Congenital epidermolysis bullosa is a rare, genetic condition in which even slight stimulation can cause blistering of the skin or mucosa. While previous reports of treatments requiring general anesthesia in these patients were focused on anesthesia-related procedures, such as endotracheal intubation, no report has described specific management required for these patients during surgery, such as preparation of the surgical site, fixation of infusion lines and other tubes, and adjustment of the operation table. This is probably the first report to address these issues. This report presents a case of recessive dystrophic congenital epidermolysis bullosa in which open hepatectomy was safely performed.
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| http://dx.doi.org/10.1155/2018/1786786 | DOI Listing |
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Article Synopsis
- Recessive dystrophic epidermolysis bullosa (RDEB) is a severe skin condition that causes blisters and other complications, and this study tested the safety of losartan, a medication that may slow its progression.
- The study was a phase 1/2 trial involving children aged 2-16 with confirmed RDEB, focusing on identifying any serious side effects of losartan over a treatment period of 10 months.
- Primary safety concerns monitored included severe drops in blood pressure, allergic reactions, and changes in potassium levels, along with evaluating the drug's effectiveness through various clinical scores.
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