Bullosa Epidermolysis, Osteopenia, Osteoporosis, Phosphorus and Calcium Disorders, Vitamin D Deficiency
Conditions
Keywords
Children, Only
Brief summary
This retrospective prospective study is aimed at studying the level of vitamin D supply and identifying markers of bone tissue remodeling in order to develop approaches to the prevention of osteopenia and osteoporosis in children with congenital epidermolysis bullosa.
Detailed description
This retrospective prospective interventional study will examine vitamin D availability and its relationship with clinical disease, patient gender and age, and season. The mechanisms of impairment of phosphorus-calcium metabolism and bone tissue metabolism will be analyzed on the basis of biochemical parameters and instrumental research methods in children with dystrophic epidermolysis bullosa in order to develop personalized approaches for correcting vitamin D status and bone metabolism disorders in children with epidermolysis bullosa, followed by an assessment of their clinical efficacy.
Interventions
DRUGVitamin D
Adding vitamin D preparations to the therapy in the individual required dosage to correct the deficient state
DIETARY_SUPPLEMENTOral nutritional supplement
Adding оral nutritional supplement to the therapy depending on the degree of protein-energy malnutrition
Sponsors
National Medical Research Center for Children's Health, Russian Federation
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
0 Years to 17 Years
Healthy volunteers
No
Inclusion criteria
* Verified diagnosis of epidermolysis bullosa; * Signing by parents (legal representatives) of informed consent to participate in the study and fulfill the requirements of the study
Exclusion criteria
* not planned
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Vitamin D level | Baseline | Study of the provision of vitamin D in children with dystrophic and simple forms of epidermolysis bullosa based on the level of 25 (OH) vitamin D (25 hydroxycholecalciferol) in the blood |
| Phosphorus-calcium metabolism assessment | Baseline | Study of levels of calcium, phosphorus, parathyroid hormone, creatinine, magnesium, albumin in the blood |
| Assessment of the state of bone tissue | Baseline | Study of levels of alkaline phosphatase, osteocalcin, b-CrossLaps, P1NP in the blood |
| Assessment of indicators of physical development | Baseline | Z-score calculation (weight-for-height z-score) |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Assessment of bone mineral density | Baseline | dual-energy X-ray densitometry |
| Bone age assessment | Baseline | X-ray of the bones of the hand with an assessment of bone age |
Countries
Russia
Outcome results
None listed