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SHORT REPORTS |
Renal Division1 Department of Internal Medicine Department of Anatomopathology2 University Hospital Ghent, Belgium
* e-mail: Jill.Vanmassenhove{at}ugent.be
Primary hyperoxaluria type 1 (PH1) is a rare metabolic disorder caused
by a defect in glyoxylate metabolism attributable to low or absent activity of
the liver-specific peroxisomal enzyme alanine/glyoxylate aminotransferase.
This defect leads to enhanced conversion of glyoxylate to poorly soluble
oxalate, which is then excreted into the urine. This process may lead to
deposition of calcium oxalate crystals in many tissues as well as in the
kidneys, resulting in nephrolithiasis, nephrocalcinosis, and/or renal
failure.
We present a 39-year-old patient with end-stage renal failure due to
PH1, who was admitted with symptoms of feeling bloated, vomiting, diarrhea,
and abdominal pain related to encapsulating peritoneal sclerosis (EPS). He had
been treated with peritoneal dialysis for a total period of 5 years.
EPS is a rare condition characterized by fibrosis and adhesions of the
peritoneum to loops of the small intestine and has been described secondary to
treatment with peritoneal dialysis. It also occurs in a variety of other
clinical conditions such as autoimmune diseases and peritoneal and
intra-abdominal malignancies.
The calcium oxalate crystals found in the peritoneal fascia of this
particular patient may suggest a causative relationship between crystal
deposits and evolution to fibrosis and sclerosis of the peritoneum. The degree
of impact of the peritoneal dialysis treatment itself on the development of
EPS, however, is uncertain.
KEY WORDS: Encapsulating peritoneal sclerosis; primary hyperoxaluria type 1; systemic oxalosis.
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