American journal of kidney diseases : the official journal of the National Kidney Foundation
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A 15-year-old patient with severe bone disease (with bilateral fractures of hips and shoulders) due to primary hyperoxaluria type 1 (PH1) was treated with combined liver-kidney transplantation after a 4-year hemodialysis period. Normalization of excessive oxalate synthesis brought in by the liver graft combined with the slow excretion of skeletal oxalate stores by the renal graft led to progressive improvement of clinical, radiological, and histological evidence of oxalate osteopathy. This allowed bilateral hip replacement 3 years after transplantation, which led to complete physical rehabilitation of the crippled patient. Combined liver-kidney transplantation constitutes the treatment of choice for end-stage renal failure due to PH1, even in the face of severe oxalate bone disease.
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Resistant hypertension and paroxystic hemodynamic changes are common features in patients on regular hemodialysis. We describe a hemodialyzed patient who developed malignant hypertension. The finding of elevated plasma catecholamines suggested the diagnosis of pheochromocytoma, which was confirmed by surgical removal of the tumor.