公开(公告)号：US20150024083A1

公开(公告)日：2015-01-22

申请号：US14383989

申请日：2013-03-08

申请人： N.V. Nutricia

发明人： Andrew Sean Lynch ,  Simon David Anders

IPC分类号： A23L1/29 ,  A23L1/305

CPC分类号： A23L33/16 ,  A23L2/02 ,  A23L2/66 ,  A23L33/175 ,  A23L33/40 ,  A23V2002/00 ,  A23V2200/00 ,  A23V2200/30 ,  A23V2200/32 ,  A23V2250/00 ,  A23V2250/032 ,  A23V2250/06 ,  A23V2250/1578 ,  A23V2250/161 ,  A23V2250/506 ,  A23V2250/5072 ,  A23V2250/5086 ,  A23V2250/70 ,  A61K31/198 ,  A61K31/723 ,  A61K31/736 ,  A61K33/06 ,  A61K2300/00

摘要： The invention relates to a liquid or gelled pasteurized or sterilized composition comprising free amino acids, calcium, a digestible carbohydrate, and preferably an indigestible carbohydrate, wherein the free amino acids are present in a concentration between 5 and 30 g/100 ml, and the dry weight of the total composition is at least 40 g/100 ml, and preferably lower than 60 g/100 ml. The composition may in particular be used in the treatment of a human suffering from a disorder selected from the group consisting of phenylketonuria, homocystinuria, maple syrup urine disease, tyrosinaemia, propionic acidaemia, methylmalonic acidaemia, isovaleric acidaemia, urea cycle disorders and glutaric aciduria.

摘要翻译： 本发明涉及包含游离氨基酸，钙，可消化碳水化合物，优选消化不良的碳水化合物的液体或凝胶化巴氏消毒或灭菌组合物，其中游离氨基酸以5至30g / 100ml的浓度存在， 总组合物的干重至少为40g / 100ml，优选低于60g / 100ml。 组合物可特别用于治疗患有选自苯丙酮尿症，高尿酸尿症，枫糖浆尿病，酪氨酸血症，丙酸血症，甲基丙二酸血症，异戊酸性血症，尿素循环障碍和戊二酸尿症的病症的人。