Effect of acid-base changes on urinary hydrolases in Fabry's disease after renal transplantation

Rozlyn M. Berty, Sheldon Adler, Alakananda Basu, Robert H. Glew

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Fabry's disease, which is characterized by α-galactosidase A (AG) deficiency, causes early renal failure. Kidney transplants do not reliably supply the deficient enzyme. To assess both urinary excretion of AG by the transplant and the relationship between urine and serum hydrolase activity, acute and chronic acid-base studies were performed in normal control subjects and in the patients with Fabry's disease who had undergone renal transplantation. For the acute studies, alkalasis was induced by intravenous infusion of sodium bicarbonate and acidosis was induced by ingestion of ammonium chloride. The chronic study involved long-term ingestion by NH4Cl by only the patient with Fabry's disease. The results show that AG is secrteted by the renal graft. Urinary hydrolase excretion was increased by acute alkalinization and decreased by acute acidification. Acute, but not chronic, acidification increased the patient's serum AG activity, indicating that long-term acidification is not useful for treating Fabry's disease after transplantation. The large changes in hydrolyase excretion induced by acute and chronic acid-base changes show the difficulty of using lysosomal enzymuria as a diagnostic marker for renal disorders without knowledge of acid-base conditions.

Original languageEnglish
Pages (from-to)696-703
Number of pages8
JournalThe Journal of Laboratory and Clinical Medicine
Volume115
Issue number6
StatePublished - 1 Jan 1990

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Fabry Disease
Hydrolases
Kidney Transplantation
Acids
Transplants
Kidney
Eating
Galactosidases
Ammonium Chloride
Sodium Bicarbonate
Knowledge Bases
Acidosis
Serum
Intravenous Infusions
Renal Insufficiency
Transplantation
Urine
Enzymes

Cite this

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abstract = "Fabry's disease, which is characterized by α-galactosidase A (AG) deficiency, causes early renal failure. Kidney transplants do not reliably supply the deficient enzyme. To assess both urinary excretion of AG by the transplant and the relationship between urine and serum hydrolase activity, acute and chronic acid-base studies were performed in normal control subjects and in the patients with Fabry's disease who had undergone renal transplantation. For the acute studies, alkalasis was induced by intravenous infusion of sodium bicarbonate and acidosis was induced by ingestion of ammonium chloride. The chronic study involved long-term ingestion by NH4Cl by only the patient with Fabry's disease. The results show that AG is secrteted by the renal graft. Urinary hydrolase excretion was increased by acute alkalinization and decreased by acute acidification. Acute, but not chronic, acidification increased the patient's serum AG activity, indicating that long-term acidification is not useful for treating Fabry's disease after transplantation. The large changes in hydrolyase excretion induced by acute and chronic acid-base changes show the difficulty of using lysosomal enzymuria as a diagnostic marker for renal disorders without knowledge of acid-base conditions.",
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Effect of acid-base changes on urinary hydrolases in Fabry's disease after renal transplantation. / Berty, Rozlyn M.; Adler, Sheldon; Basu, Alakananda; Glew, Robert H.

In: The Journal of Laboratory and Clinical Medicine, Vol. 115, No. 6, 01.01.1990, p. 696-703.

Research output: Contribution to journalArticle

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AB - Fabry's disease, which is characterized by α-galactosidase A (AG) deficiency, causes early renal failure. Kidney transplants do not reliably supply the deficient enzyme. To assess both urinary excretion of AG by the transplant and the relationship between urine and serum hydrolase activity, acute and chronic acid-base studies were performed in normal control subjects and in the patients with Fabry's disease who had undergone renal transplantation. For the acute studies, alkalasis was induced by intravenous infusion of sodium bicarbonate and acidosis was induced by ingestion of ammonium chloride. The chronic study involved long-term ingestion by NH4Cl by only the patient with Fabry's disease. The results show that AG is secrteted by the renal graft. Urinary hydrolase excretion was increased by acute alkalinization and decreased by acute acidification. Acute, but not chronic, acidification increased the patient's serum AG activity, indicating that long-term acidification is not useful for treating Fabry's disease after transplantation. The large changes in hydrolyase excretion induced by acute and chronic acid-base changes show the difficulty of using lysosomal enzymuria as a diagnostic marker for renal disorders without knowledge of acid-base conditions.

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