Critical clinical situations in adult patients with Mucopolysaccharidoses (MPS).

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 le

SUBMITTER: Stepien KM 

PROVIDER: S-EPMC7227065 | biostudies-literature | 2020 May

REPOSITORIES: biostudies-literature