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Study to Evaluate Romosozumab in Children and Adolescents With Osteogenesis Imperfecta

Phase 1
Completed
Conditions
Osteogenesis Imperfecta
Interventions
Dietary Supplement: Calcium
Dietary Supplement: Vitamin D
Registration Number
NCT04545554
Lead Sponsor
Amgen
Brief Summary

The primary objective of this study is to evaluate the pharmacokinetics (PK) profile following multiple subcutaneous (SC) doses of romosozumab in children and adolescents with Osteogenesis Imperfecta (OI).

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
25
Inclusion Criteria
  • Ambulatory male or female children 5 to less than 18 years of age upon entry into screening
  • Clinical diagnosis of OI defined as a clinical history consistent with type I-IV OI as determined by presence of expected phenotype and lack of additional features unrelated to type I-IV OI

Exclusion Criteria

  • History of an electrophoresis pattern inconsistent with type I to type IV OI
  • History of known mutation in a gene other than collagen type I alpha 1/collagen type I alpha 2 (COL1AI/COL1A2) causing OI or other metabolic bone disease
  • History of other bone diseases that affect bone metabolism (eg, osteoporosis pseudoglioma syndrome, idiopathic juvenile osteoporosis, osteopetrosis, hypophosphatasia)
  • History of Kawasaki disease, rheumatic myocarditis, ischemic cardiomyopathy, inherited cardiomyopathies, nephrotic syndrome, familial hypercholesterolemia, stroke, or any thromboembolic disorder
  • Unhealed fracture as defined by orthopedic opinion
  • Symptoms associated with skull abnormalities such as basilar invagination, basilar impression or Chiari malformation
  • Prior treatment with anti-sclerostin antibody, fluoride or strontium, parathyroid hormone (PTH) within 12 months prior to screening, denosumab within 12 months or zoledronic acid within 6 months prior to first dose
  • Less than 2 evaluable vertebrae by DXA evaluation in the region of interest, L1 - L4, as confirmed by the central imaging laboratory.
  • Clinically significant valvular heart disease based on local echocardiogram (ECHO) results.
Exclusion Criteria

Not provided

Study & Design

Study Type
INTERVENTIONAL
Study Design
SEQUENTIAL
Arm && Interventions
GroupInterventionDescription
Romosozumab: 12 - < 18 Years of AgeCalciumParticipants will receive 1 of 3 dose levels of romosozumab. All participants also received calcium and vitamin D.
Romosozumab: 5 - < 12 Years of AgeVitamin DParticipants will receive 1 of 3 dose levels of romosozumab. All participants also received calcium and vitamin D.
Romosozumab: 5 - < 12 Years of AgeCalciumParticipants will receive 1 of 3 dose levels of romosozumab. All participants also received calcium and vitamin D.
Romosozumab: 12 - < 18 Years of AgeVitamin DParticipants will receive 1 of 3 dose levels of romosozumab. All participants also received calcium and vitamin D.
Romosozumab: 5 - < 12 Years of AgeRomosozumabParticipants will receive 1 of 3 dose levels of romosozumab. All participants also received calcium and vitamin D.
Romosozumab: 12 - < 18 Years of AgeRomosozumabParticipants will receive 1 of 3 dose levels of romosozumab. All participants also received calcium and vitamin D.
Primary Outcome Measures
NameTimeMethod
Time to Cmax (Tmax) of RomosozumabSingle blood samples were taken on days 1, 8, 15, 29, 57, 64, 71, 85, 113, and 169 (end of study); pre-specified PK analysis took place on Days 1 and 57

Median tmax values following Days 1 and 57 are presented.

Terminal Half-life of RomosozumabSingle blood samples were taken on days 1, 8, 15, 29, 57, 64, 71, 85, 113, and 169 (end of study); pre-specified PK analysis took place on Day 57

Median terminal half-life values at Day 57 are presented.

Maximum Observed Serum Concentration (Cmax) of RomosozumabSingle blood samples were taken on days 1, 8, 15, 29, 57, 64, 71, 85, 113, and 169 (end of study); pre-specified PK analysis took place on Days 1 and 57

Mean Cmax values following Days 1 and 57 are presented.

Area Under the Serum Concentration Time Curve (AUC) From Time 0 to Day 28 (AUC[0-28]) of RomosozumabSingle blood samples were taken on days 1, 8, 15, 29, 57, 64, 71, and 85; pre-specified PK analysis took place on Days 1 and 57

Mean AUC(0-28) values following Days 1 and 57 are presented.

Accumulation Ratio of RomosozumabSingle blood samples were taken on days 1, 8, 15, 29, 57, 64, 71, and 85; pre-specified PK analysis took place on Days 1 and 57

The accumulation ratio was calculated as AUC(0-28) at Day 57/AUC(0-28) at Day 1. Mean accumulation ratio values based on analysis at Days 1 and 57 are presented, as pre-specified.

Secondary Outcome Measures
NameTimeMethod
Percentage Change From Baseline in Bone Mineral Density (BMD) of the Lumbar SpineDXA scans were during screening (baseline) and at Day 85 and Day 169

BMD was assessed by dual energy X-ray absorptiometry (DXA) scans of the anteroposterior lumbar spine (L1 through L4) and analyzed by a central imaging laboratory. At least 2 lumbar vertebrae from L1 - L4 must be evaluable by DXA.

Number of Participants With Changes From Baseline in Cranial Nerve VII Examination Findings at Day 57, Day 85, and Day 169Baseline (Day 1), Day 57, Day 85, and Day 169

Facial nerve (cranial nerve VII) function was assessed clinically by facial symmetry inspection at rest, followed by assessment of the symmetry of specific facial movements: raising eyebrows, closing the eyes, blowing out the cheeks, smiling, pursing and closing the lips. Results of the cranial nerve examination were classed as 0 = Normal; 1 = Abnormal not clinically significant; and 2 = Abnormal clinically significant. An increase from baseline indicates an increase in abnormal clinical findings on the cranial nerve VII examination.

Number of Participants With Treatment-Emergent Adverse Events (TEAEs)Day 1 to end of study (up to Day 169); median duration on study was 5.55 months

TEAEs were adverse events (AEs) that started on or after first dose of investigational product up to the end of study (up to Day 169). Any clinically significant changes in vital signs, electrocardiogram parameters, physical exam findings, and clinical laboratory parameters were reported as TEAEs.

Injection site reactions were events of interest (EOI) for this study.

Percentage Change From Baseline in Serum Concentrations of Serum Type 1 Collagen C-Telopeptide (CTX)Blood samples were taken Days 1 (baseline), 8, 15, 29, 57, 64, 71, 85, 113, and 169

Serum concentrations of the bone turnover marker CTX were determined at pre-specified time points.

Number of Participants With Anti-romosozumab AntibodiesBlood samples for anti-romosozumab antibodies were taken Day 1, Day 15, Day 29, Day 85, and Day 169

Treatment-boosted anti-romosozumab antibody was defined as binding antibody positive at baseline with a \>4 x increase in magnitude post-baseline. Transient results were defined as negative results at the participant's last time point tested within the study period.

Percentage Change From Baseline in Serum Concentrations of Procollagen Type 1 N-terminal Propeptide (P1NP)Blood samples were taken Days 1 (baseline), 8, 15, 29, 57, 64, 71, 85, 113, and 169

Serum concentrations of the bone turnover marker P1NP were determined at pre-specified time points.

Percentage Change From Baseline in Bone Mineral Content (BMC) of the Lumbar SpineDXA scans were during screening (baseline) and at Day 85 and Day 169

BMC was assessed by DXA scans of the anteroposterior lumbar spine (L1 through L4) and analyzed by a central imaging laboratory. At least 2 lumbar vertebrae from L1 - L4 must be evaluable by DXA.

Percentage Change From Baseline in Lumbar Spine Bone AreaDXA scans were during screening (baseline) and at Day 85 and Day 169

Bone area was assessed by DXA scans of the anteroposterior lumbar spine (L1 through L4) and analyzed by a central imaging laboratory. At least 2 lumbar vertebrae from L1 - L4 must be evaluable by DXA.

Mean Change From Baseline in Lumbar Spine BMD Z-ScoreDXA scans were during screening (baseline) and at Day 85 and Day 169

Lumbar spine BMD was assessed by DXA scans. The results were then converted to Z-scores. The Z-score indicated the number of standard deviations away from the reference population and a score of 0 is equal to the mean. Positive changes from baseline indicated an improvement in lumbar spine BMD.

Trial Locations

Locations (15)

General Children Hospital Panagioti and Aglaias Kyriakou

🇬🇷

Athens, Greece

IRCCS Ospedale Pediatrico Bambino Gesu

🇮🇹

Roma, Italy

Koc Universitesi Hastanesi

🇹🇷

Istanbul, Turkey

Kepler Universitaetsklinikum GmbH

🇦🇹

Linz, Austria

Uniklinik Köln

🇩🇪

Koeln, Germany

Riley Hospital for Children

🇺🇸

Indianapolis, Indiana, United States

Hospital Universitario de Getafe

🇪🇸

Getafe, Madrid, Spain

Gazi Universitesi Tip Fakultesi

🇹🇷

Ankara, Turkey

Hospital de Cruces

🇪🇸

Baracaldo, País Vasco, Spain

Hospital Sant Joan de Deu

🇪🇸

Esplugues de Llobregat, Cataluña, Spain

Hospital Universitari i Politecnic La Fe

🇪🇸

Valencia, Comunidad Valenciana, Spain

Vanderbilt University Medical Center

🇺🇸

Nashville, Tennessee, United States

Semmelweis Egyetem

🇭🇺

Budapest, Hungary

The Medical College of Wisconsin

🇺🇸

Milwaukee, Wisconsin, United States

Ege Universitesi Ilac Gelistirme ve Farmakokinetik Arastirma Uygulama Merkezi (ARGEFAR)

🇹🇷

Izmir, Turkey

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