Remuzzi G, Galbusera M, Salvadori M, Rizzoni G, Paris S,
Bilateral nephrectomy stopped disease progression in plasma-
resistant hemolytic uremic syndrome with neurologic signs and
(Jan) 49:282-285 1996
Hemolytic uremic syndrome (HUS), an example of thrombotic and
microangiopathic process causing acute glomerular injury and renal failure,
not commonly seen in adults, although it is more common in childhood.
manifestations commonly include acute renal failure, involvement of CNS,
hypertension, and schistocytes and thrombocytopenia. Treatment consists of
control of hypertension and plasma infusion or exchange. Although success
of 80 - 90 % are reported with this approach there are patients in whom
and neurologic manifestations remain resistant with fatal outcome. Remuzzi
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
Comment: This report is significant for many reasons. It offers a
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
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
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.