Critical clinical situations in adult patients with Mucopolysaccharidoses (MPS)
Open Access
- 14 May 2020
- journal article
- research article
- Published by Springer Science and Business Media LLC in Orphanet Journal of Rare Diseases
- Vol. 15 (1), 1-12
- https://doi.org/10.1186/s13023-020-01382-z
Abstract
Background Mucopolysaccharidoses (MPS) are rare, inherited disorders associated with enzyme deficiencies that result in glycosaminoglycan (GAG) accumulation in multiple organ systems. Management of MPS is evolving as patients increasingly survive to adulthood and undergo multiple surgeries throughout their lives. As surgeries in these patients are considered to be high risk, this can result in a range of critical clinical situations in adult patients. Results We discuss strategies to prepare for and manage critical clinical situations in adult patients with MPS, including supporting the multidisciplinary team, preoperative and airway assessments, surgical preparations, and postoperative care. We also present eight critical clinical cases (age range: 21-38 years) from four leading inherited metabolic disease centres in Europe to highlight challenges and practical solutions to optimise the care of adult patients with MPS. Critical clinical situations included surgical procedures, pregnancy and a thrombus in a port-a-cath. Conclusions Individualised strategies to manage critical clinical situations need to be developed for each patient to compensate for the heterogeneous symptoms that may be present and the potential complications that may occur. These strategies should include input from the wider MDT, and be coordinated by metabolic specialists with expertise in the management of MPS disorders and surgery in adult patients with MPS.Other Versions
This publication has 46 references indexed in Scilit:
- Anaesthesia and airway management in mucopolysaccharidosisJournal of Inherited Metabolic Disease, 2012
- How safe is diathermy in patients with cochlear implants?The Annals of The Royal College of Surgeons of England, 2012
- Anesthetic care and perioperative complications of children with Morquio syndromePediatric Anesthesia, 2012
- Cognitive decline after anaesthesia and critical careContinuing Education in Anaesthesia Critical Care & Pain, 2012
- Overview of the mucopolysaccharidosesRheumatology, 2011
- Mortality and cause of death in mucopolysaccharidosis type II—a historical review based on data from the Hunter Outcome Survey (HOS)Journal of Inherited Metabolic Disease, 2009
- Cognitive Effects of Antiepileptic DrugsJournal of Clinical Neurology, 2008
- Management Guidelines for Mucopolysaccharidosis VIPEDIATRICS, 2007
- The Role of Postoperative Analgesia in Delirium and Cognitive Decline in Elderly Patients: A Systematic ReviewAnesthesia & Analgesia, 2006
- Management of mucopolysaccharidosis type III.Archives of Disease in Childhood, 1993