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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Group 1 - Vitamin D | Experimental | Patients receiving vitamin D supplements in therapy |
|
| Group 2 - Oral nutritional supplement | Experimental | Patients receiving oral nutritional supplement |
|
| Group 3 - Vitamin D+Oral nutritional supplement | Experimental | Patients receiving vitamin D supplements and oral nutritional supplement in therapy |
|
| Group 4 - No intervention | No Intervention | Patients with dystrophic form of congenital epidermolysis bullosa who have not taken vitamin D supplements and/or oral nutritional supplement |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Vitamin D | Drug | Adding vitamin D preparations to the therapy in the individual required dosage to correct the deficient state |
|
| Measure | Description | Time Frame |
|---|---|---|
| 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 | 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 | 24 week |
| Phosphorus-calcium metabolism assessment | Study of levels of calcium, phosphorus, parathyroid hormone, creatinine, magnesium, albumin in the blood | Baseline |
| Phosphorus-calcium metabolism assessment | Study of levels of calcium, phosphorus, parathyroid hormone, creatinine, magnesium, albumin in the blood | 24 week |
| Assessment of the state of bone tissue | Study of levels of alkaline phosphatase, osteocalcin, b-CrossLaps, P1NP in the blood | Baseline |
| Assessment of the state of bone tissue | Study of levels of alkaline phosphatase, osteocalcin, b-CrossLaps, P1NP in the blood | 24 week |
| Assessment of indicators of physical development | Z-score calculation (weight-for-height z-score) | Baseline |
| Measure | Description | Time Frame |
|---|---|---|
| Assessment of bone mineral density | dual-energy X-ray densitometry | Baseline |
| Assessment of bone mineral density | dual-energy X-ray densitometry |
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Inclusion Criteria:
Exclusion Criteria:
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| National Medical Research Center for Children's Health | Recruiting | Moscow | 119296 | Russia |
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| Oral nutritional supplement | Dietary Supplement | Adding оral nutritional supplement to the therapy depending on the degree of protein-energy malnutrition |
|
| Assessment of indicators of physical development | Z-score calculation (weight-for-height z-score) | 24 wee |
| 24 week |
| Bone age assessment | X-ray of the bones of the hand with an assessment of bone age | Baseline |
| Bone age assessment | X-ray of the bones of the hand with an assessment of bone age | 24 week |
| ID | Term |
|---|---|
| D004820 | Epidermolysis Bullosa |
| D014808 | Vitamin D Deficiency |
| D010024 | Osteoporosis |
| D001851 | Bone Diseases, Metabolic |
| ID | Term |
|---|---|
| D012868 | Skin Abnormalities |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D012873 | Skin Diseases, Genetic |
| D030342 | Genetic Diseases, Inborn |
| D012871 | Skin Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D012872 | Skin Diseases, Vesiculobullous |
| D001361 | Avitaminosis |
| D003677 | Deficiency Diseases |
| D044342 | Malnutrition |
| D009748 | Nutrition Disorders |
| D009750 | Nutritional and Metabolic Diseases |
| D001847 | Bone Diseases |
| D009140 | Musculoskeletal Diseases |
| D008659 | Metabolic Diseases |
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| ID | Term |
|---|---|
| D014807 | Vitamin D |
| D019587 | Dietary Supplements |
| ID | Term |
|---|---|
| D012632 | Secosteroids |
| D013256 | Steroids |
| D000072473 | Fused-Ring Compounds |
| D011083 | Polycyclic Compounds |
| D005502 | Food |
| D000066888 | Diet, Food, and Nutrition |
| D010829 | Physiological Phenomena |
| D019602 | Food and Beverages |
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