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Microscopic nephrocalcinosis in cystic fibrosis
S M Katz1, L J Krueger, B Falkner
1Division of Renal Pathology, Hahnemann University, Philadelphia, PA 19102.
The New England Journal of Medicine
|August 4, 1988
Summary
Cystic fibrosis (CF) patients show kidney defects, with 92% exhibiting microscopic nephrocalcinosis and 36% having hypercalciuria. These renal calcium deposits appear early, suggesting a primary CF-related metabolic abnormality.
Area of Science:
- Nephrology
- Genetics
- Pediatrics
Background:
- Cystic fibrosis (CF) is characterized by abnormal ion transport in epithelial cells.
- The kidney, a critical organ for ion transport, was investigated for similar defects in CF patients.
Purpose of the Study:
- To determine if CF patients exhibit renal calcium deposition (nephrocalcinosis) and abnormal calcium excretion (hypercalciuria).
- To investigate the potential link between early-onset nephrocalcinosis and the underlying genetic defect in CF.
Main Methods:
- Analysis of 38 kidney tissue specimens for calcium deposits.
- Measurement of 24-hour urinary calcium excretion in 14 CF patients and 15 controls.
Main Results:
- Microscopic nephrocalcinosis was found in 92% of CF kidney specimens.
- Hypercalciuria was observed in 36% of CF patients.
- Nephrocalcinosis was detected in infants, including neonates, suggesting an early-onset defect.
Conclusions:
- Early-onset nephrocalcinosis in CF patients indicates a primary abnormality in kidney calcium metabolism.
- These findings suggest that renal manifestations are integral to cystic fibrosis pathophysiology.
- Further research into CF kidney pathology may reveal key molecular alterations.