The German etanercept registry for treatment of juvenile idiopathic arthritis.

Objective: To describe a registry set up to monitor children treated with etanercept in Germany and Austria.

Methods: Giannini's criteria, duration of morning stiffness, number of swollen, tender and contracted joints, adverse events, and reasons for discontinuation were assessed.

Results: 322 patients with juvenile idiopathic arthritis (JIA) and 12 additional patients with non-JIA rheumatic diagnoses were included.

[Evidence-based use of methotrexate in children with rheumatic disorders. Consensus statement of the Working Group for Children and Adolescents with Rheumatic Diseases in Germany (AGKJR) and the Working Group Pediatric Rheumatology Austria].

Juvenile idiopathic arthritis (JIA) is the most common diagnosis in children and adolescents with rheumatic disorders. In many children and adolescents, JIA is successfully treated with nonsteroidal antiinflammatory drugs (NSAR) and physiotherapy. Still, in a significant number of cases the disease is resistant to this therapy and treatment with "second line" disease modifying antirheumatic drugs (DMARDs) is required.

Monitoring neutrophil activation in juvenile rheumatoid arthritis by S100A12 serum concentrations.

Objective: Phagocytes are extensively involved in the synovial inflammation associated with chronic arthritis. The aim of our study was to determine neutrophil activation in juvenile rheumatoid arthritis (JRA) by analyzing S100A12 (EN-RAGE; calgranulin C), a proinflammatory protein secreted by human neutrophils.

Methods: S100A12 serum concentrations were determined in 124 patients with chronic active polyarticular-, oligoarticular-, or systemic-onset JRA.

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.

Growth reconstitution in juvenile idiopathic arthritis treated with etanercept.

Objective: Growth failure is a leading problem in uncontrolled juvenile idiopathic arthritis. It also affects 10% of patients who are not treated with corticosteroids. The influence of proinflammatory cytokines like interleukin-1 beta, interleukin-6 and tumour necrosis factor on the neuroendocrine axis as well as on the production of insulin-like growth factors (IGFs) has been postulated.

Infliximab in two patients with juvenile ankylosing spondylitis.

Infliximab, a monoclonal chimeric anti-tumor necrosis factor alpha (anti-TNF-alpha) antibody, was tried in two patients suffering from severe refractory juvenile ankylosing spondylarthritis with disease duration of more than 10 years. To assess the response, validated clinical activity parameters were monitored prospectively. In both patients, treatment with infliximab at a dosage of 5 mg/kg body weight already led to considerable improvement with loss of joint pain the day after it was given.

Long-term follow-up of autologous stem cell transplantation for refractory juvenile idiopathic arthritis.

Since 1997, autologous stem cell transplantation (ASCT) had been applied to more than 40 children with polyarticular or systemic juvenile idiopathic arthritis (JIA). For this review, results of the follow-up are available from 25 children with systemic JIA and six with polyarticular JIA that were reported in detail from eight different pediatric European transplant centers. Before ASCT all children had progressive disease despite the use of corticosteroids, methotrexate (MTX) up to 1 mg/kg/week, cyclosporin (2.

[Therapy of osteoid osteomas -- always surgically?].

Osteoidosteomas are common bone tumours in childhood. Frequently they occur in the long bones of the lower extremities, less frequent in the humeri, phalanxes or the axial skeleton. The tumour is benign and noninfiltrative.

[Guillain-Barré syndrome as a rare cause of acute respiratory insufficiency].

Background: Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating disease of peripheral nerves. Antibodies acting against antigens on the myelin or the axons seem to play a causative role. In up to 80 % the onset of GBS follows an antecedent respiratory or gastrointestinal infection.

[Not Available].

Juvenile idiopathic arthritis is a chronic inflammatory disease with uncertain outcome. Patients may suffer from severe joint damage leading to mutilations as well as from extra-articular manifestations. Prognosis is variable and depends in part on the number of affected joints and the occurrence of extra-articular manifestations.

Treatment with cyclosporin A in a patient with Omenn's syndrome.

Unless treated with haematopoetic stem cell transplantation, Omenn's syndrome, a rare variant of severe combined immunodeficiency, is associated with a fatal outcome. We describe a male infant showing all the typical features of Omenn's syndrome, who was successfully treated with cyclosporin A to improve clinical condition prior to haematopoetic stem cell transplantation.

Herpes zoster by reactivated vaccine varicella zoster virus in a healthy child.

Unlabelled: Varicella can be prevented by vaccination using the live-attenuated Oka vaccine strain of varicella zoster virus (VZV). Only mild breakthrough disease has been reported in seronegative vaccinees when exposed to the wild-type virus. The latent varicella vaccine virus has rarely caused herpes zoster in childhood and adolescence.

Pulmonary function in children with juvenile idiopathic arthritis and effects of methotrexate therapy.

Objective: To evaluate impairment of lung function as an adverse effect associated with methotrexate therapy in patients with juvenile idiopathic arthritis (JIA).

Methods: We performed pulmonary function testing including diffusion capacity for carbon monoxide as measured by the single breath method (DLCO-SB) in 89 children with juvenile idiopathic arthritis. Forty (45%) were treated with methotrexate for a median of 24 months (range 3 to 120 months).

[Prader-Labhart-Willi syndrome in infants].

We report an infant with severe hypotonia, feeding problems and failure to thrive in the neonatal period, followed by developmental delay. In addition, pale skin, eyelid and pedal edema, cryptorchidism and micrognathia were present. The tentative diagnosis of Prader-Labhart-Willi syndrome was made and confirmed by specific molecular testing at the age of 5 months.

Systemic activation of the immune system during ganciclovir treatment following intratumoral herpes simplex virus type 1 thymidine kinase gene transfer in an adolescent ependymoma patient.

During ganciclovir treatment of an adolescent ependymoma patient two weeks after intracranial implantation of HSVtk retroviral vector producer cells, increasing numbers of peripheral T- and B-cells were found as well as enhanced T-cell activation and elevated serum levels of interleukin 12 and soluble Fas ligand. These findings suggest the systemic activation of the immune system during ganciclovir treatment in our patient. The induction of an immune response by HSVtk/ganciclovir supports the concept of an anti-tumor vaccination effect by prodrug activating gene therapy systems and may open new promising perspectives for enhancing therapeutic efficiency by combined prodrug activating and immunological gene therapy strategies.

Glycogenosis type V (McArdle's disease) mimicking atypical myositis.

A 13-year-old girl was referred to our clinic because of a positive rheumatoid factor test, muscle pain and weakness. Laboratory evaluation revealed an increased ESR, hypergammaglobulinaemia, antinuclear antibodies, circulating immune complexes, complement consumption and elevated serum creatine kinase (CK) activity. A needle biopsy of the dolent muscle showed normal routine histology.

A combination of etanercept and methotrexate for the treatment of refractory juvenile idiopathic arthritis: a pilot study.

Objective: To study the efficacy of combination therapy with etanercept and methotrexate in patients with refractory juvenile idiopathic arthritis.

Methods: Seven children with active juvenile idiopathic arthritis refractory to at least combination therapy with methotrexate and sulfasalazine or cyclosporin A were studied. Concomitant treatment, consisting of non-steroidal drugs, corticosteroids, and methotrexate, remained unchanged.

Apoptosis in T-lymphocyte subsets in human immunodeficiency virus-infected children measured immediately ex vivo and following in vitro activation.

Phosphatidylserine molecules are translocated to the outer plasma membrane of lymphocytes undergoing apoptosis and can be detected by the binding of fluorochrome-conjugated annexin V. Using the annexin V assay, we examined CD4 and CD8 T cells from human immunodeficiency virus (HIV)-infected children for apoptosis upon isolation or following in vitro culture. Immediate ex vivo analysis or overnight culture showed significantly higher levels of apoptosis in CD8 cells than in CD4 cells.

Panniculitis, pancreatitis and very severe aplastic anemia in childhood: a challenge to treat.

In childhood, aplastic anemia is a rare disease of unknown etiology. Besides toxic effects, also an infectious or an autoimmune origin are discussed. We report on an 8-year-old boy with very severe aplastic anemia (VSAA) who developed pancreatitis together with panniculitis.

Relapsing polychondritis in childhood--case report and short review.

Relapsing polychondritis (RP) is a disease of unknown etiology and it is characterized by inflammation of the cartilage. While the clinical picture of RP in adults is well described, RP in childhood is poorly documented. We describe a young girl presenting with acute dyspnea, stridor and polyarthritis.

[Mollusca contagiosa in HIV-infected children receiving optimal antiretroviral therapy].

Two children with symptomatic HIV-infection suffered from extended mollusca contagiosa. Intensified antiretroviral therapy including a protease inhibitor, resulted in a decrease of HIV RNA plasma concentration and a dramatic increase of CD4 T cells. Mollusca contagiosa nearly completely disappeared.

Treatment-resistant expansion of CD8+CD28-cells in pediatric HIV infection.

There is a disease stage-dependent loss of CD28 expression on T cells in HIV-infected children. In this study, T cell recovery, in particular CD28 expression on T cells, was analyzed after initiation of highly active antiretroviral therapy in a group of eight mostly treatment-naive HIV-infected children. Plasma HIV-RNA levels were recorded, and numbers of CD4, CD8, CD4+CD28+, and CD8+CD28+ cells were determined by two-color flow cytometry.

Diversity, functionality, and stability of the T cell repertoire derived in vivo from a single human T cell precursor.

In this report, we have analyzed the human T cell repertoire derived in vivo from a single T cell precursor. A unique case of X-linked severe combined immunodeficiency in which a reverse mutation occurred in an early T cell precursor was analyzed to this end. It was determined that at least 1,000 T cell clones with unique T cell receptor-beta sequences were generated from this precursor.