Severe CNS involvement in a subset of long-term treated children with infantile-onset Pompe disease.

INTRODUCTION: The standard of care for patients with infantile-onset Pompe disease (IOPD) is enzyme replacement therapy (ERT), which does not cross the blood brain barrier. While neuromuscular manifestations of IOPD are well-described, central nervous system (CNS) manifestations of this disorder are far less characterized. Here we describe severe CNS-related neurological manifestations including seizures and encephalopathy in six individuals with IOPD. METHOD: We identified six children with IOPD who developed CNS manifestations such as seizures and/or encephalopathy. We studied their brain magnetic resonance imaging scans (MRIs) and graded the severity of white matter hyperintensities (WMHI) using the Fazekas scale scoring system as previously published. Longitudinal cognitive measures were available from 4/6 children. RESULTS: All six IOPD patients (4 males/2 females) had been treated with ERT for 12-15 years. Seizures and/or encephalopathy were noted at a median age at onset of 11.9 years (range 9-15 years). All were noted to have extensive WMHI in the brain MRIs and very high Fazekas scores which preceded the onset of neurological symptoms. Longitudinal IQ scores from four of these children suggested developmental plateauing. DISCUSSION: Among a subset of IOPD patients on long-term ERT, CNS manifestations including hyperreflexia, encephalopathy and seizures may become prominent, and there is likely an association between these symptoms and significant WMHI on MRI. Further study is needed to identify risk factors for CNS deterioration among children with IOPD and develop interventions to prevent neurological decline.

Epilepsy in cardiofaciocutaneous syndrome: Clinical burden and response to anti-seizure medication.

Treatment-resistant epilepsy is among the most serious complications of cardiofaciocutaneous syndrome (CFCS), a rare disorder caused by germline variants in the RAS-MAPK signaling pathway. This study analyzed the clinical characteristics of epilepsy and response to anti-seizure medications (ASMs) in a multinational CFCS cohort. A caregiver survey provided data regarding seizure history, use of ASMs and other treatment approaches, adverse effects, caregiver perception of treatment response, and neurological disease burden impact among individuals with CFCS. Results from 138 survey responses were quantitatively analyzed in conjunction with molecular genetic results and neurological records. The disease burden impact of CFCS was higher among individuals with epilepsy (n = 74/138), especially those with more severe seizure presentation. Oxcarbazepine, a sodium-channel blocker, had the best seizure control profile with relatively infrequent adverse effects. The most commonly prescribed ASM, levetiracetam, demonstrated comparatively poor seizure control. ASM efficacy was generally similar for individuals with BRAF and MAP2K1 gene variants. The high proportion of patients with CFCS who experienced poor seizure control despite use of multiple ASMs highlights a substantial unmet treatment need. Prospective study of ASM efficacy and clinical trials of therapies to attenuate RAS-MAPK signaling may improve avenues for clinical management.

Reported Impact of COVID-19 Workload and Stressors on School Nurses' Provision of Care During the 2021-2022 School Year: A Secondary Analysis of U.S. School Nurse Survey Data.

Many school nurses experienced increased work burden and stress during the COVID-19 pandemic. This analysis examined data from a Centers for Disease Control and Prevention cross-sectional, nationwide survey of school nurses in March 2022 to examine associations between school nurses' ability to conduct their core responsibilities and selected nurse and school factors among school nurses during the 2021-2022 school year and COVID-19 pandemic. Perceived adequate staffing and financial compensation reduced the odds of reported difficulties across all core school nursing tasks. Nurses without a registered nurse license and with higher caseloads were more likely to report difficulty in implementing specific tasks. The impact of these factors varied, with inadequate financial compensation having the largest association with school nurses' difficulty implementing all the core responsibilities. The study results improve our understanding of school nurses' challenges in implementing core school nursing responsibilities during the COVID-19 pandemic in the 2021-2022 school year.

The trends in cost associated with liver transplantation in the US: Analysis of weighted hospital data.

This study aims to evaluate recent annualized trends in the cost-burden of inpatient hospitalizations associated with liver transplantation (LT) in the US as stratified by patient demographics and medical characteristics. From 2016 to 2019 National Inpatient Sample was used to select patients who underwent LT, from which the weighted charge estimates were derived and converted to admission costs using inflation-adjusted charge-to-cost ratios. The adjusted values were stratified using select patient variables and graphed across the respective years to derive goodness-of-fit for each trend (expressed with R2 and p -values). From 2016 to 2019, the estimated total number of LT-related hospitalizations in the US were 6685, 7075, 7260, and 7815 cases respectively. There was a general increase in the total cost of LT-related hospitalizations over the years: $945.75, $1010.23, $1052.46, and $1143.84 in millions of dollars (0.98, 0.01). Furthermore, positive trends in total cost were observed in the following strata: patients aged 35-49 (0.92, 0.04) and above 65 (0.91, 0.05), Whites (0.99, 0.01), those with congestive heart failure (0.98, 0.01), ≥2 comorbidities (0.97, 0.02), hepatic encephalopathy (0.93, 0.04), and those with private insurance (0.93, 0.04), as well as LT performed in the Northeast (0.94, 0.03), Midwest (0.92, 0.04), and South (0.91, 0.04). Total cost associated with hepatitis C declined significantly (0.94, 0.03). With respect to mean costs, positive trends were observed in the following strata: those with other or cryptogenic liver disease (0.93, 0.03), ≥2 comorbidities (0.96, 0.02), and LT performed in the Northeast region (0.93, 0.04). The number of liver transplants performed in the US, as well as the associated costs, are rising. Given the apparent rising costs in specific patient populations, economic and public health policies must focus on cost containment within these groups to ensure appropriate usage of resources.

The Trends in Health Care Utilization and Costs Associated With Primary Liver Cancer: An Analysis of United States Hospitals Between 2016 and 2019.

BACKGROUND: Primary liver cancer (PLC) has placed an increasing economic and resource burden on the health care system of the United States. We attempted to quantify its epidemiology and associated costs using a national inpatient database. METHODS: Hospital discharge and insurance claims data from the National Inpatient Sample were used to conduct this analysis. Patients diagnosed with PLC (hepatocellular carcinoma or cholangiocarcinoma) were included in the study population, which was then stratified using patient demographics, comorbidities, degree of cancer spread, liver disease complications, and other descriptors. Trends were analyzed via regression curves for each of these strata from the years 2016 to 2019, with special attention to patterns in hospitalization incidence, inpatient mortality rate, total costs, and average per-capita costs. The resulting curves were evaluated using goodness-of-fit statistics and P-values. RESULTS: Aggregate hospitalization incidence, inpatient mortality rates, and total costs were found to significantly increase throughout the study period (P=0.002, 0.002, and 0.02, respectively). Relative to their demographic counterparts, males, White Americans, and those older than 65 years of age contributed the largest proportions of total costs. These population segments also experienced significant increases in total expenditure (P=0.04, 0.03, and 0.02, respectively). Admissions deemed to have multiple comorbidities were associated with progressively higher total costs throughout the study period (P=0.01). Of the categorized underlying liver diseases, only admissions diagnosed with alcoholic liver disease or nonalcoholic fatty liver disease saw significantly increasing total costs (P=0.006 and 0.01), although hepatitis C was found to be the largest contributor to total expenses. CONCLUSIONS: From 2016 to 2019, total costs, admission incidence, and inpatient mortality rates associated with PLC hospitalization increased. Strata-specific findings may be reflective of demographic shifts in the PLC patient populations, as well as changes in underlying chronic liver disease etiologies.

Infantile epileptic spasms syndrome in children with cardiofaciocutanous syndrome: Clinical presentation and associations with genotype.

Gene variants that dysregulate signaling through the RAS-MAPK pathway cause cardiofaciocutaneous syndrome (CFCS), a rare multi-system disorder. Infantile epileptic spasms syndrome (IESS) and other forms of epilepsy are among the most serious complications. To investigate clinical presentation, treatment outcomes, and genotype-phenotype associations in CFCS patients with IESS, molecular genetics and clinical neurological history were reviewed across two large clinical research cohorts (n = 180). IESS presented in 18/180 (10%) cases, including 16 patients with BRAF variants and 2 with MAP2K1 variants. Among IESS patients with BRAF variants, 16/16 (100%) had sequence changes affecting the protein kinase domain (exons 11-16), although only 57% of total BRAF variants occurred in this domain. Clinical onset of spasms occurred at a median age of 5.4 months (range: 1-24 months). Among 13/18 patients whose IESS resolved with anti-seizure medications, 10 were treated with ACTH and/or vigabatrin. A substantial majority of CFCS patients with IESS subsequently developed other epilepsy types (16/18; 89%). In terms of neurodevelopmental outcomes, gross motor function and verbal communication were more limited in patients with a history of IESS compared to those without IESS. These findings can inform clinical neurological care guidelines for CFCS and development of relevant pre-clinical models for severe epilepsy phenotypes.

Neurologic and neurodevelopmental complications in cardiofaciocutaneous syndrome are associated with genotype: A multinational cohort study.

PURPOSE: Dysregulation of RAS or its major effector pathway is the molecular mechanism of RASopathies, a group of multisystemic congenital disorders. Neurologic complications are especially challenging in the management of the rare RASopathy cardiofaciocutaneous (CFC) syndrome. This study evaluated clinical neurologic and neurodevelopmental features and their associations with CFC syndrome gene variants. METHODS: A multinational cohort of 138 individuals with CFC syndrome (BRAF = 90, MAP2K1 = 36, MAP2K2 = 10, KRAS = 2) was recruited. Neurologic presentation was captured via clinician review of medical records and caregiver-completed electronic surveys. Validated measures of seizure severity, adaptive function, and gross motor function were obtained. RESULTS: The overall frequency of intellectual disability and seizures was 82% and 55%, respectively. The frequency and severity of seizures was higher among individuals with BRAF or MAP2K1 variants than in those with MAP2K2 variants. A disproportionate incidence of severe, treatment-resistant seizures was observed in patients with variants in the catalytic protein kinase domain of BRAF and at the common p.Y130 site of MAP2K1. Neurodevelopmental outcomes were associated with genotype as well as seizure severity. CONCLUSION: Molecular genetic testing can aid in prediction of epilepsy and neurodevelopmental phenotypes in CFC syndrome. Study results identified potential CFC syndrome-associated variants in the development of relevant animal models for neurologic, neurocognitive, and motor function impairment.

Treatment of CSF1R-Related Leukoencephalopathy: Breaking New Ground.

BACKGROUND: Colony-stimulating factor-1 receptor (CSF1R)-related leukoencephalopathy is a rapidly progressive neurodegenerative disease for which there is currently no cure. Hematopoietic stem cell transplantation (HSCT) has been proposed as a disease-modifying treatment. OBJECTIVE: The objective of this study was to determine the effect of HSCT on disease progression. METHODS: We collected all available clinical data from a cohort of 7 patients with CSF1R-related leukoencephalopathy who underwent HSCT at our institutions. Clinical data included detailed neurological examination by a board-certified neurologist, serial cognitive screens, formal neuropsychological evaluations, and serial brain magnetic resonance imaging (MRI). RESULTS: Our patients had an average disease duration of 27.6 months at the time of transplant, and we have 87 months of total posttransplant follow-up time (median, 11; range, 2-27). One patient died in the periprocedural period. The remaining patients showed a variable response to treatment, with 6 of 7 patients trending toward stabilization on motor examination, cognitive scores, and/or MRI abnormalities, especially with white matter lesion burden. CONCLUSIONS: This is the largest series of patients with CSF1R-related leukoencephalopathy receiving HSCT. We conclude that HSCT can stabilize the disease in some patients. Variability in patient responsiveness suggests that measures of disease heterogeneity and severity need to be considered when evaluating a patient's candidacy for transplant. HSCT appears to be the first disease-modifying therapy for CSF1R-related leukoencephalopathy. This milestone may serve as a foothold toward better understanding the disease's pathomechanism, thus providing new opportunities for better disease-specific therapies. © 2021 International Parkinson and Movement Disorder Society.

Differential outcomes for frontal versus posterior demyelination in childhood cerebral adrenoleukodystrophy.

In the most common variant of childhood cerebral adrenoleukodystrophy (cALD), demyelinating brain lesions are distributed predominately in parieto-occipital white matter. Less frequently, lesions first develop in frontal white matter. This matched cohort study examined whether outcomes after standard treatment with hematopoietic cell transplantation (HCT) differ in patients with early stage frontal lesions as compared to parieto-occipital lesions. Retrospective chart review identified seven pediatric patients with frontal cALD lesions and MRI severity score < 10 who underwent a single HCT at our center between 1990 and 2019. Concurrent MRI, neurocognitive and psychiatric outcomes at last comprehensive follow-up (mean 1.2 years; range 0.5-2.1 years) were compared with a group of seven boys with the parieto-occipital variant matched on pre-HCT MRI severity score. Both groups showed similar rates of transplant complications and radiographic disease advancement. Neurocognitive outcomes were broadly similar, with more frequent working memory deficits among individuals with frontal lesions. Psychiatric problems (hyperactivity, aggression, and atypical behavior) were considerably more common and severe among patients with frontal lesions. Aligned with the critical role of the frontal lobes in emotional and behavioral regulation, functional disruption of self-regulation skills is widely observed among patients with frontal lesions. Comprehensive care for cALD should address needs for psychiatric care and management.

The introduction of a standardised national licensing exam as a driver of change in medical education: A qualitative study from Switzerland.

INTRODUCTION: Only a few studies have described the impacts, strengths and needs for further development of national licensing exams (NLE). To gain such insights regarding the Swiss NLE, which includes a multiple-choice and a standardised clinical skills exam, we explored the perceptions of involved experts and stakeholders. METHODS: We explored participants' perceptions in four focus group discussions. The interviews were recorded, transcribed verbatim and qualitatively analysed using a thematic analysis approach. RESULTS: The analysis resulted in five perceived impacts, two strengths and two needs for further developments of the NLE. Perceived impacts were (1) steering students' learning behaviour, (2) supporting teachers and assessors to align teaching to competencies, (3) elevating the importance of the Swiss Catalogue of Learning Objectives, (4) setting incentives for the further development of curricula, and (5) fostering the collaboration between the faculties of medicine. Perceived strengths were the blend of assessment formats, including their competency-based orientation, and the collaborative development approach. Perceived needs lay in the NLE's further development to sustain its fit for purpose and in incentives for people involved. CONCLUSION: According to our study, this NLE had, and has, notable impacts on medical education in Switzerland. Our insights can be useful for others planning a similar undertaking.

Enhanced gastrointestinal passive paracellular permeability contributes to the obesity-associated hyperoxaluria.

Most kidney stones (KS) are composed of calcium oxalate and small increases in urine oxalate enhance the stone risk. Obesity is a risk factor for KS, and urinary oxalate excretion increases with increased body size. We previously established the obese ob/ob ( ob) mice as a model (3.3-fold higher urine oxalate) to define the pathogenesis of obesity-associated hyperoxaluria (OAH). The purpose of this study was to test the hypothesis that the obesity-associated enhanced small intestinal paracellular permeability contributes to OAH by increasing passive paracellular intestinal oxalate absorption. ob Mice have significantly higher jejunal (1.6-fold) and ileal (1.4-fold) paracellular oxalate absorption ex vivo and significantly higher (5-fold) urine [13C]oxalate following oral gavage with [13C]oxalate, indicating increased intestinal oxalate absorption in vivo. The observation of higher oxalate absorption in vivo compared with ex vivo suggests the possibility of increased paracellular permeability along the entire gut. Indeed, ob mice have significantly higher fractions of the administered sucrose (1.7-fold), lactulose (4.4-fold), and sucralose (3.1-fold) excreted in the urine, reflecting increased gastric, small intestinal, and colonic paracellular permeability, respectively. The ob mice have significantly reduced gastrointestinal occludin, zonula occludens-1, and claudins-1 and -3 mRNA and total protein expression. Proinflammatory cytokines and oxidative stress, which are elevated in obesity, significantly enhanced paracellular intestinal oxalate absorption in vitro and ex vivo. We conclude that obese mice have significantly higher intestinal oxalate absorption and enhanced gastrointestinal paracellular permeability in vivo, which would likely contribute to the pathogenesis of OAH, since there is a transepithelial oxalate concentration gradient to drive paracellular intestinal oxalate absorption. NEW & NOTEWORTHY This study shows that the obese ob/ob mice have significantly increased gastrointestinal paracellular oxalate absorption and remarkably enhanced paracellular permeability along the entire gut in vivo, which are likely mediated by the obesity-associated increased systemic and intestinal inflammation and oxidative stress. A transepithelial oxalate concentration gradient driving gastrointestinal paracellular oxalate absorption exists, and therefore, our novel findings likely contribute to the hyperoxaluria observed in the ob/ob mice and hence to the pathogenesis of obesity-associated hyperoxaluria.

A report on state-wide implementation of newborn screening for X-linked Adrenoleukodystrophy.

Minnesota became the fourth state to begin newborn screening (NBS) for X-linked adrenoleukodystrophy (X-ALD) in 2017. As there is limited retrospective data available on NBS for X-ALD, we analyzed Minnesota's NBS results from the first year of screening. C26:0 lysophosphatidylcholine (C26:0-LPC) screening results of 67,836 infants and confirmatory testing (ABCD1 gene and serum VLCFA analysis) for screen positives were obtained. Fourteen infants (nine males, five females) screened positive for X-ALD and all were subsequently confirmed to have X-ALD, with zero false positives. The birth prevalence of X-ALD in screened infants was 1 in 4,845 and 1 in 3,878 males, more than five times previous reported incidences. Pedigrees of affected infants were analyzed, and 17 male (mean age of 17) and 24 female relatives were subsequently diagnosed with X-ALD. Phenotypes of these family members included self-reported mild neuropathy symptoms in two males and seven females, and childhood cerebral disease (ccALD) and adrenal insufficiency in one male. We observed fewer cases of ccALD and adrenal insufficiency than expected in male family members (5.9% of males for both) compared to previous observations. Together, these findings suggest that the spectrum of X-ALD may be broader than previously described and that milder cases may previously have been underrepresented. Other challenges included a high frequency of variants of uncertain significance in ABCD1 and an inability to predict phenotypic severity. We posit that thoughtful planning to address these novel challenges and coordination by dedicated specialists will be imperative for successful implementation of population-based screening for X-ALD.

Adenosinergic signaling inhibits oxalate transport by human intestinal Caco2-BBE cells through the A2B adenosine receptor.

Most kidney stones (KS) are composed of calcium oxalate, and small increases in urine oxalate affect the stone risk. Intestinal oxalate secretion mediated by anion exchanger SLC26A6 (PAT1) plays a crucial role in limiting net absorption of ingested oxalate, thereby preventing hyperoxaluria and related KS, reflecting the importance of understanding regulation of intestinal oxalate transport. We previously showed that ATP and UTP inhibit oxalate transport by human intestinal Caco2-BBE cells (C2). Since ATP is rapidly degraded to adenosine (ADO), we examined whether intestinal oxalate transport is regulated by ADO. We measured [14C]oxalate uptake in the presence of an outward Cl gradient as an assay of Cl-oxalate exchange activity, ≥49% of which is PAT1-mediated in C2 cells. We found that ADO significantly inhibited oxalate transport by C2 cells, an effect completely blocked by the nonselective ADO receptor antagonist 8- p-sulfophenyltheophylline. ADO also significantly inhibited oxalate efflux by C2 cells, which is important since PAT1 mediates oxalate efflux in vivo. Using pharmacological antagonists and A2B adenosine receptor (A2B AR) siRNA knockdown studies, we observed that ADO inhibits oxalate transport through the A2B AR, phospholipase C, and PKC. ADO inhibits oxalate transport by reducing PAT1 surface expression as shown by biotinylation studies. We conclude that ADO inhibits oxalate transport by lowering PAT1 surface expression in C2 cells through signaling pathways including the A2B AR, PKC, and phospholipase C. Given higher ADO levels and overexpression of the A2B AR in inflammatory bowel disease (IBD), our findings have potential relevance to pathophysiology of IBD-associated hyperoxaluria and related KS.

Reduced active transcellular intestinal oxalate secretion contributes to the pathogenesis of obesity-associated hyperoxaluria.

Most kidney stones are composed of calcium oxalate, and minor changes in urine oxalate affect the stone risk. Obesity is a risk factor for kidney stones and a positive correlation of unknown etiology between increased body size, and elevated urinary oxalate excretion has been reported. Here, we used obese ob/ob (ob) mice to elucidate the pathogenesis of obesity-associated hyperoxaluria. These ob mice have significant hyperoxaluria (3.3-fold) compared with control mice, which is not due to overeating as shown by pair-feeding studies. Dietary oxalate removal greatly ameliorated this hyperoxaluria, confirming that it is largely enteric in origin. Transporter SLC26A6 (A6) plays an essential role in active transcellular intestinal oxalate secretion, and ob mice have significantly reduced jejunal A6 mRNA (- 80%) and total protein (- 62%) expression. While net oxalate secretion was observed in control jejunal tissues mounted in Ussing chambers, net absorption was seen in ob tissues, due to significantly reduced secretion. We hypothesized that the obesity-associated increase in intestinal and systemic inflammation, as reflected by elevated proinflammatory cytokines, suppresses A6-mediated intestinal oxalate secretion and contributes to obesity-associated hyperoxaluria. Indeed, proinflammatory cytokines (elevated in ob mice) significantly decreased intestinal oxalate transport in vitro by reducing A6 mRNA and total protein expression. Proinflammatory cytokines also significantly reduced active mouse jejunal oxalate secretion, converting oxalate transport from net secretion in vehicle-treated tissues to net absorption in proinflammatory cytokines-treated tissues. Thus, reduced active intestinal oxalate secretion, likely secondary to local and systemic inflammation, contributes to the pathogenesis of obesity-associated hyperoxaluria. Hence, proinflammatory cytokines represent potential therapeutic targets.

Febrile infection-related epilepsy syndrome treated with anakinra.

Febrile infection-related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with limited treatment options and an unclear etiology. Anakinra is a recombinant version of the human interleukin-1 receptor antagonist used to treat autoinflammatory disorders. This is the first report of anakinra for treatment of a child with super-refractory status epilepticus secondary to FIRES. Anakinra was well tolerated and effective. Cerebral spinal fluid analysis revealed elevated levels of proinflammatory cytokines before treatment that normalized on anakinra, suggesting a potential pathogenic role for neuroinflammation in FIRES. Further studies are required to assess anakinra efficacy and dosing, and to further delineate disease etiology. Ann Neurol 2016;80:939-945.

Emission of hydrogen sulfide (H2S) at a waterfall in a sewer: study of main factors affecting H2S emission and modeling approaches.

Hydrogen sulfide (H2S) represents one of the main odorant gases emitted from sewer networks. A mathematical model can be a fast and low-cost tool for estimating its emission. This study investigates two approaches to modeling H2S gas transfer at a waterfall in a discharge manhole. The first approach is based on an adaptation of oxygen models for H2S emission at a waterfall and the second consists of a new model. An experimental set-up and a statistical data analysis allowed the main factors affecting H2S emission to be studied. A new model of the emission kinetics was developed using linear regression and taking into account H2S liquid concentration, waterfall height and fluid velocity at the outlet pipe of a rising main. Its prediction interval was estimated by the residual standard deviation (15.6%) up to a rate of 2.3 g H2S·h-1. Finally, data coming from four sampling campaigns on sewer networks were used to perform simulations and compare predictions of all developed models.

Differences in Receipt of Three Preventive Health Care Services by Race/Ethnicity in Medicare Advantage Plans: Tracking the Impact of Pay for Performance, 2010 and 2013.

INTRODUCTION: In 2012, the Centers for Medicare and Medicaid Services (CMS) introduced the Quality Bonus Payment Demonstration, a pay-for-performance (P4P) program, into Medicare Advantage plans. Previous studies documented racial/ethnic disparities in receipt of care among participants in these plans. The objective of this study was to determine whether P4P incentives have affected these disparities in Medicare Advantage plans. METHODS: We studied 411 Medicare Advantage health plans that participated in the Medicare Health Outcome Survey in 2010 and 2013. Preventive health care was defined as self-reported receipt of health care provider communication or treatment to reduce risk of falling, improve bladder control, and monitor physical activity among individuals reporting these problems. Logistic regression stratified by health care plan was used to examine racial/ethnic disparities in receipt of preventive health care before and after the introduction of the P4P program in 2012. RESULTS: We found similar racial/ethnic differences in receipt of preventive health care before and after the introduction of P4P. Blacks and Asians were less likely than whites to receive advice to improve bladder control and more likely to receive advice to reduce risk of falling and improve physical activity. Hispanics were more likely to report receiving advice about all 3 health issues than whites. After the introduction of P4P, the gap decreased between Hispanics and whites for improving bladder control and monitoring physical activity and increased between blacks and whites for monitoring physical activity. CONCLUSION: Racial/ethnic differences in receipt of preventive health care are not always in the expected direction. CMS should consider developing a separate measure of equity in preventive health care services to encourage health plans to reduce gaps among racial/ethnic groups in receiving preventive care services.

Discharge disposition for home health care patients with Alzheimer's disease and related dementia: The role of living arrangements and rural living.

PURPOSE: To examine the relationship between living arrangements and discharge disposition, and how this relationship differs by the rural or urban characteristics of the patient's residence among home health care patients with Alzheimer's disease and related dementia (ADRD). METHODS: This retrospective study used the 2019 Outcome and Assessment Information Set and the Master Beneficiary Summary File. Our study was based on 531,269 Medicare fee-for-service patients with ADRD. We used linear probability regression models to examine the relationship between discharge disposition (to the community vs. an institution) and living arrangements, including an interaction term for rural-urban residence. FINDINGS: Patients in rural areas (19.8%) were more likely to live alone than those in urban areas (15.2%). Our main results show that patients living at home with others (coefficient: -0.02, p-value < 0.001) or alone (coefficient: -0.03, p-value < 0.001) were less likely to be discharged to the community compared to patients who lived in congregate settings. Also, for patients with ADRD who lived in rural areas, living at home with others (rural*home with others; coefficient: -0.02, p-value < 0.001) or living alone (rural*home alone; coefficient: -0.03, p-value<0.001) were associated with additional lower probabilities of being discharged to their communities. CONCLUSIONS: A multidimensional approach considering living arrangements to support home health care patients with ADRD could be critical to achieving better health outcomes. Furthermore, implementing area-specific target interventions could be important for improving the care and health of patients with ADRD as well as reducing rural-urban disparities in discharge disposition.

The Role of Neighborhood Socioeconomic Status in Institutionalization of Home Health Care Patients With and Without Alzheimer's Disease and Related Dementias.

OBJECTIVES: To assess whether neighborhood socioeconomic status (SES) moderates the association between Alzheimer's disease and related dementias (ADRD) and successful discharge to the community. In addition, to explore whether the role of neighborhood SES on successful discharge for patients with ADRD varies by the severity of ADRD. DESIGN: This is a retrospective cohort study. SETTING AND PARTICIPANTS: Medicare Fee-for-service beneficiaries, aged 65 or older, who received home health care in 2019. METHODS: We used linear probability regression models with successful discharge to the community as the main outcome, and neighborhood SES and ADRD as independent variables. Also, we modified the Functional Assessment Staging Tool (FAST) to measure ADRD severity. RESULTS: Our study results show ADRD and residing in neighborhoods with lower socioeconomic conditions were independently associated with lower probabilities of successful discharge to the community. We also found that the differences in probabilities of remaining at home between patients with and without ADRD were larger among those in neighborhoods with lower SES (ADRD∗less disadvantaged neighborhood, coeff: -0.01, P < .001; ADRD∗more disadvantaged neighborhood, coeff: -0.02, P < .001; ADRD∗most disadvantaged neighborhood, coeff: 0.032, P < .001). Among patients with ADRD, patients with the most advanced ADRD were less likely to remain in their homes and community when living in neighborhoods with lower SES. CONCLUSIONS AND IMPLICATIONS: Our study results show that when patients with ADRD receiving home health care live in neighborhoods with lower SES, they face further challenges to remaining in their homes and community. Public health officials and community planners should consider using area-level interventions to improve care and health outcomes for patients with ADRD. Also, further research aimed at identifying the specific factors and resources influencing lower care quality and poorer health outcomes in socioeconomically disadvantaged neighborhoods, particularly for patients with ADRD, can provide valuable insights for the development and implementation of targeted interventions.