L. Kragelj
Enota Šentvid, Zdravstveni dom Ljubljana, Ljubljana, Slovenija

T. Kersnik Levart
Klinični oddelek za nefrologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Abstract

Haemolytic uraemic syndrome (HUS) is a clinical entity generally defined by the simultaneous occurrence of microangiopathic non-immune haemolytic anaemia, thrombocytopenia and acute kidney injury. It is among the most common causes of the latter in children. Traditionally, HUS had been divided into diarrhoea-positive (typical HUS) and diarrhoea-negative HUS (atypical HUS). Based on new findings, the definitions of primary or atypical HUS and secondary HUS have been established. Primary or atypical HUS is due to alternative complement pathway dysregulation, whereas secondary HUS is the consequence of another coexisting disease (most commonly infection). In this article, we describe the basic characteristics of HUS, followed by a case report of a nine-year-old girl with a relatively typical course of secondary HUS, caused by Escherichia coli infection. We outline the features that could have altered the clinical decision-making and course of the disease.

Key words: haemolytic uraemic syndrome, Escherichia coli, child.