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Encapsulating peritoneal sclerosis in a patient with primary hyperoxaluria type 1: a case report
Jill Vanmassenhove1, Raymond Vanholder, Ramses Forsyth
1Renal Division, Department of Internal Medicine, University Hospital Ghent, Belgium. Jill.Vanmassenhove@ugent.be
Summary
Primary hyperoxaluria type 1 (PH1), a metabolic disorder, can lead to kidney failure. A PH1 patient developed encapsulating peritoneal sclerosis (EPS) during dialysis, possibly linked to oxalate crystal deposition.
Area of Science:
- Nephrology
- Metabolic Disorders
- Gastroenterology
Background:
- Primary hyperoxaluria type 1 (PH1) is a rare genetic disorder of glyoxylate metabolism.
- Defective alanine/glyoxylate aminotransferase activity increases oxalate production, leading to kidney damage and renal failure.
Observation:
- A 39-year-old patient with end-stage renal failure due to PH1 developed encapsulating peritoneal sclerosis (EPS) after 5 years of peritoneal dialysis.
- EPS is characterized by peritoneal fibrosis and intestinal adhesions, often associated with peritoneal dialysis.
Findings:
- Calcium oxalate crystals were found in the patient's peritoneal fascia.
- This suggests a potential link between oxalate crystal deposition and the development of EPS.
Implications:
- The findings suggest a possible causative relationship between calcium oxalate deposition and EPS in PH1 patients undergoing peritoneal dialysis.
- Further research is needed to clarify the role of peritoneal dialysis in EPS development in these patients.
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