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Renal Urate Hypoexcretion Preceding Renal Disease in a New Kindred with Familial Juvenile Gouty Nephropathy (FJGN)

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Purine and Pyrimidine Metabolism in Man VII

Abstract

We have reported the syndrome of FJGN in numerous kindreds as well as isolated young subjects of either sex (reviewed in 1). The latter have generally had a strong family history of gout and sometimes renal disease. Subsequent investigations have invariably revealed FJGN in other seemingly healthy family members. The hallmark of the disorder has been a grossly reduced fractional uric acid clearance [FEur] for the young age, sex and renal function, as discussed elswhere in this Symposium (Cameron).

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References

  1. G. Calabrese, H.A. Simmonds, and J.S. Cameron. Precocious familial gout with reduced fractional urate clearance and normal purine enzymes. Quart J Med 75:441–450 (1990).

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  2. F. Moro, C.S. Ogg, J.S. Cameron, H.A. Simmonds, J.A. Duley, and M.B. McBride, P.M. Davies. Familial juvenile gouty nephropathy with renal urate hypoexcretion preceding renal disease. Clin Nephrol 35:263 (1991).

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  3. J.S. Cameron, C.S. Ogg, F. Moro, C. Chantier, and H.A. Simmonds. Precocious familial gout (letter). Lancet ii;745 (1990).

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© 1991 Springer Science+Business Media New York

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McBride, M.B. et al. (1991). Renal Urate Hypoexcretion Preceding Renal Disease in a New Kindred with Familial Juvenile Gouty Nephropathy (FJGN). In: Harkness, R.A., Elion, G.B., Zöllner, N. (eds) Purine and Pyrimidine Metabolism in Man VII. Advances in Experimental Medicine and Biology, vol 309A. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-2638-8_43

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  • DOI: https://doi.org/10.1007/978-1-4899-2638-8_43

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4899-2640-1

  • Online ISBN: 978-1-4899-2638-8

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