Bullous pemphigoid (BP) is characterized by substantial skin and blood eosinophilia as well as intensive pruritus. Since the pruritogenic cytokine interleukin (IL)-31 is increased in inflammatory skin diseases the aim of this study was to determine whether IL-31 plays a role in BP. Using immunofluorescence, IL-31 expression was analysed in eosinophils derived from blister fluids and skin from patients with BP and IL-31 levels in blister fluids, serum and culture supernatants were determined by enzyme-linked immunoassay (ELISA). High levels of IL-31 expression were observed in BP blister fluids, but they were only marginally elevated in BP serum compared with healthy controls. Eosinophils from either BP blister fluids or skin biopsies showed strong expression of IL-31. Furthermore, peripheral blood eosinophils from patients with BP, but not healthy controls, released high levels of IL-31, reflecting those in blister fluids. In conclusion, eosinophils are a major source of IL-31 in BP and this cytokine may contribute to itch in patients with BP.

Highlights Osteosarcoma is the most common primary bone sarcoma. Neoadjuvant chemotherapy combined with limb salvage surgery (LSS) or amputation are the main strategies in treating limb osteosarcoma. LSS is associated with a higher 5-year overall survival. Local recurrence was more frequently encountered in patients treated with LSS, however not affecting overall survival.

Pilocytic astrocytomas (PA) comprise the most common childhood central nervous system (CNS) tumor. Exploiting registry-based data from Southern and Eastern Europe (SEE) and SEER, US, we opted to examine incidence, time trends, survival and tentative outcome disparities of childhood PA by sociodemographic and clinical features. Childhood PA were retrieved from 12 SEE registries (N = 552; 1983-2014) and SEER (N = 2723; 1973-2012). Age-standardized incidence rates (ASR) were estimated and survival was examined via Kaplan-Meier and Cox regression analysis. ASR of childhood PA during 1990-2012 in SEE was 4.2/10, doubling in the USA (8.2/10). Increasing trends, more prominent during earlier registration years, were recorded in both areas (SEE: +4.1 %, USA: +4.6 %, annually). Cerebellum comprised the most common location, apart from infants in whom supratentorial locations prevailed. Age at diagnosis was 1 year earlier in SEE, whereas 10-year survival was 87 % in SEE and 96 % in SEER, improving over time. Significant outcome predictors were age <1 year at diagnosis diagnosis (hazard ratio, HR [95% confidence intervals]: 3.96, [2.28-6.90]), female gender (HR: 1.38, [1.01-1.88]), residence in SEE (HR: 4.07, [2.95-5.61]) and rural areas (HR: 2.23, [1.53-3.27]), whereas non-cerebellar locations were associated with a 9- to 12-fold increase in risk of death. The first comprehensive overview of childhood PA epidemiology showed survival gains but also outcome discrepancies by geographical region and urbanization pointing to healthcare inequalities. The worse prognosis of infants and, possibly, females merits further consideration, as it might point to treatment adjustment needs, whereas expansion of systematic registration will allow interpretation of incidence variations.

Disseminated Trichosporon infection in neutropenic patients carries a poor prognosis. Clinical evidence on the use of voriconazole for this infection is limited. The authors report a case of Trichosporon asahii fungemia refractory to liposomal amphotericin B treatment in a boy with acute lymphoblastic leukemia, which resolved after the addition of voriconazole. Both voriconazole and amphotericin B exhibited low minimal inhibitory concentrations and minimal fungicidal concentrations, and their combination was indifferent in vitro. The use of voriconazole for the treatment of trichosporonosis in patients with hematologic malignancies deserves further study.

No abstract

No abstract

To our knowledge, this is the first reported case of a severe phototoxic reaction during treatment with pirfenidone. Our aim by presenting this case is to increase the awareness of clinicians for severe phototoxic effects of oral pirfenidone.

Infections frequently complicate the treatment course in children with hematologic malignancies undergoing chemotherapy. Febrile neutropenia (FN) remains a major cause of hospital admissions in this population, and respiratory tract is often proven to be the site of infection even without respiratory signs and symptoms. Clinical presentation may be subtle due to impaired inflammatory response. Common respiratory viruses and bacteria are widely identified in these patients, while fungi and, less commonly, bacteria are the causative agents in more severe cases. A detailed history, thorough clinical and basic laboratory examination along with a chest radiograph are the first steps in the evaluation of a child presenting signs of a pulmonary infection. After stratifying patient’s risk, prompt initiation of the appropriate empirical antimicrobial treatment is crucial and efficient for the majority of the patients. High-risk children should be treated with an intravenous antipseudomonal beta lactam agent, unless there is suspicion of multi-drug resistance when an antibiotic combination should be used. In unresponsive cases, more invasive procedures, including bronchoalveolar lavage (BAL), computed tomography (CT)-guided fine-needle aspiration or open lung biopsy (OLB), are recommended. Overall mortality rate can reach 20% with higher rates seen in cases unresponsive to initial therapy and those under mechanical ventilation.