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一名儿童合并黏多糖贮积症 VI 型和先天性肾上腺皮质增生症：麻醉注意事项

Combined mucopolysaccharidosis type VI and congenital adrenal hyperplasia in a child: Anesthetic considerations.

作者信息

Bansal Abhishek, Das Jyotirmoy, Kumar Raj, Khanna Sangeeta, Sapra Harsh, Mehta Yatin

机构信息

Medanta Institute of Critical Care and Anaesthesiology, Medanta - The Medicity, Gurgaon, Haryana, India.

出版信息

J Anaesthesiol Clin Pharmacol. 2012 Jul;28(3):364-7. doi: 10.4103/0970-9185.98343.

DOI:10.4103/0970-9185.98343
PMID:22869947
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3409950/
Abstract

We present a child posted for magnetic resonance imaging of brain under general anesthesia with the rare combination of mucopolysachharidosis type VI and congenital adrenal hyperplasia. The presence of both these disorders has important anesthetic implications. The pathophysiology of this rare combination of disease is reviewed with emphasis on the anesthesia management.

摘要

我们报告一例在全身麻醉下准备进行脑部磁共振成像检查的儿童，其患有罕见的Ⅵ型黏多糖贮积症和先天性肾上腺皮质增生症。这两种疾病的并存具有重要的麻醉意义。本文回顾了这种罕见疾病组合的病理生理学，并重点讨论了麻醉管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a6c8/3409950/acb8ab955e43/JOACP-28-364-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a6c8/3409950/a52fbd5d142e/JOACP-28-364-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a6c8/3409950/26f2199ff2c7/JOACP-28-364-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a6c8/3409950/46d883df16fe/JOACP-28-364-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a6c8/3409950/acb8ab955e43/JOACP-28-364-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a6c8/3409950/a52fbd5d142e/JOACP-28-364-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a6c8/3409950/26f2199ff2c7/JOACP-28-364-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a6c8/3409950/46d883df16fe/JOACP-28-364-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a6c8/3409950/acb8ab955e43/JOACP-28-364-g004.jpg

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J Anaesthesiol Clin Pharmacol. 2012 Jul;28(3):364-7. doi: 10.4103/0970-9185.98343.
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引用本文的文献

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本文引用的文献

1
Anesthesia in a patient with mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome).六型黏多糖贮积症（马罗托克斯-拉米综合征）患者的麻醉。
J Anesth. 2010 Dec;24(6):945-8. doi: 10.1007/s00540-010-1029-8. Epub 2010 Oct 26.
2
Approach to the adult with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.21-羟化酶缺乏所致先天性肾上腺皮质增生症成人患者的诊治方法
J Clin Endocrinol Metab. 2008 Mar;93(3):653-60. doi: 10.1210/jc.2007-2417.
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Management guidelines for mucopolysaccharidosis VI.黏多糖贮积症VI型管理指南。
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Clinical and biochemical study of 28 patients with mucopolysaccharidosis type VI.28例VI型黏多糖贮积症患者的临床与生化研究。
Clin Genet. 2004 Sep;66(3):208-13. doi: 10.1111/j.1399-0004.2004.00277.x.
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[A NEW DYSOSTOSIS WITH URINARY ELIMINATION OF CHONDROITIN SULFATE B].[一种伴有硫酸软骨素B经尿液排泄的新型骨发育不全症]
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Congenital adrenal hyperplasia owing to 21-hydroxylase deficiency.由于21-羟化酶缺乏所致的先天性肾上腺皮质增生症。
Endocrinol Metab Clin North Am. 2001 Mar;30(1):31-59, vi. doi: 10.1016/s0889-8529(08)70018-5.
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Disordered breathing during sleep in patients with mucopolysaccharidoses.
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Study of three patients with congenital adrenal hyperplasia treated by bilateral adrenalectomy.双侧肾上腺切除术治疗三名先天性肾上腺增生症患者的研究。
World J Surg. 2000 Nov;24(11):1347-52. doi: 10.1007/s002680010223.
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Congenital adrenal hyperplasia.先天性肾上腺增生症
Pediatr Rev. 2000 May;21(5):159-70; quiz 171. doi: 10.1542/pir.21-5-159.
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The daily cortisol production reinvestigated in healthy men. The serum and urinary cortisol production rates are not significantly different.对健康男性的每日皮质醇分泌进行了重新研究。血清和尿皮质醇分泌率无显著差异。
J Clin Endocrinol Metab. 1998 Apr;83(4):1247-52. doi: 10.1210/jcem.83.4.4694.