Thromboprophylaxis in congenital nephrotic syndrome: 15-year experience from a national cohort
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ORIGINAL ARTICLE
Thromboprophylaxis in congenital nephrotic syndrome: 15-year experience from a national cohort Laurence J. Dobbie 1
&
Angela Lamb 2 & Lucy Eskell 2 & Ian J. Ramage 1,2 & Ben C. Reynolds 1,2
Received: 16 April 2020 / Revised: 2 September 2020 / Accepted: 19 September 2020 # The Author(s) 2020
Abstract Introduction Congenital nephrotic syndrome (CNS) is an ultra-rare disease associated with a pro-thrombotic state and venous thromboembolisms (VTE). There is very limited evidence evaluating thromboprophylaxis in patients with CNS. This study aimed to determine the doses and duration of treatment required to achieve adequate thromboprophylaxis in patients with CNS. Methods From 2005 to 2018 children in Scotland with a confirmed genetic or histological diagnosis of CNS were included if commenced on thromboprophylaxis. The primary study endpoint was stable drug monitoring. Secondary outcomes included VTE or significant haemorrhage. Results Eight patients were included; all initially were commenced on low-molecular weight heparin (enoxaparin). Four patients maintained therapeutic anti-Factor Xa levels (time 3–26 weeks, dose 3.2–5.07 mg/kg/day), and one patient developed a thrombosis (Anti-Factor Xa: 0.27 IU/ml). Four patients were subsequently treated with warfarin. Two patients maintained therapeutic INRs (time 6–11 weeks, dose 0.22–0.25 mg/kg/day), and one patient had two bleeding events (Bleed 1: INR 6, Bleed 2: INR 5.5). Conclusions Achieving thromboprophylaxis in CNS is challenging. Similar numbers of patients achieved stable anticoagulation on warfarin and enoxaparin. Enoxaparin dosing was nearly double the recommended starting doses for secondary thromboprophylaxis. Bleeding events were all associated with supra-therapeutic anticoagulation. Keywords Infantile nephrotic . Warfarin . Low molecular weight heparin . Venous thromboembolism . Anticoagulation
Abbreviations BNFc British National Formulary for Children CNS Congenital Nephrotic Syndrome CVVH Continuous veno-venous hemofiltration eGFR Estimated glomerular filtration rate INR International Normalised Ratio LMWH Low molecular weight heparin SVC Superior vena cava VTE Venous Thromboembolism UPCR Urinary protein:creatinine ratio Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00467-020-04793-z) contains supplementary material, which is available to authorized users. * Ben C. Reynolds [email protected] 1
University of Glasgow, Glasgow, UK
2
Department of Paediatric Nephrology, Royal Hospital for Children, 1345 Govan Road, Glasgow G51 4TF, UK
Introduction Congenital nephrotic syndrome (CNS) is a rare disease characterised by heavy proteinuria and severe oedema developing within 3 months of birth [1, 2]. Glomerular filtration barrier proteins are defective due to genetic mutations or more rarely secondary to congenital viral infection. Complications arising from severe proteinuria include venous thromboembolism (VTE), recurrent infection, fluid and electrolyte disturbance, and impair
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