NCT02125721

Brief Summary

The purpose of this study is to evaluate the effect of escalating doses of cystine biding thiol drugs, including tiopronin and d-penicillamine, on the urinary cystine capacity, which is a measure of the amount of cystine in the urine, in patients with cystinuria. The overall goal will be to help guide therapy and ultimately minimize unnecessary side effects caused by larger doses.

Trial Health

87
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
10

participants targeted

Target at below P25 for phase_4

Timeline
Completed

Started Jun 2012

Longer than P75 for phase_4

Geographic Reach
1 country

1 active site

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

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Study Timeline

Key milestones and dates

Study Start

First participant enrolled

June 1, 2012

Completed
1.9 years until next milestone

First Submitted

Initial submission to the registry

April 24, 2014

Completed
5 days until next milestone

First Posted

Study publicly available on registry

April 29, 2014

Completed
3.6 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

December 1, 2017

Completed
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

December 1, 2017

Completed
3.7 years until next milestone

Results Posted

Study results publicly available

August 23, 2021

Completed
Last Updated

August 23, 2021

Status Verified

July 1, 2021

Enrollment Period

5.5 years

First QC Date

April 24, 2014

Results QC Date

July 17, 2019

Last Update Submit

July 28, 2021

Conditions

Keywords

cystinuriaD-penicillamine (Cuprimine®)tiopronin (Thiola®)

Outcome Measures

Primary Outcomes (1)

  • Cystine Capacity

    We will test for a negative correlation between progressive increases in doses of thiol drugs and cystine capacity

    4 weeks on assigned therapy

Study Arms (1)

CBTD Patients

EXPERIMENTAL

Part 1: patients will stop taking CBTDs for seven days and perform a 24-hour urine collection on day 7 Part 2: patients will take their usual CBTD, either tiopronin or d-penicillamine, 1g per day for 7 days, taken as 500 mg twice a day Part 3: patients will take a total of 2g of tiopronin or D-penicillamine daily for 7 days Part 4: patients will take a total of 3g/d of tiopronin or D-penicillamine, also for a 7 day period

Drug: Tiopronin 1g per dayDrug: Tiopronin 2g per dayDrug: Tiopronin 3g per day

Interventions

500mg PO BID x 7 days

Also known as: Thiola or tiopronin; d-penicillamine
CBTD Patients

1g PO BID x 7 days

Also known as: Thiola or tiopronin; d-penicillamine
CBTD Patients

1.5g PO BID x 7 days

Also known as: Thiola or tiopronin; d-penicillamine
CBTD Patients

Eligibility Criteria

Age18 Years - 80 Years
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)

You may qualify if:

  • Be a patient with a confirmed diagnosis of cystinuria.
  • Be already taking a CBTD (either D-penicillamine (Cuprimine®) or tiopronin (Thiola®) as part of your medication regimen.
  • Be between 18 and 80 years of age
  • Be enrolled in the Cystinuria Registry.

You may not qualify if:

  • You are not a patient with cystinuria
  • You are not already taking a cystine binding thiol drug
  • You have renal colic (if you are passing a stone)
  • You are scheduled to undergo a urologic procedure
  • You are unwilling or unable to provide informed consent in order to be able to participate.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (1)

NYU Langone Medical Center

New York, New York, 10016, United States

Location

MeSH Terms

Conditions

Cystinuria

Interventions

TioproninPenicillamine

Condition Hierarchy (Ancestors)

Renal AminoaciduriasRenal Tubular Transport, Inborn ErrorsKidney DiseasesUrologic DiseasesFemale Urogenital DiseasesFemale Urogenital Diseases and Pregnancy ComplicationsUrogenital DiseasesMale Urogenital DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and Abnormalities

Intervention Hierarchy (Ancestors)

Amino Acids, SulfurSulfur CompoundsOrganic ChemicalsAmino AcidsAmino Acids, Peptides, and ProteinsN-substituted GlycinesGlycine

Results Point of Contact

Title
David Goldfarb
Organization
NYU Langone Health

Study Officials

  • David S Goldfarb, MD

    NYU Langone Medical Center

    PRINCIPAL INVESTIGATOR

Publication Agreements

PI is Sponsor Employee
Yes

Study Design

Study Type
interventional
Phase
phase 4
Allocation
NA
Masking
NONE
Purpose
TREATMENT
Intervention Model
SINGLE GROUP
Sponsor Type
OTHER
Responsible Party
SPONSOR

Study Record Dates

First Submitted

April 24, 2014

First Posted

April 29, 2014

Study Start

June 1, 2012

Primary Completion

December 1, 2017

Study Completion

December 1, 2017

Last Updated

August 23, 2021

Results First Posted

August 23, 2021

Record last verified: 2021-07

Locations