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Management of severe polyuria in idiopathic Fanconi syndrome
Guido Filler1,2,3,4,5, Rishika Geda6,7, Fabio Salerno8,9
1Department of Pediatrics, Schulich School of Medicine and Dentistry, University of Western Ontario, 800 Commissioners Road East, E3-206, Ontario, N6A 5W9, London, Canada. guido.filler@lhsc.on.ca.
Insights
Indomethacin effectively treated severe polyuria in a patient with idiopathic Fanconi syndrome, reducing urine output and sodium wasting. This offers a potential new treatment for this debilitating condition.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Pharmacology
Background:
- Polyuria is a significant challenge in tubular diseases like chronic kidney disease (CKD) and Fanconi syndrome.
- Current treatment guidelines for polyuria are lacking, and vasopressin is often ineffective.
- Idiopathic Fanconi syndrome can cause debilitating polyuria, impacting quality of life.
Observation:
- A 13-year-old female with idiopathic Fanconi syndrome experienced severe polyuria (5 L/day), affecting mood and academic performance.
- Despite literature discouraging its use, indomethacin was administered at 2 mg/kg/day.
- Treatment resulted in a significant reduction in urine output and sodium wasting.
Findings:
- Urine output decreased from 5 L to 2.5 L per day.
- Daily sodium wasting reduced from 24.6 to 7.7 mmol/kg/day.
- While eGFR showed a temporary dip, it stabilized, and 23Na MRI indicated changes in skin and muscle sodium levels.
Implications:
- Indomethacin presents a potential safe and effective therapeutic option for managing polyuria in idiopathic Fanconi syndrome.
- This case highlights a novel approach in the absence of established treatment protocols.
- Further research is warranted to confirm the long-term efficacy and safety of indomethacin for polyuria in Fanconi syndrome.
Background:
Polyuria is a common problem in patients with tubular diseases, especially for those with CKD and high-output Fanconi syndrome. There are currently no guidelines on how to treat debilitating polyuria, in children or adults, and vasopressin is usually not effective.
Case-Diagnosis/Treatment:
A 13-year-old female with idiopathic Fanconi syndrome and an eGFR of 69 mL/min/1.73 m2 was severely affected by polyuria of 5 L per day (voiding at least 11 times during the day and up to 8 times at night), impacting her mood (measured by the RCADS-child) and academic performance at school. In the absence of guidelines and with literature discouraging the use of indomethacin in this condition, we attempted indomethacin treatment at a dose of 2 mg/kg divided in two doses with substantial success. Urine output dropped to 2.5L and this was accompanied by a substantial decrease of her sodium wasting from 24.6 to 7.7 mmol/kg/day. Over the course of 18 months, the patient's eGFR dropped temporarily to 60 mL/min/1.73 m2 and was 68 mL/min/1.73 m2 at last follow-up. However, a sodium-23 (23Na) MRI of her thigh revealed ongoing moderate sodium decrease in her skin and substantial Na+ decrease in her muscle when compared to age-matched peers with normal kidney function.
Conclusions:
Indomethacin may be a safe and effective treatment option for polyuria in idiopathic Fanconi syndrome.
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