PT  - JOURNAL ARTICLE
AU  - Filipe Santos Mira
AU  - Ana Luísa Nunes
AU  - Ana Rita Elvas
AU  - Nuno Oliveira
TI  - Atypical haemolytic uremic syndrome from multiple missenses to a full-blown disease
AID  - 10.1136/bcr-2019-230026
DP  - 2019 Jun 01
TA  - BMJ Case Reports
PG  - e230026
VI  - 12
IP  - 6
4099  - http://casereports.bmj.com/content/12/6/e230026.short
4100  - http://casereports.bmj.com/content/12/6/e230026.full
SO  - BMJ Case Reports2019 Jun 01; 12
AB  - A 72-year-old woman was admitted to the hospital because of dorsal, lumbar and lower abdomen pain that had started 4 days before. She had a history of age-related macular degeneration (treated with intraocular bevacizumab). Blood tests showed anaemia, thrombocytopaenia, acute kidney injury, elevated liver enzymes and total bilirubin (mainly because of the indirect fraction). Viral serologies and ADAMTS13 activity levels were normal, and stool testing was negative for Escherichia coli-producing Shiga toxins. E. coli was isolated in urine. Atypical haemolytic uremic syndrome triggered by a urinary tract infection or by the vascular endothelial growth factor-inhibitor bevacizumab were the most likely hypothesis. The patient started urgent plasmapheresis and dialysis that lasted for a total of 18 days. There was complete remission and recovery of kidney function allowing for treatment discontinuation, and she was discharged home. After 6 months of follow-up, she shows no signs of relapse.