scholarly journals Newborn Screening in Japan—2021

2022 ◽  
Vol 8 (1) ◽  
pp. 3
Author(s):  
Toshihiro Tajima
Keyword(s):  
Amino Acid ◽  
Newborn Screening ◽  
Mass Screening ◽  
Amino Acid Metabolism ◽  
Acid Metabolism ◽  
National Level ◽  
National Project ◽  
Maple Syrup ◽  
Newborn Mass Screening

Japan’s Newborn Mass Screening (NBS) was started in 1977 for amino acid metabolism disorders (phenylketonuria (PKU), homocystinuria, maple syrup urine, histidineemia (discontinued in 1993)) and galactosemia at the national level as a national project [...]

Dietary Management of Inborn Errors of Amino Acid Metabolism With Protein-Modified Diets

1992 ◽  
Vol 7 (1_suppl) ◽  
pp. S92-S111 ◽  
Author(s):  
Enolia Thomas
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Amino Acid Metabolism ◽  
Acid Metabolism ◽  
Propionic Acidemia ◽  
Methylmalonic Aciduria ◽  
Inborn Errors ◽  
Amino Acid Requirement ◽  
Maple Syrup ◽  
Lyase Deficiency

This paper presents experiences encountered with protein-modified diets (PMD) in the management of 67 patients, aged 1 day to 14 years, followed in the Pediatric Nutrition Clinic in the past 5 years. All had inborn errors of amino acid metabolism : maple syrup urine disease (MSUD), 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) lyase deficiency, propionic acidemia (PPA), or methylmalonic aciduria (MMA). In early infancy, the diet prescription is frequently adjusted to search for the infant's tolerance level of restricted amino acids. The levels must be established when natural foods other than milk are added to the PMD. The amino acids restricted are leucine, isoleucine, and valine in MSUD; leucine in HMG-CoA lyase deficiency; and isoleucine, methionine, threonine, and valine in PPA and MMA. Efficacy of the PMD depends on accuracy in prediction of the restricted amino acid requirement and the willingness and ability of parents and patients to conform to its demands. (J Child Neurol 1992;7(Suppl):S92-S111.)

Amino Acid Metabolism

1979 ◽  
Vol 7 (1) ◽  
pp. 261-262
Author(s):  
E. V. ROWSELL
Keyword(s):  
Amino Acid ◽  
Amino Acid Metabolism ◽  
Acid Metabolism

Effect of intravenous bicarbonate administration on postoperative amino acid metabolism

1985 ◽  
Vol 4 ◽  
pp. 141-146 ◽  
Author(s):  
K VESTERBERG ◽  
J BERGSTROM ◽  
P FURST ◽  
U LEANDER ◽  
E VINNARS
Keyword(s):  
Amino Acid ◽  
Amino Acid Metabolism ◽  
Acid Metabolism

Different sensitivity of glucose and amino acid metabolism to insulin in NIDDM

Diabetes ◽  
1993 ◽  
Vol 42 (12) ◽  
pp. 1868-1877 ◽  
Author(s):  
L. Luzi ◽  
A. S. Petrides ◽  
R. A. De Fronzo
Keyword(s):  
Amino Acid ◽  
Amino Acid Metabolism ◽  
Acid Metabolism
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