Liver involvement in hereditary hemorrhagic telangiectasia: consensus recommendations.
OBJECTIVE
To formulate recommendations about clinical management of liver involvement in hereditary hemorrhagic telangiectasia (HHT), using a formal consensus development process.


METHODS
A nominal group technique was used. A list of main clinical, diagnostic and therapeutic issues about liver involvement in HHT was generated by the organizing committee. Panel members then scored their agreement with each statement; the median score, and standard deviation for each statement were determined for each of the three successive panel rounds. These consensus statements formed the basis for recommendations graded with the strength and quality of supporting evidence.
Doppler US is sufficiently accurate and suitable for first-line imaging of the liver in the general HHT population. Liver biopsy in any patient with proven or suspected HHT should be avoided. Liver involvement in HHT is generally asymptomatic; in the minority of patients where it is symptomatic, morbidity and mortality can be substantial. The prevalence of focal nodular hyperplasia is much higher in patients with liver involvement by HHT than in the general population. Invasive therapies for liver involvement by HHT (namely liver transplantation) should be considered only in patients who have failed to respond to intensive medical therapy.
Buscarini
Elisabetta
E
Gastroenterology Department, Maggiore Hospital, Crema, Italy. ebuscarini@rim.it
Plauchu
Henry
H
Garcia Tsao
Guadalupe
G
White
Robert I
RI
Sabbà
Carlo
C
Miller
Franklin
F
Saurin
Jean Cristophe
JC
Pelage
Jean Pierre
JP
Lesca
Gaetan
G
Marion
Marie Jeanne
MJ
Perna
Annalisa
A
Faughnan
Marie E
ME
D002779:Cholestasis / Q000209:etiology; D004621:Embolization, Therapeutic; D020518:Focal Nodular Hyperplasia / Q000209:etiology; D006801:Humans; D008107:Liver Diseases / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D016031:Liver Transplantation; D017410:Practice Guidelines as Topic; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*; D014463:Ultrasonography; D018608:Ultrasonography, Doppler