Document Detail

Review article: the hepatic manifestations of hereditary haemorrhagic telangiectasia.
MedLine Citation:
PMID:  18573106     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Liver involvement in hereditary haemorrhagic telangiectasia is mainly characterized by vascular malformations, such as telangiectasies and arteriovenous shunts, which are found in up to 80% of patients. AIM: To analyse the current knowledge and controversies regarding the epidemiological, pathological, clinical, diagnostic and therapeutic aspects of liver involvement in hereditary haemorrhagic telangiectasia. Methods Systematic survey analysis of the indexed studies dealing with the above mentioned topics. RESULTS: No more than 8% of patients with hepatic vascular abnormalities will have a symptomatic liver disease, mainly consisting in high-output heart failure, portal hypertension or biliary disease. CONCLUSIONS: Colour Doppler ultrasonography is a non-invasive, highly accurate and relatively low-cost procedure for the screening of liver involvement in patients with hereditary haemorrhagic telangiectasia; computed tomography, magnetic resonance imaging and angiography can be reserved for the characterization of focal lesions and the study of severely ill patients in whom invasive therapeutic procedures are advisable. Patients with asymptomatic liver involvement should not receive any treatment, while the therapeutic options for symptomatic patients include treatment of the specific complication, invasive procedures for shunt reduction and liver transplantation. The newly developed antiangiogenetic therapies appear to be very promising, but still require further evaluation in clinical trials.
C Sabbà; M Pompili
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Publication Detail:
Type:  Journal Article; Review     Date:  2008-06-20
Journal Detail:
Title:  Alimentary pharmacology & therapeutics     Volume:  28     ISSN:  1365-2036     ISO Abbreviation:  Aliment. Pharmacol. Ther.     Publication Date:  2008 Sep 
Date Detail:
Created Date:  2008-08-14     Completed Date:  2008-09-24     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8707234     Medline TA:  Aliment Pharmacol Ther     Country:  England    
Other Details:
Languages:  eng     Pagination:  523-33     Citation Subset:  IM    
Unit of Internal Medicine, Department of Internal Medicine and Public Health, Interdepartmental HHT Centre, University of Bari, Bari, Italy.
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MeSH Terms
Biliary Tract Diseases / etiology
Heart Failure / etiology
Hypertension, Portal / etiology
Liver Diseases / diagnosis,  etiology*,  therapy
Liver Transplantation
Magnetic Resonance Imaging
Telangiectasia, Hereditary Hemorrhagic / complications*,  diagnosis,  therapy
Tomography, X-Ray Computed
Ultrasonography, Doppler, Color
Vascular Fistula / diagnosis,  etiology

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