Morquio综合征合并肢端肥大症患者的麻醉挑战。

IF 1.8 Q4 NEUROSCIENCES Annals of Neurosciences Pub Date : 2022-10-01 DOI:10.1177/09727531221137403
Nidhi Singh, Rashi Sarna, Manjul Tripathi, Rajeev Chauhan, Sanjay Kumar
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引用次数: 0

摘要

Morquio综合征是一种罕见的储存性疾病,与骨、软骨、心脏瓣膜和角膜中硫酸角蛋白和硫酸软骨素-6的过度沉积有关。虽然大多数患有这种综合症的人在出生时表现正常;骨骼异常通常发生在生命的第一年。呼吸受限、关节僵硬和心脏异常也很常见。这些患者的多系统参与带来了独特的麻醉挑战,并且缺乏关于这种情况下患者的麻醉管理的文献。我们报告一例罕见的34岁男性Morquio综合征患者,其表现为肢端肥大症,并计划在全身麻醉下手术切除肿瘤。这种罕见的疾病主要需要对疾病,其表现和管理策略有透彻的了解,以获得更好的结果。牢记多系统参与,适当的团队合作和各种专业的协调变得至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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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 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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来源期刊
Annals of Neurosciences
Annals of Neurosciences NEUROSCIENCES-
CiteScore
2.40
自引率
0.00%
发文量
39
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