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

Fabry's disease, which is characterized by alpha-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,...

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Vydané v:The Journal of laboratory and clinical medicine Ročník 115; číslo 6; s. 696
Hlavní autori: Berty, R M, Adler, S, Basu, A, Glew, R H
Médium: Journal Article
Jazyk:English
Vydavateľské údaje: United States 01.06.1990
Predmet:
Adult
alpha-N-Acetylgalactosaminidase
Ammonium Chloride - therapeutic use
beta-N-Acetyl-Galactosaminidase
beta-N-Acetylhexosaminidases - urine
Bicarbonates - therapeutic use
Electrolytes - blood
Electrolytes - urine
Fabry Disease - enzymology
Fabry Disease - surgery
Fabry Disease - urine
Glycoside Hydrolases - urine
Hexosaminidases - urine
Humans
Hydrogen-Ion Concentration
Kidney Transplantation
Male
Sodium - therapeutic use
Sodium Bicarbonate
Time Factors
ISSN:0022-2143
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Popis
Shrnutí:Fabry's disease, which is characterized by alpha-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 patient with Fabry's disease who had undergone renal transplantation. For the acute studies, alkalosis was induced by intravenous infusion of sodium bicarbonate and acidosis was induced by ingestion of ammonium chloride. The chronic study involved long-term ingestion of NH4Cl by only the patient with Fabry's disease. The results show that AG is secreted 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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ISSN:0022-2143