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Anesthetic management of a pediatric patient with Rett syndrome - A case report -
Anesth Pain Med 2018;13(2):173-5
Published online April 30, 2018
© 2018 The Korean Society of Anesthesiologists.

A Ran Lee, Hyung Kwan Lee, Young Ung Kim, Jae Ho Lee, Ho Jun Kang, and Se Hun Park
Department of Anesthesiology and Pain Medicine, Ulsan University Hospital, Ulsan, Korea
Correspondence to: Se Hun Park, M.D., Ph.D.
Department of Anesthesiology and Pain Medicine, Ulsan University Hospital, 877 Bangeojinsunhwandoro, Dong-gu, Ulsan 44033, Korea
Tel: 82-52-250-7248
Fax: 82-52-250-7249
Received September 28, 2017; Revised November 1, 2017; Accepted November 1, 2017.
cc This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Rett syndrome is a neurodevelopmental disease that almost always affects female patients. It is caused by mutations in MeCP2 in the majority of cases. Patients diagnosed with Rett syndrome may experience serious adverse events even with smaller amounts of medication for sedation and anesthesia. The major anesthetic concerns associated with Rett syndrome are lack of cooperation, abnormal continuous limb movements, abnormal respiratory control, difficult positioning secondary to scoliosis, and altered sensitivity to painful stimuli. Because of the risks caused by these problems, anesthesiologists should be aware of the specific anesthetic concerns of patients with Rett syndrome in order to safely administer anesthesia. Here, we describe the management of a pediatric patient diagnosed with Rett syndrome.
Key Words : MeCP2 mutations, Postnatal neurological disorder, Rett syndrome.

April 2018, 13 (2)
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