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

Hvidovre University Hospital

Status: Unknown

Conditions: Hypophosphatasia (HPP)

Treatments: Other: Microindentation; Biological: Biochemical analysis of different bone markers.; Other: High resolution peripheral quantitative computed tomography (HRpQCT)

Study type: Observational

Funder types: Other

Identifiers

NCT04181164

HvH-BABS-Study

Details and patient eligibility

About

The study aims to evaluate the bone architecture and bone strength in adults with Hypophosphatasia (HPP).

Enrollment: 30 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: Accepts Healthy Volunteers

Inclusion and 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

Trial design

30 participants in 2 patient groups

HPP-Group

Description:

Adults with hypophosphatasia.

Treatment:

Other: Microindentation

Other: High resolution peripheral quantitative computed tomography (HRpQCT)

Biological: Biochemical analysis of different bone markers.

Control-Group

Description:

Healthy control subjects.

Treatment:

Other: Microindentation

Other: High resolution peripheral quantitative computed tomography (HRpQCT)

Biological: Biochemical analysis of different bone markers.