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Thromboembolic Disease in Haemophilic Patients Undergoing Major Orthopaedic Surgery: Is Thromboprophylaxis Mandatory?

Haemophilia is a rare genetic disorder, that results from various degrees of deficiency of coagulation factor VIII (haemophilia A), or factor IX (haemophilia B), with an X-linked transmission. The patients affected are in the majority of cases males (who inherit the affected X-chromosome from the ma...

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Autores principales: Badulescu, Oana Viola, Bararu Bojan, Iris, Badescu, Minerva Codruta, Filip, Nina, Chelsău, Alina, Ciocoiu, Manuela, Vladeanu, Maria, Filip, Alexandru, Forna, Norin, Sirbu, Mihnea Theodor, Ungureanu, Carmen, Sîrbu, Paul-Dan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9818461/
https://www.ncbi.nlm.nih.gov/pubmed/36611305
http://dx.doi.org/10.3390/diagnostics13010013
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author Badulescu, Oana Viola
Bararu Bojan, Iris
Badescu, Minerva Codruta
Filip, Nina
Chelsău, Alina
Ciocoiu, Manuela
Vladeanu, Maria
Filip, Alexandru
Forna, Norin
Sirbu, Mihnea Theodor
Ungureanu, Carmen
Sîrbu, Paul-Dan
author_facet Badulescu, Oana Viola
Bararu Bojan, Iris
Badescu, Minerva Codruta
Filip, Nina
Chelsău, Alina
Ciocoiu, Manuela
Vladeanu, Maria
Filip, Alexandru
Forna, Norin
Sirbu, Mihnea Theodor
Ungureanu, Carmen
Sîrbu, Paul-Dan
author_sort Badulescu, Oana Viola
collection PubMed
description Haemophilia is a rare genetic disorder, that results from various degrees of deficiency of coagulation factor VIII (haemophilia A), or factor IX (haemophilia B), with an X-linked transmission. The patients affected are in the majority of cases males (who inherit the affected X-chromosome from the maternal side), with rare cases of females with haemophilia (FVIII or FIX < 40 IU/dL), situations in which both X-chromosomes are affected, or one is affected, and the other one is inactive (known as carrier). The hypocoagulable state due to the deficiency of clotting factors, manifests as an excessive, recurrent tendency to bleeding, which positively correlates with plasmatic levels. Severe haemophilia results in hemarthrosis, although recent data have shown that moderate or even mild disease can lead to joint bleeding. Recurrent episodes of haemorrhages, usually affecting large joints such as knees, elbows, or ankles, lead to joint remodelling and subsequent haemophilic arthropathy, which may require arthroplasty as a last therapeutic option. Orthopaedic patients have the highest risk among all for deep vein thrombosis (DVT) and venous thromboembolism (VTE) with morbid and potentially fatal consequences. While for the rest of the population thromboprophylaxis in orthopaedic surgery is efficient, relatively safe, and widely used, for patients with haemophilia who are considered to have a low thromboembolic risk, there is great controversy. The great heterogeneity of this particular population, and the lack of clinical trials, with only case reports or observational studies, makes thromboprophylaxis in major orthopaedic surgery a tool to be used by every clinician based on experience and case particularities. This review aims to briefly summarise the latest clinical data and to offer an insight into the current recommendations that readers would find useful in daily practice.
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spelling pubmed-98184612023-01-07 Thromboembolic Disease in Haemophilic Patients Undergoing Major Orthopaedic Surgery: Is Thromboprophylaxis Mandatory? Badulescu, Oana Viola Bararu Bojan, Iris Badescu, Minerva Codruta Filip, Nina Chelsău, Alina Ciocoiu, Manuela Vladeanu, Maria Filip, Alexandru Forna, Norin Sirbu, Mihnea Theodor Ungureanu, Carmen Sîrbu, Paul-Dan Diagnostics (Basel) Review Haemophilia is a rare genetic disorder, that results from various degrees of deficiency of coagulation factor VIII (haemophilia A), or factor IX (haemophilia B), with an X-linked transmission. The patients affected are in the majority of cases males (who inherit the affected X-chromosome from the maternal side), with rare cases of females with haemophilia (FVIII or FIX < 40 IU/dL), situations in which both X-chromosomes are affected, or one is affected, and the other one is inactive (known as carrier). The hypocoagulable state due to the deficiency of clotting factors, manifests as an excessive, recurrent tendency to bleeding, which positively correlates with plasmatic levels. Severe haemophilia results in hemarthrosis, although recent data have shown that moderate or even mild disease can lead to joint bleeding. Recurrent episodes of haemorrhages, usually affecting large joints such as knees, elbows, or ankles, lead to joint remodelling and subsequent haemophilic arthropathy, which may require arthroplasty as a last therapeutic option. Orthopaedic patients have the highest risk among all for deep vein thrombosis (DVT) and venous thromboembolism (VTE) with morbid and potentially fatal consequences. While for the rest of the population thromboprophylaxis in orthopaedic surgery is efficient, relatively safe, and widely used, for patients with haemophilia who are considered to have a low thromboembolic risk, there is great controversy. The great heterogeneity of this particular population, and the lack of clinical trials, with only case reports or observational studies, makes thromboprophylaxis in major orthopaedic surgery a tool to be used by every clinician based on experience and case particularities. This review aims to briefly summarise the latest clinical data and to offer an insight into the current recommendations that readers would find useful in daily practice. MDPI 2022-12-21 /pmc/articles/PMC9818461/ /pubmed/36611305 http://dx.doi.org/10.3390/diagnostics13010013 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Badulescu, Oana Viola
Bararu Bojan, Iris
Badescu, Minerva Codruta
Filip, Nina
Chelsău, Alina
Ciocoiu, Manuela
Vladeanu, Maria
Filip, Alexandru
Forna, Norin
Sirbu, Mihnea Theodor
Ungureanu, Carmen
Sîrbu, Paul-Dan
Thromboembolic Disease in Haemophilic Patients Undergoing Major Orthopaedic Surgery: Is Thromboprophylaxis Mandatory?
title Thromboembolic Disease in Haemophilic Patients Undergoing Major Orthopaedic Surgery: Is Thromboprophylaxis Mandatory?
title_full Thromboembolic Disease in Haemophilic Patients Undergoing Major Orthopaedic Surgery: Is Thromboprophylaxis Mandatory?
title_fullStr Thromboembolic Disease in Haemophilic Patients Undergoing Major Orthopaedic Surgery: Is Thromboprophylaxis Mandatory?
title_full_unstemmed Thromboembolic Disease in Haemophilic Patients Undergoing Major Orthopaedic Surgery: Is Thromboprophylaxis Mandatory?
title_short Thromboembolic Disease in Haemophilic Patients Undergoing Major Orthopaedic Surgery: Is Thromboprophylaxis Mandatory?
title_sort thromboembolic disease in haemophilic patients undergoing major orthopaedic surgery: is thromboprophylaxis mandatory?
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9818461/
https://www.ncbi.nlm.nih.gov/pubmed/36611305
http://dx.doi.org/10.3390/diagnostics13010013
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