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The Effect of pH Changes on the Levels of Alpha-Galactosidase A in a Patient with Fabry's Disease, Post Renal Transplant

Brown, Lois E. (1989) The Effect of pH Changes on the Levels of Alpha-Galactosidase A in a Patient with Fabry's Disease, Post Renal Transplant. Master's Thesis, University of Pittsburgh.

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Abstract

Fabry's disease (alpha-galactosidase A deficiency) leads to early renal failure. Unfortunately, kidney transplants do not always reliably supply the deficient enzyme. To assess urinary and serum activity of alpha-galactosidase A by the transplant as well as the relationship between urinary and serum hydrolase activity, acid-base studies were performed in normal controls and in a patient with Fabry' s disease post renal transplant. Alkalinization was induced by intravenous sodium bicarbonate and acidification by acetazolamide administration. The results show that alpha-galactosidase A is secreted by the renal graft, but that administration of sodium bicarbonate and acetazolamide variably alter the excretion and reabsorption of alpha-galactosidase, as well as other lysosomal hydrolases, specifically beta-hexosaminidase and beta-glucuronidase. Therefore, these methods do not appear useful for treating Fabry' s disease post renal transplant. The large changes in hydrolase excretion induced by the pH changes also demonstrate the difficulty of using lysosomal enzymuria as a diagnostic marker for renal disorders without knowledge of acid-base conditions.


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Details

Item Type: Other Thesis, Dissertation, or Long Paper (Master's Thesis)
Status: Unpublished
Creators/Authors:
CreatorsEmailPitt UsernameORCID
Brown, Lois E.
Contributors:
ContributionContributors NameEmailPitt UsernameORCID
Committee ChairGlew, RobertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Committee MemberChen, AliceUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Committee MemberKlinosky, BernardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Date: 1989
Date Type: Completion
Number of Pages: 65
Institution: University of Pittsburgh
Schools and Programs: School of Health and Rehabilitation Sciences > Health and Rehabilitation Sciences
Degree: MS - Master of Science
Thesis Type: Master's Thesis
Refereed: Yes
Date Deposited: 24 Jul 2024 13:53
Last Modified: 24 Jul 2024 13:53
URI: http://d-scholarship.pitt.edu/id/eprint/46738

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