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

Oldakovsky, V. I.; Мурашкин, Н. Н.; Lokhmatov, Maksim; Гусев, А А; Tupylenko, A.V.; Budkina, T.N.; Yatzik, Sergey; Dyakonova, E. E.; Abaykhanov, Rasul; Фисенко, А. П.

doi:10.1016/j.jpedsurg.2022.11.001

Cited by 6 publications

(2 citation statements)

References 24 publications

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“…TGF-β is a major factor in the mechanism of mesenchymal cell synthesis in vitro and during tissue fibrosis 20 , 21 . In the study of Oldakovskiy 22 , losartan was used to prevent restenosis after balloon dilation in children with dystrophic epidermolysis bullosa (DEB). These findings demonstrated that losartan contributed to a decrease in restenosis frequency and an improvement in the nutritional status of children after balloon dilation.…”

Section: Discussionmentioning

confidence: 99%

Paclitaxel-coated balloon catheter for benign esophageal stenosis in a rabbit model

Ren,

Wang,

et al. 2024

Sci Rep

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Most patients with benign esophageal stenosis require multiple or even continuous balloon dilation treatments to achieve symptom relief. In this study, eighteen rabbits were used to establish an esophageal benign stenosis model and were divided into a control group (n = 6), a balloon group (n = 6) and a PTX-coated balloon group (n = 6) to evaluate the feasibility and effectiveness of paclitaxel (PTX)-coated balloons for the rabbit esophageal benign stenosis model. The weight and esophageal diameter were recorded every 2 weeks until 8 weeks post-surgery. Hematoxylin–eosin staining, Masson’s trichrome staining and immunohistochemical staining were performed for pathological analysis. Four weeks post-operation, there was a significant difference in weight between the control group and the balloon group (p = 0.01) and between the control group and the PTX balloon group (p = 0.01). There was a significant difference in the esophageal diameter between the balloon group and the PTX balloon group at 8 weeks post-operation (p = 0.02). Four weeks post-operation, the degree of inflammatory cell infiltration in the PTX balloon group was significantly lower than that in the control group (p = 0.002) and balloon group (p = 0.001). The degree of collagen deposition in the PTX balloon group was significantly lower than that in the control group (p = 0.002) and balloon group (p = 0.03). Eight weeks post-operation, the percentage of cells positive for TGF-β (p < 0.001), the degree of inflammatory cell infiltration (p = 0.02) and the degree of collagen deposition (p = 0.02) in the PTX balloon group were significantly lower than those in the balloon group. Therefore, PTX-coated balloons may alleviate the local inflammatory response and collagen deposition when used during dilation treatment of benign esophageal stenosis.

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Section: Discussionmentioning

confidence: 99%

Paclitaxel-coated balloon catheter for benign esophageal stenosis in a rabbit model

Ren,

Wang,

et al. 2024

Sci Rep

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“…Interim analysis of 18 treated cases showed positive safety data, while final evaluation is awaited. 109 Interest in oral losartan for RDEB has sparked a few other small case reports 108,110,111 but consensus opinion is that the larger trial data results should be awaited before clinical recommendations on the use of losartan are made.…”

Section: Anti-fibrosis: Losartanmentioning

confidence: 99%

Innovations in the Treatment of Dystrophic Epidermolysis Bullosa (DEB): Current Landscape and Prospects

Hou,

del Agua,

Lwin

et al. 2023

TCRM

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Dystrophic epidermolysis bullosa (DEB) is one of the major types of EB, a rare hereditary group of trauma-induced blistering skin disorders. DEB is caused by inherited pathogenic variants in the COL7A1 gene, which encodes type VII collagen, the major component of anchoring fibrils which maintain adhesion between the outer epidermis and underlying dermis. DEB can be subclassified into dominant (DDEB) and recessive (RDEB) forms. Generally, DDEB has a milder phenotype, while RDEB patients often have more extensive blistering, chronic inflammation, skin fibrosis, and a propensity for squamous cell carcinoma development, collectively impacting on daily activities and life expectancy. At present, best practice treatments are mostly supportive, and thus there is a considerable burden of disease with unmet therapeutic need. Over the last 20 years, considerable translational research efforts have focused on either trying to cure DEB by direct correction of the COL7A1 gene pathology, or by modifying secondary inflammation to lessen phenotypic severity and improve patient symptoms such as poor wound healing, itch, and pain. In this review, we provide an overview and update on various therapeutic innovations for DEB, including gene therapy, cell-based therapy, protein therapy, and disease-modifying and symptomatic control agents. We outline the progress and challenges for each treatment modality and identify likely prospects for future clinical impact.

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Epidermolysis Bullosa: A Review of Wound Care and Emerging Treatments

Bermudez,

Warp,

Hargis

et al. 2024

Curr Derm Rep

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

Cited by 6 publications

References 24 publications

Paclitaxel-coated balloon catheter for benign esophageal stenosis in a rabbit model

Paclitaxel-coated balloon catheter for benign esophageal stenosis in a rabbit model

Innovations in the Treatment of Dystrophic Epidermolysis Bullosa (DEB): Current Landscape and Prospects

Epidermolysis Bullosa: A Review of Wound Care and Emerging Treatments

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