Partial splenectomy for portal hypertension in cystic fibrosis related liver disease

2007 ◽  
Vol 42 (12) ◽  
pp. 1173-1180 ◽  
Author(s):  
Dominique Louis ◽  
Mai Linh Pham Duc ◽  
Philippe Reix ◽  
Jean-Pierre Chazalette ◽  
Isabelle Durieu ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Partial Splenectomy ◽  
Related Liver Disease

P069 Cystic fibrosis-related liver disease – a decade of experience

2021 ◽  
Vol 20 ◽  
pp. S59-S60
Author(s):  
A. Mocic Pavic ◽  
M. Masic ◽  
I. Trivic ◽  
S. Sila ◽  
T. Niseteo ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Disease ◽  
Related Liver Disease

scholarly journals Cystic Fibrosis Related Liver Disease and Endocrine Considerations

2021 ◽  
pp. 100283
Author(s):  
Jordan S. Sherwood ◽  
Jagdeesh Ullal ◽  
Katherine Kutney ◽  
Kara S. Hughan
Keyword(s):  
Cystic Fibrosis ◽  
Liver Disease ◽  
Related Liver Disease

Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension

2019 ◽  
Vol 17 (10) ◽  
pp. 2134-2136 ◽  
Author(s):  
Hao Wu ◽  
Megan Vu ◽  
Sadhna Dhingra ◽  
Ruth Ackah ◽  
John A. Goss ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Obliterative Portal Venopathy

scholarly journals Cystic fibrosis-related liver disease: a single-center experience

2011 ◽  
Vol 3 (3) ◽  
pp. 21 ◽  
Author(s):  
Paula Catarino Costa ◽  
Celeste Canha Barreto ◽  
Luisa Pereira ◽  
Maria Luisa Lobo ◽  
Maria Adília Costa ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Disease ◽  
Liver Function ◽  
Pediatric Patients ◽  
Liver Function Tests ◽  
Liver Involvement ◽  
Clinical Expression ◽  
Function Tests ◽  
Related Liver Disease

Prospective studies concerning liver disease in pediatric cystic fibrosis patients are scarce. The present study aimed to describe the prevalence and clinical expression of cystic fibrosis - related liver disease, in a cohort of 62 pediatric patients. Descriptive study, resulting from the prospective evaluation, between 1994 and 2009, of 62 pediatric patients (age <18 years) with cystic fibrosis. The follow-up protocol included a clinical assessment every 2 months, liver function tests every 6 months and annual liver ultrasonography. The cumulative prevalence of liver disease was 11.2% (7/62 cases). All patients had ΔF508 mutation and pancreatic insufficiency, none had meconium ileus. The liver involvement became clinically evident at a mean age of 8 years (3-15 years), revealed by hepatomegaly or hepatosplenomegaly (3 cases) and/ or abnormalities of liver function tests (3 cases) changes of liver ultrasound (7 cases) with evidence of portal hypertension (2 cases). Four patients were submitted to liver biopsy; biliary fibrosis was documented in one case, focal biliary cirrhosis in 2 cases and multilobular cirrhosis in another case. Within a median 11.6 years follow-up period (all patients under UDCA therapy after liver disease diagnosis), progression of liver disease was observed in 2 patients; one patient developed refractory variceal bleeding and progressive hepatic failure, requiring liver transplant. The results of the present study agree with those of previous pediatric studies, further documenting clinical expression of liver disease in CF patients, which is usually detected in the first decade of life and emphasize the contribution of ultrasound to early diagnosis of liver involvement. Moreover, although advanced liver disease is a relatively rare event, early isolated liver transplantation may have to be considered at this age group.

scholarly journals GP285 The effect of modulator therapy on liver function in patients with cystic fibrosis-related liver disease (CFLD)

2019 ◽  
Author(s):  
Sharon Dempsey ◽  
John Travers ◽  
Gerardine Leer ◽  
Geraldine Connell ◽  
Shona Quinn ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Disease ◽  
Liver Function ◽  
Related Liver Disease

scholarly journals ARFI and transient elastography for characterization of cystic fibrosis related liver disease: First longitudinal follow-up data in adult patients

2013 ◽  
Vol 12 (6) ◽  
pp. 826-827 ◽  
Author(s):  
Thomas Karlas ◽  
Marie Neuschulz ◽  
Annett Oltmanns ◽  
Hubert Wirtz ◽  
Volker Keim ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Disease ◽  
Transient Elastography ◽  
Adult Patients ◽  
Related Liver Disease
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