Vitamin D Provision in Children with Congenital Epidermolysis Bullosa: Cross-Sectional Study
https://doi.org/10.15690/vsp.v20i5.2315
Abstract
Background. Children with congenital epidermolysis bullosa (CEB) can have vitamin D deficiency due to its malabsorption in intestine and reduced synthesis in skin as these patients have restrictions on staying in the sun. However, the prevalence of vitamin D insufficiency/deficiency among patients with CEB remains not fully studied due to the small samples' sizes in previously studies. Objective. Our aim was to study vitamin D provision in children with CEB. Methods. The study included children aged from 3 to 18 years old with simplex and dystrophic types of CEB hospitalized in our department. The serum level of 25(OH)D was determined via chemiluminescence immunoassay. Vitamin D deficiency was established at 25(OH)D concentration of 20-30 ng/ml, deficiency — < 10-20 ng/ml, deep deficiency — < 10 ng/ml. Results. The study included 129 children with CEB (62 (48%) males, median age 6 (3; 10) years). 101 patients had dystrophic type of disease, 28 — simplex. The median 25(OH)D serum concentration in children with CEB was 21.7 (13.0; 36.6) ng/ml. Vitamin D insufficiency was revealed in 36 (28%) patients, deficiency — in 38 (29%), deep deficiency — in 16 (12%). Independent predictors of 25(OH)D concentration were the type of CEB (concentration was higher in children with simplex type) and age (negative association), but not the patients' gender and the examination season, according to multivariate regression analysis. Conclusion. The study has shown low level of vitamin D provision in children with CEB, whilst 25(OH)D concentration depended on the type of disease and the age of patients.
About the Authors
Irina Yu. ProninaRussian Federation
Moscow.
Disclosure of interest:
No conflict of interests.
Nikolay N. Murashkin
Russian Federation
Moscow.
Disclosure of interest:
Nikolay N. Murashkin — receiving research grants from pharmaceutical companies Janssen, Eli Lilly, Novartis. Scientific consultant of Galderma, Pierre Fabre, Bayer, LEO Pharma, Pfizer, AbbVie, Amryt Pharma, Zeldis Pharma.
Svetlana G. Makarova
Russian Federation
Moscow.
Disclosure of interest:
No conflict of interests.
Elena L. Semikina
Russian Federation
Moscow.
Disclosure of interest:
No conflict of interests.
Dmitry S. Yasakov
Russian Federation
Moscow.
Disclosure of interest:
No conflict of interests.
Stepan G. Grigoriev
Russian Federation
Saint Petersburg.
Disclosure of interest:
No conflict of interests.
References
1. Bardhan A, Bruckner-Tuderman L, Chapple I, et al. Epidermolysis bullosa. Nat Rev Dis Primers. 2020;6(1):78. doi: 10.1038/s41572-020-0210-0
2. Bulleznyi epidermoliz: Guide for doctors. Murashkin NN, Namazova-Baranova LS, eds. Moscow: Pediatr; 2019. 444 p. (In Russ).
3. Fine JD, Bruckner-Tuderman L, Eady RAJ, et al. Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol. 2014;70(6):1103-1126. doi: 10.1016/j.jaad.2014.01.903
4. Fine JD. Epidemiology of inherited epidermolysis bullosa based on incidence and prevalence estimates from the National Epidermolysis Bullosa Registry. JAMA Dermatol. 2016;152(11):1231-1238. doi: 10.1001/jamadermatol.2016.2473
5. Has C, Bauer JW, Bodemer C, et al. Consensus reclassification of inherited epidermolysis bullosa and other disorders with skin fragility. Br J Dermatol. 2020;183(4):614-627. doi: 10.1111/bjd.18921
6. Mariath LM, Santin JT, Schuler-Faccini L, Kiszewski AE. Inherited epidermolysis bullosa: update on the clinical and genetic aspects. An Bras Dermatol. 2020;95(5):551-569. doi: 10.1016/j.abd.2020.05.001
7. Albanova VI, Smolyannikova VA, Golchenko VA. Kindler syndrome — a rare type of hereditary epidermolysis bullosa. Vestnik Dermatologii i Venerologii. 2015;(4):95-104. (In Russ).
8. Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. J Am Acad Dermatol. 2009;61(3):367-384. doi: 10.1016/j.jaad.2009.03.052
9. Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organs. J Am Acad Dermatol. 2009;61(3):387-402. doi: 10.1016/j.jaad.2009.03.053
10. Murashkin NN, Makarova SG, Epishev RV, et al. Assessment of nutritional imbalance in children with epidermolysis bullosa. Kremlin Medicine Journal. 2018;(1):61-65. (In Russ).
11. Reyes ML, Cattani A, Gajardo H, et al. Bone metabolism in children with epidermolysis bullosa. J Pediatr. 2002;140(4):467-469. doi: 10.1067/mpd.2002.123287
12. Salera S, Tadini G, Rossetti D, et al. A nutrition-based approach to epidermolysis bullosa: Causes, assessments, requirements and management. Clin Nutr. 2020;39(2):343-352. doi: 10.1016/j.clnu.2019.02.023
13. Institute of Medicine, Food and Nutrition Board. Dietary Reference Intakes for Calcium and Vitamin D. Washington, DC: National Academy Press; 2010.
14. Cranney C, Horsely T, O'Donnell S, et al. Effectiveness and safety of vitamin D. Evid Rep Technol Asses (Full Rep). 2007;(158):1-235.
15. Pludowski P, Holick MF, Pilz S, et al. Vitamin D effects on musculoskeletal health, immunity, autoimmunity, cardiovascular disease, cancer, fertility, pregnancy, dementia and mortality-a review of recent evidence. Autoimmun Rev. 2013;12(10):976-989. doi: 10.1016/j.autrev.2013.02.004
16. Martinez AE, Mellerio JE. Osteopenia and osteoporosis in epidermolysis bullosa. Dermatol Clin. 2010;28(2):353-355. doi: 10.1016/j.det.2010.01.006
17. Varki R, Sadowski S, Uitto J, Pfendner E. Epidermolysis bullosa. II. Type VII collagen mutations and phenotype-genotype correlations in the dystrophic subtypes. J Med Genet. 2007;44(3):181-192. doi: 10.1136/jmg.2006.045302
18. Vahidnezhad H, Youssefian L, Zeinali S, et al. Dystrophic Epidermolysis Bullosa: COL7A1 Mutation Landscape in a Multi-Ethnic Cohort of 152 Extended Families with High Degree of Customary Consanguineous Marriages. J Invest Dermatol. 2017;137:660-669. doi: 10.1016/j.jid.2016.10.023
19. Rodari G, Guez S, Manzoni F, et al. Birmingham epidermolysis severity score and vitamin D status are associated with low BMD in children with epidermolysis bullosa. Osteoporos Int. 2017;28(4):1385-1392. doi: 10.1007/s00198-016-3883-1
20. Makarova SG, Namazova-Baranova LS, Murashkin NN, et al. Correction of Nutritional Status in Complex Therapy for Children Suffering from Dystrophic Forms of Innate Epidermolysis Bullosa. Pediatricheskaya farmakologiya — Pediatric pharmacology. 2016;13(6):577-586. (In Russ). doi: 10.15690/pf.v13i6.1672
21. Reimer A, Hess M, Schwieger-Briel A, et al. Natural history of growth and anaemia in children with epidermolysis bullosa: a retrospective cohort study. Br J Dermatol. 2020;182(6):1437-1448. doi: 10.1111/bjd.18475
22. Epishev RV. Nutritivnaya podderzhka detei s vrozhdennym bulleznym epidermolizom. [dissertation]. Moscow; 2018. (In Russ).
23. Fine JD, Tamura T, Johnson L. Blood vitamin and trace metal levels in epidermolysis bullosa. Arch Dermatol. 1989;125:374-379.
24. Ingen-Housz-Oro S, Blanchet-Bardon C, Vrillat M, Dubertret L. Vitamin and trace metal levels in recessive dystrophic epidermolysis bullosa. J Eur Acad Dermatol Venereol. 2004;18(6):649-653. doi: 10.1111/j.1468-3083.2004.01067.x
25. Petrushkina AA, Pigarova EA, Rozhinskaya LYa. The prevalence of vitamin D deficiency in Russian Federation. Osteoporosis and Bone Diseases. 2018;21(3):15-20. (In Russ). doi: 10.14341/osteo10038
26. Natsional'naya programma “Nedostatochnost' vitamina D u detei i podrostkov Rossiiskoi Federatsii: sovremennye podkhody k korrektsii”. Moscow: Pediatr; 2018. 96 p. (In Russ).
Review
For citations:
Pronina I.Yu., Murashkin N.N., Makarova S.G., Semikina E.L., Yasakov D.S., Grigoriev S.G. Vitamin D Provision in Children with Congenital Epidermolysis Bullosa: Cross-Sectional Study. Current Pediatrics. 2021;20(5):407-412. https://doi.org/10.15690/vsp.v20i5.2315