Estradiol congeners/etanercept
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Atypical haemolytic uraemic syndrome (first report) treated with eculizumab: case report A 50-year-old woman with rheumatoid arthritis developed atypical haemolytic uraemic syndrome (HUS) while receiving etanercept and an estrogen patch [duration of treatments to reaction onset not stated]. She was successfully treated with eculizumab. The woman presented with malaise, abdominal pain and bloody diarrhoea while receiving twice weekly etanercept [route not stated; dosage details incomplete] and an estrogen patch [dosage and indication not stated]. Treatment with ceftriaxone, ciprofloxacin and dexamethasone was initiated for colitis, but she was transferred to an ICU with pancolitis, acute renal failure, sepsis and thrombocytopenia 3 days after presentation. Post-transfer, she was maintained on broad-spectrum antimicrobials and methylprednisolone. Laboratory investigations revealed the following: haemoglobin 12.7 g/dL, WBC count 31.4 × 109/L, creatinine 3.8 mg/dL, platelet count 28 × 109/L, AST 118 U/L, LDH 1199 U/L, reticulocyte count 2.7 %, haptoglobin < 6 mg/dL, C3 35 mg/dL and C4 6 mg/dL. Bibasilar opacities and small bilateral pulmonary effusions were evident on chest x-ray. By hospital day 5, occasional schistocytes were noted on peripheral smear. The woman underwent total abdominal colectomy and end ileostomy on the day of transfer; fresh-frozen plasma, pooled platelets and packed RBCs were administered during the procedure. After surgery, her platelet count increased without further infusions until day 5. Pathology findings were consistent with microangiopathy. Her mental and respiratory status deteriorated; she became unresponsive to voice and painful stimuli on hospital day 3, and developed seizure activity on day 4. An EEG was suggestive of diffuse cerebral dysfunction. On day 4, continuous veno-venous haemodialysis was initiated for worsening renal function. Atypical HUS was suspected, and IV eculizumab 900mg was begun on day 6, followed by four weekly doses and subsequent maintenance therapy (scheduled at 1200mg every 2 weeks); she also underwent plasma exchange, and received meningococcal vaccine and ciprofloxacin. Her LDH, C3 and C4 levels started to improve 4 days after her first eculizumab dose, and she opened her eyes and started localising to painful stimuli 3 days later. She was able to nod, follow commands and interact with her family 2 days after her second eculizumab dose. Dialysis frequency was reduced the next day, and she was weaned from the ventilator after another day. Her memory and cognition had recovered 3 days after the third eculizumab dose, and her urine output had increased to 875mL. Dialysis was stopped after the fourth dose, and LDH, haptoglobin, C3 and C4 levels had normalised after the sixth dose. Findings from an MRI taken 26 days after her first eculizumab dose were suggestive of extensive thrombotic microangiopathic brain injury. Maintenance therapy was associated with nausea, and her seventh dose was delayed by 1 day; treatment was then continued at 600mg weekly in additi
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