PURPOSE OF REVIEW: Streptococcus pneumoniae associated hemolytic uremic syndrome (SpHUS) is defined by the occurrence of acute hemolytic anemia, thrombocytopenia and acute kidney injury in a patient with a S. pneumoniae infection. We review the pathophysiology, clinical course, treatment and prognosis for SpHUS. We also describe an expanded classification system that uses additional diagnostic criteria to identify more patients with a high likelihood of having SpHUS. RECENT FINDINGS: SpHUS often may be underdiagnosed because of overlapping features with disseminated intravascular coagulation (DIC) and the lack of strict diagnostic criteria. The epidemiology has changed with the emergence of different pneumococcal serotypes as newer pneumococcal vaccines have been introduced. SUMMARY: SpHUS accounts for 5-15% of all HUS cases. The majority of SpHUS patients have pneumonia and a low mortality rate in contrast to those with meningitis, who have a more severe clinical course. Although the pathogenesis of SpHUS remains unknown, the Thomsen-Friedenreich antigen seems to play a central role. S. pneumoniae produces neuraminidase, thereby exposing the Thomsen-Friedenreich antigen on the surface of cell membranes. Thomsen-Friedenreich antigen exposure can result in hemolysis and direct endothelial injury leading to HUS phenotype. Early identification of these patients is critical so that fresh frozen plasma may be avoided.
PURPOSE OF REVIEW: Streptococcus pneumoniae associated hemolytic uremic syndrome (SpHUS) is defined by the occurrence of acute hemolytic anemia, thrombocytopenia and acute kidney injury in a patient with a S. pneumoniae infection. We review the pathophysiology, clinical course, treatment and prognosis for SpHUS. We also describe an expanded classification system that uses additional diagnostic criteria to identify more patients with a high likelihood of having SpHUS. RECENT FINDINGS: SpHUS often may be underdiagnosed because of overlapping features with disseminated intravascular coagulation (DIC) and the lack of strict diagnostic criteria. The epidemiology has changed with the emergence of different pneumococcal serotypes as newer pneumococcal vaccines have been introduced. SUMMARY: SpHUS accounts for 5-15% of all HUS cases. The majority of SpHUS patients have pneumonia and a low mortality rate in contrast to those with meningitis, who have a more severe clinical course. Although the pathogenesis of SpHUS remains unknown, the Thomsen-Friedenreich antigen seems to play a central role. S. pneumoniae produces neuraminidase, thereby exposing the Thomsen-Friedenreich antigen on the surface of cell membranes. Thomsen-Friedenreich antigen exposure can result in hemolysis and direct endothelial injury leading to HUS phenotype. Early identification of these patients is critical so that fresh frozen plasma may be avoided.
Authors: Chantal Loirat; Fadi Fakhouri; Gema Ariceta; Nesrin Besbas; Martin Bitzan; Anna Bjerre; Rosanna Coppo; Francesco Emma; Sally Johnson; Diana Karpman; Daniel Landau; Craig B Langman; Anne-Laure Lapeyraque; Christoph Licht; Carla Nester; Carmine Pecoraro; Magdalena Riedl; Nicole C A J van de Kar; Johan Van de Walle; Marina Vivarelli; Véronique Frémeaux-Bacchi Journal: Pediatr Nephrol Date: 2015-04-11 Impact factor: 3.714
Authors: Helene Werner; Kathrin Buder; Markus A Landolt; Thomas J Neuhaus; Guido F Laube; Giuseppina Spartà Journal: Pediatr Nephrol Date: 2016-12-23 Impact factor: 3.714
Authors: Dejan Lavrek; Sebastiano A G Lava; Gregorio P Milani; Giacomo D Simonetti; Mario G Bianchetti; Olivier Giannini Journal: J Nephrol Date: 2018-10-17 Impact factor: 3.902
Authors: Karen van Hoeve; Corinne Vandermeulen; Marc Van Ranst; Elena Levtchenko; Lambert van den Heuvel; Djalila Mekahli Journal: Eur J Pediatr Date: 2017-01-21 Impact factor: 3.183
Authors: Ravi Shankar Keshari; Narcis Ioan Popescu; Robert Silasi; Girija Regmi; Cristina Lupu; Joe H Simmons; Alonso Ricardo; K Mark Coggeshall; Florea Lupu Journal: Proc Natl Acad Sci U S A Date: 2021-09-14 Impact factor: 11.205