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Mucopolysaccharidosis Type I (MPS I)

Anesthesia Implications

Clinically reviewed by anesthesia providers · Updated

Difficult AirwayC-spineVideo LaryngoscopeCopious SecretionsReactive AirwayPulmonary HypertensionIncreased ICPArrhythmiasCareful PositioningObstructive Sleep ApneaRestrictive Lung DiseaseValvular Heart Disease

Pathophysiology

Mucopolysaccharidosis type I (MPS I) is an autosomal recessive lysosomal storage disease caused by deficiency of alpha-L-iduronidase, the enzyme that degrades the glycosaminoglycans (GAGs) dermatan...

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