Pediatric Nephrology Research Center(Ped NRC), Shahid Beheshti Medical University, Tehran, Iran.
Iran J Kidney Dis. 2020 Jul;14(4):278-281.
A few data on the prevalence of renal involvement in cystic fibrosis and its spectrum in childhood is available. In the present study, we conducted a prospective study on children who had cystic fibrosis and evaluated their renal involvement. In fact, the aim of the study was to provide data on the clinical consequences of proper identification of kidney disease in a group of children with cystic fibrosis.
This prospective study was conducted on 55 consecutive patients with previous diagnosis of cystic fibrosis during a threeyear period and at least 3 months to over 5 years or more follow-up. The inclusion criteria was the diagnosis of cystic fibrosis which was made by clinical presentation of cystic fibrosis and laboratory results. Initially, patients' medical records were reviewed and relevant data were collected. A 24-hour urine collection (or a random urine sampling in very young infants) was used to assess crystalluria and renal function was evaluated by blood sampling.
Totally, 55 patients with cystic fibrosis were admitted in two hospitals with the mean age of 8.22 ± 5.66 years. GFR totally reduced in 34.5%. The overall prevalence of hypercalciuria was estimated to be 60%, while hyperoxaluria, hypocitraturia, and hyperuricosuria in 41.8%, 24.5%, and 47.3%; respectively.
Crystalluria is a common consequence of cystic fibrosis in childhood. The prevailing crystalluric finding includes hypercalciuria followed by hyperuricosuria, and hyperoxaluria. During disease GFR may be decreased due to several reasons such as nephrotoxic drugs usage.
关于囊性纤维化中肾脏受累及其在儿童中的表现,已有少量数据。在本研究中,我们对患有囊性纤维化的儿童进行了一项前瞻性研究,并评估了他们的肾脏受累情况。实际上,该研究的目的是提供一组囊性纤维化儿童中正确识别肾脏疾病的临床后果的数据。
这是一项前瞻性研究,纳入了 55 例连续诊断为囊性纤维化的患者,随访时间为 3 年,至少随访 3 个月至 5 年以上。纳入标准为临床诊断为囊性纤维化,实验室检查结果符合囊性纤维化的表现。最初,回顾了患者的病历并收集了相关数据。通过 24 小时尿液收集(或非常年幼的婴儿随机尿液抽样)来评估结晶尿,并通过采血来评估肾功能。
共有 55 例囊性纤维化患者入住了两家医院,平均年龄为 8.22 ± 5.66 岁。GFR 总共有 34.5%的下降。高钙尿症的总体患病率估计为 60%,而高草酸尿症、低柠檬酸尿症和高尿酸尿症的患病率分别为 41.8%、24.5%和 47.3%。
结晶尿是儿童囊性纤维化的常见后果。主要的结晶尿发现包括高钙尿症,其次是高尿酸尿症和高草酸尿症。在疾病过程中,由于使用肾毒性药物等原因,GFR 可能会降低。