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Phase 2 N=29 Randomized Double-blind Treatment

A Pilot Therapeutic Trial Using Hydroxyurea in Type I Spinal Muscular Atrophy Patients

Muscular Atrophy, Spinal

Enrolled (actual)
29
Serious AEs
Results posted
Oct 2019
Primary outcome: Primary: Safety: Frequency of Adverse Events/Lab Abnormalities

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
Hydroxyurea (Drug); Placebo to match hydroxyurea (Drug)
Age
Pediatric
Sex
All
Sponsor
Stanford University
Primary completion
Feb 2012

Outcome Measures

OutcomeResultp-value
PRIMARY
Safety: Frequency of Adverse Events/Lab Abnormalities
PRIMARY
Efficacy: Length of Survival (LOS) and Age of Ventilator Dependence (AVD)
SECONDARY
Motor Unit Number Estimation (MUNE)
SECONDARY
Biomarker Assays: SMN Protein and SMN mRNA

Summary

The objectives of this trial are: to establish a safety profile for use of Hydroxyurea in children with Type I Spinal Muscular Atrophy; to identify reliable outcome measures for HU treatment in Type I SMA; and to detect the clinical efficacy of HU treatment in children with Type I SMA.

Eligibility Criteria

Inclusion Criteria:1. Laboratory confirmation of a homozygous deletion or mutation of the SMN1 gene 2. Clinical Diagnosis of Type I SMA (never achieved independent sitting) 3. Onset of disease before the age of 6 months 4. Enrollment in study within 6 months of diagnosis Exclusion Criteria:1. Known hematological disorders, such as chronic anemia (defined as platelet count less than 100,000/mm^3) in two contiguous measures in two weeks 2. Severe systemic disorders such as congenital heart disease, other major birth defects involving internal organs, or severe birth asphyxia 3. Participation in SMA clinical trials for other experimental drugs 4. Requiring continuous respiratory support before the initiation of HU treatment
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00568698). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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