CHU de Bordeaux, Centre de Ressources et de Compétences de la Mucoviscidose pédiatrique, Hôpital Pellegrin Enfants, Bordeaux, France.
J Cyst Fibros. 2010 Jul;9(4):263-8. doi: 10.1016/j.jcf.2010.03.006. Epub 2010 Apr 21.
Due to the improvement in life expectancy in cystic fibrosis (CF), co-morbidities such as renal function impairment may be more frequent.
To determine the prevalence of renal disease in children with CF and to identify associated risk factors.
A single-center retrospective study analyzing the genetic, clinical and therapeutic characteristics of 112 children. The estimated glomerular filtration rate (GFR), microalbuminuria and lithiasic risk factors were assessed.
The median calculated GFR (Schwartz) was 123, 161 and 155ml/min/1.73m(2) in children aged 1, 6 and 15years, respectively. The cumulative dose of aminoglycosides was not correlated to GFR. Microalbuminuria was present in 22/38 patients. Hyperoxaluria was observed in 58/83 patients and was associated with a severe genotype, pancreas insufficiency and liver disease. Hypercalciuria, hyperuricuria and hypocitraturia were identified in 16/87, 15/83 and 57/76 patients, respectively.
Renal impairment in CF has various presentations. There appears to be low levels of renal impairment in children with CF. However, the risk of oxalocalcic urolithiasis is enhanced, and GFR may be underestimated by the Schwartz formula. Further studies using measured GFR techniques are thus warranted.
由于囊性纤维化 (CF) 患者的预期寿命延长，肾功能损害等合并症可能更为常见。
确定 CF 患儿中肾脏病的患病率，并确定相关的危险因素。
对 112 名儿童进行了单中心回顾性研究，分析了其遗传、临床和治疗特征。评估了估计肾小球滤过率 (GFR)、微量白蛋白尿和结石形成危险因素。
年龄为 1、6 和 15 岁的儿童的中位计算 GFR（Schwartz）分别为 123、161 和 155ml/min/1.73m(2)。氨基糖苷类药物的累积剂量与 GFR 无关。38 名患者中有 22 名存在微量白蛋白尿。58/83 名患者存在高草酸尿症，与严重基因型、胰腺功能不全和肝病有关。16/87、15/83 和 57/76 名患者分别存在高钙尿症、高尿酸尿症和低柠檬酸尿症。
CF 患者的肾功能损害表现多样。CF 患儿的肾功能损害似乎处于低水平。然而，草酸钙结石形成的风险增加，并且 Schwartz 公式可能低估了 GFR。因此，有必要使用测量 GFR 的技术进行进一步研究。
