The Importance of Early Pericardiectomy in Mulibrey Nanism Syndrome, a Case Report

Mulibrey (Muscle-Liver-Brain-Eye) Nanism syndrome is an extremely rare genetic disorder with multiorgan involvement. Constrictive pericarditis and diastolic dysfunction are the most common causes of mortality. We present a case of a patient with Mulibrey nanism syndrome who underwent pericardiectomy...

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Detalles Bibliográficos
Autores principales: Cordova Sanchez, Andres, Vasigh, Mostafa, Carhart, Robert
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2022
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8908392/
https://www.ncbi.nlm.nih.gov/pubmed/35257621
http://dx.doi.org/10.1177/23247096221077816
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author Cordova Sanchez, Andres
Vasigh, Mostafa
Carhart, Robert
author_facet Cordova Sanchez, Andres
Vasigh, Mostafa
Carhart, Robert
author_sort Cordova Sanchez, Andres
collection PubMed
description Mulibrey (Muscle-Liver-Brain-Eye) Nanism syndrome is an extremely rare genetic disorder with multiorgan involvement. Constrictive pericarditis and diastolic dysfunction are the most common causes of mortality. We present a case of a patient with Mulibrey nanism syndrome who underwent pericardiectomy at 12 years old and was able to live 44 years more with relatively stable and asymptomatic diastolic congestive heart failure (CHF). This case highlights the importance of early recognition and treatment of constrictive pericarditis in these patients.
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spelling pubmed-89083922022-03-11 The Importance of Early Pericardiectomy in Mulibrey Nanism Syndrome, a Case Report Cordova Sanchez, Andres Vasigh, Mostafa Carhart, Robert J Investig Med High Impact Case Rep Case Report Mulibrey (Muscle-Liver-Brain-Eye) Nanism syndrome is an extremely rare genetic disorder with multiorgan involvement. Constrictive pericarditis and diastolic dysfunction are the most common causes of mortality. We present a case of a patient with Mulibrey nanism syndrome who underwent pericardiectomy at 12 years old and was able to live 44 years more with relatively stable and asymptomatic diastolic congestive heart failure (CHF). This case highlights the importance of early recognition and treatment of constrictive pericarditis in these patients. SAGE Publications 2022-03-08 /pmc/articles/PMC8908392/ /pubmed/35257621 http://dx.doi.org/10.1177/23247096221077816 Text en © 2022 American Federation for Medical Research https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Cordova Sanchez, Andres
Vasigh, Mostafa
Carhart, Robert
The Importance of Early Pericardiectomy in Mulibrey Nanism Syndrome, a Case Report
title The Importance of Early Pericardiectomy in Mulibrey Nanism Syndrome, a Case Report
title_full The Importance of Early Pericardiectomy in Mulibrey Nanism Syndrome, a Case Report
title_fullStr The Importance of Early Pericardiectomy in Mulibrey Nanism Syndrome, a Case Report
title_full_unstemmed The Importance of Early Pericardiectomy in Mulibrey Nanism Syndrome, a Case Report
title_short The Importance of Early Pericardiectomy in Mulibrey Nanism Syndrome, a Case Report
title_sort importance of early pericardiectomy in mulibrey nanism syndrome, a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8908392/
https://www.ncbi.nlm.nih.gov/pubmed/35257621
http://dx.doi.org/10.1177/23247096221077816
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