2 documents found, page 1 of 1
The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Rev...
Pena, M.; Pinto, A.; Daly, A.; MacDonald, A.; Azevedo, L.; Rocha, J.; Borges, N.In phenylketonuria (PKU), synthetic protein derived from L-amino acids (AAs) is essential in a low-phenylalanine (Phe) diet. Glycomacropeptide (GMP), an intact protein, is very low in Phe in its native form. It has been modified and adapted for PKU to provide an alternative protein source through supplementation with rate-limiting amino acids (GMP-AAs), although it still contains residual Phe. This review aims ...
International practices in the dietary management of fructose 1-6 biphosphatase...
Pinto, A.; Alfadhel, M.; Akroyd, R.; Atik Altınok, Y.; Bernabei, S.; Bernstein, L.; Bruni, G.; Caine, G.; Cameron, E.; Carruthers, R.; Cochrane, B.Background: In fructose 1,6 bisphosphatase (FBPase) deficiency, management aims to prevent hypoglycaemia and lactic acidosis by avoiding prolonged fasting, particularly during febrile illness. Although the need for an emergency regimen to avoid metabolic decompensation is well established at times of illness, there is uncertainty about the need for other dietary management strategies such as sucrose or fructose...