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Our experience of using Losartan for esophageal stenosis in children with dystrophic form of congenital epidermolysis bullosa

Published:November 18, 2022DOI:https://doi.org/10.1016/j.jpedsurg.2022.11.001

      Highlights

      • Esophageal stenosis is one of the most common surgical complications in children with dystrophic epidermolysis bullosa.
      • Balloon dilatation under X-ray control is the most efficient therapeutic technique, but the variable recurrence rate necessitates conservative treatment to prevent restenosis.
      • The use of the drug losartan is promising due to its antifibrotic effect through the suppression of transforming growth factor-β1 as a predominantly signaling pathway of the scar formation.

      Abstract

      Introduction

      Dystrophic epidermolysis bullosa (DEB) is one of the most severe forms of congenital epidermolysis bullosa and characterized by the formation of many surgical complications. Esophageal stenosis is a common complication of DEB and occurs in almost 76% of cases. Balloon dilatation (BD) under X-ray control is the main therapeutic technique, however conservative treatment is necessary to prevent restenosis. The use of the drug losartan is promising due to its antifibrotic effect through the suppression of transforming growth factor-β1 (TGF-β1).

      Purpose

      To evaluate the efficacy of losartan in the prevention of restenosis after BD of esophageal stenosis in children with DEB.

      Materials and Methods

      The study included 19 children from 2 to 16 years old (mean age 9.2 ± 3.58 years) with DEB and X-ray confirmed esophageal stenosis. All children underwent BD. In the main group 9 children after BD have received losartan, in the control group of 10 children - only standard therapy. The observation period was 12 months.

      Results

      In the main group, 1 child (11.1%) required repeated dilatation, in the control group - 4 children (40%). Indicators of nutritional deficiency (THINC scale) and the disease severity index (EBDASI) were significantly lower in the group of children treated with losartan. No undesirable actions of the drug were recorded.

      Conclusions

      In this study losartan showed its safety, contributed to a decrease in the restenosis frequency and an improvement in the nutritional status of children with DEB after BD. However, further studies are required to confirm its effectiveness.

      Keywords

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