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Anesthetic Challenges in a Patient of Morquio Syndrome Associated with Acromegaly

Morquio syndrome is one of the rare storage disorders associated with excessive deposition of keratin sulfate and chondroitin-6-sulfate in bones, cartilages, heart valves, and cornea. Although most individuals with this syndrome appear normal at birth; skeletal abnormalities often develop within the...

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Autores principales: Singh, Nidhi, Sarna, Rashi, Tripathi, Manjul, Chauhan, Rajeev, Kumar, Sanjay
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10101157/
https://www.ncbi.nlm.nih.gov/pubmed/37064287
http://dx.doi.org/10.1177/09727531221137403
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author Singh, Nidhi
Sarna, Rashi
Tripathi, Manjul
Chauhan, Rajeev
Kumar, Sanjay
author_facet Singh, Nidhi
Sarna, Rashi
Tripathi, Manjul
Chauhan, Rajeev
Kumar, Sanjay
author_sort Singh, Nidhi
collection PubMed
description Morquio syndrome is one of the rare storage disorders associated with excessive deposition of keratin sulfate and chondroitin-6-sulfate in bones, cartilages, heart valves, and cornea. Although most individuals with this syndrome appear normal at birth; skeletal abnormalities often develop within the first year of life. Restricted breathing, joint stiffness, and cardiac abnormalities are also common. The multisystem involvement in these patients poses unique anesthestic challenges and there is a paucity of literature regarding the anesthetic management of patients with this condition. We report a successful management of a rare case of 34 years old male with Morquio syndrome who presented an association of acromegaly and was planned for surgical resection of the tumor under general anesthesia. Such rare disorders primarily require thorough knowledge about the disease, its presentation and management strategies for a better outcome. Keeping in mind the multisystem involvement a proper teamwork and coordination of various specialities becomes an utmost importance.
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spelling pubmed-101011572023-04-14 Anesthetic Challenges in a Patient of Morquio Syndrome Associated with Acromegaly Singh, Nidhi Sarna, Rashi Tripathi, Manjul Chauhan, Rajeev Kumar, Sanjay Ann Neurosci Case Reports Morquio syndrome is one of the rare storage disorders associated with excessive deposition of keratin sulfate and chondroitin-6-sulfate in bones, cartilages, heart valves, and cornea. Although most individuals with this syndrome appear normal at birth; skeletal abnormalities often develop within the first year of life. Restricted breathing, joint stiffness, and cardiac abnormalities are also common. The multisystem involvement in these patients poses unique anesthestic challenges and there is a paucity of literature regarding the anesthetic management of patients with this condition. We report a successful management of a rare case of 34 years old male with Morquio syndrome who presented an association of acromegaly and was planned for surgical resection of the tumor under general anesthesia. Such rare disorders primarily require thorough knowledge about the disease, its presentation and management strategies for a better outcome. Keeping in mind the multisystem involvement a proper teamwork and coordination of various specialities becomes an utmost importance. SAGE Publications 2023-01-31 2022-10 /pmc/articles/PMC10101157/ /pubmed/37064287 http://dx.doi.org/10.1177/09727531221137403 Text en © The Author(s) 2023 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 page (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Reports
Singh, Nidhi
Sarna, Rashi
Tripathi, Manjul
Chauhan, Rajeev
Kumar, Sanjay
Anesthetic Challenges in a Patient of Morquio Syndrome Associated with Acromegaly
title Anesthetic Challenges in a Patient of Morquio Syndrome Associated with Acromegaly
title_full Anesthetic Challenges in a Patient of Morquio Syndrome Associated with Acromegaly
title_fullStr Anesthetic Challenges in a Patient of Morquio Syndrome Associated with Acromegaly
title_full_unstemmed Anesthetic Challenges in a Patient of Morquio Syndrome Associated with Acromegaly
title_short Anesthetic Challenges in a Patient of Morquio Syndrome Associated with Acromegaly
title_sort anesthetic challenges in a patient of morquio syndrome associated with acromegaly
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10101157/
https://www.ncbi.nlm.nih.gov/pubmed/37064287
http://dx.doi.org/10.1177/09727531221137403
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