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Background & aims: \ud Obesity prevalence in people with phenylketonuria (PKU) is comparable to that of the general population but the underlying aetiology remains unknown. To assess body composition, dietary intake, moderate physical activity duration (MPAD) and energy expenditure (MPAEE), resting metabolic rate (RMR), diet-induced thermogenesis (DIT), fasting and postprandial fat (FOx) and carbohydrate oxidation (CHOOx), in PKU people and healthy Controls.\ud \ud Methods: \ud Participants were PKU people (n = 16) and healthy controls (n = 15). Body composition was measured with stable isotopes using deuterium as tracer, dietary intake from 4-day food diaries, MPAD and MPAEE from 7-day activity counts measured by triaxial accelerometers, calibrated against individual rates of oxygen consumption and carbon dioxide production, RMR, DIT, FOx and CHOOx by indirect calorimetry.\ud \ud Results: \ud Body composition, DIT, FOx, CHOOx and RMR did not differ between the PKU and the Control groups. MPAD (PKU, 73 ± 26 min/week; Control, 152 ± 43 min/week) and MPAEE (PKU, 404 ± 127 kcal/week; Control, 741 ± 153 kcal/week) were lower (P < 0.05) in the PKU than the Control group. Raised phenylalanine levels were inversely related with MPAD and MPAEE. Energy intake and energy provided by protein did not differ between the groups, while energy proportion obtained from carbohydrate was higher (PKU, 60 ± 2%; Control, 51 ± 2%; P < 0.05) and from fat lower (PKU, 24 ± 2%; Control, 35 ± 3%; P < 0.05) in the PKU than in the Control group.\ud \ud Conclusion: \ud People with PKU spent less time and expend less energy in moderate physical activity and have a higher intake of energy from CHO which may be involved in the underlying mechanisms of obesity in PKU.

Depressive symptoms are associated with age-related cognitive impairment, but the relative risk of specific subtypes of mild cognitive impairment (MCI) conferred by depressive symptoms is unclear. The purpose of this exploratory study was to determine the longitudinal association between baseline depressive symptoms and incident cases of MCI subtypes (amnestic vs. non-amnestic) and probable dementia (PD) (Alzheimer's disease, vascular, mixed) among postmenopausal women.Depressive symptoms were assessed at study baseline using an 8-item Burnam algorithm in 7043 postmenopausal women who participated in the Women's Health Initiative Memory Study (WHIMS) and the WHIMS-Epidemiology of Cognitive Health Outcomes (WHIMS-ECHO) extension study. During the median 9.4-year follow-up interval, the presence of MCI and PD was classified by a central adjudication committee. Classification of participants by MCI subtype (amnestic single and multi-domain, non-amnestic single and multi-domain) was done algorithmically based on established criteria using data from annual cognitive testing.At baseline, 557 women (7.9%) had clinically significant depressive symptoms based on Burnam algorithm cut-point of 0.06. Depressive symptoms at baseline were associated with an increased risk of incident amnestic MCI (hazard ratio [HR] = 1.91, 95% confidence interval [CI] 1.32-2.78, p 0.0001), but not non-amnestic MCI (HR = 1.39, 95% CI 0.91-2.14, p = 0.13) after controlling for demographic factors. This relationship between depressive symptoms and amnestic MCI remained consistent after controlling for lifestyle variables, cardiovascular risk factors, antidepressant use, and history of hormone therapy. There were no significant associations between depressive symptoms and incidence of PD.Depressive symptoms at baseline among postmenopausal older women are associated with higher incidence of amnestic MCI, suggesting that they may be an independent risk factor or part of the early prodrome of dementia.

A low amino acid (AA)/protein diet is the principal treatment for many inherited amino acid disorders (IMDs). Due to their low AA content, plant foods constitute an essential part of diet therapy. However, data on their AA composition are limited, which leads to an estimation of AA intake from protein content rather than an accurate calculation of true AA intake. This study describes the AA content of a total of 73 plant foods (fruits, n = 12; vegetables, n = 51; and other plant foods, n = 10), with the analysis commissioned by the UK National Society for Phenylketonuria (NSPKU) over 15 years. For all fruits and some vegetables (e.g., rocket, watercress and pea shoots), raw samples were used during analysis. All other vegetables were cooked prior to analysis to represent the usual condition of the food at the time of serving. AA analysis was performed with ion exchange chromatography. The median percentage of protein was 2.0% [0.6–5.4%] for the fruits and vegetables analysed (n = 56), although higher in vegetables than in fruits. Each of the five reported AAs (leucine, lysine, phenylalanine, tyrosine, and methionine) supplied 1–5% per g of protein content. From the heterogeneous range of plant foods analysed, the AA/protein ratios differed significantly (2–5% in fruits and 1–9% in vegetables). There was a strong correlation between the amounts of each of the five AAs in the plant foods, but only a small, moderate correlation between the protein and AA content. Overall, this study provides data on the AA content of several plant foods, which are suitable for patients treated with a low AA/protein diet, including many novel plant options. However, only a limited range of fruits and vegetables were analysed due to the high costs of analysis. Hence, more extensive studies with an increased number of plant foods prepared by different cooking methods and replicate samples are necessary, particularly to examine the relationship between the protein and AA content in depth.

Declining physical function significantly worsens quality of life in dementia care dyads. Caring for people living with dementia (PLWD) at home is both physically demanding and emotionally stressful. Physical exercise is strongly recommended as a way of sustaining or improving overall function in dementia care dyads. However, little is known regarding the impact of home-based dyadic exercise interventions. We present results from a secondary analysis of data from a multicomponent translational study, Reducing Disability in Alzheimer's Disease – North West (RDAD-NW). We report results from an examination of the mediating role of exercise on the trajectory of change in physical function over time for both PLWD and their care partners.

To study the relationship between well-being and all-cause mortality, we estimated mortality among women in four classes of well-being using the well-being profile from the Women’s Health Initiative Study (WHI). Demographic characteristics were self-reported at enrollment (1993-98). All-cause mortality included death from any cause between 2012-2020. We used logistic regression to examine all-cause mortality risk across the classes, using Class 4 (highest hedonic and eudaemonic well-being scores) as the referent, adjusting for age and race. Compared to Class 4, all other classes had higher age- and race-adjusted odds of death. Highest risks were in Class 1 women (OR=2.61; 95% CI: 2.46-2.76) and Class 3 women (OR=1.62; 95% CI: 1.55-1.68). Women in Class 4 had the lowest risk of all-cause mortality over an 18-year follow-up. These results confirm the utility of a profile of well-being for predicting all-cause mortality while preserving ability to identify the differences among well-being indicators across classes.

Introduction The nutritional compositions of infant foods for special medical purposes (iFSMPs) are governed by the EU, and new regulations (2016/127; 2016/128) were implemented to ensure standardisation and implementation of latest nutritional recommendations, scheduled to take effect by February 2020. Amongst the changes required, nutrient minimum and maximum levels were redefined, as well as mandatory supplementation of docosahexaenoic acid (DHA). Anecdotal evidence from clinical practice suggests that changes to formulations, minor or otherwise, may affect tolerance and acceptance in infants taking iFSMPs, especially those with complex medical conditions and backgrounds. A case-study series was conducted to evaluate iFSMPs reformulated by Nutricia Ltd to understand any possible impact on patient care. Aim A multi-centre case-study series was conducted in infants and children who took iFSMPs manufactured by Nutricia Ltd for a range of clinical conditions. Gastro-intestinal tolerance, acceptance and compliance were evaluated over 28 days in each case-study. Methods From 17 paediatric centres across the UK, 44 infants and children were recruited [mean age 16.5 m; range 1.5–87], receiving one of the following iFSMPs prescribed for nutrition support relevant to their clinical condition: Infatrini (n=9), Infatrini Peptisorb (n=3), Neocate LCP (n=9), Neocate Syneo (n=1), Kindergen (n=4), Monogen (n=5), Energivit (n=4), Locasol (n=4), Galactomin 19 (n=1), PKU Anamix Infant (n=4). Mean intake of baseline iFSMP was 683±275 ml (which met 97% of prescribed daily volume), of which n=16 administered iFSMPs via enteral feeding tubes (the remaining orally). The managing Dietitian determined the prescribed daily volume of the reformulated iFSMP. Medical history was recorded at baseline, and growth, gastrointestinal tolerance, compliance and acceptance was measured at baseline and end of case-study. Results Forty patients completed the 28-day evaluation (n=4 did not due to medical and other reasons, days on case-study ranged between 1–17). Gastrointestinal tolerance remained stable in the majority of case studies (n=41 including n=1 drop out), and any deviations were not attributed to the reformulated iFSMPs. For the patients that completed the 28-day evaluation, compliance remained stable (n=33), and any reduction was related to increased complementary feeding or medical reasons. Mean intake of reformulated iFSMP was 579±254 ml (which met 91% of prescribed daily volume), where the majority of patients directly transitioned onto the reformulation (n=41). No deterioration in medical conditions or growth were reported as a result of using the reformulated iFSMPs during any of the case studies. Furthermore, caregiver and HCP satisfaction was positively recorded in 89% of case studies. Conclusion This multi-centre, case-study series demonstrates that the minor reformulation of iFSMPs manufactured by Nutricia Ltd in line with the Commission Delegated Regulations (2016/127; 2016/128) to amend nutrient levels and include DHA are well tolerated, accepted and complied with in infants and children with various medical backgrounds. Furthermore, the reformulated iFSMPs continued to support growth and achieved positive caregiver and HCP satisfaction which is paramount to patient care. The reformulated iFSMPs used in this case study series have since been implemented into clinical practice in the UK, with support from Nutricia Ltd, and are now widely accepted.

Summary Background Dietary management of phenylketonuria (PKU) requires the replacement of natural protein-containing foods with special low protein foods. The effect of a PKU type diet on factors contributing to energy balance requires investigation. Objective To investigate the impact of a PKU type meal on appetite ratings, gut appetite hormones, thermic effect of feeding (TEF) and fat oxidation. Methods Twenty-three healthy adults (mean ± SD age: 24.3 ± 5.1 years; BMI: 22.4 ± 2.5 kg/m 2 ) participated in a randomized, crossover design study. Each participant conducted two (PKU and Control) experimental trials which involved consumption of a PKU type meal and protein substitute drink or an isocaloric and weight matched ordinary meal and protein-enriched milk. Appetite, metabolic rate, fat oxidation measurements and blood collections were conducted for the duration of 300 min. On the completion of the measurements ad libitum buffet dinner was served. Results Responses of appetite ratings, plasma concentrations of GLP-1 and PYY ( P > 0.05, trial effect, two-way ANOVA) and energy intake during ad libitum buffet dinner ( P > 0.05, paired t -test) were not significantly different between the two trials. The TEF (PKU, 10.2 ± 1.5%; Control, 13.2 ± 1.0%) and the total amount of fat oxidized (PKU, 18.90 ± 1.10 g; Control, 22.10 ± 1.10 g) were significantly ( P t -tests) lower in the PKU than in the Control trial. The differences in TEF and fat oxidation were significant ( P t -tests) for the post-meal period. Conclusions Consumption of a meal composed of special low protein foods has no detrimental impact on appetite and appetite hormones but produces a lower TEF and postprandial fat oxidation than an ordinary meal. These metabolic alterations may contribute to the increased prevalence of obesity reported in patients with PKU on contemporary dietary management. Clinical trial registration : The trial has been registered in ClinicalTrials as NCT02440932 .

Our aim was to develop a profile of well-being preserving the ability to estimate differential effects of both hedonic and eudaemonic dimensions of well-being on health outcomes. Numerous indicators of well-being from over 80,000 aging women included hedonic (enjoyment of life, happiness, satisfaction with life, quality of life) and eudaemonic (personal growth, purpose in life, environmental mastery, control, self mastery) dimensions. Using latent class analysis, we identified groups of women with distinct profiles of well-being. A four-class solution had both good statistical fit and made conceptual sense. Class 1 (n=9,146, 11%) had the lowest scores on hedonic and eudaemonic indicators, while Class 4 (n=38,246 47%) had the highest levels of all well-being indicators. Class 2 (n=7,106, 9%) had higher hedonic and lower eudaemonic scores and Class 3 (n=26,650, 33%) had lower hedonic and higher eudaemonic scores. These classes form a well-being profile useful for estimating differential effects on health outcomes.

Solid food introduction may create anxiety for parents of children with phenylketonuria (PKU) due to the burden associated with protein substitute (PS) administration and natural protein restriction. In a longitudinal, prospective study, 20 mothers of children with PKU and 20 non-PKU control mothers completed 4 questionnaires (mealtime emotions, feed-time, Beck&rsquo, s anxiety inventory and the coping health inventory for parents), examining parent/child mealtime emotions, anxiety, stress and coping strategies at child ages: weaning start, 8 months (m), 12 m, 15 m, 18 m and 24 m. Overall, mothers of children with PKU cope well with solid food introduction when applying a low-phenylalanine diet, with comparable low levels of stress and anxiety reported in both PKU and non-PKU groups. However, mothers of children with PKU reported peak scores in anxiety for emotive/cognitive symptoms at a child age of 15 m, and higher use of coping strategies at 15 m and 24 m (p &lt, 0.05) of age. Generally, there was a trend that maternal anxiety regarding child rejection of PS increased with time, peaking between 12&ndash, 24 m. In PKU, a child age of 12&ndash, 18 m is identified as a key period when mothers feel most anxious/stressed with feeding, coinciding with raised blood phenylalanine levels probably associated with teething, illness and developing independence. Health professionals should be conscious of this vulnerable period and be prepared to offer more directional support as required.

Growth issues have been observed in young children with phenylketonuria (PKU), but studies are conflicting. In infancy, there is an increasing trend to introduce a second-stage semi-solid weaning protein substitute (WPS) but there is concern that this may not meet energy requirements. In this longitudinal, prospective study, 20 children with PKU transitioning to a WPS, and 20 non-PKU controls were observed monthly from weaning commencement (4&ndash, 6 months) to 12 m and at 15, 18 and 24 months of age for: weight, length, head circumference, body mass index (BMI), energy and macronutrient intake. Growth parameters were within normal range at all ages in both groups with no significant difference in mean z-scores except for accelerated length in the PKU group. No child with PKU had z-scores &lt, &minus, 2 for any growth parameter at age 2 years. Total protein and energy intake in both groups were similar at all ages, however, from 12&ndash, 24 months in the PKU group, the percentage of energy intake from carbohydrate increased (60%) but from fat decreased (25%) and inversely for controls (48% and 36%). In PKU, use of low volume WPS meets Phe-free protein requirements, facilitates transition to solid foods and supports normal growth. Further longitudinal study of growth, body composition and energy/nutrient intakes in early childhood are required to identify any changing trends.

Weaning is complex for children with phenylketonuria (PKU). Breastmilk/infant formula and phenylalanine (Phe)-free infant protein-substitute (PS) are gradually replaced with equivalent amounts of Phe-containing food, a semi-solid/spoonable weaning PS and special low-protein foods. In PKU, feeding patterns/practices during weaning in PKU have not been formally evaluated. In this longitudinal, prospective, case-control study (n = 20) infants with PKU transitioning to a second-stage PS, were recruited at weaning (4&ndash, 6 months) for a comparison of feeding practices and development with non-PKU infants. Subjects were monitored monthly to 12 months and at age 15 months, 18 months and 24 months for: feeding progression, food textures, motor skill development and self-feeding, feeding environment, gastrointestinal symptoms, and negative feeding behaviours. Children with PKU had comparable weaning progression to non-PKU infants including texture acceptance, infant formula volume and self-feeding skills. However, children with PKU had more prolonged Phe-free infant formula bottle-feeding and parental spoon feeding than controls, fewer meals/snacks per day, and experienced more flatulence (p = 0.0005), burping (p = 0.001), retching (p = 0.03), and less regurgitation (p = 0.003). Negative behaviours associated with PS at age 10&ndash, 18 months, coincided with the age of teething. Use of semi-solid PS in PKU supports normal weaning development/progression but parents require support to manage the complexity of feeding and to normalise the social inclusivity of their child&rsquo, s family food environment. Further study regarding parental anxiety associated with mealtimes is required.

In phenylketonuria (PKU), variable dietary advice provided by health professionals and social media leads to uncertainty for patients/caregivers reliant on accurate, evidence based dietary information. Over four years, 112 consensus statements concerning the allocation of foods in a low phenylalanine diet for PKU were developed by the British Inherited Metabolic Disease Dietitians Group (BIMDG-DG) from 34 PKU treatment centres, utilising 10 rounds of Delphi consultation to gain a majority (&ge, 75%) decision. A mean of 29 UK dietitians (range: 18&ndash, 40) and 18 treatment centres (range: 13&ndash, 23) contributed in each round. Statements encompassed all foods/food groups divided into four categories based on defined protein/phenylalanine content: (1) foods high in protein/phenylalanine (best avoided), (2) foods allowed without restriction including fruit/vegetables containing phenylalanine &le, 75 mg/100 g and most foods containing protein &le, 0.5 g/100 g, (3) foods that should be calculated/weighed as an exchange food if they contain protein exchange ingredients (categorized into foods with a protein content of: &gt, 0.1 g/100 g (milk/plant milks only), &gt, 0.5 g/100 g (bread/pasta/cereal/flours), &gt, 1 g/100 g (cook-in/table-top sauces/dressings), &gt, 1.5 g/100 g (soya sauces)), and (4) fruit/vegetables containing phenylalanine &gt, 75 mg/100 g allocated as part of the protein/phenylalanine exchange system. These statements have been endorsed and translated into practical dietary management advice by the medical advisory dietitians for the National Society for PKU (NSPKU).

WOS: 000442229500021, PubMed ID: 30101073, Background: In infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe. Methods: We sent a cross sectional, survey monkey (R) questionnaire to European health professionals working in IMD. It contained 31 open and multiple-choice questions. The results were analysed according to different geographical regions. Results: Ninety-five centres from 21 countries responded. Over 60% of centres commenced diet in infants by age 10 days, with 58% of centres implementing newborn screening by day 3 post birth. At diagnosis, infant hospital admission occurred in 61% of metabolic centres, mainly in Eastern, Western and Southern Europe. Breastfeeding fell sharply following diagnosis with only 30% of women still breast feeding at 6 months. 53% of centres gave pre-measured Phe-free infant formula before each breast feed and 23% alternated breast feeds with Phe-free infant formula. With standard infant formula feeds, measured amounts were followed by Phe-free infant formula to satiety in 37% of centres (n = 35/95), whereas 44% (n = 42/95) advised mixing both formulas together. Weaning commenced between 17 and 26 weeks in 85% centres, >= 26 weeks in 12% and < 17 weeks in 3%. Discussion: This is the largest European survey completed on PKU infant feeding practices. It is evident that practices varied widely across Europe, and the practicalities of infant feeding in PKU received little focus in the PKU European Guidelines (2017). There are few reports comparing different feeding techniques with blood Phe control, Phe fluctuations and growth. Controlled prospective studies are necessary to assess how different infant feeding practices may influence longer term feeding development., Vitaflo, We thank Vitaflo for supporting the publication cost of this paper.

WOS: 000423688500002, PubMed ID: 29370874, Background: In fructose 1,6 bisphosphatase (FBPase) deficiency, management aims to prevent hypoglycaemia and lactic acidosis by avoiding prolonged fasting, particularly during febrile illness. Although the need for an emergency regimen to avoid metabolic decompensation is well established at times of illness, there is uncertainty about the need for other dietary management strategies such as sucrose or fructose restriction. We assessed international differences in the dietary management of FBPase deficiency. Methods: A cross-sectional questionnaire (13 questions) was emailed to all members of the Society for the Study of Inborn Errors of Metabolism (SSIEM) and a wide database of inherited metabolic disorder dietitians. Results: Thirty-six centres reported the dietary prescriptions of 126 patients with FBPase deficiency. Patients' age at questionnaire completion was: 1-10y, 46% (n = 58), 11-16y, 21% (n = 27), and >16y, 33% (n = 41). Diagnostic age was: 16y, 2% (n = 2). Seventy-five per cent of centres advocated dietary restrictions. This included restriction of: high sucrose foods only (n = 7 centres, 19%); fruit and sugary foods (n = 4, 11%); fruit, vegetables and sugary foods (n = 13, 36%). Twenty-five per cent of centres (n = 9), advised no dietary restrictions when patients were well. A higher percentage of patients aged >16y rather than 16y, 85% (n = 35/41). Patients classified as having a normal fasting tolerance increased with age from 30% in 1-10y, to 36% in 11-16y, and 58% in >16y, but it was unclear if fasting tolerance was biochemically proven. Twenty centres (56%) routinely prescribed uncooked cornstarch (UCCS) to limit overnight fasting in 47 patients regardless of their actual fasting tolerance (37%). All centres advocated an emergency regimen mainly based on glucose polymer for illness management. Conclusions: Although all patients were prescribed an emergency regimen for illness, use of sucrose and fructose restricted diets with UCCS supplementation varied widely. Restrictions did not relax with age. International guidelines are necessary to help direct future dietary management of FBPase deficiency., ERDF - Programa Operacional Competitividade e Internacionalizacao - COMPETE [POCI-01-0145-FEDER-007746]; FCT - Fundacao para a Ciencia e a Tecnologia within CINTESIS, RD Unit [UID/IC/4255/2013], This article was supported by ERDF through the operation POCI-01-0145-FEDER-007746 funded by the Programa Operacional Competitividade e Internacionalizacao - COMPETE2020 and by National Funds through FCT - Fundacao para a Ciencia e a Tecnologia within CINTESIS, R&D Unit (reference UID/IC/4255/2013).

Background There is scarce evidence available with respect to an evaluation of the role of low protein staple foods (LPSF) in the management of phenylketonuria (PKU). The present study explored beliefs, acceptability and issues around the use of LPSF by people with PKU or their carers. Methods A semi-anonymous questionnaire was posted to 178 people with PKU in Scotland (104 children, aged 2–17 years, and 74 adults). Questions explored were: the type and amount of LPSF ordered; perceptions on use and usefulness of LPSF; acceptability of the LPSF sensory properties (i.e. taste, smell, texture, appearance); support for the supply and use of LPSF; and comments from primary healthcare professionals regarding dispensing and prescription. Results Eighty-two individuals responded (46% response rate): 97% perceived that LPSF were useful for PKU management; more than 85% reported that LPSF were important for phenylalanine control, satisfying appetite, and diet variety. The most common LPSF ordered were pasta/rice/cous cous, flour, biscuits and bread. Fifty percent of respondents ordered

Genome-wide association studies (GWASs) primarily performed in European-ancestry (EA) populations have identified numerous loci associated with body mass index (BMI). However, it is still unclear whether these GWAS loci can be generalized to other ethnic groups, such as African Americans (AAs). Furthermore, the putative functional variant or variants in these loci mostly remain under investigation. The overall lower linkage disequilibrium in AA compared to EA populations provides the opportunity to narrow in or fine-map these BMI-related loci. Therefore, we used the Metabochip to densely genotype and evaluate 21 BMI GWAS loci identified in EA studies in 29,151 AAs from the Population Architecture using Genomics and Epidemiology (PAGE) study. Eight of the 21 loci (SEC16B, TMEM18, ETV5, GNPDA2, TFAP2B, BDNF, FTO, and MC4R) were found to be associated with BMI in AAs at 5.8 × 10(-5). Within seven out of these eight loci, we found that, on average, a substantially smaller number of variants was correlated (r(2) > 0.5) with the most significant SNP in AA than in EA populations (16 versus 55). Conditional analyses revealed GNPDA2 harboring a potential additional independent signal. Moreover, Metabochip-wide discovery analyses revealed two BMI-related loci, BRE (rs116612809, p = 3.6 × 10(-8)) and DHX34 (rs4802349, p = 1.2 × 10(-7)), which were significant when adjustment was made for the total number of SNPs tested across the chip. These results demonstrate that fine mapping in AAs is a powerful approach for both narrowing in on the underlying causal variants in known loci and discovering BMI-related loci.

Social workers are key professionals in assessing social care need and providing support.However, needs in the palliative care stage of chronic illness are not always wellmanaged. In this study, we set out to examine the social worker’s role in the deliveryof palliative care to clients with Parkinson’s disease (PD). Two focus groups and one individualinterview were undertaken with thirteen social workers with experience ofworking with clients with PD in community and hospice settings. Differing perceptionsof palliative care emerged: from a holistic approach to specialist care near the end oflife. Negative associations of palliative care were identified. Other barriers also exist,such as lack of knowledge and experience of PD and resources that hinder the deliveryof palliative care to clients with chronic conditions. Different interpretations of palliativecare affect its delivery to clients with long-term chronic conditions. Very fewclients with PD are referred to specialist palliative care specifically for management oftheir symptoms, which must prevent holistic care. Social workers have an important,yet underdeveloped, role in identifying and addressing palliative care needs. Policiesand procedures should be clarified regarding prioritisation and access for clients withchronic long-term conditions to appropriate palliative care.

How to cite this article MacDonald A., Evans S., Cochrane B. & Wildgoose J. (2012) Weaning infants with phenylketonuria: a review. J Hum Nutr Diet.25, 103–110 Abstract Careful weaning is particularly important in phenylketonuria (PKU). Dietary phenylalanine intake is severely restricted, and the diet is supplemented with phenylalanine-free amino acids and special low protein foods. In PKU, there are no evidence-based weaning guidelines and no studies assessing the introduction of solid foods. We critically review the literature and examine current UK weaning practices. Ideally, weaning in PKU should closely reflect the ‘model’ for healthy infants. However, the requirement for optimal blood phenylalanine control and the demands of diet therapy overshadow the social aspects of weaning. Solid food intake is established with very low protein foods first, and then 50 mg phenylalanine exchanges (equivalent to 1 g of intact protein) gradually replace breast/formula feeds. Introducing solids before the recommended 6 months of age may be advantageous because there is a less persistent neophobic food response, possibly leading to better food acceptance. Infants with PKU also require a special phenylalanine-free protein substitute. Between 6 and 12 months, a second concentrated source of phenylalanine-free protein substitute is required. This is commonly given as an additional liquid, although the prescribed volume may adversely affect appetite. Alternatively, a second-stage protein substitute administered as a paste may better suit feeding development. Further research aiming to examine the weaning process in PKU with a focus on biological, maternal, infant, social and environmental factors is required. This will help provide evidence for the effect of protein substitute on appetite and help in the development of evidence-based guidelines.

A review of the literature highlights the important role informal carers play in the provision of palliative care in the community. In order to explore the caring experience of relatives with Parkinson’s Disease (PD), interviews were conducted with 26 informal family caregivers. Interviews were taped, transcribed and subjected to content analysis. All caregivers were spouses, the majority female ( n = 17) and all were responsible for providing physical, social and emotional care in the home. Although they viewed care giving as their role and duty, the results highlight the widespread burden of providing care on the emotional and physical health of the caregivers. The financial implications for providing care were outlined, with many reporting difficulty in accessing benefits. From the point of diagnosis, which had a huge emotional impact on relatives and carers, carers did not feel health professionals integrated them within the caring journey. Since diagnosis, carers commented on the lack of continued and coordinated care plans for relatives, resulting in symptoms being mismanaged and care opportunities for relatives and carers missed. Stereotypes of the meaning and timing of palliative care were common with many viewing it as being synonymous with cancer and not applicable to a person with PD. As the well-being of the informal carer directly influences the care of the person with PD, support interventions are required to relieve their burden, maximize outcomes and ensure targeting of services.

Although most people with Parkinson's disease are cared for in the community, little is known about family members' lived experiences of palliative or end-of-life care. The aim of this study was to explore former carers' lived experiences of palliative and end-of-life care. In total, 15 former family caregivers of patients who had died with Parkinson's disease were interviewed using a semi-structured topic list. Findings indicated that some palliative and end-of-life care needs had not been fully addressed. Lack of communication, knowledge and coordination of services resulted in many people caring for someone with Parkinson's disease not accessing specialist palliative care services. Participants also reflected upon the physical and psychological impact of caring in the advanced stage of Parkinson's. A multi-disciplinary team-based approach was advocated by participants. These findings provide important insights into the experience of caregiving to patients with Parkinson's disease in the home at the end-of-life stage. According to palliative care standards, patients and their carers are the unit of care; in reality, however, this standard is not being met. © 2010 The Author(s).

Objectives The Women's Health Initiative (WHI) randomized trial of calcium/vitamin D supplementation found reduced bone loss with active treatment compared to placebo. Now we examine whether the treatment affected self-reported physical functioning and objective measures of physical functioning. Design A randomized, double-blind, placebo-controlled trial of 1,000 mg calcium carbonate plus 400 IU vitamin D 3 per day or matching placebo pills. Subjects/setting The study included 33,067 women (50 to 79 years old) at 40 US study centers. Main outcome measures Physical functioning was assessed by questionnaire at enrollment in WHI, 1 year prior to calcium/vitamin D trial randomization and at study close-out (average follow-up 7.1 years). Objective physical performance and self-reported exercise measures were collected at WHI baseline (1 year prior to calcium/vitamin D enrollment) and 2 years and 4 years after calcium/vitamin D trial enrollment in a subsample (n=3,137). Statistical analyses performed Calcium/vitamin D effects were tested in unadjusted and interaction linear models for each of the physical function measures. Covariates were baseline total calcium intake, fracture risk score, treatment arm in the hormone therapy and dietary modification trials (ie, active drug or placebo, low-fat diet intervention or usual diet, respectively) and age. Results Neither intention to treat nor high adherence analyses produced substantial effects of calcium/vitamin D compared to placebo on physical functioning or performance. The interaction analyses also did not result in differences because of calcium/vitamin D. Conclusions As the first long-term randomized trial to examine the effectiveness of calcium and vitamin D in protecting against decline of physical functioning in older women, the results did not support benefit.

Palliative care encompasses spiritual as well as physical, social and psychological aspects. Spiritual care has been identified as a key concern of dying patients. During an audit of the Northern Ireland Hospice chaplaincy service against the national Standards for Hospice and Palliative Care Chaplaincy (2003), 62 patients' spiritual needs along with their interactions with the hospice chaplains were assessed by using a questionnaire survey and reviewing data recorded on their pastoral care notes. Findings suggest that the Standards were useful for assessing and addressing spiritual needs. Access to the chaplaincy service (Standard 1) was partially met and Standard 2's spiritual criteria were fully met. The participants, of whom 92% had a faith in God or a Higher Being, highlighted their top six spiritual needs as: to have the time to think; to have hope; to deal with unresolved issues; to prepare for death; to express true feelings without being judged; to speak of important relationships. The majority of the participants (82%) felt their spiritual needs had been addressed and viewed their interaction with the chaplaincy service positively. Recommendations were made relating to improve communication of chaplaincy services. Palliative Medicine 2007; 21: 519—525

Background: There appears little consensus concerning protein requirements in phenylketonuria (PKU). Methods: A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. Results: The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n = 24 centres) (infants 2-3 g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n = 10 centres) (infants 2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n = 25 centres) giving the least (infants 2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day). Conclusions: The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population. (C) 2015 Elsevier Inc. All rights reserved.

Objective This exploratory study investigated the influence of adding a patented, specific mixture of prebiotic oligosaccharides (scGOS/lcFOS [9:1 ratio], Danone Research) to a protein substitute suitable for infants with Phenylketonuria (PKU); PKU Anamix Infant (Nutricia). Design This was an 8-week open-label, single-arm, pilot intervention study in 9 infants (8-week median age) diagnosed with PKU. On study entry, infants were prescribed PKU Anamix Infant to replace an infant phenylalanine-free protein substitute without prebiotics (IPS). Blood phenylalanine concentrations were monitored and stool samples analyzed for pH/bacterial groups. Results PKU Anamix infant was well tolerated and accepted with no adverse events reported. Overall, plasma phenylalanine and tyrosine concentrations were maintained within target ranges throughout the study (120–360 μmol/l phenylalanine, 30–100 μmol/l tyrosine). All infants exhibited microbiota dominated by bifidobacteria (median 58.97% at Week 8), although no statistically significant change from baseline was observed at study endpoint. No infants showed abnormally high levels of Clostridium histolyticum/lituseburense or potentially pathogenic enterobacteriaceae at any point during the study. A significant reduction in median stool pH versus baseline was observed at Week 4 (pH reduced from 6.79 to 5.83), but this significance was not present at Week 8 (pH = 6.61). Conclusions PKU Anamix Infant maintains phenylalanine control in line with established IPS without prebiotics and maintains levels of bifidobacteria and lowers stool pH. In exclusively breast-fed infants the latter two factors have been associated with a reduced risk of infection and may be of particular importance in infants with PKU.

Aim: This study explored the experiences of palliative care that bereaved carers had while providing care to a dying loved one with chronic obstructive pulmonary disease (COPD). Method: Semi-structured interviews were undertaken with nine carers who had lost a loved one in the preceding 6 to 24 months. These interviews explored levels of satisfaction with disease management, symptom management, and end-of-life care. With permission, interviews were tape recorded, transcribed, and subjected to content analysis. Findings: Three themes emerged from the data: the impact of the caring experience, the lack of support services, and end-of-life and bereavement support. Carers experienced carer burden, lack of access to support services, a need for palliative care, and bereavement support. Conclusion: The findings provide a first insight into the experiences of carers of patients with advanced COPD. Bereaved carers of patients who had suffered advanced COPD reported that they had received inadequate support and had a range of unmet palliative care needs. Special attention should be paid to educating and supporting carers during their caring and bereavement periods to ensure that their quality of life is maintained or enhanced.

This article describes health and social care professionals' perceptions of palliative care and facilitators and barriers to the delivery of such care for patients with advanced chronic obstructive pulmonary disease. Health professionals participated in semi structured interviews and focus groups which were analysed using content analysis. According to participants, care of patients with chronic obstructive pulmonary disease is focused upon the management of symptoms, with emphasis focused predominately on an acute model of care. Key barriers towards the delivery of palliative care included the reluctance to negotiatie end-of-life decisions and a perceived lack of understanding among patients and carers regarding the illness trajectory. Consequently the delivery of palliative care was viewed as a specialist role rather than an integral component of care. There is a need for education and training for health and social care professions to plan and provide high quality end-of-life care. © 2009 SAGE Publications.

Background: The aim of this study was to determine the magnitude and significance of the associations between coronary artery calcium (CAC) and different measures of blood pressure among postmenopausal women. Methods: Subjects were a subset of women aged 50 to 59 at baseline in the Women’s Health Initiative (WHI) clinical trial of conjugated equine estrogen (CEE) who underwent a one-time computed tomography (CT) scan of the chest following the end of the trial to determine CAC. At the baseline WHI clinic visit, CEE trial participants provided data on a wide range of factors to include blood pressure measurements which were measured twice with the participant in the seated position using a conventional mercury sphygmomanometer and appropriately sized cuffs. Results: The sample consisted of 1,064 women with a mean age of 55.1 (2.8) years at WHI randomization and 64.8 (2.9) years at CAC ascertainment. The prevalence of a CAC score > 0 was 47%, while the prevalence of a CAC score >= 10 and > 100 was 39 and 19%, respectively. There was a linear association between the log-odds of any CAC and SBP while there was a curvilinear and inverse association with DBP that was independent of CVD risk factors and blood pressure medication status. For any value of diastolic blood pressure, the probability of CAC increased with higher levels of SBP. Conversely, for any given value of SBP, the probability of any CAC decreased with higher levels of DBP. Those with a PP >= 55 mmHg had nearly a two-fold higher odds for having any CAC (Odds Ratio: 1.95, 95% CI: 1.24 – 3.06) while individuals with isolated systolic hypertension (SBP >= 140 and DBP < 90) had a 73% higher odds for CAC (95% CI: 1.03 – 2.90, p = 0.04) independent of other risk factors and blood pressure medication status. There was no significant associations between mean arterial pressure and CAC. Conclusions: In postmenopausal women over the age of 50 years, both SBP and DBP are relevant for determining the risk for coronary artery calcium. Notably, higher levels of pulse pressure and systolic blood pressure were strong determinants of CAC, while diastolic blood pressure was inversely related. Consequently, isolated systolic hypertension may be relevant in women. These results may be of clinical relevance for the prevention of coronary artery disease. This research has received full or partial funding support from the American Heart Association, AHA National Center.

Context: Women discharged with a diagnosis of nonspecific chest pain (NSCP) may have an increased risk of subsequent coronary heart disease (CHD) events. The influence of hormone therapy on NSCP is unknown. Methods: The WHI enrolled postmenopausal women aged 50 –79 years. After excluding women with prior cardiovascular disease, 9427 women in the estrogen alone (E-Alone) trial and 15,105 women in the estrogen plus progestin (E + P) trial were included in this analysis. NSCP, defined as having a primary hospital discharge diagnosis of NSCP by ICD-9 code, was reported in 322 E-Alone and 249 E + P women. Risks of subsequent CHD events were estimated using intent-to-treat Cox proportional hazard ratio (HR) models stratified by clinic and adjusted for age and other risk factors. Results: In the fully adjusted models of the combined trials, women with NSCP had a 2-fold greater risk of subsequent nonfatal CHD events including nonfatal myocardial infarction [2.3% vs 1.7%, HR 2.10 (1.11–3.98)], revascularization [3.5% vs 2.6%, HR 1.99 (1.20 –3.30)], and hospitalized angina [3.7% vs 2.3%, HR 2.39 (1.46 –3.92)]. Hormone therapy did not have a significant effect on either the incidence of NSCP hospitalizations [HRs (95% CIs): E-Alone 1.04 (0.81–1.32); E + P 0.78 (0.59 –1.02)] or on the risk of a subsequent CHD event over 7.1 or 5.6 years of follow-up, respectively. Conclusion: A NSCP hospitalization doubles the risk of a subsequent CHD event in postmenopausal women over the next 5 to 7 years. Further research is needed to identify appropriate diagnosis and treatment strategies for women with NSCP.

Day hospice allows patients to remain living at home while attending a specialist palliative care center. This paper describes patient feedback (n=26) on medical, social and therapeutic out-patient palliative care services provided at one such facility in Northern Ireland. Medical records of all attending patients (n=50) were reviewed, and patients completed a semi-structured questionnaire. Patients' comments on day hospice were largely positive. A number of suggestions for new service activities were made, and the need for education of multi-professional team members was recognized. Respondents recognized that day hospice allowed respite care for the family. Patients were very appreciative of the care that was offered--their concern for its continuance perhaps restraining any criticism they had. Such findings provide the opportunity for health professionals to gain further insights into day care to better meet patients' needs.

Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low-volume (amino acids 72 g/100 g), low-carbohydrate, phenylalanine-free protein substitute with added vitamins and minerals designed for people with PKU over 8 years of age. In an open intervention study, the aim was to investigate its acceptability and effectiveness in a group of teenagers and adults with PKU. Twenty-three subjects (15 female; 8 male) with PKU, who had a median age of 17 years (range 8-37 years) took the substitute for 8 weeks. A 3-day prospective diet diary, height, weight, plasma amino acids, biochemical and haematological nutritional analytes were measured at weeks 0 and 8. Skin-puncture bloods for plasma phenylalanine were collected every 2 weeks. The median weight of protein substitute (with or without vitamin and mineral supplements) consumed decreased by 33% from 150 g (range 140-180) daily to 100 g (range 100-125) daily ( p

Dietary therapy remains the first line of treatment for patients with high blood cholesterol levels. Among free-living persons, compliance with National Cholesterol Education Program (NCEP) dietary recommendations is uncertain.We performed a cross-sectional, baseline analysis of 91,627 postmenopausal women enrolled in the Women's Health Initiative Observational Study. Among women with self-reported hypercholesterolemia, we ascertained factors associated with compliance with National Cholesterol Education Program dietary recommendations, defined for the Step II diet asor=30% of total calories from fat,7% of calories from saturated fat, and daily dietary cholesterol200 mg.Of the 13,777 participants who reported having high cholesterol levels requiring drug therapy, only 20% reported total fat, saturated fat, and dietary cholesterol consumption consistent with Step II dietary goals. Factors associated with Step II dietary compliance included having a college degree (odds ratio [OR] = 1.26; 95% confidence interval [CI]: 1.14 to 1.40), a prior cardiovascular event (OR = 1.48; 95% CI: 1.28 to 1.70), and consumption of five or more daily servings of fruits or vegetables (OR = 3.0; 95% CI: 2.7 to 3.3). Being married, smoking, a sedentary lifestyle, and a higher body mass index were all associated with reduced compliance (all P0.0001). In the subsample in which plasma lipid levels were measured, dietary compliance was associated with higher levels of low-density lipoprotein cholesterol (P = 0.02).Since the inception of the NCEP in 1985, health care providers, public health programs, and patients have not successfully implemented the dietary recommendations.

A prospective study of injuries affecting 50 highly com petitive young female gymnasts was conducted over a period of 1 year. Many of the findings of this investi gation were consistent with previous studies and sug gest particular injury trends in women's gymnastics. These results included injury location, injury severity, nature of onset, event, and activity at the time of injury. Some of the descriptive results, however, provided information that was heretofore unreported or incon sistent with previous investigations. These findings in volved injury rate, reinjury rate, time loss, injury type, hours of practice, and incidence of physician-seen in juries. Some of these findings were disturbing and echo concerns registered in the professional literature. In particular, the reinjury rate is alarming and points to the need for complete rehabilitation before return to full participation. The results of the analytic component of the study alluded to the potential role of competitive level and maturation rate in the profile of the injury- prone gymnast. Specifically, rapid periods of growth and advanced levels of training and competition ap peared to be related to injury proneness. Pursuant to the descriptive and analytic results of the investigation, recommendations for injury prevention and continued research are made.

Mary Waldron, W George Kernohan, Felicity Hasson, Susan Foster, Barbara Cochrane, Cathy Payne Aims: Allied health professionals (AHPs) have a key role in the delivery of palliative care through rehabilitation. However, evidence suggests that rehabilitation remains an area of neglect in palliative care. This study explores the views of allied health professionals in delivering rehabilitation in palliative care to people with Parkinson's disease. Methods: Twelve allied health professionals, from both generalist and specialist settings, who had experience of working with people with advanced Parkinson's disease and/or palliative care took part in interviews and/or focus groups. Findings: Participants viewed palliative care as holistic care, however, the rehabilitation care they provided was impeded by a number of personal and organisational barriers, and negative perceptions. Misconceptions as to the value of rehabilitation in the palliative stage and a lack of training resulted in uncertainty as to when and how it could be introduced. While the importance of early referral to AHP rehabilitation services was highlighted, organisational and administrative barriers prevented many people from accessing such care. Conclusions: Many health professionals have a role in palliative rehabilitation, but barriers prevent early referral and ongoing access to such services. There is a need to educate professionals in the principles of palliative rehabilitation, combined with providing targeted resources to promote shared care and responsibility.

It has long been recognised that the majority of care provided in chronic illness comes not from health and social care professionals, but from family and friends. One such illness is chronic obstructive pulmonary disease (COPD), a leading cause of morbidity and mortality in the developed world. To explore the specific care needs of informal caregivers of patients with advanced COPD, interviews were conducted with seven active family caregivers. Interviews were taped, transcribed and content analysed to obtain the caregivers’ needs. Results confirm that family caregivers provide direct care with little support and assistance. Participants reported restricted activities of daily living and some emotional distress. There were knowledge deficiencies among caregivers relating to the COPD illness trajectory and little awareness of the potential of palliative care. Family caregivers need social and professional support while caring for a patient at home. This would help to ensure that their physical and emotional health does not suffer. There is a need to devise interventions to ensure family caregivers are supported.

This study aims to explore the potential for palliative care among people living with advanced chronic obstructive pulmonary disease (COPD). Individual semi-structured interviews (n=13) were conducted with people who had a diagnosis of advanced COPD and were on optimal tolerated drug therapy, with their breathing volume (forced expiratory volume at less than 30%) or were on long-term oxygen therapy or non-invasion ventilation. Participants raised concerns about the uncertain trajectory of the illness and reported unmet palliative care needs with poor access to palliative care services. For most people, palliative care was associated with end of life; therefore, they were unwilling to discuss the issue. There was a wide acceptance that, medically, nothing more could be done. Findings also suggest that patients had unmet palliative care needs, requiring information and support. The research suggests the need for palliative care to be extended to all (regardless of diagnosis), with packages of care developed to target specific needs.