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In Brief Purpose: Recent advancements in medical treatment of Hurler syndrome have resulted in longer life expectancies and a greater need for therapeutic services. The purpose of this case series is to provide recommendations for assessing children with Hurler syndrome after umbilical cord blood transplant (UCBT). Clinical descriptions: Two children with Hurler syndrome were seen for longitudinal assessments following an UCBT for Hurler syndrome. Methods: The raw scores and percentage of fine and gross motor items each child completed on the Motor Scale of the Bayley Scales of Infant Development II (BSID-II) were reviewed. Results: Both children gained new motor skills with each successive motor assessment. Both children were able to complete a higher percentage of fine motor skills than gross motor skills in the most advanced item set assessed. Discussion: The children presented in these two case reports both had better fine motor skills than gross motor skills, which inflated their standard scores on the BSID-II. Clinicians assessing children with Hurler syndrome should use standardized assessments that allow for differentiation of fine and gross motor skills to prevent this situation. Using two illustrative cases that had inflated motor scores on the Bayley II, the authors recommend that clinicians assessing children with Hurler syndrome should use standardized tests that allow for differentiation of fine and gross motor skills.
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Dusing et al. (2005) studied this question.
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