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Renal function in cystic fibrosis.

B M Assael, G Marra, A S Tirelli

    The International Journal of Pediatric Nephrology
    |October 1, 1986
    PubMed
    Summary
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    Patients with cystic fibrosis (CF) exhibit altered renal function, showing increased glomerular filtration rate and renal plasma flow after saline expansion. This suggests a generalized electrolyte transport disorder in CF affects kidney function.

    Area of Science:

    • Nephrology
    • Physiology
    • Genetics

    Background:

    • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
    • Previous research suggests potential electrolyte imbalances in CF patients.
    • The impact of extracellular volume expansion on renal function in CF is not well understood.

    Purpose of the Study:

    • To investigate the effects of saline extracellular volume expansion on renal function in patients with cystic fibrosis.
    • To compare renal hemodynamic and tubular handling responses in CF patients versus healthy controls.

    Main Methods:

    • Administered saline extracellular volume expansion (4 ml/min/10 kg b.w. X 60 min) to CF patients and age-matched controls.
    • Measured glomerular filtration rate (GFR), renal plasma flow (RPF), and tubular sodium and chloride (Na, Cl) handling.

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  • Assessed renin and aldosterone levels.

    • Main Results:

    • CF patients showed significant increases in GFR and RPF post-saline expansion, unlike controls.
    • Increased proximal tubular reabsorption of NaCl was observed in CF patients, contrasting with reduced reabsorption in controls.
    • Renin and aldosterone levels did not indicate chronic volume contraction or salt loss as the cause of increased NaCl reabsorption.

    Conclusions:

    • Renal hemodynamics are altered in cystic fibrosis patients.
    • The renal tubule appears involved in the generalized electrolyte transport disorder seen in CF.
    • Observed changes suggest a potential tubulo-glomerular feedback mechanism in CF-related renal alterations.