Developmental Defects of Enamel in Phenylketonuria Patients

Source: Journal of Dentistry for Children - Category: Dentistry Authors: Tags: Clinical Article Source Type: research

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Source: BMJ News - Category: General Medicine Source Type: research
AbstractLimited published research has evaluated mental health in parents of children with phenylketonuria (PKU) and their coping strategies. We aimed to assess anxiety, depression and coping in parents of Tunisian children treated for PKU. We conducted a cross-sectional study in the reference center for the management of inherited metabolic diseases in Tunisia. We used the Hospital Anxiety and Depression (HAD) scale and the Brief-COPE. Dependent variables were anxiety, depression, HAD Depression (HADD) and Anxiety (HADA) scores, and coping scores. Linear regression and logistic binary regression were used for multivariate...
Source: Journal of Developmental and Physical Disabilities - Category: Disability Source Type: research
Condition:   Adult Phenylketonuria Non Treated Patients Intervention:   Dietary Supplement: Dietary Supplement for PKU patients Sponsors:   University Hospital, Tours;   INSERM 1415-TOURS Not yet recruiting
Source: - Category: Research Source Type: clinical trials
In this study we compared the gut microbial communities of children with PKU and with mild hyperphenylalaninemia (MHP, unrestricted diet). For each group, we enrolled 21 children (4–18 years old), for a total dataset of 42 subjects. We assessed dietary intake and performed gut microbiota analysis by sequencing the V3–V4 hypervariable regions of the 16S rRNA gene. Short chain fatty acids (SCFAs) were quantified by gas chromatographic analysis. While alpha-diversity analysis showed no significant differences between PKU and MHP groups, microbial community analysis highlighted a significant separation of the gut m...
Source: Frontiers in cellular and infection microbiology - Category: Microbiology Source Type: research
Can the Microbiome Deliver? A Proof-of-Concept Engineered E. coli PKU Therapeutic. Cell Host Microbe. 2019 Apr 10;25(4):473-474 Authors: Alteri CJ Abstract Phenylketonuria (PKU) is a rare genetic disorder that causes phenylalanine toxicity in the brain. Two studies, Crook et al. (2019), in this issue of Cell Host &Microbe, and Isabella et al. (2018), employ synthetic biology to develop a live bacterial therapeutic to treat PKU and potentially other metabolic diseases. PMID: 30974078 [PubMed - in process]
Source: Cell Host and Microbe - Category: Microbiology Authors: Tags: Cell Host Microbe Source Type: research
Mehdi Taslimifar, Stefano Buoso, Francois Verrey, Vartan Kurtcuoglu
Source: Frontiers in Physiology - Category: Physiology Source Type: research
AbstractPhenylketonuria ’s (PKU) treatment based on low-protein diet may affect other metabolic pathways, such as that of asymmetric dimethylarginine (ADMA). The aim of this study was to evaluate the reliability of ADMA as a biomarker of adequate metabolic control and possible nutritional risk in a long-term PKU patient population. One hundred and six dietary-treated PKU patients from four hospitals in Spain were enrolled in this cross-sectional study. Their lipid profile, total homocysteine, ADMA, and symmetric dimethylarginine (SDMA) concentrations were analyzed and compared with a control group. Sensitivity, spe c...
Source: European Journal of Pediatrics - Category: Pediatrics Source Type: research
Phenylketonuria is a rare inborn error of metabolism. The build-up of phenylalanine in the blood and body tissues can have significant impact on the brain's development. High phenylalanine levels have been shown to be associated with an increase in neuropsychiatric symptoms, including mood, anxiety, and attention problems; decreased social competence; and low self-esteem. This case report highlights such a presentation in an adolescent with phenylketonuria.
Source: Journal of Pediatric Health Care - Category: Pediatrics Authors: Tags: Case Report Source Type: research
Authors: Hozyasz KK, Żółkowska J Abstract Dietetic treatment of phenylketonuria (PKU) includes a low-phenylalanine (phe) diet that provides sufficient phe for maintenance and growth plus special phe-free formulas with amino acids to meet requirements for protein, energy and micronutrients. PMID: 30907078 [PubMed - as supplied by publisher]
Source: Journal of Biological Regulators and Homeostatic Agents - Category: Biomedical Science Tags: J Biol Regul Homeost Agents Source Type: research
Publication date: June 2019Source: Molecular Genetics and Metabolism Reports, Volume 19Author(s): Hardo Lilleväli, Karit Reinson, Kai Muru, Siret Saarsalu, Kadi Künnapas, Tiina Kahre, Ülle Murumets, Katrin ÕunapAbstractBlood phenylalanine (Phe) values from the dried blood spots of all Estonian phenylketonuria (PKU) patients have been deposited into a unified electronic laboratory database for eight years, providing an opportunity to assess the adherence of the patients to dietary recommendations over time and to observe patient practices both individually and collectively. Our results demonstrate gene...
Source: Molecular Genetics and Metabolism Reports - Category: Genetics & Stem Cells Source Type: research
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