05203nam 2201309z- 450 991061946290332120231214133051.03-0365-5320-7(CKB)5670000000391646(oapen)https://directory.doabooks.org/handle/20.500.12854/93272(EXLCZ)99567000000039164620202210d2022 |y 0engurmn|---annantxtrdacontentcrdamediacrrdacarrierDiet Therapy and Nutritional Management of PhenylketonuriaMDPI - Multidisciplinary Digital Publishing Institute20221 electronic resource (376 p.)3-0365-5319-3 Phenylketonuria (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.Research & information: generalbicsscBiology, life sciencesbicsscFood & societybicsscphenylketonuriaspecial low protein foodsnutritional compositionUKmacronutrientsphenylketonuria (PKU)consensusDelphi methodfood labellingphenylalaninePheproteinexchangesPKUglycomacropeptideamino acidabsorptiondiet therapyprotein substitutegastrointestinal symptomsprolonged releasesatietymacronutrient intakeprotein contentfree fromgluten freeaspartamesugar taxphenylalanine hydroxylase deficiencyhyperphenylalaninemiamedical formulaamino acid mixturetetrahydrobiopterinsapropterinBH4body compositionbone mineral densitybone turnover markersosteoporosisblood biochemistrycasein glycomacropeptideamino acid protein substitutelow-protein dietnewborn screeningmetabolic controlguidelinesnitrogen balanceamino acid catabolismblood urea nitrogenbody mass indexobesityoverweightphenylalanine restrictionphenylalanine-restricted dietfoodschoolIHCPparent/caregiver experiencesEnglandprescribing patternscostsadult phenylketonuriastandard operating procedureinherited metabolic disordersdietary managementeating outlow protein foodrestaurantsamino acidsadherenceepigeneticshealthpreconceptionwomendietary patternsfood frequency questionnairevalidationreproducibilityResearch & information: generalBiology, life sciencesFood & societyMacDonald Anitaedt1319281MacDonald AnitaothBOOK9910619462903321Diet Therapy and Nutritional Management of Phenylketonuria3033695UNINA05326nam 22007695 450 991029984160332120200630031130.03-319-20813-610.1007/978-3-319-20813-8(CKB)3710000000444411(EBL)3567619(SSID)ssj0001534494(PQKBManifestationID)11875456(PQKBTitleCode)TC0001534494(PQKBWorkID)11494684(PQKB)10666019(DE-He213)978-3-319-20813-8(MiAaPQ)EBC3567619(PPN)187687072(EXLCZ)99371000000044441120150704d2015 u| 0engur|n|---|||||txtccrMaintenance Overtime Policies in Reliability Theory Models with Random Working Cycles /by Toshio Nakagawa, Xufeng Zhao1st ed. 2015.Cham :Springer International Publishing :Imprint: Springer,2015.1 online resource (126 p.)Lecture Notes in Production Engineering,2194-0525Description based upon print version of record.3-319-20812-8 Includes bibliographical references at the end of each chapters.Preface; References; Contents; 1 Age Replacement Overtime; 1.1 Age and Random Replacements; 1.2 Replacement Overtime; 1.3 Comparisons of Age and Random Replacements; 1.4 Replacement Overtime Last; 1.5 Finite Interval; 1.6 Working Number; 1.7 Parallel System; References; 2 Periodic Replacement Overtime; 2.1 Periodic and Random Replacements; 2.2 Replacement Overtime; 2.3 Comparisons of Periodic and Random Replacements; 2.4 Replacement Overtime Last; 2.5 Replacement Overtime with Number of Failures; 2.5.1 Replacement Overtime First with Number of Failures2.5.2 Replacement Overtime Last with Number of Failures2.6 Replacement Overnumber; 2.6.1 Replacement Over Number N; 2.6.2 Replacement over Number K; 2.7 Preventive Maintenance Overtime; References; 3 Inspection Overtime; 3.1 Periodic and Random Inspections; 3.2 Inspection First and Last; 3.2.1 Inspection First; 3.2.2 Inspection Last; 3.2.3 Comparison of Inspection First and Last; 3.3 Inspection Overtime; 3.3.1 Comparisons with Other Policies; 3.4 General Failure Times; 3.5 Periodic and Random Backup; 3.6 Backup Overtime; References; 4 Replacement Overtime with Three Variables4.1 Replacement with Three Variables4.2 Replacement Overtime with Working Cycle; 4.3 Replacement Overtime with Failure Number; 4.4 Modified Replacement with Three Variables; 4.5 General Replacement Policies; References; AppendicesThis book introduces a new concept of replacement in maintenance and reliability theory. Replacement overtime, where replacement occurs at the first completion of a working cycle over a planned time, is a new research topic in maintenance theory and also serves to provide a fresh optimization technique in reliability engineering. In comparing replacement overtime with standard and random replacement techniques theoretically and numerically, 'Maintenance Overtime Policies in Reliability Theory' highlights the key benefits to be gained by adopting this new approach and shows how they can be applied to inspection policies, parallel systems and cumulative damage models. Utilizing the latest research in replacement overtime by internationally recognized experts, readers are introduced to new topics and methods, and learn how to practically apply this knowledge to actual reliability models. This book will serve as an essential guide to a new subject of study for graduate students and researchers and also provides a valuable resource for reliability engineers and managers who have difficulties maintaining computer and production systems with random working cycles.Lecture Notes in Production Engineering,2194-0525Quality controlReliabilityIndustrial safetyManagementIndustrial managementComputer software—ReusabilityQuality Control, Reliability, Safety and Riskhttps://scigraph.springernature.com/ontologies/product-market-codes/T22032Innovation/Technology Managementhttps://scigraph.springernature.com/ontologies/product-market-codes/518000Performance and Reliabilityhttps://scigraph.springernature.com/ontologies/product-market-codes/I12077Quality control.Reliability.Industrial safety.Management.Industrial management.Computer software—Reusability.Quality Control, Reliability, Safety and Risk.Innovation/Technology Management.Performance and Reliability.620.00452Nakagawa Toshioauthttp://id.loc.gov/vocabulary/relators/aut724944Zhao Xufengauthttp://id.loc.gov/vocabulary/relators/autMiAaPQMiAaPQMiAaPQBOOK9910299841603321Maintenance Overtime Policies in Reliability Theory2521966UNINA