title: High rate of postoperative mortality in patients with mucopolysaccharidosis I: findings from the MPS I Registry.
abstract: OBJECTIVE
  Mucopolysaccharidosis I (MPS I) is a rare lysosomal storage disorder caused by deficiency of α-L-iduronidase, which results in progressive multisystemic disease. Patients with MPS I often require multiple common and uncommon surgeries and are at risk for surgical and anesthetic complications because of respiratory and cardiac disease. Surgery often precedes diagnosis; thus, surgeons and anesthesiologists may be unaware of potential risks.
  
  
  METHODS
  We analyzed data from the MPS I Registry, a voluntary observational database, for deaths occurring within 1 month of a surgical procedure among the 932 patients enrolled as of July 2010.
  
  
  RESULTS
  Among the 196 deceased patients, 186 reported 1 surgery or more, and 32 had 1 surgery or more within 1 month of death, including 20 who had 1 surgery or more within 10 days of death. Surgeries before death included hernia repair, central line placement, spinal surgery, tracheostomy, and ventriculo-peritoneal shunt. Most patients (28/32) had severe MPS I (Hurler), and 20 of 32 patients (all Hurler) died at 3 years or younger. In 6 of 32 patients, surgery was directly noted in the cause of death, including 4 patients with an attenuated form of MPS I.
  
  
  CONCLUSIONS
  Patients with mucopolysaccharidosis have a high postoperative mortality because of underlying respiratory and cardiac diseases.
authors.affiliation: The Nemours Children's Clinic, Jacksonville, FL 32207, USA. parn@nemours.org
authors.forename: Pamela
authors.initials: P
authors.lastname: Arn
authors.forename: Chester
authors.initials: C
authors.lastname: Whitley
authors.forename: J Edmond
authors.initials: JE
authors.lastname: Wraith
authors.forename: H Warner
authors.initials: HW
authors.lastname: Webb
authors.forename: Lisa
authors.initials: L
authors.lastname: Underhill
authors.forename: Lakshmi
authors.initials: L
authors.lastname: Rangachari
authors.forename: Gerald F
authors.initials: GF
authors.lastname: Cox
mesh_terms: D000293:Adolescent; D000328:Adult; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007431:Intraoperative Complications / Q000401:mortality*; D008297:Male; D008059:Mucopolysaccharidosis I / Q000150:complications / Q000401:mortality* / Q000601:surgery; D011183:Postoperative Complications / Q000401:mortality*; D012042:Registries; D055815:Young Adult