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Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report

INTRODUCTION: Bardet-Biedl syndrome, which compromises airway management and the cardiovascular and renal systems, is a rare ciliopathic syndrome characterized by multisystem involvement and varying genetic etiologies and clinical manifestations. PATIENT CONCERNS: A 13-year-old female patient had a...

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Autores principales: Yaman, Ferda, Çekmen, Nedim
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7505336/
https://www.ncbi.nlm.nih.gov/pubmed/32957390
http://dx.doi.org/10.1097/MD.0000000000022300
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author Yaman, Ferda
Çekmen, Nedim
author_facet Yaman, Ferda
Çekmen, Nedim
author_sort Yaman, Ferda
collection PubMed
description INTRODUCTION: Bardet-Biedl syndrome, which compromises airway management and the cardiovascular and renal systems, is a rare ciliopathic syndrome characterized by multisystem involvement and varying genetic etiologies and clinical manifestations. PATIENT CONCERNS: A 13-year-old female patient had a history of chronic renal failure, hypothyroidism, mental retardation, hypogonadotropic hypogonadism, obesity, and retinitis pigmentosa and was undergoing 4-hour hemodialysis 3 days a week. DIAGNOSIS: We diagnosed Bardet-Biedl syndrome based on the results of genetic tests. INTERVENTIONS: We performed renal transplantation under general anesthesia while considering the perioperative risks of airway obstruction and hypothermia. OUTCOMES: Multidisciplinary preoperative evaluation is crucial to avoid perioperative complications. The risk of an obstructed airway should be considered. Hypothyroidism is a rare consequence of Bardet-Biedl syndrome. Rocuronium and sugammadex are safe for anesthetic management during renal transplantation to address Bardet-Biedl syndrome. CONCLUSION: Safe anesthetic management can be achieved with the rigorous preoperative assessment of perioperative complications.
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spelling pubmed-75053362020-09-24 Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report Yaman, Ferda Çekmen, Nedim Medicine (Baltimore) 3300 INTRODUCTION: Bardet-Biedl syndrome, which compromises airway management and the cardiovascular and renal systems, is a rare ciliopathic syndrome characterized by multisystem involvement and varying genetic etiologies and clinical manifestations. PATIENT CONCERNS: A 13-year-old female patient had a history of chronic renal failure, hypothyroidism, mental retardation, hypogonadotropic hypogonadism, obesity, and retinitis pigmentosa and was undergoing 4-hour hemodialysis 3 days a week. DIAGNOSIS: We diagnosed Bardet-Biedl syndrome based on the results of genetic tests. INTERVENTIONS: We performed renal transplantation under general anesthesia while considering the perioperative risks of airway obstruction and hypothermia. OUTCOMES: Multidisciplinary preoperative evaluation is crucial to avoid perioperative complications. The risk of an obstructed airway should be considered. Hypothyroidism is a rare consequence of Bardet-Biedl syndrome. Rocuronium and sugammadex are safe for anesthetic management during renal transplantation to address Bardet-Biedl syndrome. CONCLUSION: Safe anesthetic management can be achieved with the rigorous preoperative assessment of perioperative complications. Lippincott Williams & Wilkins 2020-09-18 /pmc/articles/PMC7505336/ /pubmed/32957390 http://dx.doi.org/10.1097/MD.0000000000022300 Text en Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 (https://creativecommons.org/licenses/by/4.0/)
spellingShingle 3300
Yaman, Ferda
Çekmen, Nedim
Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report
title Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report
title_full Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report
title_fullStr Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report
title_full_unstemmed Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report
title_short Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report
title_sort anesthetic management of a patient with bardet-biedl syndrome undergoing renal transplantation: a case report
topic 3300
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7505336/
https://www.ncbi.nlm.nih.gov/pubmed/32957390
http://dx.doi.org/10.1097/MD.0000000000022300
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