Hemolysis in a patient with alkaptonuria and chronic kidney failure.

In alkaptonuria, the absence of homogentisic acid oxidase results in the accumulation of homogentisic acid (HGA) in the body. Fatal disease cases are infrequent, and death often results from kidney or cardiac complications. We report a 24-year-old alkaptonuric man with severe decreased kidney function who developed fatal metabolic acidosis and intravascular hemolysis. Hemolysis may have been caused by rapid and extensive accumulation of HGA and subsequent accumulation of plasma soluble melanins. Toxic effects of plasma soluble melanins, their intermediates, and reactive oxygen side products are increased when antioxidant mechanisms are overwhelmed. A decrease in serum antioxidative activity has been reported in patients with chronic decreased kidney function. However, despite administration of large doses of an antioxidant agent and ascorbic acid and intensive kidney support, hemolysis and acidosis could not be brought under control and hemolysis led to the death of the patient.

[Aortocoronary bypass, haemodialysis and internal mammary artery steal]. / Pontage aortocoronarien, hémodialyse et vol thoracique interne.

The dramatic occurrence, during haemodialysis sessions, of hemodynamic and cerebral symptoms in a 53-year-old haemodialysed woman with a history of aortocoronary bypass leads to discovery and treatment by percutaneous angioplasty and stenting of a significant stenosis of proximal subclavian artery, ipsilateral to the arteriovenous fistula, with retrograde flow in internal mammary artery graft and vertebral artery. All symptoms resume after interventional radiology. Analysis of literature confirms possibility of coronary artery steal induced by use of the arteriovenous fistula in haemodialysed patients with ipsilateral internal mammary bypass, especially if there is concomitant subclavian artery stenosis, and leading us to discuss the diagnostic and therapeutic implications.