Bile acid synthetic defects and liver disease

被引:37
作者
Bove, KE
Daugherty, CC
Tyson, W
Mierau, G
Heubi, JE
Balistreri, WF
Setchell, KDR
机构
[1] Childrens Hosp, Med Ctr, Div Pediat Pathol, Cincinnati, OH 45229 USA
[2] Univ Cincinnati, Coll Med, Cincinnati, OH 45229 USA
[3] Childrens Hosp, Med Ctr, Div Mass Spect, Cincinnati, OH 45229 USA
[4] Childrens Hosp, Med Ctr, Div Pediat Gastroenterol, Cincinnati, OH 45229 USA
[5] Denver Childrens Hosp, Dept Pathol, Denver, CO 80218 USA
关键词
bile acid synthesis; metabolic defects; liver disease; neonatal hepatitis; fat-soluble vitamin deficiency;
D O I
10.1007/s100240050001
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
[No abstract available]
引用
收藏
页码:1 / 16
页数:16
相关论文
共 38 条
[1]  
AGAMANOLIS D, 1988, PEDIATR PATHOL, V6, P657
[2]  
BALISTRERI W, 1999, SCHIFFS DIS LIVER, P1415
[3]  
Balistreri WF, 1995, FALK SYMP, V80, P333
[4]  
BOWEN P, 1964, B JOHNS HOPKINS HOSP, V114, P402
[5]   LACK OF 3-BETA-HYDROXY-DELTA-5-C-27-STEROID DEHYDROGENASE ISOMERASE IN FIBROBLASTS FROM A CHILD WITH URINARY-EXCRETION OF 3-BETA-HYDROXY-DELTA-5-C-27-BILE ACIDS - A NEW INBORN ERROR OF METABOLISM [J].
BUCHMANN, MS ;
KVITTINGEN, EA ;
NAZER, H ;
GUNASEKARAN, T ;
CLAYTON, PT ;
SJOVALL, J ;
BJORKHEM, I .
JOURNAL OF CLINICAL INVESTIGATION, 1990, 86 (06) :2034-2037
[6]   FAMILIAL GIANT-CELL HEPATITIS ASSOCIATED WITH SYNTHESIS OF 3-BETA, 7-ALPHA-DIHYDROXY-5-CHOLENOIC AND 3-BETA, 7-ALPHA, 12-ALPHA-TRIHYDROXY-5-CHOLENOIC ACIDS [J].
CLAYTON, PT ;
LEONARD, JV ;
LAWSON, AM ;
SETCHELL, KDR ;
ANDERSSON, S ;
EGESTAD, B ;
SJOVALL, J .
JOURNAL OF CLINICAL INVESTIGATION, 1987, 79 (04) :1031-1038
[7]   FAMILIAL GIANT-CELL HEPATITIS WITH LOW BILE-ACID CONCENTRATIONS AND INCREASED URINARY-EXCRETION OF SPECIFIC BILE ALCOHOLS - A NEW INBORN ERROR OF BILE-ACID SYNTHESIS [J].
CLAYTON, PT ;
CASTEELS, M ;
MIELIVERGANI, G ;
LAWSON, AM .
PEDIATRIC RESEARCH, 1995, 37 (04) :424-431
[8]   Ataxia associated with increased plasma concentrations of pristanic acid, phytanic acid and C-27 bile acids but normal fibroblast branched-chain fatty acid oxidation [J].
Clayton, PT ;
Johnson, AW ;
Mills, KA ;
Lynes, GW ;
Wilson, J ;
Casteels, M ;
Mannaerts, G .
JOURNAL OF INHERITED METABOLIC DISEASE, 1996, 19 (06) :761-768
[9]   CEREBRO-HEPATO-RENAL SYNDROME OF ZELLWEGER - REPORT OF 8 CASES WITH COMMENTS UPON INCIDENCE, LIVER LESION, AND A FAULT IN PIPECOLIC ACID METABOLISM [J].
DANKS, DM ;
TIPPETT, P ;
ADAMS, C ;
CAMPBELL, P .
JOURNAL OF PEDIATRICS, 1975, 86 (03) :382-387
[10]   RESOLUTION OF LIVER-BIOPSY ALTERATIONS IN 3 SIBLINGS WITH BILE-ACID TREATMENT OF AN INBORN ERROR OF BILE-ACID METABOLISM (DELTA(4)-3-OXOSTEROID 5-BETA-REDUCTASE DEFICIENCY) [J].
DAUGHERTY, CC ;
SETCHELL, KDR ;
HEUBI, JE ;
BALISTRERI, WF .
HEPATOLOGY, 1993, 18 (05) :1096-1101