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| Name | Class |
|---|---|
| Odense University Hospital | OTHER |
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The study aims to evaluate the bone architecture and bone strength in adults with Hypophosphatasia (HPP).
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| HPP-Group | Adults with hypophosphatasia. |
| |
| Control-Group | Healthy control subjects. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Microindentation | Other | Microindentation is a new technology directly measuring bone strength by a minimal invasive technique. |
|
| Measure | Description | Time Frame |
|---|---|---|
| Differences in Bone Mineral Strength Index (BMSi) between the two groups, assessed by microindentation (OsteoProbe®). | Differences in BMSi between the HPP- and Control-Group will be evaluated by microindentation (OsteoProbe®). Microindentation is a technology directly measuring bone strength by a minimal invasive technique. By applying a standardized pressure with a probe, which at the same time measures the indentation depth in the tibia bone, a measure of bone strength is obtained and calculated as Bone Mineral Strength Index (BMSi) [1]. | 1. October 2019 - 31.July 2020 |
| Measure | Description | Time Frame |
|---|---|---|
| Correlation between BMSi and fracture prevalence in the HPP-Group and the Control-Group. | BMSi will be evaluated by microindentation (described above). In addition, information about the occurrence of fractures in the HPP- and Control-Group will be obtained by data from the Danish National Patient Register and a structured clinical interview. | 1. October 2019 - 31.July 2020 |
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Inclusion criteria HPP-Group:
Inclusion Criteria Control-Group:
Exclusion criteria HPP-Group:
Exclusion Criteria Control-Group:
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Adults, diagnosed with HPP (genetic verified) (n=15), matched 1:1 in case of gender, age (± 5 years), BMI (± 3 kg/m2), postmenopausal status (± 2 years) with healthy controls.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hvidovre University Hospital | Hvidovre | Capital Region | 2650 | Denmark |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 27840302 | Background | Herrera S, Diez-Perez A. Clinical experience with microindentation in vivo in humans. Bone. 2017 Feb;95:175-182. doi: 10.1016/j.bone.2016.11.003. Epub 2016 Nov 11. |
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| High resolution peripheral quantitative computed tomography (HRpQCT) | Other | HRpQCT scan can assess the cross-sectional geometry of the bone and is an appropriate investigation to evaluate bone quality. |
|
| Biochemical analysis of different bone markers. | Biological | Measurement of different bone markers by biochemical analysis of blood samples. |
|
| Evaluation of differences in bone microarchitecture between the HPP- and Control-Group by high resolution peripheral quantitative computed tomography (HRpQCT). | To asses differences in bone architecture between the two groups, the non-dominant distal radius and non-dominant distal tibia will be examined by HRpQCT, which will provide data about total, trabecular and cortical BMD, trabecular thickness, cortical thickness, trabecular number, stiffness and finite element failure load of the radius and tibia. | 1. October 2019 - 31.July 2020 |
| Evaluation of differences in bone homeostasis between the two groups by biochemical analysis of different bone markers (P1NP, CTx, BALP, Trab-5, Sclerostin, Osteocalcin and FGF23) | Blood samples will be collected for biochemical analysis of different bone markers (P1NP, CTx, BALP, Trab-5, Sclerostin, Osteocalcin and FGF23). BALP = Bone specific alkaline phosphatase CTx = Carboxy-terminal collagen crosslinks FGF-23 = Fibroblast growth factor 23 P1NP = Procollagen type 1 amino-terminal propeptide Trab-5 = Tartrate-resistant acid phosphatase-5 | 1. October 2019 - 31.July 2020 |
| ID | Term |
|---|---|
| D007014 | Hypophosphatasia |
| ID | Term |
|---|---|
| D008664 | Metal Metabolism, Inborn Errors |
| D008661 | Metabolism, Inborn Errors |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
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