Current clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)

van Haalen F.M.; Kaya M.; Pelsma I.C.M.; Cannegieter S.C.; Huisman M.V.; van Vlijmen B.J.M.; Feelders R.A.; Stochholm K.; Fliers E.; Castinetti F.; Brue T.; Bertherat J.; Scaroni C.; Colao A.; Giordano R.; Druce M.R.; Beckers A.; Spranger J.; Driessens N.; Maiter D.; Feldt-Rasmussen U.; Feelders R.; Webb S.M.; Dattani M.; Husebye E.; Zilaitiene B.; Gaztambide S.; Gatto F.; Ferone D.; Persani L.; Chiodini I.; Höybye C.; Pereira A.M.; Biermasz N.R.; Klok F.A.; Dekkers O.M.; Meijer O.C.; Reincke M.; Vila G.; Perry C.; Heck A.; Stancampiano M.R.; van de Ven A.; Johannsson G.; Ragnarsson O.; Tóth M.; Volke V.; Toumba M.; Canu L.; Vojtková J.; Al-Mrayat M.; Fassnacht M.; Detomas M.; Karavitaki N.; van der Klauw M.M.; Groselj U.; Elenkova A.; Unuane D.

Current clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)

Background: Cushing’s syndrome (CS) is associated with an hypercoagulable state and an increased risk of venous thromboembolism (VTE). Evidence-based guidelines on thromboprophylaxis strategies in patients with CS are currently lacking. We aimed to map the current clinical practice for thromboprophy...

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Authors:	van Haalen F.M.; Kaya M.; Pelsma I.C.M.; Cannegieter S.C.; Huisman M.V.; van Vlijmen B.J.M.; Feelders R.A.; Stochholm K.; Fliers E.; Castinetti F.; Brue T.; Bertherat J.; Scaroni C.; Colao A.; Giordano R.; Druce M.R.; Beckers A.; Spranger J.; Driessens N.; Maiter D.; Feldt-Rasmussen U.; Feelders R.; Webb S.M.; Dattani M.; Husebye E.; Zilaitiene B.; Gaztambide S.; Gatto F.; Ferone D.; Persani L.; Chiodini I.; Höybye C.; Pereira A.M.; Biermasz N.R.; Klok F.A.; Dekkers O.M.; Meijer O.C.; Reincke M.; Vila G.; Perry C.; Heck A.; Stancampiano M.R.; van de Ven A.; Johannsson G.; Ragnarsson O.; Tóth M.; Volke V.; Toumba M.; Canu L.; Vojtková J.; Al-Mrayat M.; Fassnacht M.; Detomas M.; Karavitaki N.; van der Klauw M.M.; Groselj U.; Elenkova A.; Unuane D.
Format:	Article
Status:	Published version
Publication date:	2022
Country:	España
Institution:	Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
Repository:	r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
OAI Identifier:	oai:iibsantpau.fundanetsuite.com:p15745
Online Access:	https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=15745 https://www.scopus.com/inward/record.uri?eid=2-s2.0-85129997291&doi=10.1186%2fs13023-022-02320-x&partnerID=40&md5=1bce48e3562061040f7701f9182966ce
Access Level:	Open access
Keyword:	antithrombin III apixaban dabigatran edoxaban low molecular weight heparin rivaroxaban anticoagulant agent activated partial thromboplastin time adrenalectomy Article clinical practice Cushing syndrome deep vein thrombosis geographic distribution haplotype human hypercoagulability hypercortisolism hyperglycemia hypertension immobilization medical history postoperative care prothrombin time questionnaire risk assessment thrombophilia thrombosis prevention transsphenoidal surgery treatment duration venous thromboembolism complication endocrine disease endometriosis female rare disease Anticoagulants Cushing Syndrome Endocrine System Diseases Endometriosis Female Humans Rare Diseases Venous Thromboembolism

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Summary:	Background: Cushing’s syndrome (CS) is associated with an hypercoagulable state and an increased risk of venous thromboembolism (VTE). Evidence-based guidelines on thromboprophylaxis strategies in patients with CS are currently lacking. We aimed to map the current clinical practice for thromboprophylaxis management in patients with CS across reference centers (RCs) of the European Reference Network on Rare Endocrine Conditions (Endo-ERN), which are endorsed specifically for the diagnosis and treatment of CS. Using the EU survey tool, a primary screening survey, and subsequently a secondary, more in-depth survey were developed. Results: The majority of the RCs provided thromboprophylaxis to patients with CS (n = 23/25), although only one center had a standardized thromboprophylaxis protocol (n = 1/23). RCs most frequently started thromboprophylaxis from CS diagnosis onwards (n = 11/23), and the majority stopped thromboprophylaxis based on individual patient characteristics, rather than standardized treatment duration (n = 15/23). Factors influencing the initiation of thromboprophylaxis were ‘medical history of VTE’ (n = 15/23) and ‘severity of hypercortisolism’ (n = 15/23). Low-Molecular-Weight-Heparin was selected as the first-choice anticoagulant drug for thromboprophylaxis by all RCs (n = 23/23). Postoperatively, the majority of RCs reported ‘severe immobilization’ as an indication to start thromboprophylaxis in patients with CS (n = 15/25). Most RCs (n = 19/25) did not provide standardized testing for variables of hemostasis in the postoperative care of CS. Furthermore, the majority of the RCs provided preoperative medical treatment to patients with CS (n = 23/25). About half of these RCs (n = 12/23) took a previous VTE into account when starting preoperative medical treatment, and about two-thirds (n = 15/23) included ‘reduction of VTE risk’ as a goal of treatment. Conclusions: There is a large practice variation regarding thromboprophylaxis management and perioperative medical treatment in patients with CS, even in Endo-ERN RCs. Randomized controlled trials are needed to establish the optimal prophylactic anticoagulant regimen, carefully balancing the increased risk of (perioperative) bleeding, and the presence of additional risk factors for thrombosis. © 2022, The Author(s).

Current clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)

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