Document Detail


Large spectrum of liver vascular lesions including high prevalence of focal nodular hyperplasia in patients with hereditary haemorrhagic telangiectasia: the Belgian Registry based on 30 patients.
MedLine Citation:
PMID:  20631626     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: We report the Belgian Registry of 30 patients (19 women and 11 men) with hereditary haemorrhagic telangiectasia (HHT) and liver involvement.
RESULTS: Twenty-three patients (77%) were asymptomatic. Within the seven symptomatic patients (23%), four suffered from high output cardiac failure, two died before liver transplantation and one was transplanted. Three patients developed symptomatic biliary disease, two were transplanted and one was listed. Intrahepatic shunts and a large hepatic artery (6-14 mm, mean: 9.3 mm) were found in all patients and are characteristic of liver involvement. We observed a high prevalence (47%) of asymptomatic hepatic tumours with radiological and histological characteristics of focal nodular hyperplasia (FNH) for the majority of these tumours. The histological examination of the three explanted livers revealed the coexistence of a large spectrum of hepatic vascular lesions including intrahepatic shunts, FNH, nodular regenerative hyperplasia, sinusoidal dilatation and ischaemic cholangiopathy. All these lesions should be diagnosed early to avoid invasive procedures even if a liver biopsy was performed in six of our patients without complications. The liver biopsy led to the diagnosis of HHT in one patient and to FNH in another one.
CONCLUSION: Liver involvement in HHT is characterized by a high prevalence of FNH and a large spectrum of vascular lesions such as intrahepatic shunts, nodular regenerative hyperplasia, sinusoidal dilatation and ischaemic cholangiopathy that may coexist simultaneously in the same patient.
Authors:
Réginald Brenard; Xavier Chapaux; Pierre Deltenre; Jean Henrion; Stephane De Maeght; Yves Horsmans; Ivan Borbath; Anne Leenaerts; Jacques Van Cauter; Sven Francque; Thomas Sersté; Christophe Moreno; Hans Orlent; Philippe Mengeot; Jan Lerut; Christine Sempoux
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  European journal of gastroenterology & hepatology     Volume:  22     ISSN:  1473-5687     ISO Abbreviation:  Eur J Gastroenterol Hepatol     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-09-09     Completed Date:  2010-12-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9000874     Medline TA:  Eur J Gastroenterol Hepatol     Country:  England    
Other Details:
Languages:  eng     Pagination:  1253-9     Citation Subset:  IM    
Affiliation:
Hepato-Gastroenterology Unit, Grand Hôpital de Charleroi, Site St Joseph Gilly, Gilly, Belgium. reginald.brenard@ghdc.be
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Belgium / epidemiology
Biliary Tract / pathology
Biopsy
Female
Focal Nodular Hyperplasia / epidemiology*,  pathology*,  radiography
Hepatic Artery / pathology
Hepatic Veins / pathology
Humans
Liver / pathology*
Magnetic Resonance Imaging
Male
Middle Aged
Organ Size
Prevalence
Registries
Telangiectasia, Hereditary Hemorrhagic / epidemiology*,  pathology*,  radiography
Tomography, X-Ray Computed
Young Adult

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