Search

Background & aims: \ud Obesity prevalence in people with phenylketonuria (PKU) is comparable to that of the general population but the underlying aetiology remains unknown. To assess body composition, dietary intake, moderate physical activity duration (MPAD) and energy expenditure (MPAEE), resting metabolic rate (RMR), diet-induced thermogenesis (DIT), fasting and postprandial fat (FOx) and carbohydrate oxidation (CHOOx), in PKU people and healthy Controls.\ud \ud Methods: \ud Participants were PKU people (n = 16) and healthy controls (n = 15). Body composition was measured with stable isotopes using deuterium as tracer, dietary intake from 4-day food diaries, MPAD and MPAEE from 7-day activity counts measured by triaxial accelerometers, calibrated against individual rates of oxygen consumption and carbon dioxide production, RMR, DIT, FOx and CHOOx by indirect calorimetry.\ud \ud Results: \ud Body composition, DIT, FOx, CHOOx and RMR did not differ between the PKU and the Control groups. MPAD (PKU, 73 ± 26 min/week; Control, 152 ± 43 min/week) and MPAEE (PKU, 404 ± 127 kcal/week; Control, 741 ± 153 kcal/week) were lower (P < 0.05) in the PKU than the Control group. Raised phenylalanine levels were inversely related with MPAD and MPAEE. Energy intake and energy provided by protein did not differ between the groups, while energy proportion obtained from carbohydrate was higher (PKU, 60 ± 2%; Control, 51 ± 2%; P < 0.05) and from fat lower (PKU, 24 ± 2%; Control, 35 ± 3%; P < 0.05) in the PKU than in the Control group.\ud \ud Conclusion: \ud People with PKU spent less time and expend less energy in moderate physical activity and have a higher intake of energy from CHO which may be involved in the underlying mechanisms of obesity in PKU.

Tourist activity is characterized by its variable and varied nature, as it is a human activity that includes various features that constantly need to be satisfied through the different types of tourism. Meanwhile, the global transformations and developments that occurred during the past two decades have led to an increasing demand for more specialized types of tourism, among which, is medical tourism. The importance of the foreign market to the destination country has attracted scholars' interest in understanding the motivations of tourists to travel abroad. The success of marketing destinations must be guided by a comprehensive analysis of tourists ’motivations, which is why this study adopts a model that relies on push and pull factors as a conceptual framework. Whereas, the push factors is that a person desires to travel and they are mainly internal psychological motives while pull factors is an external factor that affects a person's travel to satisfy a need or desire. A person will choose one destination that meets his needs and desires. Since little information has been documented about the motives for travel to Egypt, this study attempts to determine the motivation factors for foreign tourists toward Egypt as different tourism markets show different areas of behavior. This may have important marketing implications for the Egypt especially with regard to examining drivers in segmenting markets, designing promotional programs and deciding on destination development. Despite the already published work around medical tourism in the last two decades, there continues to be, firstly, a jungle of similar and mixed concepts (most of the previous studies have analyzed different aspects of this type of tourism, mainly medical tourism, without providing a clear and integrated framework). And, secondly, an area of scarcity of research on mature destinations (mostly African countries have received the attention).

Purpose- This study examines the effect of international patient motivators to travel (push factors and pull factors) on international patient intention to visit Egypt as medical tourism destination. It proposes a model which examines the relationship between push and pull factors and patient intention toward Egypt as a medical tourism destination. Also, this study presents an integrated approach to understanding the tourist's motivations and attempts to expand the empirical evidence on the relationships between push and pull motives in order to determine the extent to which these motivating factors will contribute to the overall perception of a destination Findings- The research findings reveal that; push factors have a positive impact on intention of patient except for rest and relaxation which has negative impact, although, rest and relaxation is the most effective push factor. Also, both pull factors (healthcare provider specific, destination specific) have positive impact on intention of patient. While, destination specific is the most effective pull factors. More specifically, the destination specific can be arranged as following: Environment and safety, Culture and historical attractions, then the tourism facilities. Moreover, the Healthcare provider service factors can be arranged as following: the Service quality, Waiting time, and Medical cost. Also, there are significant positive relationships between pull factors on push factors and vies versa with the same strength. Finally, the results revealed three push and six pull factor dimensions and the results of the correlation analysis indicate that significant relationships were found between the three push and the six pull factor dimensions

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 (&ge, 75%) decision. A mean of 29 UK dietitians (range: 18&ndash, 40) and 18 treatment centres (range: 13&ndash, 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 &le, 75 mg/100 g and most foods containing protein &le, 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: &gt, 0.1 g/100 g (milk/plant milks only), &gt, 0.5 g/100 g (bread/pasta/cereal/flours), &gt, 1 g/100 g (cook-in/table-top sauces/dressings), &gt, 1.5 g/100 g (soya sauces)), and (4) fruit/vegetables containing phenylalanine &gt, 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).

Background In the treatment of phenylketonuria (PKU), there was disparity between UK dietitians regarding interpretation of how different foods should be allocated in a low phenylalanine diet (allowed without measurement, not allowed, or allowed as part of phenylalanine exchanges). This led to variable advice being given to patients. Methodology In 2015, British Inherited Metabolic Disease Group (BIMDG) dietitians (n = 70) were sent a multiple-choice questionnaire on the interpretation of protein from food-labels and the allocation of different foods. Based on majority responses, 16 statements were developed. Over 18-months, using Delphi methodology, these statements were systematically reviewed and refined with a facilitator recording discussion until a clear majority was attained for each statement. In Phase 2 and 3 a further 7 statements were added. Results The statements incorporated controversial dietary topics including: a practical ‘scale’ for guiding calculation of protein from food-labels; a general definition for exchange-free foods; and guidance for specific foods. Responses were divided into paediatric and adult groups. Initially, there was majority consensus (≥86%) by paediatric dietitians (n = 29) for 14 of 16 statements; a further 2 structured discussions were required for 2 statements, with a final majority consensus of 72% (n = 26/36) and 64% (n = 16/25). In adult practice, 75% of dietitians agreed with all initial statements for adult patients and 40% advocated separate maternal-PKU guidelines. In Phase 2, 5 of 6 statements were agreed by ≥76% of respondents with one statement requiring a further round of discussion resulting in 2 agreed statements with a consensus of ≥71% by dietitians in both paediatric and adult practice. In Phase 3 one statement was added to elaborate further on an initial statement, and this received 94% acceptance by respondents. Statements were endorsed by the UK National Society for PKU. Conclusions The BIMDG dietitians group have developed consensus dietetic statements that aim to harmonise dietary advice given to patients with PKU across the UK, but monitoring of statement adherence by health professionals and patients is required.

The nutritional and metabolic characteristics of adult phenylketonuria (PKU) patients in the UK with varying dietary adherence is unknown. In other countries, nutritional and metabolic abnormalities have been reported in nonadherent patients compared to adherent counterparts. A pooled analysis of primary baseline data from two UK multi-centre studies was therefore performed to establish whether this is true from a UK perspective. Adult PKU patients who had provided 3-day food records and amino acid blood samples were included and grouped according to dietary adherence (adherent, n = 16 vs. nonadherent, n = 14). Nonadherent patients consumed greater amounts of natural protein compared to adherent patients (61.6 &plusmn, 30.7 vs. 18.3 &plusmn, 7.7 g/day, q &lt, 0.001). In contrast, the contribution of protein substitutes to total protein intake was lower in nonadherent compared to adherent patients (3.9 &plusmn, 9.2 g/day vs. 58.6 &plusmn, 10.2 g/day, 0.001). Intakes of iron, zinc, vitamin D3, magnesium, calcium, selenium, iodine, vitamin C, vitamin A and copper were significantly lower in nonadherent compared to adherent patients and were below UK Reference Nutrient Intakes. Similarly, intakes of thiamin, riboflavin, niacin, vitamin B6 and phosphorus were significantly lower in nonadherent compared to adherent patients but met the UK Reference Nutrient Intakes. Phenylalanine concentrations in nonadherent patients were significantly higher than adherent patients (861 &plusmn, 348 vs. 464 &plusmn, 196 &micro, mol/L, q=0.040) and fell outside of European treatment target ranges. This study shows the nutritional and metabolic consequences of deviation from phenylalanine restriction and intake of PKU protein substitutes in nonadherent adult PKU patients. Collectively, these data further underlie the importance of life-long adherence to the PKU diet.

Noncompliance is widespread in adults with PKU and is associated with adverse metabolic, nutritional and cognitive abnormalities. Returning to the PKU diet is important for this at-risk population, yet for many this is challenging to achieve. Strategies that ease the return to the PKU diet, while offering nutritional and cognitive advantages, are needed. Twelve PKU adults (33.7 &plusmn, 2.6 years), who had been noncompliant for 4.5 years (range: 1 to 11 years), took 33 g of a low-volume, nutrient-enriched, protein substitute daily for 28 days. Outcomes of eating behaviour, nutrient intake and mood were assessed at entry (baseline, days 1&ndash, 3) and after the intervention period (days 29&ndash, 31). At baseline, intakes of natural protein and estimated phenylalanine were high (66.4 g and 3318.5 mg, respectively) and intakes of calcium, magnesium, iron, zinc, iodine and vitamin D were below country-specific recommendations. With use of the experimental protein substitute, natural protein and estimated phenylalanine intake declined (p = 0.043 for both). Fat and saturated fat intakes also decreased (p = 0.019 and p = 0.041, respectively), while energy and carbohydrate intake remained unchanged. Micronutrient intake increased (p &le, 0.05 for all aforementioned) to levels well within reference nutrient intake recommendations. Blood vitamin B12 and vitamin D increased by 19.8% and 10.4%, respectively. Reductions in anxiety and confusion were also observed during the course of the study yet should be handled as preliminary data. This study demonstrates that reintroducing a low-volume, nutrient-enriched protein substitute delivers favourable nutritional and possible mood benefits in noncompliant PKU patients, yet longer-term studies are needed to further confirm this. This preliminary knowledge should be used in the design of new strategies to better facilitate patients&rsquo, return to the PKU diet, with the approach described here as a foundation.

Uterine fibroids are the most common gynecologic tumors with a significant medical and financial burden. Several genetic, hormonal, and biological factors have been shown to contribute to the development and growth of fibroid tumors. Of these factors, estrogen is particularly critical since fibroids are considered estrogen dependent because no prepubertal cases have been described in the literature and tumors tend to regress after menopause. Understanding the role of estrogen in fibroids is not only important for understanding the pathobiology of fibroids but also for the development of successful therapeutics. In this review, we discuss the types and structure of estrogen receptors (nuclear and membrane bound, including α and β receptors and G protein-coupled estrogen receptor 1 GPER1). Estrogen-signaling pathways in fibroids include genomic (direct and indirect) and nongenomic including Ras-Raf-MEK (MAPK/Erk Kinase)-mitogen-activated protein kinase (MAPK) and phosphatidylinositide 3-kinase (PI3K)-phosphatidylinositol-3,4,5-trisphosphate (PIP3)-Akt (Protein kinase B)-mammalian target of rapamycin (mTOR) pathways; shortly Ras-Raf-MEK-MAPK and PI3K-PIP3-Akt-mTOR pathways. Several aberrations in estrogen receptors and signaling pathways are implicated in fibroid pathobiology. Current therapeutic and research agents targeting ERs/signaling include gonadotropin-releasing hormone (GnRH) agonists, GnRH antagonists, aromatase inhibitors, selective ER modulators, gene therapy, and others. Future research can identify potential targets for the development of novel treatments. In particular, epigenomics of estrogen activity and individualized (precision) medicine appear to be attractive areas for future research.