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Background: Special low-protein foods are essential in the dietary treatment of phenylketonuria (PKU). In the UK, these are available on prescription through the General Practitioners (GPs) and distributed via nutritional home delivery companies or pharmacies. Methods: A 58-item online non-validated semi-structured questionnaire was emailed to British Inherited Metabolic Disease Group (BIMDG) dietitians and dietetic support workers (DSW)/administrators working in PKU to ascertain the main system issues and errors with the supply of low-protein prescription foods (LPPF). Results: 73% (n = 53/73) of dietitians and 72% (n = 18/25) of DSW/administrators responded. A total of 80 questionnaires (representing 44 paediatric and 36 adult PKU centres) were completed. A total of 50% (n = 40/80) of respondents reported patient/caregiver problems accessing LPPF at least weekly. The most common problems were unavailable products (82%), missing LPPF in deliveries (79%), and delayed deliveries (66%). For 64% of respondents, >25% of their patients had recurring problems accessing LPPF, and 69% of respondents spent ≥1 h/week and 11% >5 h/week correcting LPPF patient supply issues. The most common foods patients experienced supply issues with were bread (96%), pasta/rice (41%) and milk replacements (35%). This was associated with GP prescription errors (65%), LPPF prescriptions sent to incorrect dispensers/suppliers (60%), and manufacturer supply issues (54%). Problems with patients/caregivers included not ordering LPPF in a timely way (81%), not responding to messages from home delivery companies (73%) and poor understanding of the ordering process (70%). The majority (93%) of respondents reported that prescription issues impacted their patients’ blood Phe control. Suggestions for improving access to LPPF included centralisation of the system to one supplier (76%) and apps for ordering LPPF (69%). Conclusions: The supply of LPPF for PKU in the UK is problematic; it may adversely affect the ability of patients to adhere to dietary management, and a review investigating patient access to LPPF is urgently required.

Patients with phenylketonuria (PKU) are reliant on special low protein foods (SLPFs) as part of their dietary treatment. In England, several issues regarding the accessibility of SLPFs through the national prescribing system have been highlighted. Therefore, prescribing patterns and expenditure on all SLPFs available on prescription in England (n = 142) were examined. Their costs in comparison to regular protein-containing (n = 182) and ‘free-from’ products (n = 135) were also analysed. Similar foods were grouped into subgroups (n = 40). The number of units and costs of SLPFs prescribed in total and per subgroup from January to December 2020 were calculated using National Health Service (NHS) Business Service Authority (NHSBSA) ePACT2 (electronic Prescribing Analysis and Cost Tool) for England. Monthly patient SLPF units prescribed were calculated using patient numbers with PKU and non-PKU inherited metabolic disorders (IMD) consuming SLPFs. This was compared to the National Society for PKU (NSPKU) prescribing guidance. Ninety-eight percent of SLPF subgroups (n = 39/40) were more expensive than regular and ‘free-from’ food subgroups. However, costs to prescribe SLPFs are significantly less than theoretical calculations. From January to December 2020, 208,932 units of SLPFs were prescribed (excluding milk replacers), costing the NHS £2,151,973 (including milk replacers). This equates to £962 per patient annually, and prescribed amounts are well below the upper limits suggested by the NSPKU, indicating under prescribing of SLPFs. It is recommended that a simpler and improved system should be implemented. Ideally, specialist metabolic dietitians should have responsibility for prescribing SLPFs. This would ensure that patients with PKU have the necessary access to their essential dietary treatment, which, in turn, should help promote dietary adherence and improve metabolic control.

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.

Funding Information: No funding was required for this project. All work was conducted as part of routine service by all dietitians involved. Publisher Copyright: © 2022 by the authors. 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.

Funding Information: Conflicts of Interest: A.M. (Anita MacDonald) is a member of, the advisory board ELEMENT Danone-Nutricia, the advisory board for Arla and Applied Pharma Research, and received research funding and honoraria from Nutricia, Vitaflo International, Biomarin, MetaHealth, Metax and Merck Serono. S.E. receives research funding from Nutricia, and has received financial support and honoraria from Nutricia and Vitaflo to attend/speak at study days and conferences. A.P. received an educational grant from Cambrooke Therapeutics and grants from Vitaflo International, Nutricia, Merck Serono, Biomarin and Mevalia to attend scientific meetings. A.D. received research funding from Vitaflo International, financial support from Nutricia, Mevalia and Vitaflo International to attend study days and conferences. J.C.R. is a member of the European Nutritionist Expert Panel (Biomarin), the Advisory Board for Applied Pharma Research and Nutricia, and has received honoraria as a speaker from APR, Merck Serono, Biomarin, Nutricia, Vitaflo, Cambrooke, PIAM, and Lifediet. S.F. (Suzanne Ford) is a member of the advisory board for Nutricia, and MetaHealth and has received financial support and honoraria from Cambrooke and Vitaflo. R.S. has received sponsorship to attend conferences and study days, payment to present at conferences from Nutricia Metabolics, Vitaflo Internation and Mevalia. C.E. has received honoraria and educational grants to attend events from Vitaflo, Nutricia, Meta Healthcare and SOBI and is a member of the Advisory Commitee on Borderline Substances. S.F. (Sarah Firman) has received funding to attend conferences and study days from Nutricia, Vitaflo International and Dr. Schär UK Ltd., and consulting fees from Vitaflo International and Meta Healthcare Ltd. C.N. has received financial support and honoraria from Nutricia and Vitaflo to attend/speak at study days and conferences. L.R. is a member of the Nutricia adult advisory board and received honorarium from Nutricia and Vitaflo in the past. F.J.W. receives honoraria, educational and travel grants from Nutricia and Vitaflo. M.H. is a member of advisory board Nutricia Danone and Applied Pharma and has received financial support and honoraria from Nutricia, Vitaflo, Cambrooke, Mevalia, Promin for attendance at conferences/meetings and speakers fee. A.T. (Allyson Terry) has received payment from Vitaflo International for speaking at a patient event. K.vW. receives honoraria and educational grants from Nutricia, Vitaflo and Meta Healthcare. S.T. is expected to receive honoraria from Vitaflo International for providing feedback and reviewing guidelines for PKU Sphere. G.W., M.F., L.G., C.P., S.A., A.M. (Anita MacDonald), A.T. (Alison Tooke), A.W., E.G., L.W., I.H., H.S., J.G., A.E., S.C., R.P. have no conflicts of interest to declare. Publisher Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. Patients with phenylketonuria (PKU) are reliant on special low protein foods (SLPFs) as part of their dietary treatment. In England, several issues regarding the accessibility of SLPFs through the national prescribing system have been highlighted. Therefore, prescribing patterns and expenditure on all SLPFs available on prescription in England (n = 142) were examined. Their costs in comparison to regular protein-containing (n = 182) and ‘free-from’ products (n = 135) were also analysed. Similar foods were grouped into subgroups (n = 40). The number of units and costs of SLPFs prescribed in total and per subgroup from January to December 2020 were calculated using National Health Service (NHS) Business Service Authority (NHSBSA) ePACT2 (electronic Prescribing Analysis and Cost Tool) for England. Monthly patient SLPF units prescribed were calculated using patient numbers with PKU and non-PKU inherited metabolic disorders (IMD) consuming SLPFs. This was compared to the National Society for PKU (NSPKU) prescribing guidance. Ninety-eight percent of SLPF subgroups (n = 39/40) were more expensive than regular and ‘free-from’ food subgroups. However, costs to prescribe SLPFs are significantly less than theoretical calculations. From January to December 2020, 208,932 units of SLPFs were prescribed (excluding milk replacers), costing the NHS £2,151,973 (including milk replacers). This equates to £962 per patient annually, and prescribed amounts are well below the upper limits suggested by the NSPKU, indicating under prescribing of SLPFs. It is recommended that a simpler and improved system should be implemented. Ideally, specialist metabolic dietitians should have responsibility for prescribing SLPFs. This would ensure that patients with PKU have the necessary access to their essential dietary treatment, which, in turn, should help promote dietary adherence and improve metabolic control.

Abstract Introduction Advocacy and commercially funded education successfully reduced barriers to the provision of long-term opioid analgesia. The subsequent escalation of opioid prescribing for chronic noncancer pain has seen increasing harms without improved pain outcomes. Methods This was a one-group pretest-posttest design study. A multidisciplinary team developed a chronic pain educational package for general practitioner trainees emphasizing limitations, risk-mitigation, and deprescribing of opioids with transition to active self-care. This educational intervention incorporated prereadings, a resource kit, and a 90-minute interactional video case-based workshop incorporated into an education day. Evaluation was via pre- and postintervention (two months) questionnaires. Differences in management of two clinical vignettes were tested using McNemar’s test. Results Of 58 eligible trainees, 47 (response rate = 81.0%) completed both questionnaires (36 of whom attended the workshop). In a primary analysis including these 47 trainees, therapeutic intentions of tapering opioid maintenance for pain (in a paper-based clinical vignette) increased from 37 (80.4%) pre-intervention to 44 (95.7%) postintervention (P = 0.039). In a sensitivity analysis including only trainees attending the workshop, 80.0% pre-intervention and 97.1% postintervention tapered opioids (P = 0.070). Anticipated initiation of any opioids for a chronic osteoarthritic knee pain clinical vignette reduced from 35 (74.5%) to 24 (51.1%; P = 0.012) in the primary analysis and from 80.0% to 41.7% in the sensitivity analysis (P = 0.001). Conclusions Necessary improvements in pain management and opioid harm avoidance are predicated on primary care education being of demonstrable efficacy. This brief educational intervention improved hypothetical management approaches two months subsequently. Further research measuring objective changes in physician behavior, especially opioid prescribing, is indicated.

BackgroundAustralian and international guidelines recommend benzodiazepines and related drugs (hereafter “benzodiazepines”) as second-line, short-term medications only. Most benzodiazepines are prescribed by general practitioners (GPs; family physicians). Australian GP registrars (“trainees” or “residents” participating in a post-hospital training, apprenticeship-like, practice-based vocational training program), like senior GPs, prescribe benzodiazepines at high rates. Education within a training program, and experience in general practice, would be expected to reduce benzodiazepine prescribing.ObjectiveTo establish if registrars’ prescribing of benzodiazepines decreases with time within a GP training programDesignLongitudinal analysis from the Registrar Clinical Encounters in Training multi-site cohort studyParticipantsRegistrars of five of Australia’s 17 Regional Training Providers. Analyses were restricted to patients ≥ 16 years.Main MeasuresThe main outcome factor was prescription of a benzodiazepine. Conditional logistic regression was used, with registrar included as a fixed effect, to assess within-registrar changes in benzodiazepine-prescribing rates. The “time” predictor variable was “training term” (6-month duration Terms 1–4). To contextualize these “within-registrar” changes, a mixed effects logistic regression model was used, including a random effect for registrar, to assess within-program changes in benzodiazepine-prescribing rates over time. The “time” predictor variable was “year” (2010–2015).Key ResultsOver 12 terms of data collection, 2010–2015, 1161 registrars (response rate 96%) provided data on 136,809 face-to-face office-based consultations. Two thousand six hundred thirty-two benzodiazepines were prescribed (for 1.2% of all problems managed). In the multivariable model, there was a significant reduction in within-program benzodiazepine prescribing over time (year) (p = < 0.001, OR = 0.94, CI = 0.90, 0.97). However, there was no significant change in ‘within-registrar’ prescribing over time (registrar Term) (p = 0.92, OR = 1.00 [95% CI = 0.94–1.06]).ConclusionsDespite a welcome temporal trend for reductions in overall benzodiazepine prescribing from 2010 to 2015, there is still room for improvement and our findings suggest a lack of effect of specific GP vocational training program education and, thus, an opportunity for targeted education.

Purpose: To explore the association of Australian general practitioner (GP) registrars' responses to uncertainty with their in-consultation information-, advice- and assistance-seeking. Design/methodology/approach: A cross-sectional analysis of data from the Registrar Clinical Encounters in Training (ReCEnT) cohort study in four Australian states. In ReCEnT, GP registrars record details of 60 consecutive consultations, six-monthly, three times during training. Outcome factors in logistic regression models included whether the registrar sought in-consultation information or assistance from (i) their supervisor or (ii) an electronic or paper-based source. Independent variables were the four independent subscales of the Physicians' Reaction to Uncertainty (PRU) instrument, as well as registrar, practice and consultation variables. Findings: 589 registrars contributed details of 70,412 consultations. On multivariable analysis, scores on the two 'affective' PRU subscales 'anxiety regarding diagnosis/management' (OR 1.03; 95% confidence intervals [CIs] [1.01, 1.05], p = 0.003) and 'concern about a bad outcome' (OR 1.03; 95% CIs [1.01, 1.06], p = 0.008) were significantly associated with seeking supervisor assistance. There was no association with 'behavioural' subscales 'reluctance to disclose uncertainty to patients' and 'reluctance to disclose mistakes to physicians'. None of the PRU subscales were significantly associated with information-seeking from electronic or hard copy sources. Research implications: Further research is required to explore the role of uncertainty within registrar-supervisor interactions and to define the role of supervisors in registrars' functional adaptation to clinical uncertainty (including how best to support and train supervisors in this role). Practical implications: GP registrars' 'affective' responses to clinical uncertainty are associated with assistance-seeking from clinical supervisors. While in-consultation assistance-seeking may promote registrars' tolerance of uncertainty, it may also contribute to supervisor workload. Originality/value: This is the first study to examine trainees' levels of uncertainty and their seeking of information and assistance. Limitations: We have not investigated whether registrars' seeking assistance resolved or attenuated, for the index problem, their anxiety or concern.

Batrachochytrium dendrobatidis (Bd) is commonly termed the ‘amphibian chytrid fungus’ but thus far has been documented to be a pathogen of only batrachian amphibians (anurans and caudatans). It is not proven to infect the limbless, generally poorly known, and mostly soil-dwelling caecilians (Gymnophiona). We conducted the largest qPCR survey of Bd in caecilians to date, for more than 200 field-swabbed specimens from five countries in Africa and South America, representing nearly 20 species, 12 genera, and 8 families. Positive results were recovered for 58 specimens from Tanzania and Cameroon (4 families, 6 genera, 6+ species). Quantities of Bd were not exceptionally high, with genomic equivalent (GE) values of 0.052–17.339. In addition, we report the first evidence of lethal chytridiomycosis in caecilians. Mortality in captive (wild-caught, commercial pet trade) Geotrypetes seraphini was associated with GE scores similar to those we detected for field-swabbed, wild animals.