Publications by authors named "Sharon Evans"

130 Publications

Casein Glycomacropeptide: An Alternative Protein Substitute in Tyrosinemia Type I.

Nutrients 2021 Sep 16;13(9). Epub 2021 Sep 16.

Birmingham Women's and Children's Hospital, Birmingham B4 6NH, UK.

Tyrosinemia type I (HTI) is treated with nitisinone, a tyrosine (Tyr) and phenylalanine (Phe)-restricted diet, and supplemented with a Tyr/Phe-free protein substitute (PS). Casein glycomacropeptide (CGMP), a bioactive peptide, is an alternative protein source to traditional amino acids (L-AA). CGMP contains residual Tyr and Phe and requires supplementation with tryptophan, histidine, methionine, leucine, cysteine and arginine.

Aims: a 2-part study assessed: (1) the tolerance and acceptability of a low Tyr/Phe CGMP-based PS over 28 days, and (2) its long-term impact on metabolic control and growth over 12 months.

Methods: 11 children with HTI were recruited and given a low Tyr/Phe CGMP to supply all or part of their PS intake. At enrolment, weeks 1 and 4, caregivers completed a questionnaire on gastrointestinal symptoms, acceptability and ease of PS use. In study part 1, blood Tyr and Phe were assessed weekly; in part 2, weekly to fortnightly. In parts 1 and 2, weight and height were assessed at the study start and end.

Results: Nine of eleven children (82%), median age 15 years (range 8.6-17.7), took low Tyr/Phe CGMP PS over 28 days; it was continued for 12 months in = 5 children. It was well accepted by 67% ( = 6/9), tolerated by 100% ( = 9/9) and improved gastrointestinal symptoms in 2 children. The median daily dose of protein equivalent from protein substitute was 60 g/day (range 45-60 g) with a median of 20 g/day (range 15 to 30 g) from natural protein. In part 2 ( = 5), a trend for improved blood Tyr was observed: 12 months pre-study, median Tyr was 490 μmol/L (range 200-600) and Phe 50 μmol/L (range 30-100); in the 12 months taking low Tyr/Phe CGMP PS, median Tyr was 430 μmol/L (range 270-940) and Phe 40 μmol/L (range 20-70). Normal height, weight and BMI z scores were maintained over 12 months.

Conclusions: In HTI children, CGMP was well tolerated, with no deterioration in metabolic control or growth when studied over 12 months. The efficacy of CGMP in HTI needs further investigation to evaluate the longer-term impact on blood Phe concentrations and its potential influence on gut microflora.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu13093224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8467066PMC
September 2021

Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study.

Nutrients 2021 Aug 31;13(9). Epub 2021 Aug 31.

Birmingham Women's and Children's Hospital, Birmingham B4 6NH, UK.

In a retrospective review, we aimed to assess long-term growth in 17 patients ( = 11 males) with hereditary tyrosinaemia type I (HTI). Median age at assessment was 15.6 years (5.7-26.6 years) and median age at diagnosis was 1 month (range: 0-16 months), with 35% ( = 6/17) symptomatic on presentation. From the age of 8 years, there was a noticeable change in median height, weight, and body-mass-index [BMI]-z-scores. Median height-for-age z-scores were consistently ≤ -1 (IQR -1.6, -0.5) during the first 8 years of life but increased with age. Weight-for-age z-scores ranged between -1 to 0 (IQR -1.2, 0.1) in the first 8 years; then increased to > 0.5 (IQR -0.3, 1.3) by age 16 years, and BMI-for-age z-scores ranged from 0 to 1 (IQR -0.7, 1.3) up to 8 years, and >1 (IQR -0.2, 1.9) until 16 years. The percentage of overweight and obesity was lowest in children aged < 5 years, and consistently > 40% in patients aged between 7 to 16 years. The prescribed total protein intake was associated with improved height growth (p < 0.01). Impaired growth in early life improved with age achieving normal population standards. Further studies are needed to investigate factors that influence growth outcome in HTI patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu13093070DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8472760PMC
August 2021

Effect of Personalized Messages Sent by a Health System's Patient Portal on Influenza Vaccination Rates: a Randomized Clinical Trial.

J Gen Intern Med 2021 Sep 1. Epub 2021 Sep 1.

Department of Pediatrics, UCLA Mattel Children's Hospital, University of California at Los Angeles, Los Angeles, CA, USA.

Background: Adult influenza vaccination rates are low. Tailored patient reminders might raise rates.

Objective: Evaluate impact of a health system's patient portal reminders: (1) tailored to patient characteristics and (2) incorporating behavioral science strategies, on influenza vaccination rates among adults.

Design: Pragmatic 6-arm randomized trial across a health system during the 2019-2020 influenza vaccination season. The setting was one large health system-53 adult primary care practices.

Participants: All adult patients who used the patient portal within 12 months, stratified by the following: young adults (18-64 years, without diabetes), older adults (≥65 years, without diabetes), and those with diabetes (≥18 years).

Interventions: Patients were randomized within strata to either (1) pre-commitment reminder alone (1 message, mid-October), (2) pre-commitment + loss frame messages, (3) pre-commitment + gain frame messages, (4) loss frame messages alone, (5) gain frame messages alone, or (6) standard of care control. Patients in the pre-commitment group were sent a message in mid-October, asking if they planned on getting an influenza vaccination. Patients in loss or gain frame groups were sent up to 3 portal reminders (late October, November, and December, if no documented influenza vaccination in the EHR) about importance and safety of influenza vaccine.

Main Measures: Receipt of 1 influenza vaccine from 10/01/2019 to 03/31/2020.

Key Results: 196,486 patients (145,166 young adults, 29,795 older adults, 21,525 adults with diabetes) were randomized. Influenza vaccination rates were as follows: for young adults 36.8%, for older adults 55.6%, and for diabetics 60.6%. On unadjusted and adjusted (for age, gender, insurance, race, ethnicity, and prior influenza vaccine history) analyses, influenza vaccination rates were not statistically different for any study group versus control.

Conclusions: Patient reminders sent by a health system's patient portal that were tailored to patient demographics (young adults, older adults, diabetes) and that incorporated two behavioral economic messaging strategies (pre-commitment and loss/gain framing) were not effective in raising influenza vaccination rates.

Trial Registration: This trial was registered with ClinicalTrials.gov (NCT04110314).
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s11606-021-07023-wDOI Listing
September 2021

Patient Portal Reminders for Pediatric Influenza Vaccinations: A Randomized Clinical Trial.

Pediatrics 2021 Aug;148(2)

Department of Pediatrics and Mattel Children's Hospital.

Objectives: In a large health system, we evaluated the effectiveness of electronic health record patient portal reminders in increasing pediatric influenza vaccination rates.

Methods: We conducted an intention-to-treat randomized clinical trial of 22 046 children from 6 months to <18 years of age in 53 primary care practices. Patients (or parent and/or proxies) who were active portal users were randomly assigned to receive reminder messages framed as gains or losses or no messages. They were separately randomly assigned to receive a precommitment message before the influenza season. The primary outcome was receipt of ≥1 seasonal influenza vaccinations. Additionally, children 6 months to <3 years of age due for a second influenza vaccine were randomly assigned to receive a reminder or no reminder for the second vaccination.

Results: First-dose influenza vaccination rates were 56.9% in the control group, 58.0% in the loss-frame reminders group ( = .07), and 58.0% in the gain-frame group ( = .47). Rates were 58.3% in the precommitment group versus 57.0% in the control group ( = .11). Adjusted risk ratios for first vaccination were 1.02 (95% confidence interval [CI]: 1.00-1.04) for loss-frame reminders, 1.01 (95% CI: 0.98-1.05) for gain-frame reminders, and 1.02 (95% CI: 1.00-1.04) for precommitment messages versus controls. Second-dose vaccination rates were 44.1% in the control group and 55.0% in the reminder group, with an adjusted risk ratio of 1.25 (95% CI: 1.07-1.45).

Conclusions: Patient portal reminders for influenza vaccines in children, whether framed as gains or losses, did not increase first-dose influenza vaccination rates but were highly effective for the second dose of the vaccine.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1542/peds.2020-048413DOI Listing
August 2021

A Three-Year Longitudinal Study Comparing Bone Mass, Density, and Geometry Measured by DXA, pQCT, and Bone Turnover Markers in Children with PKU Taking L-Amino Acid or Glycomacropeptide Protein Substitutes.

Nutrients 2021 Jun 17;13(6). Epub 2021 Jun 17.

Birmingham Women's and Children's Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK.

In patients with phenylketonuria (PKU), treated by diet therapy only, evidence suggests that areal bone mineral density (BMDa) is within the normal clinical reference range but is below the population norm.

Aims: To study longitudinal bone density, mass, and geometry over 36 months in children with PKU taking either amino acid (L-AA) or casein glycomacropeptide substitutes (CGMP-AA) as their main protein source.

Methodology: A total of 48 subjects completed the study, 19 subjects in the L-AA group (median age 11.1, range 5-16 years) and 29 subjects in the CGMP-AA group (median age 8.3, range 5-16 years). The CGMP-AA was further divided into two groups, CGMP100 (median age 9.2, range 5-16 years) ( = 13), children taking CGMP-AA only and CGMP50 (median age 7.3, range 5-15 years) ( = 16), children taking a combination of CGMP-AA and L-AA. Dual X-ray absorptiometry (DXA) was measured at enrolment and 36 months, peripheral quantitative computer tomography (pQCT) at 36 months only, and serum blood and urine bone turnover markers (BTM) and blood bone biochemistry at enrolment, 6, 12, and 36 months.

Results: No statistically significant differences were found between the three groups for DXA outcome parameters, i.e., BMDa (L2-L4 BMDa g/cm), bone mineral apparent density (L2-L4 BMAD g/cm) and total body less head BMDa (TBLH g/cm). All blood biochemistry markers were within the reference ranges, and BTM showed active bone turnover with a trend for BTM to decrease with increasing age.

Conclusions: Bone density was clinically normal, although the median z scores were below the population mean. BTM showed active bone turnover and blood biochemistry was within the reference ranges. There appeared to be no advantage to bone density, mass, or geometry from taking a macropeptide-based protein substitute as compared with L-AAs.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu13062075DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8233747PMC
June 2021

Growth and Body Composition in PKU Children-A Three-Year Prospective Study Comparing the Effects of L-Amino Acid to Glycomacropeptide Protein Substitutes.

Nutrients 2021 Apr 16;13(4). Epub 2021 Apr 16.

Birmingham Women's and Children's Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK.

Protein quality and quantity are important factors in determining lean body (muscle) mass (LBM). In phenylketonuria (PKU), protein substitutes provide most of the nitrogen, either as amino acids (AA) or glycomacropeptide with supplementary amino acids (CGMP-AA). Body composition and growth are important indicators of long-term health. In a 3-year prospective study comparing the impact of AA and CGMP-AA on body composition and growth in PKU, 48 children were recruited. = 19 (median age 11.1 years, range 5-15 years) took AA only, = 16 (median age 7.3 years, range 5-15 years) took a combination of CGMP-AA and AA, (CGMP50) and 13 children (median age 9.2 years, range 5-16 years) took CGMP-AA only (CGMP100). A dual energy X-ray absorptiometry (DXA) scan at enrolment and 36 months measured LBM, % body fat (%BF) and fat mass (FM). Height was measured at enrolment, 12, 24 and 36 months. No correlation or statistically significant differences (after adjusting for age, gender, puberty and phenylalanine blood concentrations) were found between the three groups for LBM, %BF, FM and height. The change in height z scores, (AA 0, CGMP50 +0.4 and CGMP100 +0.7) showed a trend that children in the CGMP100 group were taller, had improved LBM with decreased FM and % BF but this was not statistically significant. There appeared to be no advantage of CGMP-AA compared to AA on body composition after 3-years of follow-up. Although statistically significant differences were not reached, a trend towards improved body composition was observed with CGMP-AA when it provided the entire protein substitute requirement.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu13041323DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8073059PMC
April 2021

Accidental Consumption of Aspartame in Phenylketonuria: Patient Experiences.

Nutrients 2021 Feb 23;13(2). Epub 2021 Feb 23.

Birmingham Women's and Children's NHS Foundation Trust, Steelhouse Lane, Birmingham B4 6NH, UK.

Aspartame is a phenylalanine containing sweetener, added to foods and drinks, which is avoided in phenylketonuria (PKU). However, the amount of phenylalanine provided by aspartame is unidentifiable from food and drinks labels. We performed a cross-sectional online survey aiming to examine the accidental aspartame consumption in PKU. 206 questionnaires (58% female) were completed. 55% of respondents ( = 114) were adults with PKU or their parent/carers and 45% ( = 92) were parents/carers of children with PKU. 74% ( = 152/206) had consumed food/drinks containing aspartame. Repeated accidental aspartame consumption was common and more frequent in children ( < 0.0001). The aspartame containing food/drinks accidentally consumed were fizzy drinks (68%, = 103/152), fruit squash (40%, = 61/152), chewing gum (30%, = 46/152), flavoured water (25%, = 38/152), ready to drink fruit squash cartons (23%, = 35/152) and sports drinks (21%, = 32/152). The main reasons described for accidental consumption, were manufacturers' changing recipes (81%, = 123/152), inability to check the ingredients in pubs/restaurants/vending machines (59%, = 89/152) or forgetting to check the label (32%, = 49/152). 23% (= 48/206) had been prescribed medicines containing aspartame and 75% ( = 36/48) said that medicines were not checked by medics when prescribed. 85% ( = 164/192) considered the sugar tax made accidental aspartame consumption more likely. Some of the difficulties for patients were aspartame identification in drinks consumed in restaurants, pubs, vending machines (77%, = 158/206); similarities in appearance of aspartame and non-aspartame products (62%, = 127/206); time consuming shopping/checking labels (56%, = 115/206); and unclear labelling (55%, = 114/206). These issues caused anxiety for the person with PKU (52%, = 106/206), anxiety for parent/caregivers (46%, = 95/206), guilt for parent/carers (42%, = 87/206) and social isolation (42%, = 87/206). It is important to understand the impact of aspartame and legislation such as the sugar tax on people with PKU. Policy makers and industry should ensure that the quality of life of people with rare conditions such as PKU is not compromised through their action.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu13020707DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7926728PMC
February 2021

Protein Substitutes in PKU; Their Historical Evolution.

Nutrients 2021 Feb 2;13(2). Epub 2021 Feb 2.

Birmingham Women's and Children's Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK.

Protein substitutes developed for phenylketonuria (PKU) are a synthetic source of protein commonly based on L-amino acids. They are essential in the treatment of phenylketonuria (PKU) and other amino acid disorders, allowing the antagonistic amino acid to be removed but with the safe provision of all other amino acids necessary for maintaining normal physiological function. They were first formulated by a chemist and used experimentally on a 2-year-old girl with PKU and their nutritional formulations and design have improved over time. Since 2008, a bioactive macropeptide has been used as a base for protein substitutes in PKU, with potential benefits of improved bone and gut health, nitrogen retention, and blood phenylalanine control. In 2018, animal studies showed that physiomimic technology coating the amino acids with a polymer allows a slow release of amino acids with an improved physiological profile. History has shown that in PKU, the protein substitute's efficacy is determined by its nutritional profile, amino acid composition, dose, timing, distribution, and an adequate energy intake. Protein substitutes are often given little importance, yet their pharmacological actions and clinical benefit are pivotal when managing PKU.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu13020484DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7912909PMC
February 2021

Breast Tumor Microenvironment in Black Women: A Distinct Signature of CD8+ T-Cell Exhaustion.

J Natl Cancer Inst 2021 Aug;113(8):1036-1043

Department of Cancer Prevention and Control, Roswell Park Comprehensive Cancer Center, Buffalo, NY, USA.

Background: Blacks tend to have a stronger inflammatory immune response than Whites. We hypothesized that racial differences in host immunity also manifest in the tumor microenvironment, constituting part of a distinct aggressive tumor biology underlying higher mortality in Black women.

Methods: Pathological and gene expression profiling approaches were used for characterizing infiltrating immune cells in breast tumor microenvironment from 1315 patients from the Women's Circle of Health Study. Racial differences in tumor immune phenotypes were compared, with results validated in a publicly accessible dataset. Prognostic associations of immune phenotypes were assessed in 3 independent cohorts.

Results: We found marked and consistent differences in tumor immune responses between Black and White patients. Not only did tumors from Blacks display a stronger overall immune presence but also the composition and quality of immune infiltrates differed, regardless of tumor subtypes. Black patients had a stronger CD4+ and B-cell response, and further, a more exhausted CD8+ T-cell profile. A signature indicating a higher ratio of exhausted CD8+ T cells to total CD8+ T cells (ExCD8-r) was consistently associated with poorer survival, particularly among hormone receptor-positive patients. Among hormone receptor-negative patients, combinations of the absolute fraction of CD8+ T cells and ExCD8-r signature identified the CD8lowExCD8-rhigh subgroup, the most prevalent among Blacks, with the worst survival.

Conclusions: Our findings of a distinct exhausted CD8+ T-cell signature in Black breast cancer patients indicate an immunobiological basis for their more aggressive disease and a rationale for the use of immune checkpoint inhibitors targeting the exhaustion phenotype.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/jnci/djaa215DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8328978PMC
August 2021

Protein Labelling Accuracy for UK Patients with PKU Following a Low Protein Diet.

Nutrients 2020 Nov 10;12(11). Epub 2020 Nov 10.

Birmingham Women's and Children's NHS Foundation Trust, Steelhouse Lane, Birmingham B4 6NH, UK.

A phenylalanine (protein)-restricted diet is the primary treatment for phenylketonuria (PKU). Patients are dependent on food protein labelling to successfully manage their condition. We evaluated the accuracy of protein labelling on packaged manufactured foods from supermarket websites for foods that may be eaten as part of a phenylalanine-restricted diet. Protein labelling information was evaluated for 462 food items ("free from", = 159, regular, = 303), divided into 16 food groups using supermarket website data. Data collection included protein content per portion/100 g when food was "as sold", "cooked" or "prepared"; cooking methods, and preparation instructions. Labelling errors affecting protein content were observed in every food group, with overall protein labelling unclear in 55% ( = 255/462) of foods. There was misleading, omitted, or erroneous (MOE) information in 43% ( = 68/159) of "free from" foods compared with 62% ( = 187/303) of regular foods, with fewer inaccuracies in "free from" food labelling ( = 0.007). Protein analysis was available for uncooked weight only but not cooked weight for 58% ( = 85/146) of foods; 4% ( = 17/462) had misleading protein content. There was a high rate of incomplete, misleading, or inaccurate data affecting the interpretation of the protein content of food items on supermarket websites. This could adversely affect metabolic control of patients with PKU and warrants serious consideration.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu12113440DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7696494PMC
November 2020

Dietary Management, Clinical Status and Outcome of Patients with Citrin Deficiency in the UK.

Nutrients 2020 Oct 29;12(11). Epub 2020 Oct 29.

Birmingham Women's and Children's Hospital, Birmingham B4 6NH, UK.

Background: Little is known about the optimal dietary treatment for citrin deficiency. Our aim is to describe the management of UK citrin deficiency patients.

Methods: A longitudinal retrospective review was performed. Data were collected from medical records on presenting signs and symptoms, dietary management and clinical outcome.

Results: data were collected on 32 patients from 21 families. 50% were females (16/32). Median age at diagnosis was 4 y (5 days-35 y) with 12 patients diagnosed in the neonatal period with neonatal intrahepatic cholestasis (NICCD), eight later in childhood (FTTDCD) and 12 by family screening based on index cases from five families. No patient had adult-onset type II citrullinemia. The patient age at the time of data collection was a median of 11 y (1-44 y). 91% (29/32) of patients had normal physical and neurological development, 47% (15/32) experienced recurrent unexplained abdominal pain and 9% (3/32) episodes of hypoglycaemia. Siblings had different phenotypes (5 families had > 1 affected patient). Most patients preferred high protein foods, limiting sugar-containing foods. Only 41% (13/32) were prescribed a low CHO, high protein, high fat diet (restriction varied) and two used medium chain triglyceride (MCT) supplements. No patient was prescribed drug therapy. Twenty-five per cent (8/32) of patients were underweight and 41% (13/32) had height <-1 z-scores.

Conclusions: patients presented with various phenotypes, symptoms and suboptimal growth. Symptoms and biochemical markers improved with age, but height remained low in some. More research is necessary to assess the effectiveness of dietary approaches in improving clinical outcomes and symptoms in citrin deficiency.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu12113313DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7693899PMC
October 2020

A 3 Year Longitudinal Prospective Review Examining the Dietary Profile and Contribution Made by Special Low Protein Foods to Energy and Macronutrient Intake in Children with Phenylketonuria.

Nutrients 2020 Oct 15;12(10). Epub 2020 Oct 15.

Dietetic Department, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.

The nutritional composition of special low protein foods (SLPFs) is controlled under EU legislation for 'Foods for Special Medical Purposes (FSMP)'. They are designed to meet the energy needs of patients unable to eat a normal protein containing diet. In phenylketonuria (PKU), the macronutrient contribution of SLPFs has been inadequately examined.

Aim: A 3-year longitudinal prospective study investigating the contribution of SLPFs to the macronutrient intake of children with early treated PKU.

Methods: 48 children (27 boys) with a mean recruitment age of 9.3 y were studied. Semi-quantitative dietary assessments and food frequency questionnaires (FFQ) were collected three to four times/year for 3 years.

Results: The mean energy intake provided by SLPFs was 33% (SD ± 8), and this figure was 42% (SD ± 13) for normal food and 21% (SD ± 5) for protein substitutes (PS). SLPFs supplied a mean intake of 40% carbohydrate (SD ± 10), 51% starch (SD ± 18), 21% sugar (SD ± 8), and 38% fat (SD ± 13). Fibre intake met 83% of the Scientific Advisory Committee on Nutrition (SACN) reference value, with 50% coming from SLPFs with added gums and hydrocolloids. Low protein bread, pasta and milk provided the highest energy contribution, and the intake of sweet SLPFs (e.g., biscuits, cakes, and chocolate) was minimal. Children averaged three portions fruit/vegetable daily, and children aged ≥ 12 y had irregular meal patterns.

Conclusion: SLPFs provide essential energy in phenylalanine restricted diets. Optimising the nutritional quality of SLPFs deserves more attention.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu12103153DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7602523PMC
October 2020

The Impact of the Use of Glycomacropeptide on Satiety and Dietary Intake in Phenylketonuria.

Nutrients 2020 Sep 4;12(9). Epub 2020 Sep 4.

Dietetic Department, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.

Protein is the most satiating macronutrient, increasing secretion of gastrointestinal hormones and diet induced thermogenesis. In phenylketonuria (PKU), natural protein is restricted with approximately 80% of intake supplied by a synthetic protein source, which may alter satiety response. Casein glycomacropeptide (CGMP-AA), a carbohydrate containing peptide and alternative protein substitute to amino acids (AA), may enhance satiety mediated by its bioactive properties.

Aim: In a three-year longitudinal; prospective study, the effect of AA and two different amounts of CGMP-AA (CGMP-AA only (CGMP100) and a combination of CGMP-AA and AA (CGMP50) on satiety, weight and body mass index (BMI) were compared.

Methods: 48 children with PKU completed the study. Median ages of children were: CGMP100; ( = 13), 9.2 years; CGMP50; ( = 16), 7.3 years; and AA ( = 19), 11.1 years. Semi-quantitative dietary assessments and anthropometry (weight, height and BMI) were measured every three months.

Results: The macronutrient contribution to total energy intake from protein, carbohydrate and fat was similar across the groups. Adjusting for age and gender, no differences in energy intake, weight, BMI, incidence of overweight or obesity was apparent between the groups.

Conclusion: In this three-year longitudinal study, there was no indication to support a relationship between CGMP and satiety, as evidenced by decreased energy intake, thereby preventing overweight or obesity. Satiety is a complex multi-system process that is not fully understood.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu12092704DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7576483PMC
September 2020

An Observational Study Evaluating the Introduction of a Prolonged-Release Protein Substitute to the Dietary Management of Children with Phenylketonuria.

Nutrients 2020 Sep 3;12(9). Epub 2020 Sep 3.

Dietetic Department, Birmingham Women's and Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.

Dietary restriction of phenylalanine combined with a protein substitute prevents intellectual disability in patients with phenylketonuria (PKU). However, current protein substitutes are associated with low adherence owing to unpalatability and burdensome administration regimens. This prospective, observational acceptability study in children with PKU assessed the use of a prolonged-release protein substitute designed with an ethyl cellulose and arginate coating masking the bitter taste, smell and reducing the osmolarity of free amino acids. The study product was mixed with the subject's food or drink and replaced ≥1 dose per day of the subject's usual protein substitute for 7 days. Seven of 13 subjects were able to take their prescribed dose over the 7 day period. Most subjects mixed the test protein substitute with food or fruit juice. Reduced blood phenylalanine levels ( = 5) and improved phenylalanine/tyrosine ratio ( = 4) were recorded from baseline to Day 7, respectively. Four subjects reported fewer gastrointestinal symptoms compared to baseline. There were no cases of diarrhoea, constipation, bloating, nausea or vomiting. No adverse reactions were reported. In conclusion, the novel prolonged-release protein substitute was taken in a different way to a typical protein substitute and enabled satisfactory blood phenylalanine control. The study product was well tolerated; subjects experienced fewer gastrointestinal symptoms than with their previous treatment. Although the results of this pilot study provide reassuring data, longer-term studies evaluating adherence and blood phenylalanine control are necessary.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu12092686DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7551143PMC
September 2020

Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria.

Nutrients 2020 Aug 14;12(8). Epub 2020 Aug 14.

Dietetic Department, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.

In Phenylketonuria (PKU), the peptide structure of the protein substitute (PS), casein glycomacropeptide (CGMP), is supplemented with amino acids (CGMP-AA). CGMP may slow the rate of amino acid (AA) absorption compared with traditional phenylalanine-free amino acids (Phe-free AA), which may improve nitrogen utilization, decrease urea production, and alter insulin response.

Aim: In children with PKU, to compare pre and postprandial AA concentrations when taking one of three PS's: Phe-free AA, CGMP-AA 1 or 2.

Methods: 43 children (24 boys, 19 girls), median age 9 years (range 5-16 years) were studied; 11 took CGMP-AA1, 18 CGMP-AA2, and 14 Phe-free AA. Early morning fasting pre and 2 h postprandial blood samples were collected for quantitative AA on one occasion. A breakfast with allocated 20 g protein equivalent from PS was given post fasting blood sample.

Results: There was a significant increase in postprandial AA for all individual AAs with all three PS. Postprandial AA histidine ( < 0.001), leucine ( < 0.001), and tyrosine ( < 0.001) were higher in CGMP-AA2 than CGMP-AA1, and leucine ( < 0.001), threonine ( < 0.001), and tyrosine ( = 0.003) higher in GCMP-AA2 than Phe-free AA. This was reflective of the AA composition of the three different PS's.

Conclusions: In PKU, the AA composition of CGMP-AA influences 2 h postprandial AA composition, suggesting that a PS derived from CGMP-AA may be absorbed similarly to Phe-free AA, but this requires further investigation.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu12082443DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7468934PMC
August 2020

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

Nutrients 2020 Jul 24;12(8). Epub 2020 Jul 24.

Bristol Royal Hospital for Children, Bristol BS2 8BJ, UK.

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).
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu12082205DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7468820PMC
July 2020

Special Low Protein Foods in the UK: An Examination of Their Macronutrient Composition in Comparison to Regular Foods.

Nutrients 2020 Jun 25;12(6). Epub 2020 Jun 25.

Birmingham Women's and Children's NHS Foundation Trust, Steelhouse Lane, Birmingham B4 6NH, UK.

Special low protein foods (SLPFs) are essential in a low phenylalanine diet for treating phenylketonuria (PKU). With little known about their nutritional composition, all SLPFs on UK prescription were studied ( = 146) and compared to equivalent protein-containing foods ( = 190). SLPF nutritional analysis was obtained from suppliers/manufacturers. Comparable information about regular protein-containing foods was obtained from online UK supermarkets. Similar foods were grouped together, with mean nutritional values calculated for each subgroup ( = 40) and percentage differences determined between SLPFs and regular food subgroups. All SLPF subgroups contained 43-100% less protein than regular foods. Sixty-three percent ( = 25/40) of SLPF subgroups contained less total fat with palm oil (25%, = 36/146) and hydrogenated vegetable oil (23%, = 33/146) key fat sources. Sixty-eight percent ( = 27/40) of SLPF subgroups contained more carbohydrate, with 72% ( = 105/146) containing added sugar. Key SLPF starch sources were maize/corn (72%; = 105/146). Seventy-seven percent ( = 113/146) of SLPFs versus 18% ( = 34/190) of regular foods contained added fibre, predominantly hydrocolloids. Nine percent of SLPFs contained phenylalanine > 25 mg/100 g and sources of phenylalanine/protein in their ingredient lists. Stricter nutritional composition regulations for SLPFs are required, identifying maximum upper limits for macronutrients and phenylalanine, and fat and carbohydrate sources that are associated with healthy outcomes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu12061893DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7353443PMC
June 2020

Risk factors for renal disease and diabetes in remote Australia - findings from The Western Desert Kidney Health Project.

Rural Remote Health 2020 06 9;20(2):5440. Epub 2020 Jun 9.

La Trobe University, Kingsbury Drive, Bundoora, Vic. 3086, Australia

Introduction: The Western Desert Kidney Health Project (WDKHP) aimed to determine the prevalence of type 2 diabetes (T2DM), kidney disease and associated risk factors in Aboriginal and non-Aboriginal people in a remote area of Western Australia.

Methods: The project, featuring whole-of-community cross-sectional surveys and health assessments using point-of-care testing, was conducted in five small towns and six remote Aboriginal communities in the Goldfields of Western Australia between 2010 and 2014. Initial health assessments were completed by 597 adults (424 Aboriginal) and 502 children (393 Aboriginal). This included almost 80% of the Aboriginal population. All non-Aboriginal people residing in the six remote Aboriginal communities participated.

Results: Risk factors for renal disease and T2DM were present in participants of all ages, including children as young as 2 years. There was no significant difference between Aboriginal and non-Aboriginal children. Aboriginal and non-Aboriginal adult participants had twice the burden of T2DM than the standard Australian population. More than 12% of all children had elevated albumin-creatinine ratio (ACR). Adults had markers of kidney disease at higher rates than expected: 51% of Aboriginal adults and 27% of non-Aboriginal adults had at least one marker of kidney disease (haematuria, proteinuria or elevated ACR). Aboriginal women were the highest risk group (32% T2DM, 40% elevated ACR). Haematuria and low urine pH were common findings, 21% of people had haematuria (greater than trace) and 71% had urine pH of 6 or less; there was no difference in this finding between Aboriginal and non-Aboriginal people.

Conclusion: The WDKHP found higher than expected rates of risk factors for T2DM and renal disease compared with Australian Bureau of Statistics rates for Australian Aboriginal and non-Aboriginal adults and children, with Aboriginal women the highest risk group. The rates for non-Aboriginal participants were higher than expected, suggesting exposures in common might be more important than ethnicity.The high prevalence of aciduria and haematuria found in both Aboriginal and non-Aboriginal participants in this study suggests that factors contributing to a chronic metabolic acidosis and inflammation or irritation of the urinary tract need to be explored. Drinking water quality in this remote area is known to be poor and may be an important contributing factor. Many of the contributing factors are potentially modifiable - such as water quality, food supply, exercise opportunities and living conditions - offering scope for interventions to reduce the risk and burden of these diseases.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.22605/RRH5440DOI Listing
June 2020

Effect of Patient Portal Reminders Sent by a Health Care System on Influenza Vaccination Rates: A Randomized Clinical Trial.

JAMA Intern Med 2020 07;180(7):962-970

Department of Pediatrics, UCLA (University of California, Los Angeles) Mattel Children's Hospital, UCLA.

Importance: Influenza vaccination rates across the US are low. Because few practices send patient reminders for influenza vaccination, a scalable patient reminder system is needed.

Objective: To evaluate the effect of patient reminders sent via a health care system's electronic health record patient portal on influenza vaccination rates.

Design, Setting, And Participants: This pragmatic, 4-arm randomized clinical trial was performed from October 1, 2018, to March 31, 2019, across the UCLA (University of California, Los Angeles) health care system. A total of 164 205 patients in 52 primary care practices who had used the patient portal within 12 months were included.

Interventions: Patients due for an influenza vaccine were sent a letter via the patient portal of the health care system reminding them about the importance of influenza vaccination, safety of the vaccine, and morbidity associated with influenza. Patients were randomized within primary care practices to 1 of 4 study groups (no reminder [n = 41 070] vs 1 reminder [n = 41 055], 2 reminders [n = 41 046], or 3 reminders [n = 41 034]).

Main Outcomes And Measures: The primary outcome was receipt of 1 or more influenza vaccines as documented in the electronic health record, which was supplemented with influenza vaccination data from external sources (eg, pharmacies). Secondary outcomes were influenza vaccination rates among subgroups and influenza vaccinations self-reported by patients in reply to the portal-based query as having been received elsewhere.

Results: A total of 164 205 patients (mean [SD] age, 46.2 [19.6] years; 95 779 [58.3%] female) were randomly allocated to 1 of the 4 study arms. In the primary analysis across all ages and not including patient self-reported vaccinations in reply to portal reminders, influenza vaccination rates were 37.5% for those receiving no reminders, 38.0% for those receiving 1 reminder (P = .008 vs no reminder), 38.2% for those receiving 2 reminders (P = .03 vs no reminder), and 38.2% for those receiving 3 reminders (P = .02 vs no reminder). In the secondary analysis not including patient self-reported vaccinations, among adults aged 18 to 64 years (vaccination rates: 32.0% in the control group, 32.8% in the 1-reminder group, 32.8% in the 2-reminder group, and 32.8% in the 3-reminder group; P = .001), male patients (vaccination rates: 37.3% vs 38.3%, 38.6%, and 38.8%; P = .001), non-Hispanic patients (vaccination rates: 37.6% vs 38.2%, 38.3%, and 38.2%; P = .004), and those who were not vaccinated in the prior 2 years (vaccination rates: 15.3% vs 15.9%, 16.3%, and 16.1%; P < .001), vaccination rates were higher in the portal reminder groups than in the control group; the findings in these 3 subgroups mirrored the findings in the entire population. When self-reported vaccinations received elsewhere were included, influenza vaccination rates were 1.4 to 2.9 percentage points higher in the portal reminder groups, with a dose-response effect (0 reminders: 15 537 [37.8%]; 1 reminder: 16 097 [39.2%]; 2 reminders: 16 426 [40.0%]; and 3 reminders: 16 714 [40.7%]; P < .001).

Conclusions And Relevance: Generic patient portal reminders were effective in minimally increasing influenza vaccination rates, but more intensive or more targeted patient motivational strategies appear to be needed.

Trial Registration: ClinicalTrials.gov Identifier: NCT03666026.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1001/jamainternmed.2020.1602DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7235900PMC
July 2020

Natural Protein Tolerance and Metabolic Control in Patients with Hereditary Tyrosinaemia Type 1.

Nutrients 2020 Apr 19;12(4). Epub 2020 Apr 19.

Birmingham Women's and Children's Hospital, Birmingham B4 6NH, UK.

In a longitudinal retrospective study, we aimed to assess natural protein (NP) tolerance and metabolic control in a cohort of 20 Hereditary Tyrosinaemia type I (HTI) patients. Their median age was 12 years ([3.2-17.7 years], = 11 female, = 8 Caucasian, = 8 Asian origin, = 2 Arabic and = 2 Indian). All were on nitisinone (NTBC) with a median dose of 0.7 g/kg/day (range 0.4-1.5 g/kg/day) and were prescribed a tyrosine (Tyr)/phenylalanine (Phe)-restricted diet supplemented with Tyr/Phe-free L-amino acids. Data were collected on clinical signs at presentation, medical history, annual dietary prescriptions, and blood Phe and Tyr levels from diagnosis until transition to the adult service (aged 16-18 years) or liver transplantation (if it preceded transition). The median age of diagnosis was 2 months (range: 0 to 24 months), with = 1 diagnosed by newborn screening, = 3 following phenylketonuria (PKU) screening and = 7 by sibling screening. Five patients were transplanted (median age 6.3 years), and one died due to liver cancer. The median follow-up was 10 years (3-16 years), and daily prescribed NP intake increased from a median of 5 to 24 g/day. Lifetime median blood Tyr (370 µmol/L, range 280-420 µmol/L) and Phe (50 µmol/L, 45-70 µmol/L) were maintained within the target recommended ranges. This cohort of HTI patients were able to increase the daily NP intake with age while maintaining good metabolic control. Extra NP may improve lifelong adherence to the diet.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu12041148DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7230348PMC
April 2020

A Fever-Th17 Cell Immune Axis: Some SMADs Like It Hot.

Immunity 2020 02;52(2):209-211

Department of Immunology, Roswell Park Comprehensive Cancer Center, Buffalo, NY, USA.

Fever has beneficial effects on immune responses; however, its impact on T cell polarization is poorly understood. In this issue of Immunity, Wang et al. show that fever acts through a T cell-intrinsic SMAD4-dependent mechanism that selectively drives Th17 cell differentiation and pathogenicity in autoimmunity.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.immuni.2020.01.010DOI Listing
February 2020

Dietary practices in methylmalonic acidaemia: a European survey.

J Pediatr Endocrinol Metab 2020 Jan;33(1):147-155

Evelina London Children's Hospital, Guy's and St. Thomas' NHS Foundation Trust, London, UK.

Background The dietary management of methylmalonic acidaemia (MMA) is a low-protein diet providing sufficient energy to avoid catabolism and to limit production of methylmalonic acid. The goal is to achieve normal growth, good nutritional status and the maintenance of metabolic stability. Aim To describe the dietary management of patients with MMA across Europe. Methods A cross-sectional questionnaire was sent to European colleagues managing inherited metabolic disorders (IMDs) (n=53) with 27 questions about the nutritional management of organic acidaemias. Data were analysed by different age ranges (0-6 months; 7-12 months; 1-10 years; 11-16 years; >16 years). Results Questionnaires were returned from 53 centres. Twenty-five centres cared for 80 patients with MMA vitamin B12 responsive (MMAB12r) and 43 centres managed 215 patients with MMA vitamin B12 non-responsive (MMAB12nr). For MMAB12r patients, 44% of centres (n=11/25) prescribed natural protein below the World Health Organization/Food and Agriculture Organization/United Nations University (WHO/FAO/UNU) 2007 safe levels of protein intake in at least one age range. Precursor-free amino acids (PFAA) were prescribed by 40% of centres (10/25) caring for 36% (29/80) of all the patients. For MMAB12nr patients, 72% of centres (n=31/43) prescribed natural protein below the safe levels of protein intake (WHO/FAO/UNU 2007) in at least one age range. PFAA were prescribed by 77% of centres (n=33/43) managing 81% (n=174/215) of patients. In MMAB12nr patients, 90 (42%) required tube feeding: 25 via a nasogastric tube and 65 via a gastrostomy. Conclusions A high percentage of centres used PFAA in MMA patients together with a protein prescription that provided less than the safe levels of natural protein intake. However, there was inconsistent practices across Europe. Long-term efficacy studies are needed to study patient outcome when using PFAA with different severities of natural protein restrictions in patients with MMA to guide future practice.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1515/jpem-2019-0277DOI Listing
January 2020

Introduction to .

Int J Hyperthermia 2019 11;36(sup1):1-2

Roswell Park Cancer Institute, Buffalo, NY, USA.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1080/02656736.2019.1659427DOI Listing
November 2019

thermal ablation augments antitumor efficacy of adoptive T cell therapy.

Int J Hyperthermia 2019 11;36(sup1):22-36

Department of Immunology, Roswell Park Comprehensive Cancer Center, Buffalo, NY, USA.

The aim of this study is to investigate whether radiofrequency ablation (RFA) improves the efficacy of adoptive T cell immunotherapy in preclinical mouse cancer models. Mice implanted subcutaneously () with syngeneic colon adenocarcinoma or melanoma were treated with sub-curative RFA (90 °C, 1 min). Trafficking of T cells to lymph nodes (LN) or tumors was quantified by homing assays and intravital microscopy (IVM) after sham procedure or RFA. Expression of trafficking molecules (CCL21 and intercellular adhesion molecule-1 [ICAM-1]) on high endothelial venules (HEV) in LN and tumor vessels was evaluated by immunofluorescence microscopy. Tumor-bearing mice were pretreated with RFA to investigate the therapeutic benefit when combined with adoptive transfer of -activated tumor-specific CD8 T cells. RFA increased trafficking of naïve CD8 T cells to tumor-draining LN (TdLN). A corresponding increase in expression of ICAM-1 and CCL21 was detected on HEV in TdLN but not in contralateral (c)LN. IVM revealed that RFA substantially enhanced secondary firm arrest of lymphocytes selectively in HEV in TdLN. Furthermore, strong induction of ICAM-1 in tumor vessels was associated with significantly augmented trafficking of adoptively transferred -activated CD8 T cells to tumors after RFA. Finally, preconditioning tumors with RFA augmented CD8 T cell-mediated apoptosis of tumor targets and delayed growth of established tumors when combined with adoptive T cell transfer immunotherapy. These studies suggest that in addition to its role as a palliative therapeutic modality, RFA may have clinical potential as an immune-adjuvant therapy by augmenting the efficacy of adoptive T cell therapy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1080/02656736.2019.1653500DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6897324PMC
November 2019

Impact of a Nurse-Led Advance Care Planning Intervention on Satisfaction, Health-Related Quality of Life, and Health Care Utilization Among Patients With Severe Respiratory Disease: A Randomized Patient-Preference Trial.

J Pain Symptom Manage 2020 04 30;59(4):848-855. Epub 2019 Nov 30.

Sir Charles Gairdner Hospital, Perth, WA, Australia.

Context: Previous work has found that facilitated advance care planning (ACP) interventions are effective in increasing ACP uptake among patients with severe respiratory disease.

Objectives: The objective of this study was to investigate whether a nurse-led, facilitated ACP intervention among participants with severe respiratory disease impacts self-reported or clinical outcomes.

Methods: A multicenter, open-label, patient-preference, randomized controlled trial of a nurse-led facilitated ACP intervention was performed. Outcome measures included self-report scales (health care satisfaction and EQ-5D-5L health-related quality of life at three- and six-month follow-up), 12-month mortality, and health care utilization during the final 90 days of life.

Results: One hundred forty-nine participants were recruited across two study settings (metropolitan tertiary hospital respiratory department and rural sites) and 106 were allocated to receive the ACP intervention. There was no effect of the intervention on satisfaction with health care, health-related quality of life, or 12-month mortality rates. Among those participants who died during the follow-up period (N = 54), those allocated to the ACP intervention had significantly fewer outpatient consultations (7.51 vs. 13.6, P < 0.001). There were no changes in emergency department attendances, total hospital admissions or length of stay, or home nursing visits. Among those allocated to the ACP intervention, there was a reduced length of stay in acute hospital settings (7.76 vs. 11.5 nights, P < 0.001) and increased length of stay in palliative hospital settings (5.54 vs. 2.08, P < 0.001) during the final 90 days of life.

Conclusion: A facilitated ACP intervention among patients with severe respiratory disease did not have an impact on satisfaction, health-related quality of life, or 12-month mortality rate. Facilitated ACP may be associated with a different type of health care utilization during the end-of-life period.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpainsymman.2019.11.018DOI Listing
April 2020

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

Nutrients 2019 Nov 21;11(12). Epub 2019 Nov 21.

Dietetic Department, Birmingham Women's and Children's Hospital NHS Foundation Trust, Birmingham B4 6NH, UK.

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 ( < 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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu11122857DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6950038PMC
November 2019

Investigation of paediatric PKU breath malodour, comparing glycomacropeptide with phenylalanine free L-amino acid supplements.

J Breath Res 2019 10 21;14(1):016001. Epub 2019 Oct 21.

School of Engineering, University of Warwick, Coventry CV4 7AL, United Kingdom.

In clinical practice, caregivers of children with phenylketonuria (PKU) report that their children have breath malodour. This might be linked to the regular consumption of low phenylalanine (Phe)/Phe-free protein substitutes (PS), which are an essential component of a low-Phe diet. Oral malodour can negatively affect interpersonal communication, lead to bullying, low self-esteem and social isolation. In this longitudinal cross-over study, exhaled volatile organic compounds (VOCs) were measured using gas chromatography-ion mobility spectrometry. 40 children (20 PKU, 20 controls) were recruited. Subjects with PKU took either L-Amino Acid (L-AA) or Casein Glycomacropeptide (CGMP-AA) exclusively for 1 week, in a randomised order. On the seventh day, seven exhaled breath samples were collected over a 10 h period. Subjects then transferred to the other PS for a week and on day seven, provided seven further breath samples. All subjects had a standardised menu using low-Phe food alternatives and all food intake was measured and recorded. In the PKU group, the aim was to collect samples 30 min after consuming PS. In 3 subjects, breath was collected 5 min post-PS consumption. Fasted L-AA and CGMP-AA breath samples contained a similar number of VOC peaks (10-12) as controls. Longitudinal breath testing results demonstrate that there was no significant difference in the number of exhaled VOCs, comparing L-AA or CGMP-AA with controls, or between PS (12-18 VOC peaks). Breath analysed immediately after consumption of PS (n = 3) showed an immediate increase in the number of VOC peaks (25-30), but these were no longer detectable at 30 min post-consumption. This suggests PS have a transient effect on exhaled breath. Measurements taken 30 min after consuming L-AA or CGMP-AA were not significantly different to controls. This indicates that timing food and drinks with PS consumption may be a potential solution for carers to reduce or eliminate unpleasant PS-related breath odours.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1088/1752-7163/ab4097DOI Listing
October 2019

Feedback learning opportunities from medical student logs of paediatric patients.

BMC Med Educ 2019 Apr 11;19(1):107. Epub 2019 Apr 11.

The Rural Clinical School of Western Australia, Faculty of Health and Medical Sciences, University of Western Australia, M706, 35 Stirling Highway, Crawley, WA, 6009, Australia.

Background: Feedback can alter medical student logging practices, although most learners feel feedback is inadequate. A varied case mix in rural and urban contexts offers diverse clinical encounters. Logs are an indicator of these clinical experiences, and contain opportunities for feedback, which can greatly influence learning: we labelled these 'feedback learning opportunities' (FLOs). We asked: How often do FLOs occur? What are the case complexities of rural compared to urban paediatric logs? Do more complex cases result in more FLOs?

Methods: In Western Australia, 25% of medical students are dispersed in a Rural Clinical School (RCSWA) up to 2175 miles (3500 km) from the city. Urban students logged 20 written cases; rural students logged a minimum of 25 paediatric cases electronically. These were reviewed to identify FLOs, using a coding convention. FLO categories provided a structure for feedback: medical, professionalism, insufficient, clinical reasoning, student wellbeing, quality and safety, and sociocultural. Each log was assigned an overall primary, secondary or tertiary case complexity.

Results: There were 76 consenting students in each urban and rural group, providing 3034 logs for analysis after exclusions. FLOs occurred in more than half the logs, with significantly more rural (OR 1.35 95% CI 1.17, 1.56; p < 0.0001). Major FLOs occurred in over a third of logs, but with no significant difference between rural and urban (OR 1.10 95% CI 0.94, 1.28; p = 0.24). Medical FLOs were the most common, accounting for 64.0% of rural and 75.2% of urban FLOs (OR 1.71 95% CI 1.37, 2.12; p < 0.0001). Students logged cases with a variety of complexities. Most cases logged by urban students in a tertiary healthcare setting were of primary and secondary complexity. Major medical FLOs increased with increasing patient complexity, occurring in 32.1% of tertiary complexity cases logged by urban students (p < 0.001).

Conclusions: Case logs are a valuable resource for medical educators to enhance students' learning by providing meaningful feedback. FLOs occurred often, particularly in paediatric cases with multiple medical problems. This study strengthens recommendations for regular review and timely feedback on student logs. We recommend the FLOs categories as a framework for medical educators to identify FLOs.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12909-019-1533-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6460648PMC
April 2019

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.

Nutrients 2019 Mar 5;11(3). Epub 2019 Mar 5.

Birmingham Women's and Children's Hospital NHS Foundation Trust, Birmingham B4 6NH, UK.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu11030552DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6471165PMC
March 2019

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

Nutrients 2019 Feb 28;11(3). Epub 2019 Feb 28.

Birmingham Women's and Children's Hospital NHS Foundation Trust, Birmingham B4 6NH, UK.

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 ( = 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 ( = 0.0005), burping ( = 0.001), retching ( = 0.03); and less regurgitation ( = 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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu11030529DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6470524PMC
February 2019
-->