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NCT05848271Recruiting

Natural History Study of Patients with HPDL Mutations

University of California, San Diego

Start Date

5/18/2023

Completion Date

12/31/2027

Summary

This study uses medical records that allow retrospective data extraction of clinical manifestation to assess the natural history of HPDL mutations

Detailed Description

A novel mitochondrial disease arises from mutations in HPDL, which codes for 4-hydroxyphenylpyruvate dioxygenase-like protein. The main purpose of this study is to establish a patient registry to gather medical data from consenting HPDL mutation patients worldwide. From longitudinal data, we will be able to figure out the natural history of the disease, and genotype-phenotype correlation. Dry blood spots will be collected to develop biomarkers to understand the disease better.

Eligibility Criteria

Age Range: No minimum to No maximum

Inclusion Criteria: * Any individuals diagnosed with HPDL variants * Clinical diagnosis can include: * HPDL-related hereditary spastic paraplegia (HSP) * HPDL-related neonatal mitochondrial encephalopathy * Spastic paraplegia -83 (SPG83) * Neurodevelopmental disorder with progressive spasticity and brain white matter abnormalities (NEDSWMA) Exclusion Criteria: * Any known genetic abnormality (other than HPDL mutation) * Any condition that, in the opinion of the Site Investigator, could put the participant at undue risk and/or would ultimately prevent the completion of study procedures

Interventions

OTHER

Patient Registry

OTHER

Dry blood spots sampling

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Conditions

Mitochondrial EncephalomyopathiesHereditary Spastic ParaplegiaSpastic ParaplegiaWhite Matter DiseaseNeonatal EncephalopathyMutationGenetic Disease

Locations

Eun Hae Lee

San Diego, California 92093

United States