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- W2783239571 abstract "Abstract Background: Severe congenital protein C deficiency (SCPCD) is a rare inherited disorder characterized by recurrent thrombosis, disseminated intravascular coagulation, and purpura fulminans. Due to limitations in traditional therapeutic options (anticoagulation, FFP, PCC), plasma-derived protein C concentrates have been used for both prophylactic and therapeutic indications. However, there are few reports of protein C infusion therapy in adults, with limited understanding of pharmacokinetics and no dosing guidelines available. We report the use of protein C concentrate in an individualized dose-finding study, in an adult patient in preparation for renal transplantation. Case: A 35 year old female (68 kg) with SCPCD (baseline PC activity Results: The patient was admitted to hospital for this dose-finding study. She was administered Ceprotin® (human plasma-derived, virally-inactivated protein C concentrate) with an initial intravenous bolus (59 IU/kg) followed by an infusion (5 IU/kg/hr) to maintain a target PC level of 0.65-1.30 U/mL (see Table). Protein C activity was measured every 6 hours, and target levels were achieved over the 30-hour duration of the trial. The patient experienced no adverse events during the infusion trial. Conclusion: In this case report, target protein C levels were maintained using bolus and continuous infusion rates of protein C concentrate that were approximately half of those recommended by the manufacturer. This personalized trial, while resource-intensive, provided a feasible and safe method of preparing for a high-risk surgical procedure that could be adapted to other similar clinical situations. Download : Download high-res image (89KB) Download : Download full-size image Table . Disclosures Crowther: Leo Pharma: Research Funding; Shinogi: Consultancy; Boehringer Ingelheim: Speakers Bureau; Bayer: Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Alexion: Speakers Bureau; Pfizer: Honoraria; Portola: Consultancy." @default.
- W2783239571 created "2018-01-26" @default.
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- W2783239571 date "2017-12-07" @default.
- W2783239571 modified "2023-09-28" @default.
- W2783239571 title "Protein C Infusion Therapy in an Adult Case of Severe Congenital Protein C Deficiency" @default.
- W2783239571 doi "https://doi.org/10.1182/blood.v130.suppl_1.4916.4916" @default.
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