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AutorSolanki, Guirish A.
AutorAlden, Tord D.
AutorBurton, Barbara K.
AutorGiugliani, Roberto
AutorHorovitz, Dafne Dain Gandelman
AutorJones, Simon A.
AutorLampe, Christina
AutorMartin, Kenneth W.
AutorRyan, Maura E.
AutorSchaefer, Matthias K.
AutorSiddiqui, Aisha
AutorWhite, Klane K.
AutorHarmatz, Paul
Fecha de acceso2014-12-15T18:06:34Z
Fecha de disponibilización2014-12-15T18:06:34Z
Fecha de publicación2012
ReferenciaSOLANKI, Guirish A. et al. A multinational, multidisciplinary consensus for the diagnosis and management of spinal cord compression among patients with mucopolysaccharidosis VI. Molecular Genetics and Metabolism, Orlando, v. 107, p. 15-24, 2012.pt_BR
ISSN1096-7192pt_BR
URIhttps://www.arca.fiocruz.br/handle/icict/9173
Idiomaengpt_BR
EditorAcademic Presspt_BR
Derechos de autorrestricted access
TítuloA multinational, multidisciplinary consensus for the diagnosis and management of spinal cord compression among patients with mucopolysaccharidosis VIpt_BR
Tipo del documentoArticle
DOI10.1016/j.ymgme.2012.07.018
Resumen en InglésCervical cord compression is a sequela of mucopolysaccharidosis VI, a rare lysosomal storage disorder, and has devastating consequences. An international panel of orthopedic surgeons, neurosurgeons, anesthesiologists, neuroradiologists, metabolic pediatricians, and geneticists pooled their clinical expertise to codify recommenda-tions for diagnosing, monitoring, and managing cervical cord compression; for surgical intervention criteria; and for best airway management practices during imaging or anesthesia. The recommendations offer ideal best prac-tices but also attempt to recognize the worldwide spectrum of resource availability. Functional assessments and clinical neurological examinations remain the cornerstone for identification of early signs ofmyelopathy, butmagnetic resonance imaging is the gold standard for identificationof cervical cord com-pression. Difficult airways of MPS VI patients complicate the anesthetic and, thus, the surgical management of cervical cord compression. All patients with MPS VI require expert airway management during any surgical pro-cedure. Neurophysiological monitoring of the MPS VI patient during complex spine or head and neck surgery is considered standard practice but should also be considered for other procedures performed with the patient under general anesthesia, depending on the lengthand type of theprocedure. Surgical interventionsmay include cervical decompression, stabilization, or both. Specific techniques vary widely among surgeons. The onset, presentation, and rate of progression of cervical cord compression vary among patients with MPS VI. The availability of medical resources, the expertise and experience of members of the treatment team, and the standard treatment practices vary among centers of expertise. Referral to specialized, experienced MPS treatment centers should be considered for high-risk patients and those requiring complex procedures. Therefore, the key to optimal patient care is to implement best practices through meaningful communication among treatment team members at each center and among MPS VI specialists worldwide.pt_BR
AfiliaciónBirmingham Children's Hospital. Department of Paediatric Neurosurgery. Birmingham, NH, UK.pt_BR
AfiliaciónLurie Children's Hospital of Chicago. Lurie Children's Hospital of Chicago. Chicago, USA.pt_BR
AfiliaciónLurie Children's Hospital of Chicago. Division of Genetics. Chicago, USA.pt_BR
AfiliaciónInstituto Nacional de Genética Médica Populacional. Hospital de Clínicas de Porto Alegre. Serviço de Genética Médica. Porto Alegre, RS, Brasil.pt_BR
AfiliaciónInstituto Nacional de Genética Médica Populacional. Universidade Federal do Rio Grande do Sul. Departamento de Genética. Porto Alegre, RS, Brasil. / Instituto Nacional de Genética Médica Populacional. Instituto Nacional de Genética Médica Populacional. Porto Alegre, RS, Brasil.pt_BR
AfiliaciónFundação Oswaldo Cruz. Instituto de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira. Centro de Genética Médica. Rio de Janeiro, RJ, Brasil.pt_BR
AfiliaciónCentral Manchester University Hospitals. Manchester Academic Health Sciences Centre. St. Mary's Hospital. Genetic Medicine. Manchester, UK.pt_BR
AfiliaciónChildren's Hospital and Research Center Oakland. Department of Medical Imaging. Chicago, USA.pt_BR
AfiliaciónEmergency Medicine and Pain Therapy. Department of Anesthesiology. Intensive Care. Koblenz, Germany.pt_BR
AfiliaciónLurie Children's Hospital of Chicago. Division of Anesthesiology. Chicago, USA.pt_BR
AfiliaciónSeattle Children's Hospital. Department of Orthopedics and Sports Medicine. Seattle, USA.pt_BR
AfiliaciónChildren's Hospital & Research Center Oakland. Oakland, USA.pt_BR
Palavras clave en InglêsAirway Managementpt_BR
Palavras clave en InglêsCervical Cord Compressionpt_BR
Palavras clave en InglêsPractice Guidelinept_BR
Palavras clave en InglêsSpinal Cord Compressionpt_BR
Palavras clave en InglêsSurgical Decompressionpt_BR
DeCSCompressão da Medula Espinalpt_BR
DeCSDescompressão Cirúrgicapt_BR


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