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Hyperuricosuria due to high-dose pancreatic extract therapy in cystic fibrosis
Insights
Over-supplementing pancreatic enzymes in cystic fibrosis (CF) patients can lead to hyperuricosuria, increasing the risk of kidney problems. Adhering to minimal effective enzyme doses is crucial for managing CF and preventing complications.
Area of Science:
- Pediatric Nephrology
- Gastroenterology
- Genetic Disorders
Background:
- Cystic Fibrosis (CF) management often involves pancreatic enzyme replacement therapy.
- Hyperuricosuria, the excess excretion of uric acid in urine, can lead to kidney stones and renal injury.
- Normal serum uric acid levels do not preclude urinary uric acid abnormalities.
Observation:
- A child with CF presented with dysuria, uric acid crystalluria, and hyperuricosuria despite normal serum uric acid.
- Hyperuricosuria in three pediatric patients was directly correlated with high intake of pancreatic extract.
- A significant percentage (15/32) of randomly screened CF patients in a clinic were over-medicated with pancreatic enzymes, with most exhibiting hyperuricosuria.
Findings:
- Reducing pancreatic extract dosage by 85% in affected children led to substantial decreases in purine intake and urinary uric acid excretion.
- Over-medication with pancreatic enzymes, often initiated by parental decisions, was identified as the cause of hyperuricosuria.
- A strong association was observed between excessive pancreatic enzyme dosage and hyperuricosuria in pediatric CF patients.
Implications:
- Determining and adhering to the minimal effective dose of pancreatic extract is recommended for all children with cystic fibrosis.
- Preventing hyperuricosuria through appropriate enzyme dosing may mitigate the risk of renal injury in CF patients.
- This highlights the importance of careful monitoring and dose adjustment of pancreatic enzyme therapy in cystic fibrosis care.
Abstract:
Dysuria, uric acid crystalluria and hyperuricosuria developed in a child with cystic fibrosis and normal serum uric acid. Hyperuricosuria in this patient and two other children was directly related to ingestion of large amounts of pancreatic extract. In these three children, reducing pancreatic extract dosage by 85 percent lowered their purine intake by 307, 225, and 148 mg, respectively; urinary uric acid excretion decreased by 245, 239, and 158 mg. Overmedication resulted from parents' decisions to increase enzyme dosages. In our cystic fibrosis clinic, 15 of 32 patients screened at random were taking higher than the prescribed dose of pancreatic enzymes, and 14 of these 15 children were hyperuricosuric. On the basis of this information, we suggest that the minimal effective dose of pancreatic extract should be determined and adhered to for each child with cystic fibrosis to avoid potential renal injury from hyperuricosuria.