RESUMO
Loeys-Dietz syndrome is an autosomal dominant genetic disorder which is associated with significant and often crucial vascular manifestations. This review is aimed to examine current evidence on pathophysiology and management of Loeys-Dietz syndrome in current era. A comprehensive electronic search was done to identify the articles that discussed all the aspects of Loeys-Dietz syndrome, combined key words and Medical Subject Headings (MeSH) terms were used. Relevant articles have been summarized in each relevant section. Loeys-Dietz syndrome is an autosomal dominant genetic disorder which has combined and multi-systemic manifestations. The increased breakdown of extracellular matrix predisposes an individual to developing aneurysms in the aortic tree which is undoubtedly the most significant complication of this disorder. Understanding the pathophysiology and natural history of Loeys-Dietz syndrome and regular surveillance is important to plan prophylactic interventions to prevent life-threatening aortic emergencies which can be fatal. Loeys-Dietz syndrome is an aggressive genetic condition that predisposes an individual to the development of life-threatening aortic aneurysms. Our understanding of Loeys-Dietz syndrome remains ever-changing and it is likely that the knowledge regarding its diagnosis and treatment will become more clearly defined in the coming years with deeper genetic studies.
Assuntos
Aorta/cirurgia , Implante de Prótese Vascular , Procedimentos Endovasculares , Síndrome de Loeys-Dietz/cirurgia , Remodelação Vascular , Adulto , Idoso , Aorta/patologia , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/mortalidade , Dilatação Patológica , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/mortalidade , Feminino , Predisposição Genética para Doença , Humanos , Síndrome de Loeys-Dietz/genética , Síndrome de Loeys-Dietz/mortalidade , Síndrome de Loeys-Dietz/patologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Fatores de Risco , Resultado do TratamentoRESUMO
Arising from the city of Wuhan, Hubei province in China, a novel coronavirus named severe acute respiratory syndrome coronavirus 2 has been rapidly spreading since its first presentation in late 2019. The World Health Organization declared a pandemic on the 11th March 2020, and as of 29th of April 2020 more than 3 million cases have been reported worldwide with over 225 000 confirmed deaths. Where mechanical ventilation may not be enough, extracorporeal membrane oxygenation (ECMO) could play a role as a form of rescue therapy and may provide beneficial results in the hands of skilled clinicians in centers with experience of using ECMO appropriately in selected patients. Our understanding of COVID-19 is ever-changing and the need for intensive care beds is rising, which means that ECMO will surely play a key role in the near future.
