Document Detail


Individualized management of hepatic diseases in hereditary hemorrhagic telangiectasia.
MedLine Citation:
PMID:  21375837     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Liver involvement in patients with hereditary hemorrhagic telangiectasia (HHT) has not been fully characterized in China. The clinical manifestations, imaging studies, results of treatment in six patients and symptomatic liver involvement were analyzed. Patients included three women and three men with age from 35 to 62 years old. Two patients presented with shortness of breath, one patient with anemia and splenomegaly, and one with chronic gastrointestinal bleeding; the remaining two were asymptomatic. CT and CT angiography (CTA) showed arterioportal and arteriovenous shunting in liver. CTA showed at least one enlarged hepatic artery in all patients. One patient received ligation of the enlarged arteries with subsequent disappearance of symptoms at 56-month follow-up. The patient with gastrointestinal bleeding received interventional embolotherapy and resolved; interventional therapy to embolize the enlarged hepatic arteries was unsuccessful in another patient and the patient died of heart failure and liver dysfunction 38 months later. The patient with splenomegaly received a splenectomy and bandage of an enlarged hepatic artery. One of the two patients with no symptoms died of liver dysfunction 41 months after diagnosis. The other showed abnormal liver function and ascites, and traditional Chinese medicinal herb was used with no effect 21 months later. The symptoms disappeared after systemic medical treatment. Individualized and active therapy is advantageous and proper for patients with HHT.
Authors:
Xie Song; Hong-Qiang Chen; Yu-Xin Chen; Yu Cheng; Chuan-Qiang Qu; En-Yu Liu; Sen Guo; Ke-Sen Xu; Jun Niu; Nan-Hai Shou
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The American surgeon     Volume:  77     ISSN:  0003-1348     ISO Abbreviation:  Am Surg     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-03-07     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370522     Medline TA:  Am Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  281-5     Citation Subset:  IM    
Affiliation:
Department of Hepatobiliary Surgery , Qilu Hospital, Shandong University, Jinan, China.
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