Nutritional Quality and Degree of Processing of Children's Foods Assessment on the French Market.

Food packaging marketing techniques which appeal to children (such as cartoon characters and brand mascots) affect children's choices, preferences, and eating habits. Several studies have assessed the nutritional quality of food intended to children in various countries and concluded that most were high in fat, salt, and sugar (HFSS) and ultra-processed foods. The aim of this study is to analyse products intended for children over the age of 3 (foods and beverages with relevant marketing elements on the packaging) available on the French market as regards: (1) nutritional quality, based on the Nutri-Score labelling system, (2) compliance with expected nutritional profile suitable for children, according to the criteria of the WHO Europe Nutrient Profile Model, and (3) degree of processing, as defined by the NOVA classification, from packaging collected in 20 stores (hyper/supermarkets, hard-discount retail chains, and organic food stores). The marketing strategies most often used on children's products are cartoons (97.22%; n = 1120) and mascots (77.78%; n = 896). A total of 1155 products were included in the study, most of which were sugary foods: almost a quarter of the products in the sample (23.81%; n = 275) list a sweetener as the first ingredient, and most of them (89.52%; n = 1034) contain free sugars according to the WHO definition. All the products included in our study feature marketing elements targeting on the packaging, yet 94.88% do not meet the criteria of the WHO Europe Nutrient Profile Model. Most (58.68%; n = 676) belong to Nutri-Score groups D and E, with the highest proportion in group D (39.32%; n = 453) and are ultra-processed (87.97%; n = 1016), especially through the use of flavourings and ultra-processed sugars. Using the Nutri-Score, the WHO Europe Nutrient Profile Model, and the NOVA classification, this study suggests that a significant share of pre-packaged foods marketed to children do not have an adequate nutritional profile. As such, measures are needed to regulate what marketing elements aimed at children can be included on packaging, based on these criteria.

Dietary practices in methylmalonic acidaemia: a European survey.

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.

A series of three case reports in patients with phenylketonuria performing regular exercise: first steps in dietary adjustment.

Background Phenylketonuria (PKU), a rare, inherited metabolic condition, is treated with a strict low-phenylalanine (Phe) diet, supplemented with Phe-free protein substitute. The optimal nutritional management of a sporting individual with PKU has not been described. Therefore, guidelines for the general athlete have to be adapted. Case presentation Three clinical scenarios of sporting patients with PKU are given, illustrating dietary adaptations to usual management and challenges to attain optimal sporting performance. Therefore, the main objectives of sports nutrition in PKU are to (1) maintain a high carbohydrate diet; (2) carefully monitor hydration status; and (3) give attention to the timing of protein substitute intake in the immediate post-exercise recovery phase. Optimal energy intake should be given prior to, during and post exercise training sessions or competition. Fortunately, a usual low-Phe diet is rich in carbohydrate, but attention is required on the types of special low-protein foods chosen. Acute exercise does not seem to influence blood Phe concentrations, but further evidence is needed. Summary Well-treated PKU patients should be able to participate in sports activities, but this is associated with increased nutritional requirements and dietary adjustments. Conclusions It should be the goal of all sporting patients with PKU to maintain good metabolic Phe control and attain maximal athletic performance.

Angiotensin converting enzyme inhibitor intoxication: Naloxone to the rescue? Naloxone for ACE inhibitor intoxication.

We report here the case of a patient with perindopril intoxication inducing severe bradycardia together with a profound hypotension. Initiation of a naloxone infusion completely resolved those symptoms. As a consequence, we could recommend as a first step the use of naloxone in order to prevent the use of more invasive therapeutic tools.

Evaluation of nutritional care of hospitalized children in a tertiary pediatric hospital.

BACKGROUND AND AIMS: Hospitalized children are at risk of malnutrition. The aim of the present study was to evaluate a clinical practice in a tertiary hospital. The nutritional team developed a specific software for screening of malnutrition and risk of malnutrition (Evalnut) that provides also recommendations for the nutritional management of the patient. The data recorded into this program and the tool itself were analyzed and optimizations are highlighted. METHODS: A retrospective study analyzed the data collected in 2015 during 4931 consecutive hospitalizations (3984 children) at the University Children's Hospital Queen Fabiola. Pivot tables analysis (Excel) of the database of the screening tool was compared with the clinical practice of the dietitians. First data processing excluded records with abnormal or missing values. Impact of nutritional care analysis needs at least 2 evaluations and a positive patient's height trend. In case of height equality, only length of hospital stays less than 2 weeks were kept. RESULTS: This study highlighted inaccurate database records related to imperfections of the computer program, missing or erroneous measures and incomplete encoding. First analysis on 3219 valid hospitalizations showed statistical correlations. Prevalence of malnutrition on admission was 33%, split into 14,5% acute malnutrition, 15% chronic malnutrition and 3,5% mixed malnutrition. Overall, 30,3% of the children were categorized at risk of developing malnutrition during their stay. Positive impact of nutritional management on the resulting nutritional status was demonstrated on the second data selection (352 hospitalizations): WFH median (interquartile range) increased from 96,1% (87,1-106,4) on admission to 96,9% (89,1-106,1) (p < 0,01) on discharge. An optimization of the existing software was finally proposed. CONCLUSION: In our hospital, the dietitians are the most aware on the importance of nutritional assessment and management during hospitalization. Encouraging results are obtained. Inclusion of a nutritional program in the medical file is useful to raise interest amongst caregivers and is particularly valuable for the nutritional follow up of the patients by the nutrition team.

Weight Management in Phenylketonuria: What Should Be Monitored.

BACKGROUND: Severe intellectual disability and growth impairment have been overcome by the success of early and continuous treatment of patients with phenylketonuria (PKU). However, there are some reports of obesity, particularly in women, suggesting that this may be an important comorbidity in PKU. It is becoming evident that in addition to acceptable blood phenylalanine control, metabolic dieticians should regard weight management as part of routine clinical practice. SUMMARY: It is important for practitioners to differentiate the 3 levels for overweight interpretation: anthropometry, body composition and frequency and severity of associated metabolic comorbidities. The main objectives of this review are to suggest proposals for the minimal standard and gold standard for the assessment of weight management in PKU. While the former aims to underline the importance of nutritional status evaluation in every specialized clinic, the second objective is important in establishing an understanding of the breadth of overweight and obesity in PKU in Europe. KEY MESSAGES: In PKU, the importance of adopting a European nutritional management strategy on weight management is highlighted in order to optimize long-term health outcomes in patients with PKU.

Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile.

BACKGROUND: Special low protein foods (SLPF) are essential in the nutritional management of patients with phenylketonuria (PKU). The study objectives were to: 1) identify the number of SLPF available for use in eight European countries and Turkey and 2) analyse the nutritional composition of SLPF available in one of these countries. METHODS: European Nutritionist Expert Panel on PKU (ENEP) members (Portugal, Spain, Belgium, Italy, Germany, Netherlands, UK, Denmark and Turkey) provided data on SPLF available in each country. The nutritional composition of Portuguese SLPF was compared with regular food products. RESULTS: The number of different SLPF available in each country varied widely with a median of 107 [ranging from 73 (Portugal) and 256 (Italy)]. Food analysis of SLPF available from a single country (Portugal) indicated that the mean phenylalanine content was higher in low protein baby cereals (mean 48 mg/100 g) and chocolate/energy bars/jelly (mean 41 mg/100 g). The energy content of different foods from a sub-group of SLPF (cookies) varied widely between 23 and 96 kcal/cookie. Low protein bread had a high fat content [mean 5.8 g/100 g (range 3.7 to 10)] compared with 1.6 g/100 g in regular bread. Seven of the 12 SLPF sub-groups (58 %) did not declare any vitamin content, and only 4 (33 %) identified a limited number of minerals. CONCLUSIONS: Whilst equal and free access to all SLPF is desirable, the widely variable nutritional composition requires careful nutritional knowledge of all products when prescribed for individual patients with PKU. There is a need for more specific nutritional standards for special low protein foods.

Consensus on the guidelines for the dietary management of classical galactosemia.

BACKGROUND AND AIM: Worldwide there is scientific discussion about the dietary management of galactosemia. The dietary management is very different in several countries among Europe, the US and Canada. The main points of discussion are related to the fact that i) despite a strict diet some patients still have poor outcomes; ii) there is lack of scientific knowledge about the role of endogenous production of galactose on disease evolution, with or without diet. The aim of the current work was the creation of a Belgian consensus on dietary guidelines for the management of galactosemia. METHODS: A step-wise approach was used to achieve a consensus, including: a workshop, a Delphi round, discussion groups and a round table of different Belgian experts. RESULTS: The consensus is an agreement between strict guidelines (strict limitation of fruits, vegetables and soybean products/French guidelines) and the more liberal guidelines (comparable with a diet free of lactose/guidelines of UK and the Netherlands). The consensus document consists of different modules, including the medical context, the theoretical background of dietary guidelines and the age-specific practical dietary guidelines. CONCLUSION: A Belgian consensus on the guidelines for the dietary management of classical galactosemia was developed despite the uncertainties of the efficacy and practical application of these guidelines. The final consensus is based on scientific knowledge and practical agreement among experts. In the future, regular revision of the guidelines is recommended and a uniform European guideline is desirable.

When should social service referral be considered in phenylketonuria?

Lifelong low-phenylalanine (Phe) dietary management is the foundation of care in phenylketonuria (PKU). However, strict monitoring of food intake places a burden on patients and their caregivers, and adherence to the required diet frequently decreases in later childhood and adolescence. Rarely, parents of children with PKU refuse to recognise the importance of treatment and follow-up for this chronic condition. Here, two case studies are presented that document consideration of placement of children into foster care or kinship homes as a last resort to improve persistently high Phe concentrations. In the first case, social service referral led to a 3-year-old girl being placed in a kinship home with her grandparents, resulting in excellent Phe control thereafter. In the second case, discussion with the parents of possible placement of a 12-year-old child into foster care was sufficient to have a positive effect on Phe control. A staged approach for managing intractable non-adherence in PKU is proposed.

A Belgian consensus-statement on growing-up milks for children 12-36 months old.

UNLABELLED: Growing-up milks (GUM) are milk-based drinks with low protein and added minerals and vitamins intended for children 12-36 months. Since the advantages of GUM are heavily debated, we reviewed the literature. A literature search was done using the classic databases (Pubmed, Embase, Cochrane) on the use of GUM in 12- to 36-month-old young children. Only limited data are available. GUM have a highly variable composition as their marketing is not regulated. Nevertheless, all papers conclude that GUM help to cover nutritional requirements of 12- to 36-month-old infants. CONCLUSION: Appropriate intakes of macro- and micronutrients in 1- to 3-year-old children have long-term health benefits. Present diets offered to toddlers do in general not meet the requirements. Supplemented foods are therefore helpful, of which GUM is a possibility.

Optimising growth in phenylketonuria: current state of the clinical evidence base.

Patients with phenylketonuria (PKU) must follow a strict low-phenylalanine (Phe) diet in order to minimise the potentially disabling neuropsychological sequelae of the disorder. Research in this area has unsurprisingly focussed largely on managing blood Phe concentrations to protect the brain. Protein requirements in dietary management of PKU are met mostly from Phe-free protein substitutes with the intake of natural protein restricted to patient tolerance. Several reports have suggested that growth in early childhood in PKU is sub-optimal, relative to non-PKU control groups or reference populations. We reviewed the literature searching for evidence regarding PKU and growth as well as possible links between dietary management of PKU and growth. The search retrieved only limited evidence on the effect of PKU and its dietary management on growth. Physical development in PKU remains an under-studied aspect of this disorder.

A pilot randomized trial of a smoking cessation nursing intervention in cardiac patients after hospital discharge.

BACKGROUND: One fifth of Canadians are smokers despite the availability of community-based smoking cessation programs. It was hypothesized that offering a post-discharge smoking cessation program to cardiac patients would decrease smoking rates at six months. METHOD: This pilot randomized study explored the feasibility, acceptability and preliminary efficacy of a smoking cessation intervention delivered by a smoking cessation nurse specialist (SCNS) to cardiac patients after hospital discharge. SAMPLE: Participants (N=40) were randomized to either a postdischarge telephone intervention delivered weekly for the first month and then monthly until the third month (experimental group [EG]), or referral to usual community care (control group [CG]). FINDINGS: The researchers confirmed the feasibility of recruitment and acceptability of the intervention, but dfficulty with follow-up. The intention-to-treat analysis showed similar smoking cessation rates in both groups at six months (25% EG versus 30% CG; p = 0.72). CONCLUSION: An intensifed follow-up protocol, or a more intensive, comprehensive and multidisciplinary intervention might be required, given the characteristics of the smokers.

[A pre assessment for nursing intervention to support tobacco cessation in patients hospitalized for cardiac problems: a pilot study (So-Live)]. / Evaluation préliminaire d'une intervention infirmière de soutien a la cessation tabagique chez des patients hospitalisés pour un problème cardiaque: etude pilote (So-Live).

OBJECTIVE: The aim of this study was to evaluate the effect of a smoking cessation intervention provided after discharge from a specialized cardiac hospital. DESIGN: A randomized pilot study (N = 40); after discharge, the experimental group (EG) received 6 phone calls from a nurse specialized in tobacco cessation counselling. RESULTS: Patients in the EG showed improved scores on two aspects of illness representations (perceive their illness as chronic and reported less negative emotional representations). No significant difference in smoking cessation was observed at 6 months (p = 0.72). CONCLUSION: The non-significant difference may be explained in part by the smoking characteristics within this sample exemplifying the more nicotine dependent "hard core" smokers who persist in their smoking habits despite the serious health consequences incurred by continued smoking. This population of smokers may require a more intensive, specialized intervention to achieve smoking cessation.

Adjusting diet with sapropterin in phenylketonuria: what factors should be considered?

The usual treatment for phenylketonuria (PKU) is a phenylalanine-restricted diet. Following this diet is challenging, and long-term adherence (and hence metabolic control) is commonly poor. Patients with PKU (usually, but not exclusively, with a relatively mild form of the disorder) who are responsive to treatment with pharmacological doses of tetrahydrobiopterin (BH4) have either lower concentrations of blood phenylalanine or improved dietary phenylalanine tolerance. The availability of a registered formulation of BH4 (sapropterin dihydrochloride, Kuvan®) has raised many practical issues and new questions in the dietary management of these patients. Initially, patients and carers must understand clearly the likely benefits (and limitations) of sapropterin therapy. A minority of patients who respond to sapropterin are able to discontinue the phenylalanine-restricted diet completely, while others are able to relax the diet to some extent. Care is required when altering the phenylalanine-restricted diet, as this may have unintended nutritional consequences and must be undertaken with caution. New clinical protocols are required for managing any dietary change while maintaining control of blood phenylalanine, ensuring adequate nutrition and preventing nutritional deficiencies, overweight or obesity. An accurate initial evaluation of pre-sapropterin phenylalanine tolerance is essential, and the desired outcome from treatment with sapropterin (e.g. reduction in blood phenylalanine or relaxation in diet) must also be understood by the patient and carers from the outset. Continuing education and support will be required thereafter, with further adjustment of diet and sapropterin dosage as a young patient grows.

Dietary management practices in phenylketonuria across European centres.

BACKGROUND: Dietary phenylalanine restriction is the cornerstone of phenylketonuria (PKU) management. However, there are no European consensus guidelines for its optimal dietary care. METHODS: Detailed information on the routine dietary management of PKU was obtained from 10 European centres using structured questionnaires. Each centre was represented by one dietitian/nutritionist or physician (European Nutritionist Expert Panel). RESULTS: All centres screened for PKU within the first 10 days of life. PKU prevalence was highest in Turkey. The training, roles and responsibilities of dietitians and nutritionists varied widely; in some centres dietitians were responsible for managing the diet, while in others this was performed by a physician. There were marked differences in target blood phenylalanine concentrations, the dosages of protein substitutes, systems for allocating daily phenylalanine allowance, and the definition of foods that could be eaten without restriction ('free foods'). Eighty percent (n=8/10) of centres encouraged breastfeeding together with protein substitute in infants with PKU. CONCLUSIONS: Important differences exist among centres across Europe in the dietary management of PKU, and in support systems designed to assist patients in managing their diets. Further studies are needed to compare different dietary treatments with the aim of identifying best practice to optimise phenylalanine control and dietary adherence.