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Portal diversion for the treatment of glycogen storage disease in humans

Starzl, TE and Putnam, CW and Porter, KA and Halgrimson, CG and Corman, J and Brown, BI and Gotlin, RW and Rodgerson, DO and Greene, HL (1973) Portal diversion for the treatment of glycogen storage disease in humans. Annals of Surgery, 178 (4). 525 - 539. ISSN 0003-4932

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Seven patients with Types I, III, or VI glycogen storage disease were treated with portal diversion from 5 1/2 mth to 9 1/2 yr ago. The first 2 patients had portacaval transposition with 1 early death. The last 5 patients had the technically safer procedure of end to side portacaval shunt without any deaths and with no late findings of hepatic encephalopathy. The convalescence of the patients with either kind of portal diversion was characterized by accelerated body growth and bone mineralization, incomplete relief of hypoglycemia and metabolic acidosis, striking amelioration of the hyperlipidemia of Type I disease, liver shrinkage in Types I and VI disease, and variable relief of such diverse other derangements as abnormal bleeding and elevated serum uric acid concentrations. The liver concentrations of glycogen were not affected by portal diversion. Hepatocytes were decreased in size in subsequent biopsies, thereby accounting for the reduction of liver size in most of the cases without a major alteration in glycogen. There was a high incidence of pre existing coincidental liver disease in patients, including transaminase elevations and hepatic fibrosis or even cirrhosis. These abnormalities were particularly striking in Type III patients but did not appear in any of the cases to be made worse by portal diversion. The observations in these 7 patients and in 6 more reported from other centers and followed up with personal examination indicate that portal diversion should have an important role in carefully selected cases of glycogen storage disease. Recent work was reviewed which suggests that the effects of portacaval shunt are due more to the rerouting of pancreatic hormones around the liver than to the bypassing of alimentary glucose.


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Item Type: Article
Status: Published
CreatorsEmailPitt UsernameORCID
Starzl, TEtes11@pitt.eduTES11
Putnam, CW
Porter, KA
Halgrimson, CG
Corman, J
Brown, BI
Gotlin, RW
Rodgerson, DO
Greene, HL
Centers: Other Centers, Institutes, Offices, or Units > Thomas E. Starzl Transplantation Institute
Date: 1 January 1973
Date Type: Publication
Journal or Publication Title: Annals of Surgery
Volume: 178
Number: 4
Page Range: 525 - 539
DOI or Unique Handle: 10.1097/00000658-197310000-00015
Institution: University of Pittsburgh
Refereed: Yes
ISSN: 0003-4932
Other ID: uls-drl:31735062109172, Starzl CV No. 312
Date Deposited: 08 Apr 2010 17:05
Last Modified: 02 Feb 2019 13:55


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