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Post-Partum Hemorrhage (PPH) as Implicated by Osler-Weber-Rendu Syndrome

Obstetrical management of patients with genetic disorders bears its own challenges in the overall care and outcomes of the patient. Hereditary hemorrhagic telangiectasia (HHT), or Osler-Weber-Rendu Syndrome, is an inherited genetic disorder that, due to the nature of the pathophysiology of arteriove...

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Autores principales: Powers, Patrick, Combs, Shanna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10495189/
https://www.ncbi.nlm.nih.gov/pubmed/37700945
http://dx.doi.org/10.7759/cureus.43388
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author Powers, Patrick
Combs, Shanna
author_facet Powers, Patrick
Combs, Shanna
author_sort Powers, Patrick
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description Obstetrical management of patients with genetic disorders bears its own challenges in the overall care and outcomes of the patient. Hereditary hemorrhagic telangiectasia (HHT), or Osler-Weber-Rendu Syndrome, is an inherited genetic disorder that, due to the nature of the pathophysiology of arteriovenous malformation and dysplasia, has been highly associated with an increased tendency of bleeding. This case presents a patient diagnosed with HHT prior to pregnancy who developed severe postpartum hemorrhage (PPH) after cesarean section delivery. Clinical considerations were made proactively with the knowledge and understanding of the genetic disorder, but due to unforeseen changes in the manifestation of the PPH, clinical decisions were dynamically modified in order to save the patient from exsanguination, ultimately resulting in an emergency hysterectomy and a total of 15 units of transfused packed red blood cells (pRBC). As a result, this case report serves as a preliminary recount for future research and clinical management of similar cases. 
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spelling pubmed-104951892023-09-12 Post-Partum Hemorrhage (PPH) as Implicated by Osler-Weber-Rendu Syndrome Powers, Patrick Combs, Shanna Cureus Medical Education Obstetrical management of patients with genetic disorders bears its own challenges in the overall care and outcomes of the patient. Hereditary hemorrhagic telangiectasia (HHT), or Osler-Weber-Rendu Syndrome, is an inherited genetic disorder that, due to the nature of the pathophysiology of arteriovenous malformation and dysplasia, has been highly associated with an increased tendency of bleeding. This case presents a patient diagnosed with HHT prior to pregnancy who developed severe postpartum hemorrhage (PPH) after cesarean section delivery. Clinical considerations were made proactively with the knowledge and understanding of the genetic disorder, but due to unforeseen changes in the manifestation of the PPH, clinical decisions were dynamically modified in order to save the patient from exsanguination, ultimately resulting in an emergency hysterectomy and a total of 15 units of transfused packed red blood cells (pRBC). As a result, this case report serves as a preliminary recount for future research and clinical management of similar cases.  Cureus 2023-08-12 /pmc/articles/PMC10495189/ /pubmed/37700945 http://dx.doi.org/10.7759/cureus.43388 Text en Copyright © 2023, Powers et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Medical Education
Powers, Patrick
Combs, Shanna
Post-Partum Hemorrhage (PPH) as Implicated by Osler-Weber-Rendu Syndrome
title Post-Partum Hemorrhage (PPH) as Implicated by Osler-Weber-Rendu Syndrome
title_full Post-Partum Hemorrhage (PPH) as Implicated by Osler-Weber-Rendu Syndrome
title_fullStr Post-Partum Hemorrhage (PPH) as Implicated by Osler-Weber-Rendu Syndrome
title_full_unstemmed Post-Partum Hemorrhage (PPH) as Implicated by Osler-Weber-Rendu Syndrome
title_short Post-Partum Hemorrhage (PPH) as Implicated by Osler-Weber-Rendu Syndrome
title_sort post-partum hemorrhage (pph) as implicated by osler-weber-rendu syndrome
topic Medical Education
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10495189/
https://www.ncbi.nlm.nih.gov/pubmed/37700945
http://dx.doi.org/10.7759/cureus.43388
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