Evaluation of Bone Architecture and Bone Strength in Adults With Hypophosphatasia (HPP)

• Register: ClinicalTrials.gov
• Last refreshed on: 13 September 2021
• Main ID: NCT04181164
• Date of registration: 18/11/2019
• Prospective Registration: No
• Primary sponsor: Hvidovre University Hospital
• Public title: Evaluation of Bone Architecture and Bone Strength in Adults With Hypophosphatasia (HPP) BABS
• Scientific title: Evaluation of Bone Architecture and Bone Strength in Adults With Hypophosphatasia (HPP)
• Date of first enrolment: October 1, 2019
• Target sample size: 30
• Recruitment status: Active, not recruiting
• URL: https://clinicaltrials.gov/show/NCT04181164
• Study type: Observational

Countries of recruitment

Denmark

Key inclusion & exclusion criteria

Inclusion criteria HPP-Group:

- Genetically verified HPP

- Age: = 18 years

- Persistently low levels of alkaline phosphatase (ALP) = 35 U/L (normal range 35-105
U/L)

- At least one of the following symptoms: a) dental manifestations; b) musculoskeletal
pain; c) history of fracture(s)

- Submitted informed consent

Inclusion Criteria Control-Group:

- No ALP measurements = 45 U/l and = 50% of all ALP measurements, registered in the
electronical clinical journal = 55 U/l

- Normal parathyroid hormone (PTH) and Pyridoxal-5´-phosphate (PLP)

- Vitamin D3 = 25 nmol/L

- Submitted informed consent

Exclusion criteria HPP-Group:

- Pregnancy

- Skin infection or severe skin affection in the measurement area of microindentation

- Known allergy to Lidocain

- Former or current medical treatment influencing bone metabolism (oral corticosteroid >
12 weeks, former or current anti-osteoporosis treatment at any time (regardless drug
holiday), all kind of sex steroids (excluding oral contraception), anti-convulsants)

- Current malignant disorders

Exclusion Criteria Control-Group:

- Family history of a genetic metabolic bone disease (HPP, Osteogenesis imperfecta)

- Rickets in childhood

- Former or current Osteoporosis

- Former or current Osteomalacia

- Known diabetes

- Former or current medical treatment influencing bone metabolism (oral corticosteroid >
12 weeks, former or current anti-osteoporosis treatment at any time (regardless drug
holiday), all kind of sex steroids (excluding oral contraception), anti- convulsants)

- Skin infection or severe skin affection in the measurement area of microindentation

- Chronic liver or gallbladder disease

- Current malignant disorders

- Former or current thyrotoxicosis (T4 over normal range = 6 months)

- Cushing disease

- Pregnancy



Age minimum: 18 Years
Age maximum: N/A
Gender: All

Health Condition(s) or Problem(s) studied

Hypophosphatasia (HPP)

Intervention(s)

Other: High resolution peripheral quantitative computed tomography (HRpQCT)

Other: Microindentation

Biological: Biochemical analysis of different bone markers.

Primary Outcome(s)

Differences in Bone Mineral Strength Index (BMSi) between the two groups, assessed by microindentation (OsteoProbe®). [Time Frame: 1. October 2019 - 31.July 2020]

Secondary Outcome(s)

Correlation between BMSi and fracture prevalence in the HPP-Group and the Control-Group. [Time Frame: 1. October 2019 - 31.July 2020]

Evaluation of differences in bone microarchitecture between the HPP- and Control-Group by high resolution peripheral quantitative computed tomography (HRpQCT). [Time Frame: 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) [Time Frame: 1. October 2019 - 31.July 2020]

Secondary ID(s)

HvH-BABS-Study

Source(s) of Monetary Support

Please refer to primary and secondary sponsors

Secondary Sponsor(s)

Odense University Hospital