Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease

被引：22
|
作者：
Wen, Jessica [1 ,2 ]
机构：
[1] Childrens Hosp Philadelphia, Div Gastroenterol Hepatol & Nutr, Philadelphia, PA 19104 USA
[2] Univ Penn, Sch Med, Dept Pediat, Philadelphia, PA 19104 USA
来源：
CTS-CLINICAL AND TRANSLATIONAL SCIENCE | 2011年 / 4卷 / 06期
关键词：
liver disease; pediatrics; cirrhosis; RENIN-ANGIOTENSIN SYSTEM; TISSUE GROWTH-FACTOR; CLINICAL-EXPERIENCE; BILIARY DYSGENESIS; INTRACELLULAR CA2+; MAMMALIAN TARGET; EPITHELIAL-CELLS; LIVER FIBROSIS; DETECT CHANGES; PRIMARY CILIUM;
D O I：
10.1111/j.1752-8062.2011.00306.x
中图分类号：
R-3 [医学研究方法]; R3 [基础医学];
学科分类号：
1001 ;
摘要：
Autosomal recessive polycystic kidney disease (ARPKD) is a developmental disorder that mainly affects the kidneys and the biliary tract. Affected patients often have massively enlarged cystic kidneys as well as congenital hepatic fibrosis (CHF) characterized by dilated bile ducts and associated peribiliary fibrosis. This review will examine what is known about ARPKD-associated liver disease and will highlight areas of ongoing research into its pathogenesis and potential treatment. Clin Trans Sci 2011; Volume 4: 460465
引用
收藏
页码：460 / 465
页数：6
相关论文
共 50 条
  • [21] PKHD1 sequence variations in 78 children and adults with autosomal recessive polycystic kidney disease and congenital hepatic fibrosis
    Gunay-Aygun, Meral
    Tuchman, Maya
    Font-Montgomery, Esperanza
    Lukose, Linda
    Edwards, Hailey
    Garcia, Angelica
    Ausavarat, Surasawadee
    Ziegler, Shira G.
    Piwnica-Worms, Katie
    Bryant, Joy
    Bernardini, Isa
    Fischer, Roxanne
    Huizing, Marjan
    Guay-Woodford, Lisa
    Gahl, William A.
    MOLECULAR GENETICS AND METABOLISM, 2010, 99 (02) : 160 - 173
  • [22] Renin-angiotensin System Activation in Congenital Hepatic Fibrosis in the PCK Rat Model of Autosomal Recessive Polycystic Kidney Disease
    Goto, Miwa
    Hoxha, Nita
    Osman, Rania
    Wen, Jessica
    Wells, Rebecca G.
    Dell, Katherine MacRae
    JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 2010, 50 (06): : 639 - 644
  • [23] Pregnancy in a patient with autosomal-dominant polycystic kidney disease and congenital hepatic fibrosis
    Klinkert, J
    Koopman, MG
    Wolf, H
    EUROPEAN JOURNAL OF OBSTETRICS GYNECOLOGY AND REPRODUCTIVE BIOLOGY, 1998, 76 (01): : 45 - 47
  • [24] CONGENITAL HEPATIC-FIBROSIS IN AUTOSOMAL-DOMINANT POLYCYSTIC KIDNEY-DISEASE
    COBBEN, JM
    BREUNING, MH
    SCHOOTS, C
    TENKATE, LP
    ZERRES, K
    KIDNEY INTERNATIONAL, 1990, 38 (05) : 880 - 885
  • [25] Pathology teach and tell: Congenital hepatic fibrosis and autosomal dominant polycystic kidney disease
    Kaczorowski, JM
    Halterman, JS
    Spitalnik, P
    Mannick, E
    Fong, CT
    Seaman, JM
    PEDIATRIC PATHOLOGY & MOLECULAR MEDICINE, 2001, 20 (03): : 245 - 248
  • [26] Congenital Hepatic Fibrosis in a Case of Autosomal Dominant Polycystic Kidney Disease: A Case Report
    Majethia, Nikhil
    Haria, Pankti
    Patel, R. D.
    Gadgil, Nitin
    Kalgutkar, Alka
    INTERNATIONAL JOURNAL OF SCIENTIFIC STUDY, 2014, 2 (08) : 243 - 245
  • [27] AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE: HEPATIC MANIFESTATIONS IN RELATION TO KIDNEY FAILURE
    Piscopo, G.
    Carbone, V.
    Melchionda, S.
    Gesualdo, L.
    Giordano, M.
    DIGESTIVE AND LIVER DISEASE, 2015, 47 : E254 - E254
  • [28] Autosomal recessive polycystic kidney disease
    Swanson, Kate
    AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY, 2021, 225 (05) : B7 - B8
  • [29] Autosomal recessive polycystic kidney disease
    K. Zerres
    Sabine Rudnik-Schöneborn
    Carsten Steinkamm
    Jutta Becker
    Gabi Mücher
    Journal of Molecular Medicine, 1998, 76 : 303 - 309
  • [30] Autosomal recessive polycystic kidney disease
    Zerres, K
    RudnikSchoneborn, S
    Steinkamm, C
    Mucher, G
    NEPHROLOGY DIALYSIS TRANSPLANTATION, 1996, 11 : 29 - 33
12345