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Phase 2 N=38 Treatment

CARNIVAL Type I: Valproic Acid and Carnitine in Infants With Spinal Muscular Atrophy (SMA) Type I

Spinal Muscular Atrophy Type I

Enrolled (actual)
38
Serious AEs
76.3%
Results posted
Jun 2015
Primary outcome: Primary: Laboratory Safety Data

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
Valproic Acid and Levocarnitine (Drug)
Age
Pediatric · 0+ yrs
Sex
All
Sponsor
University of Utah
Primary completion
May 2012

Outcome Measures

OutcomeResultp-value
PRIMARY
Laboratory Safety Data
PRIMARY
Anthropometric Measures of Nutritional Status (Body Mass Index [BMI] Z-scores, Weight for Length Ratios, Lean/Fat Mass Via DEXA, Growth Parameters, and Triceps Skinfold Measures)
4317.15; 4993.92; 5133.83; 3011.37; 3618.25; 4316.08
SECONDARY
Time to Death or Ventilator Dependence (Defined as >16 Hours/Day)
SECONDARY
Primary Caregiver Functional Rating Scale for SMA Type I Subjects (PCFRS)
SECONDARY
Functional Motor Assessments: TIMPSI Scores
SECONDARY
Quantitative SMN mRNA and Protein Measures
SECONDARY
Maximum Ulnar CMAP Amplitude/Area and MUNE
SECONDARY
Whole Body DEXA Scanning for Lean Body Mass and Total Bone Mineral Density/ Content

Summary

This is a multi-center trial to test safety and evaluate early treatment intervention with valproic acid and carnitine in moderating SMA symptoms of Type I infants.

Eligibility Criteria

Inclusion Criteria

  • Laboratory documentation of SMN mutation/deletion consistent with a genetic diagnosis of SMA
  • Clinical diagnosis of SMA type I
  • Age 2 weeks to 12 months
  • Written informed consent of parents/guardian

Exclusion Criteria

  • Any clinical or laboratory evidence of hepatic or pancreatic insufficiency.
  • Laboratory results drawn within 14 days prior to start of study drug demonstrating:

Liver transaminases (AST, ALT), lipase, amylase: > 1.5 x ULN White Blood Cell Count: < 3 Neutropenia: <1 Platelet: <100K Hematocrit: <30, persisting over a 30-day period

  • Serious illness requiring systemic treatment and/or hospitalization within two weeks prior to study entry.
  • Use of medications or supplements within 30 days of study enrollment that interfere with VPA or carnitine metabolism; that increase the potential risks of VPA or carnitine; or that are hypothesized to have a beneficial effect in SMA animal models or human neuromuscular disorders, including riluzole, valproic acid, hydroxyurea, oral use of albuterol, sodium phenylbutyrate, butyrate derivatives, creatinine, growth hormone, anabolic steroids, probenecid, oral or parenteral use of corticosteroids at entry, or agents anticipated to increase or decrease muscle strength or agents with presumed histone deacetylase (HDAC) inhibition.
  • Infants who have participated in a treatment trial for SMA within 30 days of study entry or who will become enrollees in any other treatment trial during the course of this study.
  • Unwillingness to travel for study assessments.
  • Coexisting medical conditions that contradict use of VPA/carnitine or travel to and from study site.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00661453). 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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