Studies of uric acid metabolism in glycogen storage disease associated with gouty arthritis

Jakovcic, Smilja; Sørensen, Leif

doi:10.1002/art.1780100207

Cited by 54 publications

(20 citation statements)

References 21 publications

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“…Specific activity of urine uric acid and 6-day incorporation of [I-I4C]glycine into [ITIuric acid decreased when nocturnal feeding by nasogastric feeding was begun, as shown in Figure 1 and Table 1, but remained greater than control. The patient with GSD type 111 had a [I-14C]glycine uptake into uric acid similar to that of previous control subjects (14,15).…”

Section: Resultssupporting

confidence: 77%

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Uric Acid Metabolism in Therapy of Glycogen Storage Disease Type I

Benke

Gold

1978

Pediatr Res

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SummaryFactors which may explain lower serum uric acid in a new therapy of patients with glycogen storage disease (GSD) type I have been studied. [1-14C]Glycine incorporation into urine uric acid was 0.68% of the injected dose during a 6-day period of frequent high carbohydrate feedings, 0.40% with the same diet and nocturnal nasogastric feeding by Vivonex, and 0.18% in a control patient with GSD type 111. Fractional renal uric acid excretion in the patient with GSD type I increased from 11.3% to 26.3% after beginning nocturnal nasogastric feeding of Vivonex. Red cell phosphoribosylpyrophosphate levels were not changed by the therapy. Addition of Vivonex nocturnal feedings to frequent high carbohydrate feedings ( I ) decreased the accelerated de novo purine synthesis to a level still higher than control and (2) increased fractional renal uric acid excretion. SpeculationNear normalization of purine metabolism with nocturnal feeding should decrease the risk of gout in GSD type I.

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Section: Resultssupporting

confidence: 77%

“…Elevated serum ketone concentrations potentially decrease renal uric acid excretion ( 6 ) . Patients have an acceleration of de novo svnthesis of uric acid from precursors (12,14,15). Uric acid binding to increased levels of lipoproteins may be a.factor, and excessive breakdown of cellular ~u r i n e s to uric acid has been proposed (8).…”

Section: Speculationmentioning

confidence: 99%

Uric Acid Metabolism in Therapy of Glycogen Storage Disease Type I

Benke

Gold

1978

Pediatr Res

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“…Hyperuricemia in GSD-I has been shown previously to result from at least two mechanisms: (a) decreased urinary excretion due to chronic lacticacidemia and ketonemia (34) and (b) increased production of uric acid (2)(3)(4)(5)(6)(7). The second mechanism has not been clearly defined although an increase in purine synthesis de novo is suggested by an increase in labeled glycine incorporation into urinary uric acids (3-7).…”

Section: Discussionmentioning

confidence: 99%

ATP Depletion, a Possible Role in the Pathogenesis of Hyperuricemia in Glycogen Storage Disease Type I

Greene¹,

Wilson²,

Hefferan³

et al. 1978

J. Clin. Invest.

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A B S T R A C T Other investigators have shown that fructose infusion in normal man and rats acutely depletes hepatic ATP and Pi and increases the rate of uric acid formation by the degradation ofpreformed nucleotides. We postulated that a similar mechanism of ATP depletion might be present in patients with glucose-6-phosphatase deficiency (GSD-I) except for glucose-6-phosphate, returned toward preinfusion levels within 20 min.Treatment consisted of continuous intragastric feedings of a high glucose dietary mixture. Such treatment increased blood glucose from a mean level of 62 (range 28-96) to 86 (range 71-143) mg/dl (P < 0.02), decreased plasma glucagon from a mean of 190 (range 171-208) to 56 (range 30-70) pg/ml (P < 0.01), but caused no significant change in insulin levels. Urate output measured in three patients showed an initial increase, coinciding with a decrease in plasma lactate and triglyceride levels, then decreased to normal within 3 days after treatment. Normalization of urate excretion was associated with normalization of serum uric acid.We suggest that the maintenance of blood glucose levels above 70 mg/dl is effective in reducing serum urate levels and that transient and recurrent depletion of hepatic ATP due to glycogenolysis is contributory in the genesis of hyperuricemia in untreated patients with GSD

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“…Several types ot glycogen storage disease have, as an accompaniment, hyperuricemia (40)(41)(42)(43)(44). I n fact at a relatively early age patients with Type I glycogen storage disease develop gouty arthritis as one of their major medical problems.…”

Section: Purine Metabolism J Edwin Seeghiillermentioning

confidence: 99%

Purine metabolism

Seegmiller

1975

Arthritis & Rheumatism

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Studies of uric acid metabolism in glycogen storage disease associated with gouty arthritis

Cited by 54 publications

References 21 publications

Uric Acid Metabolism in Therapy of Glycogen Storage Disease Type I

Uric Acid Metabolism in Therapy of Glycogen Storage Disease Type I

ATP Depletion, a Possible Role in the Pathogenesis of Hyperuricemia in Glycogen Storage Disease Type I

Purine metabolism

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