OBJECTIVES: To study the lipid profile in a group of treated phenylketonuric patients (PKU; n = 61) compared with a group of inborn error of intermediary metabolism patients (IEM; n = 22), a group of hyperphenylalaninemic children (HPA; n = 37), and a control group without dietary restriction (n = 41). DESIGN AND METHODS: Phenylalanine was analyzed by ion exchange chromatography and triglycerides, cholesterol and HDL were determined by standard procedures with the Cobas Integra analyzer. RESULTS: Serum total cholesterol concentrations were significantly lower in PKU patients compared with IEM patients (whose cholesterol daily intake was similar to those of PKU patients), HPA children and the control group. A negative correlation was observed between cholesterol and phenylalanine concentrations in the PKU patients. CONCLUSIONS: Our findings support the hypothesis of a relationship between high plasma phenylalanine levels and an inhibition of cholesterogenesis, although the low cholesterol intake of the special diets may also decrease serum cholesterol values.
OBJECTIVES: To study the lipid profile in a group of treated phenylketonuricpatients (PKU; n = 61) compared with a group of inborn error of intermediary metabolismpatients (IEM; n = 22), a group of hyperphenylalaninemicchildren (HPA; n = 37), and a control group without dietary restriction (n = 41). DESIGN AND METHODS: Phenylalanine was analyzed by ion exchange chromatography and triglycerides, cholesterol and HDL were determined by standard procedures with the Cobas Integra analyzer. RESULTS: Serum total cholesterol concentrations were significantly lower in PKUpatients compared with IEM patients (whose cholesterol daily intake was similar to those of PKUpatients), HPAchildren and the control group. A negative correlation was observed between cholesterol and phenylalanine concentrations in the PKUpatients. CONCLUSIONS: Our findings support the hypothesis of a relationship between high plasma phenylalanine levels and an inhibition of cholesterogenesis, although the low cholesterol intake of the special diets may also decrease serum cholesterol values.
Authors: Bridget M Stroup; Nivedita Nair; Sangita G Murali; Katarzyna Broniowska; Fran Rohr; Harvey L Levy; Denise M Ney Journal: J Nutr Date: 2018-02-01 Impact factor: 4.798
Authors: Fernando Andrade; Olalla López-Suárez; Marta Llarena; María L Couce; Luis Aldámiz-Echevarría Journal: Medicine (Baltimore) Date: 2017-07 Impact factor: 1.889
Authors: Radu Tanacli; Jan-Hendrik Hassel; Rolf Gebker; Alexander Berger; Michael Gräfe; Christopher Schneeweis; Patrick Doeblin; Eckart Fleck; Christian Stehning; Frank Tacke; Burkert Pieske; Joachim Spranger; Ursula Plöckinger; Athanasia Ziagaki; Sebastian Kelle Journal: J Am Heart Assoc Date: 2021-08-21 Impact factor: 5.501