Biochemical studies on the treatment of primary hyperoxaluria.
نویسندگان
چکیده
The increased urinary oxalate excretion which is characteristic of primary hyperoxaluria causes recurrent urinary calculi and nephrocalcinosis, with death at an early age (Hall, Scowen, and Watts, 1960; Hockaday, Clayton, Frederick, and Smith, 1964), and no effective treatment for the disease has been reported. Glyoxylate is the main immediate metabolic precursor of oxalate, and if the enzyme or enzymes which catalyse the oxidation
منابع مشابه
Hyperoxalurias and their treatment
Hyperoxaluria is characterized by an increased in excretion of oxalate by kidney.There are two distinct clinical expressions of hyperoxaluria, named primary and secondary hyperoxaluria. Primary hyperoxaluria is a genetic disorder due to defective enzyme activity .In contrast , secondary hyperoxaluria , is caused by increased dietary ingestion of oxalate or oxalate precursors. There are three ma...
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PH1 (primary hyperoxaluria type 1) is a severe inborn disorder of glyoxylate metabolism caused by a functional deficiency of the peroxisomal enzyme AGXT (alanine-glyoxylate aminotransferase), which converts glyoxylate into glycine using L-alanine as the amino-group donor. Even though pre-genomic studies indicate that other human transaminases can convert glyoxylate into glycine, in PH1 patients...
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Primary hyperoxaluria (PH) disorder causes end-stage renal disease (ESRD). Missed diagnosis or relapse in transplanted kidney is common. We present a 36-year-old patient with a history of recurrent renal calculus which progressed to end-stage renal disease. He underwent kidney-transplant surgery. Renal function tests had worsening progressively at first-week post-transplant. Transplanted kidney...
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BACKGROUND Primary hyperoxaluria is a rare disease with an estimated prevalence of 1 to 3 cases per million. It is due to a hepatic enzyme deficiency responsible for an endogenous overproduction of oxalate. Oxalate crystals commonly deposit in the kidney and more rarely in bone marrow. The literature has reported, to the best of our knowledge, only two cases of hyperoxaluria diagnosed by bone m...
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VON WARTBURG, J.P., BETHUNE, J.L. & VALLEE, B.L. (1964) Human liver alcohol dehydrogenase: kinetic and physiochemical properties. Biochemistry, 3, 1775. WACKER, W.E.C., HAYNES, H., DRUYAN, R., FISHER, W. & COLEMAN, J.E. (1965) Treatment of ethylene glycol poisoning with ethyl alcohol Journal of the American Medical Association, 194, 1231. WALLS, J., MORLEY, A.R. & KERR, D.N.S. (1969) Primary hy...
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ورودعنوان ژورنال:
- Archives of disease in childhood
دوره 42 225 شماره
صفحات -
تاریخ انتشار 1967