Haemolytic Uremic Syndrome – The Impact of Early and Targeted Intervention on the Course of Potentially Life-Threatening Disease

Eva Bormane, Georgijs Moisejevs, Dace Trumpika, Regina Baufale, Anda Grigane

Research output: Contribution to journalMeeting Abstractpeer-review

Abstract

30-year-old female was admitted with complaints about abdominal and back pain, nausea, and vomiting, bloody urine. These symptoms appeared 2 days prior hospitalization. On admission patient was suspicious to have renal colic due to nephrolithiasis. Patient history demonstrated that she had been hospitalized with similar complaints in UK four years ago – haemolytic uremic syndrome (HUS) was found and rapid improvement with plasma exchange and steroid therapy was achieved. Another episode was in her early childhood with spontaneous remission but there was lack of detailed information about this case. She had no relevant family history.
On admission her BP was 180/110 mm Hg, weight 75 kg, she had decreased renal function (creatinine 151 μmol/l; eGFR 37.18 ml/min MDRD), thrombocytopenia (22x103/μl), haemolysis (LDH 2324 U/l; haemolysis test 0.26 g/dl; ref. range <0.05 g/dl). US of abdomen had no relevant finding.
Taking into account her past medical history and these results, a diagnosis of HUS was established. CVC was placed in femoral vein and urgent therapeutic plasma exchange (TPE) procedure was performed already within the first 4 hours after the admission. TPE was continued daily for the first week. During each session 0.5 plasma volume was exchanged to six units of fresh frozen plasma and crystalloids. Due to continued decrease of renal function she also had 2 sessions of IHD. Overall, she received 20 TPE procedures (daily for the first week, then 3–4–5 times a week.) as well as red blood cell and platelet transfusions, pulse steroids, ACEI. The duration of her treatment was one month with significant improvement – LDH reached to reference range, platelet count, Hb value gradually increased, renal function improved.
Thanks to our Lithuanian colleagues, patient had an opportunity for detailed genetic testing of complement for possible mutations. These tests revealed that she has a combined genetic predisposition to aHUS (1 mutation, 3 frequent risk variations),
ADAMTS13 activity was 81% (on the background of TPE).
She is now in our close outpatient observation and is doing quite well already for one and a half year with stabile remission. She is on small dose of ACEI, her clinical condition is stable and all clinical analyses are in reference range, so there is no data of relapse until now.
The presentation aimed to show:
• Favourable outcome of a potentially life-threatening disease.
• The impact of very early diagnostics and intervention on the course of the disease.
Original languageEnglish
Article number13
Pages (from-to)165-166
JournalBlood Purification
Volume44
Publication statusPublished - Jun 2017
Externally publishedYes
Event35th Vicenza Course on AKI & CRRT
- Vicenza, Italy
Duration: 13 Jun 201716 Jun 2017

Field of Science

  • 3.2 Clinical medicine

Publication Type

  • 3.4. Other publications in conference proceedings (including local)

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