The use of amino acid formulas in the treatment of children with congenital epidermolysis bullosa
Congenital epidermolysis bullosa is a group of rare (orphan) diseases characterized by disruption of intercellular connections in the epidermis or epidermal-dermal junction and leading to the formation of blisters on the skin and/or mucous membranes even with minor injury. In addition to the violati...
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Open Systems Publication
2023-04-01
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| Series: | Лечащий Врач |
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| Online Access: | https://journal.lvrach.ru/jour/article/view/1050 |
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| author | M. A. Kosareva V. S. Nikonova O. S. Orlova |
| author_facet | M. A. Kosareva V. S. Nikonova O. S. Orlova |
| author_sort | M. A. Kosareva |
| collection | DOAJ |
| description | Congenital epidermolysis bullosa is a group of rare (orphan) diseases characterized by disruption of intercellular connections in the epidermis or epidermal-dermal junction and leading to the formation of blisters on the skin and/or mucous membranes even with minor injury. In addition to the violation of the integrity of the skin and the need to care for it, in patients with congenital epidermolysis bullosa, there is a lesion of the gastrointestinal tract, and therefore, the nutrition of children with congenital epidermolysis bullosa is of particular attention. The presence of multiple non-healing erosions and large transdermal losses of protein, serous fluid, and sometimes blood, as well as increased heat transfer, increase the need for energy and macronutrients in patients with congenital epidermolysis bullosa. Daily energy needs correspond to 115-150% of the age norms of consumption, and even more with pronounced erosion. The protein requirements are 115-200% of the age norm, in liquid – 150-200 ml/kg. Also, patients with congenital epidermolysis bullosa need to replenish ω-3 fatty acids, additional subsidies of microelements (zinc, iron, etc.) and vitamins (D, C, A, B12, B6, etc.). In case of formula feeding patient with BE it is extremely important to select appropriate formula. The loss of barrier properties by skin and mucous membranes causes an excessive intake of antigens, incl. allergens. Violation of barriers can cause sensitization to cow's milk proteins, with the subsequent development of a clinically significant allergy. In this regard, amino acid formula should be used as baseline nutrition, which ensure the absorption of nutrients under conditions of significant inhibition of the digestive tract's digesting and absorption capacity, and also minimize the antigenic load. Thus, patients with congenital epidermolysis bullosa require constant monitoring and assessment of nutritional status, with the earliest possible initiation of adequate nutritional support. Effective maintenance therapy and nutritional correction can improve the quality of life of patients and compensate for the loss of vital macro- and micronutrients. |
| format | Article |
| id | doaj-art-8617467c8f914e8a8b7fe25e7c73324e |
| institution | DOAJ |
| issn | 1560-5175 2687-1181 |
| language | Russian |
| publishDate | 2023-04-01 |
| publisher | Open Systems Publication |
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| series | Лечащий Врач |
| spelling | doaj-art-8617467c8f914e8a8b7fe25e7c73324e2025-08-20T03:20:31ZrusOpen Systems PublicationЛечащий Врач1560-51752687-11812023-04-0102727610.51793/OS.2023.26.2.0111045The use of amino acid formulas in the treatment of children with congenital epidermolysis bullosaM. A. Kosareva0V. S. Nikonova1O. S. Orlova2State Budgetary Healthcare Institution of the Nizhny Novgorod Region Children's City Clinical Hospital No. 1Federal State Budgetary Scientific Institution Medical and Genetic Research Center named after Academician N. P. BochkovFederal State Autonomous Institution National Medical Research Center for Children's Health of the Ministry of Health of the Russian Federation; State Budgetary Healthcare Institution of the Moscow Region Research Clinical Institute of Childhood of the Ministry of Health of the Moscow RegionCongenital epidermolysis bullosa is a group of rare (orphan) diseases characterized by disruption of intercellular connections in the epidermis or epidermal-dermal junction and leading to the formation of blisters on the skin and/or mucous membranes even with minor injury. In addition to the violation of the integrity of the skin and the need to care for it, in patients with congenital epidermolysis bullosa, there is a lesion of the gastrointestinal tract, and therefore, the nutrition of children with congenital epidermolysis bullosa is of particular attention. The presence of multiple non-healing erosions and large transdermal losses of protein, serous fluid, and sometimes blood, as well as increased heat transfer, increase the need for energy and macronutrients in patients with congenital epidermolysis bullosa. Daily energy needs correspond to 115-150% of the age norms of consumption, and even more with pronounced erosion. The protein requirements are 115-200% of the age norm, in liquid – 150-200 ml/kg. Also, patients with congenital epidermolysis bullosa need to replenish ω-3 fatty acids, additional subsidies of microelements (zinc, iron, etc.) and vitamins (D, C, A, B12, B6, etc.). In case of formula feeding patient with BE it is extremely important to select appropriate formula. The loss of barrier properties by skin and mucous membranes causes an excessive intake of antigens, incl. allergens. Violation of barriers can cause sensitization to cow's milk proteins, with the subsequent development of a clinically significant allergy. In this regard, amino acid formula should be used as baseline nutrition, which ensure the absorption of nutrients under conditions of significant inhibition of the digestive tract's digesting and absorption capacity, and also minimize the antigenic load. Thus, patients with congenital epidermolysis bullosa require constant monitoring and assessment of nutritional status, with the earliest possible initiation of adequate nutritional support. Effective maintenance therapy and nutritional correction can improve the quality of life of patients and compensate for the loss of vital macro- and micronutrients.https://journal.lvrach.ru/jour/article/view/1050congenital epidermolysis bullosaamino acid formulaallergy to cow's milk proteinschildrenenteral nutritionspecialized products |
| spellingShingle | M. A. Kosareva V. S. Nikonova O. S. Orlova The use of amino acid formulas in the treatment of children with congenital epidermolysis bullosa Лечащий Врач congenital epidermolysis bullosa amino acid formula allergy to cow's milk proteins children enteral nutrition specialized products |
| title | The use of amino acid formulas in the treatment of children with congenital epidermolysis bullosa |
| title_full | The use of amino acid formulas in the treatment of children with congenital epidermolysis bullosa |
| title_fullStr | The use of amino acid formulas in the treatment of children with congenital epidermolysis bullosa |
| title_full_unstemmed | The use of amino acid formulas in the treatment of children with congenital epidermolysis bullosa |
| title_short | The use of amino acid formulas in the treatment of children with congenital epidermolysis bullosa |
| title_sort | use of amino acid formulas in the treatment of children with congenital epidermolysis bullosa |
| topic | congenital epidermolysis bullosa amino acid formula allergy to cow's milk proteins children enteral nutrition specialized products |
| url | https://journal.lvrach.ru/jour/article/view/1050 |
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