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Streptococcus pneumoniae-associated hemolytic uremic syndrome (HUS) is an underrecognized condition that mainly occurs in young children. Early diagnosis is important because of the potential to improve morbidity and mortality rates. The purposes of this report are to review the clinical and laboratory features of 14 patients with pneumococcal HUS and present a modified classification to capture cases that may not have been documented with a diagnosis of pneumococcal HUS. We thereby provide a rationale for including patients with concurrent disseminated intravascular coagulopathy and/or those whose culture results were negative, and we highlight the emergence of serotype 19A subsequent to the introduction of 7-valent pneumococcal protein conjugate vaccine (Prevnar). This is the largest series of such subjects (to our knowledge) from a single center. Sixty-four percent of the patients recovered without any long-term sequelae. Three patients developed chronic kidney disease, 1 developed end-stage renal failure, and 1 died in the acute phase. The greatest risk factor for the development of chronic kidney disease is the need for acute dialysis for >20 days, and death in the acute phase is rare unless meningitis is the primary infection. [ABSTRACT FROM AUTHOR]