Background and ObjectivesRecent data suggest increasing global prevalence of multiple sclerosis (... more Background and ObjectivesRecent data suggest increasing global prevalence of multiple sclerosis (MS). Early diagnosis of MS reduces the burden of disability-adjusted life years and associated health care costs. Yet diagnostic delays persist in MS care and even within national health care systems with robust resources, comprehensive registries, and MS subspecialist referral networks. The global prevalence and characteristics of barriers to expedited MS diagnosis, particularly in resource-restricted regions, have not been extensively studied. Recent revisions to MS diagnostic criteria demonstrate potential to facilitate earlier diagnosis, but global implementation remains largely unknown.MethodsThe Multiple Sclerosis International Federation third edition of the Atlas of MS was a survey that assessed the current global state of diagnosis including adoption of MS diagnostic criteria; barriers to diagnosis with respect to the patient, health care provider, and health system; and existence of national guidelines or national standards for speed of MS diagnosis.ResultsCoordinators from 107 countries (representing approximately 82% of the world population), participated. Eighty-three percent reported at least 1 “major barrier” to early MS diagnosis. The most frequently reported barriers included the following: “lack of awareness of MS symptoms among general public” (68%), “lack of awareness of MS symptoms among health care professionals” (59%), and “lack of availability of health care professionals with knowledge to diagnose MS” (44%). One-third reported lack of “specialist medical equipment or diagnostic tests.” Thirty-four percent reported the use of only 2017 McDonald criteria (McD-C) for diagnosis, and 79% reported 2017 McD-C as the “most commonly used criteria.” Sixty-six percent reported at least 1 barrier to the adoption of 2017 McD-C, including “neurologists lack awareness or training” by 45%. There was no significant association between national guidelines pertaining to MS diagnosis or practice standards addressing the speed of diagnosis and presence of barriers to early MS diagnosis and implementation of 2017 McD-C.DiscussionThis study finds pervasive consistent global barriers to early diagnosis of MS. While these barriers reflected a lack of resources in many countries, data also suggest that interventions designed to develop and implement accessible education and training can provide cost-effective opportunities to improve access to early MS diagnosis.
Background-Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that typically res... more Background-Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that typically results in death within 2-5 years of initial symptom onset. Multidisciplinary ALS clinics (MDCs) have been established to provide specialty care to people living with the disease. Objective-To estimate the proximity of ALS prevalence cases to the nearest MDC in the US to help evaluate one aspect of access to care. Methods-Using 2013 prevalence data from the National ALS Registry, cases were geocoded by city using geographic information system (GIS) software, along with the locations of all MDCs in operation during 2013. Case-to-MDC proximity was calculated and analyzed by sex, race, and age group. Results-During 2013, there were 72 MDCs in operation in 30 different states. A total of 15,633 ALS cases were geocoded and were distributed throughout all 50 states. Of these, 62.6% were male, 77.9% were white, and 76.2% were 50-79 years old. For overall case-to-MDC proximity, nearly half (44.9%) of all geocoded cases in the US lived >50 miles from an MDC, including
Our objective was to evaluate the completeness of the United States National ALS Registry (Regist... more Our objective was to evaluate the completeness of the United States National ALS Registry (Registry). We compared persons with ALS who were passively identified by the Registry with those actively identified in the State and Metropolitan Area ALS Surveillance project. Cases in the two projects were matched using a combination of identifiers, including, partial social security number, name, date of birth, and sex. The distributions of cases from the two projects that matched/did not match were compared and Chi-square tests conducted to determine statistical significance. There were 5883 ALS cases identified by the surveillance project. Of these, 1116 died before the Registry started, leaving 4767 cases. We matched 2720 cases from the surveillance project to those in the Registry. The cases identified by the surveillance project that did not match cases in the Registry were more likely to be non-white, Hispanic, less than 65 years of age, and from western states. The methods used by the Registry to identify ALS cases, i.e. national administrative data and self-registration, worked well but missed cases. These findings suggest that developing strategies to identify and promote the Registry to those who were more likely to be missing, e.g. non-white and Hispanic, could be beneficial to improving the completeness of the Registry.
The Agency for Toxic Substances and Disease Registry (ATSDR) conducted a cross-sectional health s... more The Agency for Toxic Substances and Disease Registry (ATSDR) conducted a cross-sectional health study at the Fort Hall Indian Reservation in Idaho to investigate concerns about the health effects on reservation residents that might be attributed to two phosphate-processing plants located near the reservation`s southern border. In addition to increased particulates, air emissions from these plants included phosphorus pentoxide, cadmium, chromium, fluoride, uranium, and its daughter radionuclides. A total of 515 participants -- 229 from Fort Hall and 286 from a comparison group at the Duck Valley Indian Reservation -- were interviewed in person by trained American Indian interviewers. Approximately 100 residents of each reservation performed pulmonary function tests and provided urine specimens that were analyzed for cadmium, chromium, fluoride, and several renal biomarkers.
A human exposure study was conducted to determine the effect of mine tailings contaminated with l... more A human exposure study was conducted to determine the effect of mine tailings contaminated with lead, arsenic, and cadmium (Silver Creek Mine Tailings Site, Utah) on biological levels of these elements among persons living in the immediate vicinity. Cross-sectional data were collected from the target area and from a comparison area located off site. Biological measurements included blood lead, erythrocytic protoporphyrin, hemoglobin, urine beta-2-microglobulin, urine arsenic, and urine cadmium (adjusted to urine creatinine and specific gravity). In addition, all participants were surveyed to identify independent or potentially confounding factors that might influence the measurements. There was no evidence of excess exposure to lead, arsenic, or cadmium at clinically meaningful levels among residents living on or adjacent to the site. Although blood lead levels were higher in the target area than in the comparison area among children 9-71 months of age, the differing values were within the normal range. Further, the mean blood lead levels for both areas were well below the national mean levels (1976-1980) for this age group. Other behavioral factors found to increase blood lead levels in this age group were identified.
AbstractObjective: To develop a valid algorithm for identifying multiple sclerosis (MS) cases in ... more AbstractObjective: To develop a valid algorithm for identifying multiple sclerosis (MS) cases in administrative health claims (AHC) datasets. Methods: We used 4 AHC datasets from the Veterans Administration (VA), Kaiser Permanente Southern California (KPSC), Manitoba (Canada), and Saskatchewan (Canada). In the VA, KPSC, and Manitoba, we tested the performance of candidate algorithms based on inpatient, outpatient, and disease-modifying therapy (DMT) claims compared to medical records review using sensitivity, specificity, positive and negative predictive values, and interrater reliability (Youden J statistic) both overall and stratified by sex and age. In Saskatchewan, we tested the algorithms in a cohort randomly selected from the general population. Results: The preferred algorithm required ≥3 MS-related claims from any combination of inpatient, outpatient, or DMT claims within a 1-year time period; a 2-year time period provided little gain in performance. Algorithms including DMT...
ImportanceRacial, ethnic, and geographic differences in multiple sclerosis (MS) are important fac... more ImportanceRacial, ethnic, and geographic differences in multiple sclerosis (MS) are important factors to assess when determining the disease burden and allocating health care resources.ObjectiveTo calculate the US prevalence of MS in Hispanic, non-Hispanic Black (hereafter referred to as Black), and non-Hispanic White individuals (hereafter referred to as White) stratified by age, sex, and region.Design, Setting, and ParticipantsA validated algorithm was applied to private, military, and public (Medicaid and Medicare) administrative health claims data sets to identify adult cases of MS between 2008 and 2010. Data analysis took place between 2019 and 2022. The 3-year cumulative prevalence overall was determined in each data set and stratified by age, sex, race, ethnicity, and geography. The insurance pools included 96 million persons from 2008 to 2010. Insurance and stratum-specific estimates were applied to the 2010 US Census data and the findings combined to calculate the 2010 prev...
All hazardous substances spill databases created before the inception of HSEES were developed as ... more All hazardous substances spill databases created before the inception of HSEES were developed as passive reporting systems, relying on voluntary reporting by responsible parties or others. As a result, the problems associated with selective reporting and reporting bias in these ...
End-stage renal disease (ESRD) is characterized by total renal failure making hemodialysis or org... more End-stage renal disease (ESRD) is characterized by total renal failure making hemodialysis or organ transplantation necessary. Some ESRD can be explained by underlying disease processes such as diabetes mellitus or polycystic kidney disease (1). The etiologic origin ...
Archives of Environmental Health: An International Journal, 1988
Exposure registries are new tools for use as part of the public health response to toxic occupati... more Exposure registries are new tools for use as part of the public health response to toxic occupational and environmental exposures. These registries can help fill a gap that currently exists in the effective handling of problems following the revelation of an exposure situation. In the past, such a tool was lacking and potentially high-risk groups may not have received needed services. Recent legislation, however, has mandated the establishment of exposure registries. These registries can help provide the basis for a unified response to what historically has been described as a fragmented and ineffective approach to managing high-risk cohorts and assessing the impact of toxic substances. Certain exposure registries are now mandated by law (PL 96-510), and they have a place alongside other disease control and public health tools. Their establishment and use is not without problems, but the gain from them could be substantial. They fill a gap in the current public health response to problems of groups found to be at increased risk of exposure to toxic substances.
Mass-casualty and hospital preparedness has been analyzed widely. However, information regarding ... more Mass-casualty and hospital preparedness has been analyzed widely. However, information regarding the types of areas where these events occur is limited. Therefore, the characteristics of acute hazardous substances releases resulting in death/multiple-victim events occuring in rural/agricultural areas and in all other areas were studied and compared. Data reported to the Hazardous Substances Emergency Events Surveillance (HSEES) system from 16 state health departments during 1993-2000 were used to examine factors associated with events with death/multiple victims involving acute release of hazardous substances. A death/multiple-victim event is defined as any event resulting in a death and/or at least five people being transported to a hospital. Of a total of 43,133 events, 6661 occurred in rural/agricultural areas. Of these, 107 were death/multiple-victim events with 632 victims, of whom 91 died and 77 were hospitalized. All other areas had 472 death/multiple-victim events with 7981 victims, of whom 116 died and 413 were hospitalized. Death/multiple-victim events in rural/agricultural areas were more likely to be associated with transportation (Proportional Ratio (PR) = 4.1, 95% CI = 3.1-5.4) and fires and/or explosions (PR = 1.4, 95% CI = 0.95-2.0) than were death/multiple-victim events in all other areas. Among transportation-related events in rural/agricultural areas, 19 were associated with air transport--mainly crop dusters--and resulted in 18 deaths. Responders were three times more likely to be injured in rural/agricultural areas. Of responders, volunteer firefighters constituted 52% compared with 6.7% in all other areas. The most frequently released chemicals in rural/agricultural areas were ammonia, chlorine, and pesticides. In all other areas, ammonia, chlorine, hydrochloric acid, carbon monoxide, and 0-chlorobenzylidene malononitrile, a tearing agent often associated with an illegal or unauthorized act, were released most frequently. Findings from this analysis suggest that remedial actions should address safety measures in both transportation and fixed facilities containing acute hazardous substances. These include regular maintenance of equipment, education of workers about the substances used in their facility, rigorous training and licensing of drivers and crop duster operators, and education and training of employees and first responders in the use of protective equipment. These activities may reduce the number of events, casualties, and costs associated with hazardous substance events.
and Surveillance Branch children with elevared blood lead levels living in the vicinity of a defu... more and Surveillance Branch children with elevared blood lead levels living in the vicinity of a defunct mining and sntelring facility. Methods: Study individuals were recruitedfiom rhe 1992 Silver Valley blood lewlscrecningpanicipants. The cases were children with a bloodlead level > I 0 microgromwper deciliter (pg/dLJ. The controls were children with a blood lead level <I0 pg/dL, who were nultched ro cases by age w~d sex. Duro 012 risk factors were obtained through personnl interviews. Results: Of the variables exanzined, yurd soil remediation showed the strongest association with changes in blobdlead levels. This variable was found to be a protective factor for ebvafed blood lead levels in children (odds ratio, 0.28: confidence inrental, 0. 0 8 4 9 2). Conclusion: The results suggest rhdt ren~ovuiof lea~coniaminated,~oilfion~ residential yards was effective in reducing blood lead levels in children. INTROQUCTION The Bunker Hill Superfund site is located in Silver Valley. Idaho. Bunker ifill has been mined for lead for over 100 years and has a smelting facility that operated for over 60 years until it closedin 1981. T h e rniningand smeltingprocess wastes generated at the Bunker Hill complex during-(ha[ time left widespread lead contamination in the area (ATSDR.1989). Although remediation programs have been underway since the closure of the complex, childhood lead toxicity is a concern among area residents. Due to community concerns over lead contamination m d the fact that some children continued to have elevited blood lead levels (IDWW. 1992),the Agency for Toxic Substances and Disease Registry (ATSDR) rccruited children who voluntarily participated in the 1992 Silver Valley blood lead screening to conduct a case-control study. The study was designed u, identify which risk factors, known or suspected to affect childhood blood lcad levels. were more prevalent among children with elevated blood lcad levels.
Objective To develop a valid algorithm for identifying multiple sclerosis (MS) cases in administr... more Objective To develop a valid algorithm for identifying multiple sclerosis (MS) cases in administrative health claims (AHC) datasets. Methods We used 4 AHC datasets from the Veterans Administration (VA), Kaiser Permanente Southern California (KPSC), Manitoba (Canada), and Saskatchewan (Canada). In the VA, KPSC, and Manitoba, we tested the performance of candidate algorithms based on inpatient, outpatient, and disease-modifying therapy (DMT) claims compared to medical records review using sensitivity, specificity, positive and negative predictive values, and interrater reliability (Youden J statistic) both overall and stratified by sex and age. In Saskatchewan, we tested the algorithms in a cohort randomly selected from the general population. Results The preferred algorithm required ≥3 MS-related claims from any combination of inpatient, outpatient, or DMT claims within a 1-year time period; a 2-year time period provided little gain in performance. Algorithms including DMT claims performed better than those that did not. Sensitivity (86.6%-96.0%), specificity (66.7%-99.0%), positive predictive value (95.4%-99.0%), and interrater reliability (Youden J = 0.60-0.92) were generally stable across datasets and across strata. Some variation in performance in the stratified analyses was observed but largely reflected changes in the composition of the strata. In Saskatchewan, the preferred algorithm had a sensitivity of 96%, specificity of 99%, positive predictive value of 99%, and negative predictive value of 96%. Conclusions The performance of each algorithm was remarkably consistent across datasets. The preferred algorithm required ≥3 MS-related claims from any combination of inpatient, outpatient, or DMT use within 1 year. We recommend this algorithm as the standard AHC case definition for MS.
Exposure registries are new tools for use as part of the public health response to toxic occupati... more Exposure registries are new tools for use as part of the public health response to toxic occupational and environmental exposures. These registries can help fill a gap that currently exists in the effective handling of problems following the revelation of an exposure situation. In the past, such a tool was lacking and potentially high-risk groups may not have received needed services. Recent legislation, however, has mandated the establishment of exposure registries. These registries can help provide the basis for a unified response to what historically has been described as a fragmented and ineffective approach to managing high-risk cohorts and assessing the impact of toxic substances. Certain exposure registries are now mandated by law (PL 96-510), and they have a place alongside other disease control and public health tools. Their establishment and use is not without problems, but the gain from them could be substantial. They fill a gap in the current public health response to problems of groups found to be at increased risk of exposure to toxic substances.
Amyotrophic lateral sclerosis & frontotemporal degeneration, Dec 20, 2017
Background: Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that typically re... more Background: Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that typically results in death within 2-5 years of initial symptom onset. Multidisciplinary ALS clinics (MDCs) have been established to provide specialty care to people living with the disease. Objective: To estimate the proximity of ALS prevalence cases to the nearest MDC in the US to help evaluate one aspect of access to care. Methods: Using 2013 prevalence data from the National ALS Registry, cases were geocoded by city using geographic information system (GIS) software, along with the locations of all MDCs in operation during 2013. Case-to-MDC proximity was calculated and analyzed by sex, race, and age group. Results: During 2013, there were 72 MDCs in operation in 30 different states. A total of 15,633 ALS cases were geocoded and were distributed throughout all 50 states. Of these, 62.6% were male, 77.9% were white, and 76.2% were 50-79 years old. For overall case-to-MDC proximity, nearly half (44.9%) of all geocoded cases in the US lived 450 miles from an MDC, including approximately a quarter who lived 4100 miles from an MDC. There was a statistically significant difference between distance to MDC by race and age group. Conclusions: The high percentage of those living more than 50 miles from the nearest specialized clinic underscores one of the many challenges of ALS. Having better access to care, whether at MDCs or through other modalities, is likely key to increasing survivability and obtaining appropriate end-of-life treatment and support for people with ALS.
ABSTRACT Kimsey Addition is a neighborhood community immediately adjacent to Tinker Air Force Bas... more ABSTRACT Kimsey Addition is a neighborhood community immediately adjacent to Tinker Air Force Base in the metropolitan area of Oklahoma City. This community was exposed to airborne volatile organic compounds (VOCs) from 1956 until August 1993. The study included term live births for the periods 1956–1967 and 1975–1993. Low birth weight and mean birth weight deficit were evaluated for three defined periods. The adjusted odds ratios (ORs) for term low birth weight were elevated for the periods 1956–1967 (OR=3.09; 90% CI=0.96–10.05) and 1975–1985 (OR=2.04; 90% CI=0.54–6.85). There was a deficit in mean birth weight of 59.2 grams during 1956–1967 but not for the later periods (+54.1 grams during 1975–1985 and +8.7 grams during 1986–1993). The results suggested that residential exposure to airborne VOCs during pregnancy might have adversely affected fetal development.
Objective: To determine mortality rates for ALS in the United States, 2011-2013, using Internatio... more Objective: To determine mortality rates for ALS in the United States, 2011-2013, using International Classification of Disease, 10th Revision (ICD-10), code G12.2, Motor Neuron Disease (MND), and excluding deaths from causes other than ALS. Background: Unlike the 9th Revision, ICD-10 does not include a code specific for ALS. In addition, other non-MND conditions (e.g., supranuclear palsy) were assigned to G12.2. Past mortality studies have reported rates for G12.2 without excluding those that were not ALS which leads to inflated mortality rates. This analysis focuses only on the mortality for ALS. Methods: Mortality data for ALS were obtained from the Centers for Disease Control and Prevention, National Center for Health Statistics, for the period 2011-2013. Text strings for multiple-cause-of death were reviewed for all deaths with a code of G12.2. Deaths without ALS listed in one of the multiple-cause of death fields were excluded. ALS death rates per 100,000 persons were age-adjusted to the 2000 US standard population. Results: Approximately 21[percnt] of ICD-10 code G12.2 deaths in each year were not ALS, the majority being supranuclear palsy. There were 18,036 ALS deaths in the US during 2011-2013, mostly white (n=16,487; 91[percnt]) and male (n=10,065; 56[percnt]). The overall death rate was 1.7 per 100,000 persons (95[percnt] CI 1.7, 1.7). The death rate for males was 2.1 per 100,000 persons (95[percnt] CI 2.0, 2.1) and for females was 1.4 per 100,000 persons (95[percnt] CI 1.4, 1.4). Conclusions: Previous reports of ALS mortality in the U.S., showed similar age, sex, and race distributions. However, other reports showed slightly greater age-adjusted mortality rates due to the inclusion of other diseases in the case definition. Until there is a specific ICD-10 code for ALS, an additional review of multiple-cause of death data is required to give an accurate estimate of ALS deaths in the United States. Disclosure: Dr. Larson has nothing to disclose. Dr. Kaye has received personal compensation for activities with McKing Consulting Corporation as an employee. Dr. Mehta has nothing to disclose. Dr. Horton has nothing to disclose.
Morbidity and Mortality Weekly Report, Nov 23, 2018
Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and... more Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and fatal neuromuscular disease; the majority of ALS patients die within 2-5 years of receiving a diagnosis (1). Familial ALS, a hereditary form of the disease, accounts for 5%-10% of cases, whereas the remaining cases have no clearly defined etiology (1). ALS affects persons of all races and ethnicities; however, whites, males, non-Hispanics, persons aged ≥60 years, and those with a family history of ALS are more likely to develop the disease (2). No cure for ALS has yet been identified, and the lack of proven and effective therapeutic interventions is an ongoing challenge. Treatments currently available, Edaravone and Riluzole, do not cure ALS, but slow disease progression in certain patients (3,4). This report presents National ALS Registry findings regarding ALS prevalence in the United States for the period January 1-December 31, 2015. In 2015, the estimated prevalence of ALS cases was 5.2 per 100,000 population with a total of 16,583 cases identified. Overall, these findings are similar to the 2014 ALS prevalence and case count (5.0 per 100,000; 15,927 cases) (2). Prevalence rates by patient characteristics (most common in whites, males, and persons aged ≥60 years) and U.S. Census regions are consistent with ALS demographics and have not changed from 2014 to 2015 calendar years. The algorithm used to identify cases from national administrative databases was updated from the International Classification of Diseases, Ninth Revision (ICD-9) to the ICD-10 codes for claims starting on October 1, 2015, with no apparent effect on case ascertainment. Data collected by the National ALS Registry are being used to better describe the epidemiology of ALS in the United States and to facilitate research on the genetics, potential biomarkers, environmental pollutants, and etiology for ALS.
Background and ObjectivesRecent data suggest increasing global prevalence of multiple sclerosis (... more Background and ObjectivesRecent data suggest increasing global prevalence of multiple sclerosis (MS). Early diagnosis of MS reduces the burden of disability-adjusted life years and associated health care costs. Yet diagnostic delays persist in MS care and even within national health care systems with robust resources, comprehensive registries, and MS subspecialist referral networks. The global prevalence and characteristics of barriers to expedited MS diagnosis, particularly in resource-restricted regions, have not been extensively studied. Recent revisions to MS diagnostic criteria demonstrate potential to facilitate earlier diagnosis, but global implementation remains largely unknown.MethodsThe Multiple Sclerosis International Federation third edition of the Atlas of MS was a survey that assessed the current global state of diagnosis including adoption of MS diagnostic criteria; barriers to diagnosis with respect to the patient, health care provider, and health system; and existence of national guidelines or national standards for speed of MS diagnosis.ResultsCoordinators from 107 countries (representing approximately 82% of the world population), participated. Eighty-three percent reported at least 1 “major barrier” to early MS diagnosis. The most frequently reported barriers included the following: “lack of awareness of MS symptoms among general public” (68%), “lack of awareness of MS symptoms among health care professionals” (59%), and “lack of availability of health care professionals with knowledge to diagnose MS” (44%). One-third reported lack of “specialist medical equipment or diagnostic tests.” Thirty-four percent reported the use of only 2017 McDonald criteria (McD-C) for diagnosis, and 79% reported 2017 McD-C as the “most commonly used criteria.” Sixty-six percent reported at least 1 barrier to the adoption of 2017 McD-C, including “neurologists lack awareness or training” by 45%. There was no significant association between national guidelines pertaining to MS diagnosis or practice standards addressing the speed of diagnosis and presence of barriers to early MS diagnosis and implementation of 2017 McD-C.DiscussionThis study finds pervasive consistent global barriers to early diagnosis of MS. While these barriers reflected a lack of resources in many countries, data also suggest that interventions designed to develop and implement accessible education and training can provide cost-effective opportunities to improve access to early MS diagnosis.
Background-Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that typically res... more Background-Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that typically results in death within 2-5 years of initial symptom onset. Multidisciplinary ALS clinics (MDCs) have been established to provide specialty care to people living with the disease. Objective-To estimate the proximity of ALS prevalence cases to the nearest MDC in the US to help evaluate one aspect of access to care. Methods-Using 2013 prevalence data from the National ALS Registry, cases were geocoded by city using geographic information system (GIS) software, along with the locations of all MDCs in operation during 2013. Case-to-MDC proximity was calculated and analyzed by sex, race, and age group. Results-During 2013, there were 72 MDCs in operation in 30 different states. A total of 15,633 ALS cases were geocoded and were distributed throughout all 50 states. Of these, 62.6% were male, 77.9% were white, and 76.2% were 50-79 years old. For overall case-to-MDC proximity, nearly half (44.9%) of all geocoded cases in the US lived >50 miles from an MDC, including
Our objective was to evaluate the completeness of the United States National ALS Registry (Regist... more Our objective was to evaluate the completeness of the United States National ALS Registry (Registry). We compared persons with ALS who were passively identified by the Registry with those actively identified in the State and Metropolitan Area ALS Surveillance project. Cases in the two projects were matched using a combination of identifiers, including, partial social security number, name, date of birth, and sex. The distributions of cases from the two projects that matched/did not match were compared and Chi-square tests conducted to determine statistical significance. There were 5883 ALS cases identified by the surveillance project. Of these, 1116 died before the Registry started, leaving 4767 cases. We matched 2720 cases from the surveillance project to those in the Registry. The cases identified by the surveillance project that did not match cases in the Registry were more likely to be non-white, Hispanic, less than 65 years of age, and from western states. The methods used by the Registry to identify ALS cases, i.e. national administrative data and self-registration, worked well but missed cases. These findings suggest that developing strategies to identify and promote the Registry to those who were more likely to be missing, e.g. non-white and Hispanic, could be beneficial to improving the completeness of the Registry.
The Agency for Toxic Substances and Disease Registry (ATSDR) conducted a cross-sectional health s... more The Agency for Toxic Substances and Disease Registry (ATSDR) conducted a cross-sectional health study at the Fort Hall Indian Reservation in Idaho to investigate concerns about the health effects on reservation residents that might be attributed to two phosphate-processing plants located near the reservation`s southern border. In addition to increased particulates, air emissions from these plants included phosphorus pentoxide, cadmium, chromium, fluoride, uranium, and its daughter radionuclides. A total of 515 participants -- 229 from Fort Hall and 286 from a comparison group at the Duck Valley Indian Reservation -- were interviewed in person by trained American Indian interviewers. Approximately 100 residents of each reservation performed pulmonary function tests and provided urine specimens that were analyzed for cadmium, chromium, fluoride, and several renal biomarkers.
A human exposure study was conducted to determine the effect of mine tailings contaminated with l... more A human exposure study was conducted to determine the effect of mine tailings contaminated with lead, arsenic, and cadmium (Silver Creek Mine Tailings Site, Utah) on biological levels of these elements among persons living in the immediate vicinity. Cross-sectional data were collected from the target area and from a comparison area located off site. Biological measurements included blood lead, erythrocytic protoporphyrin, hemoglobin, urine beta-2-microglobulin, urine arsenic, and urine cadmium (adjusted to urine creatinine and specific gravity). In addition, all participants were surveyed to identify independent or potentially confounding factors that might influence the measurements. There was no evidence of excess exposure to lead, arsenic, or cadmium at clinically meaningful levels among residents living on or adjacent to the site. Although blood lead levels were higher in the target area than in the comparison area among children 9-71 months of age, the differing values were within the normal range. Further, the mean blood lead levels for both areas were well below the national mean levels (1976-1980) for this age group. Other behavioral factors found to increase blood lead levels in this age group were identified.
AbstractObjective: To develop a valid algorithm for identifying multiple sclerosis (MS) cases in ... more AbstractObjective: To develop a valid algorithm for identifying multiple sclerosis (MS) cases in administrative health claims (AHC) datasets. Methods: We used 4 AHC datasets from the Veterans Administration (VA), Kaiser Permanente Southern California (KPSC), Manitoba (Canada), and Saskatchewan (Canada). In the VA, KPSC, and Manitoba, we tested the performance of candidate algorithms based on inpatient, outpatient, and disease-modifying therapy (DMT) claims compared to medical records review using sensitivity, specificity, positive and negative predictive values, and interrater reliability (Youden J statistic) both overall and stratified by sex and age. In Saskatchewan, we tested the algorithms in a cohort randomly selected from the general population. Results: The preferred algorithm required ≥3 MS-related claims from any combination of inpatient, outpatient, or DMT claims within a 1-year time period; a 2-year time period provided little gain in performance. Algorithms including DMT...
ImportanceRacial, ethnic, and geographic differences in multiple sclerosis (MS) are important fac... more ImportanceRacial, ethnic, and geographic differences in multiple sclerosis (MS) are important factors to assess when determining the disease burden and allocating health care resources.ObjectiveTo calculate the US prevalence of MS in Hispanic, non-Hispanic Black (hereafter referred to as Black), and non-Hispanic White individuals (hereafter referred to as White) stratified by age, sex, and region.Design, Setting, and ParticipantsA validated algorithm was applied to private, military, and public (Medicaid and Medicare) administrative health claims data sets to identify adult cases of MS between 2008 and 2010. Data analysis took place between 2019 and 2022. The 3-year cumulative prevalence overall was determined in each data set and stratified by age, sex, race, ethnicity, and geography. The insurance pools included 96 million persons from 2008 to 2010. Insurance and stratum-specific estimates were applied to the 2010 US Census data and the findings combined to calculate the 2010 prev...
All hazardous substances spill databases created before the inception of HSEES were developed as ... more All hazardous substances spill databases created before the inception of HSEES were developed as passive reporting systems, relying on voluntary reporting by responsible parties or others. As a result, the problems associated with selective reporting and reporting bias in these ...
End-stage renal disease (ESRD) is characterized by total renal failure making hemodialysis or org... more End-stage renal disease (ESRD) is characterized by total renal failure making hemodialysis or organ transplantation necessary. Some ESRD can be explained by underlying disease processes such as diabetes mellitus or polycystic kidney disease (1). The etiologic origin ...
Archives of Environmental Health: An International Journal, 1988
Exposure registries are new tools for use as part of the public health response to toxic occupati... more Exposure registries are new tools for use as part of the public health response to toxic occupational and environmental exposures. These registries can help fill a gap that currently exists in the effective handling of problems following the revelation of an exposure situation. In the past, such a tool was lacking and potentially high-risk groups may not have received needed services. Recent legislation, however, has mandated the establishment of exposure registries. These registries can help provide the basis for a unified response to what historically has been described as a fragmented and ineffective approach to managing high-risk cohorts and assessing the impact of toxic substances. Certain exposure registries are now mandated by law (PL 96-510), and they have a place alongside other disease control and public health tools. Their establishment and use is not without problems, but the gain from them could be substantial. They fill a gap in the current public health response to problems of groups found to be at increased risk of exposure to toxic substances.
Mass-casualty and hospital preparedness has been analyzed widely. However, information regarding ... more Mass-casualty and hospital preparedness has been analyzed widely. However, information regarding the types of areas where these events occur is limited. Therefore, the characteristics of acute hazardous substances releases resulting in death/multiple-victim events occuring in rural/agricultural areas and in all other areas were studied and compared. Data reported to the Hazardous Substances Emergency Events Surveillance (HSEES) system from 16 state health departments during 1993-2000 were used to examine factors associated with events with death/multiple victims involving acute release of hazardous substances. A death/multiple-victim event is defined as any event resulting in a death and/or at least five people being transported to a hospital. Of a total of 43,133 events, 6661 occurred in rural/agricultural areas. Of these, 107 were death/multiple-victim events with 632 victims, of whom 91 died and 77 were hospitalized. All other areas had 472 death/multiple-victim events with 7981 victims, of whom 116 died and 413 were hospitalized. Death/multiple-victim events in rural/agricultural areas were more likely to be associated with transportation (Proportional Ratio (PR) = 4.1, 95% CI = 3.1-5.4) and fires and/or explosions (PR = 1.4, 95% CI = 0.95-2.0) than were death/multiple-victim events in all other areas. Among transportation-related events in rural/agricultural areas, 19 were associated with air transport--mainly crop dusters--and resulted in 18 deaths. Responders were three times more likely to be injured in rural/agricultural areas. Of responders, volunteer firefighters constituted 52% compared with 6.7% in all other areas. The most frequently released chemicals in rural/agricultural areas were ammonia, chlorine, and pesticides. In all other areas, ammonia, chlorine, hydrochloric acid, carbon monoxide, and 0-chlorobenzylidene malononitrile, a tearing agent often associated with an illegal or unauthorized act, were released most frequently. Findings from this analysis suggest that remedial actions should address safety measures in both transportation and fixed facilities containing acute hazardous substances. These include regular maintenance of equipment, education of workers about the substances used in their facility, rigorous training and licensing of drivers and crop duster operators, and education and training of employees and first responders in the use of protective equipment. These activities may reduce the number of events, casualties, and costs associated with hazardous substance events.
and Surveillance Branch children with elevared blood lead levels living in the vicinity of a defu... more and Surveillance Branch children with elevared blood lead levels living in the vicinity of a defunct mining and sntelring facility. Methods: Study individuals were recruitedfiom rhe 1992 Silver Valley blood lewlscrecningpanicipants. The cases were children with a bloodlead level > I 0 microgromwper deciliter (pg/dLJ. The controls were children with a blood lead level <I0 pg/dL, who were nultched ro cases by age w~d sex. Duro 012 risk factors were obtained through personnl interviews. Results: Of the variables exanzined, yurd soil remediation showed the strongest association with changes in blobdlead levels. This variable was found to be a protective factor for ebvafed blood lead levels in children (odds ratio, 0.28: confidence inrental, 0. 0 8 4 9 2). Conclusion: The results suggest rhdt ren~ovuiof lea~coniaminated,~oilfion~ residential yards was effective in reducing blood lead levels in children. INTROQUCTION The Bunker Hill Superfund site is located in Silver Valley. Idaho. Bunker ifill has been mined for lead for over 100 years and has a smelting facility that operated for over 60 years until it closedin 1981. T h e rniningand smeltingprocess wastes generated at the Bunker Hill complex during-(ha[ time left widespread lead contamination in the area (ATSDR.1989). Although remediation programs have been underway since the closure of the complex, childhood lead toxicity is a concern among area residents. Due to community concerns over lead contamination m d the fact that some children continued to have elevited blood lead levels (IDWW. 1992),the Agency for Toxic Substances and Disease Registry (ATSDR) rccruited children who voluntarily participated in the 1992 Silver Valley blood lead screening to conduct a case-control study. The study was designed u, identify which risk factors, known or suspected to affect childhood blood lcad levels. were more prevalent among children with elevated blood lcad levels.
Objective To develop a valid algorithm for identifying multiple sclerosis (MS) cases in administr... more Objective To develop a valid algorithm for identifying multiple sclerosis (MS) cases in administrative health claims (AHC) datasets. Methods We used 4 AHC datasets from the Veterans Administration (VA), Kaiser Permanente Southern California (KPSC), Manitoba (Canada), and Saskatchewan (Canada). In the VA, KPSC, and Manitoba, we tested the performance of candidate algorithms based on inpatient, outpatient, and disease-modifying therapy (DMT) claims compared to medical records review using sensitivity, specificity, positive and negative predictive values, and interrater reliability (Youden J statistic) both overall and stratified by sex and age. In Saskatchewan, we tested the algorithms in a cohort randomly selected from the general population. Results The preferred algorithm required ≥3 MS-related claims from any combination of inpatient, outpatient, or DMT claims within a 1-year time period; a 2-year time period provided little gain in performance. Algorithms including DMT claims performed better than those that did not. Sensitivity (86.6%-96.0%), specificity (66.7%-99.0%), positive predictive value (95.4%-99.0%), and interrater reliability (Youden J = 0.60-0.92) were generally stable across datasets and across strata. Some variation in performance in the stratified analyses was observed but largely reflected changes in the composition of the strata. In Saskatchewan, the preferred algorithm had a sensitivity of 96%, specificity of 99%, positive predictive value of 99%, and negative predictive value of 96%. Conclusions The performance of each algorithm was remarkably consistent across datasets. The preferred algorithm required ≥3 MS-related claims from any combination of inpatient, outpatient, or DMT use within 1 year. We recommend this algorithm as the standard AHC case definition for MS.
Exposure registries are new tools for use as part of the public health response to toxic occupati... more Exposure registries are new tools for use as part of the public health response to toxic occupational and environmental exposures. These registries can help fill a gap that currently exists in the effective handling of problems following the revelation of an exposure situation. In the past, such a tool was lacking and potentially high-risk groups may not have received needed services. Recent legislation, however, has mandated the establishment of exposure registries. These registries can help provide the basis for a unified response to what historically has been described as a fragmented and ineffective approach to managing high-risk cohorts and assessing the impact of toxic substances. Certain exposure registries are now mandated by law (PL 96-510), and they have a place alongside other disease control and public health tools. Their establishment and use is not without problems, but the gain from them could be substantial. They fill a gap in the current public health response to problems of groups found to be at increased risk of exposure to toxic substances.
Amyotrophic lateral sclerosis & frontotemporal degeneration, Dec 20, 2017
Background: Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that typically re... more Background: Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that typically results in death within 2-5 years of initial symptom onset. Multidisciplinary ALS clinics (MDCs) have been established to provide specialty care to people living with the disease. Objective: To estimate the proximity of ALS prevalence cases to the nearest MDC in the US to help evaluate one aspect of access to care. Methods: Using 2013 prevalence data from the National ALS Registry, cases were geocoded by city using geographic information system (GIS) software, along with the locations of all MDCs in operation during 2013. Case-to-MDC proximity was calculated and analyzed by sex, race, and age group. Results: During 2013, there were 72 MDCs in operation in 30 different states. A total of 15,633 ALS cases were geocoded and were distributed throughout all 50 states. Of these, 62.6% were male, 77.9% were white, and 76.2% were 50-79 years old. For overall case-to-MDC proximity, nearly half (44.9%) of all geocoded cases in the US lived 450 miles from an MDC, including approximately a quarter who lived 4100 miles from an MDC. There was a statistically significant difference between distance to MDC by race and age group. Conclusions: The high percentage of those living more than 50 miles from the nearest specialized clinic underscores one of the many challenges of ALS. Having better access to care, whether at MDCs or through other modalities, is likely key to increasing survivability and obtaining appropriate end-of-life treatment and support for people with ALS.
ABSTRACT Kimsey Addition is a neighborhood community immediately adjacent to Tinker Air Force Bas... more ABSTRACT Kimsey Addition is a neighborhood community immediately adjacent to Tinker Air Force Base in the metropolitan area of Oklahoma City. This community was exposed to airborne volatile organic compounds (VOCs) from 1956 until August 1993. The study included term live births for the periods 1956–1967 and 1975–1993. Low birth weight and mean birth weight deficit were evaluated for three defined periods. The adjusted odds ratios (ORs) for term low birth weight were elevated for the periods 1956–1967 (OR=3.09; 90% CI=0.96–10.05) and 1975–1985 (OR=2.04; 90% CI=0.54–6.85). There was a deficit in mean birth weight of 59.2 grams during 1956–1967 but not for the later periods (+54.1 grams during 1975–1985 and +8.7 grams during 1986–1993). The results suggested that residential exposure to airborne VOCs during pregnancy might have adversely affected fetal development.
Objective: To determine mortality rates for ALS in the United States, 2011-2013, using Internatio... more Objective: To determine mortality rates for ALS in the United States, 2011-2013, using International Classification of Disease, 10th Revision (ICD-10), code G12.2, Motor Neuron Disease (MND), and excluding deaths from causes other than ALS. Background: Unlike the 9th Revision, ICD-10 does not include a code specific for ALS. In addition, other non-MND conditions (e.g., supranuclear palsy) were assigned to G12.2. Past mortality studies have reported rates for G12.2 without excluding those that were not ALS which leads to inflated mortality rates. This analysis focuses only on the mortality for ALS. Methods: Mortality data for ALS were obtained from the Centers for Disease Control and Prevention, National Center for Health Statistics, for the period 2011-2013. Text strings for multiple-cause-of death were reviewed for all deaths with a code of G12.2. Deaths without ALS listed in one of the multiple-cause of death fields were excluded. ALS death rates per 100,000 persons were age-adjusted to the 2000 US standard population. Results: Approximately 21[percnt] of ICD-10 code G12.2 deaths in each year were not ALS, the majority being supranuclear palsy. There were 18,036 ALS deaths in the US during 2011-2013, mostly white (n=16,487; 91[percnt]) and male (n=10,065; 56[percnt]). The overall death rate was 1.7 per 100,000 persons (95[percnt] CI 1.7, 1.7). The death rate for males was 2.1 per 100,000 persons (95[percnt] CI 2.0, 2.1) and for females was 1.4 per 100,000 persons (95[percnt] CI 1.4, 1.4). Conclusions: Previous reports of ALS mortality in the U.S., showed similar age, sex, and race distributions. However, other reports showed slightly greater age-adjusted mortality rates due to the inclusion of other diseases in the case definition. Until there is a specific ICD-10 code for ALS, an additional review of multiple-cause of death data is required to give an accurate estimate of ALS deaths in the United States. Disclosure: Dr. Larson has nothing to disclose. Dr. Kaye has received personal compensation for activities with McKing Consulting Corporation as an employee. Dr. Mehta has nothing to disclose. Dr. Horton has nothing to disclose.
Morbidity and Mortality Weekly Report, Nov 23, 2018
Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and... more Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and fatal neuromuscular disease; the majority of ALS patients die within 2-5 years of receiving a diagnosis (1). Familial ALS, a hereditary form of the disease, accounts for 5%-10% of cases, whereas the remaining cases have no clearly defined etiology (1). ALS affects persons of all races and ethnicities; however, whites, males, non-Hispanics, persons aged ≥60 years, and those with a family history of ALS are more likely to develop the disease (2). No cure for ALS has yet been identified, and the lack of proven and effective therapeutic interventions is an ongoing challenge. Treatments currently available, Edaravone and Riluzole, do not cure ALS, but slow disease progression in certain patients (3,4). This report presents National ALS Registry findings regarding ALS prevalence in the United States for the period January 1-December 31, 2015. In 2015, the estimated prevalence of ALS cases was 5.2 per 100,000 population with a total of 16,583 cases identified. Overall, these findings are similar to the 2014 ALS prevalence and case count (5.0 per 100,000; 15,927 cases) (2). Prevalence rates by patient characteristics (most common in whites, males, and persons aged ≥60 years) and U.S. Census regions are consistent with ALS demographics and have not changed from 2014 to 2015 calendar years. The algorithm used to identify cases from national administrative databases was updated from the International Classification of Diseases, Ninth Revision (ICD-9) to the ICD-10 codes for claims starting on October 1, 2015, with no apparent effect on case ascertainment. Data collected by the National ALS Registry are being used to better describe the epidemiology of ALS in the United States and to facilitate research on the genetics, potential biomarkers, environmental pollutants, and etiology for ALS.
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