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Abstract 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.

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.

Abstract 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.

Abstract 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.

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. [ABSTRACT FROM AUTHOR]