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Endothelial dysfunction in patients with various forms of congenital epidermolysis bullosa

https://doi.org/10.24884/1607-4181-2022-29-3-101-105

Abstract

Introduction. The endothelial system is an important component of vascular-platelet hemostasis, capable of actively responding to mechanical and inflammatory agents. Patients with congenital epidermolysis bullosa are prone to mechanical damage to the skin and the development of a chronic inflammatory syndrome with a high probability of endothelial dysfunction.

The study objective was to assess the state of the endothelial system and to reveal the dependence of endothelial dysfunction on the form of epidermolysis bullosa.

Methods and materials. The study used venous blood of 57 patients (27 men and 30 women) with congenital epidermolysis bullosa. In patients with simple and dystrophic forms of epidermolysis bullosa, the platelet count, P-selectin, fibrinogen, albumin, C-reactive protein, von Willebrand factor antigen concentration, and factor VIII activity were determined.

Results. Comparative results of endothelial dysfunction depending on the form of epidermolysis bullosa were represented and endothelial dysfunction’s dependence on the concentration of albumin, C-reactive protein, and platelet count was determined.

Conclusions. In patients with a dystrophic form of epidermolysis bullosa, endothelial dysfunction is accompanied by an increase in the expression of P-selectin, factor VIII activity, and the concentration of von Willebrand factor antigen. Chronic inflammation and impaired nutritional status with a decrease in albumin contribute to the development of endothelial dysfunction. 

About the Authors

V. I. Kornev
A.M. Nikiforov Russian Center of Emergency and Radiation Medicine; Charitable Foundation «BELA. Children-Butterflies»
Russian Federation

Cand. of Sci. (Med.), Head of the Department of Anesthesiology and Reanimatology № 1, 4/2, Academica Lebedeva str., Saint Petersburg, 194044;

Moscow



O. N. Startseva
A.M. Nikiforov Russian Center of Emergency and Radiation Medicine
Russian Federation

Cand. of Sci. (Biol.), Biologist of the Clinical Chemistry Laboratory of Laboratory Diagnostic Department, 

4/2, Academica Lebedeva str., Saint Petersburg, 194044

 



A. S. Pleshkov
A.M. Nikiforov Russian Center of Emergency and Radiation Medicine; Charitable Foundation «BELA. Children-Butterflies»
Russian Federation

Surgeon of Burn Department, 4/2, Academica Lebedeva str., Saint Petersburg, 194044;

Moscow



M. V. Nikiforov
A.M. Nikiforov Russian Center of Emergency and Radiation Medicine; Charitable Foundation «BELA. Children-Butterflies»
Russian Federation

General Practitioner of the Department of Medical Rehabilitation, 4/2, Academica Lebedeva str., Saint Petersburg, 194044;

Moscow



References

1. Dorofienko N. N. The role of vasculare endothelium in the organism and the universal mechanisms of changing its activity (review) // Bulletin Physiology and Pathology of Respiration. 2018;(68):107–116. (In Russ.). Doi: 10.12737/article_5b1a0351210298.18315210.

2. Pizov N.A., PizovA. V., Skachkova O.A., Pizova N. V. Endothelial function in normal and pathological conditions // Meditsinsky Sovet. 2019;(6):154–159. (In Russ.). Doi: 10.21518/2079-701X-2019-6-154-159.

3. Albanova V. I., Golchenko V. A. Hereditary bullous epidermolysis. Modern concepts of etiology and patogenesis // Russian Journal of Skin and Venereal Diseases. 2013; 16(2):15. (In Russ.). Doi: 10.17816/dv36748.

4. Zinovyev G. V., Drozdovskaya D.A.,Anokhina Ye. M. et al. Squamous Cell Skin Cancer Treatment Experience in Patients with Dystrophic Form of Epidermolysis Bullosa // Effective Pharmacotherapy. 2021;17(11):38–43. (In Russ.). Doi: 10.33978/2307-3586-2021-17-11-38-43.

5. Fine J. D., Bruckner-Tuderman L., Eady R. A. 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.

6. Mel’nikova Y. S., Makarova T. P. Endothelial dysfunction as the key link of chronic diseases pathogenesis // Kazan medical journal. 2015;96(4):659–665. (In Russ.). Doi: 10.17750/KMJ2015-659.

7. Shabrov A. V., Apresyan A. G., Dobkes A. L., Ermolov S.Y., ErmolovaT. V., Manasyan S. G., Serdyukov S. V. Modern methods of evaluation of endothelial dysfunction and possibilities of their application in practical medicine // Rational pharmacotherapy in cardiology [Racional’naja farmakoterapija v kardiologii]. 2016;12(6):733–742. (In Russ.). Doi: 10.20996/1819-6446-2016-12-6-733-742.

8. Simundic A. M., Bölenius K., Cadamuro J. et al. Joint EFLM-COLABIOCLI Recommendation for venous blood sampling // Clin Chem Lab Med. 2018;56(12):2015–2038. Doi: 10.1515/cclm-2018-0602.

9. Kornev V. I., Kalinina N. M., Startseva O. N. Effect of minimally invasive extracorporeal circulationon endothelialdysfunction in cardiac ¬surgery patients // Kazan medical journal. 2020;101(2):279–283.(InRuss.).Doi: 10.17816/KMJ2020-279.

10. Chernova E. V. Von Willebrand factor // Herald of North-Western State Medical University named after I. I. Mechnikov. 2018;10(4):73–80. (In Russ.). Doi: 10.17816/mechnikov201810473-80.

11. Rajendran P., Rengarajan T., Thangavel J. et al. The vascular endothelium and human diseases // Int J Biol Sci. 2013;9(10):1057–1069. Doi: 10.7150/ijbs.7502.


Review

For citations:


Kornev V.I., Startseva O.N., Pleshkov A.S., Nikiforov M.V. Endothelial dysfunction in patients with various forms of congenital epidermolysis bullosa. The Scientific Notes of the Pavlov University. 2022;29(3):101-105. (In Russ.) https://doi.org/10.24884/1607-4181-2022-29-3-101-105

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