Results We identified 190 aPL-positive patients and randomly selected 100 patients for detailed evaluation. Sixty-two were girls and 38 boys with mean age at presentation 9.7 years. Twenty-seven (27%) patients had underlying systemic autoimmune disease (SLE 16, JIA 8). Sixty patients presented with one, 14 with two and 13 with three or more aPL-related clinical manifestations. Thromboses occurred in 10 (10%) patients and one patient had recurrent thrombosis. One or more non-thrombotic clinical manifestations were found in 77 (77%) patients including hematological disorders in 27 (thrombocytopenia 20, autoimmune hemolytic anemia 2), neurological disorders in 26 (migraine 15, seizures 3, chorea 2) and skin disorders in 22 patients (livedo reticularis 9, Raynaud's phenomenon 4). Seventeen infants born to mothers with aPL-positive autoimmune disease had positive aPL and 4 of them exhibited aPL-related clinical manifestations.

Background The pathogenesis of PFAPA syndrome is unknown. According to Stojanov et al. cytokine profile in PFAPA syndrome suggests Th1 mediated inflammatory process resulting in continuous inflammation and reduced Th2 anti-inflammatory response. Interferon γ might be responsible for suppressing the production of IL-4 and IL10. Hung et al. showed suppressive effects of ketotifen on the expression of Th1 and Th2 related chemokines of human monocytes.

The aim of this study was to assess autoimmune response following annual influenza vaccination in apparently healthy adults, staff at a children's hospital. 92 healthy adult subjects were tested for autoantibodies including antinuclear antibodies (ANA), anti-extractable nuclear antigen antibodies (anti-ENA), antiphospholipid antibodies (aPL), namely anticardiolipin antibodies (aCL), anti-beta2-glycoprotein I antibodies (aβ2-GPI) and lupus anticoagulant (LA). Blood samples were taken from each participant before annual influenza vaccination, one month and six months after vaccination. Before influenza vaccination 26% of participants were positive for ANA, 1% for anti-ENA, 16% for aCL, 7% for aβ2-GPI and 2% for LA. One month after influenza vaccination 76% of participants showed no change in autoantibodies titres. Six months after influenza vaccination 74% of participants showed no change in autoantibodies titres. Overall, there was no statistically significant difference in the percentage of positive ANA, aCL, aβ2-GPI and LA before and 6 months after the vaccination. Five participants developed autoantibodies 6 months after the vaccination and one who was initially low positive for ANA became highly positive (1:320). Eleven participants had only transiently increased autoantibodies. Persistently positive or progressively increased levels of autoantibodies during 6 months' follow up were observed in 6 persons (7%). Our study showed a high percentage of positive autoantibody testing among healthy adult staff at a children's hospital. There was no statistically significant difference in the percentage of positive autoantibodies before and after influenza vaccination. However, our study clearly demonstrated induction of autoantibodies production in selected subjects.

Purpose of reviewAntiphospholipid syndrome is considered as the most common acquired hypercoagulation state of autoimmune disorder in children. Besides vascular occlusion, antiphospholipid antibodies have been associated with various nonthrombotic clinical manifestations. This review highlights recent clinical advances in the field of neonatal and pediatric antiphospholipid syndrome and emphasizes differences in relation to the antiphospholipid syndrome in adult population. Recent findingsNeonatal antiphospholipid syndrome is a rare clinical entity characterized by neonatal thrombotic disease due to the transplacental passage of maternal antiphospholipid antibodies. There is growing evidence that transplacentally transferred antiphospholipid antibodies act as a risk factor, but are not usually a sufficient condition for thrombosis and other thrombophilic risk factors should be systematically evaluated. Long-term studies of children born to antiphospholipid-antibody-positive mothers provided the evidence of possible neurodevelopmental changes in these children and regular neuropsychological assessments are recommended. Data from the pediatric antiphospholipid syndrome studies have confirmed that antiphospholipid-antibody-related thromboses in children are frequently associated with multiple antiphospholipid antibodies positivity and concomitant presence of inherited prothrombotic disorders. Children with antiphospholipid syndrome have frequently demonstrated associated nonthrombotic manifestations, particularly hematological, skin and neurological manifestations. Comparisons between children with primary antiphospholipid syndrome and antiphospholipid syndrome associated with autoimmune disease have recognized certain differences that suggest two distinct subgroups with specific clinical characteristics. SummaryClinical and laboratory characterization of pediatric patients with antiphospholipid syndrome continues to improve and implies some important differences between antiphospholipid syndrome in pediatric and adult populations.