Protein requirements in adults with phenylketonuria and bioavailability of glycomacropeptide compared to an l-amino acid-based product.

Background: Phenylketonuria (PKU) is caused by phenylalanine hydroxylase deficiency. Treatment is primarily a low-Phe diet combined with l-amino acid-based products (l-AA). Protein requirements in adults with PKU have not been directly determined.

Development of minimally invasive C-glucose breath test to examine different exogenous carbohydrate sources in patients with glycogen storage disease type Ia.

Background: Glycogen storage disease type Ia (GSD Ia) is an autosomal recessive disorder caused by deficiency of glucose-6-phosphatase (G6Pase), resulting in fasting hypoglycemia. Dietary treatment with provision of uncooked cornstarch (UCCS) or a novel modified cornstarch () is available to treat hypoglycemia, yet choice of carbohydrate to achieve a desirable glycemic control is debated.C-glucose breath test (C-GBT) can be used to examine glucose metabolism from different carbohydrate sources via CO in breath.

Salivary serotonin does not correlate with central serotonin turnover in adult phenylketonuria (PKU) patients.

Introduction: Phenylketonuria (PKU) is an inborn error of metabolism associated with an increased risk of behavioural and mood disorders. There are currently no reliable markers for monitoring mood in PKU. The purpose of this study was to evaluate salivary serotonin as a possible non-invasive marker of long-term mood symptoms and central serotonin activity in patients with PKU.