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BackgroundPneumocystis jirovecci pneumonia (PCP) is a life threating disease in immunodeficient patients. Liver cirrhosis itself can lead to immunodefiency, however little is known if Pneumocystis jirovecci infection affects the outcome of patients with liver cirrhosis.AimWe aimed to assess the predictors for Pneumocystis jirovecci-associated mortality in patients with Pneumocystis jirovecci infection treated at intensive care units.MethodsA total of 151 patients hospitalized between January 2013 and November 2019 with a PCR-confirmed Pneumocystis jirovecci infection were retrospectively included in this study and analysed for clinical predictors for PCJ associated mortality.ResultsThe overall mortality in our patient cohort was 60%. Out of 151 patients included in the analysis, 67 (44%) patients suffered from liver cirrhosis. Patients with an advanced liver cirrhosis (Child-Pugh class C) showed the highest mortality rate of 84.7%. The presence of a liver cirrhosis was associated with a significant increased risk of mortality (OR: 4.809) ([95%-CI: 2.32–9.97]; p

Abstract Background Metastatic Crohn’s disease is a rare disorder characterized by various granulomatous skin lesions that occur independently of gastrointestinal tract involvement. However, currently there is no standardized care or specific treatment. Therapeutic approaches include immunosuppressive agents, such as corticosteroids, azathioprine, and monoclonal antibodies targeting inflammatory cytokines like tumor necrosis factor (TNF). Case presentation We present a case of a 29-year-old western European woman with significant blind ending abdominal subcutaneous fistulas and abscesses, who sought evaluation in the dermatology department. Histological examination revealed multiple epithelioid cell granulomas. There was no evidence of infectious or rheumatologic diseases such as sarcoidosis. The tentative diagnosis was metastatic Crohn’s disease, which was not related to an intestinal manifestation of the disease. The patient responded to infliximab but had to discontinue it due to an allergic reaction. Subsequent adalimumab treatment failed to induce clinical remission; thus, therapy was switched to ustekinumab, resulting in a positive response. Written informed consent for publication of their clinical details and clinical images was obtained from the patient. For our study more than 1600 publications were screened for cases of metastatic Crohn’s disease on PubMed database. 59 case reports with 171 patients were included in the analysis and evaluated for localization, diagnostic and therapeutic approaches, and complications and were summarized in this review. Conclusion The successful ustekinumab treatment of a patient with metastatic Crohn's disease underscores the potential of this minimally investigated therapeutic option, highlighting the need for future treatment guidelines given the increasing prevalence of such cases.

Background: Idiopathic pulmonary fibrosis (IPF) is a serious illness with an unpredictable disease course and survival rates comparable with some cancers. Patients with IPF suffer considerable symptom burden, declining quality of life, and high health care resource utilization. Patients and caregivers report many unmet needs, including a desire for more education regarding diagnosis and assistance with navigating disease trajectory. Compelling evidence suggests that palliative care (PC) provides an extra layer of support for patients with serious illness. Research Question: The purpose of this survey was to gain perspectives regarding PC for patients with IPF by board-certified pulmonologists in South Carolina (SC). Study Design and Methods: A 24-item survey was adapted (with permission) from the Pulmonary Fibrosis Foundation PC Survey instrument. Data were analyzed and results are presented. Results: Pulmonologists (n?=?32, 44%) completed the survey; 97% practice in urbanized settings. The majority agreed that PC and hospice do not provide the same service. There were varying views about comfort in discussing prognosis, disease trajectory, and addressing advance directives. Options for ambulatory and inpatient PC are limited and early PC referral does not occur. None reported initiating a PC referral at time of initial IPF diagnosis. Interpretation: Pulmonologists in SC who participated in this survey are aware of the principles of PC in providing comprehensive care to patients with IPF and have limited options for PC referral. PC educational materials provided early in the diagnosis can help facilitate and guide end-of-life planning and discussions. Minimal resources exist for patients in underserved communities.

Abstract Background Research studies demonstrate that palliative care can improve patient outcomes such as quality of life, symptom burden and patient satisfaction with care (Gomes B, et al. Effectiveness and cost-effectiveness of home palliative care services for adults with advanced illness and their caregivers. Cochrane Database Syst Rev. 2013(6):CD00776) (World Health Organization. Palliative Care. Published 2020.). While 76% of patients who need palliative care live in limited-resource countries, access to high quality palliative services in these countries is minimal (Worldwide Hospice and Palliative Care Association and World Health Organization. Global Atlas of Palliative Care (2nd ed). 2020.). In 2014 the Worldwide Hospice Palliative Care Alliance, with strong endorsement by the WHO, released the Palliative Care Toolkit to provide a training and implementation toolkit for empowering community members to deliver palliative care in resource poor settings (Worldwide Hospice and Palliative Care Association and World Health Organization. Global Atlas of Palliative Care at the End of Life. Geneva, Switzerland 2014.). They encouraged researchers and public health practitioners to conduct rigorous evaluation of the toolkit in diverse settings and contexts. To address this need, we will conduct a pilot randomized controlled trial (RCT) to examine implementation and explore potential effect of an intervention based upon the Palliative Care Toolkit, as adapted and used by community health workers (CHWs) working with a cancer center in Kolkata, India to deliver home-based palliative care for rural patients. Methods Utilizing a randomized controlled trial design, intervention patients (n = 45) receive home-based palliative services (Pal-Care) delivered by community health workers (CHWs), with comparison against a control group of patients (n = 45) who receive usual cancer-center-based palliative services. Primary outcome measures include evaluation of CHW training outcomes, roles and responsibilities of the CHWS and how they assist patients, trial recruitment, stakeholder perceptions of the intervention, and fidelity to study protocol. Secondary outcomes measure patient self-report of health-related quality of life, symptom burden, palliative needs and patient care experience, outcomes The RE-AIM framework guides our evaluation plan to measure the reach, effectiveness, adoption, implementation and maintenance of the Pal-Care intervention (Gaglio B, et al. The RE-AIM framework: a systematic review of use over time. Am J Public Health. 2013;103(6):e38?46.). Data will be analyzed in SAS. All measures will be evaluated overall and by patient age, gender and cancer type and by CHW caseload. Discussion Pal-Care is a RCT funded by the NCI to explore utilization of CHWs to deliver a home-based palliative care intervention built upon the WHO Palliative Care toolkit (PCT), as compared to a usual care control group. The long-term goal of this research is to develop an effective and sustainable model for delivering home-based palliative care for cancer patients in underserved areas. Trial registration (TRN) ClinicalTrials.gov ID# NCT04972630.

Fatigue is the most reported symptom in patients with sarcoidosis (SPs) and is a significant predictor of decreased quality of life that is strongly associated with stress and negative mood states. Few medications exist for treating fatigue in SPs, and outpatient physical rehabilitation programs are limited by availability and cost. Sarcoidosis in the US predominantly impacts minorities and underserved populations who are of working age and often have limited resources (e.g., financial, transportation, time off work) that may prevent them from attending in-person programs. The use of mobile health (mHealth) is emerging as a viable alternative to provide access to self-management resources to improve quality of life. The Sarcoidosis Patient Assessment and Resource Companion (SPARC) App is a sarcoidosis-specific mHealth App intended to improve fatigue and stress in SPs. It prompts SPs to conduct breathing awareness meditation (BAM) and contains educational modules aimed at improving self-efficacy.Herein we describe the design and methods of a 3-month randomized control trial comparing use of the SPARC App (10-min BAM twice daily) to standard care in 50 SPs with significant fatigue (FAS ≥22). A Fitbit® watch will provide immediate heartrate feedback after BAM sessions to objectively monitor adherence. The primary outcomes are feasibility and usability of the SPARC App (collected monthly). Secondary endpoints include preliminary efficacy at improving fatigue, stress, and quality of life. We expect the SPARC App to be a useable and feasible intervention that has potential to overcome barriers of more traditional in-person programs.

BackgroundPeople with spinal cord injury (SCI) report feeling unprepared to manage their disability upon discharge to the community. This situation is exacerbated when they return to settings where self-management support and resources are sparse, thus increasing the risk of costly secondary conditions and rehospitalizations. These factors make a compelling case for implementing innovative community-based SCI self-management programs that empower and engage individuals with SCI. Using a community-engaged research (CEnR) approach, we developed a peer-supported SCI self-management intervention, known as PHOENIX (Peer-supported Health Outreach, Education, and Information Exchange), which integrates online educational content and support from peer navigators (PNs) through telehealth, to promote health and community participation after SCI. ObjectiveThe objective of this pilot study is to evaluate the feasibility and acceptability of PHOENIX and the study design, and to obtain estimates of the variability of relevant outcome measures. MethodsWe conducted a pilot randomized waitlist-controlled trial (n=30) in collaboration with the South Carolina Spinal Cord Injury Association (SCSCIA), our long-standing community-based nonprofit organization research partner. We recruited 4 PNs through our SCSCIA collaboration using its existing network of trained peer mentors. Our study design supported comparison of the following 2 randomly assigned groups: PHOENIX intervention group and waitlist enhanced usual care (EUC) group. The PHOENIX intervention was administered online by PNs over 16 weeks through scheduled “video visits.” The EUC group participated in the study for 16 weeks with usual community services and no navigation, and received 4 monthly newsletters from the SCSCIA on a variety of SCI-relevant topics. At the end of the waitlist period, the waitlist EUC group received the full PHOENIX intervention. Measures of feasibility included PN and participant recruitment and retention, PN workload, protocol adherence, and incidence of technical issues. We conducted qualitative interviews with participants and PNs to evaluate the acceptability of PHOENIX and the study design. Outcome measures, including community participation, quality of life, and the occurrence and subjective impact of medically serious secondary conditions and rehospitalizations, were assessed at baseline after randomization and at subsequent time points to allow between-group comparisons. ResultsPN hiring and training were completed in August 2018. Recruitment began in November 2018. A total of 30 participants were recruited across South Carolina, and 28 participants completed follow-up by August 2020. An analysis of the results is being finalized, and the results are expected to be published in 2023. ConclusionsThis study will provide valuable information to guide future research seeking to address unmet self-management needs and improve outcomes in individuals with SCI. Feasibility findings of this study will provide evidence from CEnR guided by people with SCI and SCI service providers to inform further development, testing, and dissemination of effective and scalable self-management strategies for people with SCI. International Registered Report Identifier (IRRID)RR1-10.2196/42688

Abstract Background Children with sickle cell anemia are at risk for stroke. Ischemic stroke risk can be identified among children ages 2–16 years with sickle cell anemia using transcranial Doppler ultrasound. Despite strong recommendations for transcranial Doppler screening in guidelines released by the National Heart, Lung, and Blood Institute, implementation of transcranial Doppler screening in sickle cell anemia remains suboptimal. The purpose of this study was to identify barriers and facilitators to transcranial Doppler screening in a large national consortium to inform subsequent implementation interventions. Methods A qualitative descriptive approach was used to conduct 52 semi-structured interviews with a sample of patients with sickle cell anemia, their parents or primary caregivers, and healthcare providers dispersed across the United States. Interviews took place from September 2018 through March 2019. Directed content analysis was conducted with an adapted version of the Multilevel Ecological Model of Health as an initial coding framework, completed July 2019. Frequency analysis was conducted to determine predominant barrier and facilitator themes. Results Fourteen barrier themes and 12 facilitator themes emerged representing all levels of the ecological framework. Two barrier themes (Logistical Difficulties and Competing Life Demands and Gaps in Scheduling and Coordination), and 5 facilitator themes (Coordination, Scheduling and Reminders; Education and Information; Provider and Staff Investment and Assistance; Positive Patient Experience; and Convenient Location) were predominant. Conclusions Barriers and facilitators to transcranial Doppler screening in children with sickle cell anemia are complex and occur across multiple ecological levels. One barrier theme and 3 facilitator themes were found to be optimal to address in subsequent implementation interventions.

Abstract Background Chronic venous leg ulcers (CVLUs) are the most common type of lower extremity wound. Even when treated with evidenced-based care, 30–50% of CVLUs fail to heal. A specific gap exists about the association between psychosocial stressors, particularly loneliness, and biomarkers of inflammation and immunity. Loneliness is highly prevalent in persons with CVLUs, has damaging effects on health, and contributes to the development of multiple chronic conditions, promotes aberrant inflammation, and diminishes healing. However, the confluence of loneliness, inflammation and the wound healing trajectory has not been elucidated; specifically whether loneliness substantially mediates systemic inflammation and alters healing over time. This study seeks to address whether there is a specific biomarker profile associated with loneliness, CVLUs, and wound healing that is different from non-lonely persons with CVLUs. Methods An observational prospective study will identify, characterize and explore associations among psychosocial stressors, symptoms and biomarkers between 2 CVLU groups, with loneliness+ (n = 28) and without loneliness- (n = 28) during 4 weeks of wound treatment, measured at 3 time points. We will examine psychosocial stressors and symptoms using psychometrically-sound measures include PROMIS® and other questionnaires for loneliness, social isolation, depression, anxiety, stigma, sleep, fatigue, pain, quality of life, cognition, and function. Demographics data including health history, sex, age, wound type and size, wound age, and treatment will be recorded from the electronic health record. We will characterize a biomarker panel of inflammatory genes including chemotaxic and growth factors, vascular damage, and immune regulators that express in response to loneliness to loneliness and CVLUs using well-established RNA sequence and PCR methods for whole blood samples. In an exploratory aim we will explore whether age and sex/psychological stressors and symptoms indicate potential moderation/mediation of the effect of loneliness on the biomarker profile over the study period. Discussion This study will provide insight into the influence of psychosocial stressors, symptoms, and biological mechanisms on wound healing, towards advancing a future healing prediction model and interventions to address these stressors and symptoms experienced by persons with CVLUs.

Abstract Background Persons with dementia with mild to moderate cognitive impairment are at risk for developing impairments with activities of daily living such as the ability to feed oneself, that negatively influence health. Lack of caregiver skills related to mealtime planning for persons with dementia and the ability to cope with dysfunctional behaviors are well-documented factors that influence nutritional status outcomes, lead to weight loss, poor quality of life, and impact their ability to remain at home. Methods A cluster randomized controlled trial with a parallel mixed methods evaluation processes will be conducted to examine the efficacy of a train-the-trainer program using non-paid volunteers in respite care centers to deliver a telehealth mealtime intervention guided by the theory-based C3P Model—Change the Person, Change the People, Change the Place (C3P). In this six-month study, dyads of caregivers and persons with mild to moderate dementia receiving respite care services will be randomized to receive either the telehealth intervention or enhanced usual care. Within the intervention group, dyads will be partnered with a C3P trained volunteer who will work with caregivers via videoconference to devise and implement mealtime plans. Under usual care, dyads will receive standardized educational materials modified from The Savvy Caregiver Program for Alzheimer’s disease. The primary outcomes include weight maintenance or gain of the person with dementia and quality of life of the caregiver. A multi-level evaluation process utilizing respite center administrators and directors, volunteers, and caregivers will explore intervention fidelity, acceptability and sustainability. Using both the CONSORT and SPIRIT checklists as guidance, the comprehensive study design is more fully described in this manuscript. Discussion In this trial, we will lay the groundwork to examine the efficacy and sustainability of a train-the-trainer telehealth program that could be widely disseminated by national Alzheimer’s organizations and readily adopted by community agencies to provide additional resources to assist families in managing mealtimes at home, while promoting the quality of life of both the caregiver and the person with dementia. Trial registration This study was registered with clinicaltrials.gov: NCT03622814 on August 9, 2018..

Abstract Background A poor quality diet is a well-known risk factor for many chronic diseases. However, eating a healthful diet is not always simple as many underlying factors can impede adherence. Individuals with fewer barriers are more likely to eat a healthful diet than those who have more barriers. Accurately measuring barriers to eating a healthful diet could inform personalized interventions, particularly those aiming to prevent chronic diseases. The aim of this study was to establish content validity for selected items obtained from the National Health and Nutrition Examination Survey (NHANES) database to be considered for inclusion as items on the conceptualized Dietary Health Status (DHS) instrument, which is designed to measure barriers to eating a healthful diet in adults. Methods The Behavioral Change Wheel hub COM-B and the Theoretical Domains Framework (TDF) were the two theoretical frameworks underpinning the development of the DHS instrument. Seven steps were conducted to create the instrument: 1) development of operational definitions for each TDF domain; 2) identification of items from the NHANES database 2011–2012; 3) screening of items to ensure inclusion of all relevant items; 4) assigning items to a theory-based domain; 5) evaluation of the items against inclusion/exclusion criteria; 6) solicitation of feedback from expert reviewers to reach consensus on inclusion into a domain; and 7) validation of items. Results A total of 170 items representing twelve domains were identified as potential barriers to eating a healthful diet-- knowledge, optimism, beliefs about consequences, beliefs about capabilities, reinforcement, memory, attention and decision processes, environmental context and resources, social influences, emotion, behavioral regulation, health identity, and functional status. Conclusion Expert review consultation and a consensus approach established content validity for 12 theory-based domains comprised of 170 items identified as potential barriers to eating a healthful diet. The use of these explanatory domains may: assist researchers to better understand barriers to adult dietary practices; inform the development of a screening tool that could be used in a community setting to measure barriers to eating a healthful diet; and inform individualized interventions.

Background: Cancer is the second-leading cause of death in the United States. Clinical trials translate basic science discoveries into treatments needed by cancer patients. Inadequate accrual of trial participants is one of the most significant barriers to the completion of oncology clinical trials. Objective: The purpose of this study was to investigate trial-level factors that affect accrual and/or completion of oncology clinical trials, identify gaps in the literature, and indicate opportunities for future research. Design: A systematic review of the literature on trial-level factors that affect accrual and/or completion of oncology clinical trials was performed. Searches in PubMed and Scopus identified 6582 studies. Based on eligibility criteria, 16 studies were selected for the review. Results were analyzed according to the following: a) background factors, b) disease-related, c) treatment-related, and d) trial design. Results: Background factors that were investigated in relation to oncology clinical trial accrual and/or completion included sponsor, number and location of participating institutions, competing trials, time of trial opening, and fast-track status. Disease-related factors included the annual incidence and type(s) of targeted cancer. Several types of treatment such as drugs, radiation and surgery were examined in the studies. Trial design factors included trial development time, eligibility criteria, randomization, sample size, trial phase, placebo use, and required protocol procedures and their timing. Conclusion: With low patient participation rates in oncology clinical trials that hold promise for future treatments, it is imperative that trial-level factors affecting accrual be identified and addressed to facilitate the completion of trials.

Abstract Introduction We describe findings from a large study that provide empirical support for the emerging construct of cognitive frailty and put forth a theoretical framework that may advance the future study of complex aging conditions. While cognitive impairment and physical frailty have long been studied as separate constructs, recent studies suggest they share common etiologies. We aimed to create a population predictive model to gain an understanding of the underlying biological mechanisms for the relationship between physical frailty and cognitive impairment. Methods Data were obtained from the longitudinal “Invecchaiare in Chianti” (Aging in Chianti, InCHIANTI Study) with a representative sample (n = 1453) of older adults from two small towns in Tuscany, Italy. Our previous work informed the candidate 132 single nucleotide polymorphisms (SNPs) and 155 protein biomarkers we tested in association with clinical outcomes using a tree boosting, machine learning (ML) technique for supervised learning analysis. Results We developed two highly accurate predictive models, with a Model I area under the curve (AUC) of 0.88 (95% confidence interval [CI] 0.83‐0.90) and a Model II AUC of 0.86 (95% CI 0.80‐0.90). These models indicate cognitive frailty is driven by dysregulation across multiple cellular processes including genetic alterations, nutrient and lipid metabolism, and elevated levels of circulating pro‐inflammatory proteins. Discussion While our results establish a foundation for understanding the underlying biological mechanisms for the relationship between cognitive decline and physical frailty, further examination of the molecular pathways associated with our predictive biomarkers is warranted. Our framework is in alignment with other proposed biological underpinnings of Alzheimer's disease such as genetic alterations, immune system dysfunction, and neuroinflammation.

Background Cardiovascular risk factor burden in the absence of clinical or radiological “events” is associated with mild cognitive impairment. Magnetic resonance imaging techniques exploring the integrity of neuronal fiber connectivity within white matter networks supporting cognitive processing could be used to measure the impact of cardiovascular disease on brain health and be used beyond bedside neuropsychological tests to detect subclinical changes and select or stratify participants for entry into clinical trials. Methods and Results We assessed the relationship between verbal IQ and brain network integrity and the effect of cardiovascular risk factors on network integrity by constructing whole‐brain structural connectomes from magnetic resonance imaging diffusion images (N=60) from people with various degrees of cardiovascular risk factor burden. We measured axonal integrity by calculating network density and determined the effect of fiber loss on network topology and efficiency, using graph theory. Multivariate analyses were used to evaluate the relationship between cardiovascular risk factor burden, physical activity, age, education, white matter integrity, and verbal IQ. Reduced network density, resulting from a disproportionate loss of long‐range white matter fibers, was associated with white matter network fragmentation (r=−0.52, P

Background. The purpose of the Living Organ Video Educated Donors (LOVED) program is to address living donor kidney transplantation (LDKT) disparities in African Americans who experience half the rates of LDKTs compared with whites in the United States. Methods. LOVED is an iterative-designed, distance-based, navigator-led, mobile health educational program, developed via guidance from patients, Self-determination Theory and Social Cognitive Theory. The purpose of this study was to assess the feasibility of LOVED using a proof-of-concept design to increase African Americans’ knowledge about the living donor process to improve their willingness to approach others about being a potential kidney donor. The 8-week LOVED program consisted of (1) a computer tablet-delivered education program, (2) group video chat sessions with an African American LDKT recipient navigator, and (3) additional communication between group members and the navigator. Results. Twenty-five participants completed the LOVED study. Poststudy focus groups were analyzed using inductive and deductive qualitative descriptive techniques and analysts found 6 themes reporting LOVED being highly acceptable with the program being overall empowering and helpful. The study had 0% attrition, 90.9% adherence to the education videos and 88% adherence to the video chat sessions. Surveys showed statistically significant improvements in LDKT knowledge, higher willingness to communicate to others about their need, lower donor concerns, and higher self-efficacy about LDKT. Conclusions. Findings indicate that participation in LOVED can lead to improvements in predictors to increase the number of evaluations and LDKTs in African Americans; however, these findings need to be confirmed in adequately powered, randomized controlled trials. Using LOVED may reduce barriers to broadly address LDKT disparities. Findings from this study will inform the design of a future statewide randomized controlled trials.

Purpose: To explore older rural women’s perceptions of an Internet assisted weight loss program. Sample: The sample (n = 24) included white women (70%) and black women (30%) age 55 and older recruited from two rural senior centers in the Appalachian region of North Carolina. Method: A qualitative design was used to explore rural older obese women’s perceptions of an Internet assisted weight loss program using semi-structured interviews. A total of six focus groups were held with a convenience sample (n=24) of Appalachian adult women (age 55 and older) from two community sites. Findings: The 24 women gave their perspective on the use of the Internet for weight loss. Six themes emerged from the two groups were: awareness, use of the Internet, personal relationships, self-image, peer support, and obstacles Conclusions: Weight loss is a sensitive subject matter for rural obese older women. Evidence from this study suggests that rural women lack social support for weight loss. Nurses may assist women living in rural areas with weight loss through the use of the Internet weight loss social support program in which older obese women can access in the privacy of their own homes. Keywords: Rural, Technology, Appalachian Region, and Older Adults DOI: http://dx.doi.org/10.14574/ojrnhc.v14i2.324

The population of older adults with chronic disease is increasing, yet little is known about their perception of chronic disease and self-management. To develop successful and sustainable chronic disease self-management interventions in the older adult population, health care providers must first understand older adults’ attitudes toward health status and behavior change. This pilot study was conducted to determine the feasibility of the study design. The purpose of this pilot study was to assess the appropriateness of using a mixed-methods research design to investigate Appalachian older adult’s attitude toward the chronic disease experience and health behavior change. A convergent, parallel mixed-method design included a quantitative questionnaire and qualitative focus groups in churches in northeast Tennessee. The aim of the study was met, and the methodology of the study was found to be feasible for larger studies. Divergence of data was found when evaluating qualitative and quantitative data. The study instrument was found to be reliable for future use. The implications of the results suggest that the study design is appropriate for the purpose of the study.

Purpose: Children with complex chronic conditions (CCC) have high health care needs and utilization. The purpose of this study was to identify place-related risk and protective factors associated with hospital utilization among children with CCC. Methods: A mixed methods study was conducted to generate a risk profile of place-related factors associated with hospital utilization. The quantitative phase of the study consisted of retrospective review of 216 urban hospital medical records of infants and young children with CCC. The qualitative phase of the study included interviews with parents/caregivers and focus groups with health care providers (HCPs) of children with CCC. Results from multiple regression modeling and directed content analysis were merged using a side-by-side table organized by ecological level. Findings: Place-related risk and protective factors on multiple ecological levels were identified. Key place-related factors associated with hospital utilization were more complex conditions, positive relationships with HCPs, more parent/family resources, and having fewer place-related resources. Conclusions: The results of this study suggest patterns of health care utilization among infants and young children with CCC are influenced by place-related factors. Parent relationships with HCPs and comfort with care, hospital resources, and parent resources determine location of care. Results of this study also imply place-related disparities in access to care, especially among children in smaller metropolitan areas and rural-dwelling children. DOI: http://dx.doi.org/ 10.14574/ojrnhc.v15i2.364 Keywords: Rural population, Rural health services, Infant, Child, Preschool, Chronic disease

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (OMIM 201450) is the most common inherited disorder of fatty acid metabolism presenting with hypoglycaemia, hepatopathy and Reye-like symptoms during catabolism. In the past, the majority of patients carried the prevalent c.985A>G mutation in the ACADM gene. Since the introduction of newborn screening many other mutations with unknown clinical relevance have been identified in asymptomatic newborns. In order to identify functional effects of these mutant genotypes we correlated residual MCAD (OMIM 607008) activities as measured by octanoyl-CoA oxidation in lymphocytes with both genotype and relevant medical reports in 65 newborns harbouring mutant alleles. We identified true disease-causing mutations with residual activities of 0 to 20%. In individuals carrying the c.199T>C or c.127G>A mutation on one allele, residual activities were much higher and in the range of heterozygotes (31%-60%). Therefore, both mutations cannot clearly be associated with a clinical phenotype. This demonstrates a correlation between the octanoyl-CoA oxidation rate in lymphocytes and the clinical outcome. With newborn screening, the natural course of disease is difficult to assess. The octanoyl-CoA oxidation rate, therefore, allows a risk assessment at birth and the identification of new ACADM genotypes associated with asymptomatic disease variants.

Objective. Current generation smartphones' video camera technologies enable photoplethysmographic (PPG) acquisition and heart rate (HR) measurement. The study objective was to develop an Android application and compare HRs derived from a Motorola Droid to electrocardiograph (ECG) and Nonin 9560BT pulse oximeter readings during various movement-free tasks. Materials and Methods. HRs were collected simultaneously from 14 subjects, ages 20 to 58, healthy or with clinical conditions, using the 3 devices during 5-minute periods while at rest, reading aloud under observation, and playing a video game. Correlation between the 3 devices was determined, and Bland-Altman plots for all possible pairs of devices across all conditions assessed agreement. Results. Across conditions, all device pairs showed high correlations. Bland-Altman plots further revealed the Droid as a valid measure for HR acquisition. Across all conditions, the Droid compared to ECG, 95% of the data points (differences between devices) fell within the limits of agreement. Conclusion. The Android application provides valid HRs at varying levels of movement free mental/perceptual motor exertion. Lack of electrode patches or wireless sensor telemetric straps make it advantageous for use in mobile-cell-phone-delivered health promotion and wellness programs. Further validation is needed to determine its applicability while engaging in physical movement-related activities.

Walking, more specifically gait, is an essential component of daily living. Walking is a very different activity for individuals with a Body Mass Index (BMI) of 40 or more (Class III obesity) compared with those who are overweight or obese with a BMI between 26–35. Yet all obesity weight classes receive the same physical activity guidelines and recommendations. This observational study examined the components of function and disability in a group with Class III obesity and a group that is overweight or has Class I obesity. Significant differences were found between the groups in the areas of gait, body size, health condition, and activity capacity and participation. The Timed Up and Go test, gait velocity, hip circumference, and stance width appear to be most predictive of activity capacity as observed during gait assessment. The findings indicate that Class III-related gait is pathologic and not a normal adaptation.

BACKGROUND:Newborn screening for medium- and very long-chain acyl-CoA dehydrogenase (MCAD and VLCAD, respectively) deficiency, using acylcarnitine profiling with tandem mass spectrometry, has increased the number of patients with fatty acid oxidation disorders due to the identification of additional milder, and so far silent, phenotypes. However, especially for VLCADD, the acylcarnitine profile can not constitute the sole parameter in order to reliably confirm disease. Therefore, we developed a new liquid chromatography tandem mass spectrometry (LC-MS/MS) method to rapidly determine both MCAD- and/or VLCAD-activity in human lymphocytes in order to confirm diagnosis. METHODOLOGY:LC-MS/MS was used to measure MCAD- or VLCAD-catalyzed production of enoyl-CoA and hydroxyacyl-CoA, in human lymphocytes. PRINCIPAL FINDINGS:VLCAD activity in controls was 6.95+/-0.42 mU/mg (range 1.95 to 11.91 mU/mg). Residual VLCAD activity of 4 patients with confirmed VLCAD-deficiency was between 0.3 and 1.1%. Heterozygous ACADVL mutation carriers showed residual VLCAD activities of 23.7 to 54.2%. MCAD activity in controls was 2.38+/-0.18 mU/mg. In total, 28 patients with suspected MCAD-deficiency were assayed. Nearly all patients with residual MCAD activities below 2.5% were homozygous 985A>G carriers. MCAD-deficient patients with one other than the 985A>G mutation had higher MCAD residual activities, ranging from 5.7 to 13.9%. All patients with the 199T>C mutation had residual activities above 10%. CONCLUSIONS:Our newly developed LC-MS/MS method is able to provide ample sensitivity to correctly and rapidly determine MCAD and VLCAD residual activity in human lymphocytes. Importantly, based on measured MCAD residual activities in correlation with genotype, new insights were obtained on the expected clinical phenotype.