Recommendations on the dietary management of phenylketonuria. Report of Medical Research Council Working Party on Phenylketonuria.
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Design and Development of a Minimum Data Set for Phenylketonuria Disease
Introduction: Phenylketonuria is one of the most common autosomal recessive metabolic diseases, characterized by a wide range of neuropsychological and neurocognitive disorders. Without proper care, control, and management, this disease can lead to severe mental retardation and neurobehavioral disorders. Therefore, the objective of this study was to design and develop a Minimum Data Set (MDS) f...
متن کاملDietary amino acid intakes associated with a low-phenylalanine diet combined with amino acid medical foods and glycomacropeptide medical foods and neuropsychological outcomes in subjects with phenylketonuria
This article provides original data on median dietary intake of 18 amino acids from amino acid medical foods, glycomacropeptide medical foods, and natural foods based on 3-day food records obtained from subjects with phenylketonuria who consumed low-phenylalanine diets in combination with amino acid medical foods and glycomacropeptide medical foods for 3 weeks each in a crossover design. The sa...
متن کاملEfect of a controlled diet program on behavioral-emotional disorders in patients with phenylketonuria
Introduction: Phenylketonuria is a disorder due to deficiency of the phenylalanine hydroxylase enzyme. Delay or untreatement would cause impacts on motor-cognitive and individual-social skills. This study aimed to investigate the effect of a controlled diet program on behavioral-emotional disorders in patients with phenylketonuria. Materials and Methods: In a quasi-experimental study, 60 patien...
متن کاملDesign and Development of a Minimum Data Set for Phenylketonuria Disease
Introduction: Phenylketonuria is one of the most common autosomal recessive metabolic diseases, characterized by a wide range of neuropsychological and neurocognitive disorders. Without proper care, control, and management, this disease can lead to severe mental retardation and neurobehavioral disorders. Therefore, the objective of this study was to design and develop a Minimum Data Set (MDS) f...
متن کاملSelective Screening of Phenylketonuria, Tyrosinemia and Maple Syrup Urine Disease in Southern Iran
Inborn errors of amino-acids metabolism and other inherited Mendeliandisorders are common in the MiddleEast.The number of diagnosed inborn errors of amino acid metabolism is growing constantly on account of and availability and improved of analytical techniques. The aim of this work was to determine a rough estimate of the incidence rates of phenylketonuria (PKU), tyrosinemia, and maple syrup ...
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ورودعنوان ژورنال:
- Archives of disease in childhood
دوره 68 3 شماره
صفحات -
تاریخ انتشار 1993