The objective of this study was to assess epidemiology, diagnostic work-up, treatment and follow-up of children presenting to emergency department (ED) with headache. Records of visits for non-traumatic headache to the ED of a pediatric hospital over a period of 12 months were retrospectively reviewed. Headache center charts were analyzed one year after. Five-hundred and fifty patients (1% of all ED visits) were included. Spectrum of diagnoses was: primary headache (56.7%), with 9.6% of migraine; secondary headache (42%); unclassified headache (1.3%). Viral illnesses accounted for 90.5% of secondary headaches. A serious disorder was found in 4% of patients. Forty-four patients (8%) underwent neuroimaging studies, with 25% of abnormal findings. Only 223 patients (40.5%) received pharmacological treatment. On discharge, 212 patients (38.5%) were referred to headache center and 114 (20.7% of all patients) attended it. ED diagnosis was confirmed in 74.6% of cases. Most of ED repeated visits (82.6%) occurred in patients not referred to headache center at discharge from first ED visit. The most frequent diagnosis was primary headache; viral illnesses represented the majority of secondary headaches. Underlying serious disorders were associated with neurological signs, limiting the need of diagnostic investigations. Well structured prospective studies are needed to evaluate appropriate diagnostic tools, as well as correct therapeutic approach of pediatric headache in emergency. Collaboration with headache center might limit repeated visits and provide a correct diagnostic definition.

Fas (CD95) is a transmembrane molecule that induces programmed cell death (PCD) of lymphocytes. We examined its function in children with chronic thrombocytopenia, serum autoantibodies, and lymphadenopathy and/or splenomegaly. We found that T-cell lines from six of seven patients with this autoimmune/lymphoproliferative disease (ALD) were relatively resistant to PCD induced by monoclonal antibodies to Fas. By contrast, Fas function was normal in control patients with typical chronic idiopathic thrombocytopenic purpura (ITP) without lymphadenopathy. The defect was not due to decreased Fas expression, nor to over-production of soluble forms of Fas. Moreover, it specifically involved the Fas system because PCD was induced in the normal way by methylprednisolone. Complementary DNA sequencing of the Fas gene did not identify any causal mutation in patients with ALD. This distinguished them from patients with the human autoimmune lymphoproliferative syndrome (ALPS), who carry mutations of the Fas gene. Moreover, patients with ALD did not show the peripheral expansion of CD4/CD8 double-negative T cells that characterizes the ALPS phenotype. Fas signaling involves activation of a sphingomyelinase-catalyzing production of ceramide. We found that ceramide-induced PCD was defective in patients with ALD and not in patients with typical chronic ITP. These data suggest that the ALD patient defect involves the Fas signaling pathway downstream from the sphingomyelinase and that Fas gene mutations and double-negative T-cell expansion are not the only signs of a defective Fas system.

References1 Raskin NH. The hypnic headache syndrome. Headache 1988; 28: 536-6. 2 Dodick DW, Mosek AC, Campbell JK. The hypnic ('alarm clock') headache syndrome.

Kikuchi-Fujimoto disease, a benign and unusual self-limiting histiocytic necrotizing lymphadenitis of unknown origin, should be included in the differential diagnosis of lymphadenopathy and fever of unknown origin. This disease mostly affects young Asian women and has rarely been reported in children, thus remaining a poorly recognized entity that is frequently confused with malignant lymphoma. The authors describe two children with Kikuchi-Fujimoto disease, with particular attention to diagnostic approach and clinical and histologic features of the disease.

An elongated C-terminal hemoglobin variant, due to the deletion of nucleotide A in codon 144 (nucleotide 63600 GenBank entry UO1317) was found in a 31-year-old woman from Trento (northeastern Italy). This deletion led to the replacement of lysine at beta144 by a serine residue, the disappearance of the stop codon at position 147, and the presence of 12 additional residues, identical to those observed in Hbs Saveme, Tak and Cranston, which result from a similar mechanism. Hb Trento, amounting to 29% of the total hemoglobin, was unstable and had, as the other variants of this group, an increased oxygen affinity. It led to a mild compensated hemolytic anemia with red cell inclusion bodies. Functional studies of the isolated abnormal hemoglobin were difficult to perform because of autoxidation, precipitation, and formation of hybrids with Hb A.