SECONDARY CARNITINE DEFICIENCY IN HYPERAMMONEMIC ATTACKS OF ORNITHINE TRANSCARBAMYLASE DEFICIENCY

被引:49
作者
OHTANI, Y
OHYANAGI, K
YAMAMOTO, S
MATSUDA, I
机构
[1] KUMAMOTO UNIV, SCH MED, DEPT PEDIAT, KUMAMOTO 860, JAPAN
[2] KUMAMOTO UNIV, SCH MED, DEPT CHILD DEV, KUMAMOTO 860, JAPAN
[3] SAPPORO MED COLL, DEPT PEDIAT, SAPPORO, HOKKAIDO 060, JAPAN
[4] CHIBA UNIV, SCH MED, DEPT PEDIAT, CHIBA, JAPAN
关键词
D O I
10.1016/S0022-3476(88)80321-4
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
引用
收藏
页码:409 / 414
页数:6
相关论文
共 30 条
[1]   RISK OF SERIOUS ILLNESS IN HETEROZYGOTES FOR ORNITHINE TRANSCARBAMYLASE DEFICIENCY [J].
BATSHAW, ML ;
MSALL, M ;
BEAUDET, AL ;
TROJAK, J .
JOURNAL OF PEDIATRICS, 1986, 108 (02) :236-241
[2]   TREATMENT OF INBORN-ERRORS OF UREA SYNTHESIS - ACTIVATION OF ALTERNATIVE PATHWAYS OF WASTE NITROGEN SYNTHESIS AND EXCRETION [J].
BATSHAW, ML ;
BRUSILOW, S ;
WABER, L ;
BLOM, W ;
BRUBAKK, AM ;
BURTON, BK ;
CANN, HM ;
KERR, D ;
MAMUNES, P ;
MATALON, R ;
MYERBERG, D ;
SCHAFER, IA .
NEW ENGLAND JOURNAL OF MEDICINE, 1982, 306 (23) :1387-1392
[3]  
BROWN GW, 1959, J BIOL CHEM, V234, P1769
[4]   URINARY-EXCRETION OF L-CARNITINE AND ACYLCARNITINES BY PATIENTS WITH DISORDERS OF ORGANIC-ACID METABOLISM - EVIDENCE FOR SECONDARY INSUFFICIENCY OF L-CARNITINE [J].
CHALMERS, RA ;
ROE, CR ;
STACEY, TE ;
HOPPEL, CL .
PEDIATRIC RESEARCH, 1984, 18 (12) :1325-1328
[5]   EFFECTS OF L-CARNITINE ON UREA SYNTHESIS FOLLOWING ACUTE AMMONIA INTOXICATION IN MICE [J].
COSTELL, M ;
OCONNOR, JE ;
MIGUEZ, MP ;
GRISOLIA, S .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1984, 120 (03) :726-733
[6]   CARNITINE METABOLISM AND INBORN-ERRORS [J].
ENGEL, AG ;
REBOUCHE, CJ .
JOURNAL OF INHERITED METABOLIC DISEASE, 1984, 7 :38-43
[7]   PRIMARY SYSTEMIC CARNITINE DEFICIENCY .2. RENAL HANDLING OF CARNITINE [J].
ENGEL, AG ;
REBOUCHE, CJ ;
WILSON, DM ;
GLASGOW, AM ;
ROMSHE, CA ;
CRUSE, RP .
NEUROLOGY, 1981, 31 (07) :819-825
[8]  
HASS R, 1981, NEUROLOGY, V31, P1473
[9]   EVIDENCE OF INHERITED UREA CYCLE DEFECT IN A CASE OF FATAL VALPROATE TOXICITY [J].
HJELM, M ;
DESILVA, LVK ;
SEAKINS, JWT ;
OBERHOLZER, VG ;
ROLLES, CJ .
BRITISH MEDICAL JOURNAL, 1986, 292 (6512) :23-24
[10]  
KAY JDS, 1986, LANCET, V2, P1283