TY - JOUR
T1 - Characterization of Surgical Procedures in Patients with Mucopolysaccharidosis Type I: Findings from the MPS I Registry
AU - Arn, Pamela
AU - Wraith, J. Edmond
AU - Underhill, Lisa
PY - 2009/6
Y1 - 2009/6
N2 - Objective: To clarify the extent and chronology of surgical burden in relation to symptom onset and diagnosis in patients with mucopolysaccharidosis I (MPS I) as reported in the MPS I Registry, an international observational database. Study design: Analysis of surgical data from 544 patients enrolled in the MPS I Registry. Among all patients with at least 1 reported surgery, the number and frequency of procedures, and age at procedure, diagnosis, and symptom onset were collected overall, by patient, and by reported phenotype (Hurler, Hurler-Scheie, Scheie). Results: Overall and by phenotype, ∼75% of patients in the MPS I Registry reported at least 1 surgery. The most common were myringotomies and related procedures, hernia repair, adenoidectomy/tonsillectomy, and carpal-tunnel release. Median age at first surgery was
AB - Objective: To clarify the extent and chronology of surgical burden in relation to symptom onset and diagnosis in patients with mucopolysaccharidosis I (MPS I) as reported in the MPS I Registry, an international observational database. Study design: Analysis of surgical data from 544 patients enrolled in the MPS I Registry. Among all patients with at least 1 reported surgery, the number and frequency of procedures, and age at procedure, diagnosis, and symptom onset were collected overall, by patient, and by reported phenotype (Hurler, Hurler-Scheie, Scheie). Results: Overall and by phenotype, ∼75% of patients in the MPS I Registry reported at least 1 surgery. The most common were myringotomies and related procedures, hernia repair, adenoidectomy/tonsillectomy, and carpal-tunnel release. Median age at first surgery was
U2 - 10.1016/j.jpeds.2008.12.024
DO - 10.1016/j.jpeds.2008.12.024
M3 - Article
C2 - 19217123
SN - 0022-3476
VL - 154
SP - 859-e3
JO - Journal Of Pediatrics
JF - Journal Of Pediatrics
IS - 6
ER -