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Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
Osmoregulation in children with cystic fibrosis
Yury V Natochin1, Alla A Kuznetsova2,3, Anastasia V Prokopenko2,3
1I.M. Sechenov Institute of Evolutionary Physiology and Biochemistry, Laboratory of Renal Physiology and Water Salt Balance, Russian Academy of Sciences, Pr. Torez 44, Saint Petersburg, Russia, 194223. natochin1@mail.ru.
Insights
Children with cystic fibrosis maintain normal kidney function for water balance. This study found preserved renal concentrating and diluting capacity in pediatric cystic fibrosis patients, suggesting osmoregulation is intact.
Area of Science:
- Pediatric Nephrology
- Endocrinology
- Cystic Fibrosis Research
Background:
- Hyponatremia is a recognized complication in cystic fibrosis patients.
- Previous hypotheses suggested potential endocrine and renal imbalances in water and salt homeostasis contributing to hyponatremia.
Purpose of the Study:
- To investigate the renal concentrating and diluting abilities in children with cystic fibrosis.
- To evaluate the hypothesis of a generalized endocrine and renal imbalance in water and salt homeostasis in this population.
Main Methods:
- Assessed urinary concentrating ability via overnight water deprivation in 12 cystic fibrosis patients and control groups.
- Evaluated renal diluting ability using a water-load test in the same groups.
- Compared urine osmolality, diuresis, and solute-free water excretion among groups.
Main Results:
- No significant differences in urine osmolality were observed between cystic fibrosis patients and control groups after water deprivation.
- The water-load test demonstrated comparable decreases in urine osmolality and increases in diuresis and solute-free water excretion across all groups.
- These findings indicate preserved renal function in managing water balance.
Conclusions:
- Children with cystic fibrosis exhibit normal renal concentrating and diluting capacities.
- A generalized endocrine and renal imbalance in water and salt homeostasis is unlikely to be the primary cause of hyponatremia in cystic fibrosis.
- Osmoregulation appears to be preserved in pediatric cystic fibrosis patients.
Abstract:
Hyponatremia is not rare in cystic fibrosis and might be due to several mechanisms. An endocrine and renal imbalance in water and salt homeostasis was suggested. To address this hypothesis, we assessed the urinary concentrating and diluting ability in 12 cystic fibrosis patients (6 females, 6 males) and in two control groups: 14 children with pneumonia (9 females, 5 males) and in 13 healthy children (9 females, 4 males). Renal concentrating ability was evaluated following overnight water deprivation. Urine osmolality was not significantly different between groups. Renal diluting ability was assessed by means of a water-load test. This provoked a decrease in urine osmolality, as well as an increase in diuresis and solute-free water excretion. These changes were comparable among groups.Conclusion: Children with cystic fibrosis show a preserved renal concentrating and diluting capacity. A generalized endocrine and renal imbalance in water and salt homeostasis therefore appears unlikely.What is Known:•Hyponatremia sometimes occurs in cystic fibrosis.What is New:•Osmoregulation is normal in cystic fibrosis.
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