Plasma
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Plasma Lack of response: case report
A 39-year-old woman exhibited a lack of response during treatment with plasma for pregnancy associated atypical haemolytic uremic syndrome (p-aHUS). The woman underwent in vitro fertilisation resulting in twin pregnancy. The pregnancy was unremarkable until gestational week 32, when she was referred to the unit of obstetrics and gynecology for hypertension. Initially, gestational hypertension was suspected, and she was treated with methyldopa [α-methyldopa] 250mg twice daily. After 4 days, she again presented to the hospital due to strong epigastrium pain. Based on investigations, she was diagnosed with haemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. Hence, a caesarean section was performed, and she delivered the healthy dizygotic twins weighing 2.190Kgs and 2.050Kgs. After the procedure, she was scheduled for a subtotal hysterectomy due to severe bleeding. Therefore, she was shifted to the ICU. Persistent high BP was noted with anaemia. On postpartum day 1 and 2, she received 4 units of infusion of plasma [frozen fresh plasma]. On postpartum day 5, nephrology was consulted due to marked fluctuations in laboratory findings. Despite treatment, a lack of response to plasma infusion was noted. The low C3 level and no effect to plasma infusion suggested paHUS. Hence, her treatment was started with eculizumab, and subsequent imprvment was noted in her condition. Her eculizumab treatment was discontinued after 1 year. During follow up examination one year after cessation of treatment, her kidney function was noted as normal without proteinuria or haematuria. Also, genetic analysis was performed by direct sequencing which showed a novel heterozygous G-to-A mutation, and H3 risk haplotype for aHUS on the CFH gene-3 and gene-2 genetic variants on the MG3 region of C3 and the CFI gene. Santoro D, et al. Identification of a New Complement Factor H Mutation in a Patient With Pregnancy-Related Acute Kidney Injury. Kidney International Reports 5: 1603-1607, No. 9, Sep 2020. Available from: URL: http://doi.org/10.1016/j.ekir.2020.06.028
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Reactions 3 Oct 2020 No. 1824
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