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Hypercalciuria and nephrocalcinosis in cystic fibrosis patients
Uğur Ozçelik1, Nesrin Beşbaş, Ayhan Göçmen
1Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Insights
Cystic fibrosis (CF) patients frequently experience hypercalciuria and nephrocalcinosis, suggesting a primary kidney calcium metabolism issue. Further research is needed to understand these common CF complications.
Area of Science:
- Nephrology
- Pediatrics
- Genetics
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs.
- Nephrocalcinosis and hypercalciuria are potential complications in CF patients.
- Understanding the causes of these renal abnormalities in CF is crucial for patient management.
Purpose of the Study:
- To determine the frequency of nephrocalcinosis and hypercalciuria in pediatric CF patients.
- To investigate potential causes and correlations of these renal findings in CF.
Main Methods:
- Analysis of plasma and urine biochemistry, including calcium and vitamin D levels.
- Assessment of renal function (GFR) and microscopic hematuria.
- Renal ultrasonography and correlation with clinical data (Shwachman score, Pseudomonas colonization, pseudo Bartter's syndrome, CF mutations).
Main Results:
- Hypercalciuria detected in 34.2% and nephrocalcinosis in 23.2% of CF patients.
- Borderline correlation between 25-hydroxyvitamin-D and hypercalciuria; no significant correlations with age or Shwachman score.
- No association found with Pseudomonas colonization, pseudo Bartter's syndrome, or specific CF mutations.
Conclusions:
- The study suggests a primary abnormality in kidney calcium metabolism contributes to hypercalciuria and nephrocalcinosis in CF.
- These renal complications appear independent of common CF clinical parameters and genetic factors.
- Further investigation into calcium regulation pathways in CF kidneys is warranted.
Abstract:
The objective of this study was to determine the frequency of nephrocalcinosis and hypercalciuria in cystic fibrosis (CF) patients, and to search possible causes of this phenomenon. Forty-three CF children (24 boys, 19 girls; mean age 64.9 months, range 5 months-18 years) were included in this study. Plasma sodium, potassium, chloride, BUN, creatinine, calcium, phosphorus, magnesium, alkaline phosphatase; spot urine sodium, potassium, chloride, creatinine, calcium, magnesium; and serum 25-hydroxyvitamin-D levels were measured in all patients. Urine samples were examined for microscopic hematuria. Fractional sodium, potassium, chloride excretion and estimated glomerular filtration rate (GFR) were calculated. All patients underwent renal ultrasonography. Hypercalciuria, nephrocalcinosis and microscopic hematuria were detected in 15 patients (34.2%), 10 patients (23.2%) and two patients (5%), respectively. There was no significant but borderline correlation between 25-hydroxyvitamin-D levels and hypercalciuria (r: 0.308, p:0.05). There were no correlations between Shwachman clinical scoring system results and hypercalciuria (r: 0.221, p: 0.148) and age and hypercalciuria (r: -0.229, p: 0.135). Patients with chronic Pseudomonas colonization showed no hypercalciuria or nephrocalcinosis. There was no difference for plasma biochemical results, renal function tests, hypercalciuria and nephrocalcinosis between CF patients who had or had not experienced pseudo Bartter's syndrome (PBS) before. There was no relation between detected CF mutations of the patients and hypercalciuria and nephrocalcinosis. These results suggested that it is a primary abnormality of calcium metabolism in the kidney.
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