NEONATAL HYPERAMMONEMIA WITH COMPLETE ABSENCE OF LIVER CARBAMYL-PHOSPHATE SYNTHETASE-ACTIVITY

被引:21
|
作者
MANTAGOS, S
TSAGARAKI, S
BURGESS, EA
OBERHOLZER, V
PALMER, T
SACKS, J
BAIBAS, S
VALAES, T
机构
[1] ST SOPHIA CHILDRENS HOSP,INST CHILD HLTH,ATHENS,GREECE
[2] QUEEN ELIZABETH HOSP CHILDREN,DEPT BIOCHEM,LONDON E2 8PS,ENGLAND
关键词
D O I
10.1136/adc.53.3.230
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
引用
收藏
页码:230 / 234
页数:5
相关论文
共 50 条
  • [1] EFFECT OF ARGININE ON CARBAMYL-PHOSPHATE SYNTHETASE-ACTIVITY
    GOODMAN, MW
    HEPATOLOGY, 1982, 2 (05) : 677 - 677
  • [2] LETHAL NEONATAL HYPERAMMONEMIA SECONDARY TO CARBAMYL-PHOSPHATE SYNTHETASE DEFICIENCY
    GELEHRTE.TD
    SNODGRAS.PJ
    AMERICAN JOURNAL OF HUMAN GENETICS, 1973, 25 (06) : A29 - A29
  • [3] RAPID PREPARATION OF ORNITHINE CARBAMYL TRANSFERASE WITHOUT CARBAMYL-PHOSPHATE SYNTHETASE-ACTIVITY
    RABIER, D
    CATHELINEAU, L
    KAMOUN, P
    ANNALES DE BIOLOGIE CLINIQUE, 1978, 36 (05) : 449 - 451
  • [4] LETHAL NEONATAL DEFICIENCY OF CARBAMYL-PHOSPHATE SYNTHETASE
    GELEHRTER, TD
    SNODGRASS, PJ
    NEW ENGLAND JOURNAL OF MEDICINE, 1974, 290 (08): : 430 - 433
  • [5] TREATMENT OF HYPERAMMONEMIA COMA IN CARBAMYL-PHOSPHATE SYNTHETASE (CPS) DEFICIENCY
    THIRSK, J
    ROBSON, WL
    SNYDER, F
    PARSONS, H
    CLINICAL AND INVESTIGATIVE MEDICINE-MEDECINE CLINIQUE ET EXPERIMENTALE, 1985, 8 (03): : A172 - A172
  • [6] LETAL NEONATAL DEFICIENCY OF CARBAMYL-PHOSPHATE SYNTHETASE
    PLOCHL, E
    BACHMANN, C
    STOLLINGER, O
    COLOMBO, JP
    RASSEM, T
    CZIHAK, G
    PADIATRIE UND PADOLOGIE, 1982, 17 (02): : 371 - 382
  • [7] HYPERORNITHINEMIA, HYPERAMMONEMIA, AND HOMOCITRULLINURIA ASSOCIATED WITH DECREASED CARBAMYL-PHOSPHATE SYNTHETASE-I ACTIVITY
    GATFIELD, PD
    TALLER, E
    WOLFE, DM
    HAUST, MD
    PEDIATRIC RESEARCH, 1975, 9 (05) : 488 - 497
  • [8] MASKING OF CARBAMYL-PHOSPHATE SYNTHETASE-ACTIVITY IN TOXOPLASMA-GONDII - EVIDENCE FOR AN ENDOGENOUS INHIBITOR
    OSULLIVAN, WJ
    ASAI, T
    KOBAYASHI, M
    TATIBANA, M
    BIOCHEMISTRY INTERNATIONAL, 1981, 3 (01): : 81 - 87
  • [9] CARBAMYL-PHOSPHATE SYNTHETASE DEFICIENCY WITH LETHAL NEONATAL OUTCOME
    JAEKEN, J
    DEVLIEGER, H
    BACHMANN, C
    VANAERDE, J
    CORBEEL, L
    EGGERMONT, E
    EUROPEAN JOURNAL OF PEDIATRICS, 1982, 139 (01) : 72 - 75
  • [10] NEONATAL DEATH DUE TO CARBAMYL-PHOSPHATE SYNTHETASE DEFICIENCY
    WILSON, RG
    MASTERS, PL
    AUSTRALIAN PAEDIATRIC JOURNAL, 1977, 13 (02): : 119 - 121