Disease relapse was reported in 23 out of 46 patients (50%) within a half to 26 months. At relapse, 8 patients received corticosteroids monotherapy (35%) and 15 patients received additional immunosuppressive therapy (65%), usually cyclophosphamide, and in one case

mycophenolate-mofetil. The mortality of CSS is substantial, 10 out of 47 patients (21%) died after a disease duration of 2–26 months. Two patients died within short time when therapy was refused or stopped. Reported causes of death are: respiratory insufficiency (n = 1), pulmonary abscess and sepsis (n = 1), cardiac failure (n = 3), cardiac arrest and severe myocarditis (n = 1), intestinal perforation (n = 2), intestinal perforation and septicaemia (n = 1), and gastrointestinal inflammation with necrosis and sepsis (n = 1). Zwerina et al compared DNA Damage inhibitor CSS in childhood with CSS in adults.2 Asthma is frequent in all patients.2 Other organ involvement is different among children

and adults.2 Pulmonary infiltrates and cardiac disease are seen more frequently in children.2 Peripheral nerve and musculoskeletal symptoms are seen more frequently in adults.2 ANCA are found in about 1/3 of adult and in 1/4 of childhood patients with CSS, respectively.2 The outcome in childhood seems to be worse.2 Reasons for these differences remains elusive.2 In conclusion, we present signaling pathway a 12-year-old boy with an ANCA-negative Churg–Strauss syndrome. The clinical presentation of childhood CSS can be diverse, with involvement of different organ systems. The respiratory tract is most frequently involved, with pulmonary infiltrates, asthma symptoms and sinusitis. Early recognition of childhood CSS is important as delayed diagnosis can lead to severe organ involvement and a fatal outcome. Written informed consent was obtained from the patient and his parents for publication of this case report and images. A copy of the written consent is available for review

by the Editor-in-Chief of this journal. The authors did not receive any funding. FR, JH, GJV, LD, JJ, JB and PR were involved in diagnostics and treatment Ibrutinib chemical structure of the patient. FR and PR drafted the manuscript. All authors contributed to writing and editing the manuscript. All authors read and approved the final manuscript. The authors have no conflict of interest. We wish to thank our patient and his parents for their approval and collaboration. “
“The mammalian target of rapamycin inhibitor (mTORi) sirolimus was introduced into clinical transplantation in 1999.1 It is frequently used either in the induction phase or for maintenance immunosuppression to prevent acute and chronic rejection. Sirolimus is often used to achieve adequate immunosuppression while decreasing the dose and possible toxicity of primary agents, such as calcineurin inhibitors. Dose related myelosuppression and hyperlipidemia are the most common side effects.