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RecruitingNCT05588167Updated Aug 31, 2026

Establishment of Genomic and Phenotypic Database for Niemann-Pick Disease, Type C

An observational study in Niemann-Pick Disease, Type C, sponsored by Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD). Recruiting at 1 site in United States. Open to participants aged 3 Months and older. Per ClinicalTrials.gov, last updated 2026-08-31.

Sponsored by Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) · Observational

Study type
Observational
Model
Cohort
Time perspective
Retrospective
Enrollment
100
Ages
3 Months and older
Sex
All
01

Study summary

Background:

Niemann-Pick type C (NPC) disease is a rare, progressive neurodegenerative disease that affects mainly the brain, liver, and spleen but also other parts of the body. There is no cure for NPC, and symptoms only get worse over time. Symptoms can include seizures, difficulty moving or talking, or dementia. But symptoms can vary among different people with the disease. Some may have seizures, while others do not, for example. Some people begin showing symptoms in childhood; in others, symptoms may not appear until they are adults. Researchers want to learn more about why NPC affects people differently. This natural history study will gather data from people with NPC in order to understand more about the disease and how it affects the body.

Objective:

This study will create the first and largest database about NPC.

Eligibility:

People of any age who have NPC.

Design:

Participants will have blood drawn from a vein. This will happen only once. The blood will be used to analyze the participants DNA.

The participants medical records will be reviewed. The study team will collect data on participants NPC diagnosis and symptoms; they will record how long participants have had each symptom. The study team will also collect data on each participants age, sex, race, height, weight, medications, and other test results.

The study team will communicate with participants. They will discuss the study and answer any questions.

Participants will receive up to $190.

Read the detailed description

Study Description:

The primary objective of this protocol is to investigate the phenotypic heterogeneity of NPC by using clinical and genomic data, and to establish a comprehensive database to facilitate future investigations.

Objectives:

  1. Identify correlations between NPC clinical phenotypic findings and genomic markers to facilitate the understanding of the heterogeneity of this disease.
  2. Identify genetic contributions to NPC disease progression that can be utilized as potential therapeutic targets.
  3. Establish the first and largest database of genomic and phenotypic information for NPC to benefit the NPC research and patient community.
02

Conditions studied

  • Niemann-Pick Disease, Type C

Keywords

  • Genetic
  • Clinical
  • DNA
  • Medical
  • History
  • Natural History
03

Who can participate

Ages eligible
3 Months and older
Sexes eligible
All
Accepts healthy volunteers
No
Sampling method
Non-probability sample

Study population

Individuals with Niemann-Pick Disease, type C

Inclusion criteria

In order to be eligible to participate in this study, an individual must meet all of the following criteria:

  1. Provision of signed and dated informed consent form
  2. Stated willingness to comply with all study procedures and availability for the duration of the study
  3. Male or female, any age, demographic or ethnic background will be eligible for this study
  4. Diagnosis of NPC will be based on clinical, biochemical or molecular testing.

Exclusion criteria

EXCLUSION CRITERIA:

An individual who meets any of the following criteria will be excluded from participation in this study:

  1. Unwilling to provide consent
  2. Unable to provide biospecimen to obtain DNA
  3. Unable to provide medical records or clinical data
04

Study design

Observational model
Cohort
Time perspective
Retrospective
Enrollment
100 participants (estimated)

Groups and cohorts

  • Affected

    Patients with Niemann-Pick Disease, type C

05

What researchers measure

Primary outcomes

  1. Clinical data, genomic markers

    Identify correlations between NPC clinical phenotypic findings and genomic markers to facilitate the understanding of the heterogeneity of this disease.

    Time frame: 2 years

  2. Whole genome

    Identify genetic contributions to NPC disease progression that can be utilized as potential therapeutic targets.

    Time frame: 2 years

06

Study locations

1 of 1 sites recruiting
  • National Institutes of Health Clinical Center
    Bethesda, Maryland 20892, United States
    • For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR) · Contact · ccopr@nih.gov · 800-411-1222
    Recruiting
07

References and documents

08

Registry details

Key details

Study ID
NCT05588167
Lead sponsor
Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
Responsible party
Sponsor
First posted
Oct 20, 2022
Start date
Nov 28, 2022
Primary completion
Sep 30, 2026 (estimated)
Completion
Sep 30, 2026 (estimated)
Last update
Aug 31, 2026

Study contacts

Desiree A Labor, C.R.N.P.
Contact
desiree.labor@nih.gov
(240) 678-7868
Forbes D Porter, M.D.
Contact
fdporter@mail.nih.gov
(301) 435-4432
Forbes D Porter, M.D.
principal investigator · Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)

Oversight

FDA-regulated drug
No
FDA-regulated device
No
View the source record on ClinicalTrials.gov ↗

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