Results 21 to 30 of about 48,194 (317)

Is the Phenylalanine-Restricted Diet a Risk Factor for Overweight or Obesity in Patients with Phenylketonuria (PKU)? A Systematic Review and Meta-Analysis

Nutrients, 2021
Although there is a general assumption that a phenylalanine (Phe)-restricted diet promotes overweight in patients with phenylketonuria (PKU), it is unclear if this presumption is supported by scientific evidence. This systematic review aimed to determine
C. Rodrigues, A. Pinto, A. Faria, D. Teixeira, A. V. van Wegberg, K. Ahring, F. Feillet, C. Calhau, A. Macdonald, A. Moreira-Rosário, J. C. Rocha   +10 more
semanticscholar   +1 more source

Protein Substitutes in PKU; Their Historical Evolution

Nutrients, 2021
Protein substitutes developed for phenylketonuria (PKU) are a synthetic source of protein commonly based on L-amino acids. They are essential in the treatment of phenylketonuria (PKU) and other amino acid disorders, allowing the antagonistic amino acid ...
A. Daly, S. Evans, A. Pinto, C. Ashmore, A. Macdonald   +4 more
semanticscholar   +1 more source

Special Low Protein Foods Prescribed in England for PKU Patients: An Analysis of Prescribing Patterns and Cost

Nutrients, 2021
Patients with phenylketonuria (PKU) are reliant on special low protein foods (SLPFs) as part of their dietary treatment. In England, several issues regarding the accessibility of SLPFs through the national prescribing system have been highlighted ...
Georgina Wood, A. Pinto, S. Evans, A. Daly, S. Adams, S. Costelloe, J. Gribben, C. Ellerton, A. Emm, Sarah Firman, Suzanne Ford, M. French, L. Gaff, Emily Giuliano, M. Hill, I. Hunjan, C. Newby, Allison Mackenzie, R. Pereira, Celine Prescott, L. Robertson, Heidi Seabert, R. Skeath, Simon Tapley, A. Terry, A. Tooke, K. van Wyk, F. White, Lucy White, A. Woodall, J. C. Rocha, A. Macdonald   +31 more
semanticscholar   +1 more source

Intestinal peroxisome proliferator‐activated receptor α‐fatty acid‐binding protein 1 axis modulates nonalcoholic steatohepatitis

Hepatology, EarlyView., 2022
Abstract Background and Aims Peroxisome proliferator‐activated receptor α (PPARα) regulates fatty acid transport and catabolism in liver. However, the role of intestinal PPARα in lipid homeostasis is largely unknown. Here, intestinal PPARα was examined for its modulation of obesity and NASH. Approach and Results Intestinal PPARα was activated and fatty
Tingting Yan, Yuhong Luo, Nana Yan, Keisuke Hamada, Nan Zhao, Yangliu Xia, Ping Wang, Changdong Zhao, Dan Qi, Shoumei Yang, Lulu Sun, Jie Cai, Qiong Wang, Changtao Jiang, Oksana Gavrilova, Kristopher W. Krausz, Daxesh P. Patel, Xiaoting Yu, Xuan Wu, Haiping Hao, Weiwei Liu, Aijuan Qu, Frank J. Gonzalez   +22 more
wiley   +1 more source

Measuring Burden of Illness in Phenylketonuria (PKU): Development of the PKU Symptom Severity and Impacts Scale as a Robust Patient-Reported Outcome

Advances in Therapy, 2021
Phenylketonuria (PKU) is a rare, metabolic genetic disorder that can cause various neuropsychological symptoms that often affect patients’ health-related quality of life, even for patients with good metabolic control.
J. Quinn, A. Georgiadis, H. Lewis, E. Jurecki   +3 more
semanticscholar   +1 more source

Allelic phenotype prediction of phenylketonuria based on the machine learning method

Human Genomics, 2023
Background Phenylketonuria (PKU) is caused by mutations in the phenylalanine hydroxylase (PAH) gene. Our study aimed to predict the phenotype using the allelic genotype.
Yang Fang, Jinshuang Gao, Yaqing Guo, Xiaole Li, Enwu Yuan, Erfeng Yuan, Liying Song, Qianqian Shi, Haiyang Yu, Dehua Zhao, Linlin Zhang   +10 more
doaj   +1 more source

Dental Status and Periodontal Health of Patients with Phenylketonuria in Latvia

Acta Stomatologica Croatica, 2022
Objectives: Phenylketonuria (PKU) is an autosomal recessive inherited disorder of phenylalanine metabolism resulting from a deficiency of phenylalanine hydroxylase.
Iveta Abola, Darta Elizabete Emulina, Ingus Skadins, Anda Brinkmane, Linda Gailite, Madara Auzenbaha   +5 more
doaj   +1 more source

Comparison of IQ scores between children with phenylketonuria and healthy children referring to Besat Hospital in Sanandaj between 2017 and 2018 [PDF]

مجله علمی دانشگاه علوم پزشکی کردستان, 2019
Background and Aim: Phenylketonuria is a genetic and congenital metabolic disorder with autosomal recessive transmission. In this disease phenylalanine is not converted to tyrosine due to lack of  phenylalanine hydroxylase enzyme which results in ...
Asadollah Fatholahpuor, Sima Alimoradi, Fayegh Yousefi, Hajar Kashefi   +3 more
doaj   +1 more source

The cardiovascular phenotype of adult patients with phenylketonuria [PDF]

, 2019
BACKGROUND: Patients with Phenylketonuria (PKU) are exposed to multiple cardiovascular risk factors, but the clinical significance of these abnormalities is yet unknown.
Azabdaftari, Aline, Giet, Markus van der, Hennermann, Julia B., Plöckinger, Ursula, Querfeld, Uwe, Schuchardt, Mirjam   +5 more
core   +1 more source

Nutrient Status among Latvian Children with Phenylketonuria

Children, 2023
(1) Introduction: Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism. The main treatment for PKU is to manage nutrition, thereby restricting phenylalanine intake. Part of patient management is analyzing eating habits
Olga Lubina, Linda Gailite, Julija Borodulina, Madara Auzenbaha   +3 more
doaj   +1 more source