Neuro-Behçet disease presenting as secondary pseudotumor syndrome: case report.

Behçet's disease is a multisystemic, recurrent, inflammatory disorder, which has a three-symptom complex comprising uveitis, oral aphtae and genital ulcerations. It is rare in childhood. The prevalence of neurologic involvement in BD is range of 10-49%, and shows a wide spectrum from isolated headache to subacute encephalopathy and severe psychosis. We report a 12-year-old Behçet's disease patient with secondary pseudotumor syndrome due to cerebral vein thrombosis and aim to review the literature.

The effect of iron deficiency anemia on the function of the immune system.

We aimed to study the effect of iron deficiency anemia (IDA) on immunity. In 32 children with IDA and 29 normal children, the percentage of T-lymphocyte subgroups, the level of serum interleukin-6 (IL-6); and the phagocytic activity, the oxidative burst activity of neutrophils and monocytes and the levels of immunoglobulins were compared. There was no difference in the distribution of T-lymphocyte subgroups. The mean IL-6 levels was 5.6+/-3.9 pg/ml in children with IDA and 10.3+/-5.3 pg/ml in the control group (P<0.001). The percentage of neutrophils with oxidative burst activity when stimulated with pma was 53.4+/-32.7% in children with IDA and 81.7+/-14.3% in the control group (P=0.005). The percentage of monocytes with oxidative burst activity was 13.8+/-11.7% in children with IDA and 35+/-20.0% in the control group (P<0.001) when stimulated with pma. and 4.3+/-3.1 versus 9.7+/-6.0% (P=0.008) when stimulated with fMLP. The ratio of neutrophils with phagocytic activity was 58.6+/-23.3% in the anemic group; and 74.2+/-17.7% in the control group (P=0.057). The ratio of monocytes with phagocytic activity was 24.3+/-12.0% in the anemic group; and 42.9+/-13.4% in the control group (P=0.001). IgG4 level was 16.7+/-16.6 mg/dl in children with IDA and 51.8+/-40.7 mg/dl in healthy children (P<0.05). These results suggest that humoral, cell-mediated and nonspecific immunity and the activity of cytokines which have an important role in various steps of immunogenic mechanisms are influenced by iron deficiency anemia.

Pulmonary tuberculosis in children with Hodgkin's lymphoma.

The clinical presentation of tuberculosis (TB) and Hodgkin's lymphoma (HL) with pulmonary involvement is similar and raises problems of differential diagnosis. It may also be difficult to distinguish TB from relapsed lymphoma. The purpose of this study was to evaluate the association of HL and pulmonary TB and to discuss differential diagnosis. Medical records of 70 children were reviewed retrospectively. A total of 27 patients (38%) had mediastinal-pulmonary involvement initially. Systemic symptoms were present in 37 (52%) patients. In all, 14 patients (20%) had pulmonary TB; three of them were diagnosed as having TB before HL, two of them had TB and HL concomittantly at initial diagnosis, seven of them during lymphoma therapy and two of them after the cessation of lymphoma treatment. PPD was positive (>10 mm) only in seven patients. In all, 11 patients with pulmonary TB had diffuse pulmonary infiltrations and mediastinal enlargement at lung contrast-enhanced computed tomography and X-ray, which was difficult to differentiate from HL. Biopsies were performed in five patients. No mortality because of the infection was seen. Only one patient had been lost as relapsed-resistant HL. To evaluate mediastinal lymphadenopathies is very crucial and the differential diagnosis is difficult; hence the association between HL and the TB must be considered especially in countries where TB is highly endemic.

Role of Mycoplasma pneumoniae and Chlamydia pneumoniae in children with community-acquired pneumonia in Istanbul, Turkey.

BACKGROUND: To investigate the role of Mycoplasma pneumoniae and Chlamydia pneumoniae infection in pediatric pneumonia, in Istanbul, Turkey, we conducted a prospective study covering all the children between 2 months and 15 years hospitalized for community-acquired pneumonia. METHODS: A total of 140 children (85 males, median age 2.5 years) with community-acquired pneumonia were enrolled. Acute and convalescent sera were tested for IgM and IgG antibodies to M. pneumoniae (enzyme-linked immunosorbent assay, Serion ELISA classic) and for IgM and IgG antibodies to C. pneumoniae (microimmunofluorescence, Savyon, Israel). RESULTS: Mycoplasma pneumoniae infection was diagnosed in 38 patients (27%) and C. pneumoniae infection in 7 (5%). In 2 children M. pneumoniae and C. pneumoniae co infection was observed. The average age of the M. pneumoniae cases was 5.3 years and that of the C. pneumoniae was 1.5 years. The average age of pneumonia cases caused by other pathogens was 3.4 years (p<0.05). No significant difference was observed in clinical onset, signs, symptoms and laboratory parameters in children with M. pneumoniae and C. pneumoniae infection and in those without M. pneumoniae and C. pneumoniae infection. CONCLUSIONS: The results of this study suggest a remarkable role for M. pneumoniae and C. pneumoniae in childhood community-acquired pneumonia, and the knowledge of the true prevalence of these two types of infections discovered in the community might lead to modifications in the present empirical treatment of bacterial pneumonia.

Neuro-Behçet syndrome presenting as acute meningeal syndrome.

Behçet's disease is a systemic vasculitic disease characterized mainly by recurrent oral and genital aphthous ulcers, uveitis and skin findings. Central nervous system involvement is a serious manifestation. A case of acute meningeal syndrome secondary to Behçet's disease is presented. Behçet's disease should always be considered in differential diagnosis of acute meningeal syndrome without signs of viral infection, particularly in the context of multisystem manifestations.

Presentation of interleukin-12/-23 receptor beta1 deficiency with various clinical symptoms of Salmonella infections.

Clinical disease caused by weakly pathogenic mycobacterial species, Mycobacterium bovis Bacille Calmette-Guérin (BCG) and non-tuberculous environmental mycobacteria (EM), which is known as Mendelian susceptibility to mycobacterial disease (MSMD), is a rare entity defined recently. Infections with the more virulent Mycobacterium species, M. tuberculosis, may have largely gone unnoticed in these patients due to early death. Mutations in five proteins (IFNgammaR1, IFNgammaR2, IL-12/IL-23Rbeta1, IL-12/IL-23p40 and STAT1) have been found in MSMD. These patients are prone to surprisingly few other infectious diseases mainly to salmonellosis. Here we present three IL-12/IL-23Rbeta1 deficient patients from three different families and with different genetic mutations, who presented exclusively with Salmonella infections. Bacteremia and lymph node involvement were common clinical expressions. Leukocytoclastic vasculitis developed in one of these patients. Two patients were not inoculated with BCG, the third patient did not develop BCG infection although BCG vaccine had been given twice at ages of 1 and 7 years. All three patients responded well to antibiotic treatment. In conclusion, patients with chronic, recurrent or complicated Salmonella infections should be screened for MSMD, particularly for IL-12/IL-23p40/IL-12R/-23Rbeta1 deficiency. Conversely, in patients with genetic IL-12/-23Rbeta1 deficiency a full evaluation for Salmonella infection is required. IL-12/IL-23p40/IL-12R/IL-23Rbeta1 deficiency seem to be underdiagnosed in patients with salmonellosis, and since such patients need prolonged therapy, diagnosis is important.

Myofibroblastic tumors involving bilateral adrenal glands and skin in a patient with common variable immunodeficiency.

Common variable immunodeficiency (CVID) is a heterogeneous form of primary immune deficiency characterized by hypogammaglobulinemia, recurrent bacterial infections, and various immunologic abnormalities. In addition to recurrent infections, patients with this syndrome have an increased incidence of autoimmune diseases and malignancy. A patient with CVID in whom myofibroblastic tumors affecting both adrenal glands and skin is presented.

Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to Activation-Induced Cytidine Deaminase deficiency.

Mutations of the Activation-Induced Cytidine Deaminase (AID) gene have been found in patients with autosomal recessive hyper-IgM (HIGM) syndrome type 2. We retrospectively analyzed clinical, immunologic and genetic characteristics of 29 patients from 22 families with AID deficiency. Patients' median age at diagnosis and at last evaluation was 4.9 years (range: 0 to 53) and 14.2 years (range: 2.7 to 63), respectively. Most patients had suffered from recurrent and severe infections, however, intravenous immunoglobulin (IVIG) replacement therapy resulted in a dramatic decrease in the number of infections. Lymphoid hyperplasia developed in 22 patients and persisted in 7 at last follow-up. It is striking to note that six patients developed autoimmune or inflammatory disorders including diabetes mellitus, polyarthritis, autoimmune hepatitis, hemolytic anemia, immune thrombocytopenia, Crohn's disease and chronic uveitis. Fifteen distinct AID mutations were found but there was no significant genotype-phenotype correlation. In conclusion, AID-deficient patients are prone to infections and lymphoid hyperplasia, which may be prevented by early-onset IVIG replacement, but also to autoimmune and inflammatory disorders.