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selexipag

Phase 3

Pulmonary Arterial Hypertension | Small molecule | Cardiovascular |Johnson & Johnson|Last Updated: Jul 20, 2026

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Trial Design
RandomizedDouble-BlindPLACEBO_CONTROLLEDDMC
Total Trials3
Total Enrollment1,253
FDA Designations
No designations recorded
Clinical trial landscape

selexipag · 12 trials · 9 indications

Phase 3 4Phase 2 2Phase 1 6
NCT04565990A Study of Selexipag in Participants Who Participated in a Previous Selexipag StudyHypertension, Pulmonary
COMPLETED43 Analytics
NCT04175600A Study of Selexipag as Add-On Treatment to Standard of Care in Children With Pulmonary Arterial HypertensionHypertension, Pulmonary
ACTIVE NOT_RECRUITING138 Analytics
NCT02471183Study to Assess the Tolerability and the Safety of the Transition From Inhaled Treprostinil to Oral Selexipag in Patients With Pulmonary Arterial HypertensionPulmonary Arterial Hypertension
COMPLETED34 Analytics
NCT01106014Selexipag (ACT-293987) in Pulmonary Arterial HypertensionPulmonary Arterial Hypertension
COMPLETED1,156 Analytics
PHASE3COMPLETED
A Study of Selexipag in Participants Who Participated in a Previous Selexipag Study
Hypertension, PulmonaryUnlock trial analytics
PHASE3ACTIVE NOT_RECRUITING
A Study of Selexipag as Add-On Treatment to Standard of Care in Children With Pulmonary Arterial Hypertension
Hypertension, PulmonaryUnlock trial analytics
PHASE3COMPLETED
Study to Assess the Tolerability and the Safety of the Transition From Inhaled Treprostinil to Oral Selexipag in Patients With Pulmonary Arterial Hypertension
Pulmonary Arterial HypertensionUnlock trial analytics
PHASE3COMPLETED
Selexipag (ACT-293987) in Pulmonary Arterial Hypertension
Pulmonary Arterial HypertensionUnlock trial analytics
Study Endpoints
Primary Endpoints
Number of Participants With Treatment-emergent Adverse Events (TEAEs)
From Day 1 up to 3 days after last dose of drug (up to 28 months 3 days)

Number of participants with TEAEs were reported. Adverse event (AE) was defined as any untoward medical occurrence in a clinical study participant administered a pharmaceutical (investigational or non investigational) product. An AE did not necessarily have a causal relationship with the pharmaceutical/biological agent under study. TEAEs were defined as AEs occurring at or after the initial administration of study intervention through the day of last dose plus 3 days. Data includes all TEAEs irrespective of whether they were serious or non-serious.

Number of Participants With TEAEs Leading to Premature Discontinuation of Selexipag
From Day 1 up to 3 days after last dose of drug (up to 28 months 3 days)

Number of participants with TEAEs leading to premature discontinuation of selexipag were reported. AE was defined as any untoward medical occurrence in a clinical study participant administered a pharmaceutical (investigational or non investigational) product. An AE did not necessarily have a causal relationship with the pharmaceutical/biological agent under study. TEAEs were defined as AEs occurring at or after the initial administration of study intervention through the day of last dose plus 3 days.

Number of Participants With Treatment-emergent Serious Adverse Events (TESAEs)
From Day 1 up to 3 days after last dose of drug (up to 28 months 3 days)

Number of participants with TESAEs were reported. AE was defined as any untoward medical occurrence in a clinical study participant administered a pharmaceutical (investigational or non investigational) product. An AE did not necessarily have a causal relationship with the pharmaceutical/biological agent under study. A SAE was any untoward medical occurrence at any dose that: resulted in death, was life threatening, required inpatient hospitalization or prolongation of existing hospitalization, resulted in persistent or significant disability/incapacity, or resulted in congenital anomaly/birth defect. TESAEs were defined as TSAEs occurring at or after the initial administration of study intervention through the day of last dose plus 3 days.

Number of Participants With Treatment-emergent Deaths
From Day 1 up to 3 days after last dose of drug (up to 28 months 3 days)

Number of participants with treatment-emergent deaths during the study were reported.

Number of Pregnant Females With Maternal Exposure to Selexipag
From Day 1 up to 30 days after last dose of drug (up to 29 months)

Number of pregnant females with maternal exposure to selexipag were reported.

Double-blind Period: Time to Disease Progression
Placebo arm: Randomization (Day 1) up to 7 days after last dose in the DB treatment (maximum up to 199 weeks); Selexipag arm: Randomization (Day 1) up to 7 days after last dose in the DB treatment (maximum up to 213 weeks)

Time to disease progression: time from randomization to first Clinical Event Committee (CEC)-confirmed disease progression event occurring between date of randomization until double-blind end date (DBED) + 7 days. Disease progression event as adjudicated by CEC, defined as any of the following: death (all causes); atrial septostomy or Potts' anastomosis, or registration on lung transplant list; hospitalization due to worsening pulmonary arterial hypertension (PAH); clinical worsening of PAH. Clinical worsening of PAH was defined as initiation of new PAH-specific therapy or intravenous diuretics or continuous oxygen use and at least 1 of the following: worsening in World Health Organization (WHO) functional class (FC); new occurrence or worsening: of syncope, of at least 2 PAH symptoms (shortness of breath/dyspnea, chest pain, cyanosis, dizziness/near syncope, or fatigue), of signs of right heart failure not responding to oral diuretics. Kaplan-Meier method was used for estimation.

Percentage of Subjects With Sustained Treatment Transition
At Week 16

A sustained treatment transition is considered if the 3 following criteria are met a) being on study treatment (selexipag) at Week 16, and b) not having a study treatment interruption(s) of a total of 8 days or more prior to Week 16, and c) absence of inhaled treprostinil or any prostanoid treatment after Week 8 up to Week 16. The percentage of subjects with a sustained treatment transition is calculated with 95% confidence interval (CI) using the Clopper-Pearson method.

Percentage of Subjects With Treatment-emergent Adverse Events (AEs),
26 weeks on average (from the first dose of selexipag up to 30 days after the last dose of selexipag)

Percentage of subjects with treatment-emergent AEs (serious and non serious), regardless of relationship to selexipag

Number of Subjects With Adverse Events Leading to Premature Discontinuation of Selexipag
Up to 22 weeks on average

Number of subjects with adverse events leading to premature discontinuation of selexipag is determined from the first dose of selexipag up to the last dose of selexipag

Absolute Change From Baseline Over Time in Blood Pressure
Baseline, Week 4, Week 12, Week 16

Both systolic(SBP) and diastolic (DBP) arterial blood pressure were measured in a sitting position after at least 5 minutes of rest at scheduled time points. Median change from baseline to pre-specified post-baseline visits are calculated

Absolute Change From Baseline Over Time in Heart Rate (HR)
Baseline, Week 4, Week 12, Week 16

Pulse rate is measured after at least 5 minutes of rest in a sitting position. Median change from baseline to pre-specified post-baseline visits are calculated.

Maximal Tolerated Dose
At Week 12, in subjects still on selexipag at Week 16

This is the individual maximal tolerated dose (MTD) observed at Week 12 in the subjects still on selexipag at Week 16. MTD is defined as the dose of selexipag reached with the last dose change up to Week 12

Time to Discontinuation of Inhaled Treprostinil.
Baseline to Week 16

Median time from baseline (Day1) to the end of down-titration of inhaled treprostinil is calculated

Time From Randomization to the First Morbidity Event or Death (All Causes) up to 7 Days After the Last Study Drug Intake
Up to 7 days after end of double-blind treatment (maximum: 4.3 years)

Time from randomization to the first occurrence of a morbidity event or death (all causes) was analyzed with the Kaplan-Meier method (event-free KM estimates at different time points). Morbidity event was defined as any of the following events confirmed by the Critical Event committee: * Hospitalization for worsening of pulmonary arterial hypertension (PAH), * Worsening of PAH resulting in need for lung transplantation or balloon atrial septostomy, * Initiation of parenteral prostanoid therapy or chronic oxygen therapy due to worsening of PAH, * Disease progression which was defined by a decrease in 6-minute walk distance from baseline (\>=15%, confirmed by a 2nd test on a different day) combined with worsening of WHO FC for patients belonging to WHO FC II/III at baseline, or combined with the need for additional PAH-specific therapy for patients belonging to WHO FC III/IV at baseline. Note: The number of patients at risk decreased over time but this cannot be captured below

Area Under the Plasma Concentration-time Curve Over a Dose Interval at Steady State of Selexipag and Its Metabolite ACT-333679 Combined (AUCτ, ss, Combined)
Week 1,Week 12: pre-dose, 1, 2, 4, 6, 8 and 12 h post-morning dose. Week 2, 4 and 6: pre-dose (Up to Week 12)

AUCτ, ss, combined was defined as the area under the plasma concentration-time curve over one dosing interval at steady state. AUCτ,ss,combined was calculated as 1/38 AUCτ,ss,selexipag plus 37/38 AUCτ,ss,ACT-333679.

Average number of Raynaud's phenomenon (RP) attacks per week during the maintenance treatment period
From Day 26 to Day 56 ( +/- 7 days)

The number of RP attacks is determined from daily entries in electronic Diaries (eDiary).

Area under the plasma concentration-time profile of selexipag and ACT-333679 during a dose interval (AUC-tau)
Blood samples at pre-dose, 0.5, 1, 2, 3, 4, 5, 6, 7, 8, 10 and 12 hours post-dose on Day 3, Day 4 and Day 10

AUC-tau for selexipag and ACT-333679 is calculated on the basis of the actual blood sampling time points drawn during the 12-hour interval after the morning administration of selexipag administered either alone (Day 3) or concomitantly with clopidogrel (Day 4 and Day 10)

Maximal plasma concentration of selexipag and ACT-333679 (Cmax)
Blood samples at pre-dose, 0.5, 1, 2, 3, 4, 5, 6, 7, 8, 10 and 12 hours post-dose on Day 3, Day 4 and Day 10

Cmax is directly derived from the individual plasma concentration-time curves for selexipag and ACT-333679, following administration of selexipag alone (Day 3) or concomitantly with clopidogrel (Day 4 and Day 10)

Cmax of midazolam following administration of midazolam alone and in combination with selexipag
From pre-dose up to 24 hours after midazolam admisnitration for each treatment period

Cmax is the maximum observed plasma concentration and is directly derived from the individual plasma concentration time curves of midazolam

AUC(0-inf) of midazolam following administration of midazolam alone and in combination with selexipag
From pre-dose up to 24 hours after midazolam admisnitration for each treatment period

AUC(0-inf) is the area under the plasma concentration-time curves of midazolam, calculated from time 0 (pre-dose) to the extrapolated infinite time

Area under plasma concentration-time curve [AUC(0-inf)] of selexipag and ACT-333679
From predose until 72 hours postdose for each treatment period

AUC(0-inf) is the area under plasma concentration-time curves for selexipag and its metabolite (ACT-333679), calculated from zero to the extrapolated infinite time

Maximum plasma concentration (Cmax) of selexipag and ACT-333679
From predose until 72 hours postdose for each treatment period

Cmax is directly derived from the individual plasma concentration time curves for selexipag and its metabolite ACT-333679

Area under the plasma concentration-time curve from zero to infinity [AUC(0-inf)] of selexipag and ACT-333679
Blood samples at different time points from pre-dose up to 72 hours after selexipag administration in each study period (except for the period with co-administration of gemfibrozil: up to 144 hours)

AUC(0-inf) is calculated for selexipag and its metabolite, ACT-333679, following administration of selexipag alone or concomitantly with gemfibrozil (Part I) or rifampicin (Part II)

Area under the plasma concentration-time curve (AUCt) for selexipag
23 days

Blood will be collected by direct venipuncture or via an intravenous catheter placed in an arm vein after 4.5 days' treatment with 1600 μg selexipag b.i.d. during Treatments A and B. Selexipag will be quantified in plasma samples using a validated liquid chromatography with tandem mass spectrometry (LC-MS/MS) assay. AUCt will be calculated according to the linear trapezoidal rule using the measured concentration-time values above the limit of quantification during one dosing interval.

Maximum plasma concentration at steady state (Cmax,ss) for selexipag
23 days

Blood will be collected by direct venipuncture or via an intravenous catheter placed in an arm vein after 4.5 days' treatment with 1600 μg selexipag b.i.d. during Treatments A and B. Selexipag will be quantified in plasma samples using a validated liquid chromatography with tandem mass spectrometry (LC-MS/MS) assay. The measured individual plasma concentrations of selexipag will be used to directly obtain Cmax,ss.

Baseline adjusted placebo corrected change in corrected QTc interval (time interval from beginning of the Q wave until end of the T wave) (ΔΔQTcI) at various time points
24 days

Electrocardiograms (ECGs) will be extracted in replicate at predefined timepoints from replicated 12-lead Holter ECGs. Cardiodynamic endpoints will be assessed at steady-state for 800 μg selexipag on Day 11 and at steady-state for 1600 μg selexipag on Day 23. Based on prospective criteria, QTcI will be the primary method for heart rate (HR) correction.

Secondary Endpoints
Double-blind Period: Change in log2 N-terminal Pro-brain Natriuretic Peptide (NT-proBNP) From Baseline to Week 24
Baseline (Day 1), Week 24
Double-blind Period: Number of Participants With Treatment-emergent Adverse Events (TEAEs) and Treatment-emergent Serious AEs (TESAEs)
Placebo arm: Start of treatment (Day 1) up to 3 days after last dose in the DB treatment (maximum up to 198.42 weeks); Selexipag arm: Start of treatment (Day 1) up to 3 days after last dose in the DB treatment (maximum up to 212.42 weeks)
Double-blind Period: Number of Participants With AEs Leading to Premature Discontinuation of Study Treatment
Placebo arm: Start of treatment (Day 1) up to last dose in the DB treatment (maximum up to 198 weeks); Selexipag arm: Start of treatment (Day 1) up to last dose in the DB treatment (maximum up to 212 weeks)
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Study Design & Arms
AllocationNA
MaskingNONE
ModelSINGLE_GROUP
PurposeTREATMENT
Treatment Arms
ArmTypeDescription
SelexipagEXPERIMENTALParticipants will receive selexipag tablets twice daily with the dose strength corresponding to their individual maximum tolerated dose (iMTD) from the parent study.
PlaceboPLACEBO_COMPARATORParticipants will receive matching placebo based on the body weight on Day 1 and will continue thereafter with twice daily dosing. Participants will continue to receive PAH-specific concomitant therapies such as ERAs, PDE-5 inhibitors, and soluble guanylate cyclase stimulator as per local standard-of-care.
Open-Label Extension Period: SelexipagEXPERIMENTALParticipants with a positive benefit/risk ratio of selexipag for PAH will be offered selexipag in the open label extension period. Participants on selexipag during the double-blind treatment period will continue treatment at their iMTD during the OLEP, for those previously on placebo, the iMTD will uptitrate selexipag during first 12 weeks until participant reaches iMTD. Participants will continue to receive PAH-specific concomitant therapies such as ERAs, PDE-5 inhibitors, and soluble guanylate cyclase stimulator as per local standard-of-care.
Selexipag, Open LabelEXPERIMENTALSubjects on inhaled treprostinil treatment participate in a 16-week main treatment period including down-titration of treprostinil to end of Week 8 and parallel up-titration of selexipag to the maximum tolerated dose (MTD) up to Week 12, for each individual patient but not above 1600 mcg twice daily. From Week 12 up to Week 16, patients continue selexipag at their individual MTD. Patients could continue the study drug selexipag during the extended treatment period from Week 16 until commercial availability of selexipag.
1EXPERIMENTALSelexipag is up-titrated from Day 1 to Week 12 to each patient's maximum tolerated dose in the range of 200-1600 µg twice a day (b.i.d.) in 200 µg steps starting with one 200 µg oral tablet on Day 1. From Day 2 onwards, a b.i.d. dose regimen with an interval of approximately 12 hours is followed. If this dose (selexipag 200 μg b.i.d.) is well-tolerated, selexipag is up-titrated with weekly increments of 200 µg. Up-titration is followed by a stable maintenance treatment period from Week 12 onwards, up to Week 26, at the maximum tolerated dose
2PLACEBO_COMPARATORMatching placebo is administered orally with a dosing interval of approximately12 h. A (mock) up-titration scheme is followed
open label selexipagEXPERIMENTALThe first dose of selexipag (Uptravi) will be administered in the evening of Day 1 and will be based on the body weight. Thereafter selexipag will be administered twice daily (morning and evening). Selexipag will be up-titrated during the first 12 weeks, with weekly increments equal to the starting dose until the participants reach their individual maximum tolerated dose (iMTD) or until a maximum dose corresponding to their baseline weight category is achieved (which will be 8-fold of the corresponding starting dose). Up-titration is followed by a stable maintenance treatment period from Week 12 to Week 16, at the maximum tolerated dose. Thereafter, participants will be treated with selexipag as long as the treatment is beneficial to the participants, as per investigator's decision.
Sequential treatment armEXPERIMENTALSubjects receive 1 tablet of selexipag twice daily from Day 1 to Day 9 and 1 tablet in the morning of Day 10. In the morning of Day 4 and 1 hour before the administration of selexipag, they receive 4 tablets of clopidogrel. Then from Day 5 to Day 10, 1 hour before the morning administration of selexipag, they receive 1 tablet of clopidogrel .
Sequence ABEXPERIMENTALSubjects participate in two study periods: During the first period, they receive a single oral dose of midazolam on Day 1. During the second period, they receive oral selexipag alone from Day 1 to Day 11 and selexipag + midazolam on Day 12. There is a washout period of 14 to 21 days between the two periods.
Sequence BAEXPERIMENTALSubjects participate in two study periods: During the first period, they receive oral selexipag alone from Day 1 to Day 11 and selexipag + midazolam on Day 12. During the second period, they receive a single oral dose of midazolam on Day 1. There is a washout period of 14 to 21 days between the two periods.
Part 1, sequence ABEXPERIMENTALSubjects participate in two study periods: During the first period (Treatment A), they receive oral selexipag on Day 1. During the second period (Treatment B), they receive multiple oral dose of gemfibrozil from Day 1 to Day 9. Subjects also receive a single oral dose of selexipag on Day 4 concomitantly with gemfibrozil. There is a washout period of 14 to 21 days between the two periods.
Part 1, sequence BAEXPERIMENTALSubjects participate in two study periods: During the first period (Treatment B), they receive multiple oral dose of gemfibrozil from Day 1 to Day 9. They also receive a single oral dose of selexipag on Day 4 concomitantly with gemfibrozil. During the second period (Treatment A) they receive oral selexipag on Day 1. There is a washout period of 14 to 21 days between the two periods.
Part 2, sequence ABEXPERIMENTALSubjects participate in two study periods: During the first period (Treatment A), they receive oral selexipag on Day 1. During the second period (Treatment B), they receive rifampicin once daily from Day 1 to Day 9. Subjects also receive a single oral dose of selexipag on Day 7 together with the dose of rifampicin.There is a washout period of 14 to 21 days between the two periods.
Part 2, sequence BAEXPERIMENTALSubjects participate in two study periods: During the first period (Treatment B), they receive rifampicin once daily from Day 1 to Day 9. Subjects also receive a single oral dose of selexipag on Day 7 together with the dose of rifampicin. During the second period (Treatment A), they receive oral selexipag on Day 1. There is a washout period of 14 to 21 days between the two periods.
Treatment Sequence ABEXPERIMENTALSubjects will receive Treatment A in Period 1 followed by Treatment B in Period 2. There will be a washout period lasting at least 6 days between treatments. Treatment A: up-titration from Day 1-18 will performed in 200 μg steps every fourth day with multiples of 200 μg film-coated tablets starting with 400 μg selexipag b.i.d. The up-titration will be followed by treatment with 8 film-coated tablets, 200 μg each, b.i.d. from Day 19 to the morning dose of Day 23. Treatment B: up-titration from Day 1-18 will be performed in 200 μg steps every fourth day with multiples of 200 μg film-coated tablets starting with 400 μg selexipag b.i.d. The up-titration will be followed by treatment with one single film-coated tablet, 1600 μg, b.i.d. from Day 19 to the morning dose of Day 23.
Treatment Sequence BAEXPERIMENTALSubjects will receive Treatment B in Period 1 followed by Treatment A in Period 2. There will be a washout period lasting at least 6 days between treatments. Treatment A: up-titration from Day 1-18 will performed in 200 μg steps every fourth day with multiples of 200 μg film-coated tablets starting with 400 μg selexipag b.i.d. The up-titration will be followed by treatment with 8 film-coated tablets, 200 μg each, b.i.d. from Day 19 to the morning dose of Day 23. Treatment B: up-titration from Day 1-18 will be performed in 200 μg steps every fourth day with multiples of 200 μg film-coated tablets starting with 400 μg selexipag b.i.d. The up-titration will be followed by treatment with one single film-coated tablet, 1600 μg, b.i.d. from Day 19 to the morning dose of Day 23.
Group AEXPERIMENTALSubjects in Group A receive selexipag on Days 3 to 23 and moxifloxacin-matching placebo on Days 2 and 24. Selexipag administered orally, twice a day, for 21 days according to the following multiple dose up-titration regimen: 400 μg on Days 3-5, 600 μg on Days 6-8, 800 μg on Days 9-11, 1000 μg on Days 12-14, 1200 μg on Days 15-17, 1400 μg on Days 18-20, and 1600 μg on Days 21-23 (only morning dose on Day 23).
Group B1EXPERIMENTALSubjects in Group B1 receive 400 mg moxifloxacin, orally on Day 2 and moxifloxacin-matching placebo, orally on Day 24. Subjects receive placebo for selexipag, orally on Days 3 to 23.
Group B2EXPERIMENTALSubjects in Group B2 receive moxifloxacin-matching placebo, orally on Day 2 and 400 mg moxifloxacin, orally on Day 24. Subjects receive placebo for selexipag, orally on Days 3 to 23.
Interventions
NameTypeDescription
SelexipagDRUGSelexipag tablets will be administered orally at all dose strengths (200, 400, 600, 800, 1000, 1200, 1400 and 1600 microgram) twice daily.
PlaceboDRUGMatching placebo tablets will be administered orally.
Standard of Care (SOC): Endothelin receptor antagonistDRUGERAs will be administered as SOC therapy.
SOC: Phosphodiesterase type 5 (PDE-5) inhibitorDRUGPDE-5 inhibitor will be administered as SOC therapy.
SOC: Soluble guanylate cyclase stimulatorDRUGSoluble guanylate cyclase stimulator will be administered as SOC therapy.
selexipag (Uptravi)DRUGFilm-coated tablets for oral administration
ClopidogrelDRUGEach film-coated tablet containing 75 mg of clopidogrel (oral use)
MidazolamDRUGSingle oral dose of 7.5 mg midazolam (tablet)
Selexipag (adult formulation)DRUGOne selexipag film-coated tablet of 200 µg
Selexipag (pediatric formulation)DRUGFour selexipag film-coated tablets of 50 µg
GemfibrozilDRUGGemfibrozil film-coated tablet of 600 mg administered orally b.i.d. from Day 1 to Day 9
RifampicinDRUGRifampicin film-coated tablet of 600 mg administered orally o.d.from Day 1 to Day 9
placebo for selexipagDRUG -
moxifloxacinDRUG -
moxifloxacin-matching placeboDRUG -
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Eligibility Criteria
Age Range18 Years to N/A
SexALL
Healthy VolunteersNo
Study Sites13

Inclusion Criteria: * Treated with selexipag at the end of a parent study and: a) the parent study has established efficacy with a favorable benefit/risk profile for the indication under investigation; b) participant may continue to benefit from treatment with selexipag; c) has completed the end of...

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Recent Changes (Last 90 Days)
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