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Main Study Up to 100 subjects, both non-CF volunteers and Cystic Fibrosis (CF) patients, will participate in a single study visit that will include a DEXA scan, micro CT, and blood collection.
Denosumab (Prolia) Sub study Approximately 10 adult subjects with CF who participated in the main study and have results indicating bone disease will receive treatment with Denosumab for up to 5 years. They will be asked to return for repeat DEXA scans, micro CT, blood collection, and documenting of any adverse events.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Main Study | No Intervention | Up to 100 subjects, both non-CF volunteers and Cystic Fibrosis (CF) patients, will participate in a single study visit that will include a DEXA scan (if not completed as standard of care within 6 months prior to research visit), micro CT, and blood collection. | |
| Denosomab Sub-study | Experimental | Approximately 10 subjects with CF that have completed the main study and have results which indicate bone disease are eligible to participate in the sub study. Subjects who consent will receive treatment with denosumab (60 mg/ml via subcutaneous injection in the upper arm, upper thigh, or abdomen every 6 months) for up to 5 years. These subjects will be asked to return every 6 months for injections and annually (+/- 6 months) for up to 5 years for a DEXA scan, micro CT, and blood collection. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Denosumab | Drug | treatment with denosumab every 6 months for up to 5 years |
|
| Measure | Description | Time Frame |
|---|---|---|
| DEXA results | Z and/or T scores | Looking at single timepoints and well as changes for up to 5 years for those in the sub-study |
| Micro CT results | Trabecular bone volume to tissue volume (BV/TV), cortical and trabecular thickness (Tb.Th, microns), trabecular spacing (Tb.Sp; microns), and structure model index (SMI; index of trabecular rod vs plate-like shape/ stoutness) will be assessed. Change in BV/TV and cortical thickness will be taken to indicate increased/decreased bone mass accrual by microCT. | Looking at single timepoints and well as changes for up to 5 years for those in the sub-study |
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Cystic Fibrosis Main Study Inclusion Criteria:
Sub-study Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Ashley Keller | Contact | 2146482817 | ashley.keller@utsouthwestern.edu |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| UT Southwestern Medical Center | Recruiting | Dallas | Texas | 75390 | United States |
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| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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| ID | Term |
|---|---|
| D000069448 | Denosumab |
| ID | Term |
|---|---|
| D061067 | Antibodies, Monoclonal, Humanized |
| D000911 | Antibodies, Monoclonal |
| D000906 | Antibodies |
| D007136 | Immunoglobulins |
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| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |
| D007162 |
| Immunoproteins |
| D001798 | Blood Proteins |
| D011506 | Proteins |
| D000602 | Amino Acids, Peptides, and Proteins |
| D012712 | Serum Globulins |
| D005916 | Globulins |