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A Study Evaluating the Efficacy, Safety, Pharmacokinetics and Pharmacodynamics of Crovalimab in Pediatric Participants With Atypical Hemolytic Uremic Syndrome (aHUS)

Phase 3
Active, not recruiting
Conditions
Atypical Hemolytic Uremic Syndrome
Interventions
Registration Number
NCT04958265
Lead Sponsor
Hoffmann-La Roche
Brief Summary

This study aims to evaluate the efficacy and safety of crovalimab in pediatric participants with aHUS.

Detailed Description

Not available

Recruitment & Eligibility

Status
ACTIVE_NOT_RECRUITING
Sex
All
Target Recruitment
41
Inclusion Criteria
  • Body weight >= 5 kg at screening.
  • Vaccination against Neisseria meningitis serotypes A, C, W, and Y; vaccination against serotype B, according to national vaccination recommendations.
  • Vaccination against Haemophilus influenzae type B and Streptococcus pneumoniae, according to national vaccination recommendations.
  • For patients continuing to receive other therapies concomitantly with crovalimab (e.g., immunosuppressants, corticosteroids, mammalian target of rapamycin inhibitor (mTORi), or calcineurin inhibitors): stable dose for >=28 days prior to screening and up to the first crovalimab administration.
  • For female participants of childbearing potential: an agreement to remain abstinent or use contraception.
  • Participants with a prior kidney transplant are eligible if they have a known history of complement-mediated aHUS prior to the kidney transplant.
  • Onset of initial TMA presentation within 28 days prior to the first dose of crovalimab (for Naive Cohort only).
  • Documented treatment with either eculizumab or ravulizumab (for Switch Cohort only).
  • Clinical evidence of response to a C5 inhibitor (for Switch Cohort only).
  • Poorly controlled TMA following treatment with another C5 inhibitor (for C5 SNP participants in the Pretreated Cohort only).
  • Known C5 polymorphism (for C5 SNP participants in the Pretreated Cohort only).
Exclusion Criteria
  • TMA associated with non-aHUS related renal disease.
  • Positive direct Coombs test.
  • Chronic dialysis within 90 days prior to first crovalimab administration , and /or end stage renal disease
  • Identified drug exposure-related TMA.
  • Presence or history of a condition that could trigger TMA, such as malignancy, bone marrow or organ transplant (other than kidney transplant) or autoimmune disease.
  • History of a kidney disease, other than aHUS.
  • History of Neisseria meningitidis infection within 6 months of study enrollment.
  • Known or suspected immune deficiency (e.g., history of frequent recurrent infections).
  • Positive HIV test.
  • Active systemic bacterial, viral, or fungal infection within 14 days before first crovalimab administration.
  • Presence of fever (>= 38°C) before the first crovalimab administration (If fevers are solely due to the underlying aHUS pathology, and there is no evidence or suspicion of a systemic infection, participants may enroll).
  • Multi-system organ dysfunction or failure.
  • Recent intravenous immunoglobulin (IVIg) treatment.
  • Pregnant or breastfeeding or intending to become pregnant.
  • Participation in another interventional treatment study with an investigational agent or use of any experimental therapy within 28 days of screening or within five half lives of that investigational product, whichever is greater.
  • Recent use of tranexamic acid.
  • Current or previous treatment with a complement inhibitor (for Naive Cohort only).
  • First initiation of plasma exchange/plasma infusions (PE/PI) should not be more than 28 days prior to first crovalimab administration (for Naive Cohort only).
  • Last PE/PI completed less than 2 hours prior to first crovalimab administration (for Naive Cohort only).
  • Receiving PE/PI within 8 weeks of the first crovalimab administration (Switch Cohort only).
  • Normalization of serum creatinine values at baseline (<97.5th percentile for age), (for Naive Cohort only).
  • Positive for active Hepatitis B and/or C infections (HBV/HCV) (for Switch Cohort and switching C5 SNP Pretreated Cohort participants who recently received C5 inhibitor treatment).
  • Cryoglobulinemia at screening (for Switch Cohort and C5 SNP Cohort participants who recently received C5 inhibitor treatment).
  • Diagnosis of a condition leading to non-aHUS TMA: Thrombotic Thrombocytopenic Purpura (TTP), Shiga Toxin producing Escherichia Coli (STEC)-TMA, Pneumococcal HUS, TMA secondary to cobalamin C defect (as demonstrated by either increased total blood homocysteine levels or MMACHC gene mutation) and TMA related to Diacylglycerol kinase ε (DGKE) nephropathy.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
CrovalimabCrovalimabParticipants will be enrolled in three cohorts: \[1\] Naive Cohort - participants who have not been previously treated with complement inhibitor therapy; \[2\] Switch Cohort - participants who switch to crovalimab from another C5 inhibitor and \[3\] Pretreated Cohort (includes C5 SNP (Single Nucleotide Polymorphism) participants) - participants who received treatment with another C5 inhibitor and subsequently discontinued it.
Primary Outcome Measures
NameTimeMethod
Percentage of Participants with complete TMA response (cTMAr) (Naive Cohort only)Baseline up to Week 25 (after 24 weeks on treatment)
Secondary Outcome Measures
NameTimeMethod
Observed Value in Platelet Count (Naive and Switch Cohorts)Baseline up to Week 25 (after 24 weeks on treatment)
Observed Value in Lactate Dehydrogenase (LDH) (mg/dL) (Naive and Switch Cohorts)Baseline up to Week 25 (after 24 weeks on treatment)
Observed Value in Hemoglobin (mg/dL) (Naive and Switch Cohorts)Baseline up to Week 25 (after 24 weeks on treatment)
Change from Baseline in Platelet Count (Naive and Switch Cohorts)Baseline up to Week 25 (after 24 weeks on treatment)
Change in Estimated Glomerular Filtration Rate (eGFR) (Naive and Switch Cohorts)Baseline up to Week 25 (after 24 weeks on treatment)
Percentage of Participants with Change from Baseline in Chronic Kidney Disease (CKD) stage (Naive and Switch Cohorts)Baseline up to Week 25 (after 24 weeks on treatment)
Change from Baseline in Dialysis StatusBaseline up to Week 25 (after 24 weeks on treatment)
Change from Baseline in Lactate Dehydrogenase (LDH) (mg/dL) (Naive and Switch Cohorts)Baseline up to Week 25 (after 24 weeks on treatment)
Change from Baseline in Hemoglobin (mg/dL) (Naive and Switch Cohorts)Baseline up to Week 25 (after 24 weeks on treatment)
Percentage of Participants with Platelet Count >= LLN (Naive Cohort only)Baseline up to Week 25 (after 24 weeks on treatment)
Percentage of Participants with Normalisation of LDH (i.e., =< Upper Limit Normal (ULN)) (Naive Cohort only)Baseline up to Week 25 (after 24 weeks on treatment)
Percentage of Participants with >=25% decrease in Serum Creatinine (Naive Cohort only)Baseline up to Week 25 (after 24 weeks on treatment)
Time to complete TMA response (cTMAr) (Naive Cohort only)Up to 8 years
Duration of complete TMA response (cTMAr) (Naive Cohort only)Up to 8 years
Percentage of Participants with ongoing complete TMA response (cTMAr) (Naive Cohort only)At Week 25
Percentage of Participants with maintained TMA control (mTMAc) (Switch Cohort only)Baseline through Week 25 (after 24 weeks on treatment)
Percentage of Participants with Adverse Events (AEs)Up to 8 years
Percentage of Participants with Injection-Site Reactions, Infusion-Related Reactions, Hypersensitivity, Malignant Hypertension (including malignant renal hypertension) and Infections (including meningococcal meningitis)Up to 8 years
Percentage of Participants with Adverse Events (AEs) leading to Study Drug DiscontinuationUp to 8 years
Percentage of Participants with Clinical Manifestations of Drug-Target-Drug Complex (DTDC) formation amongst participants who switched to crovalimab treatment from eculizumab/ravulizumab treatment (Switch Cohort and switching Pretreated participants)Up to Week 25
Serum Concentrations of Crovalimab over timeUp to 8 years
Prevalence of Anti-Crovalimab Antibodies at BaselineBaseline
Percentage of Participants with Anti-Crovalimab AntibodiesUp to 8 years
Observed value of Pharmacodynamic Markers (CH50, Free/Total C5)Up to 8 years

Trial Locations

Locations (38)

Chiba Children's Hospital

🇯🇵

Chibashi, Chibaken, Japan

Tongji Hosp, Tongji Med. Col, Huazhong Univ. of Sci. & Tech

🇨🇳

Wuhan City, China

Hôpital Arnaud de Villeneuve

🇫🇷

Montpellier, France

Hôpital Robert Debré

🇫🇷

Paris, France

Gh Necker Enfants Malades

🇫🇷

Paris, France

CHU de Toulouse - Hôpital des Enfants

🇫🇷

Toulouse, France

Szegedi Tudományagyetem

🇭🇺

Szeged, Hungary

Institute of Kidney Diseases and Research Centre

🇮🇳

Ahmadabad CITY, Gujarat, India

Medanta-The Medicity

🇮🇳

Gurgaon, Haryana, India

Yokohama City University Medical Center

🇯🇵

Kanagawa, Japan

Instytut ?Centrum Zdrowia Matki Polki

🇵🇱

Lodz, Poland

Red Cross War Memorial Children's Hospital

🇿🇦

Observatory, South Africa

University of California Davis Medical Center

🇺🇸

Sacramento, California, United States

Children's Hospital Colorado

🇺🇸

Aurora, Colorado, United States

Memorial Healthcare Systems

🇺🇸

Hollywood, Florida, United States

Emory Children's Center

🇺🇸

Atlanta, Georgia, United States

University of Nebraska

🇺🇸

Omaha, Nebraska, United States

Hackensack University Medical Center

🇺🇸

Hackensack, New Jersey, United States

Cincinnati Children's Hospital Medical Center

🇺🇸

Cincinnati, Ohio, United States

UT Health Science Center

🇺🇸

San Antonio, Texas, United States

University Of Utah Hosp & Clin

🇺🇸

Salt Lake City, Utah, United States

UZ Gent

🇧🇪

Gent, Belgium

UZ Leuven Gasthuisberg

🇧🇪

Leuven, Belgium

CHC MontLégia

🇧🇪

Liege, Belgium

Santa Casa de Belo Horizonte

🇧🇷

Belo Horizonte, Minas Gerais, Brazil

UPECLIN Hospital das Clinicas da Faculdade de Medicina de Botucatu

🇧🇷

Botucatu, São Paulo, Brazil

Inst. Da Criança- Faculdade de Medicina Usp

🇧🇷

Sao Paulo, São Paulo, Brazil

British Columbia Children's Hospital

🇨🇦

Vancouver, British Columbia, Canada

CHU Sainte-Justine

🇨🇦

Montreal, Quebec, Canada

Peking University First Hospital

🇨🇳

Beijing City, China

Beijing Children's Hospital, Capital Medical University

🇨🇳

Beijing City, China

The children's hospital , Zhejiang university school of medicine

🇨🇳

Hangzhou City, China

All India Institute Of Medical Sciences (AIIMS)

🇮🇳

New Delhi, Delhi, India

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico

🇮🇹

Milano, Lombardia, Italy

Aichi Children?s Health and Medical Center

🇯🇵

Aichi, Japan

Okinawa Prefectural Nanbu Medical Center & Children's Medical Center

🇯🇵

Okinawa, Japan

Hospital de Especialidades Puerta de Hierro S.A de C.V.

🇲🇽

Zapopan, Mexico

Uniwersyteckie Centrum Kliniczne

🇵🇱

Gdansk, Poland

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