A Study to See Iftolvaptan is Safe in Infants and Children Who at Enrollment Are 28 Days to Less Than 18 Years Old withAutosomal Recessive Polycystic Kidney Disease (ARPKD)

NCT ID: NCT04782258

Last Updated: 2026-01-21

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE3

Total Enrollment

20 participants

Study Classification

INTERVENTIONAL

Study Start Date

2023-01-23

Study Completion Date

2028-08-14

Brief Summary

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To evaluate the safety of tolvaptan in pediatric subjects with ARPKD

Detailed Description

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This study is a multinational, multicenter, open-label, non-randomized trial. The study consist of three periods: Screening Period, Treatment period and Follow-up period.

Tolvaptan has been demonstrated to delay the decline of kidney function in adults with rapidly progressing ADPKD (CKD stages 1 to 4), a closely related indication to ARPKD, as measured by estimated glomerular filtration rate (eGFR) and Total Kidney Volume (TKV).

Participants in this study will be assigned to tolvaptan and followed for 18 months over the course of the study.

The overall trial duration is expected to be approximately 3.5 years.

Conditions

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Autosomal Recessive Polycystic Kidney (ARPKD)

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Tolvaptan Suspension

Tolvaptan suspension will be administered orally or via feeding/nasogastric tube at doses of 0.15 mg/kg once daily in the AM, 0.30 mg/kg once daily in the AM, 0.5 mg/kg once daily in the AM, 0.75 mg/kg split dose (0.5 mg/kg AM and 0.25 mg/kg 8 hours later), and 1 mg/kg split dose (0.67 mg/kg AM and 0.33 mg/kg 8 hours later) based on age. Treatment duration is 18 months.

Group Type EXPERIMENTAL

Tolvaptan Suspension

Intervention Type DRUG

Syrup

Tolvaptan Tablets

Tolvaptan tablets will be administered orally as split-dose regimens (15/7.5 mg, 30/15 mg, and 45/15 mg) upon awakening and 8 hours later (twice daily) based on weight if able to swallow tablets. Treatment duration is 18 months.

Group Type EXPERIMENTAL

Tolvaptan Tablets

Intervention Type DRUG

Tolvaptan (OPC-41061) Tolvaptan tablets will be administered orally as split-dose regimens (15/7.5 mg, 30/15 mg, and 45/15 mg) upon awakening and 8 hours later (twice daily) based on weight if able to swallow tablets.

Interventions

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Tolvaptan Suspension

Syrup

Intervention Type DRUG

Tolvaptan Tablets

Tolvaptan (OPC-41061) Tolvaptan tablets will be administered orally as split-dose regimens (15/7.5 mg, 30/15 mg, and 45/15 mg) upon awakening and 8 hours later (twice daily) based on weight if able to swallow tablets.

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

1. Male or female subjects between 28 days and less than 18 years of age, with clinical features that are consistent with a diagnosis of ARPKD.
2. Ability for parent/legal guardian to provide written, informed consent prior to initiation of any trial-related procedures, and ability, in the opinion of the principal investigator, to comply with all the requirements of the trial. Ability to provide written informed assent from all subjects old enough per local laws to provide assent.

Exclusion Criteria

1. Premature birth (≤ 32 weeks gestational age) for infants 28 days to \< 12 weeks of age.
2. Anuria or RRT defined as intermittent or continuous hemodialysis, peritoneal dialysis, hemofiltration, hemodiafiltration or history of kidney transplantation.
3. Evidence of syndromic conditions associated with renal cysts (other than ARPKD).
4. Abnormal liver function tests including ALT and AST, \> 1.2 × ULN (upper limit of normal).
5. Has splenomegaly or portal hypertension (HTN).
6. Parents with renal cystic disease.
7. Receiving chronic diuretic that could not be adjusted after tolvaptan initiation.
8. Cannot be monitored for fluid balance.
9. Has or at risk of having sodium and potassium electrolyte imbalances, as determined by the investigator.
10. Has or at risk of having significant hypovolemia as determined by investigator.
11. Clinically significant anemia, as determined by investigator.
12. Platelets \< 50000 µL.
13. Severe systolic dysfunction defined as ejection fraction \< 14%.
14. Serum sodium levels \< 130 mmol/L or \>145 mmol/L.
15. Taking any other experimental medications.
16. Require ventilator support.
17. Taking medications known to induce CYP3A4 (CYP = Cytochrome P).
18. Having an infection including viral that would require therapy disruptive to IMP dosing.
19. Females who are breast-feeding or who have a positive pregnancy test result prior to receiving IMP.
20. Subjects with a history of substance abuse (within the last 6 months).
21. Subjects who have bladder dysfunction and/or difficulty voiding.
22. Subjects taking a vasopressin agonist (eg, desmopressin).
23. Subjects with a history of persistent noncompliance with antihypertensive or other important medical therapy.
24. Subjects taking medications or having concomitant illnesses likely to confound endpoint assessments, including taking approved (ie, marketed) therapies for the purpose of affecting PKD cysts such as tolvaptan, vasopressin antagonists, anti-sense ribonucleic acid (RNA) therapies, rapamycin, sirolimus, everolimus, or somatostatin analogs (ie, octreotide, sandostatin).
25. Received or are scheduled to receive a liver transplant.
26. History of cholangitis within the last 6 months.
27. Has findings consistent with clinically significant portal hypertension (eg, varices, variceal bleeding, hypersplenism indicated by thrombocytopenia).
Minimum Eligible Age

28 Days

Maximum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Otsuka Pharmaceutical Development & Commercialization, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Children's National Medical Center

Washington D.C., District of Columbia, United States

Site Status RECRUITING

Emory University Hospital

Atlanta, Georgia, United States

Site Status WITHDRAWN

Northwestern University Feinberg School of Medicine - Ann & Robert H. Lurie Children's Hospital of Chicago - Neonatology

Chicago, Illinois, United States

Site Status RECRUITING

Riley Hospital for Children

Indianapolis, Indiana, United States

Site Status WITHDRAWN

Children's Hospital - New Orleans

New Orleans, Louisiana, United States

Site Status WITHDRAWN

Johns Hopkins Pediatric Specialty Clinic

Baltimore, Maryland, United States

Site Status RECRUITING

C.S. Mott Children's Hospital

Ann Arbor, Michigan, United States

Site Status RECRUITING

Mayo Clinic - Rochester

Rochester, Minnesota, United States

Site Status RECRUITING

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status RECRUITING

Cleveland Clinic

Cleveland, Ohio, United States

Site Status RECRUITING

Children's Hospital of Pittsburgh of UPMC

Pittsburgh, Pennsylvania, United States

Site Status WITHDRAWN

Primary Children's Hospital

Salt Lake City, Utah, United States

Site Status WITHDRAWN

Université Catholique De Louvain And Cliniques St Luc

Brussels, Brussels Capital, Belgium

Site Status RECRUITING

Universitair Ziekenhuis Gent

Ghent, Oost-Vlaanderen, Belgium

Site Status RECRUITING

UZ Leuven

Leuven, Vlaams Brabant, Belgium

Site Status RECRUITING

Universitätsklinikum Köln

Cologne, North Rhine-Westphalia, Germany

Site Status RECRUITING

Instytut "Pomnik - Centrum Zdrowia Dziecka"

Warsaw, Masovian Voivodeship, Poland

Site Status WITHDRAWN

Uniwersytecki Dzieciecy Szpital Kliniczny im. L. Zamenhofa

Bialystok, , Poland

Site Status WITHDRAWN

Universitat de Barcelona - Hospital Sant Joan de Deu Barcelona (HSJDB)

Esplugues de Llobregat, Barcelona, Spain

Site Status RECRUITING

Hospital Universitari Parc Tauli

Sabadell, Barcelona, Spain

Site Status WITHDRAWN

Hospital Universitari Vall D Hebron

Barcelona, , Spain

Site Status RECRUITING

Hospital Universitario Virgen del Rocío Avenida Manuel Siurot

Seville, , Spain

Site Status WITHDRAWN

Great Ormond Street Hospital for Children NHS Trust

London, , United Kingdom

Site Status RECRUITING

Countries

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United States Belgium Germany Poland Spain United Kingdom

Central Contacts

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Otsuka Call Center

Role: CONTACT

844-687-8522

Other Identifiers

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2020-005992-10

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

156-201-00307

Identifier Type: -

Identifier Source: org_study_id

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