800x600 Abstract Macrophage activation syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (SJIA). MAS is characterized by systemic inflammation caused by excessive or uncontrolled release of proinflammatory cytokines (cytokine storm). The diagnostic hallmark are hemophagocytic macrophages, that could be present in bone marrow, liver, spleen or lymph nodes. Clinical features are similar to a flare of the underlying rheumatic disease which makes early recognition and choice of the appropriate treatment difficult. Diagnosis is made according to the preliminary diagnostic guidelines for MAS complicating SJIA. We report a case of an 11 years old girl with MAS as an initial presentation of SJIA. She was successfully treated with high doses of glucocorticoid and cyclosporine. After discontinuation of glucocorticoid therapy she developed a new flare of the disease which was successfully treated with interleukin 1 blocking agent anakinra.

Systemic capillary leak syndrome is a rare condition, characterized by hypotension, edema, hemoconcentration and hypoalbuminemia. We describe 2 episodes of systemic capillary leak syndrome, following influenza A virus infection, occurring during 2 subsequent influenza seasons, in an 8-year-old boy.

Anaphylaxis is a severe life-threatening generalized or systemic hypersensitivity reaction. All doctors and other medical staff should be familiar with the treatment of anaphylaxis. Food, insect bites and drugs and are principal agents responsible for anaphylaxis in children and adolescents. In the absence of treatment, the reaction may rapidly progress with severe manifestations including fatal outcome. Intramuscular adrenaline is first-line therapy for anaphylaxis. Additional measures, such as removing the trigger, call for help, the correct position of the child or adolescent, high-flow oxygen, volume support, bronchodilator and adrenaline inhalations, systemic antihistamine and glucocorticoid, are supplementary to adrenaline. At discharge from hospital it is necessary to assess the risk of future anaphylaxis to determine the individualized management plan in case of anaphylaxis and to prescribe adrenaline auto-injector. Training of the child, parents and others who take care of the child, on when and how to use the self-injectable devices of adrenaline is necessary. Allergy assessment at an allergists office is obligatory in all children with a history of anaphylaxis in order to determine the cause of anaphylaxis, to provide detailed instructions on allergen avoidance and, if possible, to start with specific immunotherapy.