2 documents found, page 1 of 1
Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis
Ilgaz, Fatma; Pinto, Alex; Gökmen-Özel, Hülya; Rocha, Júlio César; van Dam, Esther; Ahring, Kirsten; Bélanger-Quintana, Amaya; Dokoupil, KatharinaThere 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 of this systematic review was to investigate the effects of a phenylalanine (Phe)-restricted diet on long-term growth in patients with PKU. Four electronic databases were searched for articles ...
Dietary practices in methylmalonic acidaemia: a European survey
Pinto, Alex; Evans, Sharon; Daly, Anne; Almeida, Manuela Ferreira; Assoun, Murielle; Bélanger-Quintana, Amaya; Bernabei, Silvia MariaBackground -The dietary management of methylmalonic acidaemia (MMA) is a low-protein diet providing sufficient energy to avoid catabolism and to limit production of methylmalonic acid. The goal is to achieve normal growth, good nutritional status and the maintenance of metabolic stability. Aim -To describe the dietary management of patients with MMA across Europe. Methods - A cross-sectional questionnaire was s...