学术探索
学术期刊
文章热点
数据分析
智能智评

Assessment of liver disease in cystic fibrosis

被引：12
作者：
Davison, Suzanne [1 ]
机构：
[1] Leeds Teaching Hosp NHS Trust, Leeds Childrens Hosp, Great George St, Leeds LS1 3EX, W Yorkshire, England
来源：
PAEDIATRIC RESPIRATORY REVIEWS | 2018年 / 27卷
关键词：
Cystic fibrosis liver disease; Portal hypertension; Oesophageal varices; Liver transplantation; TRANSIENT ELASTOGRAPHY; IMPROVEMENT; STIFFNESS; CHILDREN;
D O I：
10.1016/j.prrv.2018.05.010
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
Liver disease in cystic fibrosis has many causes, with biliary fibrosis due to abnormal CFTR protein predominating. Assessment requires aetiology to be defined. Biliary fibrosis may progress to cirrhosis and portal hypertension, which although initially asymptomatic, may cause varices and splenomegaly. Monitoring progression includes clinical and ultrasound assessment with endoscopic assessment of varices for those at risk. Extrapolated primarily from longitudinal assessment of viral hepatitis in adults, non-invasive elastography has a potential role. Evidence is lacking to support intervention strategies, but ursodeoxycholic acid and ligation of varices are widely applied. Indication and timing of liver transplantation are not clearly defined. Multidisciplinary approach is needed to tailor assessment and guide management. (C) 2018 Elsevier Ltd. All rights reserved.
引用
收藏
页码：24 / 27
页数：4
相关论文
共 20 条
  • [1] Correlation of Transient Elastography With Severity of Cystic Fibrosis-related Liver Disease
    Aqul, Amal
    Jonas, Maureen M.
    Harney, Sarah
    Raza, Roshan
    Sawicki, Gregory S.
    Mitchell, Paul D.
    Fawaz, Rima
    [J]. JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 2017, 64 (04) : 505 - 511
  • [2] Genetic Modifiers of Liver Disease in Cystic Fibrosis
    Bartlett, Jaclyn R.
    Friedman, Kenneth J.
    Ling, Simon C.
    Pace, Rhonda G.
    Bell, Scott C.
    Bourke, Billy
    Castaldo, Giuseppe
    Castellani, Carlo
    Cipolli, Marco
    Colombo, Carla
    Colombo, John L.
    Debray, Dominique
    Fernandez, Adriana
    Lacaille, Florence
    Macek, Milan, Jr.
    Rowland, Marion
    Salvatore, Francesco
    Taylor, Christopher J.
    Wainwright, Claire
    Wilschanski, Michael
    Zemkova, Dana
    Hannah, William B.
    Phillips, M. James
    Corey, Mary
    Zielenski, Julian
    Dorfman, Ruslan
    Wang, Yunfei
    Zou, Fei
    Silverman, Lawrence M.
    Drumm, Mitchell L.
    Wright, Fred A.
    Lange, Ethan M.
    Durie, Peter R.
    Knowles, Michael R.
    Clancy, J. P.
    Sindel, L. J.
    Roberts, D. M.
    Roberts, V.
    Radford, P. J.
    Argel, N.
    Morgan, W. J.
    Douthit, J. L.
    Schellhase, D. E.
    Anderson, P.
    Taggart, A.
    Morrissey, B.
    Platzker, A. C. G.
    Woo, M. S.
    Fukushima, L.
    Hsu, E.
    [J]. JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION, 2009, 302 (10): : 1076 - 1083
  • [3] Ursodeoxycholic acid for cystic fibrosis-related liver disease (Review)
    Cheng, Katharine
    Ashby, Deborah
    Smyth, Rosalind L.
    [J]. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, 2017, (09):
  • [4] Platelet count, spleen length, and platelet count-to-spleen length ratio for the diagnosis of oesophageal varices in people with chronic liver disease or portal vein thrombosis
    Colli, Agostino
    Cristobal Gana, Juan
    Yap, Jason
    Adams-Webber, Thomasin
    Rashkovan, Natalie
    Ling, Simon C.
    Casazza, Giovanni
    [J]. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, 2017, (04):
  • [5] Cystic Fibrosis-related Liver Disease: Research Challenges and Future Perspectives
    Debray, Dominique
    Narkewicz, Michael R.
    Bodewes, Frank A. J. A.
    Colombo, Carla
    Housset, Chantal
    de Jonge, Hugo R.
    Jonker, Johan W.
    Kelly, Deirdre A.
    Ling, Simon C.
    Poynard, Thierry
    Sogni, Philippe
    Trauner, Michael
    Witters, Peter
    Baumann, Ulrich
    Wilschanski, Michael
    Verkade, Henkjan J.
    [J]. JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 2017, 65 (04) : 443 - 448
  • [6] Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease
    Debray, Dominique
    Kelly, Deirdre
    Houwen, Roderick
    Strandvik, Birgitta
    Colombo, Carla
    [J]. JOURNAL OF CYSTIC FIBROSIS, 2011, 10 : S29 - S36
  • [7] Improvement of Hepatic Steatosis in Cystic Fibrosis With Ivacaftor Therapy
    Hayes, Don, Jr.
    Warren, Patrick S.
    McCoy, Karen S.
    Sheikh, Shahid I.
    [J]. JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 2015, 60 (05) : 578 - 579
  • [8] Utility of transient elastography in the non-invasive evaluation of cystic fibrosis liver disease
    Kitson, Matthew T.
    Kemp, William W.
    Iser, David M.
    Paul, Eldho
    Wilson, John W.
    Roberts, Stuart K.
    [J]. LIVER INTERNATIONAL, 2013, 33 (05) : 698 - 705
  • [9] Assessment of pathologic increase in liver stiffness enables earlier diagnosis of CFLD: Results from a prospective longitudinal cohort study
    Klotter, Victoria
    Gunchick, Caroline
    Siemers, Enno
    Rath, Timo
    Hudel, Helge
    Naehrlich, Lutz
    Roderfeld, Martin
    Roeb, Elke
    [J]. PLOS ONE, 2017, 12 (06):
  • [10] Pilot study on the use of acoustic radiation force impulse imaging in the staging of cystic fibrosis associated liver disease
    Manco, Melania
    Lo Zupone, Cristina
    Alghisi, Federico
    D'Andrea, Maria Luisa
    Lucidi, Vincenzina
    Monti, Lidia
    [J]. JOURNAL OF CYSTIC FIBROSIS, 2012, 11 (05) : 427 - 432
12