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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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
SAGE Publications
2023
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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. |
format | Online Article Text |
id | pubmed-10101157 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
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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