Phenylketonuria, co-morbidity, and ageing: A review.

Phenylketonuria (PKU) is a metabolic condition which, left untreated, results in severe and irreversible brain damage. Newborn screening and the development of the low phenylalanine (Phe) diet have transformed the outcomes for people with PKU. Those who have benefited from early treatment are now approaching their fifth and sixth decade. It is therefore timely to consider multi-morbidity in PKU and the effects of ageing, in parallel with the wider benefits of emerging treatment options in addition to dietary relaxation. We have conducted the first literature review of co-morbidity and ageing in the context of PKU. Avenues explored have emerged from limited study of multi-morbidity to date and the knowledge and critical enquiry of the authors. Findings suggest PKU to have a wider impact than brain development, and result in several intriguing questions that require investigation to attain the best outcomes for people with PKU in adulthood moving through to older age. We recognise the difficulty in studying longitudinal outcomes in rare diseases and emphasise the necessity to develop PKU registries and cohorts that facilitate well-designed studies to answer some of the questions raised in this review. Whilst awaiting new information in these areas we propose that clinicians engage with patients to make personalised and well-informed decisions around Phe control and assessment for co-morbidity.

Nutrition, Health, and Regulatory Aspects of Digestible Maltodextrins.

Digestible maltodextrins are low-sweet saccharide polymers consisting of D-glucose units linked primarily linearly with alpha-1,4 bonds, but can also have a branched structure through alpha-1,6 bonds. Often, maltodextrins are classified by the amount of reducing sugars present relative to the total carbohydrate content; between 3 and 20 percent in the case of digestible maltodextrins. These relatively small polymers are used as food ingredients derived by hydrolysis from crops naturally rich in starch. Through advances in production technology, the application possibilities in food products have improved during the last 20 years. However, since glucose from digested maltodextrins is rapidly absorbed in the small intestine, the increased use has raised questions about potential effects on metabolism and health. Therefore, up-to-date knowledge concerning production, digestion, absorption, and metabolism of maltodextrins, including potential effects on health, were reviewed. Exchanging unprocessed starch with maltodextrins may lead to an increased glycemic load and therefore post meal glycaemia, which are viewed as less desirable for health. Apart from beneficial food technological properties, its use should accordingly also be viewed in light of this. Finally, this review reflects on regulatory aspects, which differ significantly in Europe and the United States, and, therefore, have implications for communication and marketing.

Symptom's resolution and growth outcome of children with cow's milk protein allergy consuming two hydrolyzed formulas: A retrospective study in Mexico.

Background: Cow's milk protein allergy (CMPA) is the leading cause of food allergy in infants and young children. An extensively hydrolyzed formula (eHF) is the first choice of dietary management, however, not all of them have similar peptide profiles and degree of hydrolysis. The aim of this retrospective study was to investigate the use of two commercially available infant formulas in the clinical management of CMPA in Mexico in terms of symptoms' resolution and growth trajectories. Methods: Medical records of 79 subjects from four sites in Mexico were included to retrospectively evaluate the trajectory of atopic dermatitis, other symptoms of cow's milk protein allergy and growth outcomes. The study formulas were based on hydrolyzed whey protein (eHF-W) and hydrolyzed casein protein (eHF-C). Results: Medical records of 79 patients were enrolled, 3 were excluded from analysis based on previous formula consumption. Seventy-six children with confirmed CMPA based on skin prick test and/or serum specific IgE levels were included in the analysis. 82% of patients (n = 65) consumed the eHF-C, reflecting the doctors' preference for formulas with a higher grade of hydrolysis and the high incidence of positive reactions to beta-lactoglobulin amongst subjects. During their first visit to the doctors, 55% of the subjects consuming the casein-based formula and 45% of subjects consuming the whey-based formula presented with mild or moderate dermatological symptoms. Other frequently reported symptoms included respiratory issues, enteropathies and colitis which improved during the consumption of both formulas. All CMPA-related symptoms showed improvement during formula consumption. During the period of retrospective observation, growth significantly improved for both groups. Conclusion: Consumption of eHF-C and eHF-W effectively improved symptoms' resolution and growth outcomes among children with CMPA in Mexico. More preference was reported towards eHF-C due to its hydrolysate profile and lack of b-lactoglobulin. Trial registration: The study was registered at ClinicalTrials.gov: NCT04596059.