Standardised practices in the networked management of congenital hyperinsulinism: a UK national collaborative consensus.

Congenital hyperinsulinism (CHI) is a condition characterised by severe and recurrent hypoglycaemia in infants and young children caused by inappropriate insulin over-secretion. CHI is of heterogeneous aetiology with a significant genetic component and is often unresponsive to standard medical therapy options. The treatment of CHI can be multifaceted and complex, requiring multidisciplinary input. It is important to manage hypoglycaemia in CHI promptly as the risk of long-term neurodisability arising from neuroglycopaenia is high. The UK CHI consensus on the practice and management of CHI was developed to optimise and harmonise clinical management of patients in centres specialising in CHI as well as in non-specialist centres engaged in collaborative, networked models of care. Using current best practice and a consensus approach, it provides guidance and practical advice in the domains of diagnosis, clinical assessment and treatment to mitigate hypoglycaemia risk and improve long term outcomes for health and well-being.

Self-efficacy in nurse entrepreneurs: A concept analysis.

BACKGROUND: Nursing entrepreneurship represents an important opportunity for the nursing profession to address population health challenges and improve health care access. Although nurse entrepreneurs can be agents of change, the role of self-efficacy, as a determinant of nurses' success in business, is under-studied conceptually and in research. PURPOSE: This paper presents an in-depth concept analysis and model of self-efficacy within the context of the nurse entrepreneur role. METHODS: We used Walker and Avant's concept analysis methodology. DISCUSSION: A systematic understanding of self-efficacy in nurse entrepreneurs provides insights into how it may influence their judgments and actions. Our analysis sets the stage for research on how self-efficacy in nurse entrepreneurs impacts success in the nursing business. CONCLUSION: A conceptual model of self-efficacy in nurse entrepreneurs can guide their development through education and networking activities that incorporate experiential components to improve leadership and managerial skills for success in the health care business.

Risk factors and early prevention of skin cancer in rural older outdoor workers: A scoping review.

Skin cancer risk increases with age and prolonged exposure to ultraviolet radiation (UVR), particularly in rural geographical locations and for individuals with light skin complexions. However, the impact of UVR-induced skin cancer risk and sun-protective behaviors in rural older populations working outdoors has yet to be explored. A scoping review was conducted to fill this gap, with 12 articles meeting the inclusion criteria of aged 50 years and older among rural outdoor workers. Skin cancer risk factors, prevention strategies, and barriers to sun-protective behaviors were summarized for each study. The scoping review addressed some key differences in age-related effects of UVR among rural older outdoor workers compared to studies among adults in general. Findings have policy and research implications that highlight the need to design feasible preventive strategies to reduce rural disparities in cancer care and enhance access to preventive services for this high-risk population.

High satisfaction among patients at HIV clinics in Harare, Zimbabwe: a time and motion evaluation and patient satisfaction study.

Patient satisfaction is an important component of health and healthcare utilization, which measures the perceived needs and expectations for healthcare services. Patient satisfaction surveys are effective in improving health outcomes by informing health facilities on service and provider gaps and guiding the development of effective action plans and policies for quality improvement within a healthcare organization. Although patient satisfaction and patient flow analyses have been conducted in Zimbabwe, the combination of these two quality improvement measures in the context of Human Immunodeficiency Virus (HIV) clinics has not previously been evaluated. This study assessed and evaluated patient flow and patient satisfaction to enhance care quality and improve HIV service delivery to optimize patient health. We collected time and motion data from HIV patients who attended three purposively selected City of Harare Polyclinics in Harare, Zimbabwe. All patients who sought care at the clinic were given time and motion forms to track their movement and the time spent at each service area. After services were completed, patients were invited to participate in a satisfaction survey regarding their services and care. The average waiting time from clinic arrival to see the provider was 2 hr and 14 min. The areas with the longest waiting time and bottlenecks occurred at registration (49 min) and the HIV clinic waiting area (44 min). Despite these extended times, the overall patient satisfaction for HIV services was high at 72%, with over half (59%) reporting that there was nothing they did not like about their services. Patients were most satisfied with services provided (34%), timely service (27%), and antiretroviral medications (19%). The areas of least satisfaction were related to time delays (24%) and cashier delays (6%). Despite prolonged waiting times, patients' overall satisfaction with their clinic experience remains high. Perceptions of satisfaction are influenced by experience, culture, and context. However, there are still several areas of recommendations to improve service, care, and quality. Specifically, reducing or eliminating service fees, increasing clinic hour times, and having available medication were most cited. Support from the Zimbabwe Ministry of Health and Child Care, City of Harare, and other decision-makers is needed to improve patient satisfaction and address patients' recommendations within the City of Harare Polyclinic organization in accordance with the 2016-20 National Health Strategies for Zimbabwe.

Amino Acid Analyses of Plant Foods Used in the Dietary Management of Inherited Amino Acid Disorders.

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.

Phenylalanine free infant formula in the dietary management of phenylketonuria.

BACKGROUND: Phenylalanine-free infant formula is an essential source of safe protein in a phenylalanine restricted diet, but its efficacy is rarely studied. We report a multicentre, open, longitudinal, prospective intervention study on a phenylalanine-free infant formula (PKU Start: Vitaflo International Ltd.). RESULTS: This was a 2-part study: part I (28 days short term evaluation) and part II (12 months extension). Data was collected on infant blood phenylalanine concentrations, dietary intake, growth, and gastrointestinal tolerance. Ten infants (n = 8 males, 80%), with a median age of 14 weeks (range 4-36 weeks) were recruited from 3 treatment centres in the UK. Nine of ten infants completed the 28-day follow-up (one caregiver preferred the usual phenylalanine-free formula and discontinued the study formula after day 14) and 7/9 participated in study part II. The phenylalanine-free infant formula contributed a median of 57% (IQR 50-62%) energy and 53% (IQR 33-66%) of total protein intake from baseline to the end of the part II extension study. During the 12-month follow-up, infants maintained normal growth and satisfactory blood phenylalanine control. Any early gastrointestinal symptoms (constipation, colic, vomiting and poor feeding) improved with time. CONCLUSION: The study formula was well tolerated, helped maintain good metabolic control, and normal growth in infants with PKU. The long-term efficacy of phenylalanine-free infant formula should continue to be observed and monitored.

Suitability and Allocation of Protein-Containing Foods According to Protein Tolerance in PKU: A 2022 UK National Consensus.

Introduction: There is little practical guidance about suitable food choices for higher natural protein tolerances in patients with phenylketonuria (PKU). This is particularly important to consider with the introduction of adjunct pharmaceutical treatments that may improve protein tolerance. Aim: To develop a set of guidelines for the introduction of higher protein foods into the diets of patients with PKU who tolerate >10 g/day of protein. Methods: In January 2022, a 26-item food group questionnaire, listing a range of foods containing protein from 5 to >20 g/100 g, was sent to all British Inherited Metabolic Disease Group (BIMDG) dietitians (n = 80; 26 Inherited Metabolic Disease [IMD] centres). They were asked to consider within their IMD dietetic team when they would recommend introducing each of the 26 protein-containing food groups into a patient's diet who tolerated >10 g to 60 g/day of protein. The patient protein tolerance for each food group that received the majority vote from IMD dietetic teams was chosen as its tolerance threshold for introduction. A virtual meeting was held using Delphi methodology in March 2022 to discuss and agree final consensus. Results: Responses were received from dietitians from 22/26 IMD centres (85%) (11 paediatric, 11 adult). For patients tolerating protein ≥15 g/day, the following foods were agreed for inclusion: gluten-free pastas, gluten-free flours, regular bread, cheese spreads, soft cheese, and lentils in brine; for protein tolerance ≥20 g/day: nuts, hard cheeses, regular flours, meat/fish, and plant-based alternative products (containing 5−10 g/100 g protein), regular pasta, seeds, eggs, dried legumes, and yeast extract spreads were added; for protein tolerance ≥30 g/day: meat/fish and plant-based alternative products (containing >10−20 g/100 g protein) were added; and for protein tolerance ≥40 g/day: meat/fish and plant-based alternatives (containing >20 g/100 g protein) were added. Conclusion: This UK consensus by IMD dietitians from 22 UK centres describes for the first time the suitability and allocation of higher protein foods according to individual patient protein tolerance. It provides valuable guidance for health professionals to enable them to standardize practice and give rational advice to patients.

The relationship between depressive symptoms and subtypes of mild cognitive impairment in post-menopausal women: Results from the Women's Health Initiative Memory Study.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Mediterranean Diet and Fatigue among Community-Dwelling Postmenopausal Women.

We investigated cross-sectional relationships between the Mediterranean diet and overall fatigue, energy, and weariness scores among 4,563 women aged 65+ from the Women's Health Initiative study. We also used the Isocaloric Substitution approach to explore whether the substitution of fish for red and processed meat, whole for non-whole grains, and whole fruit for fruit juice relate to RAND-36 measured overall fatigue and its subdomains. The alternate Mediterranean Diet (aMED) Index quintiles (Q1-Q5) and selected Mediterranean foods available on a Food Frequency Questionnaire were exposure measures. Results showed aMED Q5 was associated with 2.99 (95% CI: 0.88, 5.11), 4.01 (95% CI: 1.51, 6.53), and 2.47 (95% CI: 0.24, 4.70) point improvements in fatigue, energy, and weariness scores, respectively, compared with aMED Q1. Substituting fish for red and processed meat and whole for non-whole grains was associated with more favorable fatigue scores, whereas substituting whole fruit for juice was not.

Fatigue in community-dwelling older adults: A review of definitions, measures, and related factors.

Fatigue is a common age-related symptom among community-dwelling adults aged 65 years and older. Yet, a systematic approach has rarely been applied to review definitions, measures, related factors, and consequences of fatigue in this population. A scoping review was conducted in December 2020 to fill the gap, and 36 articles met the inclusion criteria. Definitions, albeit diverse, included at least one of the following attributes: an early indicator of disablement, subjective, a lack of energy, multidimensional, impaired daily activities, and temporal. A summary of fatigue measures used in this population was provided, including a brief overview, number of items, reliability, and validity. In general, different measures were used with considerable variability in the content. Additionally, most measures had limited information on test-retest reliability and validity. Fatigue-related factors mapped into biological, psychological, social, and behavioral factors. Fatigue consequences were primarily declines in physical and cognitive functions. (100-150 words).

Mechanisms of obesity in children and adults with phenylketonuria on contemporary treatment.

BACKGROUND & AIMS: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

A Feasibility Study of Primary Care Liaisons: Linking Older Adults to Community Resources.

INTRODUCTION: Programs and services available through the aging services network can help community-dwelling older adults to age in place but are often not discussed in routine primary care. The primary care liaison was developed as a novel integration intervention to address this disconnect. METHODS: Employed by an Area Agency on Aging, primary care liaisons performed outreach to primary care with the goal of raising awareness of community-based programs, resources, and services available to older adults and their caregivers and facilitating referrals. The evaluation of the primary care liaison model, conducted from December 2015 to February 2019, used the Reach, Effectiveness, Adoption, Implementation, Maintenance framework and assessed reach (number of clinics contacted), adoption (number of referrals to the Area Agency on Aging), implementation (number of follow-up contacts with a practice), and effectiveness (proportion of referrals reached and provided relevant resources). RESULTS: The primary care liaisons contacted a median of 18.5 clinics per month (IQR=15-31). Primary care referrals averaged >100 per month, and referrals increased over time. Successful follow-up outreach visits had a median of 3 (IQR=2-10), and follow-up contacts had a median of 3 (IQR=1-7) per practice. Three quarters of caregivers for people with dementia reached by Area Agency on Aging staff were provided with information about relevant resources. CONCLUSIONS: The primary care liaison model is feasible, fosters ongoing interactions between primary care and Area Agencies on Aging, and connects older adults and their caregivers to relevant programs and services. Adoption of the primary care liaison model by other Area Agencies on Aging across the U.S. may help further the vision of optimized health and well-being of older adults.

Uniformity of Food Protein Interpretation Amongst Dietitians for Patients with Phenylketonuria (PKU): 2020 UK National Consensus Statements.

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 (≥75%) decision. A mean of 29 UK dietitians (range: 18-40) and 18 treatment centres (range: 13-23) 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 ≤75 mg/100 g and most foods containing protein ≤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: >0.1 g/100 g (milk/plant milks only), >0.5 g/100 g (bread/pasta/cereal/flours), >1 g/100 g (cook-in/table-top sauces/dressings), >1.5 g/100 g (soya sauces)); and (4) fruit/vegetables containing phenylalanine >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).

Healthy aging through the lens of community-based practitioners: a focus group study.

BACKGROUND: Nearly one in every seven Americans is 65 years and older, facing day-to-day challenge of aging. Although interest in healthy aging is growing, most of the efforts are directed towards understanding the perceptions of older adults. Little is known about the perspectives of community-based practitioners who work with older adults and deliver programs to promote healthy aging. The purpose of this project was to expand knowledge on healthy aging by exploring the perspectives of community-based practitioners working directly with older adults. METHODS: We purposively sampled community-based practitioners (n = 12, including nurses, physician, social workers, and other community services professionals) working with older adults, who then participated in one of three in-depth focus group discussions conducted between March and June 2016. Each focus group discussion lasted for about 2 h. Verbatim transcript data were analyzed in Atlas.ti 7 using a conventional content analysis with an inductive approach, and consensual validation of coding was achieved. RESULTS: Three core categories of healthy aging were identified: (1) characteristics of healthy aging; (2) healthy aging attainment; and (3) programs and activities for healthy aging. Practitioners identified a number of characteristics of healthy aging under person-specific (physiological, basic, psych-emotional, and cognitive needs), social aspects (creating community and contributing to the community), and spiritual dimensions (cultural views and beliefs) of healthy aging. Healthy aging attainment was represented as facilitators and barriers both with respect to care recipients and care providers, and programs and activities through promoting fitness and wellness. CONCLUSIONS: The rapidly changing demographics and aging population in the United States and their various needs suggest the implications for recognizing opportunities and developing and implementing programs to promote healthy aging. Although practitioners' perspectives had some overlap with traditional research and medical views on healthy aging, the unique and holistic conceptual framework derived in the study might provide a more refined foundation for delivering appropriate health care services to the American aging population.

Mealtime Anxiety and Coping Behaviour in Parents and Children During Weaning in PKU: A Case-Control Study.

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'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 < 0.05) of age. Generally, there was a trend that maternal anxiety regarding child rejection of PS increased with time, peaking between 12-24 m. In PKU, a child age of 12-18 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.

Prediagnostic Leukocyte Telomere Length and Pancreatic Cancer Survival.

BACKGROUND: Leukocyte telomere length has been associated with risk of subsequent pancreatic cancer. Few prospective studies have evaluated the association of prediagnostic leukocyte telomere length with pancreatic cancer survival. METHODS: We prospectively examined the association of prediagnostic leukocyte telomere length with overall survival (OS) time among 423 participants diagnosed with pancreatic adenocarcinoma between 1984 and 2008 within the Health Professionals Follow-up Study, Nurses' Health Study, Physicians' Health Study, and Women's Health Initiative. We measured prediagnostic leukocyte telomere length in banked blood samples using quantitative PCR. Cox proportional hazards models were used to estimate HRs for OS with adjustment for potential confounders. We also evaluated 10 SNPs at the telomerase reverse transcriptase locus. RESULTS: Shorter prediagnostic leukocyte telomere length was associated with reduced OS among patients with pancreatic cancer (P trend = 0.04). The multivariable-adjusted HR for OS comparing the lowest with highest quintiles of leukocyte telomere length was 1.39 (95% confidence interval, 1.01-1.93), corresponding to a 3-month difference in median OS time. In an analysis excluding cases with blood collected within 2 years of cancer diagnosis, the association was moderately stronger (HR, 1.55; 95% confidence interval, 1.09-2.21; comparing the lowest with highest quintiles; P trend = 0.01). No prognostic association or effect modification for the prognostic association of prediagnostic leukocyte telomere length was noted in relation to the studied SNPs. CONCLUSIONS: Prediagnostic leukocyte telomere length was associated with pancreatic cancer survival. IMPACT: Prediagnostic leukocyte telomere length can be a prognostic biomarker in pancreatic cancer.

How Does Feeding Development and Progression onto Solid Foods in PKU Compare with Non-PKU Children During Weaning?

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 (4⁻6 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 10⁻18 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's family food environment. Further study regarding parental anxiety associated with mealtimes is required.

Growth, Protein and Energy Intake in Children with PKU Taking a Weaning Protein Substitute in the First Two Years of Life: A Case-Control Study.

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 (4⁻6 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 < -2 for any growth parameter at age 2 years. Total protein and energy intake in both groups were similar at all ages; however, from 12⁻24 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.

Development of national consensus statements on food labelling interpretation and protein allocation in a low phenylalanine diet for PKU.

BACKGROUND: In the treatment of phenylketonuria (PKU), there was disparity between UK dietitians regarding interpretation of how different foods should be allocated in a low phenylalanine diet (allowed without measurement, not allowed, or allowed as part of phenylalanine exchanges). This led to variable advice being given to patients. METHODOLOGY: In 2015, British Inherited Metabolic Disease Group (BIMDG) dietitians (n = 70) were sent a multiple-choice questionnaire on the interpretation of protein from food-labels and the allocation of different foods. Based on majority responses, 16 statements were developed. Over 18-months, using Delphi methodology, these statements were systematically reviewed and refined with a facilitator recording discussion until a clear majority was attained for each statement. In Phase 2 and 3 a further 7 statements were added. RESULTS: The statements incorporated controversial dietary topics including: a practical 'scale' for guiding calculation of protein from food-labels; a general definition for exchange-free foods; and guidance for specific foods. Responses were divided into paediatric and adult groups. Initially, there was majority consensus (≥86%) by paediatric dietitians (n = 29) for 14 of 16 statements; a further 2 structured discussions were required for 2 statements, with a final majority consensus of 72% (n = 26/36) and 64% (n = 16/25). In adult practice, 75% of dietitians agreed with all initial statements for adult patients and 40% advocated separate maternal-PKU guidelines. In Phase 2, 5 of 6 statements were agreed by ≥76% of respondents with one statement requiring a further round of discussion resulting in 2 agreed statements with a consensus of ≥71% by dietitians in both paediatric and adult practice. In Phase 3 one statement was added to elaborate further on an initial statement, and this received 94% acceptance by respondents. Statements were endorsed by the UK National Society for PKU. CONCLUSIONS: The BIMDG dietitians group have developed consensus dietetic statements that aim to harmonise dietary advice given to patients with PKU across the UK, but monitoring of statement adherence by health professionals and patients is required.