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Portocaval shunt in patients with familial hypercholesterolemia

Starzl, TE and Chase, HP and Ahrens, EH (1983) Portocaval shunt in patients with familial hypercholesterolemia. Annals of Surgery, 198 (3). 273 - 283. ISSN 0003-4932

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Portocaval shunt was performed in ten patients with homozygous and two with heterozygous familial hypercholesterolemia (FH). Total serum cholesterol was lowered by 20% to 55.4% during follow-up periods of 14 months to almost 9 years, with commensurate decreases in LDL cholesterol. The effect on HDL cholesterol and triglyceride levels was variable. Tendinocutaneous xanthomas diminished or disappeared. Growth and development in children had proceeded or accelerated. There was no detectable emotional or intellectual deterioration. Hepatic failure did not occur, although blood ammonia concentrations and serum alkaline phosphatase levels increased relative to preoperative values. Cardiac symptoms were often improved, but evidence of reversal of cardiovascular lesions was inconclusive. Three patients with pre-existing heart disease died of cardiac complications after 4 months, 18 1/2 months, and 30 months. Portacaval shunt has been effective therapy for patients with FH who were refractory or intolerant to medical treatment; it should be performed before the development of irreversible cardiovascular damage.


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Item Type: Article
Status: Published
CreatorsEmailPitt UsernameORCID
Starzl, TEtes11@pitt.eduTES11
Chase, HP
Ahrens, EH
Centers: Other Centers, Institutes, Offices, or Units > Thomas E. Starzl Transplantation Institute
Date: 1 January 1983
Date Type: Publication
Journal or Publication Title: Annals of Surgery
Volume: 198
Number: 3
Page Range: 273 - 283
DOI or Unique Handle: 10.1097/00000658-198309000-00004
Institution: University of Pittsburgh
Refereed: Yes
ISSN: 0003-4932
Other ID: uls-drl:31735062114677, Starzl CV No. 501
Date Deposited: 08 Apr 2010 17:08
Last Modified: 02 Feb 2019 13:57


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