[Hemolytic uremic syndrome with an atypical presentation]
2010, Revue médicale de Liège
Abstract
The hemolytic uremic syndrome (HUS) is rare and of guarded prognosis in adults. It expresses a renal thrombotic microangiopathy. We report the case of a young patient whose clinical presentation and exploration make a diagnosis of HUS likely. The atypical presentation provides an opportunity not only to discuss all diagnostic elements based on recent pathophysiological hypotheses, but also to highlight recommendations for the management of this severe disease.
References (10)
- Noris M, Remuzzi G.-Atypical Hemolytic -Uremic Syndrome. N Engl J Med, 2009, 361, 1676-1687.
- Dilhuydy MS, Delclaux C, Pariente A, et al.-Syndrome hémolytique et urémique compliquant un traitement au long cours par gemcitabine. À propos d'un cas, revue de la littérature. La Revue de Médecine Interne, 2002, 23, 189-192.
- Ruggenenti P, Noris M, Remuzzi G.-Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int, 2001, 60, 831-846.
- George J.N.-Thrombotic Thrombocytopenic Purpura. N Engl J Med, 2006, 354, 1927-1935.
- Dundas S, Murphy J, Soutar RL, et al.-Effectiveness of therapeutic plasma exchange in the 1996 lanarkshire Escherichia coli 0157:H7 outbreak. Lancet, 1999, 354, 1327-1330.
- Ahmad A, Aggarwal A, Sharma D, et al.-Rituximab for treatment of refractory/relapsing thrombotic throm- bocytopenic purpura (TTP). Am J Hemat, 2004, 77, 171- 176.
- Michael M, Elliott EJ, Ridley GF, et al.-Interventions for haemolytic uraemic syndrome and thrombotic throm- bocytopenic purpura. Cochrane Database of Systematic Reviews, 2009, CD003595.
- Fakhouri F, Vernant J-P, Veyradier A, et al.-Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases. Blood, 2005, 106, 1932- 1937.
- Gruppo RA, Rother RP.-Eculizumab for Congenital Atypical Hemolytic-Uremic Syndrome. N Engl J Med, 2009, 360, 544-546.
- V. D'Orio, Service des Urgences, CHU de Liège, 4000 Liège, Belgique.