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Inicio Revista Española de Anestesiología y Reanimación (English Edition) Perioperative management of a female diagnosed with heterozygous X-linked adreno...
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Vol. 66. Issue 2.
Pages 113-116 (February 2019)
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Vol. 66. Issue 2.
Pages 113-116 (February 2019)
Case report
DOI: 10.1016/j.redare.2018.08.013
Perioperative management of a female diagnosed with heterozygous X-linked adrenoleukodystrophy for shoulder arthroscopy
Manejo perioperatorio de una mujer portadora del gen de la adrenoleucodistrofia ligada al X para artroscopia de hombro
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S. Zhang Wang
Corresponding author
serena.zhang.wang@gmail.com

Corresponding author.
, M. Álvarez Fernández, P. Mellado Miras, N. Martínez Merino, E. Herrera López, L.E. Muñoz Alameda
Servicio de Anestesiología y Reanimación, Hospital Universitario Fundación Jiménez Díaz-Quirón Salud, Madrid, Spain
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Abstract

X-linked adrenoleukodystrophy (X-ALD) belongs to a family of rare diseases due to inborn errors of metabolism. It has a wide spectrum of clinical manifestations that anaesthesiologists should recognise during the perioperative period (respiratory centre dysfunction, hypotonia, adrenal or hepatic failure, gastroesophageal reflux disease, osteopenia, seizures). The case is presented of a 42-year-old X-linked adrenoleukodystrophy female carrier, who underwent combined general and an ultrasound-guided interscalene brachial plexus block anaesthesia for shoulder arthroscopy. Induction was performed with thiopentone and fentanyl, and sevoflurane was used as inhaled maintenance agent. No events were recorded during the procedure. Her post-operative recovery was satisfactory and she was later discharged home.

Keywords:
Adrenoleukodystrophy
Anaesthesia
Long chain fatty acids
Carriers
Resumen

La adrenoleucodistrofia ligada al cromosoma X (ALD-X) pertenece al grupo de enfermedades raras debidas a errores congénitos del metabolismo. Clínicamente, presenta diferentes manifestaciones que el anestesiólogo debe tener en cuenta durante el perioperatorio (disfunción respiratoria, hipotonía, insuficiencia suprarrenal o hepática, reflujo gastroesofágico, osteopenia, crisis epilépticas). Se presenta el caso de una mujer de 42 años portadora del gen que fue sometida a anestesia general combinada para artroscopia de hombro. Se hizo bloqueo ecoguiado del plexo braquial a nivel interescalénico, inducción anestésica con tiopental y fentanilo, y mantenimiento con sevofluorano. El procedimiento transcurrió sin incidencias, la evolución postoperatoria fue favorable y la paciente fue dada de alta a domicilio.

Palabras clave:
Adrenoleucodistrofia
Anestesia
Ácidos grasos de cadena larga
Portador

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