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Cystic FibrosisNCT06216704

Skeletal Health and Bone Marrow Composition in Adolescents With Cystic Fibrosis

An observational study of Cystic Fibrosis, run by Massachusetts General Hospital at 1 site in United States.

Recruiting

Looking for participants now. Contact the study team or a site below to ask about taking part.

Sites and contacts
Official record
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Trial summary

The investigators will be evaluating bone marrow composition via magnetic resonance imaging in adolescents diagnosed with cystic fibrosis (CF) compared to healthy, matched controls. Read more about this study

Who is it for?
Ages 13 to 20 yearsHealthy volunteers can take part
Conditions the study is for
Cystic Fibrosis
What is being tested?
Magnetic resonance relaxometry, Magnetic resonance spectroscopy and Blood Draw
How many people
36 participants
Where
1 siteUnited States
Study dates
1 Apr 2024to 30 Jun 2029
Phase
Observational
Are there results?
Not yet: the study is under way

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 13 to 20 years
  • All sexes
  • Healthy volunteers accepted
  • Child, Adult

Study population: The experimental group will be adolescents aged 13-20 with CF. The control group will be matched for sex, ancestry, age (within 2 years), and pubertal stage (based on Tanner staging, ± 1 Tanner stage).

Who may be able to join

  • 13-20 years old
  • Cystic fibrosis with pancreatic insufficiency
  • Must have a stable treatment regimen, including CFTR modulator usage unchanged for the prior three months
  • Liver transplant recipients will be eligible, as long as they are at least 1 year post-transplant and are no longer on Prednisone for immunosuppressive therapy

Who cannot join

  • Diagnosis of other chronic disease affecting bone health
  • Active use (within the past 3 months) of medications that are known to affect skeletal metabolism
  • CF exacerbation or glucocorticoid exposure within the prior 1 month
  • Lung transplant

Trial locations and contacts

1 site in 1 country. Contact the study team or a site near you to ask about taking part.

United States1 site, 1 recruiting
  • Boston Children's Hospital

    Boston, Massachusetts, 02115

    Rebecca Gordon, MD, rebecca.gordon@childrens.harvard.edu

    Rebecca Gordon, MD

    Recruiting

Other trials in United States

About this study

The investigators will be evaluating bone marrow composition via magnetic resonance imaging in adolescents diagnosed with cystic fibrosis (CF) compared to healthy, matched controls. The investigators will also be assessing their bone mineral density via other imaging modalities, including dual-energy X-ray absorptiometry (DXA) and peripheral quantitative computed tomography (pQCT). This longitudinal project will focus on abnormalities in bone marrow composition, and specifically whether adolescents with diagnosed with CF exhibit increased bone marrow fat, its association with bone mineral density (BMD) and the underlying pathophysiology, including glycemic control, inflammation, and bone turnover markers.

Read the detailed description

Less than optimal bone health has been seen in children that have cystic fibrosis (CF). This can present as low bone density or altered bone structure, weakening the bones and increasing fragility and fracture risk. As adolescence is especially important in bone development, conditions such as CF during this time can lead to long term bone issues. The underlying mechanisms are not well understood, but what is known is that red bone marrow converts to fat-rich yellow marrow. This study aims to focus on abnormalities in bone marrow, and specifically whether adolescents who have been diagnosed with CF have more bone marrow fat.

The primary hypothesis is that patients with CF will have associated increased fat levels in bone marrow, which will be associated with decreased bone formation and suboptimal bone health. The central objective is to obtain longitudinal data on the differences in bone marrow between patients with CF versus healthy adolescents. Long term, the investigators want to study how abnormal marrow fat and suboptimal bone health relate to one another.

The study involves 36 adolescents diagnosed with CF and 36 matched healthy controls. Eligibility criteria include no other chronic diseases that affect bone health and limited use of bone altering medications in the prior three months. The adolescents with CF will be matched with healthy adolescents based on sex, ancestry, age, and pubertal stage. Additional data on participants with CF will be collected via a chart review that will enable us to more fully characterize their CF.

Imaging will include: MRI of the knee with quantitative marrow fat assessment; dual-energy X-ray absorptiometry (DXA); and peripheral quantitative computed tomography (pQCT). All scans will be for research purposes only. The MRIs will be evaluated for any incidental findings, and if any identified, it will be reported to their primary care physician.

Additionally, blood draws will be used to assess markers of bone formation/resorption and inflammation. In participants with CF, they will have a continuous glucose monitor to assess dysglycemia. All participants will also complete questionnaires.

There will be a baseline visit, and then a follow up visit 1 year later, with identical study procedures at both visits.

Conditions
Cystic Fibrosis
Being tested
Magnetic resonance relaxometry, Magnetic resonance spectroscopy, Blood Draw, DXA, pQCT
Keywords
Cystic Fibrosis, Bone Marrow, Dual-energy X-ray absorptiometry, Peripheral Quantitative Computed Tomography, Magnetic Resonance Imaging, pediatrics, bone health
Medical subject headings
Cystic Fibrosis

What participants receive

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

  • Magnetic resonance relaxometryDiagnostic test

    Spin-lattice relaxation (T1) relaxometry acquisition consisting of fast spin echo (FSE) acquisitions through the knee. T1 maps from the T1 relaxometry images will be generated using a two-parameter-fit iterative algorithm developed in-house using IDL software (Harris Geospatial Solutions, Melbourne, FL, USA). Mean T1 values for each region will be recorded. The anatomical locations of these regions will be consistent in size for all subjects and location. The locations chosen for the primary endpoints are ones that are known to be rich in red and yellow marrow, respectively.

  • Magnetic resonance spectroscopyDiagnostic test

    Magnetic resonance spectroscopy. MRS will be performed within a 1 mL voxel situated in the medial aspect of the distal femoral metaphysis. A single voxel point resolved spectral acquisition (PRESS) technique will be used to acquire non-water suppressed spectra at multiple echo times. Spectral fits using JMRUI MRS processing software (www.jmrui.eu) to the water and methylene/methyl resonances will be used to quantify peak areas and establish T2 corrected fat/(fat + water) ratios.

  • Blood DrawDiagnostic test

    Blood draw. Blood draws will be used to attain and assess markers of bone formation/resorption and inflammation. Specific markers of bone formation that will be assessed include osteocalcin (OC) and procollagen type 1 N-terminal propeptide (P1NP), and a marker of bone resorption, c-telopeptide (CTX). Additionally, in participants with CF, we will assess inflammation, with a c-reactive protein (CRP), and dysglycemia, with a continuous glucose monitor.

  • DXADiagnostic test

    DXA will be utilized to obtain BMD of the total body, lumbar spine, and hip using a Hologic Horizon densitometer (Hologic Inc, Bedford, MA). Body composition will be obtained from total body scans.

  • pQCTDiagnostic test

    pQCT will be utilized to obtain volumetric BMD (mg/cm3) of the left tibia. Measurements using a Stratec XCT 3000 device (Orthometrix, White Plains, NY) will be obtained at multiple locations, in relation to distal growth plate.

Study groups in NCT06216704
Study groupRoleWhat they receive
Cystic FibrosisThis group will be 36 adolescents, ages 13-20 years old, who have been diagnosed with cystic fibrosis. All participants will have a two study visits approximately one year apart during which the listed diagnostic testing will be performed.
Receives: Diagnostic Test: Magnetic resonance relaxometry; Diagnostic Test: Magnetic resonance spectroscopy; Diagnostic Test: Blood Draw; Diagnostic Test: DXA; Diagnostic Test: pQCT
ControlControls will be matched for age, Tanner staging, BMI percentile, and ancestry. All participants will have a two study visits approximately one year apart during which the listed diagnostic testing will be performed.
Receives: Diagnostic Test: Magnetic resonance relaxometry; Diagnostic Test: Magnetic resonance spectroscopy; Diagnostic Test: Blood Draw; Diagnostic Test: DXA; Diagnostic Test: pQCT

What is being measured

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

  1. Bone marrow adiposity by magnetic resonance relaxometry (MR relaxometry)

    Change in bone marrow adiposity measured by MR relaxometry

    Measured: Baseline and One Year follow-up

  2. Bone marrow adiposity by magnetic resonance spectroscopy (MRS)

    Change in bone marrow adiposity measured by MRS

    Measured: Baseline and One Year follow-up

Secondary outcomes (8)
  1. Total body bone mineral density Z-score by Dual-energy X-ray absorptiometry (DXA)

    Change in total body less head BMD Z-score

    Measured: Baseline and One Year follow-up

  2. Spine BMD Z-score by DXA

    Change in lumbar spine BMD Z-score

    Measured: Baseline and One Year follow-up

  3. Hip BMD Z-score by DXA

    Change in hip BMD Z-score

    Measured: Baseline and One year follow-up

  4. Volumetric bone mineral density (vBMD)

    Change in quantitative computed tomography (pQCT) scans will be obtained at the left tibia

    Measured: Baseline and One Year follow-up

  5. polar strength strain index

    Change in pQCT bone strength measure

    Measured: Baseline and One Year follow-up

  6. osteocalcin

    Change in bone formation assessed by osteocalcin (ng/mL)

    Measured: Baseline and One Year follow-up

  7. procollagen type 1 N-terminal propeptide

    Change in bone formation assessed by procollagen type 1 N-terminal propeptide (ng/mL)

    Measured: Baseline and One Year follow-up

  8. c-telopeptide

    Change in bone resorption assessed by c-telopeptide (pg/ml)

    Measured: Baseline and One Year follow-up

Timeline

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

  1. First posted22 Jan 2024
  2. Study start1 Apr 2024
  3. Primary completion31 Dec 2028 (estimated)
  4. Study completion30 Jun 2029 (estimated)

Resources

More details

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

Study design
Study type
Observational
Observational model
Case-control
Time perspective
Prospective
Enrolment
36 participants (estimated)
IDs, dates and publications
ClinicalTrials.gov ID
NCT06216704
Sponsor's protocol ID
IRB-P00047144
Other IDs
005960A123
Status last verified
Oct 2025
First submitted
11 Jan 2024
Last updated
30 Oct 2025
Results posted
No
Data monitoring committee
No
FDA-regulated drug
No
FDA-regulated device
No
Individual data sharing
No

Publications

  • Ullal J, Kutney K, Williams KM, Weber DR. Treatment of cystic fibrosis related bone disease. J Clin Transl Endocrinol. 2021 Dec 21;27:100291. doi: 10.1016/j.jcte.2021.100291. eCollection 2022 Mar.

    PubMed 35059303
  • Putman MS, Anabtawi A, Le T, Tangpricha V, Sermet-Gaudelus I. Cystic fibrosis bone disease treatment: Current knowledge and future directions. J Cyst Fibros. 2019 Oct;18 Suppl 2:S56-S65. doi: 10.1016/j.jcf.2019.08.017.

    PubMed 31679730
  • Weber DR, Gordon RJ, Kelley JC, Leonard MB, Willi SM, Hatch-Stein J, Kelly A, Kosacci O, Kucheruk O, Kaafarani M, Zemel BS. Poor Glycemic Control Is Associated With Impaired Bone Accrual in the Year Following a Diagnosis of Type 1 Diabetes. J Clin Endocrinol Metab. 2019 Oct 1;104(10):4511-4520. doi: 10.1210/jc.2019-00035.

    PubMed 31034056
  • Viswanathan A, Sylvester FA. Chronic pediatric inflammatory diseases: effects on bone. Rev Endocr Metab Disord. 2008 Jun;9(2):107-22. doi: 10.1007/s11154-007-9070-0. Epub 2007 Dec 29.

    PubMed 18165904
  • Gordon RJ, Pappa HM, Vajapeyam S, Mulkern R, Ecklund K, Snapper SB, Gordon CM. Bone marrow adiposity in pediatric Crohn's disease. Bone. 2022 Sep;162:116453. doi: 10.1016/j.bone.2022.116453. Epub 2022 Jun 3.

    PubMed 35667602
  • Vajapeyam S, Ecklund K, Mulkern RV, Feldman HA, O'Donnell JM, DiVasta AD, Rosen CJ, Gordon CM. Magnetic resonance imaging and spectroscopy evidence of efficacy for adrenal and gonadal hormone replacement therapy in anorexia nervosa. Bone. 2018 May;110:335-342. doi: 10.1016/j.bone.2018.02.021. Epub 2018 Feb 26.

    PubMed 29496516
  • Ecklund K, Vajapeyam S, Mulkern RV, Feldman HA, O'Donnell JM, DiVasta AD, Gordon CM. Bone marrow fat content in 70 adolescent girls with anorexia nervosa: Magnetic resonance imaging and magnetic resonance spectroscopy assessment. Pediatr Radiol. 2017 Jul;47(8):952-962. doi: 10.1007/s00247-017-3856-3. Epub 2017 Apr 22.

    PubMed 28432403
  • Ecklund K, Vajapeyam S, Feldman HA, Buzney CD, Mulkern RV, Kleinman PK, Rosen CJ, Gordon CM. Bone marrow changes in adolescent girls with anorexia nervosa. J Bone Miner Res. 2010 Feb;25(2):298-304. doi: 10.1359/jbmr.090805.

    PubMed 19653811
  • Hu L, Yin C, Zhao F, Ali A, Ma J, Qian A. Mesenchymal Stem Cells: Cell Fate Decision to Osteoblast or Adipocyte and Application in Osteoporosis Treatment. Int J Mol Sci. 2018 Jan 25;19(2):360. doi: 10.3390/ijms19020360.

    PubMed 29370110
  • Karampinos DC, Ruschke S, Gordijenko O, Grande Garcia E, Kooijman H, Burgkart R, Rummeny EJ, Bauer JS, Baum T. Association of MRS-Based Vertebral Bone Marrow Fat Fraction with Bone Strength in a Human In Vitro Model. J Osteoporos. 2015;2015:152349. doi: 10.1155/2015/152349. Epub 2015 Apr 19.

    PubMed 25969766
  • Schellinger D, Lin CS, Lim J, Hatipoglu HG, Pezzullo JC, Singer AJ. Bone marrow fat and bone mineral density on proton MR spectroscopy and dual-energy X-ray absorptiometry: their ratio as a new indicator of bone weakening. AJR Am J Roentgenol. 2004 Dec;183(6):1761-5. doi: 10.2214/ajr.183.6.01831761.

    PubMed 15547224
  • Moore SG, Dawson KL. Red and yellow marrow in the femur: age-related changes in appearance at MR imaging. Radiology. 1990 Apr;175(1):219-23. doi: 10.1148/radiology.175.1.2315484.

    PubMed 2315484
  • Javier RM, Jacquot J. Bone disease in cystic fibrosis: what's new? Joint Bone Spine. 2011 Oct;78(5):445-50. doi: 10.1016/j.jbspin.2010.11.015. Epub 2011 Jan 12.

    PubMed 21233000
  • Conway SP. Impact of lung inflammation on bone metabolism in adolescents with cystic fibrosis. Paediatr Respir Rev. 2001 Dec;2(4):324-31. doi: 10.1053/prrv.2001.0167.

    PubMed 12052304
  • Tian X, Cong F, Guo H, Fan J, Chao G, Song T. Downregulation of Bach1 protects osteoblasts against hydrogen peroxide-induced oxidative damage in vitro by enhancing the activation of Nrf2/ARE signaling. Chem Biol Interact. 2019 Aug 25;309:108706. doi: 10.1016/j.cbi.2019.06.019. Epub 2019 Jun 11.

    PubMed 31194955

Questions about this trial

What is this clinical trial studying?

An observational study of Cystic Fibrosis, run by Massachusetts General Hospital at 1 site 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 4 inclusion and 4 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: looking for participants now, as of the sponsor's last update on 30 Oct 2025. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 1 site 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.

Are results available for this study?

This study is still under way. Results are not available in the source record yet.

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

Contact Rebecca Gordon, MD ((617) 355-7476, rebecca.gordon@childrens.harvard.edu), or a site near the patient. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Bone marrow adiposity by magnetic resonance relaxometry (MR relaxometry) (time frame: Baseline and One Year follow-up). The study lists 2 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 NCT06216704, which also lists 26 linked publications.

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 30 Oct 2025.

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