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- W4294771036 abstract "In the dystrophic form of CEB (DEB), malnutrition is more often observed with prominent deficiency of micro- and macroelements, especially calcium and vitamin D, that can lead to rickets and osteoporosis with fractures. Aim of the study . To study the features of mineral and bone metabolism in patients with DHE and evaluate the effectiveness of prescribing different doses of vitamin D. Methods and mаterials . The study group included 71 children with DEB (girls 55 %, boys 45 %), the median age was 8.5 years [3.67; 11.92] and 5.75 years [3.13; 10.46], respectively. All patients underwent a complex laboratory and instrumental examination before and after 6 months of Cholecalciferol therapy. Results . We found a high rate of vitamin D insufficiency (22.5 %) and deficiency (40 %), hypocalcemia of total calcium (40 %), a decrease in bone mineral density (BMD) down to osteopenia (33.8 %) and osteoporosis (19.7 %). Osteocalcin (OC) and C-terminal telopeptide of type I collagen (CTx-1) levels in the group of patients with hypocalcemia were significantly lower than in the subgroup with normal levels of calcium (p=0.001 for OC, p=0.015 for CTx-1). Wasting of higher degree was noted in the group with hypocalcemia (p=0.001). 53.6 % of patients took Cholecalciferol irregularly (IU 1100 IU [700; 1500]), 35.2 % of patients constantly took the medicine (IU 2000 IU [1000; 3000]) (p = 0.021). After 6 months, normal levels of 25(OH)D were noted in 62 % of children; the levels of P1NP (p<0.001), phosphorus (p=0.012) decreased, and BMD improved (p=0.024). According to the plan developed in our study, with level of 25(OH)D ≤ 30 ng/ml, children aged 1–7 years old should be given at least 1500 IU; at 7–12 years – 2500 IU; at 12–18 years – 3000 IU. Conclusion . To achieve optimal levels of vitamin D, calcium, and bone turnover markers in children with DEB, it is necessary to use a personalized approach for colecalciferol prescription." @default.
- W4294771036 created "2022-09-06" @default.
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- W4294771036 date "2022-09-01" @default.
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- W4294771036 title "Mineral and bone metabolism in children with a dystrophic form of сongenital epidermolysis bullosa: the effect of Cholecalciferol therapy" @default.
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- W4294771036 doi "https://doi.org/10.33667/2078-5631-2022-16-60-69" @default.
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