caa a22 4500 475785703 CHVBK 20180406123657.0 cr unu---uuuuu 170329e20000901xx s 000 0 eng 10.1007/PL00014376 doi (NATIONALLICENCE)springer-10.1007/PL00014376 Nutrition, physical growth, and bone density in treated phenylketonuria [Elektronische Daten] [Hildegard Przyrembel, Hans-Joachim Bremer] Dietary treatment of phenylketonuria is well established to be safe and to prevent developmental and mental impairment in patients with low or absent phenylalanine hydroxylase activity. The use of semi-synthetic diets necessitates careful and longitudinal control not only of physical and intellectual development, which are both near normal in well treated patients, but also of potential diet inherent insufficiencies of essential nutrients. Concern has been raised by some reports on growth retardation in young patients on strict diets and on decreased bone density in older phenylketonuric children. The clinical significance of these findings is not known. Conclusion Changes have been found, although inconsistently, in connection with selenium, zinc, iron, retinol and polyunsaturated fatty acid status in dietetically treated patients with phenylketonuria. Both the mechanism and significance of these changes is doubtful at present. Springer-Verlag Berlin Heidelberg, 2000 Key words Bone density nationallicence Dietary treatment nationallicence Growth nationallicence Phenylketonuria nationallicence AbbreviationsAA arachidonic acid nationallicence AAS amino acid score nationallicence DHA docosahexaenoic acid nationallicence EPA eicosapentaenoic acid nationallicence IAA indispensable amino acids nationallicence Phe phenylalanine nationallicence PKU phenylketonuria nationallicence Przyrembel Hildegard Bundesinstitut für gesundheitlichen Verbraucherschutz und Veterinärmedizin, Thielallee 88-92, 14195 Berlin, Germany e-mail: h.przyrembel@bgvv.de Tel.: +49-1888 412 3221; Fax: +49-1888 412 3715, DE aut Bremer Hans-Joachim Universitäts-Kinderklinik, 69120 Heidelberg, Germany, DE aut https://doi.org/10.1007/PL00014376 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/PL00014376 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Przyrembel Hildegard Bundesinstitut für gesundheitlichen Verbraucherschutz und Veterinärmedizin, Thielallee 88-92, 14195 Berlin, Germany e-mail: h.przyrembel@bgvv.de Tel.: +49-1888 412 3221; Fax: +49-1888 412 3715, DE aut NATIONALLICENCE 700 1- Bremer Hans-Joachim Universitäts-Kinderklinik, 69120 Heidelberg, Germany, DE aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer