congenital gout

congenital gout

gutta, Latin, droplet A condition linked to 2 X-linked enzymes–hypoxanthine-guanine phosphoribosyl transferase, defective in Lesch-Nyhan disease, and 5-phosphoribosyl-1-pyrophosphate Lab ↑ serum uric acid, often ≥ 410 µmol/L–US: 7.0 mg/ml, a level found in a significant minority of ♂–90% of gout occurs in ♂; family Hx of gout is present in
±12 of cases;12 present with the classic 'podagra'–1st metatarsophalangeal joint; up to 90% suffer podagra at some time during their disease Management–Acute Colchicine, NSAIDs, corticosteroids Interval Diet Chronic Allopurinol, sulfinpyrazone, salicylates
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