Anesthesiological risks in mucopolysaccharidoses

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Annotation Summary

Term Occurence Count Dictionary
Sanfilippo syndrome 5 endocrinologydiseases
Sly syndrome 2 endocrinologydiseases
mucopolysaccharidosis 10 endocrinologydiseases

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Sanfilippo syndrome 2896 syndrome (MPS IH), Hurler-Scheie syndrome (MPS IH/S), Scheie syndrome (MPS IS), Hunter syndrome (MPS II), Sanfilippo syndrome (MPS IIIA, B, C, D), Morquio syndrome (MPS IVA, B), Maroteaux-Lamy syndrome (MPS VI), Sly syndrome (MPS
Sanfilippo syndrome 4051 may be absent in mild formIduronate sulfate sulfataseHeparan sulfateDermatan sulfateElapraseMPS IIIA Sanfilippo syndrome ADevelopmental delay, severe hyperactivity, behavioral problems. Somatic manifestations are generally
Sanfilippo syndrome 4258 are generally less severe than other MPSHeparan-S-sulfaminidaseHeparan sulfateNot availableMPS IIIB Sanfilippo syndrome BSymptoms and disease progression are less severe than IIIAN-acetyl-α-d-glucosaminidaseMPS IIICSanfilippo
Sanfilippo syndrome 4374 syndrome BSymptoms and disease progression are less severe than IIIAN-acetyl-α-d-glucosaminidaseMPS IIIC Sanfilippo syndrome CAcetyl-Co-A glucosaminidaseMPS IIIDSanfilippo syndrome DN-AcetylglucosidaseN-acyltransferaseMPS IVAMorquio
Sanfilippo syndrome 4430 than IIIAN-acetyl-α-d-glucosaminidaseMPS IIICSanfilippo syndrome CAcetyl-Co-A glucosaminidaseMPS IIID Sanfilippo syndrome DN-AcetylglucosidaseN-acyltransferaseMPS IVAMorquio syndrome ASevere skeletal dysplasia usually leading
Sly syndrome 3002 Sanfilippo syndrome (MPS IIIA, B, C, D), Morquio syndrome (MPS IVA, B), Maroteaux-Lamy syndrome (MPS VI), Sly syndrome (MPS VII), and MPS IX. The etiology, treatment options, and common symptoms are detailed in Table 1.Table
Sly syndrome 5118 incompetenceN-acetyl-galactosamine α-4-sulfate sulfataseDermatan sulfateGalsulfase, NaglazymeMPS VII Sly syndrome Highly variable developmental delay and progressive intellectual disability may be presentβ-glucuronidaseDermatan
mucopolysaccharidosis 303 (pmc-release): 11/2018Publication date (collection): /2018AbstractBackgroundPatients suffering from mucopolysaccharidosis are among the most complex from the anesthesiological point of view, especially regarding the management
mucopolysaccharidosis 617 reports and small case series.AimsTo identify useful information about experience with each subtype of mucopolysaccharidosis reported in the literature and propose a guide on the best options for airway management to the anesthesiologists
mucopolysaccharidosis 860 take care of these patients.MethodsA query of the PubMed database specific for “anesthesia” and “ mucopolysaccharidosis ” and a further query specific for “mucopolysaccharidosis and difficult airway management” was
mucopolysaccharidosis 921 database specific for “anesthesia” and “mucopolysaccharidosis” and a further query specific for “ mucopolysaccharidosis and difficult airway management” was conducted. We looked for those items that offered practical guidance
mucopolysaccharidosis 1580 and the need for an experienced team are emphasized in all the reviewed articles and for each type of mucopolysaccharidosis . Many suggestions on how to plan the perioperative period have been highlighted. Insertion of a laryngeal
mucopolysaccharidosis 1951 very useful in making intubation easier and facilitating bronchoscope passage.ConclusionsPatients with mucopolysaccharidosis are at high risk for anesthesia-related complications and require a high level of attention. However,
mucopolysaccharidosis 6053 of GAG accumulation as reported by Arn et al. [[2], [4]].Table 2Most common surgical procedures in mucopolysaccharidosis type I patients. They are also common in other types of MPS [[2], [4]]• Myringotomies and related
mucopolysaccharidosis 8186 LM and ST conducted a query of the PubMed database, specifically concerning “anesthesia” and “ mucopolysaccharidosis ” and a further query specifically concerning “mucopolysaccharidosis and difficult airway management.”
mucopolysaccharidosis 8258 concerning “anesthesia” and “mucopolysaccharidosis” and a further query specifically concerning “ mucopolysaccharidosis and difficult airway management.” Together, all the authors selected articles containing practical
mucopolysaccharidosis 28566 devices available, would be useful.Key messagesWhat is already known about the topic: anesthesia for mucopolysaccharidosis patients is associated with high morbidityWhat new information is added by this review: we suggest a

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