The effects of joint disease, inhibitors and other complications on health-related quality of life among males with severe haemophilia A in the United States.

INTRODUCTION: Health-related quality of life (HRQoL) is reduced among persons with haemophilia. Little is known about how HRQoL varies with complications of haemophilia such as inhibitors and joint disease. Estimates of preference-based HRQoL measures are needed to model the cost-effectiveness of prevention strategies. AIM: We examined the characteristics of a national sample of persons with severe haemophilia A for associations with two preference-based measures of HRQoL. METHODS: We analysed utility weights converted from EuroQol 5 Dimensions (EQ-5D) and the Short Form 6 Dimensions (SF-6D) scores from 1859 males aged ≥14 years with severe haemophilia A treated at 135 US haemophilia treatment centres in 2005-2011. Bivariate and regression analyses examined age-group-specific associations of HRQoL with inhibitor status, overweight/obesity, number of bleeds, viral infections, indicators of liver and joint disease, and severe bleeding at the time of the first HRQoL measurement. RESULTS: Overall mean HRQoL utility weight values were 0.71 using the SF-6D and 0.78 using the EQ-5D. All studied patient characteristics except for overweight/obesity were significantly associated with HRQoL in bivariate analyses. In a multivariate analysis, only joint disease was significantly associated with utility weights from both HRQoL measures and across all age groups. After adjustment for joint disease and other variables, the presence of an inhibitor was not significantly associated with HRQoL scores from either of the standardized assessment tools. CONCLUSION: Clinically significant complications of haemophilia, especially joint disease, are strongly associated with HRQoL and should be accounted for in studies of preference-based health utilities for people with haemophilia.

Tracking psychosocial health in adults with epilepsy--estimates from the 2010 National Health Interview Survey.

OBJECTIVE: This study provides population-based estimates of psychosocial health among U.S. adults with epilepsy from the 2010 National Health Interview Survey. METHODS: Multinomial logistic regression was used to estimate the prevalence of the following measures of psychosocial health among adults with epilepsy and those without epilepsy: 1) the Kessler-6 scale of serious psychological distress; 2) cognitive limitation, the extent of impairments associated with psychological problems, and work limitation; 3) social participation; and 4) the Patient-Reported Outcome Measurement Information System Global Health Scale. RESULTS: Compared with adults without epilepsy, adults with epilepsy, especially those with active epilepsy, reported significantly worse psychological health, more cognitive impairment, difficulty in participating in some social activities, and reduced health-related quality of life (HRQOL). CONCLUSIONS: These disparities in psychosocial health in U.S. adults with epilepsy serve as baseline national estimates of their HRQOL, consistent with Healthy People 2020 national objectives on HRQOL.

Development and psychometric evaluation of the public health surveillance well-being scale.

PURPOSE: To develop and psychometrically evaluate the brief Public Health Surveillance Well-Being Scale (PHS-WB) that captures mental, physical, and social components of well-being. METHODS: Using data from 5,399 HealthStyles survey respondents, we conducted bi-factor, item response theory, and differential item functioning analyses to examine the psychometric properties of a pool of 34 well-being items. Based on the statistical results and content considerations, we developed a brief 10-item well-being scale and assessed its construct validity through comparisons of demographic subgroups and correlations with measures of related constructs. RESULTS: Based on the bi-factor analyses, the items grouped into both an overall factor and individual domain-specific factors. The PHS-WB scale demonstrated good internal consistency (alpha = 0.87) and correlated highly with scores for the entire item pool (r = 0.94). The well-being scale scores differed as expected across demographic groups and correlated with global and domain-specific measures of similar constructs, supporting its construct validity. CONCLUSION: The 10-item PHS-WB scale demonstrates good psychometric properties, and its high correlation with the item pool suggests minimal loss of information with the use of fewer items. The brief PHS-WB allows for well-being assessment on national surveys or in other situations where a longer form may not be feasible.

Tetrachlorodibenzodioxin: an accidental poisoning episode in horse arenas.

Tetrachlorodibenzodioxin was identified as the apparent cause of an outbreak of poisoning in humans, horses, and other animals. Exposure was related to the spraying of contaminated waste oil on riding arenas for dust control. The contamination resulted from improper disposal of a toxic industrial waste. The pathologic effects and chemical identification of tetrachlorodibenzodioxin are described.

Prenatal and neonatal risk factors for childhood lymphatic leukemia.

BACKGROUND: Because the incidence of childhood acute lymphatic leukemia peaks between 2 and 4 years of age, the risk factors may exert their influence during the prenatal and/or the neonatal periods. Results of previous studies of perinatal risk factors have been contradictory, perhaps because most studies either have been hospital based or have been restricted to limited geographical areas. PURPOSE: A nationwide case-control study was carried out to identify maternal and perinatal risk factors for this disease. METHODS: The case-control study was nested in cohorts defined by all live births in Sweden recorded in the nationwide Medical Birth Register. Since 1973, this register has routinely collected information on all hospital births in regard to maternal demographic data, reproductive history, pregnancy, delivery, and the neonatal period. From the Swedish National Cancer Register, 613 case subjects were identified in successive birth cohorts from 1973 through 1989. Five control subjects per case subject were randomly selected from the pool of children matched by sex and month and year of birth. Conditional logistic regression was used to calculate the odds ratios (ORs) and 95% confidence intervals (CIs) for potential risk factors and to estimate their effects after adjustment for possible confounders. RESULTS: Risk of childhood lymphatic leukemia at all ages increased with Down's syndrome (OR = 20.0; 95% CI = 4.2-94.2), maternal renal disease (OR = 4.4; 95% CI = 1.6-12.1), use of supplementary oxygen (OR = 2.3; 95% CI = 1.5-3.6), postpartum asphyxia (OR = 1.8; 95% CI = 1.2-2.6), birth weight of more than 4500 g (OR = 1.7; 95% CI = 1.1-2.7), and hypertensive disease during pregnancy (OR = 1.4; 95% CI = 1.0-1.9). Down's syndrome affected risk mostly in children younger than 5 years, whereas other factors affected those children 5 years old or older. Being one of a multiple birth also increased risk among older children (OR = 2.5; 95% CI = 1.0-6.0). Use of supplementary oxygen may act as a causal intermediate (surrogate) for postpartum asphyxia and its causes, as would high birth weight for its causes. CONCLUSIONS: Several maternal and perinatal risk factors were found to be associated with childhood lymphatic leukemia, but they showed age-specific differences. Overall, only a few risk factors were identified, and these accounted for a small proportion of cases. We concluded that most risk factors for childhood lymphatic leukemia remain unidentified in very young children.

High school contact among persons with leukemia and lymphoma.

We studied frequency of contact among Connecticut high school students and teachers with leukemia and lymphoma diagnosed during or after high school from 1960 through 1971. Risk of having attended the same grade at the same school during the same year was greater among students with Hodgkin's disease (HD) than among simulated controls drawn in proportion to school enrollment (relative risk = 1.44; approximate 95% lower confidence limit = 1.05). Risk of developing HD was also greater among students enrolled simultaneously at the same school as students already diagnosed with HD than among students not so enrolled (relative risk = 2.05; 95% lower confidence limit = 1.39). However, fewer HD cases (16) were diagnosed from 1965 through 1970 at schools that formerly had patients enrolled (1959-64) than at matched schools without such patients (24). We found no evidence of increased contact among persons with non-HD lymphomas or leukemias, except between HD and non-HD lymphomas (relative risk = 1.41; approximate 95% lower confidence limit = 1.11).

Increased incidence and prevalence of multiple sclerosis in Los Alamos County, New Mexico.

The incidence and prevalence of multiple sclerosis (MS) in Los Alamos County, New Mexico, were investigated because the number of reported cases appeared to have increased. The point prevalence on November 1, 1979, was 75.7 per 100,000, and average annual incidence rates for the period 1960-1969 and 1970-1979 were 3.4 and 3.7 per 100,000, respectively. The rates of MS in Los Alamos County were greater than expected from previous epidemiologic surveys of North America. The unusual ethnic composition and high socioeconomic level of the population probably contributed to the increases.

Evaluation of surveillance programmes for colorectal cancer in ulcerative colitis patients by case-control studies: methodological considerations.

OBJECTIVES: The evaluation of the efficacy of colonoscopy screening in patients with ulcerative colitis for colorectal cancer is associated with methodological difficulties. Case-control studies can, however, be used to determine the efficacy of such a programme and the outline of the methodology in such a programme is presented. METHODS: The randomised controlled trial provides perspective for case-control studies of screening efficacy. Cases are selected from persons who have ulcerative colitis with manifestations of colorectal cancer: for example, those who have died of colorectal cancer or have symptomatic metastases. Controls are selected from persons who have ulcerative colitis, who had been alive when the case died of colorectal cancer, and who had been subject to the risk of dying from, but had not had, colorectal cancer diagnosed when the case was diagnosed with colorectal cancer. The relevant screening history for cases begins with the case's diagnosis of ulcerative colitis and ends with the cases diagnosis of colorectal cancer; that for controls should be comparable to that for cases to avoid bias. Cases and controls are compared with respect to their "exposure" to colonoscopy during their screening histories: the occurrence of any screening, which took place during the period of time that an occult tumour (or an identifiable lesion) may plausibly have been present. CONCLUSION: The proposed methodology can evaluate the efficacy of a screening programme rapidly, practically, and ethically.

Measuring health-related quality of life for public health surveillance.

In public health research and practice, quality of life is increasingly acknowledged as a valid and appropriate indicator of service need and intervention outcomes. Health-related quality of life measures, including objective and subjective assessments of health, are particularly useful for evaluating efforts in the prevention of disabling chronic diseases. Such data can inform health policy, planning, and practice. Mechanisms for routinely monitoring quality of life of populations at the State and local levels are currently lacking, however. This article discusses the rationale for and concepts measured by four quality of life questions developed for the 1993 Behavioral Risk Factor Surveillance System, a State-based telephone surveillance system. To encourage quality of life surveillance by States, the Centers for Disease Control and Prevention's National Center for Chronic Disease Prevention and Health Promotion held two related workshops, one in December 1991 and the other in June 1992. The workshops convened experts in quality of life and functional status measurement and resulted in the formulation of items for the Behavioral Risk Factor Surveillance System on self-perceived health, recent physical and mental health, and recent limitation in usual activities. The criteria, including feasibility and generalizability, considered by the Centers for Disease Control and Prevention and the workshop participants in the selection and development of these items are discussed. A model that conceptualizes the relationship of quality of life domains measured by the four survey items is presented and validated with preliminary data from the 1993 Behavioral Risk Factor Surveillance System. Finally, how States can use these measures to track progress towards the Year 2000 goal of improving quality of life is discussed.

Temporal clustering of heroin overdoses in Washington, DC.

During the 5-day period from 28 Feb. 1985 through 4 March 1985, 24 heroin overdoses occurred in the District of Columbia. Statistical tests for clustering of fatal and nonfatal overdoses during this interval identified 7 heroin-related deaths that occurred on March 1 to 2 as a statistically significant cluster (p = 0.007). An extension of the analysis for clustering to a 15-month period identified 2 additional clusters, 1 of fatal overdoses and 1 of nonfatal ones. When all victims of fatal overdose in cluster intervals were combined and compared with all other heroin-related deaths, no significant differences were noted for levels of morphine or ethanol in blood. However, bile morphine concentrations of cluster decedents were significantly lower than those of noncluster decedents (p = 0.033), suggesting that these decedents were less tolerant to the effects of narcotics than the comparison group. Heroin concentrations in street-level heroin samples collected during clusters did not differ from those collected during comparison intervals. These data conflict with the traditional explanation of overdose clusters, which attributes these events to unusually potent street-level heroin.

Neurologic evaluation of a population exposed to arsenic in Alaskan well water.

One hundred forty-seven persons exposed to arsenic from well water were evaluated by neurologic examination and nerve conduction studies. Total arsenic concentrations in well water ranged from 1 to 4781 micrograms/L and from 6 to 4964 micrograms/L in urine; a calculated index of arsenic ingestion ranged from 1 to 4521 micrograms/day. No dose-response relationship existed between arsenic ingestion and symptoms or physical findings compatible with peripheral neuropathy. Five of six persons with symptoms or physical findings suggestive of sensory neuropathy had normal nerve conduction velocities. Thirteen persons with elevated arsenic ingestion but no signs or symptoms of neuropathy had one or more abnormal nerve conduction velocities. No dose-response relationship, however, existed between arsenic ingestion and nerve conduction velocities. The authors concluded that arsenic ingestion from well water at the concentrations found in this Alaskan community did not result in clinical or subclinical neuropathy.

Increase in deaths from ischaemic heart-disease after blizzards.

Death certificates in eastern Massachusetts after six blizzards in 1974--78, including the record blizzard of Feb. 6, 1978, were examined to identify the effect on mortality of these storms. The total number of deaths was significantly higher (8%) in a "blizzard week" than in the preceding and subsequent (control) weeks (114.1 vs. 105.3 deaths per day). Deaths from ischaemic heart-disease (I.H.D.), which rose significantly by 22% in the blizzard week from 36.7 to 44.6 deaths per day, accounted for 90% of the excess total deaths. The increase was greater in males than in females (30% vs. 12%), and in both sexes there was no difference in the distribution of deaths by age between the blizzard and control weeks. I.H.D. deaths were increased for 8 days after a snowstorm, suggesting that the effect was related to activities such as snow shovelling rather than the storm itself. The identification of those at increased risk of I.H.D. death after major snowstorms and of the circumstances surrounding such deaths could lead to public-health measures to reduce these weather-related premature deaths.

Comparison of sudden and nonsudden coronary deaths in the United States.

BACKGROUND: The present study was designed to compare risk factor prevalences in coronary heart disease deaths in persons dying within 1 hour of onset of cardiovascular symptoms (sudden coronary death), those dying without such sudden symptoms (nonsudden coronary death), and those with unknown duration of symptoms before death (other coronary death). METHODS AND RESULTS: Data from the 1986 National Mortality Followback Survey and the US Bureau of the Census were examined to assess death rates for sudden, nonsudden, and other coronary deaths. Multivariate logistic regression methods were used to calculate the odds ratio (OR), compared with nonsudden and other coronary deaths, for sudden coronary death associated with socioeconomic status variables, the person's location at death, and coronary heart disease risk factors. Mortality rates for all coronary deaths increased with age, were higher for men than women, and increased with decreasing years of schooling. The rate of sudden coronary death was highest for Hispanics. In 1986, an estimated 251,000 sudden coronary deaths (95% CI = 238,000 to 263,000) occurred in the United States. Sudden coronary deaths were less likely than nonsudden coronary deaths to occur at home (OR = 0.5, 95% CI = 0.4 to 0.6), but individuals who died of sudden coronary death were more likely to have been current cigarette smokers (OR = 1.3, 95% CI = 1.0 to 1.8). No other modifiable risk factors for coronary heart disease distinguished sudden coronary deaths from nonsudden coronary deaths. CONCLUSIONS: Contrary to the commonly held view, coronary deaths in the home are more likely to be nonsudden than sudden. Cigarette smoking more likely results in sudden than nonsudden coronary death, perhaps because of nicotine-induced ventricular arrhythmias.

Paternal age and the occurrence of birth defects.

The association between paternal age and the occurrence of birth defects was studied using data collected in Metropolitan Atlanta. Paternal-age information for babies born with defects was obtained from birth certificates, hospital records, and interviews with mothers; for babies born without defects, the information was obtained from birth certificates. Several statistical techniques were used to evaluate the paternal-age-birth-defects associations for 86 groups of defects. Logistic regression analysis that controlled for maternal age and race indicated that older fathers had a somewhat higher risk for having babies with defects, when all types of defects were combined; an equivalent association for older mothers was not found. Logistic regression analyses also indicated modestly higher risks for older fathers for having babies with ventricular septal defects and atrial septal defects and substantially higher risks for having babies with defects classified in the category chondrodystrophy (largely sporadic achondroplasia) and babies with situs inversus. An association between elevated paternal age and situs inversus has not been reported before; the magnitude of the estimated increased risk for situs inversus was about the same as that found in this study for chondrodystrophy.

Risk factors for myocardial infarction associated with the Chicago snowstorm of jan 13- 15, 1979.

Sixty male survivors of acute myocardial infarction in the week after the Chicago blizzard of Jan 15, 1979, were matched by hospital and sex with 60 myocardial infarction survivors from a week without snowfall to determine whether a history of previous heart disease or of cardiac risk factors increased the risk of a postblizzard myocardial infarction. Cases did not differ significantly from control subjects with respect to age, percentage working full time, or percentage with a history of heart disease, hypertension, smoking, diabetes mellitus, obesity, or gout. Hypercholesterolemia was four times as common among cases as among controls.

Perinatal risk factors for inflammatory bowel disease: a case-control study.

To examine perinatal risk factors for Crohn's disease and ulcerative colitis, the authors analyzed birth records for 257 known case participants delivered from 1924 through 1957 at the University Hospital in Uppsala County, Sweden, and compared them with records for 514 controls delivered at the hospital. The two groups were matched by date of birth, sex, and either maternal age or parity. Eleven study variables were abstracted from standard forms that recorded health events during the pregnancy and the delivery hospitalization. Participants were more likely than controls to have a recorded health event (odds ratio (OR) = 4.4; 95% confidence interval (CI) 3.0-6.4). In a multivariate model, this increased risk was evident for infectious (OR = 3.8; 95% CI 2.6-5.8) and noninfectious (OR = 3.5; 95% CI 2.0-6.3) events. Perinatal health events may have contributed to 40% of the inflammatory bowel disease cases in our study. Infants from families with low socioeconomic status had greater risk of inflammatory bowel disease than did infants from families with high socioeconomic status (OR = 3.0, 95% CI 1.5-6.1). Perinatal health events and low socioeconomic status independently increased the risk of inflammatory bowel disease.

Lead exposure in stained glass workers.

To evaluate lead exposure in stained glass workers, we measured blood lead levels in 12 professional glass workers, in 5 hobbyists, and in 4 workers' family members. Professional workers lead levels (mean 20.7 micrograms/dl) were higher than hobbyists' (11.6 micrograms/dl) (P = 0.02) or family members' (11.3 micrograms/dl). Levels increased with years worked, hours worked per week, and percentage of work involving lead. The mean lead concentration in settled dust samples from a stained glass workshop was 11,000 parts per million. Stained glass workers are at increased risk of lead exposure.

Cytogenetic findings in persons living near the Love Canal.

Cytogenetic analyses were performed on peripheral blood from 46 present or past residents of the area surrounding Love Canal, a former dump site for chemical wastes in Niagara Falls, NY. Participants included 17 persons in whom cytogenetic analyses had been performed in 1980 and 29 persons who had been living in 1978 in seven homes that directly adjoined the canal and in which environmental tests showed elevated levels of chemicals spreading from the canal. Frequencies of chromosomal aberrations and of sister chromatid exchange (SCE) did not differ significantly from control levels. For all participants, cigarette smoking was associated with an increase in sister chromatid exchange frequency.