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Epidermolysis bullosa (EB) is a heterogeneous group of inherited rare diseases, which are characterized by trauma-induced blister formation of the skin and mucosa. The underlying cause is a functional deficiency of structural proteins of the epidermis or the dermis. Depending on the level of the blister formation, EB is divided into EB simplex (intra-epidermal), junctional EB (within the lamina lucida), dystrophic EB (below the lamina lucida) and Kindler syndrome (variable level of split formation). Besides different distinct blister formation and pain symptoms secondary problems like anaemia, oesophageal stenosis, cardiomyopathy or squamous cell carcinoma may occur. Since causal therapies are not available strict prevention of friction and trauma is essential to avoid blister formation. Anaesthesia challenges exist in the field of bedding procedures, care of the skin, monitoring, airway management und analgesia. This article gives a review over the EB and highlights in detail the corresponding anaesthesia characteristics., (© Georg Thieme Verlag Stuttgart · New York.)

Purpose: Economic assessment of an additional psychological intervention in the rehabilitation of patients with chronic low-back pain and evaluation of results by decision makers., Methods: Piggy-back cost-utility analysis of a randomised clinical trial, including a bootstrap analysis. Costs were measured by using the cost accounting systems of the rehabilitation clinics and by surveying patients. Health-related quality of life was measured using the EQ-5D. Implications of different representations of the decision problem and corresponding decision rules concerning the cost-effectiveness plane are discussed., Results: As compared with the 126 patients of the control arm, the 98 patients in the intervention arm gained 3.5 days in perfect health on average as well as 1219 euro cost saving. However, because of the uncertainty involved, the results of a bootstrap analysis cover all quadrants of the cost-effectiveness plane. Using maximum willingness-to-pay per effect unit gained, decision rules can be defined for parts of the cost-effectiveness plane. These have to be aggregated in a further valuation step., Conclusions: Study results show that decisions on stochastic economic evaluation results may require an additional valuation step aggregating the various parts of the cost-effectiveness plane.