Characterization of Surgical Procedures in Patients with Mucopolysaccharidosis Type I: Findings from the MPS I Registry

Pamela Arn, J. Edmond Wraith, Lisa Underhill

    Research output: Contribution to journalArticlepeer-review

    Abstract

    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
    Original languageEnglish
    Pages (from-to)859-e3
    JournalJournal Of Pediatrics
    Volume154
    Issue number6
    DOIs
    Publication statusPublished - Jun 2009

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