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Study on the Development of the Therapeutic Formula for Inborn Errors of Amino Acid Metabolism Consisting of Synthetic Peptide as a Nitrogen Source

Research Project

Project/Area Number 62870040
Research Category

Grant-in-Aid for Developmental Scientific Research

Allocation TypeSingle-year Grants
Research Field Pediatrics
Research InstitutionNihon University

Principal Investigator

KITAGAWA Teruo  Nihon University, School of Medicine Department of Pediatrics Professor, 医学部小児科, 教授 (50058765)

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)
Project Period (FY) 1987 – 1989
Project Status Completed (Fiscal Year 1989)
Budget Amount *help
¥12,100,000 (Direct Cost: ¥12,100,000)
Fiscal Year 1989: ¥4,100,000 (Direct Cost: ¥4,100,000)
Fiscal Year 1988: ¥4,000,000 (Direct Cost: ¥4,000,000)
Fiscal Year 1987: ¥4,000,000 (Direct Cost: ¥4,000,000)
KeywordsInborn errors of metabolism / low-phenylalanine peptide / PKU / Maternal PKU / Dietary treatment / Formula consisting of low Phe-peptide
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.

Report

(3 results)
  • 1989 Annual Research Report   Final Research Report Summary
  • 1988 Annual Research Report
  • Research Products

    (24 results)

All Other

All Publications (24 results)

  • [Publications] Arai S.et al: "Physicochemical properties of a low-phenylalanine peptide substance as a foodstuff for patients with phenylketonuria" Agric.Biol.Chem.52. 287-288 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Owada M.et al: "Successful treatment of maternal phenylket onuria with a formula consisting of low phenylalanine peptide as a protein source" J.Inher.Metab.Dis.11. 341-344 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Owada M.et al: "Dietary treatment of PKU using a low phenylalanine peptide milk" Acta.Paediatr.Jpn.30. 405-409 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Aoki K.et al: "Outcome of the patients detected by newborn screening in Japan" Acta.Paediatr.Jpn.30. 429-434 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] 青木菊麿,他: "特殊粉乳" 周産期医学. 19. 99-102 (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] 大和田操: "先天性代謝異常症と食事-フェニルケトン尿症を中心に-" 日本臨床栄養学会雑誌. 10. 62-66 (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] 北川照男(桐山 荒井編): "ペプチド栄養" 北海道大学図書刊行会, (1990)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Arai S. et al: "Physicochemical properties of a low-phenylalanine peptide substance as a foodstuff for patients with phenylketonuria." Agric. Biol. Chem.52. 287-288 (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Owada M. et al: "Successful treatment of maternal phenylketonuria with a formula consisting of low phenylalanine peptide as a protein source." J. Inher. Metab. Dis.11. 341-344 (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Owada M. et al: "Dietary treatment of PKU using a low phenylalanine peptide milk." Acta. Paediatr. Jpn.30. 405-409 (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Aoki K. et al: "Outcome of the patients detected by newborn screening in Japan." Acta. Paediatr. Jpn.30. 429-434 (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] 大和田操: "アミノ酸代謝異常症の新生児マス・スクリ-ニング" 周産期医学. 19. 665-668 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 北川照男: "小児科領域における食事療法の歩みと今後の方向" New Diet Therapy. 5. 216-226 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 北川照男,大和田操: "先天性アミノ酸代謝異常症の食事療法ー最近の進歩ー" 日本臨床栄養学会雑誌. 12. (1990)

    • Related Report
      1989 Annual Research Report
  • [Publications] 大和田操: "先天性代謝異常症と食事" 日本臨床栄養学会雑誌. 10. 62-66 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 青木菊麿: "特殊粉乳" 周産期医学. 19. 99-102 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 青木菊麿: "先天性代謝異常症と新生児マス・スクリ-ニング" 神経の進歩. 33. 799-808 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 北川照男ほか10名: "ペプチド栄養" 北海道大学図書刊行会, (1990)

    • Related Report
      1989 Annual Research Report
  • [Publications] OWADA M.: Acta Paediatr Jpn. 30. 405-409 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] OWADA M.: J.Inher.Metab.Dis.11. 341-344 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] 崎山武志: 小児科. 29. 1-10 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] 北川照男: 小児科診察. 51. 1529-1537 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] 北川照男: 小児科診察. 51. 769-774 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] 北川照男: 臨床検査. 32. 1103-1110 (1988)

    • Related Report
      1988 Annual Research Report

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Published: 1987-04-01   Modified: 2016-04-21  

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