Update on Streptococcus pneumoniae associated hemolytic uremic syndrome

Abstract

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.

Thrombotic Microangiopathy and the Kidney

“…TMA may occur in adults and children in the context of invasive Streptococcus pneumoniae infection, and the high morbidity and mortality rate usually reflects the severity of the infection (70,71). In a recently published North American cohort, pneumococcal HUS was most commonly observed in children aged ,2 years with pneumonia and empyema who were extremely unwell and frequently required prolonged hospitalization and intensive care unit admission; the mortality rate was 3%, and 33% developed ESRD (72).…”

Infectious Diseases and the Kidney in Children

“…Clues to a pneumococcal cause, in addition to culture results, include severe clinical disease, especially pneumonia, empyema, pleural effusion, or meningitis; hemolytic anemia; positive results on a direct Coombs test; and difficulties in ABO crossmatching or a positive minor crossmatch incompatibility [46]. However, when renal disease is seen in the context of severe pneumococcal infection, it is important to maintain a broad diagnostic perspective, because of the frequency of acute tubular necrosis in septic shock and DIC.…”

“…The incidence following streptococcal infection is 0.4-0.6 %. Since the introduction of the 7-and 13-valent vaccines, there have been reports of HUS cases caused by strain not covered in the vaccine [46]. In 1955, Gasser and colleagues described HUS as a clinical entity in children, and they included two infants with pneumonia among the five patients they described [47].…”

Egészségügyi szakmai irányelv – A thromboticus thrombocytopeniás purpura (TTP) és a haemolyticus uraemiás syndroma (HUS) kezeléséről

“…Neuraminidázt termelő kórokozó, leggyakrabban Streptococcus pneumoniae [68] okozta fertőzést követően alakul ki, jellemzően 2 évnél fiatalabb gyermekeken. A betegség megjelenésére súlyos klinikai állapot (leggyakrabban empyemával járó pneumonia, vagy meningitis) jellemző, gyakran kíséri DIC.…”

“…terápiája [68] Mivel az alternatív komplementaktivációs út szabályozá-sában központi szerepet játszó H-faktor a sziálsavhoz kötődik a sejtmembránon, és átfedés lehet a neuraminidázmediált és a komplementmediált aHUS formák kö-zött [69], indokolt a diagnosztikai vizsgálatok kiterjesztése a komplementrendszer irányába is.…”