LEADER 05203nam 2201309z- 450 001 9910619462903321 005 20231214133051.0 010 $a3-0365-5320-7 035 $a(CKB)5670000000391646 035 $a(oapen)https://directory.doabooks.org/handle/20.500.12854/93272 035 $a(EXLCZ)995670000000391646 100 $a20202210d2022 |y 0 101 0 $aeng 135 $aurmn|---annan 181 $ctxt$2rdacontent 182 $cc$2rdamedia 183 $acr$2rdacarrier 200 10$aDiet Therapy and Nutritional Management of Phenylketonuria 210 $cMDPI - Multidisciplinary Digital Publishing Institute$d2022 215 $a1 electronic resource (376 p.) 311 $a3-0365-5319-3 330 $aPhenylketonuria (PKU) is an established inherited amino acid disorder with a very traditional dietary therapy, but there is still more to learn and verify about its nutritional composition, application and overall effectiveness. Although in the 1950s, the first patient successfully treated with diet therapy patently established the role of a low phenylalanine protein substitute, in present times, it is still necessary to characterise the most effective source of artificial protein; defining its optimal amino acid profile; and identifying nutrient modulation that will improve the functionality of protein substitutes. It is also important to understand the impact of a life-long synthetic diet on gut microbiota, metabolomics and inflammatory status. In early-treated patients with PKU, it is unclear if co-morbidities such as overweight, obesity, hypertension and diabetes are higher than in the general population and if these are associated with increased cardiovascular risk. It is also uncertain if overweight and obesity in PKU is related to early dietary practices, the nutritional composition of protein substitutes and special low-protein foods, impact of the dietary treatment on satiety, disordered eating patterns, non-adherence with the low phenylalanine diet and poor metabolic control, or if this is even a consequence of the disorder. In a generation of ageing patients, the impact of intermittent and suboptimal dietary adherence on nutritional status deserves systematic study. 606 $aResearch & information: general$2bicssc 606 $aBiology, life sciences$2bicssc 606 $aFood & society$2bicssc 610 $aphenylketonuria 610 $aspecial low protein foods 610 $anutritional composition 610 $aUK 610 $amacronutrients 610 $aphenylketonuria (PKU) 610 $aconsensus 610 $aDelphi method 610 $afood labelling 610 $aphenylalanine 610 $aPhe 610 $aprotein 610 $aexchanges 610 $aPKU 610 $aglycomacropeptide 610 $aamino acid 610 $aabsorption 610 $adiet therapy 610 $aprotein substitute 610 $agastrointestinal symptoms 610 $aprolonged release 610 $asatiety 610 $amacronutrient intake 610 $aprotein content 610 $afree from 610 $agluten free 610 $aaspartame 610 $asugar tax 610 $aphenylalanine hydroxylase deficiency 610 $ahyperphenylalaninemia 610 $amedical formula 610 $aamino acid mixture 610 $atetrahydrobiopterin 610 $asapropterin 610 $aBH4 610 $abody composition 610 $abone mineral density 610 $abone turnover markers 610 $aosteoporosis 610 $ablood biochemistry 610 $acasein glycomacropeptide 610 $aamino acid protein substitute 610 $alow-protein diet 610 $anewborn screening 610 $ametabolic control 610 $aguidelines 610 $anitrogen balance 610 $aamino acid catabolism 610 $ablood urea nitrogen 610 $abody mass index 610 $aobesity 610 $aoverweight 610 $aphenylalanine restriction 610 $aphenylalanine-restricted diet 610 $afood 610 $aschool 610 $aIHCP 610 $aparent/caregiver experiences 610 $aEngland 610 $aprescribing patterns 610 $acosts 610 $aadult phenylketonuria 610 $astandard operating procedure 610 $ainherited metabolic disorders 610 $adietary management 610 $aeating out 610 $alow protein food 610 $arestaurants 610 $aamino acids 610 $aadherence 610 $aepigenetics 610 $ahealth 610 $apreconception 610 $awomen 610 $adietary patterns 610 $afood frequency questionnaire 610 $avalidation 610 $areproducibility 615 7$aResearch & information: general 615 7$aBiology, life sciences 615 7$aFood & society 700 $aMacDonald$b Anita$4edt$01319281 702 $aMacDonald$b Anita$4oth 906 $aBOOK 912 $a9910619462903321 996 $aDiet Therapy and Nutritional Management of Phenylketonuria$93033695 997 $aUNINA