Gender difference in clinical conditions among hospitalized adults with myotonic dystrophy.

INTRODUCTION: In this study we examined gender differences in adult hospitalizations with myotonic dystrophy (DM). METHODS: From the Nationwide Inpatient Sample (NIS) 2010-2014, we identified 1,891 adult hospitalizations with a DM diagnosis and constructed a comparison group of hospitalizations without DM using propensity score matching. We calculated relative risk by gender for 44 clinical diagnoses that each accounted for at least 5% of DM hospitalizations. RESULTS: Hospitalizations with DM were longer (4.8 vs. 4.1 days, P < 0.0001) and more costly ($13,241 vs. $11,458, P < 0.0001) than those without DM. More than half (25 of 44) of the conditions co-occurring with DM hospitalizations did not differ in their relative risks by gender. For those that differed by gender, only 5 were specific to DM, compared with hospitalizations without DM. DISCUSSION: Our findings highlight the importance of comprehensive and coordinated care for DM rather than gender-oriented care in the inpatient setting. Muscle Nerve 59:348-353, 2019.

Differences in Length of Stay and Costs Between Comparable Hospitalizations of Patients With Spina Bifida With or Without Pressure Injuries.

OBJECTIVE: To estimate differences in the length of stay and costs for comparable hospitalizations of patients with spina bifida (SB) with and without pressure injuries. DESIGN: Retrospective, cross-sectional, observational study. SETTING: Nationwide Inpatient Sample from years 2010-2014. PARTICIPANTS: Hospitalizations of patients with SB (N=7776). Hospitalizations among patients with SB and pressure injuries (n=3888) were matched to hospitalizations among patients with SB but without pressure injuries (n=3888). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Differences in length of stay and total costs between the 2 groups. RESULTS: After successful matching, multivariate modeling of costs and length of stay on matched sample showed that hospitalizations with pressure injuries had an increased 1.2 inpatient days and excess average costs of $1182 in 2014 dollars. CONCLUSIONS: The estimated average cost of hospitalization increased by 10%, and the estimated average length of stay increased by 24% in the presence of pressure injuries among hospitalized patients with SB, compared with their peers without these injuries. These results highlight the substantial morbidity associated with pressure injuries, which are potentially preventable before or during hospitalizations among persons with SB.

Clinical Follow-Up for Duchenne Muscular Dystrophy Newborn Screening: A Proposal.

New developments in the rapid diagnosis and treatment of boys with Duchenne muscular dystrophy (DMD) have led to growing enthusiasm for instituting DMD newborn screening (NBS) in the United States. Our group has been interested in developing clinical guidance to be implemented consistently in specialty care clinics charged with the care of presymptomatically identified newborns referred after DMD-NBS. We reviewed the existing literature covering patient-centered clinical follow-up after NBS, educational material from public health and advocacy sites, and federal recommendations on effective NBS follow-up. We discussed the review as a group and added our own experience to develop materials suitable for initial parent and primary care provider education. These materials and a series of templates for subspecialist encounters could be used to provide consistent care across centers and serve as the basis for ongoing quality improvement. Muscle Nerve 54: 186-191, 2016.

Characteristics and survival of patients with end stage renal disease and spina bifida in the United States renal data system.

PURPOSE: We describe the characteristics, treatments and survival of patients with spina bifida in whom end stage renal disease developed from 2004 through 2008 in the United States Renal Data System. MATERIALS AND METHODS: We used ICD-9-CM code 741.* to identify individuals with spina bifida using hospital inpatient data from 1977 to 2010, and physician and facility claims from 2004 to 2008. We constructed a 5:1 comparison group of patients with end stage renal disease without spina bifida matched by age at first end stage renal disease service, gender and race/ethnicity. We assessed the risk of mortality and of renal transplantation while on dialysis using multivariate cause specific proportional hazards survival analysis. We also compared survival after the first renal transplant from the first end stage renal disease service to August 2011. RESULTS: We identified 439 patients with end stage renal disease and spina bifida in whom end stage renal disease developed at an average younger age than in patients without spina bifida (41 vs 62 years, p <0.001) and in whom urological issues were the most common primary cause of end stage renal disease. Compared to patients with end stage renal disease without spina bifida those who had spina bifida showed a similar mortality hazard on dialysis and after transplantation. However, patients with end stage renal disease without spina bifida were more likely to undergo renal transplantation than patients with spina bifida (HR 1.51, 95% CI 1.13-2.03). Hospitalizations related to urinary tract infections were positively associated with the risk of death on dialysis in patients with end stage renal disease and spina bifida (HR 1.42, 95% CI 1.33-1.53). CONCLUSIONS: Spina bifida was not associated with increased mortality in patients with end stage renal disease on dialysis or after renal transplantation. Proper urological and bladder management is imperative in patients with spina bifida, particularly in adults.

Developing an integrated strategy to reduce ethnic and racial disparities in the delivery of clinical preventive services for older Americans.

OBJECTIVES: To determine the optimum strategy for increasing up-to-date (UTD) levels in older Americans, while reducing disparities between White, Black, and Hispanic adults, aged 65 years and older. METHODS: Data were analyzed from the 2008 Behavioral Risk Factor Surveillance System, quantifying the proportion of older Americans UTD with influenza and pneumococcal vaccinations, mammograms, Papanicolaou tests, and colorectal cancer screening. A comparison of projected changes in UTD levels and disparities was ascertained by numerically accounting for UTD adults lacking 1 or more clinical preventive services (CPS). Analyses were performed by gender and race/ethnicity. RESULTS: Expanded provision of specific vaccinations and screenings each increased UTD levels. When those needing only vaccinations were immunized, there was a projected decrease in racial/ethnic disparities in UTD levels (2.3%-12.2%). When those needing only colorectal cancer screening, mammography, or Papanicolaou test were screened, there was an increase in UTD disparities (1.6%-4.5%). CONCLUSIONS: A primary care and public health focus on adult immunizations, in addition to other CPS, offers an effective strategy to reduce disparities while improving UTD levels.

Health risk behaviors among young adults with spina bifida.

AIM: Persons with spina bifida who adopt unhealthy lifestyles could be at increased risk of adverse health outcomes because the presence of spina bifida may magnify this risk. We estimated overall and age-specific prevalence of selected health risk behaviors (HRBs) in young people with spina bifida and examined the association between HRBs and depression. METHOD: We performed analyses on data obtained from individuals with spina bifida (n=130; mean age 23y SD 4y 5mo; 64 males, 66 females; 64% lumbosacral lesion; 77% with shunt) who participated in a population-based survey conducted by the Arkansas Spinal Cord Commission in 2005. RESULTS: Compared with national estimates, young people with spina bifida tend to eat less healthy diets, do less exercise, and engage in more sedentary activities. Respondents were less likely to use substances (alcohol, tobacco, illegal drugs), which peaked among 25 to 31 year olds. About 90% saw a doctor in the previous year. Nearly one half reported mild or major depressive symptoms. In the logistic regression analysis after controlling for potential confounders (age, sex, ethnic group, education, employment, marital status, living arrangement, level of lesion, presence of shunt, mobility, self-rated health and healthcare utilization), major depressive symptoms were associated with current alcohol drinking (adjusted odds ratio: 4.74; 95% CI 1.18-19.04). INTERPRETATION: Young adults with spina bifida exhibit unhealthy behaviors that continue into their late 20s. The findings highlight the need to increase awareness of their health risk profiles in the spina bifida community and show opportunities for mental health and health risk screening and counseling by healthcare providers.

Differences in the prevalence and severity of arthritis among racial/ethnic groups in the United States, National Health Interview Survey, 2002, 2003, and 2006.

We describe the prevalence of doctor-diagnosed arthritis and its impact on activities, work, and joint pain for 6 racial/ethnic groups: non-Hispanic whites, non-Hispanic blacks, Hispanics, American Indians/Alaska Natives, Asians and Pacific Islanders, and multiracial or "other" respondents. We combined data from the 2002, 2003, and 2006 National Health Interview Survey (n = 85,784) and, after adjusting for age, sex, and body mass index, compared racial/ethnic differences. Arthritis-attributable activity limitation, arthritis-attributable work limitation, and severe joint pain were higher for non-Hispanic blacks, Hispanics, and multiracial or other respondents with arthritis compared with non-Hispanic whites with arthritis. Our finding that arthritis disproportionately affects certain racial/ethnic minorities may be useful for planning interventions.

Fragile X Syndrome-Associated Emergency Department Visits in the United States, 2006-2011.

Using national data, we examined emergency department (ED) encounters during 2006-2011 for which a diagnosis code for fragile X syndrome (FXS) was present (n = 7,217). Almost half of ED visits coded for FXS resulted in hospitalization, which is much higher than for ED visits not coded for FXS. ED visits among females coded for FXS were slightly more likely to result in hospitalization. These findings underscore the importance of surveillance systems that could accurately identify individuals with FXS, track healthcare utilization and co-occurring conditions, and monitor quality of care in order to improve care and reduce FXS-associated morbidity.

State and metropolitan variation in lack of health insurance among working-age adults, Behavioral Risk Factor Surveillance System, 2006.

OBJECTIVE: Lack of health insurance coverage for working-age adults is one of the most pressing issues facing the U.S. population, and it continues to be a concern for a large number of people. In the absence of a national solution, the states and municipalities are left to address this need. We examined the disparities in uninsurance prevalence by state and metropolitan areas in the U.S. and among racial/ethnic groups. METHOD: Data from the 2006 Behavioral Risk Factor Surveillance System (BRFSS) were analyzed for working-age adults 18 to 64 years of age. RESULTS: In 2006, according to the BRFSS data, overall 18.6% (standard error = 0.20) of working-age adults were without health insurance coverage; by state, this proportion ranged from 9.7% to 29.0%. Health insurance coverage varied by state and metropolitan area and racial/ethnic group, and a higher age-adjusted prevalence of uninsurance was observed for non-Hispanic black and Hispanic respondents. CONCLUSIONS: A substantial proportion of working-age Americans remain without health insurance coverage. Disparities in health insurance coverage were observed by population and geographic groups. Overall, black and Hispanic populations fared far worse in terms of lack of health-care coverage than working-age white Americans.

Which one? What kind? How many? Types, causes, and prevalence of disability among U.S. adults.

BACKGROUND: Quantifying the number of people with and types of disabilities is helpful for medical, policy, and public health planning. OBJECTIVE/HYPOTHESIS: To update prior estimates on types, prevalence, and main causes of disability among U.S. adults using the Survey of Income and Program Participation (SIPP) data. METHODS: We used cross-sectional data from the SIPP 2008 Panel Wave 6 interviews collected May-August 2010. Analyses were restricted to non-institutionalized adults ages ≥18 years (n = 66,410). Disability was ascertained via five non-mutually exclusive components: 1) specific activity difficulties, 2) selected impairments, 3) use of an assistive aid, 4) household work limitations, and 5) paid work limitations. Prioritized main cause of disability was established for the 95% of respondents with a disability type eligible for health condition questions. We generated weighted population estimates (number and percentage, with 95% confidence intervals (CIs)), accounting for the complex sample survey design. RESULTS: 50 million U.S. adults (21.8%) experienced a disability in 2010. Mobility-related activity limitations were the most prevalent disabilities across all five components. The most common main causes of disability were arthritis/rheumatism, 9.1 million (19.2%, 95% CI = 18.4-20.0) and back or spine problems, 8.9 million (18.6%, 95% CI = 17.9-19.3). CONCLUSIONS: A growing population with disabilities has the potential to put considerable and unsustainable demand on medical, public health, and senior service systems. Strengthening clinical community linkages and expanding the availability of existing evidence-based public health interventions to prevent, delay, and mitigate the effects of disability could improve health and outcomes for people with disabilities.

Delivery of preventive services to adults aged 50-64: monitoring performance using a composite measure, 1997-2004.

OBJECTIVE: Population-based rates for the delivery of adult vaccinations or screenings are typically tracked as individual services. The current approach is useful in monitoring progress toward national health goals but does not yield information regarding how many U.S. adults receive a combination of preventive services routinely recommended based on a person's age and gender. A composite measure is important for policymaking, for developing public health interventions, and for monitoring the quality of clinical care. During the period under study, influenza vaccination was newly recommended (2000) to be routinely delivered to adults in this age range. The objective of the study was to compare the delivery of routine clinical preventive services to U.S. adults aged 50-64 years between 1997 and 2004 using a composite measure that includes cancer screenings and vaccinations. DESIGN: Data were collected via telephone surveys in 1997, 2002, and 2004 as part of the Behavioral Risk Factor Surveillance System. PARTICIPANTS: The participants were randomly selected adults aged 50-64 years in the 50 states and the District of Columbia in the selected years. Sample sizes ranged from 24,917 to 77,244. MEASUREMENTS AND MAIN RESULTS: The composite measure includes screening for colorectal cancer, cervical cancer, breast cancer, and vaccination against influenza (2002 and 2004 only). The composite measure quantifies the percentage of adults who are up-to-date with the complete set according to recommended schedules. With the inclusion of newly recommended influenza vaccination, the percentage of men and women aged 50-64 who were up-to-date on all selected measures in 2004 was 23.4% and 23.0%, respectively, compared with 37.6% and 30.5% in 1997. Without including influenza vaccination, the percentage of up-to-date adults aged 50-64 would have risen in 2004 to 50.5% (men) and to 44.7% (women). For both sexes, results varied by education, race/ethnicity, marriage status, insurance status, health status, and state. CONCLUSION: In 2004, the percentage of adults aged 50-64 years receiving routinely recommended cancer screenings and influenza vaccination was low with fewer than 1 in 4 being up to date.

Evaluating Implementation of the Updated Care Considerations for Duchenne Muscular Dystrophy.

Care Considerations for Duchenne Muscular Dystrophy were published in 2010. However, little is known about the extent to which these considerations were implemented after publication. With this article, we provide direction on evaluating the uptake of the 2018 Duchenne Muscular Dystrophy Care Considerations. We identify key elements of care and present suggestions for their use in evaluation and research.

Muscular Dystrophy Surveillance, Tracking, and Research Network pilot: Population-based surveillance of major muscular dystrophies at four U.S. sites, 2007-2011.

BACKGROUND: For 10 years, the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet) conducted surveillance for Duchenne and Becker muscular dystrophy (DBMD). We piloted expanding surveillance to other MDs that vary in severity, onset, and sources of care. METHODS: Our retrospective surveillance included individuals diagnosed with one of nine eligible MDs before or during the study period (January 2007-December 2011), one or more health encounters, and residence in one of four U.S. sites (Arizona, Colorado, Iowa, or western New York) at any time within the study period. We developed case definitions, surveillance protocols, and software applications for medical record abstraction, clinical review, and data pooling. Potential cases were identified by International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes 359.0, 359.1, and 359.21 and International Classification of Diseases, Tenth Revision (ICD-10) codes G71.0 and G71.1. Descriptive statistics were compared by MD type. Percentage of MD cases identified by each ICD-9-CM code was calculated. RESULTS: Of 2,862 cases, 32.9% were myotonic, dystrophy 25.8% DBMD, 9.7% facioscapulohumeral MD, and 9.1% limb-girdle MD. Most cases were male (63.6%), non-Hispanic (59.8%), and White (80.2%). About, half of cases were genetically diagnosed in self (39.1%) or family (6.2%). About, half had a family history of MD (48.9%). The hereditary progressive MD code (359.1) was the most common code for identifying eligible cases. The myotonic code (359.21) identified 83.4% of eligible myotonic dystrophy cases (786/943). CONCLUSIONS: MD STARnet is the only multisite, population-based active surveillance system available for MD in the United States. Continuing our expanded surveillance will contribute important epidemiologic and health outcome information about several MDs.

Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management.

A coordinated, multidisciplinary approach to care is essential for optimum management of the primary manifestations and secondary complications of Duchenne muscular dystrophy (DMD). Contemporary care has been shaped by the availability of more sensitive diagnostic techniques and the earlier use of therapeutic interventions, which have the potential to improve patients' duration and quality of life. In part 2 of this update of the DMD care considerations, we present the latest recommendations for respiratory, cardiac, bone health and osteoporosis, and orthopaedic and surgical management for boys and men with DMD. Additionally, we provide guidance on cardiac management for female carriers of a disease-causing mutation. The new care considerations acknowledge the effects of long-term glucocorticoid use on the natural history of DMD, and the need for care guidance across the lifespan as patients live longer. The management of DMD looks set to change substantially as new genetic and molecular therapies become available.

Receipt of preventive services by elders based on composite measures, 1997-2004.

BACKGROUND: The receipt of routine vaccinations and cancer screening is typically tracked separately. Monitoring trends in this way does not measure the overall protection conferred by these services on a target population. DESIGN: Telephone surveys were conducted in 1997, 2002, and 2004 as part of the Behavioral Risk Factor Surveillance System. SETTING/PARTICIPANTS: Randomly selected adults aged 65 and older from 49 states and Washington DC. MAIN OUTCOME MEASURES: Statistically significant changes (p <0.05) in a composite measure of the prevalence of U.S. men aged 65 or older who were up to date with recommendations for colorectal cancer screening, influenza vaccination, pneumococcal vaccination; and for the prevalence of U.S. women aged 65 or older who were up to date for these measures as well as for Pap test and screening mammography. RESULTS: The percentage of men who reported being up to date on all tests increased from 24.4% (1997) to 39.6% (2002) to 41.0% (2004), and the percentage of women increased from 18.6% (1997) to 32.4% (2002) to 32.5% (2004). For both groups, results varied by education, race/ethnicity, marital status, insurance status, and state, as well as whether they had a personal doctor. CONCLUSIONS: Despite increases between 1997 and 2004 in the reported receipt of individual cancer screenings and vaccinations among U.S. adults aged 65 or older, approximately seven of ten women and six of ten men were not up to date on these routine preventive services in 2004.

Health insurance coverage and use of selected preventive services by working-age women, BRFSS, 2006.

Access to healthcare and participation in preventive screening are important to the well-being of women. Using 2006 Behavioral Risk Factor Surveillance System (BRFSS) data, we examined the prevalence of health insurance coverage among working-age women and their use of selected preventive health screening. These data were also used to determine these women's access to services and the extent to which cost was a barrier. Overall, 17.3% of working-age women reported not having health insurance coverage, and lack of coverage varied widely among the states. Those without coverage were significantly more likely to report having neither routine health examinations nor a regular provider, to report cost as a barrier to access, and to be less likely to get screened for breast, cervical, and colorectal cancers during the specified time intervals. Future research and programs need to address the public health issues of unmet healthcare needs and health insurance coverage of U.S. women.

Routine preventive services for older women: a composite measure highlights gaps in delivery.

BACKGROUND: We used a composite measure to examine the delivery of routine clinical preventive services to U.S. women aged 50-64 years and > or =65 years in 2004. METHODS: We analyzed state data from the 2004 Behavioral Risk Factor Surveillance System (BRFSS) and created a composite measure that included screening of women > or =50 years for colorectal cancer, cervical cancer, breast cancer, vaccination against influenza, and, for women aged > or =65 years only, pneumococcal vaccination. The composite measure quantified the percentage of women who were up-to-date (UTD) according to recommended schedules for these services. RESULTS: Approximately 23% of women aged 50-64 years and 32.5% of women aged > or =65 years were UTD in 2004. Results varied by education, race/ethnicity, marriage status, insurance status, and health status. There was also considerable geographic variation in state-specific UTD estimates, ranging from 16.7% (California) to 38.4% (Minnesota) for women aged 50-64 years and from 25.7% (Indiana) to 48.5% (Minnesota) for women aged > or =65 years. CONCLUSIONS: Although rates for some individual services were > or =75%, the percentage of women aged 50-64 years and > or =65 years UTD on all routinely recommended cancer screenings and vaccinations was low, with <1 in 3 being UTD.

Acculturation, weight, and weight-related behaviors among Mexican Americans in the United States.

OBJECTIVE: This analysis explores the association between acculturation and body weight, self-perceptions of weight, and attempt to lose weight among Mexican Americans. METHODS: Data were analyzed from the National Health and Nutrition Examination Survey (NHANES) for 2001-2002. Indicator of acculturation used was language assimilation. Factor analysis was used to construct the acculturation measure, and descriptive and multivariable analyses were conducted using SUDAAN. RESULTS: The acculturation measure differentiated body weight, weight-related behavior, and self-perceptions about weight. Those lower on the acculturation scale were less likely to have a high BMI (> or =30) (24% vs 32%), and their perceptions of their own weight, desired weight, and recent history of trying to lose weight differed significantly from those persons high on the acculturation scale and these varied by sex. Among Mexican Americans with a BMI > or =25, those lower on the acculturation measure were significantly less likely to perceive themselves as overweight (60% vs 73%). They were also less likely to have attempted to lose weight in the past year than those who were high on the acculturation measure (OR = 0.49; 95% CI: 0.31-0.79). CONCLUSIONS: Less acculturated Mexican Americans with BMI > or =25 were less likely to perceive themselves as overweight and to have tried to lose weight. The acculturation measure provides insights into Mexican Americans' perceptions of their own weight and their recent attempt of trying to lose weight.

Are older adults up-to-date with cancer screening and vaccinations?

INTRODUCTION: Public health organizations in the United States emphasize the importance of providing routine screening for breast cancer, cervical cancer, and colorectal cancer, as well as vaccinations against influenza and pneumococcal disease among older adults. We report a composite measure of adults aged 50 years and older who receive recommended cancer screening services and vaccinations. METHODS: We analyzed state data from the 2002 Behavioral Risk Factor Surveillance System, which included 105,860 respondents aged 50 and older. We created a composite measure that included colonoscopy or sigmoidoscopy within 10 years or a fecal occult blood test in the past year, an influenza vaccination in the past year, a Papanicolaou test within 3 years for women with an intact cervix, a mammogram, and for adults aged 65 and older, a pneumonia vaccination during their lifetime. We performed separate analyses for four age and sex groups: men aged 50 to 64, women aged 50 to 64, men aged 65 and older, and women aged 65 and older. RESULTS: The percentage of each age and sex group that was up-to-date according to our composite measure ranged from 21.1% of women aged 50 to 64 (four tests) to 39.6% of men aged 65 and older (three tests). For each group, results varied by income, education, race/ethnicity, insurance status, and whether the respondent had a personal physician. CONCLUSION: These results suggest the need to improve the delivery of cancer screenings and vaccinations among adults aged 50 and older. We propose continued efforts to measure use of clinical preventive services.