Urinary System and Renal Involvement in Children With Cystic Fibrosis
Nasrin Esfandiar, Ghamartaj Khanbabaee1, Khadijeh Riazi Kermani
1Pediatric Nephrology Research Center(Ped NRC), Shahid Beheshti Medical University, Tehran, Iran. khanbabaeegh@yahoo.com.
Insights
Cystic fibrosis commonly causes kidney issues in children, with hypercalciuria being the most frequent finding. This study highlights the prevalence of crystalluria and reduced glomerular filtration rate (GFR) in pediatric cystic fibrosis patients.
Area of Science:
- Pediatric Nephrology
- Cystic Fibrosis Research
- Renal Manifestations in Genetic Diseases
Background:
- Limited data exists on the prevalence and spectrum of renal involvement in pediatric cystic fibrosis.
- Early identification of kidney disease in cystic fibrosis is crucial for managing clinical consequences.
Purpose of the Study:
- To prospectively evaluate renal involvement in children diagnosed with cystic fibrosis.
- To provide data on the clinical spectrum of kidney disease in this pediatric population.
Main Methods:
- A prospective study involving 55 pediatric patients with cystic fibrosis over a three-year period.
- Data collection included medical record review, 24-hour urine collection for crystalluria assessment, and blood sampling for renal function evaluation.
- Inclusion criteria required a confirmed diagnosis of cystic fibrosis based on clinical presentation and laboratory results.
Main Results:
- The study included 55 cystic fibrosis patients with a mean age of 8.22 ± 5.66 years.
- A significant reduction in glomerular filtration rate (GFR) was observed in 34.5% of patients.
- The prevalence of crystalluria findings included hypercalciuria (60%), hyperoxaluria (41.8%), hypocitraturia (24.5%), and hyperuricosuria (47.3%).
Conclusions:
- Crystalluria is a common renal complication in children with cystic fibrosis.
- Hypercalciuria is the most prevalent crystalluric finding, followed by hyperuricosuria and hyperoxaluria.
- Decreased GFR in cystic fibrosis can be attributed to various factors, including the use of nephrotoxic medications.
Introduction:
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.
Methods:
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.
Results:
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.
Conclusion:
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.
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