An Exploration of People Living with Parkinson's Experience of Cardio-Drumming; Parkinson's Beats: A Qualitative Phenomenological Study.

Research has shown that physical activity has a range of benefits for people living with Parkinson's (PLwP), improving muscle strength, balance, flexibility, and walking, as well as non-motor symptoms such as mood. Parkinson's Beats is a form of cardio-drumming, specifically adapted for PLwP, and requires no previous experience nor skills. Nineteen PLwP (aged between 55 and 80) took part in the regular Parkinson's Beats sessions in-person or online. Focus group discussions took place after twelve weeks to understand the impacts of Parkinson's Beats. Through the framework analysis, six themes and fifteen subthemes were generated. Participants reported a range of benefits of cardio-drumming, including improved fitness and movement, positive mood, the flow experience, and enhanced social wellbeing. A few barriers to participation were also reported. Future research is justified, and best practice guidelines are needed to inform healthcare professionals, PLwP and their care givers.

Parental concerns about genital differences in children with congenital adrenal hyperplasia persist regardless of the selected intervention.

BACKGROUND: Congenital Adrenal Hyperplasia (CAH) due to 21-hydroxylase deficiency (21HD) can affect the in utero development of the genital anatomy of people with the 46XX karyotype. Health professionals engage parents in decision-making regarding managing genitals with this difference, including genital surgery options and patient communication. AIM: We sought to investigate parental communication with their daughters regarding clitoral size variation related to neonatal CAH. METHODS: Semistructured in-person interviews of 24 parents of chromosomal XX children with clitoral size variation attributable to a neonatal CAH diagnosis comprised 3 management categories: (1) clitoral reduction surgery (RS) (7 parents, 9 children), (2) clitoral concealment surgery (CS) (8 parents, 8 children), and no surgery on or around the clitoris (NS) (9 parents, 7 children). OUTCOMES: Four representative themes, Obvious Choice, Still Different, Parental Burden, and Ignorance Is Bliss, were common across all 3 treatment groups. RESULTS: For most parents, none of the 3 options of genital appearance alteration via clitoral reduction, clitoral concealment surgery, or avoidance of clitoral surgery ameliorated concerns, with most parents expressing an aversion to educating their child on the topic of genital differences, past treatment, or future function. CLINICAL IMPLICATIONS: Reliance on surgical treatment pathways to manage this psychosocial concern is ineffective in alleviating parental uncertainty without the application of psychosocial interventions. STRENGTHS AND LIMITATIONS: This was a qualitative study but was limited to parents of children with a specific genital difference, without direct exploration of parental values regarding the clitoris or the application of adequate psychosocial care. CONCLUSION: Healthcare services must have an impact on parental ability to engage in essential communication with their children in cases such as clitoral size variation related to neonatal CAH. Improved communication skills allow parents to engage in more genuine decision-making and adapt to enduring genital reality, including possible future sexual challenges for their adult child, without resorting to burdensome strategies focused on attempts to perpetuate a benevolent ignorance.

What is available to support pain management in Parkinson's: a scoping review protocol.

OBJECTIVE: A scoping review will be undertaken to examine and map the available evidence that has been produced in relation to pain management in Parkinson's, with a focus on behavioural interventions, resources and/or how professionals support people with Parkinson's self-management of pain. METHODS: This review will be based on the methodological framework given by Arksey and O'Malley's (2005), including enhancements by Levac et al., Peters et al. and the Joanna Briggs Institute. We will include studies from PubMed, SCOPUS, CINAHL, MEDLINE Web of Science, APA PsycINFO and ASSIA from January, 2010 onwards. Both quantitative and qualitative data will be analysed separately to identify the characteristics of support for pain management available, orientation of the approach and any identifiable behaviour change components and their outcomes. The COM-B behaviour change model and Theoretical Domains Framework will provide a theoretical framework for synthesising evidence in this review. CONCLUSION: This scoping review will help to explore studies focusing on the evidence supporting a range of interventions relating to the management of pain experienced by people living with Parkinson's. The focus will be on describing what is available to support self-management, identify what behaviour change components have been used and their effectiveness, identify barriers and enablers to pain management and explore gaps in current provision of pain management. This review will identify implications and priorities for the follow-up phases to the larger 'Pain in Parkinson's' Project which is designed to support clinicians and individuals living with Parkinson's.

Integration of nurse-led virtual reviews with growth hormone device-linked patient adherence information: a mixed methods feasibility study.

Introduction: Easypod-connect™ for childhood growth disorders is a unique connected system that enables transmission of injection adherence information for recombinant human growth hormone (r-hGH). Although this system has the potential to facilitate greater adherence, observational studies have shown declining adherence over prolonged periods when used without additional support. Supplemental nurse practitioner support has been envisaged but not investigated; in this study, we have undertaken feasibility analysis of nurse-led virtual reviews (NVR) in combination with easypod-connect™ in a single centre using quantitative and qualitative analyses. Aims: We aimed to test feasibility by assessing compliance with NVR, height standard deviation score (SDS) gain, adherence improvement and patient opinions. Methods: Patients using easypod™ r-hGH were recruited prospectively to a 12-month study with two telephone NVR appointments in addition to standard of care in-person hospital outpatient visits. A subset was recruited for a semi-structured interview for qualitative thematic analysis. Results: Forty-three patients of median (range) age 10.7 (6.7, 15.2) were recruited for a period of 1.1 (0.7, 1.8) years. Thirty-three (76.7%) patients were fully compliant with NVR integration with easypod-connect™, establishing feasibility. Median (inter-quartile range, IQR) height SDS improved from -1.85 (-2.44, -1.37) to -1.48 (-2.14, -1.07) (p<0.001) while adherence remained similar in the majority from study start [96.5 (88.8, 100.0)] to end [99.0 (94.0, 100.0)]. Qualitative analysis identified themes supporting patient benefit: practicalities of appointments, perceived purpose and significance of virtual reviews, and the importance of optimising growth. Four patients complained of injection pain, of whom two switched to an alternative r-hGH device. Conclusion: Our study has demonstrated the feasibility of nurse-led virtual review integration with easypod-connect™ in a mixed methods study, laying the foundation for research in larger groups over longer periods. Nurse practitioner supported application of easypod-connect™ offers the potential for improved growth outcomes in all r-hGH devices providing adherence information.

Macadamia nut effects on cardiometabolic risk factors: a randomised trial.

We sought to examine the effects of daily consumption of macadamia nuts on body weight and composition, plasma lipids and glycaemic parameters in a free-living environment in overweight and obese adults at elevated cardiometabolic risk. Utilising a randomised cross-over design, thirty-five adults with abdominal obesity consumed their usual diet plus macadamia nuts (~15 % of daily calories) for 8 weeks (intervention) and their usual diet without nuts for 8 weeks (control), with a 2-week washout. Body composition was determined by bioelectrical impedance; dietary intake was assessed with 24-h dietary recalls. Consumption of macadamia nuts led to increased total fat and MUFA intake while SFA intake was unaltered. With mixed model regression analysis, no significant changes in mean weight, BMI, waist circumference, percent body fat or glycaemic parameters, and non-significant reductions in plasma total cholesterol of 2⋅1 % (-4⋅3 mg/dl; 95 % CI -14⋅8, 6⋅1) and low-density lipoprotein (LDL-C) of 4 % (-4⋅7 mg/dl; 95 % CI -14⋅3, 4⋅8) were observed. Cholesterol-lowering effects were modified by adiposity: greater lipid lowering occurred in those with overweight v. obesity, and in those with less than the median percent body fat. Daily consumption of macadamia nuts does not lead to gains in weight or body fat under free-living conditions in overweight or obese adults; non-significant cholesterol lowering occurred without altering saturated fat intake of similar magnitude to cholesterol lowering seen with other nuts. Clinical Trial Registry Number and Website: NCT03801837 https://clinicaltrials.gov/ct2/show/NCT03801837?term = macadamia + nut&draw = 2&rank = 1.

RNA analysis of intronic variants in the LAMA2 gene detected by whole genome sequencing confirms a rare dual diagnosis of incontinentia pigmenti with limb-girdle muscular dystrophy.

We see that a multiple methods approach to diagnosis remains necessary in the era of whole genome sequencing. We also observe that reproductive risk genetic counseling can be a motivating factor for further testing along the diagnostic odyssey.

IgE Depletion with Ligelizumab Does Not Significantly Improve Clinical Symptoms in Patients with Moderate-to-Severe Atopic Dermatitis.

BACKGROUND: The value, if any, of anti-IgE approaches in the treatment of atopic dermatitis has not been fully clarified. Studies using the anti-IgE omalizumab have yielded conflicting results. OBJECTIVE: Antibodies with an IgE-suppressive capacity stronger than omalizumab might be more efficacious. STUDY DESIGN: We assessed the safety and efficacy of the high-affinity anti-IgE antibody ligelizumab (280 mg, subcutaneous, every other week) in 22 adult patients with moderate-to-severe atopic dermatitis in a placebo and active (cyclosporine A) controlled, randomized, multicenter, double-blind clinical trial for 12 weeks. RESULTS: We found that ligelizumab treatment resulted in either complete (patients with baseline IgE < 1,500 IU/ml) or partial (baseline IgE > 1,500 IU/ml) suppression of serum and cell-bound IgE as well as of allergic skin prick tests. On the other hand, ligelizumab-as opposed to cyclosporine A-was not significantly superior to placebo in inducing Eczema Area and Severity Index 50 response or significantly reducing pruritus and sleep disturbance. Interestingly though, patients with high baseline IgE exhibited a slightly but not significantly better treatment response than those with low baseline IgE. CONCLUSION: Our study shows that an immunologically efficacious anti-IgE approach is not clearly superior to placebo in treating atopic dermatitis. Larger studies are needed to determine whether certain patient subgroups may benefit from this strategy. TRIAL REGISTRATION: The study was registered in 2011 at clinicaltrialsregister.eu, EudraCT Number 2011-002112-84.

Application of a New Carbohydrate Food Quality Scoring System: An Expert Panel Report.

Carbohydrate foods (≥40% energy from carbohydrates) are the main source of energy in the US diet. In contrast to national-level dietary guidance, many regularly consumed carbohydrate foods are low in fiber and whole grains but high in added sugar, sodium, and/or saturated fat. Given the important contribution of higher-quality carbohydrate foods to affordable healthy diets, new metrics are needed to convey the concept of carbohydrate quality to policymakers, food industry stakeholders, health professionals, and consumers. The recently developed Carbohydrate Food Quality Scoring System is well aligned with multiple key healthy messages on nutrients of public health concern from the 2020-2025 Dietary Guidelines for Americans. Two models are described in a previously published paper: one for all non-grain carbohydrate-rich foods (e.g., fruits, vegetables, legumes) known as the Carbohydrate Food Quality Score-4 (CFQS-4), and one for grain foods only known as the Carbohydrate Food Quality Score-5 (CFQS-5). These CFQS models provide a new tool that can guide policy, programs, and people towards improved carbohydrate food choices. Specifically, the CFQS models represent a way to unify and reconcile diverse ways to describe different types of carbohydrate-rich foods (e.g., refined vs. whole, starchy vs. non-starchy, dark green vs. red/orange) and make for more useful and informative messaging that better aligns with a food's nutritional and/or health contributions. The present paper's aims are to show that the CFQS models can inform future dietary guidelines and help support carbohydrate food recommendations with other health messages aimed at promoting foods that are nutrient-dense, fiber-rich, and low in added sugar.

The global historical climate database HCLIM.

There is a growing need for past weather and climate data to support science and decision-making. This paper describes the compilation and construction of a global multivariable (air temperature, pressure, precipitation sum, number of precipitation days) monthly instrumental climate database that encompasses a substantial body of the known early instrumental time series. The dataset contains series compiled from existing databases that start before 1890 (though continuing to the present) as well as a large amount of newly rescued data. All series underwent a quality control procedure and subdaily series were processed to monthly mean values. An inventory was compiled, and the collection was deduplicated based on coordinates and mutual correlations. The data are provided in a common format accompanied by the inventory. The collection totals 12452 meteorological records in 118 countries. The data can be used for climate reconstructions and analyses. It is the most comprehensive global monthly climate dataset for the preindustrial period so far.

Anti-IL-17A blockade did not significantly reduce inflammatory lesions in a placebo-controlled pilot study in adult patients with moderate to severe acne.

BACKGROUND: CJM112 is a potent anti-IL-17A monoclonal antibody, whose clinical efficacy in psoriasis was recently documented. This study aimed to assess the effect of IL-17A blockade, using CJM112, in patients with moderate to severe acne. METHODS: A randomized, placebo-controlled, double-blind, parallel-group, proof-of-concept study was conducted on patients with moderate to severe acne. Patients received CJM112 300 mg, 75 mg, or placebo subcutaneously during Treatment Period 1 (0-12 weeks). Patients receiving placebo were re-randomized to receive CJM112 300 mg or 75 mg during Treatment Period 2 (12-24 weeks). The primary endpoint was the number of inflammatory facial lesions at Week 12. RESULTS: As the futility criterion was met during the interim analysis, only 52/75 (69.3%) patients were recruited. In total, 48/52 (92.3%) and 26/41 (63.4%) completed Treatment Periods 1 and 2, respectively. All groups exhibited a reduction in facial inflammatory lesions, with no difference observed between CJM112 and placebo (CJM112 300 mg 27.6 ± 20.7; CJM112 75 mg 30.4 ± 34.8; placebo 23.6 ± 13.6; primary endpoint). Additionally, no differences were observed between groups in other secondary and exploratory endpoints at Week 12. CONCLUSIONS: Anti-IL-17A therapy was not significantly different compared to the placebo in reducing inflammatory lesions in patients with moderate to severe acne.

Clinical and functional characterization of germline PIK3CA variants in patients with PIK3CA-related overgrowth spectrum disorders.

Mosaic variants in the PIK3CA gene, encoding the catalytic subunit of phosphoinositide 3-kinase (PI3K), produce constitutive PI3K activation, which causes PIK3CA-related overgrowth spectrum disorders. To date, fewer than 20 patients have been described with germline alterations in PIK3CA. In this study, we describe three unrelated individuals with overgrowth and germline PIK3CA variants. These variants were discovered through whole-exome sequencing and confirmed as germline by testing multiple tissue types, when available. Functional analysis using Patient 1's fibroblast cell line and two previously reported patients' cell lines showed increased phosphorylation of AKT during cellular starvation revealing constitutive activation of the phosphoinositide-3-kinase/protein kinase B/mechanistic target of rapamycin (PI3K/AKT/mTOR) pathway. Alternatively, stimulation of the cells by fetal bovine serum produced a reduced response, indicating an activated status of the PI3K complex reducing the pathway response to further external stimulation. Additional studies utilizing Biolog Phenotype Microarray technology indicated reduced energy production when cells were exposed to growth factors stimulating the PI3K/AKT/mTOR pathway, confirming the trend observed in the AKT phosphorylation test after stimulation. Furthermore, treatment with inhibitors of the PI3K/AKT/mTOR pathway rescued the normal energy response in the patients' cells. Collectively, these data demonstrate that disease-causing germline PIK3CA variants have a functional consequence, similar to mosaic variants in the PI3K/AKT/mTOR pathway.

Functional assessment of homozygous ALDH18A1 variants reveals alterations in amino acid and antioxidant metabolism.

Mono- and bi-allelic variants in ALDH18A1 cause a spectrum of human disorders associated with cutaneous and neurological findings that overlap with both cutis laxa and spastic paraplegia. ALDH18A1 encodes the bifunctional enzyme pyrroline-5-carboxylate synthetase (P5CS) that plays a role in the de novo biosynthesis of proline and ornithine. Here we characterize a previously unreported homozygous ALDH18A1 variant (p.Thr331Pro) in four affected probands from two unrelated families, and demonstrate broad-based alterations in amino acid and antioxidant metabolism. These four patients exhibit variable developmental delay, neurological deficits and loose skin. Functional characterization of the p.Thr331Pro variant demonstrated a lack of any impact on the steady-state level of the P5CS monomer or mitochondrial localization of the enzyme, but reduced incorporation of the monomer into P5CS oligomers. Using an unlabeled NMR-based metabolomics approach in patient fibroblasts and ALDH18A1-null human embryonic kidney cells expressing the variant P5CS, we identified reduced abundance of glutamate and several metabolites derived from glutamate, including proline and glutathione. Biosynthesis of the polyamine putrescine, derived from ornithine, was also decreased in patient fibroblasts, highlighting the functional consequence on another metabolic pathway involved in antioxidant responses in the cell. RNA sequencing of patient fibroblasts revealed transcript abundance changes in several metabolic and extracellular matrix-related genes, adding further insight into pathogenic processes associated with impaired P5CS function. Together these findings shed new light on amino acid and antioxidant pathways associated with ALDH18A1-related disorders, and underscore the value of metabolomic and transcriptomic profiling to discover new pathways that impact disease pathogenesis.

Mosaicism of common pathogenic MECP2 variants identified in two males with a clinical diagnosis of Rett syndrome.

Rett (RTT) syndrome, a neurodevelopmental disorder caused by pathogenic variation in the MECP2 gene, is characterized by developmental regression, loss of purposeful hand movements, stereotypic hand movements, abnormal gait, and loss of spoken language. Due to the X-linked inheritance pattern, RTT is typically limited to females. Recent studies revealed somatic mosaicism in MECP2 in male patients with RTT-like phenotypes. While detecting mosaic variation using Sanger sequencing is theoretically possible for mosaicism over ~15%-20%, several variables, including efficiency of PCR, background noise, and/or human error, contribute to a low detection rate using this technology. Mosaic variants in two males were detected by next generation sequencing (NGS; Case 1) and by Sanger re-sequencing (Case 2). Both had targeted digital PCR (dPCR) to confirm the variants. In this report, we present two males with classic RTT syndrome in whom we identified pathogenic variation in the MECP2 gene in the mosaic state (c.730C > T (p.Gln244*) in Patient 1 and c.397C > T (p.Arg133Cys) in Patient 2). In addition, estimates and measures of mosaic variant fraction were surprisingly similar between Sanger sequencing, NGS, and dPCR. The mosaic state of these variants contributed to a lengthy diagnostic odyssey for these patients. While NGS and even Sanger sequencing may be viable methods of detecting mosaic variation in DNA or RNA samples, applying targeted dPCR to supplement these sequencing technologies would provide confirmation of somatic mosaicism and mosaic fraction.

A Systematic Review on Physical Health Education Interventions for People with Parkinson's Disease: Content, Impact, and Implementation Considerations Across the Parkinson's Trajectory.

BACKGROUND: Educational interventions promoting the role of physical activity (PA) aim to address knowledge, poor exercise self-efficacy, and low outcome expectations, which are well-researched barriers to PA participation in healthy and in people with chronic conditions. However, little is known about the effectiveness of educational interventions in addressing these barriers in people with Parkinson's (PwP). OBJECTIVE: To examine the content of education interventions that promote PA behavior in PwP, and to assess their effectiveness on physical and psychosocial outcomes. METHODS: An electronic search (12/2021) of MEDLINE, EMBASE, CINAHL, PubMed PsycINFO, the Web of Science and the Cochrane Library was conducted from 1990 to 2021. Education interventions, alone or combined with other strategies, promoting PA in PwP were included. Quality was assessed using the Johanna Briggs Institute and National Institute of Health quality assessment tools. A narrative synthesis was performed. RESULTS: Six studies were identified. Five interventions were comprised of education and exercise sessions. Improvement in physical and psychosocial outcomes were suggested but delineating the exact impact of education was impeded due to lack of assessment. CONCLUSION: Few interventions exist that provide knowledge, and skills promoting PA participation, and fewer are addressed towards newly diagnosed PwP. There is lack of assessment over the effectiveness of education as a tool to facilitate PA participation in PwP. Lack of assessment poses the risk of potentially disregarding effective interventions or adopting ineffective approaches without the evidence. Education interventions can boost PA engagement by increasing factors such as exercise self-efficacy, but further interventions are required to assess this model of relationship.

SEMA6B variants cause intellectual disability and alter dendritic spine density and axon guidance.

Intellectual disability (ID) is a neurodevelopmental disorder frequently caused by monogenic defects. In this study, we collected 14 SEMA6B heterozygous variants in 16 unrelated patients referred for ID to different centers. Whereas, until now, SEMA6B variants have mainly been reported in patients with progressive myoclonic epilepsy, our study indicates that the clinical spectrum is wider and also includes non-syndromic ID without epilepsy or myoclonus. To assess the pathogenicity of these variants, selected mutated forms of Sema6b were overexpressed in Human Embryonic Kidney 293T (HEK293T) cells and in primary neuronal cultures. shRNAs targeting Sema6b were also used in neuronal cultures to measure the impact of the decreased Sema6b expression on morphogenesis and synaptogenesis. The overexpression of some variants leads to a subcellular mislocalization of SEMA6B protein in HEK293T cells and to a reduced spine density owing to loss of mature spines in neuronal cultures. Sema6b knockdown also impairs spine density and spine maturation. In addition, we conducted in vivo rescue experiments in chicken embryos with the selected mutated forms of Sema6b expressed in commissural neurons after knockdown of endogenous SEMA6B. We observed that expression of these variants in commissural neurons fails to rescue the normal axon pathway. In conclusion, identification of SEMA6B variants in patients presenting with an overlapping phenotype with ID and functional studies highlight the important role of SEMA6B in neuronal development, notably in spine formation and maturation and in axon guidance. This study adds SEMA6B to the list of ID-related genes.

A New Carbohydrate Food Quality Scoring System to Reflect Dietary Guidelines: An Expert Panel Report.

Existing metrics of carbohydrate food quality have been based, for the most part, on favorable fiber- and free sugar-to-carbohydrate ratios. In these metrics, higher nutritional quality carbohydrate foods are defined as those with >10% fiber and <10% free sugar per 100 g carbohydrate. Although fiber- and sugar-based metrics may help to differentiate the nutritional quality of various types of grain products, they may not aptly capture the nutritional quality of other healthy carbohydrate foods, including beans, legumes, vegetables, and fruits. Carbohydrate food quality metrics need to be applicable across these diverse food groups. This report introduces a new carbohydrate food scoring system known as a Carbohydrate Food Quality Score (CFQS), which supplements the fiber and free sugar components of previous metrics with additional dietary components of public health concern (e.g., sodium, potassium, and whole grains) as identified by the Dietary Guidelines for Americans. Two CFQS models are developed and tested in this study: one that includes four dietary components (CFQS-4: fiber, free sugars, sodium, potassium) and one that considers five dietary components (CFQS-5: fiber, free sugars, sodium, potassium, and whole grains). These models are applied to 2596 carbohydrate foods in the Food and Nutrient Database for Dietary Studies (FNDDS) 2017−2018. Consistent with past studies, the new carbohydrate food scoring system places large percentages of beans, vegetables, and fruits among the top scoring carbohydrate foods. The whole grain component, which only applies to grain foods (N = 1561), identifies ready-to-eat cereals, oatmeal, other cooked cereals, and selected whole grain breads and crackers as higher-quality carbohydrate foods. The new carbohydrate food scoring system shows a high correlation with the Nutrient Rich Food (NRF9.3) index and the Nutri-Score. Metrics of carbohydrate food quality that incorporate whole grains, potassium, and sodium, in addition to sugar and fiber, are strategically aligned with multiple 2020−2025 dietary recommendations and may therefore help with the implementation of present and future dietary guidelines.

Clinical assessment of lower urinary tract symptoms in adults.

Lower urinary tract symptoms (LUTS) result from conditions and diseases that affect the bladder and the urethra, and can occur in men and women of all ages. LUTS can be debilitating and affect a person's quality of life significantly. This article describes the definitions and classification of various LUTS, explains the aspects of an initial LUTS assessment and outlines the validated tools that can be used to support this process, including questionnaires and bladder diaries. It also provides information about the contributing factors associated with LUTS and the effects of these symptoms on patients' quality of life.

Fortification of grain foods and NOVA: the potential for altered nutrient intakes while avoiding ultra-processed foods.

PURPOSE: NOVA is a food classification system describing a hierarchy from minimally processed to ultra-processed foods (UPF). Research has associated intake of UPF with chronic diseases. In Australia, the primary sources of grains, both refined and whole, are breads and breakfast cereals, which are typically fortified. Most are classified as UPF, yet are recommended core foods according to the Australian Dietary Guidelines (ADG). This research aimed to identify if avoidance of ultra-processed grain foods would alter nutrient intakes in an Australian population and whether sample diets using substitute (non-UPF) foods would be likely to meet nutrient requirements. METHODS: Quantitative analysis of usual nutrient intake from the National Nutrition and Physical Activity Survey 2011-12 (n = 12,153) for all foods including and excluding UPF. Dietary modelling examined the nutritional adequacy of sample diets aligned with the ADG and another containing replacements for UPF. We particularly focused on grain foods and meeting whole-grain intake targets. RESULTS: There was a significant decrease (all p < 0.05) in modelled intake of key nutrients when UPF were excluded, specifically, thiamin, folate and iodine, as substitutions are rarely fortified. Diets with no UPF, where substitutes are carefully chosen, have the potential to meet Nutrient Reference Values, but deviation from customary food choices may mean adoption of substitutes is unlikely. CONCLUSIONS: Exclusion of UPF may result in lowered intakes of key nutrients of particular concern for at risk groups (including women of child-bearing age), negating gains made by public health policy of fortification. Substitutions may not be realistic in these at-risk populations.