Síndrome urémico hemolítico asociado a diarrea sin trombocitopenia

BALESTRACCI, ALEJANDRO; TOLEDO, ISMAEL; MENI BATTAGLIA, LUCIANA; DE LILLO, LEONARDO; MORE, NATALIA; CAO, GABRIEL; ALVARADO, CAUPOLICAN

NEFROLOGIA

SOC ESPANOLA NEFROLOGIA DR RAFAEL MATESANZ

Año: 2017 vol. 37 p. 508 - 514

0211-6995

Background: Thrombocytopenia is a hallmark of postdiarrhoeal haemolytic uraemic syn-drome (D+ HUS), although it can be transient and therefore undetected. There is scarceinformation regarding the prevalence and the course of the disease in children with D+ HUSwithout thrombocytopenia.Objective: To determine the prevalence of D+ HUS without thrombocytopenia and to describethe clinical characteristics of a series of children with this condition.Patients and methods: The medical records of patients with D+ HUS hospitalised between2000 and 2016 were reviewed to identify those without thrombocytopenia (>150,000 mm3).Demographic, clinical and laboratory parameters of the selected cases were collected anddescriptively analysed.Results: Nine cases (5.6%) without thrombocytopenia were identified among 161 patientshospitalised during the study period. Median age at diagnosis was 17 months (7?32) andmedian prodromal symptom duration was 15 days (7?21). Eight patients maintained normalurine output while the remaining one required dialysis. No patient presented with severeextrarenal compromise and/or hypertension.Conclusions: The prevalence of non-thrombocytopenic D+ HUS was 5.6% and most casesoccurred with mild forms of the disease; however, the need for dialysis in one of themindicated that normalisation of platelet count is not always an accurate marker for diseaseremittance. Our results also confirm that the time of onset of D+ HUS in patients withoutthrombocytopenia is usually delayed with respect to the initial intestinal symptoms; thus,heightened diagnostic suspicion is necessary.