HDCN Article Review/Hyperlink

Remuzzi G, Galbusera M, Salvadori M, Rizzoni G, Paris S, Ruggenenti P

Bilateral nephrectomy stopped disease progression in plasma- resistant hemolytic uremic syndrome with neurologic signs and coma

Kidney Int (Jan) 49:282-285 1996

Hemolytic uremic syndrome (HUS), an example of thrombotic and microangiopathic process causing acute glomerular injury and renal failure, is not commonly seen in adults, although it is more common in childhood. Clinical manifestations commonly include acute renal failure, involvement of CNS, severe hypertension, and schistocytes and thrombocytopenia. Treatment consists of control of hypertension and plasma infusion or exchange. Although success rates of 80 - 90 % are reported with this approach there are patients in whom renal and neurologic manifestations remain resistant with fatal outcome. Remuzzi et al report four such cases who responded to bilateral nephrectomy. Patients, all female, ranged in age from 11 to 26 years of age and all had severe hypertension, variable degrees of renal insufficiency and CNS dysfunction. Despite plasma exchanges and infusions (22-110 treatments per patient) inexorable progression of the disease was seen in all. Examination of von Willebrand factor fragmentation, an index of shear stress induced injury endothelium, during the acute phase showed a reduction in the high mol wt species and increase in the low mol wt multimers. Nephrectomy was associated with prompt resolution of CNS manifestations, thrombocytopenia and hypertension; the changes in the von Willebrand factor multimers were restored to normal.

Comment: This report is significant for many reasons. It offers a potentially life saving solution to the vexing problem of HUS resistant to conventional therapy. It also suggests that the kidneys are a major seat of endothelial injury and platelet consumption in HUS, the removal of which improves systemic manifestations of the disease. As the authors appropriately conclude, this radical approach should be reserved only for patients who have failed conventional plasma exchange/infusion , have biopsy evidence of chronic HUS in the kidney and have life threatening signs such as coma. Whether similar success is seen with a larger number of patients remains to be seen. B.S. Kasinath, M.D.