Extrarenal Manifestations in Shigatoxin-associated Haemolytic Uremic Syndrome.
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.


METHODS
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.


CONCLUSIONS
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.
Division of Pediatric Nephrology, University Children's and Adolescent's Hospital, University Hospital of Cologne, Germany.
J
J
Matthies
Division of Pediatric Gastroenterology, University Children's and Adolescent's Hospital of Cologne, Germany.
C
C
Hünseler
Division of Pediatric Nephrology, University Children's and Adolescent's Hospital, University Hospital of Cologne, Germany.
R
R
Ehren
Pediatric Study Center, University Children's Hospital of Cologne, Germany.
R
R
Volland
Division of Pediatric Radiology, Department of Radiology, University Hospital of Cologne, Germany.
F
F
Körber
Division of Pediatric Nephrology, University Children's Hospital Bonn, Germany.
B
B
Hoppe
Division of Pediatric Nephrology, University Children's and Adolescent's Hospital, University Hospital of Cologne, Germany.
L T
LT
Weber
Division of Pediatric Nephrology, University Children's and Adolescent's Hospital, University Hospital of Cologne, Germany.
S
S
Habbig
D000293:Adolescent; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D001927:Brain Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D003131:Combined Modality Therapy; D004927:Escherichia coli Infections / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006333:Heart Failure / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D007223:Infant; D007415:Intestinal Obstruction / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D008297:Male; D010195:Pancreatitis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D010951:Plasma Exchange; D012189:Retrospective Studies; D022641:Shiga Toxin 2 / Q000097:blood; D054323:Shiga-Toxigenic Escherichia coli / Q000187:drug effects / Q000472:pathogenicity*; D014774:Virulence
D061067:Antibodies, Monoclonal, Humanized; D022641:Shiga Toxin 2; C481642:eculizumab