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AzTrials

Tumor Induced Osteomalacia (TIO)Phase 2NCT02304367

Study of Burosumab (KRN23) in Adults With Tumor-Induced Osteomalacia (TIO) or Epidermal Nevus Syndrome (ENS)

A Phase 2 study testing Burosumab in people with Tumor Induced Osteomalacia (TIO) and Epidermal Nevus Syndrome (ENS), run by Kyowa Kirin, Inc. at 7 sites in United States.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record
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Trial summary

The primary objectives of this study are to evaluate the effect of burosumab treatment on: Increasing serum phosphorus levels in adults with TIO or ENS-associated osteomalacia Improvement in TIO/ENS-associated osteomalacia as determined by osteoid thickness (O.Th), osteoid surface/bone surface (OS/BS), osteoid… Read more about this study

Who is it for?
Ages 18 years and older
What is being tested?
Burosumab
Type of treatment
Injection or infusion
Could I receive a placebo?
No, everyone receives an active treatment
How many people
17 participants
Where
7 sitesUnited States
Study dates
24 Mar 2015to 21 Jan 2021
Phase
Phase 2
Are there results?
Yes, posted in the registrySummary in the doctors' view

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 18 years and older
  • All sexes
  • No healthy volunteers
  • Adult, Older adult

Who may be able to join

  • Have a clinical diagnosis of TIO/ENS-associated osteomalacia based on evidence of excessive fibroblast growth factor 23 (FGF23) that was not amenable to cure by surgical excision of the underlying tumor/lesion (documented by Investigator).
  • Be ≥ 18 years of age
  • Have a fasting serum phosphorus level < 2.5 mg/dL
  • Have an FGF23 level ≥ 100 pg/mL by Kainos assay
  • Have a ratio of renal tubular maximum reabsorption rate of phosphate to glomerular filtration rate (TmP/GFR) < 2.5 mg/dL
  • Have an estimated glomerular filtration rate (eGFR) ≥ 60 mL/min (using Cockcroft-Gault formula). Subjects with eGFR ≥ 30 but < 60 mL/min will be considered eligible as long as in the opinion of the investigator the decline in renal function is not related to nephrocalcinosis.
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  • Have a corrected serum calcium level < 10.8 mg/dL
  • Females of child-bearing potential must have a negative urine pregnancy test at Screening and Baseline and be willing to have additional pregnancy tests during the study. Females considered not to be of childbearing potential include those who have not experienced menarche, are post-menopausal (defined as having no menses for at least 12 months without an alternative medical cause) or are permanently sterile due to total hysterectomy, bilateral salpingectomy, or bilateral oophorectomy.
  • Be willing to use 2 forms of effective methods of contraception while participating in the study (sexually active subjects) and for 12 weeks after last dose of study drug.
  • Be willing to provide access to prior medical records to determine eligibility including imaging, biochemical, and diagnostic, medical, and surgical history data
  • Provide written informed consent after the nature of the study has been explained, and prior to any research-related procedures
  • Be willing and able to complete all aspects of the study, adhere to the study visit schedule and comply with the assessments (in the opinion of the investigator)

Who cannot join

  • Have a prior diagnosis of human immunodeficiency virus (HIV), hepatitis B and/or hepatitis C
  • Have a history of recurrent infection, a predisposition to infection, or a known immunodeficiency
  • Are pregnant or breastfeeding at Screening or are planning to become pregnant (self or partner) at any time during the study
  • Have participated in an investigational drug or device trial within 30 days prior to Screening or are currently enrolled in another study of an investigational product or device
  • Have used a therapeutic monoclonal antibody (mAb), including KRN23, within 90 days prior to Screening or have a history of allergic or anaphylactic reactions to any mAb
  • Have or a have a history of any hypersensitivity to KRN23 excipients that, in the judgment of the investigator, places the subject at increased risk for adverse effects
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  • Have used a pharmacologic vitamin D metabolite or its analog (e.g., calcitriol, doxercalciferol, and paricalcitol), phosphate, or aluminum hydroxide antacids (e.g., Maalox® and Mylanta®) within 2 weeks prior to Screening or during the study
  • Have used medication to suppress parathyroid hormone (PTH) (e.g., Sensipar®, cinacalcet, calcimimetics) within 2 months prior to Screening
  • Have a history of malignancy within 5 years of study entry with the exception of phosphaturic mesenchymal tumors (PMTs) of the mixed connective tissue type or non-melanoma skin cancers such as basal cell skin cancer
  • Have donated blood or blood products within 60 days prior to Screening
  • Have a history of allergic reaction to or have shown adverse reactions to a tetracycline (e.g., tetracycline hydrochloride [HCl] and demeclocycline), benzodiazepines, fentanyl or lidocaine
  • Have any condition, which in the opinion of the investigator and sponsor, could present a concern for either subject safety or difficulty with data interpretation

Trial locations and contacts

7 sites in 1 country.

7 sites in 1 country

United States7 sites
  • Johns Hopkins University

    Baltimore, Maryland, 21224

  • Duke University

    Durham, North Carolina, 27710

  • Colorado Center for Bone Research at Panorama Orthopedics and Spine Center

    Golden, Colorado, 80401

  • Houston Methodist Research Institute

    Houston, Texas, 77030

  • Indiana University Hospital

    Indianapolis, Indiana, 46202

  • Yale University School of Medicine

    New Haven, Connecticut, 06510

  • Mayo Clinic

    Rochester, Minnesota, 55905

Other trials in United States

About this study

Official title: A Phase 2 Open-Label Trial to Assess the Efficacy and Safety of KRN23, an Antibody to FGF23, in Subjects With Tumor-Induced Osteomalacia (TIO) or Epidermal Nevus Syndrome (ENS)-Associated Osteomalacia

The primary objectives of this study are to evaluate the effect of burosumab treatment on:

  • Increasing serum phosphorus levels in adults with TIO or ENS-associated osteomalacia
  • Improvement in TIO/ENS-associated osteomalacia as determined by osteoid thickness (O.Th), osteoid surface/bone surface (OS/BS), osteoid volume/bone volume (OV/BV) and mineralization lag time (MLt).
Being tested
Burosumab
Keywords
TIO, ENS, FGF23, KRN23
Medical subject headings
Oncogenic osteomalacia, Nevus, Sebaceous of Jadassohn

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

  • BurosumabBiological

    Also called KRN23, Crysvita®, UX023

    Solution for subcutaneous injection

Study groups in NCT02304367
Study groupRoleWhat they receive
BurosumabExperimentalParticipants received burosumab at a starting dose of 0.3 mg/kg administered subcutaneously (SC) every 4 weeks (Q4W). Doses may have been titrated up to a maximum of 2.0 mg/kg every 2 weeks (Q2W) in order to achieve fasting peak serum phosphorus levels within the target range of 2.5 to 4.0 mg/dL.
Receives: Biological: Burosumab

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. Percentage of Participants Achieving Mean Serum Phosphorus Levels Above 2.5 mg/dL at the Mid-Point of the Dose Intervals Between Baseline and Week 24

    The percentage of participants achieving mean serum phosphorus levels above the lower limit of normal (LLN; 2.5 mg/dL [0.81 mmol/L]) at the mid-point of the dose interval (2 weeks after dosing), as averaged across dose cycles between Baseline and Week 24 (i.e. the average of serum phosphorus levels at Weeks 2, 6, 10, 14 and 22).

    Measured: Mid-point of each dose interval from Baseline to Week 24 (Weeks 2, 6, 10, 14 and 22 [there was no study visit at Week 18])

  2. Change From Baseline to Week 48 in Osteoid Thickness

    Histomorphometry of trans-iliac crest bone biopsies was assessed by a blinded, central reader.

    Osteoid thickness is the mean thickness of osteoid seams.

    Measured: Baseline, Week 48

  3. Change From Baseline to Week 48 in Osteoid Surface/Bone Surface (OS/BS)

    Histomorphometry of trans-iliac crest bone biopsies was assessed by a blinded, central reader.

    Osteoid surface/bone surface is expressed as the percentage of bone surface covered in osteoid.

    Measured: Baseline, Week 48

  4. Change From Baseline to Week 48 in Osteoid Volume/Bone Volume (OV/BV)

    Histomorphometry of trans-iliac crest bone biopsies was assessed by a blinded, central reader. Osteoid volume/bone volume is expressed as the percentage of a given volume of bone tissue that consists of unmineralized bone (osteoid).

    Measured: Baseline, Week 48

  5. Change From Baseline to Week 48 in Mineralization Lag Time (MLt)

    Mineralization lag time is a dynamic modeling parameter representing the mean time interval between the formation of osteoid and its subsequent mineralization which can be measured using histomorphometry with double tetracycline labeling.

    Mlt was calculated by dividing the osteoid width by the mineralizing apposition rate (MAR; the average rate at which new bone mineral is being added on any actively forming surface).

    If Mlt could not be calculated directly due to low tetracycline uptake, Mlt was imputed according to the following:

    Mlt = O.Th/(MAR*MS/OS), where O.Th = osteoid thickness, MAR is imputed as 0.3 μm/day, MS/OS=mineralizing surface/osteoid surface, each measured at the same visit.

    Measured: Baseline, Week 48

Secondary outcomes (44)
  1. Percentage of Participants Achieving Mean Serum Phosphorus Levels Above 2.5 mg/dL at the End of the Dose Intervals Between Baseline and Week 24

    The percentage of participants achieving mean serum phosphorus levels above the lower limit of normal (2.5 mg/dL [0.81 mmol/L]) at the end of the dose interval (4 weeks post-dose, prior to the next dose), as averaged across dose cycles between Baseline and Week 24 (i.e. the average of serum phosphorus levels at Weeks 4, 8, 12, 16, 20, and 24).

    Measured: End of each dose interval from Baseline to Week 24 (Weeks 4, 8, 12, 16, 20, and 24)

  2. Mean Change From Baseline in Serum Phosphorus Levels at the Mid-Point of the Dose Interval, as Averaged Across Dose Cycles Between Baseline and Week 24

    Mean change from Baseline to the mid-point of the dose interval (2 weeks after dosing) averaged across dose cycles between Baseline and Week 24 (i.e. the average of serum phosphorus levels at Weeks 2, 6, 10, 14 and 22).

    Measured: Baseline and the mid-point of each dose interval from Baseline to Week 24 (Weeks 2, 6, 10, 14 and 22)

  3. Percent Change From Baseline in Serum Phosphorus Levels at the Mid-Point of the Dose Interval, as Averaged Across Dose Cycles Between Baseline and Week 24

    Mean percent change from Baseline to the mid-point of the dose interval (2 weeks after dosing) averaged across dose cycles between Baseline and Week 24 (i.e. the average of serum phosphorus levels at Weeks 2, 6, 10, 14 and 22).

    Measured: Baseline and the mid-point of each dose interval from Baseline to Week 24 (Weeks 2, 6, 10, 14 and 22)

  4. Mean Change From Baseline in Serum Phosphorus Levels at the End of the Dosing Cycle, as Averaged Across Dose Cycles Between Baseline and Week 24

    Mean change from Baseline at the end of the dose interval (4 weeks post-dose, prior to the next dose) averaged across dose cycles between Baseline and Week 24 (i.e. the average of serum phosphorus levels at Weeks 4, 8, 12, 16, 20, and 24).

    Measured: Baseline and Weeks 4, 8, 12, 16, 20, and 24

  5. Percent Mean Change From Baseline in Serum Phosphorus Levels at the End of the Dosing Cycle, as Averaged Across Dose Cycles Between Baseline and Week 24

    Mean percent change from Baseline at the end of the dose interval (4 weeks post-dose, prior to the next dose) averaged across dose cycles between Baseline and Week 24 (i.e. the average of serum phosphorus levels at Weeks 4, 8, 12, 16, 20, and 24).

    Measured: Baseline and Weeks 4, 8, 12, 16, 20, and 24

  6. Time-Adjusted Area Under the Curve (AUC) of Serum Phosphorus Levels Between Baseline and Week 24

    Serum phosphorus level versus time AUC was calculated using the trapezoidal rule. Time-adjusted AUC was calculated by dividing the AUC by duration of time included in AUC calculation.

    Measured: Pre-dose on Day 1 and at Weeks 1, 2, 4, 6, 8, 10, 12, 14, 16, 20, 21, 22, and 24

  7. Change From Baseline Over Time in Serum 1,25-dihydroxyvitamin D (1,25(OH)2D) Concentration

    Least squares (LS) means and standard errors (SE) were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline 1,25(OH)2D, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  8. Change From Baseline Over Time in Total Serum Fibroblast Growth Factor 23 (FGF23) Concentration

    Total FGF23 included free FGF23 and FGF23 bound to burosumab. Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline total FGF23, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  9. Change From Baseline Over Time in Free Serum Fibroblast Growth Factor 23 (FGF23) Concentration

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline free FGF23, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  10. Change From Baseline Over Time in 24-hour Urinary Phosphorus

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline 24-hour urinary phosphorus, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  11. Change From Baseline Over Time in Tubular Reabsorption of Phosphate (TRP)

    Tubular reabsorption of phosphate (TRP) is the fraction of filtered phosphorus that is reabsorbed by renal tubules.

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline TRP, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  12. Change From Baseline Over Time in Ratio of Renal Tubular Maximum Phosphate Reabsorption Rate to Glomerular Filtration Rate (TmP/GFR)

    TmP/GFR measures renal phosphate reabsorption (the primary mechanism by which FGF23 regulates phosphate homeostasis) by comparing the fractional absorption of phosphate relative to the estimated rate of glomerular filtration.

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline TmP/GFR, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  13. Change From Baseline Over Time in Fractional Excretion of Phosphorus (FEP)

    FEP is the percentage of phosphorus filtered by the kidney that is excreted into urine, calculated as 100% * (2-hour urine phosphorus*serum creatinine)/(2-hour urine creatinine * serum phosphorus).

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline FEP, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  14. Change From Baseline Over Time in Serum Alkaline Phosphatase (ALP)

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline ALP, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  15. Change From Baseline Over Time in Bone-Specific Alkaline Phosphatase (BALP)

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline BALP, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  16. Percent Change From Baseline Over Time in BALP

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline BALP, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  17. Change From Baseline Over Time in Carboxy Terminal Cross-Linked Telopeptide of Type 1 Collagen (CTx)

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline CTx, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  18. Percent Change From Baseline Over Time in CTx

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline CTx, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  19. Change From Baseline Over Time in Procollagen Type 1 N-Propeptide (P1NP)

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline P1NP, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  20. Percent Change From Baseline Over Time in P1NP

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline P1NP, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  21. Change From Baseline Over Time in Osteocalcin

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline osteocalcin, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  22. Percent Change From Baseline Over Time in Osteocalcin

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline osteocalcin, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  23. Change From Baseline Over Time in Hand-Held Dynamometry (HHD) Elbow Measurements

    To assess muscle strength, hand-held dynamometry was conducted using a standardized technique. Bilateral strength (defined as the average of the left and the right scores, measured in kilograms) of the elbow flexors and extensors was measured by the maximum voluntary isometric contraction against a dynamometer.

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline strength measurements, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24 and 48

  24. Change From Baseline Over Time in Hand-Held Dynamometry (HHD) Knee Measurements

    To assess muscle strength, hand-held dynamometry was conducted using a standardized technique. Bilateral strength (defined as the average of the left and the right scores, measured in kilograms) of the knee flexors and extensors was measured by the maximum voluntary isometric contraction against a dynamometer.

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline strength measurements, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24 and 48

  25. Change From Baseline Over Time in Sit-to-Stand (STS) Test

    The STS test measures lower extremity strength and mobility as a participant moves repeatedly from a seated position to standing. This study used a modified STS test that allowed participants to use the arms of the chair to help them stand and sit if necessary. The number of sit-to-stand repetitions performed in a 30-second period was recorded.

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline measurement, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24 and 48

  26. Change From Baseline Over Time in Weighted Arm Lift (WAL) Test

    The Weighted arm lift (WAL) test assesses upper extremity strength, mobility and reaching ability. The test was administered bilaterally to determine the number of times the participant could raise a 1 kg weight above the head in a 30-second period. The number of repetitions completed with each arm was recorded.

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline WAL measurements, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24 and 48

  27. Change From Baseline Over Time in Six-Minute Walk Test (6MWT)

    The 6MWT is a commonly used measure of mobility and was conducted in accordance with general principles set forth in the American Thoracic Society guidelines (ATS 2002). Participants were instructed to walk the length of a pre-measured course for 6 consecutive minutes (assistive devices could be used). The total distance walked at the end of 6 minutes was recorded in meters.

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline 6MWT measurement, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24 and 48

  28. Change From Baseline Over Time in Brief Pain Inventory (BPI) Worst Pain Score

    The Brief Pain Inventory (BPI) is a self-reported, pain-specific questionnaire with a recall period of 24 hours. Worst pain is defined as the answer to Question 3, in which participants rated their pain at its worst in the last 24 hours on a scale from 0 (no pain) to 10 (pain as bad as you can imagine).

    A negative change from Baseline score indicates improvement.

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline BPI measurement, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  29. Change From Baseline Over Time in Brief Pain Inventory (BPI) Pain Severity Score

    The Brief Pain Inventory (BPI) is a self-reported, pain-specific questionnaire with a recall period of 24 hours.

    Pain severity is defined as the average of 4 questions (Questions 3 through 6) assessing worst pain, least pain, average pain, and pain right now, rated on a scale from 0 (no pain) to 10 (pain as bad as you can imagine). Mild pain is defined as a score of 1 to 4, moderate pain is defined as a score of 5 to 6, and severe pain is defined as a score of 7 to 10. A negative change from Baseline score indicates improvement.

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline BPI measurement, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  30. Change From Baseline Over Time in Brief Pain Inventory (BPI) Pain Interference Score

    The Brief Pain Inventory (BPI) is a self-reported, pain-specific questionnaire with a recall period of 24 hours.

    Pain interference is defined as the average of 7 questions (9A through 9G) regarding the extent to which pain interfered with daily activities, including general activity, mood, walking ability, normal work, relations with other people, sleep, and enjoyment of life in the last 24 hours, rated on a scale from 0 (does not interfere) to 10 (completely interferes).

    A negative change from Baseline score indicates improvement. Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline BPI measurement, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  31. Change From Baseline Over Time in Brief Fatigue Inventory (BFI) Worst Fatigue Score

    The Brief Fatigue Inventory (BFI) is a self-reported questionnaire consisting of 9 items related to fatigue that are rated on a 0 to 10 numerical rating scale with a recall period of 24 hours.

    Worst fatigue is defined as the answer to Question 3 in which participants rated their fatigue at its worst in the last 24 hours on a scale from 0 (no fatigue) to 10 (as bad as you can imagine).

    A negative change from Baseline score indicates improvement. Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline BFI measurement, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  32. Change From Baseline Over Time in Brief Fatigue Inventory (BFI) Fatigue Severity Score

    The Brief Fatigue Inventory (BFI) is a self-reported questionnaire consisting of 9 items related to fatigue that are rated on a 0 to 10 numerical rating scale with a recall period of 24 hours.

    Fatigue severity is defined as the average of 3 questions (Questions 1 through 3) assessing fatigue right now, usual level of fatigue, and worst fatigue, rated on a scale from 0 (no fatigue) to 10 (as bad as you can imagine).

    A negative change from Baseline score indicates improvement.

    Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline BFI measurement, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  33. Change From Baseline Over Time in Brief Fatigue Inventory (BFI) Fatigue Interference Score

    The Brief Fatigue Inventory (BFI) is a self-reported questionnaire consisting of 9 items related to fatigue that are rated on a 0 to 10 numerical rating scale with a recall period of 24 hours.

    The fatigue interference score is defined as the average of 6 questions (Questions 4A through 4F) regarding the extent to which fatigue interfered with daily activities, including general activity, mood, walking ability, work, relations with others, and enjoyment of life in the last 24 hours, rated on a scale from 0 (does not interfere) to 10 (completely interferes).

    A negative change from Baseline score indicates improvement. Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline BFI measurement, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  34. Change From Baseline Over Time in Brief Fatigue Inventory (BFI) Global Fatigue Score

    The Brief Fatigue Inventory (BFI) is a self-reported questionnaire consisting of 9 items related to fatigue that are rated on a 0 to 10 numerical rating scale with a recall period of 24 hours. The first 3 questions measure fatigue severity and the next 6 questions assess the impact of fatigue on daily activities.

    The global fatigue score is defined as the average of all 9 questions on the BFI including severity and interference, and ranges from from 0 to 10, where higher scores correspond to greater levels of fatigue.

    A negative change from Baseline score indicates improvement. Least squares means and standard errors were estimated from a generalized estimation equation (GEE) model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline BFI measurement, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  35. Change From Baseline Over Time in 36-Item Short Form Health Survey (SF-36) Physical Component Summary Score

    The SF-36 v2 is a self-administered questionnaire that measures the impact of disease on overall quality of life during the past 4 weeks. The SF-36 consists of 36 questions in eight domains (physical function, pain, general and mental health, vitality, social function, physical and emotional health).

    The physical component summary score (PCS) is a weighted combination of the 8 subscales with positive weighting for physical functioning, role-physical, bodily pain, and general health. The PCS was calculated using norm-based scoring so that 50 is the average score and the standard deviation equals 10. Higher scores are associated with better functioning/quality of life; a positive change from Baseline score indicates an improvement.

    LS means and SEs were estimated from a GEE model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline SF-36, with compound symmetry covariance struct

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  36. Change From Baseline Over Time in 36-Item Short Form Health Survey (SF-36) Physical Functioning Domain Score

    The SF-36 v2 is a self-administered questionnaire that measures the impact of disease on overall quality of life during the past 4 weeks. The SF-36 consists of 36 questions in eight domains (physical function, pain, general and mental health, vitality, social function, physical and emotional health).

    The physical functioning (PF) domain includes 10 questions that assess limitations in physical activities because of health problems. The PF domain score was calculated using norm-based scoring so that 50 is the average score and the standard deviation equals 10. Higher scores are associated with better functioning/quality of life; a positive change from Baseline score indicates an improvement.

    LS means and SEs were estimated from a GEE model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline SF-36, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  37. Change From Baseline Over Time in 36-Item Short Form Health Survey (SF-36) Role Physical Domain Score

    The SF-36 v2 is a self-administered questionnaire that measures the impact of disease on overall quality of life during the past 4 weeks. The SF-36 consists of 36 questions in eight domains (physical function, pain, general and mental health, vitality, social function, physical and emotional health).

    The role-physical (RP) domain includes 4 questions that assess limitations in usual role activities because of physical health problems. The RP domain score was calculated using norm-based scoring so that 50 is the average score and the standard deviation equals 10. Higher scores are associated with better functioning/quality of life; a positive change from Baseline score indicates an improvement.

    LS means and SEs were estimated from a GEE model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline SF-36, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  38. Change From Baseline Over Time in 36-Item Short Form Health Survey (SF-36) Bodily Pain Domain Score

    The SF-36 v2 is a self-administered questionnaire that measures the impact of disease on overall quality of life during the past 4 weeks. The SF-36 consists of 36 questions in eight domains (physical function, pain, general and mental health, vitality, social function, physical and emotional health).

    The bodily pain (BP) domain includes 2 questions that assess pain level and impact of pain on normal work. The BP domain score was calculated using norm-based scoring so that 50 is the average score and the standard deviation equals 10. Higher scores are associated with better functioning/quality of life; a positive change from Baseline score indicates an improvement.

    LS means and SEs were estimated from a GEE model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline SF-36, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  39. Change From Baseline Over Time in 36-Item Short Form Health Survey (SF-36) General Health Perceptions Domain Score

    The SF-36 v2 is a self-administered questionnaire that measures the impact of disease on overall quality of life during the past 4 weeks. The SF-36 consists of 36 questions in eight domains (physical function, pain, general and mental health, vitality, social function, physical and emotional health).

    The general health perceptions (GH) domain includes 5 questions that assess participants' perception of their own general health. The GH domain score was calculated using norm-based scoring so that 50 is the average score and the standard deviation equals 10. Higher scores are associated with better functioning/quality of life; a positive change from Baseline score indicates an improvement.

    LS means and SEs were estimated from a GEE model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline SF-36, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  40. Change From Baseline Over Time in 36-Item Short Form Health Survey (SF-36) Mental Component Summary Score

    The SF-36 v2 is a self-administered questionnaire that measures the impact of disease on overall quality of life during the past 4 weeks. The SF-36 consists of 36 questions in eight domains (physical function, pain, general and mental health, vitality, social function, physical and emotional health).

    The mental component summary score (MCS) is a weighted combination of the 8 subscales with positive weighting for vitality, social function, role limitations due to emotional problems, and mental health. The MCS was calculated using norm-based scoring so that 50 is the average score and the standard deviation equals 10. Higher scores are associated with better functioning/quality of life; a positive change from Baseline indicates an improvement.

    LS means and SEs were estimated from a GEE model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline SF-36, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  41. Change From Baseline Over Time in 36-Item Short Form Health Survey (SF-36) Vitality Domain Score

    The SF-36 v2 is a self-administered questionnaire that measures the impact of disease on overall quality of life during the past 4 weeks. The SF-36 consists of 36 questions in eight domains (physical function, pain, general and mental health, vitality, social function, physical and emotional health).

    The vitality (VT) domain includes 4 questions that assess energy levels and fatigue. The VT domain score was calculated using norm-based scoring so that 50 is the average score and the standard deviation equals 10. Higher scores are associated with better functioning/quality of life; a positive change from Baseline score indicates an improvement.

    LS means and SEs were estimated from a GEE model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline SF-36, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  42. Change From Baseline Over Time in 36-Item Short Form Health Survey (SF-36) Social Functioning Domain Score

    The SF-36 v2 is a self-administered questionnaire that measures the impact of disease on overall quality of life during the past 4 weeks. The SF-36 consists of 36 questions in eight domains (physical function, pain, general and mental health, vitality, social function, physical and emotional health).

    The social functioning (SF) domain includes 2 questions that assess limitations in social activities because of physical health or emotional problems. The SF domain score was calculated using norm-based scoring so that 50 is the average score and the standard deviation equals 10. Higher scores are associated with better functioning/quality of life; a positive change from Baseline score indicates an improvement.

    LS means and SEs were estimated from a GEE model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline SF-36, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  43. Change From Baseline Over Time in 36-Item Short Form Health Survey (SF-36) Role Emotional Domain Score

    The SF-36 v2 is a self-administered questionnaire that measures the impact of disease on overall quality of life during the past 4 weeks. The SF-36 consists of 36 questions in eight domains (physical function, pain, general and mental health, vitality, social function, physical and emotional health).

    The role-emotional (RE) domain includes 3 questions that assess limitations in usual role activities because of emotional problems. The RE domain score was calculated using norm-based scoring so that 50 is the average score and the standard deviation equals 10. Higher scores are associated with better functioning/quality of life; a positive change from Baseline score indicates an improvement.

    LS means and SEs were estimated from a GEE model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline SF-36, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

  44. Change From Baseline Over Time in 36-Item Short Form Health Survey (SF-36) Mental Health Domain Score

    The SF-36 v2 is a self-administered questionnaire that measures the impact of disease on overall quality of life during the past 4 weeks. The SF-36 consists of 36 questions in eight domains (physical function, pain, general and mental health, vitality, social function, physical and emotional health).

    The mental health (MH) domain includes 5 questions that assess general mental health (psychological distress and well-being). The MH domain score was calculated using norm-based scoring so that 50 is the average score and the standard deviation equals 10. Higher scores are associated with better functioning/quality of life; a positive change from Baseline score indicates an improvement.

    LS means and SEs were estimated from a GEE model which includes the change from Baseline as the dependent variable, time as the categorical variable and adjusted for Baseline SF-36, with compound symmetry covariance structure.

    Measured: Baseline and Weeks 24, 48, 96, 144, 168, 192, 216, and 240

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted1 Dec 2014 (estimated)
  2. Study start24 Mar 2015
  3. Primary completion27 Jul 2017
  4. Study completion21 Jan 2021

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Interventional
Phase
Phase 2
Primary purpose
Treatment
Allocation
Not applicable
Intervention model
Single group
Masking
None (open label)
Enrolment
17 participants (actual)
IDs, dates and publications
ClinicalTrials.gov ID
NCT02304367
Sponsor's protocol ID
UX023T-CL201
Status last verified
May 2024
First submitted
24 Nov 2014
Last updated
6 May 2024
Data monitoring committee
No
Expanded access
Available

Publications

  • Khadora M, Mughal MZ. Burosumab treatment in a child with cutaneous skeletal hypophosphatemia syndrome: A case report. Bone Rep. 2021 Oct 1;15:101138. doi: 10.1016/j.bonr.2021.101138. eCollection 2021 Dec.

    PubMed 34660853

Questions about this trial

What is this clinical trial studying?

A Phase 2 study testing Burosumab in people with Tumor Induced Osteomalacia (TIO) and Epidermal Nevus Syndrome (ENS), run by Kyowa Kirin, Inc. at 7 sites in United States.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 12 inclusion and 12 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: finished, as of the sponsor's last update on 6 May 2024. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 7 sites in United States. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

What does this phase mean?

Phase 2 studies give the treatment to a larger group to see whether it has the intended effect and to keep checking safety.

Not every study follows the same path, and some studies (observational studies, device studies) have no phase.

Are results available for this study?

Yes. Results are posted in the registry (first posted 30 Jul 2020). A summary is in the For doctors view, and the full results are on ClinicalTrials.gov.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Percentage of Participants Achieving Mean Serum Phosphorus Levels Above 2.5 mg/dL at the Mid-Point of the Dose Intervals Between Baseline and Week 24 (time frame: Mid-point of each dose interval from Baseline to Week 24 (Weeks 2, 6, 10, 14 and 22 [there was no study visit at Week 18])). The study lists 5 primary outcomes in all. It is what the study was designed to measure, not a finding.

Where can I read the original study information?

On the ClinicalTrials.gov record for NCT02304367, which also lists 1 linked publication.

Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 6 May 2024.

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