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.
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.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
110
Adding vitamin D preparations to the therapy in the individual required dosage to correct the deficient state
Adding оral nutritional supplement to the therapy depending on the degree of protein-energy malnutrition
National Medical Research Center for Children's Health
Moscow, Russia
RECRUITINGVitamin D level
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
Time frame: Baseline
Vitamin D level
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
Time frame: 24 week
Phosphorus-calcium metabolism assessment
Study of levels of calcium, phosphorus, parathyroid hormone, creatinine, magnesium, albumin in the blood
Time frame: Baseline
Phosphorus-calcium metabolism assessment
Study of levels of calcium, phosphorus, parathyroid hormone, creatinine, magnesium, albumin in the blood
Time frame: 24 week
Assessment of the state of bone tissue
Study of levels of alkaline phosphatase, osteocalcin, b-CrossLaps, P1NP in the blood
Time frame: Baseline
Assessment of the state of bone tissue
Study of levels of alkaline phosphatase, osteocalcin, b-CrossLaps, P1NP in the blood
Time frame: 24 week
Assessment of indicators of physical development
Z-score calculation (weight-for-height z-score)
Time frame: Baseline
Assessment of indicators of physical development
Z-score calculation (weight-for-height z-score)
Time frame: 24 wee
Assessment of bone mineral density
dual-energy X-ray densitometry
Time frame: Baseline
Assessment of bone mineral density
dual-energy X-ray densitometry
Time frame: 24 week
Bone age assessment
X-ray of the bones of the hand with an assessment of bone age
Time frame: Baseline
Bone age assessment
X-ray of the bones of the hand with an assessment of bone age
Time frame: 24 week
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.