Hayabuchi Y, Matsuoka S, Takahashi Y, Akita H, Kitagawa T, Kato I, Kuroda Y
Department of Pediatrics, University of Tokushima School of Medicine, Japan.
Pediatr Cardiol. 1994 Sep-Oct;15(5):249-51. doi: 10.1007/BF00795737.
Hyperuricemia is commonly recognized in adolescents and adults with cyanotic congenital heart disease. We report a case of a male infant with hyperuricemia, Taussig-Bing anomaly, and interruption of the aortic arch. The patient underwent correction of interrupted aortic arch and pulmonary arterial banding at the age of 7 days. Hyperuricemia appeared when he was 2 months old (max 17.7 mg/dl) and persisted until he underwent a Jatene operation at the age of 10 months. The hyperuricemia improved gradually after the disappearance of hypoxia and polycythemia. The laboratory findings suggest that hyperuricemia can result from uric acid overproduction due to secondary polycythemia, impairment of uric acid excretion by the kidney, or the acceleration of anaerobic metabolism. Allopurinol and benzbromarone together were partially effective treatments for hyperuricemia in this patient with cyanotic congenital heart disease.
高尿酸血症在患有青紫型先天性心脏病的青少年和成人中较为常见。我们报告一例患有高尿酸血症、陶西格-宾畸形和主动脉弓中断的男婴病例。该患者在7日龄时接受了主动脉弓中断矫正术和肺动脉束带术。高尿酸血症在他2个月大时出现（最高达17.7mg/dl），并持续到他10个月大时接受贾滕手术。缺氧和红细胞增多症消失后，高尿酸血症逐渐改善。实验室检查结果表明，高尿酸血症可能是由于继发性红细胞增多症导致尿酸生成过多、肾脏尿酸排泄受损或无氧代谢加速所致。别嘌醇和苯溴马隆联合使用对该患有青紫型先天性心脏病的患者的高尿酸血症有部分疗效。
