Tyrosinemia type I-like disease : A possible manifestation of 3-oxo-△^4-steroid 5β-reductase deficiency
スポンサーリンク
概要
- 論文の詳細を見る
- 1998-06-01
著者
-
黒澤 隆夫
北海道大学医学部附属病院 検査部
-
KIMURA Akihiko
Department of Pediatrics and Child Health, Kurume University School of Medicine
-
Kurosawa T
Faculty Of Pharmaceutical Sciences Health Sciences University Of Hokkaido
-
Kurosawa Takao
Health Sci. Univ. Hokkaido Hokkaido Jpn
-
Kimura Akihiko
Department Of Pediatrics And Child Health Kurume University School Of Medicine
-
ENDO Fumio
Department of Pediatrics, Kumamoto University Medical School
-
KAGIMOTO Seiichi
Dividion of Infectious Diseases, Immunology, and Allergy, Saitama Children's Medical Center
-
INOUE Toshiro
Department of Pediatrics, and Child Health, Kurume University School of Medicine
-
SUZUKI Mikako
Faculty of Pharmaceutical Sciences, Health Science University of Hokkaido
-
KUROSAWA Takako
Faculty of Pharmaceutical Sciences, Health Science University of Hokkaido
-
TOHMA Masahiko
Faculty of Pharmaceutical Sciences, Health Science University of Hokkaido
-
FUJISAWA Takuji
Department of Pediatrics, and Child Health, Kurume University School of Medicine
-
Suzuki Mikako
Faculty Of Pharmaceutical Sciences Health Science University Of Hokkaido
-
Endo Fumio
Department O Pediatrics Graduate School Of Medical Science Kumamoto University
-
Kato Hirohisa
Department Of Pediatrics And Child Health Kurume University School Of Medicine
-
Fujisawa Takuji
Department Of Pediatrics And Child Health Kurume University School Of Medicine
-
Inoue Toshiro
Department Of Bioscience And Technology Faculty Of Agriculture Iwate University
-
Kimura Akihiko
Department Of Anatomy School Of Medicine Toho University
-
Kagimoto Seiichi
Dividion Of Infectious Diseases Immunology And Allergy Saitama Children's Medical Center
-
Inoue Toshiro
Department Of Pediatrics And Child Health Kurume University School Of Medicine
-
Tohma Masahiko
Faculty Of Pharmaceutical Sciences Health Science University Of Hokkaido
-
Tohma M
Faculty Of Pharmaceutical Sciences Health Science University Of Hokkaido
-
Endo Fumio
Department Of Pediatrics Kumamoto University Medical School
-
Kurosawa Takako
Faculty Of Pharmaceutical Sciences Health Science University Of Hokkaido
-
TOHMA MASAHIKO
Faculty of Pharmaceutical Science, Higashi-Nippon-Gakuen University
関連論文
- 3β-hydroxy-Δ5-C_-steroid dehydrogenase/isomeraze 欠損症の1例
- 21-P3-536 北海道医療大学におけるOSCEトライアルの評価者間による評価の問題点(薬学教育(OSCE),来るべき時代への道を拓く)
- 21-P3-535 北海道医療大学におけるOSCEトライアルの実施と事前学習による教育効果(薬学教育(OSCE),来るべき時代への道を拓く)
- 21H-07 北見地区における病院間連携によるグループ実習の試み(薬学教育(実務実習),来るべき時代への道を拓く)
- Quantitative Determination of Ursodeoxycholic Acid and Its Deuterated Derivative in Human Bile by Gas Chromatography-Mass Fragmentography
- 2型糖尿病患者に対するコレスチミド投与時の血糖降下作用と血中胆汁酸との関係
- 血中クルクミノイドのUV検出高速液体クロマトグラフィーによる分離定量法の開発
- Zellweger 症候群への経口胆汁酸療法
- C型慢性肝炎患者に対するインターフェロンα単独療法とリバビリン併用療法における血球変動の比較
- 尿中胆汁酸分析による胆汁酸異常症の診断
- 先天性胆汁酸代謝異常症の尿中胆汁酸分析依頼システムについて
- 尿中胆汁酸分析(GC/MS)による先天性胆汁酸代謝異常症および関連疾患の診断システム
- 3β-Hydroxy-Δ^5-C_-steroid dehydrogenase/isomerase 欠損症 : 診断と治療
- Maleylacetoacetate 肝細胞蓄積は 3-oxo-Δ^4-steroid 5β-reductase 活性低下をきたすか?
- ステロイド剤が有効であった副腎不全を合併する乳児肝内胆汁うっ滞症 : 二次性3-oxo- Δ^4 -steroid 5 β-reductase欠損症の1例
- 抱合型薬物の代謝研究(第2報)3-デオキシエストラジオール17-サルフェートその2その可能な代謝物の高速液体クロマトグラフィーによる定量法の確立
- 抱合型薬物の代謝研究(第1報)3-デオキシエストラジオール17-サルフェートの可能な代謝産物の合成
- 先天性胆汁酸代謝異常症の尿中胆汁酸分析依頼システム : 本邦における10年間の成績
- VASOACTIVE INTESTINAL POLYPEPTIDE AND CALBINDIN-D28K IMMUNOREACIVE NEURONS IN THE OLFACTORY BULB OF THE MUSK SHREW, SUNCUS MURINUS(Cell Biology and Morphology,Abstracts of papers presented at the 74^ Annual Meeting of the Zoological Society of Japan)
- ガスクロマトグラフィー/質量分析法による胆汁酸生合成中間体の定量法の開発(生体関連機能と分析化学)
- 原発性アルドステロン症における尿中18-ハイドロキシコルチゾーン
- 3β-Hydroxy-Δ^5-C_-steroid dehydrogenase/isomerase deficiency in a 23-year-old woman
- Tyrosinemia type I-like disease : A possible manifestation of 3-oxo-△^4-steroid 5β-reductase deficiency
- Thiolase Involved in Bile Acid Formation^1
- Determination of 3-oxo-Δ^4- and 3-oxo-Δ^-bile acids and related compounds in biological fluids of infants with cholestasis by gas chromatography-mass spectrometry
- Determination of fetal bile acids in biological fluids from neonates by gas chromatography-negative ion chemical ionization mass spectrometry
- STEREOSPECIFIC FORMATION OF (24R, 25R)-3α, 7α, 12α, 24-TETRAHYDROXY-5β-CHOLESTAN-26-OIC ACID CATALYZED WITH A PEROXISOMAL BIFUNCTIONAL D-3-HYDROXYACYL-CoA DEHYDRATASE/D-3-HYDROXYACYL-CoA DEHYDROGENASE
- Physiological Role of D-3-Hydroxyacyl-CoA Dehydratase/D-3-Hydroxyacyl-CoA Dehydrogenase Bifunctional Protein^1
- Capillary Gas Chromatographic Determination of C_-Bile Acids in Biological Samples and Its Application to the Urine of a Patient with Zellweger Syndrome
- Simultaneous Fluorometric Determination of Conjugated Fetal Bile Acids in Urine of Newborns by High-Performance Liquid Chromatography
- Profile Analysis of Bile Acids in Human Fetal and Neonatal Periods by Gas Chromatography-Mass Spectrometry
- Study on Stereospecificity of Enzyme Reaction Related to Peroxisomal Bile Acid Synthesis in Rat Liver
- Novel mutations in five Japanese patients with 3-methylcrotonyl-CoA carboxylase deficiency
- Production Efficiency and Telomere Length of the Cloned Pigs Following Serial Somatic Cell Nuclear Transfer
- Epidemiology of Helicobacter pylori infection in children : A serologic study of the Kyushu region in Japan
- Evaluation of persistence of ductus venosus with Tc-99m DTPA galactosyl human serum albumin liver scintigraphy and I-123 iodoamphetamine per-rectal portal scintigraphy
- Severe brainstem compression by an unruptured giant vertebral aneurysm : An autopsy case
- Distinct efficiencies of Impatiens necrotic spot virus transmission by five thrips vector species (Thysanoptera : Thripidae) of tospoviruses in Japan
- ガスクロマトグラフィー/質量分析法による新生児尿中の3β, 4β, 7α, 12α-テトラヒドロキシ胆汁酸の同定と定量 ( バイオサイエンスと分析化学)
- 胎児性胆汁酸測定における3α-ヒドロキシステロイドデヒドロゲナーゼの特異性 ( バイオサイエンスと分析化学)
- Synthesis of 19-HYdroxylated Bile Acids and Identification of 3α, 7α, 12α, 19-Tetrahydroxy-5β-cholan-24-oic Acid in Human Neonatal Urine
- SYNTHESIS OF 6-HYDROXYLATED BILE ACIDS AND IDENTIFICATION OF 3α, 6α, 7α, 12α-TETRAHYDROXY-5β-CHOLAN-24-OIC ACID IN HUMAN MECONIUM AND NEONATAL URINE
- Helicobacter pylori and TT virus prevalence in Japanese children
- Perinatal bile acid metabolism : bile acid analysis of meconium of preterm and full-term infants
- Acute severe hepatitis : Successful prevention of fulminant hepatic failure with early intensive medical therapy
- Two neonates with intussusception accompanying severe hypoxia
- Abnormally low ratio of cholic acid to chenodeoxycholic acid due to a deficiency of 3-oxo-Δ^4-steroid 5β-reductase
- Guidelines for care of pregnant women carrying hepatitis C virus and their infants
- Measurement of Transport Activities of Bile Acids in Human Multidrug Resistance-Associated Protein 3 Using Liquid Chromatography-Tandem Mass Spectrometry
- Measurement of the Transport Activities of Bile Salt Export Pump Using LC-MS
- An improved HPLC assay for phosphatidylcholine hydroperoxides (PCOOH) in human plasma with synthetic PCOOH as internal standard
- Determination of Regioisomeric Hydroperoxides of Fatty Acid Cholesterol Esters Produced by Photosensitized Peroxidation Using HPLC
- A possible mechanism of neonatal intrahepatic cholestasis caused by citrin deficiency
- FIBROMUSCULAR LAYER IN THE RESPIRATORY TRACT OF RED-BELLIED NEWT (CYNOPS PYRRHOGASTER)(Cell Biology and Morphology,Abstracts of papers presented at the 75^ Annual Meeting of the Zoological Society of Japan)
- DISTRIBUTION OF NEE CELLS AND NEBS IN THE RESPIRATORY TRACT OF RED-BELLIED NEWT (CYNOPS PYRRHOGASTER)(Cell Biology and Morphology,Abstracts of papers presented at the 74^ Annual Meeting of the Zoological Society of Japan)
- LOCALIZATION OF SEROTONIN-IMMUNOREACTIVE CELLS IN THE RESPIRATORY TRACT OF URODELA : THE TAILED AMPHIBIANS(Cell Biology and Morphology)(Proceedings of the Seventy-Third Annual Meeting of the Zoological Society of Japan)
- Gene expression of cytokines and growth factors in the lungs after paraquat administration in mice
- 潰瘍性大腸炎患児における血球浄化療法
- Helicobacter pylori seropositivity in patients with severe neurologic impairment
- DETERMINATION OF THE UNUSUAL BILE ACID, 3β, 12α-DI-HYDROXY-5-CHOLENOIC ACID IN HUMAN SERUM BY GAS CHROMATOGRAPHY-MASS SPECTROMETRY
- Synthesis of the 1β-Hydroxylated Bile Acids, Unusual Bile Acids in Human Biological Fluids
- SYNTHESIS OF THE 1β-HYDROXYLATED BILE ACIDS AND IDENTIFICATION OF 1β, 3α, 7α-TRIHYDROXY-AND 1β, 3α, 7α, 12α-TETRAHYDROXY-5β-CHOLAN-24-OIC ACIDS IN HUMAN MECONIUM
- Mass screening for Wilson's disease : Results and recommendations
- Newborn hearing screening in a single private Japanese obstetric hospital
- AMELIORATION OF PROLIDASE DEFICIENCY IN FIBROBLASTS USING ADENOVIRUS MEDIATED GENE TRANSFER
- An infant with 3 β -hydroxy-△^5-C_-steroid dehydrogenase/isomerase deficiency presenting with typical neonatal hepatitis syndrome : The first Japanese case
- 110 A CASE OF INSULIN ALLERGY WITH HIGH LEVEL OF SPECIFIC IGE FOR HUMAN INSULIN
- A ^C-urea breath test in children with Helicobacter pylori infection : assessment of eradication therapy and follow-up after treatment
- Histological findings in the livers of patients with neonatal intrahepatic cholestasis caused by citrin deficiency
- 44 A CASE OF CONGENITAL HYPOALDOSTERONISM CAUSED BY A MUTATION OF CYP11B2 GENE
- Serum hyaluronate level correlates to the degree of liver fibrosis in pediatric liver diseases
- Determination of Peroxidase with a Chemiluminescence Reaction of Homogentisic Acid γ-Lactone in Non-Aqueous Media
- Synthesis of 12α and 12β-Carboxyestradiol Derivatives via the Thermal Elimination of β-Ketosulfoxide
- A novel nonsense mutation of the PEPD gene in a Japanese patient with prolidase deficiency
- Urinary 1β-hydroxyursodeoxycholic acid during ursodeoxycholic acid therapy
- Transient Expression of a Brain/Embryonic-Type Myosin Heavy Chain Isoform (MIIB2) in Regenerating Rat Liver
- Structure of the respiratory tract of the red-bellied newt Cynops pyrrhogaster, with reference to serotonin-positive neuroepithelial endocrine cells
- Apoptosis under hypercytokinemia is a possible pathogenesis in influenza-associated encephalopathy
- Effect of omeprazole on the course of associated esophagitis and laryngitis
- QUANTITATIVE DETERMINATION OF CONJUGATED FETAL BILE ACIDS IN URINE BY CHEMILUMINESCENCE HIGH-PERFORMANCE LIQUID CHROMATOGRAPHY
- Measurement of Bile Salt Export Pump Transport Activities using a Fluorescent Bile Acid Derivative
- 強力ネオミノファーゲンC投与肝疾患患児の尿中グリチルリチン酸とグリチルレチン酸の同定
- 乾燥濾紙尿を用いたGC-MSによる胆汁酸分析 : 先天性胆汁酸代謝異常症への応用
- A Convenient Synthesis of Estrone and 11-Methylequilenines based on the Thermal Elimination of β-Ketosulfoxides
- 小児における尿中胆汁酸の発達
- Synthesis of C-2 Catecholic Equilin and Equilenin Derivatives for Use in Metabolic Studies
- Analyses for phosphatidylcholine hydroperoxides by LC/MS
- The cDNA sequence and expression of the AAA-family peroxin genes pex-1 and pex-6 from the nematode Caenorhabditis elegans
- 早期産児における尿中胆汁酸組成の発達的推移
- 生体成分の分析--胆汁酸
- Specificity of Antisera Raised against Estradiol 12-Bovine Serum Albumin Conjugates
- Construct Validity of Functional Balance Scale in Stroke Inpatients
- Total Synthesis of 12α- and 12β-Carboxylated Estrogens via the Thermal Elimination of β-Ketosulfoxide(Organic,Chemical)
- Complete absence of bile and pancreatic ducts in a newborn : a new entity of congenital anomaly in hepato-pancreatic development
- PFIC1型および2型における肝機能検査値と尿中胆汁酸分析
- エクソン・スキッピング誘導による治療の臨床応用に向けて、適正アンチセンスと適応患者のスクリーニング法
- 胎便中胆汁酸分析における検体採取法の検討
- 新生児ヘモクロマトーシス8例における尿中胆汁酸分析値
- Clinical application of array-based comparative genomic hybridization by two-stage screening for 536 patients with mental retardation and multiple congenital anomalies
- Cerebral hemorrhage in Fabry's disease