Kauf E, Seidel J, Winnefeld K, Dawczynski H, Häfer R, Stein F, Vogt L
Klinik für Kinder-und Jugendmedizin, Friedrich-Schiller-Universität, Jena.
Med Klin (Munich). 1997 Sep 15;92 Suppl 3:31-4. doi: 10.1007/BF03041959.
17 patients (8 female, 9 male; age 8.2 +/- 3.7 years) with phenylketonuria under phenylalanin restricted diet were investigated prior to and after 3 months of selenium substitution (sodium selenite, 115 micrograms Se/m2 BSA/d). Different parameters in blood were determined: selenium, glutathione peroxidase (Gpx) activity, thyroid hormones, blood cell count, lymphocytic antigen expression, muscle function and -enzymes, cardiac ultrasound.
The main significant results of selenium substitution are: increased plasma-selenium, blood cell selenium, plasma-Gpx activity and left ventricular cardiac index as well as decreased plasma thyroxin, free thyroxin, reverse triiodthyronin, total cholesterol, mean erythrocyte and thrombocyte volume and lymphocytic CD2 expression.
The data indicate metabolic and functional signs of selenium deficiency in patients with phenylketonuria without selenium substitution. We conclude that, despite of lacking clinical symptoms, a selenium supply in phenylketonuria patients under diet is necessary and should be performed with usefull peroral sodium selenite (115 micrograms Se/m2 BSA/d) initially, followed by a dosage between 30 and 60 micrograms Se/m2 BSA/d).
对17例接受苯丙氨酸限制饮食的苯丙酮尿症患者（8例女性，9例男性；年龄8.2±3.7岁）在补充硒（亚硒酸钠，115微克硒/平方米体表面积/天）3个月前后进行了研究。测定了血液中的不同参数：硒、谷胱甘肽过氧化物酶（Gpx）活性、甲状腺激素、血细胞计数、淋巴细胞抗原表达、肌肉功能及酶、心脏超声。
补充硒的主要显著结果为：血浆硒、血细胞硒、血浆Gpx活性及左心室心脏指数升高，以及血浆甲状腺素、游离甲状腺素、反三碘甲状腺原氨酸、总胆固醇、平均红细胞和血小板体积及淋巴细胞CD2表达降低。
数据表明未补充硒的苯丙酮尿症患者存在硒缺乏的代谢和功能体征。我们得出结论，尽管缺乏临床症状，但对饮食控制下的苯丙酮尿症患者进行硒补充是必要的，最初应以有效的口服亚硒酸钠（115微克硒/平方米体表面积/天）进行补充，随后剂量为30至60微克硒/平方米体表面积/天。
