Actively Recruiting

All Genders
NCT06669949

Natural History of Sphingosine Phosphate Lyase Insufficiency Syndrome (SPLIS)

Led by University of California, San Francisco · Updated on 2025-05-13

28

Participants Needed

1

Research Sites

297 weeks

Total Duration

On this page

Sponsors

U

University of California, San Francisco

Lead Sponsor

E

Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)

Collaborating Sponsor

AI-Summary

What this Trial Is About

This is a prospective longitudinal natural history study with a retrospective cross-sectional arm aimed at determining the natural history of sphingosine phosphate lyase insufficiency syndrome (SPLIS), a recently recognized inborn error of metabolism. The central hypothesis is that age of onset, other disease features, and disease biomarkers will be predictive of quality of life (QOL) and survival in SPLIS patients.

CONDITIONS

Official Title

Natural History of Sphingosine Phosphate Lyase Insufficiency Syndrome (SPLIS)

Who Can Participate

All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Diagnosed with SPLIS by genetic testing showing biallelic pathogenic or likely pathogenic variants in SGPL1 gene
  • Or have nucleotide changes in both SGPL1 alleles with at least one SPLIS feature or biochemical evidence of SPL protein loss of function
  • Or are a sibling of an SPLIS patient with genetic changes in both alleles and at least one SPLIS feature
  • Living or deceased patients with SPLIS
  • Parents of SPLIS patients may be included as controls
  • Provide informed consent or assent if living; PI ensures legal compliance for deceased participants
Not Eligible

You will not qualify if you...

  • Currently using or have used investigational agents in the last 30 days, except off-label medication use

AI-Screening

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Trial Site Locations

Total: 1 location

1

University of California San Francisco

San Francisco, California, United States, 94143

Actively Recruiting

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Research Team

J

Julie D Saba, MD, PhD

CONTACT

How is the study designed?

Study Type

OBSERVATIONAL

Masking

N/A

Allocation

N/A

Model

N/A

Primary Purpose

N/A

Number of Arms

3

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Natural History of Sphingosine Phosphate Lyase Insufficiency Syndrome (SPLIS) | DecenTrialz