Simulation of a perioperative (ENT) complication in a Hunter patient, presented by Dr Florian Lagler

Dr Florian Lagler MD PhD is Chair of the Research Institute for Inborn Errors of Metabolism at Paracelsus Medical University, CEO of the Clinical Research Centre and Director of the Medical Simulation Centre, Salzburg. In his clinic, he sees patients with Fabry disease, Gaucher disease, Pompe disease, mucopolysaccharidoses (MPSs) and other lysosomal storage diseases (LSDs). Dr Lagler has designed many clinical trials including pivotal studies of drug development programmes and has published more than 60 scientific papers.

In this episode of the MPS Webinar Series, Dr Lagler, presents a simulation of a perioperative complication in a Hunter Syndrome patient. During this webinar, the audience made clinical decisions in a simulation setting, deciding the outcome of the case; this has been omitted from this recording.

The mucopolysaccharidosis (MPS) diseases are a group of rare, genetic, metabolic disorders. All MPS diseases are inherited in a recessive pattern, meaning that some people might be carriers of the disease but are unaffected. MPS II is different from the other MPS diseases because its inheritance is X-linked, meaning that males are almost exclusively affected.1

People with MPS do not have enough, or any, of an enzyme that is needed to break down a sugar, called a mucopolysaccharide or glycosaminoglycan (GAG). As a result, GAGs build up throughout the body and can cause damage to cells.1

There are seven types of MPS disease that each lack different enzymes required to break down GAGs. All of the MPS disorders are progressive, meaning they worsen with time. They are also all heterogeneous, meaning that they can affect different people differently, often resulting in a spectrum of clinical severity from mildly to severely affected.1

For more information on MPS II (also known as Hunter Syndrome), please visit http://www.huntersyndrome.info/

The information found on this channel is not exhaustive, and is not intended to diagnose or advise in the treatment of any illness or disease. This information should not be used in place of advice from your general practitioner or other healthcare professional. If in doubt, please contact your doctor for advice.

For more information about upcoming webinars, please visit:
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C-ANPROM/INT//6946 January 2020

References:
1. Scarpa M et al. Mucopolysaccharidosis type II: European recommendations for the diagnosis and multidisciplinary management of a rare disease. Orphanet J Rare Dis 2011; 6: 72.