PROLONGED SURVIVAL IN 3 BROTHERS WITH SEVERE TYPE-2 CRIGLER-NAJJAR SYNDROME - ULTRASTRUCTURAL AND METABOLIC STUDIES

被引:0
|
作者
GOLLAN, JL
HUANG, SN
BILLING, B
SHERLOCK, S
机构
[1] MCGILL UNIV,DEPT PATHOL,MONTREAL,QUEBEC,CANADA
[2] ROY FREE HOSP,DEPT MED,POND ST,LONDON NW3 2Q3,ENGLAND
关键词
D O I
暂无
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
引用
收藏
页码:1543 / 1555
页数:13
相关论文
共 35 条
  • [21] IDENTIFICATION OF AN A-TO-G MISSENSE MUTATION IN EXON-2 OF THE UGT1 GENE-COMPLEX THAT CAUSES CRIGLER-NAJJAR SYNDROME TYPE-2
    MOGHRABI, N
    CLARKE, DJ
    BOXER, M
    BURCHELL, B
    GENOMICS, 1993, 18 (01) : 171 - 173
  • [22] SUPPRESSION OF BILIRUBIN PRODUCTION IN THE CRIGLER-NAJJAR TYPE-I SYNDROME - STUDIES WITH THE HEME OXYGENASE INHIBITOR TIN-MESOPORPHYRIN
    GALBRAITH, RA
    DRUMMOND, GS
    KAPPAS, A
    PEDIATRICS, 1992, 89 (02) : 175 - 182
  • [23] MECHANISMS OF INHERITED DEFICIENCIES OF MULTIPLE UDP-GLUCURONOSYLTRANSFERASE ISOFORMS IN 2 PATIENTS WITH CRIGLER-NAJJAR SYNDROME, TYPE-I
    BOSMA, PJ
    CHOWDHURY, JR
    HUANG, TJ
    LAHIRI, P
    ELFERINK, RPJO
    VANES, HHG
    LEDERSTEIN, M
    WHITINGTON, PF
    JANSEN, PLM
    CHOWDHURY, NR
    FASEB JOURNAL, 1992, 6 (10): : 2859 - 2863
  • [24] THE METABOLIC EFFECT OF HEPATOCYTE TRANSPLANTATION IS INCREASED BY PHARMACOLOGICAL ENHANCEMENT OF DONOR CELL ENGRAFTMENT IN THE GUNN RAT MODEL OF CRIGLER-NAJJAR SYNDROME TYPE 1
    Avsar, Yesim
    Zhou, Hongchao
    Wang, Xia
    Ding, Jianqiang
    Guha, Chandan
    Fox, Ira
    Roy-Chowdhury, Namita
    Roy-Chowdhury, Jayanta
    TRANSPLANT INTERNATIONAL, 2013, 26 : 117 - 117
  • [25] 3 JAPANESE PATIENTS WITH CRIGLER-NAJJAR SYNDROME TYPE-I CARRY AN IDENTICAL NONSENSE MUTATION IN THE GENE FOR UDP-GLUCURONOSYLTRANSFERASE
    KOIWAI, O
    YASUI, Y
    HASADA, K
    AONO, S
    SATO, H
    FUJIKAKE, M
    AOKI, T
    JAPANESE JOURNAL OF HUMAN GENETICS, 1995, 40 (03): : 253 - 257
  • [26] CYCLOPHOSPHAMIDE AND NAPROXEN AUGMENT THE METABOLIC EFFECT OF HEPATOCYTE TRANSPLANTATION IN THE GUNN RAT MODEL OF CRIGLER-NAJJAR SYNDROME TYPE 1 BY ENHANCING DONOR CELL ENGRAFTMENT
    Avsar, Y.
    Zhou, H.
    Wang, X.
    Ding, J.
    Guha, C.
    Fox, I. J.
    Roy-Chowdhury, N.
    Roy-Chowdhury, J.
    JOURNAL OF HEPATOLOGY, 2012, 56 : S38 - S39
  • [27] Bilirubin adsorption therapy and subsequent liver transplantation cured severe bilirubin encephalopathy in a long-term survival patient with Crigler-Najjar disease type I
    Kaneko, K
    Takei, Y
    Aoki, T
    Ikeda, S
    Matsunami, H
    Lynch, S
    INTERNAL MEDICINE, 2000, 39 (11) : 961 - 965
  • [28] COMPARATIVE-STUDY OF 3 METHODS FOR THE ASSESSMENT OF RESERVE BILIRUBIN BINDING-CAPACITY OF SERUM IN CHILDREN WITH TYPE-1 CRIGLER-NAJJAR SYNDROME
    FRANCOUAL, J
    MYARA, A
    TRIVIN, F
    LELUC, R
    ODIEVRE, M
    ARCHIVES FRANCAISES DE PEDIATRIE, 1990, 47 (10): : 721 - 723
  • [29] Paternal uniparental disomy of chromosome 2 resulting in a concurrent presentation of Crigler-Najjar syndrome type I and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency
    Knapp, Anna
    Jagla, Mateusz
    Madetko-Talowska, Anna
    Szewczyk, Katarzyna
    Ksiazek, Teofila
    Konska, Katarzyna
    Kwinta, Przemko
    AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 2022, 188 (06) : 1848 - 1852
  • [30] AAV 1 and 8 have higher efficacy than AAV 2 in correction of hyperbilirubinemia in the Gunn rat, the animal model for the metabolic liver dissorder Crigler-Najjar syndrome
    Bosma, PJ
    de Jong, B
    Looije, N
    Kunne, C
    Bakker, CT
    Seppen, J
    MOLECULAR THERAPY, 2004, 9 : S331 - S332