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Objective To describe how nursing care is delivered to patients with epidermal necrolysis in burn units/specialized units in Spain and a selection of countries. Method Descriptive cross-sectional study. Data were collected through a structured questionnaire which was sent to nurse managers in all burn units in Spain and a selection of countries. Descriptive statistics was used to summarize the results. Results All BU/SUs in Spain (n = 12) and seven BU/SUs from a selection of countries completed the questionnaire. A lack of specific nursing protocols on Epidermal Necrolysis was observed in most burn units in Spain. Skin cleansing techniques such as showering were only reported by participants from Spain. Use of antiseptics was less frequent in other countries. Conservative skin management was the most extended practice reported by all participants. The use of vaginal molds to prevent synechiae and coverage of the ocular surface with amniotic membrane to minimize sequelae were rarely reported. Pain assessment was not always documented in sedated patients and few participants reported the use of specific scales for this purpose. All nurses agreed in the need for consensus nursing care guidelines on the disease. Conclusions Nursing care in patients with epidermal necrolysis varied between burn units in Spain. Differences and similarities were observed when compared with burn units in other countries. Genital and ocular care were outdated in all BU/SUs. Pain assessment documentation was suboptimal. Evidence-based nursing care guidelines were generally demanded by all participants to help reduce mortality and morbidity of this rare and often devastating disease.

Several immunomodulatory agents are used in the treatment of epidermal necrolysis, but evidence of their efficacy is limited. The Autonomous Community of Madrid has two reference burn units to which all patients with epidermal necrolysis are referred. One burn unit has mostly used cyclosporine (CsA), and the other has used non-CsA therapies (mainly high-dose intravenous immunoglobulin). The allocation of patients to one or the other burn unit was mainly based on proximity, resembling a random assignment. Thus, we took advantage of this “natural experiment” to estimate the mortality risk ratio (MRR) of CsA (n = 26) compared with non-CsA (n = 16) treatment using hospital as an instrumental variable over the period from 2001 to 2015. We also computed the observed versus expected (O/E) MRR in a case series of 49 CsA-treated patients (including 23 patients from other regions treated in Madrid), and using the Score for Toxic Epidermal Necrolysis (i.e., SCORTEN) scale to estimate the expected values. The instrumental variable-based MRR of CsA versus non-CsA was 0.09 (95% confidence interval = 0.00–0.49). The O/E analysis also showed a reduction in mortality risk (MRROE = 0.42; 95% confidence interval = 0.14–0.99). We identified five other case series of CsA-treated patients providing MRROE and meta-analyzed their results. The pooled MRROE (including from this study) was 0.41 (95% confidence interval = 0.21–0.80). All three approaches consistently show that CsA reduces the mortality in epidermal necrolysis patients. Ministerio de Economía, Industria y Competitividad (#PI12/02267) 6.448 JCR (2017) Q1, 3/64 Dermatology 2.282 SJR (2017) Q1, 48/457 Biochemistry No data IDR 2017 UEM

Background Hemoglobin (Hb) induces vasoconstriction by heme group binding nitric oxide in an irreversible fashion. Recent in vitro studies indicate that the thiol groups in Hb reversibly bind nitric oxide and participate in trans-nitrosylation reactions with other thiols. Sepsis is a pathophysiologic state characterized by vasodilation mediated, at least in part, by an excessive release of nitric oxide. The role of nitrosothiols (RSNOs) in these changes is unknown. Objectives We tested the following in a porcine model of sepsis: (i) whether glutathione (GSH) reverses the hemodynamic effects of Hb; (ii) whether GSH induces an increase in blood flow in sepsis; (iii) whether RSNO plasma concentration increases in sepsis and is related to hypotension. Design Nonrandomized animal controlled study. Setting Animal research facility in a university hospital. Subjects Anesthetized pigs were monitored with a pulmonary artery catheter and ultrasonic blood flow probes in the mesenteric artery and the portal vein for measurement of systemic, mesenteric, and portal blood flows (Q(TOT), Q(MES), and Q(POR), respectively). Four groups of pigs were studied: nonseptic, septic, nonseptic treated with Hb (stroma-free purified porcine hemoglobin), and septic treated with Hb (n = 6 in each group). Interventions Sepsis was induced at 0 min by the administration of live Escherichia coli. Hb (400 mg/kg/hr) was administered at 240 mins, followed by glutathione (1 g iv). Measurements and main results Hb induced a pressor response and a decrease in Q(TOT), Q(MES), and Q(POR). Glutathione reversed the effects of Hb on Q(MES) and Q(POR). In septic pigs not treated with Hb, GSH induced an increase in Q(POR). RSNO plasma concentration increased after the induction of sepsis and correlated significantly with blood pressure. Conclusions These results indicate the reversibility of the effects of Hb by GSH, probably by interactions between nitric oxide and the reduced sulfhydryl groups in Hb, and suggest a role of RSNOs in the cardiovascular changes of sepsis.