1989 Fiscal Year Final Research Report Summary
Study on the Development of the Therapeutic Formula for Inborn Errors of Amino Acid Metabolism Consisting of Synthetic Peptide as a Nitrogen Source
| Project/Area Number |
62870040
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| Research Category |
Grant-in-Aid for Developmental Scientific Research
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Nihon University |
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Principal Investigator |
KITAGAWA Teruo Nihon University, School of Medicine Department of Pediatrics Professor, 医学部小児科, 教授 (50058765)
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| Co-Investigator(Kenkyū-buntansha) |
MATSUDA Ichiro Kumamoto University, School of Medicine Department of Pediatrics Professor, 医学部小児科, 教授 (10000986)
HASE Yutaka Osaka City Child Health Center Department of Internal Medicine Head of Departmen, 第一内科, 医長
IGARASHI Yutaka Tohoku University, School of Medicine Department of Pediatrics Assistant Profess, 医学部小児科, 講師 (70101144)
ARAI Soichi Tokyo University, Faculty of Agriculture Department of Agricultural Chemistry As, 農学部農芸化学科, 助教授 (20011934)
AOKI Kikumaro Aiiku Maternal and Child Health Center Head of Research Development Department (20056584)
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| Project Period (FY) |
1987 – 1989
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| Keywords | Inborn errors of metabolism / low-phenylalanine peptide / PKU / Maternal PKU / Dietary treatment / Formula consisting of low Phe-peptide |
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| Research Abstract |
During the 3 year period, 260 kg of low-phenylalanine peptides (LPP) were obtained by enzymic hydrolysis of whey protein and 1350 kg of therapeutic formula for PKU consisting of LPP as a nitrogen source were prepared. In animal experiments we confirmed that LPPs were more rapidly absorbed than amino acids. Because the palatability as well as nutritional quality seventeen infants and young children with PKU were treated with LPP formula for more than one year. Five of them have been on LPP formula for more than 4 years. In four of 17 cases who have been on LPP formula serum phenylalanine (Phe) levels were difficult to maintain below 8 mg/dl because lOOg of LPP formula contain 69 mg of Phe. In those cases, a mixture of 19 kinds of amino acids except Phe were supplemented. Normal growth was observed in all these infants and children. DQ or IQ were all within the normal range. The serum ami no acid levels in these cases were compatible to those during treatment with conventional therapeutic formula consisting of amino acids as a nitrogen source. Previously, we succeeded to have the first case of maternal PKU where a normal baby was born following dietary treatment using the LPP formula started in the preconceptional period. This woman expected to have a second baby and she received LPP formula and solid food cooked with LPP powder prior to and during pregnancy. Her blood Phe levels maintained below less than 10 mg/dl. The 2nd baby was born without any complication related to the maternal PKU. Development of LPP powder and formula resulted not only a better prognosis of infants and children with PKU but also successful prevention of maternal PKU in Japan.
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