02, 95% CI 1.06 to 3.85) and for defensive linemen compared to offensive linemen (hazard ratio 2.07, 95% CI 1.24 to 3.46). In conclusion, National Football League players from the 1959 through 1988 seasons had decreased overall mortality but those with a playing-time BMI ≥ 30 kg/m2 had 2 times the risk of CVD mortality compared to other players and African-American players and defensive linemen had higher CVD mortality compared to other players even after adjusting for playing-time BMI.     

Mortality in Behçet's disease

Objective

To report the long‐term mortality in patients with Behçet's disease (BD).

Methods

A cohort of 817 patients fulfilling the international criteria for BD from a single center in France were analyzed for causes of death, the standardized mortality ratio (SMR), and the factors associated with mortality.

Results

Among the 817 patients with BD, 41 (5%) died after a median followup of 7.7 years, of whom 95.1% were male. The mean ± SD age at death was 34.8 ± 11.9 years. Main causes of death included major vessel disease (mainly, arterial aneurysm and Budd‐Chiari syndrome) (43.9%), cancer and malignant hemopathy (14.6%), central nervous system involvement (12.2%), and sepsis (12.2%). The mortality rate at 1 year and 5 years was 1.2% and 3.3%, respectively. There was an increased mortality among patients ages 15–24 years (SMR 2.99, 95% confidence interval [95% CI] 1.54–5.39) and those ages 25–34 years (SMR 2.90, 95% CI 1.80–4.49) as compared with age‐and sex‐matched healthy controls. The mortality decreased in patients older than age 35 years (SMR 1.23, 95% CI 0.75–1.92). In multivariate analyses, male sex (hazard ratio [HR] 4.94, 95% CI 1.53–16.43), arterial involvement (HR 2.51, 95% CI 1.07–5.90), and a high number of BD flares (HR 2.37, 95% CI 1.09–5.14) were independently associated with the risk of mortality.

Conclusion

The overall mortality in our BD cohort was 5% after a median followup of 7.7 years. Male sex, arterial involvement, and the number of flares were associated with mortality in BD.

     

General and cancer mortality in a large cohort of Italian alcoholics

Background: The consumption of alcohol is an underappreciated risk factor for a wide range of conditions. Overall, it is associated with high mortality rates and causes approximately 4% of all deaths worldwide. This study aimed to evaluate the general and cancer mortality in a cohort of subjects with alcohol addiction residing in Tuscany (Central Italy). Methods: Overall, 2,272 alcoholics (1,467 men and 805 women; mean age at first examination 43.8 years ± 13.0), treated at the Alcohol Centre of Florence in the period April 1985 to September 2001, were followed until the end of the study period (median follow‐up: 9.6 years). A total of 21,855 person‐years were available for analyses. Expected deaths were estimated by using age, sex, and calendar‐specific regional mortality rates. Standardized mortality ratios (SMR) and 95% confidence intervals (CI) were calculated. Results: Six hundred and thirty‐six of the 2,272 patients (28.0%) died, yielding an SMR of 5.0 (95% CI: 4.6 to 5.4). The alcoholics had significantly elevated mortality risk from all malignant cancers (SMR = 3.8, 95% CI: 3.3 to 4.4) and a series of specific diseases (infections: SMR = 10.1, 95% CI: 4.8 to 21.1; diabetes: SMR = 3.6, 95% CI: 1.9 to 6.7; immunological system, including AIDS: SMR = 8.1, 95% CI: 4.1 to 16.2; nervous system: SMR = 3.5, 95% CI: 1.9 to 6.4; cardiovascular system: SMR = 2.4, 95% CI: 2.0 to 2.9; respiratory system: SMR = 5.8, 95% CI: 4.2 to 8.0; digestive system: SMR = 26.4, 95% CI: 22.6 to 30.8, including liver cirrhosis (SMR = 40.0, 95% CI: 33.9 to 47.1); violent causes: SMR = 6.6, 95% CI: 5.0 to 8.6). Among malignant cancers, the highest SMRs were found for cancers of the pharynx (SMR = 22.8, 95% CI: 9.5 to 54.8), oral cavity (SMR = 22.2, 95% CI: 13.2 to 37.6), liver (SMR = 13.5, 95% CI: 9.2 to 19.8), and larynx (SMR = 10.7, 95% CI: 5.8 to 19.9). Although women showed higher SMR in comparison with the general population of the area, their overall survival estimates during the follow‐up were higher than those for male alcoholics. Conclusions: This large series of Italian alcoholics showed a significant increase in total and cancer mortality in comparison with the general population, with female alcoholics reporting higher survival rates.