Trusted Resources: Education
Scientific literature and patient education texts
Disease Manifestations in Mucopolysaccharidoses and Their Impact on Anaesthesia-Related Complications-A Retrospective Analysis of 99 Patients
source: Journal of clinical medicine
year: 2021
authors: Ammer LS,Dohrmann T,Muschol NM,Lang A,Breyer SR,Ozga AK,Petzoldt M
summary/abstract:Patients with mucopolysaccharidoses (MPS) frequently require anaesthesia for diagnostic or surgical interventions and thereby experience high morbidity. This study aimed to develop a multivariable prediction model for anaesthesia-related complications in MPS. This two-centred study was performed by retrospective chart review of children and adults with MPS undergoing anaesthesia from 2002 until 2018. We retrieved the patients’ demographics, medical history, clinical manifestations, and indication by each anaesthesia. Multivariable mixed-effects logistic regression was calculated for a clinical model based on preoperative predictors preselected by lasso regression and another model based on disease subtypes only. Of the 484 anaesthesia cases in 99 patients, 22.7% experienced at least one adverse event. The clinical model resulted in a better forecast performance than the subtype-model (AICc 460.4 vs. 467.7). The most relevant predictors were hepatosplenomegaly (OR 3.10, CI 1.54-6.26), immobility (OR 3.80, CI 0.98-14.73), and planned major surgery (OR 6.64, CI 2.25-19.55), while disease-specific therapies, i.e., haematopoietic stem cell transplantation (OR 0.45, CI 0.20-1.03), produced a protective effect. Anaesthetic complications can best be predicted by surrogates for advanced disease stages and protective therapeutic factors. Further model validation in different cohorts is needed.
organization: Department of Paediatrics, International Centre for Lysosomal Disorders (ICLD), University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany.DOI: 10.3390/jcm10163518
read more
Related Content
-
Magnetic Resonance Imaging Findings of the Posterior Fossa in 47 Patients With Mucopolysaccharidoses: A Cross-Sectio...Mucopolysaccharidoses (MPS) is a group o...
-
Mucopolysaccharidosis Market: Global Industry Analysis and Opportunity Assessment 2022-2029The wide ranging Mucopolysaccharidosis M...
-
Spectrum of MPS 1 H/S Diseasehttps://www.onempsvoice.com/wp-content/u...
-
Airway Management of the Deformed Trachea Using T-Tube Stents in Patients With Mucopolysaccharidosis Type IVAIntroduction: Mucopolysaccharidosis (MPS...
-
Corneal Cloudiness: A Presenting Feature of Mucopolysaccharidosis Type IA 10-y-old ‘short-statured’ male pre...
-
MPS I-H: What Are The Symptoms?https://www.youtube.com/watch?v=h111oEHS...
-
Molecular Analysis of Vietnamese Patients With Mucopolysaccharidosis Type IMucopolysaccharidosis type I (MPS I) is ...