BackgroundIn infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe.MethodsWe sent a cross sectional, survey monkey® questionnaire to European health professionals working in IMD. It contained 31 open and multiple-choice questions. The results were analysed according to different geographical regions.ResultsNinety-five centres from 21 countries responded. Over 60% of centres commenced diet in infants by age 10 days, with 58% of centres implementing newborn screening by day 3 post birth. At diagnosis, infant hospital admission occurred in 61% of metabolic centres, mainly in Eastern, Western and Southern Europe. Breastfeeding fell sharply following diagnosis with only 30% of women still breast feeding at 6 months.53% of centres gave pre-measured Phe-free infant formula before each breast feed and 23% alternated breast feeds with Phe-free infant formula. With standard infant formula feeds, measured amounts were followed by Phe-free infant formula to satiety in 37% of centres (n = 35/95), whereas 44% (n = 42/95) advised mixing both formulas together. Weaning commenced between 17 and 26 weeks in 85% centres, ≥26 weeks in 12% and < 17 weeks in 3%.DiscussionThis is the largest European survey completed on PKU infant feeding practices. It is evident that practices varied widely across Europe, and the practicalities of infant feeding in PKU received little focus in the PKU European Guidelines (2017). There are few reports comparing different feeding techniques with blood Phe control, Phe fluctuations and growth. Controlled prospective studies are necessary to assess how different infant feeding practices may influence longer term feeding development.
BackgroundIn phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. Our aim was to assess different weaning approaches used by health professionals across Europe.MethodsA cross sectional questionnaire (survey monkey®) composed of 31 multiple and single choice questions was sent to European colleagues caring for inherited metabolic disorders (IMD). Centres were grouped into geographical regions for analysis.ResultsWeaning started at 17–26 weeks in 85% (n = 81/95) of centres, >26 weeks in 12% (n = 11/95) and < 17 weeks in 3% (n = 3/95). Infant's showing an interest in solid foods, and their age, were important determinant factors influencing weaning commencement. 51% (n = 48/95) of centres introduced Phe containing foods at 17–26 weeks and 48% (n = 46/95) at >26 weeks. First solids were mainly low Phe vegetables (59%, n = 56/95) and fruit (34%, n = 32/95).A Phe exchange system to allocate dietary Phe was used by 52% (n = 49/95) of centres predominantly from Northern and Southern Europe and 48% (n = 46/95) calculated most Phe containing food sources (all centres in Eastern Europe and the majority from Germany and Austria). Some centres used a combination of both methods.A second stage Phe-free L-amino acid supplement containing a higher protein equivalent was introduced by 41% (n = 39/95) of centres at infant age 26–36 weeks (mainly from Germany, Austria, Northern and Eastern Europe) and 37% (n = 35/95) at infant age > 1y mainly from Southern Europe. 53% (n = 50/95) of centres recommended a second stage Phe-free L-amino acid supplement in a spoonable or semi-solid form.ConclusionsWeaning strategies vary throughout European PKU centres. There is evidence to suggest that different infant weaning strategies may influence longer term adherence to the PKU diet or acceptance of Phe-free L-amino acid supplements; rendering prospective long-term studies important. It is essential to identify an effective weaning strategy that reduces caregiver burden but is associated with acceptable dietary adherence and optimal infant feeding development.
Introduction Phenylketonuria (PKU) is an inborn error of metabolism characterized by pathogenic variants of the phenylalanine hydroxylase ( PAH ) gene with a resulting accumulation of phenylalanine (Phe) to neurotoxic levels. Diagnosis of PKU in the Latvian population began in 1985 and the present study's aim was to evaluate the available data on all PKU patients in Latvia. Materials and methods The medical records of 116 - DNA sample was available in 110 patients (102 nonrelated individuals) diagnosed with PKU in Latvia were obtained. Phe concentrations were measured in dried blood spots. Genomic DNA was analyzed for pathogenic variants in the PAH gene. Biochemical data were available through follow-up visits of the 83 patients. Results In 97% of patients (99 of 102), pathogenic variants were detected on both alleles. With an occurrence of 69.6%%, the most common pathogenic variant was the severe pathogenic variant p.Arg408Trp. The available data for 83 patients revealed that metabolic control was better in younger age groups and worse in adults. Conclusion Latvia exhibits a relatively homogeneous pool of disease-causing PKU alleles with a high prevalence of the classical severe form of PKU. Dietary compliance in all patients' groups is lower than expected, especially it is poor in adult age group.
In recent years, many studies have been initiated to characterise the human gut microbiome in relation to different factors like age, lifestyle, and dietary habits. This study aimed to evaluate the impact of yoghurt intake on the gut microbiome among postmenopausal women and how overall dietary habits modulate the gut microbiome. In total, 52 participants were included in the study and two groups—a control (n = 26) and experimental group (n = 26)—were established. The study was eight weeks long. Both study groups were allowed to consume a self-selected diet, but the experimental group had to additionally consume 175 g of plain organic milk yoghurt on a daily basis for eight weeks. In addition, a series of questionnaires were completed, including a questionnaire on the subject’s sociodemographic background, health status, and lifestyle factors, as well as a food frequency questionnaire. Stool samples were collected for the analysis of the gut microbiome (both prior to and after the eight weeks of the study). Sequencing of V4-V5 regions of the 16S rRNA gene was used to determine the bacterial composition of stool samples. The dominant phylum from the gut microbiome was Firmicutes (~70% to 73%), followed by Bacteroidota (~20% to 23%). Although no significant changes in the gut microbiome were related to daily consumption of yoghurt, we report that consumption of food products like grains, grain-based products, milk and milk products, and beverages (tea, coffee) is associated with differences in the composition of the gut microbiome. Establishing nutritional strategies to shape the gut microbiome could contribute to improved health status in postmenopausal women, but further research is needed.
Objectives: Overweight and obesity has become an important worldwide health issue, that is why the risk factors for gaining excess weight are being studied a lot. Big birth weight and parental overweight are known risk factors for childhood overweight. The association between psychological issues and excess weight is bidirectional. Aim of our research was assessing if there was any association between parental weight status, birth weight or signs of depression and the exact value of already overweight child’s standardized body mass index (z-BMI). Study design: Cross-sectional study. Materials and Methods: All 303 respondents included were six to seventeen years old patients of the first weight correction programme in Baltic states. Their first day data were gathered from Children’s Clinical University Hospital electronic databases Andromeda and Saule, as well as from outpatient medical records. Height and weight data were turned into z-BMI. Depression signs had been assessed using Children Depression Inventory (by M. Kovacs, 1992). Parental weight status and child’s birth weight had also been documented. Results: From all 303 respondents 141 (47%) were boys. Median age 12 (IQR 10-15) years. The median z-BMI was significantly higher in boys than in girls (2.97 (IQR 2.59-3.37) vs. 2.59 (IQR 2.13-2.90), p<0.001). Parental weight status correlated significantly with z-BMI value in boys (r=0.17, p=0.043) and in girls (r=0.18, p=0.026). The correlation became stronger when controlled by birth weight and signs of depression: r=0.87, p=0.005 for boys; r=0.96, p<0.001 for girls. There was no significant correlation between z-BMI and either birth weight or signs of depression. Conclusions: The parental excess weight correlated significantly with the z-BMI of their son or daughter. The signs of depression and birth weight had no significant association with z-BMI.
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