Liquid nutritional formula for phenylketonuria patients

Abstract

This is a process to make a shelf-stable, liquid, ready-to-drink, aseptic nutritional formula for the treatment of PKU, tyrosinemia or other errors of metabolism. The liquid nutritional formula has caseino-glyco-macropeptide as the primary protein source and complementary essential amino acids to complete the requirement profile. The beverage also includes vitamins and minerals sufficient to meet daily requirements. When used as a complete diet, the nutritional formula includes a carbohydrate source and a fat source. The nutritional formula has a pleasant taste.

What is claimed is: 
     
         1 . A liquid nutritional formula as shown in Table 1 suitable as a complete diet for PKU. 
     
     
         2 . The nutritional formula according to  claim 1  in which protein provides 20-35% of the energy of the nutritional formula, fat provides 20% to 30% of the energy of the nutritional formula, and the carbohydrate source provides 40% to 60% of the energy of the nutritional formula. 
     
     
         3 . The nutritional formula according to  claim 1 , comprising the following complementary amino acids per gram of protein equivalent (PE), 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                   Tyrosine (mg/gm PE) 
                   93 
                 
                     
                   Tryptophan (mg/gm PE) 
                   14 
                 
                     
                   Arginine (mg/gm PE) 
                   90 
                 
                     
                   Leucine (mg/gm PE) 
                   200 
                 
                     
                   Histidine (mg/gm PE) 
                   24 
                 
                     
                     
                 
             
                
               
               
                
                
                
                
                
                
               
            
           
         
       
       all percentages being by weight of the complementary amino acids, amino acid esters or salts of amino acids. 
     
     
         4 . A liquid nutritional formula comprising components as shown in Table 2, Table 3, Table 4 or Table 5. 
     
     
         5 . The liquid nutritional formula of  claim 3 , which is an emulsion. 
     
     
         6 . A method of producing a nutritional formula suitable as a complete diet for PKU, the method comprising:
 (a) introducing, GMP, amino acids, vitamins and dry ingredients into a metered volume of water to produce a first mixture;   (b) introducing SHMP and sensitive minerals into a metered volume of water to produce a second mixture;   (c) combining the first mixture and the second mixture;   (d) combining an emulsion comprising tyrosine, OSS, SSL, cocoa butter, vegetable oil with the product of (c); and   (e) adding Vitamin C to the product of (d), thereby producing a nutritional formula.   
     
     
         7 . The method of  claim 6  further comprising subjecting the product of (e) to high-shear mixing or blending. 
     
     
         8 . The method of  claim 7 , wherein the method further comprises the step of storing the nutritional formula under aseptic conditions. 
     
     
         9 . The method of  claim 7 , wherein the method further comprises aseptically packaging the nutritional formula.