Extrarenal Manifestations in Shigatoxin-associated Haemolytic Uremic Syndrome

Abstract

Background: Shigatoxin-associated haemolytic uremic syndrome (STEC-HUS) is the most frequent cause of acute kidney injury in children worldwide. Extrarenal manifestations are the main determinants for both, short- and long-term prognosis of patients with STEC-HUS.

Patients: 46 patients treated over the last 10 years for STEC-HUS in a single center.

Methods: This retrospective study analysed the incidence and outcome of extrarenal manifestations in our cohort of children with STEC-HUS. Risk factors for extrarenal involvement and adverse outcome were assessed by detailed chart review.

Results: Eleven extrarenal manifestations occurred in 9/46 patients comprising 8 neurological, 2 gastro-intestinal, and 1 cardiovascular complication. One patient died from cerebral bleeding. Liver transplantation was required in a girl 18 months after HUS due to secondary sclerosing cholangitis. Patients with extrarenal manifestations were significantly younger and presented with higher leucocyte counts and higher alanine aminotransferase levels at admission. Renal replacement therapy was necessary for a longer period than in patients without extrarenal complications.

Conclusion: Extrarenal manifestations occurred in about 20% of our patients with STEC-HUS. The identification of risk-factors will help to provide a better management of these patients which might also include novel treatment strategies like complement inhibition.

Zusammenfassung

Hintergrund: Shigatoxin-assoziiertes hämolytisch-urämisches Syndrom (STEC-HUS) ist der häufigste Grund für ein akutes Nierenversagen bei Kindern weltweit. Extrarenale Manifestationen haben einen bedeutenden Einfluss sowohl für die kurz- als auch langfristige Prognose der Patienten.

Patienten: 46 Patienten mit STEC-HUS, die in den letzten 10 Jahre in unserem Zentrum behandelt wurden.

Methoden: Diese retrospektive Studie analysiert die Inzidenz und die Folgen von extrarenalen Komplikationen bei STEC-HUS. Zudem werden mögliche Risikofaktoren für eine extrarenale Beteiligung untersucht.

Ergebnisse: Elf extrarenale Manifestationen sind bei insgesamt 9/46 Patienten aufgetreten: 8 neurologische, 2 gastroenterologische und 1 kardiovaskuläre Komplikation. Ein Patient verstarb an einer Gehirnblutung. Eine Patientin erhielt eine Lebertransplantation 18 Monate nach STEC-HUS aufgrund einer sekundär sklerosierenden Cholangitis. Patienten mit extrarenalen Manifestationen waren signifikant jünger und hatten bei ihrer Aufnahme höhere Leukozyten- und Alanin-Aminotransferase-Werte. Ebenso war die Dialysetherapie über einen längeren Zeitraum notwendig.

Schlussfolgerung: Extrarenale Manifestationen traten bei 20% der Patienten auf. Die Identifikation von Risikofaktoren ermöglicht eine bessere Behandlung dieser Patienten, welche möglicherweise den Einsatz neuer Therapiemöglichkeiten wie Komplementinhibierung beinhalten.

Keywords

Children - Escherichia coli - Thrombotic Microangiopathy - Dialysis - Eculizumab

Schlüsselwörter

Kinder - Escherichia coli - Thrombotische Mikroangiopathie - Dialyse - Eculizumab

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