Balearic archipelago: three islands,three beta‐thalassemia population patterns

López‐Escribano H, Parera MM, Guix P, Serra JM, Gutierrez A, Balsells D, Oliva‐Berini E, Castro JA, Ramon MM, Picornell A. Balearic archipelago: three islands, three beta‐thalassemia population patterns. The mutation spectrum of 175 β‐thalassemia (β‐thal) carriers, identified in pilot carrier screening on 22,713 individuals from Balearic Islands (Spain), is reported. The β⁰ CD39 (C>T) mutation is the most frequent (61.1%), followed by β⁺ IVS‐I‐110 (G>A) (12.0%), β⁺ IVS‐I‐6 (T>C) and β⁰ IVS‐1‐1 (G>A) (3.4% both) and eight other rare mutations (2.9–0.6%); with a distinct prevalence and distribution between islands. Minorca shows the highest prevalence in Iberian populations, with a single mutation, CD39 (C>T), present in most β‐thal carriers. Ibiza is the only Western Mediterranean population where the most frequent β‐thal mutation is IVS‐I‐110 (G>A). These results can be explained by a combination of historical–demographic characteristics together with evolutionary forces such as founder effect, genetic drift and probably selection by malaria. Knowledge of the mutational spectrum in the Balearic Islands will enable to optimize mutation detection strategy for genetic diagnosis of β‐thal in these islands.     

Prediction of delirium in critically ill patients with elevated C-reactive protein

Background and Purpose

Delirium is thought to be associated with systemic inflammatory response. However, its association with the most widely used inflammatory biomarker C-reactive protein (CRP) has not been well established. We aimed to examine whether CRP on intensive care unit (ICU) entry was associated with subsequent development of delirium.

Design and Setting

This prospective observational study was conducted in a mixed 24-bed ICU in a tertiary teaching hospital.

Methods

All patients admitted to the ICU from February 2011 to June 2012 were screened for eligibility. Demographic data and clinical characteristics of included patients were recorded. Patients were screened for the presence of delirium by using the tool Confusion Assessment Method for the ICU (CAM-ICU). C-reactive protein was obtained on ICU entry and 24 hours thereafter. Eligible patients were followed up for 28 days or until death. Univariate and multivariate analyses were performed to evaluate independent risk factors for delirium. Clinical outcome included the length of stay (LOS) in the ICU, 28-day mortality, and duration of mechanical ventilation. Two-tailed P < .05 was considered statistically significant.

Results

A total of 223 patients were included during study period. In univariate analysis, patients with delirium showed significantly higher CRP values than those without (120.5 vs 57.5 mg/L; P = .0001). By adjusting for confounding variables (including age, sex, Acute Physiology and Chronic Health Evaluation II, intubation, living alone, physical restraint, alcohol drinking, smoking, type of medical condition, and hospital LOS before ICU admission) in logistic regression model, CRP remained an independent predictor of delirium (odds ratio, 1.07; 95% confidence interval, 1.01-1.15). As compared with nondelirious patients, those with delirium showed longer LOS in ICU (13 vs 5 days; P < .001) and duration of mechanical ventilation (6 vs 1 days; P < .001). An increase in CRP greater than 8.1 mg/L within 24 hours was associated with 4-fold increase in the risk of delirium (odds ratio: 4.47, 95% confidence interval, 1.28-15.60).

Conclusion

C-reactive protein measured on ICU entry and its changes within 24 hours are risk indicators of delirium. Further studies exploring the treatment of delirium according to CRP levels are warranted.     

Rapid and successful effects of combining dupilumab with ultraviolet B radiation therapy in the treatment of Papuloerythroderma of Ofuji

Papuloerythroderma of Ofuji (PEO) is an uncommon disease characterised by widespread erythroderma composed of intensely pruritic solid papules coalescing into plaques sparing the skin folds (deck-chair sign). The pathogenesis of PEO remains unclear, although T helper (Th) 2 and Th22 cells may play an important role. Dupilumab is an interleukin (IL)-4 receptor α-antagonist that effectively reduces Th2 responses, which has drawn increasing attention in the treatment of PEO patients. Here, we reported a successful case of dupilumab treatment in combination with ultraviolet B (UVB) radiation therapy, which is well known and effective for chronic itch. The patient had a significant decrease in visual analogue scale (VAS) score and eosinophil after only 1 week of treatment, which may be due to the combination effect.