Now showing items 1-6 of 6
Long-Term Growth In Phenylketonuria: A Systematic Review And Meta-Analysis
There is an ongoing debate regarding the impact of phenylketonuria (PKU) and its treatment on growth. To date, evidence from studies is inconsistent, and data on the whole developmental period is limited. The primary aim ...
Weight Management In Phenylketonuria: What Should Be Monitored?
Background: Severe intellectual disability and growth impairment have been overcome by the success of early and continuous treatment of patients with phenylketonuria (PKU). However, there are some reports of obesity, ...
When Should Social Service Referral Be Considered In Phenylketonuria?
Lifelong low-phenylalanine (Phe) dietary management is the foundation of care in phenylketonuria (PKU). However, strict monitoring of food intake places a burden on patients and their caregivers, and adherence to the ...
Special Low Protein Foods For Phenylketonuria: Availability In Europe And An Examination Of Their Nutritional Profileinfo:eu-repo/semantics/publishedVersion
Background Special low protein foods (SLPF) are essential in the nutritional management of patients with phenylketonuria (PKU). The study objectives were to: 1) identify the number of SLPF available for use in eight European ...
3-Hydroxy-3-Methylglutaryl-Coa Lyase Deficiency: A Case Report And Literature Review
(Aran Ediciones, S L, 2018)
Introduction: 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) lyase deficiency is an autosomal recessive disorder that usually presents in the neonatal period with vomiting, metabolic acidosis, hypoglycemia and absent ketonuria. ...
Adjusting Diet With Sapropterin In Phenylketonuria: What Factors Should Be Considered?
(Cambridge Univ Press, 2011)
The usual treatment for phenylketonuria (PKU) is a phenylalanine-restricted diet. Following this diet is challenging, and long-term adherence (and hence metabolic control) is commonly poor. Patients with PKU (usually, but ...