Intermediate Follow-up of Pediatric Patients With Hemolytic Uremic Syndrome During the 2011 Outbreak Caused by E. coli O104:H4

Abstract

Background. In 2011 Escherichia coli O104:H4 caused an outbreak with >800 cases of hemolytic uremic syndrome (HUS) in Germany, including 90 children. Data on the intermediate outcome in children after HUS due to E. coli O104:H4 have been lacking.Methods. Follow-up data were gathered retrospectively from the medical records of patients who had been included in the German Pediatric HUS Registry during the 2011 outbreak.Results. Seventy-two of the 89 (81%) patients were included after a median follow-up of 3.0 (0.9-4.7) years. Hypertension and proteinuria were present in 19% and 28% of these patients, respectively. Of 4 patients with chronic kidney disease (CKD) > stage 2 at short-term follow-up, 1 had a normalized estimated glomerular filtration rate, and 3 (4%) had persistent CKD > stage 2. In 1 of these patients, CKD improved from stage 4 to 3; 1 who had CKD stage 5 at presentation received kidney transplantation; and 1 patient required further hemodialysis during follow-up. One patient (1.4%) still had major neurological symptoms at the latest follow-up. Dialysis during the acute phase (P = .01), dialysis duration (P = .01), and the duration of oligo-/anuria (P = .005) were associated with the development of renal sequelae. Patients treated with eculizumab (n = 11) and/or plasmapheresis (n = 13) during the acute phase of HUS had comparable outcomes.Conclusions. The overall outcome of pediatric patients after HUS due to E. coli O104:H4 was equivalent to previous reports on HUS due to other types of Shiga toxin-producing E. coli (STEC). Regular follow-up visits in patients are recommended after STEC-HUS.

Follow‐up of children with infection‐associated haemolytic uraemic syndrome 1979–1995: Would eculizumab have improved prognosis?

“…Haemolytic uraemic syndrome (HUS) was previously the most common cause of acute kidney injury in childhood . It is characterised by microangiopathic haemolytic anaemia, thrombocytopenia and acute renal insufficiency . HUS has historically been classified into two forms: (i) typical following a diarrhoeal illness and (ii) atypical, which often has an inherited and/or acquired complement abnormality .…”

“…It is characterised by microangiopathic haemolytic anaemia, thrombocytopenia and acute renal insufficiency . HUS has historically been classified into two forms: (i) typical following a diarrhoeal illness and (ii) atypical, which often has an inherited and/or acquired complement abnormality . The classification of HUS is constantly evolving as greater understanding of the molecular basis of disease has been achieved …”

“…The primary histopathological lesion in the kidney exhibits extensive glomerular capillary and arteriole endothelial cell damage with vascular lumina occlusion by microthrombi . This is the pathological description of thrombotic microangiopathy (TMA) .…”

“…TMA can manifest with a diverse range of signs and symptoms; however, acute kidney injury is a prominent feature due to the propensity of glomerular circulation to endothelial damage and occlusion . HUS caused by the shiga toxin‐producing Escherichia coli (most frequently E. coli serotype O157:H7) is defined by the presence of shiga toxin‐producing bacteria (STEC‐HUS) . There is evidence of complement activation via an alternate pathway in STEC‐HUS patients .…”

“…In STEC‐HUS, short‐term studies in children show a 10–15% risk of permanent renal impairment. This incidence is the highest in patients with prolonged anuria or longer duration of dialysis . Brenner et al .…”