| Project/Area Number |
03670512
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Grant-in-Aid for Scientific Research (C) |
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Principal Investigator |
OWADA Misao NIHON Univ. School of Med., Associate Professor, 医学部, 助教授 (40059506)
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| Co-Investigator(Kenkyū-buntansha) |
YOSHIDA Yasuyoshi NIHON Univ. School of Med., Assistant, 医学部, 助手 (60240321)
SAKIYAMA Takeshi NIHON Univ. School of Med., Instructor, 医学部, 講師 (20130510)
岩瀬 宗則 日本大学, 医学部. 小児科, 助手 (90223396)
中林 啓記 日本大学, 医学部. 小児科, 助手 (50237369)
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| Project Period (FY) |
1991 – 1993
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| Project Status |
Completed (Fiscal Year 1993)
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| Budget Amount *help |
¥2,000,000 (Direct Cost: ¥2,000,000)
Fiscal Year 1993: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 1992: ¥600,000 (Direct Cost: ¥600,000)
Fiscal Year 1991: ¥900,000 (Direct Cost: ¥900,000)
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| Keywords | tetrahydrobiopterin (BH_4) / (2) BH_4 deficiency / cerebrospinal fluid (CSF) / BH_4 in CSF / BH_4 therapy / フエニルアラニン水酸化反応 / プテリン化合物 / Fukushima-Nixon法 / 髄液移行 / フェルアラニン水酸化反応 / フェニルケトン尿症 / FukushimaーNixon法 |
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| Research Abstract |
Detection of tetrahydrobiopterin (BH_49 deficiency amongnewborn infants with elevated blood phenylalanine (Phe) is required for the early treatment of this disorder. To differentiate BH_4 deficiency from classical phenylketonuria (PKU), analysis of pterin compounds in urine and/or blood is essential. in addition, measurement of pterins in cerebrospinal fluid (CSF) is important for evaluating the effect BH_4 therapy. The Fukushima-Nixon method has been widly used as a method for determination of pterins in body fluid ; however, BH_4 cannot be mesured directly by this method. This method also seems not to be sensitive enough for the determination of pterins in CSF.We have developed a method for dirct determination of BH_4 in urine as well as CSF.By using this method, pterin compounds such as neopterin, biopterin, pterin, BH_2 as well as BH_4 are wel separated and BH_4 was detectable not only in urine specimen but also in CSF from control subject without any pretreatment. In order to make an early diagnosis of BH_4 deficiency in newborn period, this simple and sensitive method is very useful.
In Japan, a method to synthesize R-BH_4 has been developed in recent years, and this product has been used for the treatment of BH_4 deficiency. We have been followed up 4 cases with BH_4 deficiency using the product. To evaluate the BH_4 treatment for the patients, measurement of BH_4 in urine as well as CSF have carried out and this method is also useful for the evaluation of BH_4 therapy.
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