Hyperoxaluria is characterized by nephrolithiasis and nephrocalcinosis caused by supersaturation of calcium oxalate in the urine. Primary hyperoxaluria type 1 and 2 (PH1 and PH2) are rare autosomal recessive disorders with defective glyoxalate metabolism in the liver resulting in increased oxalate production [1]. Secondary hyperoxaluria is due to reduced excretion, excessive dietary intake, or increased gut absorption of oxalate [2-4]. Idiopathic hyperoxaluria has no known associated gene defect. When left untreated, hyperoxaluria will ultimately lead to renal failure, which in turn results in oxalosis, a condition in which calcium oxalate crystals are deposited in extrarenal organs. Oxalate deposition most commonly affects the bone, bone marrow, blood vessels, central nervous system, peripheral nerves, retina, skin, and thyroid [5].
We present an interesting case of a patient with primary hyperoxaluria and oxalosis involving the kidneys, small intestine, skin, and heart. To our knowledge, intestinal deposition of calcium oxalate has not been previously reported.