Publications by authors named "K Ahring"
Article Synopsis
- In a study on phenylketonuria (PKU), data from 1323 patients across nine European and Turkish centers revealed that natural protein intake rises with age, especially during childhood and adolescence, while lifelong phenylalanine tolerance remains unclear.
- The research collected dietary intake information from 2012 to 2018, showing varying tolerances among different PKU classifications, with patients having mild PKU tolerating about 50% more phenylalanine than those with classical PKU.
- Additionally, the study highlighted that natural protein intake significantly increased for patients using sapropterin, with notable geographical differences in protein tolerance observed between Southern and Northern Europe.
Meta-analysis of bone mineral density in adults with phenylketonuria.
Orphanet J Rare Dis
September 2024
Article Synopsis
- Lifelong management of phenylketonuria (PKU) involves medical nutrition therapy, where patients have to restrict dietary phenylalanine (Phe) and often use Phe-free or low-Phe protein substitutes, which may lead to low bone mineral density (BMD). !* -
- A meta-analysis was conducted to investigate BMD specifically in adults with PKU, analyzing data from 4097 individuals across 10 studies to assess BMD Z-scores and the prevalence of low BMD. !* -
- Results indicated that adults with PKU had significantly lower mean BMD Z-scores compared to age-matched controls, although still within acceptable ranges, with about 8% of individuals
A phenylalanine-restricted diet, supplemented with protein substitutes (PSs), remains the cornerstone of phenylketonuria (PKU) management. However, adherence is challenging in adulthood, and data on the nutritional status of early and continuously treated adults with PKU (ETAwPKU) are scarce. A total of 34 ETAwPKU (16 females; mean ± SD, age: 28 ± 9 years, phenylalanine concentration: 847 ± 285 µmol/L) and 34 age- and sex-matched control subjects were compared regarding their blood nutrient status, self-reported dietary intake, and cognitive wellbeing.
View Article and Find Full Text PDFBackground: Phenylketonuria (PKU) is an inborn error of phenylalanine (Phe) metabolism that, if untreated, causes Phe accumulation in the brain leading to neurophysiologic alterations and poor outcomes. Lifelong management centers on dietary Phe restriction, yet long-term complete metabolic control is unachievable for many adults. High blood Phe levels or chronic Phe and intact protein restriction in the diet may lead to somatic comorbidities.
View Article and Find Full Text PDFArticle Synopsis
- A 2011 survey indicated that blood phenylalanine (Phe) control in patients with phenylketonuria (PKU) worsens with age, leading to new European PKU guidelines for blood Phe levels in 2017.
- The study involved nine centers across Europe and Turkey, collecting data from 1323 patients with varying PKU severity to evaluate blood Phe control from 2012 to 2018.
- Results showed that younger patients had better blood Phe control, with classical PKU patients having significantly higher mean blood Phe levels compared to mild PKU and hyperphenylalaninemia (HPA) patients, while female patients generally had a higher percentage of