Hoppe B, Hesse A, Brömme S, Rietschel E, Michalk D
University Children's Hospital Cologne, Germany.
Pediatr Nephrol. 1998 May;12(4):275-9. doi: 10.1007/s004670050452.
Patients with cystic fibrosis (CF) have an increased risk of urolithiasis/nephrocalcinosis. To determine potential mechanisms responsible, we studied the urinary excretion of lithogenic and stone-inhibitory substances and calculated the urinary saturation for calcium-oxalate (CaOx), brushite (CaHPO4), and uric acid (UA). We examined 24-h urines in 63 patients with CF (34 female, 29 male) aged 5 months to 36 years. Renal ultrasonography was performed at the time of urine collection. Hyperoxaluria was found in 25 patients (range 0.51-1.71 mmol/1.73 m2 per 24 h). Urinary Ca was increased in 13 patients (4.1-8.22 mg/kg per 24 h). Hyperuricosuria was found in 16 patients (5.2-18.0 mmol/1.73 m2 per 24 h) and hypocitraturia in 14 patients (0.07-1.14 mmol/1.73 m2 per 24 h). CaOx saturation was elevated in 26 patients, related to hyperoxaluria in 19 patients. CaHPO4 saturation was increased in 19 patients and UA saturation in 11 patients. Urolithiasis in situ was diagnosed in 1 patient; 3 patients previously had renal stones; 4 patients had present nephrocalcinosis. Elevated excretion of lithogenic substances and urinary supersaturation might lead to the higher risk of urolithiasis/nephrocalcinosis in patients with CF.
囊性纤维化（CF）患者发生尿石症/肾钙质沉着症的风险增加。为确定其潜在机制，我们研究了致石和抑石物质的尿排泄情况，并计算了草酸钙（CaOx）、磷酸氢钙（CaHPO4）和尿酸（UA）的尿饱和度。我们检测了63例年龄在5个月至36岁之间的CF患者（34例女性，29例男性）的24小时尿液。在收集尿液时进行了肾脏超声检查。发现25例患者存在高草酸尿症（范围为每24小时0.51 - 1.71 mmol/1.73 m2）。13例患者尿钙升高（每24小时4.1 - 8.22 mg/kg）。16例患者存在高尿酸尿症（每24小时5.2 - 18.0 mmol/1.73 m2），14例患者存在低枸橼酸尿症（每24小时0.07 - 1.14 mmol/1.73 m2）。26例患者的CaOx饱和度升高，其中19例与高草酸尿症有关。19例患者的CaHPO4饱和度增加，11例患者的UA饱和度增加。1例患者诊断为原位尿石症；3例患者既往有肾结石；4例患者存在肾钙质沉着症。致石物质排泄增加和尿过饱和可能导致CF患者发生尿石症/肾钙质沉着症的风险更高。
