Results 11 to 20 of about 48,194 (317)

The Adult Phenylketonuria (PKU) Gut Microbiome

Microorganisms, 2021
Phenylketonuria (PKU) is an inborn error of phenylalanine metabolism primarily treated through a phenylalanine-restrictive diet that is frequently supplemented with an amino acid formula to maintain proper nutrition.
Viviana J. Mancilla, Allison E. Mann, Yan Zhang, Michael S. Allen   +3 more
doaj   +2 more sources

Preventing maternal phenylketonuria (PKU) syndrome: important factors to achieve good metabolic control throughout pregnancy

Orphanet Journal of Rare Diseases, 2021
Background Insufficient metabolic control during pregnancy of mothers with phenylketonuria (PKU) leads to maternal PKU syndrome, a severe embryo-/fetopathy. Since maintaining or reintroducing the strict phenylalanine (Phe) limited diet in adults with PKU
Carmen Rohde, Alena Gerlinde Thiele, Christoph Baerwald, Rudolf Georg Ascherl, Dinah Lier, Ulrike Och, Christina Heller, Alexandra Jung, Kathrin Schönherr, Monika Joerg-Streller, Simone Luttat, Sabine Matzgen, Tina Winkler, Stefanie Rosenbaum-Fabian, Oxana Joos, Skadi Beblo   +15 more
doaj   +2 more sources

Living with phenylketonuria in adulthood: the PKU ATTITUDE study [PDF]

Molecular Genetics and Metabolism Reports, 2018
Dietary treatment is the cornerstone of therapy for phenylketonuria (PKU), but adherence to low- phenylalanine diet progressively decreases after adolescence.
Bensi, Giulia, Biasucci, Giacomo, Burlina, Alberto, Cazzorla, Chiara, Leuzzi, Vincenzo, Manti, Filippo, Musumeci, Antonella, Papadia, Francesco, Polo, Giulia, Stoppioni, Vera, Tummolo, Albina, Vendemiale, Marcella   +11 more
core   +3 more sources

Long-term dietary intervention with low Phe and/or a specific nutrient combination improve certain aspects of brain functioning in phenylketonuria (PKU).

PLoS ONE, 2019
IntroductionIn phenylketonuria (PKU), a gene mutation in the phenylalanine metabolic pathway causes accumulation of phenylalanine (Phe) in blood and brain.
Vibeke M Bruinenberg, Danique van Vliet, Els van der Goot, Danielle S Counotte, Mirjam Kuhn, Francjan J van Spronsen, Eddy A van der Zee   +6 more
doaj   +2 more sources

Phenylketonuria (PKU): A problem solved?

Molecular Genetics and Metabolism Reports, 2016
Phenylketonuria (PKU) is a rare metabolic disorder characterized by impaired conversion of phenylalanine (Phe) to tyrosine. If left untreated, the resultant accumulation of excess blood Phe can cause physiological, neurological, and intellectual disabilities.
Uta Lichter-Konecki, Christine S. Brown
openaire   +4 more sources

Spatiotemporally consistent global dataset of the GIMMS Normalized Difference Vegetation Index (PKU GIMMS NDVI) from 1982 to 2022

Earth System Science Data, 2023
. Global products of remote sensing Normalized Difference Vegetation Index (NDVI) are critical to assessing the vegetation dynamic and its impacts and feedbacks on climate change from local to global scales.
Muyi Li, Sen Cao, Zaichun Zhu, Zhe Wang, R. Myneni, Shilong Piao   +5 more
semanticscholar   +1 more source

Nutrition management of PKU with pegvaliase therapy: update of the web-based PKU nutrition management guideline recommendations

Orphanet Journal of Rare Diseases, 2023
Background The web-based GMDI/SERN PKU Nutrition Management Guideline, published before approval of pegvaliase pharmacotherapy, offers guidance for nutrition management of individuals with phenylketonuria (PKU) treated with dietary therapy and/or ...
A. Cunningham, F. Rohr, P. Splett, S. Mofidi, H. Bausell, A. Stembridge, A. Kenneson, Rani H. Singh   +7 more
semanticscholar   +1 more source

Dietetic Management of Adults with Phenylketonuria (PKU) in the UK: A Care Consensus Document

Nutrients, 2022
There is an increasing number of adults and elderly patients with phenylketonuria (PKU) who are either early, late treated, or untreated. The principal treatment is a phenylalanine-restricted diet.
L. Robertson, S. Adam, C. Ellerton, Suzanne Ford, M. Hill, Gemma Randles, A. Woodall, Carla Young, A. Macdonald   +8 more
semanticscholar   +1 more source

Cardiometabolic and Nutritional Morbidities of a Large, Adult, PKU Cohort from Andalusia

Nutrients, 2022
The establishment of national neonatal screening systems has resulted in improved quality of life and life expectancy in patients with phenylketonuria (PKU). This has led to the development of multidisciplinary treatment units for adult patients with PKU.
Elena Dios-Fuentes, Montserrat Gonzalo Marín, P. Remón-Ruíz, Rosa Benitez Avila, M. Bueno Delgado, J. Blasco Alonso, Viyei Kishore Doulatram Gamgaram, G. Olveira, A. Soto-Moreno, Eva Venegas-Moreno   +9 more
semanticscholar   +1 more source

Glycomacropeptide in PKU—Does It Live Up to Its Potential?

Nutrients, 2022
The use of casein glycomacropeptide (CGMP) as a protein substitute in phenylketonuria (PKU) has grown in popularity. CGMP is derived from κ casein and is a sialic-rich glycophosphopeptide, formed by the action of chymosin during the production of cheese.
A. Daly, A. Pinto, S. Evans, A. Macdonald   +3 more
semanticscholar   +1 more source