Atypical haemolytic uraemic syndrome (aHUS) is a rare disorder characterised by thrombocytopaenia and evidence of microangiopathic haemolysis. ADAMTS13 levels are normal and STEC (Shiga toxin-producing
A 46-year-old man with no past medical history and no regular medications presented to a rural hospital with an acute abdomen. An explorative laparotomy revealed a small bowel obstruction secondary to a congenital band with an associated strangulated segment that required an ileocaecal resection. The patient received antibiotic cover with cephazolin, gentamicin, and metronidazole in addition to opioid analgesia. On the tenth postoperative day he had watery diarrhoea. On the following day the patient became anuric with moderate hypertension (BP 160/80).
Blood tests revealed an acute kidney injury with a sudden rise in creatinine from 71 to 307
The stool sample was positive for
The patient received daily plasma exchange and haemodialysis in the Intensive Care Unit. Intravenous metronidazole and oral vancomycin were given as antibiotic therapy for
Eculizumab infusions were commenced with the cessation of plasma exchange. The protocol involved infusions of 900 mg weekly for four weeks followed by a single infusion of 1200 mg in the fifth week. During the maintenance phase the patient received fortnightly infusions of 1200 mg for one year.
Eculizumab treatment led to a normalisation in haemoglobin, platelet count, LDH, and haptoglobin over the following fortnight. The patient then became dialysis-independent and serum creatinine stabilised at 200
He continued to receive eculizumab as an outpatient for one year. There was no relapse of disease on eculizumab cessation. He continues to be monitored with regular blood tests. His renal function remains stable at a new baseline creatinine of 130
Atypical haemolytic uraemic syndrome is a clinical diagnosis based on microangiopathic haemolytic anaemia, thrombocytopaenia, and acute kidney injury in the absence of STEC with normal ADAMTS13 activity. Episodes may be precipitated by infection in patients with a genetic susceptibility to complement dysregulation. However, specific complement gene pathway mutations are found in only 50–60% of cases of true aHUS [
This case provides further evidence for
Published reports of
Reference | Age/sex | Kidney | Clinical features | Treatment | Dialysis | Outcome |
---|---|---|---|---|---|---|
[ | 51/female | Native | Watery diarrhoea, confusion | Vancomycin | No | Recovery |
[ | 46/female | Native | Bloody diarrhoea, vomiting | Metronidazole, plasmapheresis | Yes | Recovery |
[ | 62/female | Native | Watery diarrhoea | Metronidazole, plasmapheresis, steroids | Yes | Recovery |
[ | 73/female | Native | Watery diarrhoea, respiratory distress, chills, anuria | Metronidazole, steroids | Yes | Recovery |
[ | 29/female | Transplant | Diarrhoea, decreased urine output | Vancomycin | Yes | Complete recovery |
[ | 52/female | Transplant | Fever, diarrhoea, nausea, vomiting | Metronidazole, plasmapheresis | Yes | Allograft failure and transplant nephrectomy |
[ | 63/female | Native | Bloody diarrhoea, fever, confusion | Metronidazole, vancomycin, plasmapheresis | Yes | Partial recovery |
Current case | 46/male | Native | Watery diarrhoea, anuria | Plasmapheresis, metronidazole, vancomycin and eculizumab | Yes | Recovery |
Various treatments have been used in the reported cases of
Terminal complement inhibitors have emerged as an effective therapy for aHUS. Eculizumab has been shown to control haemolysis and lead to improvements in renal function [
The patient has given informed consent for this case report to be published.
The authors confirm that this case report has not been published previously except in abstract format.
The authors declare that they have no conflicts of interest.