Therapeutic approaches in the treatment of juvenile dermatomyositis in patients with recent-onset disease and in those experiencing disease flare: An international multicenter PRINTO study

Objective To evaluate response to therapy over a 24-month period in a large prospective international cohort of patients with juvenile dermatomyositis (DM). Methods The study included 145 patients with recent-onset juvenile DM and 130 juvenile DM patients experiencing disease flare, all of whom were \u3c18 years old. Disease activity parameters and therapeutic approaches in 4 geographic areas were analyzed at baseline and at 6, 12, and 24 months. Response was assessed according to the Pediatric Rheumatology International Trials Organization (PRINTO) juvenile DM response criteria, and data were reported as observed and in the intent-to-treat (ITT) population. Results Patients with recent-onset juvenile DM at baseline had higher baseline disease activity and greater improvement over 24 months when compared to juvenile DM patients experiencing disease flare at baseline. Methotrexate (MTX) or high-dose corticosteroids were administered more frequently to patients with recent-onset juvenile DM, compared to juvenile DM patients experiencing disease flare, who were more likely to receive cyclosporine. Compared to patients from Western and Eastern Europe, a higher proportion of patients from South and Central America and North America received pulse steroids, and the average steroid dosage was higher in the North American and South and Central American patients. The use of MTX was similar in all 4 regions, while cyclosporin A was more frequently used in Western Europe. In the as observed analysis, 57.9% of the patients with recent-onset juvenile DM and 36.4% of the patients experiencing disease flare (P \u3c 0.001) reached at least a 70% response by PRINTO criteria at 6 months; these proportions had increased at month 24 to 78.4% and 51.2%, respectively (P \u3c 0.001). Corresponding results of the ITT analysis were much lower, with only one-third of the patients able to maintain the initial assigned therapy over 24 months. Conclusion Patients with recent-onset juvenile DM are more likely to achieve significant clinical improvement over 24 months, when compared to patients experiencing flares of juvenile DM. Internationally, various therapeutic approaches are used to treat this disease. Copyright © 2011 by the American College of Rheumatology

Sterile folliculitis as an important diagnosis clue to Crohn's disease

Sterile folliculitis is known to be one of the rare cutaneous manifestations of Crohn's disease (CD). To our knowledge it has never been emphasized as a marker of significant diagnostic value, perhaps maybe even more significant than more common cutaneous manifestations such as erythema nodosum (EN)

L'Image du mois: Un cas pédiatrique de cavernome hémorragique du tronc cérébral

SCOPUS: no.jinfo:eu-repo/semantics/publishe

Une récidive de hernie diaphragmatique congénitale

Through the case report of a child who had had a congenital diaphragmatic hernia (CDH) and then relapsed 8 months after initial surgery, the various risk factors related to a CDH, its pre- and postnatal management as well as some long-term complications are discussed.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Unilateral exophthalmos secondary to cystic lymphangioma in a child

peer reviewedUne exophtalmie unilatérale de l’enfant peut être causée par de multiples étiologies. Toutefois le premier diagnostic à exclure est la néoplasie. L’échographie de l’orbite apporte des informations intéressantes pour orienter la prise en charge, avec très peu d’effets secondaires pour le patient, mais sa réalisation est opérateur-dépendant. L’IRM et la biopsie sont les deux examens indispensables pour confirmer le diagnostic

Belgique

Human herpesvirus 6 (HHV-6) has been demonstrated to be a neurotropic virus and has been linked to several neurological complications associated with a wide range of clinical outcomes. Here, we present data from immunocompetent children with a central neurological condition and intrathecal DNA detection of HHV-6 virus. Through these cases, physiopathology, investigations, and outcome of the disease will be described. Finally, specific treatment will be discussed

Contribution of NMR spectroscopy to the differential diagnosis of a recurrent cranial mass 7 years after irradiation for a pediatric ependymoma.

CASE REPORT: We describe the case of a 5-year-old-boy who underwent surgery and focal radiotherapy for an anaplastic ependymoma of the fourth ventricle. One year later, a spinal metastasis was treated the same way. Six years later, a 16-mm lesion was found on a control MRI in the posterior fossa. To help the differential diagnosis between a relapse, a radio-induced modification, and a new tumor, magnetic resonance spectroscopy was performed. The main findings were a peak at the expected resonance frequency of reduced glutathione, a prominent peak of glutamate/glutamine compounds, a low N-acetylaspartate, and the absence of elevated choline. These findings were suggestive of a meningioma, although the latency between irradiation and development of the lesion was quite short. The diagnosis was confirmed by the pathological examination. CONCLUSION: This case exemplifies the fact that magnetic resonance spectroscopy provides useful biochemical information in such a clinical setting.Case ReportsJournal ArticleSCOPUS: ar.jinfo:eu-repo/semantics/publishe

Microscopic Infiltration of Cryopreserved Ovarian Tissue in 2 Patients With Ewing Sarcoma.

We report the clinical history of 2 female patients with Ewing sarcoma and microscopic ovarian infiltration. In both cases, the initial workup found no metastasis. However, the examination of cryopreserved ovarian tissues revealed the presence of CD99 positive tumor cells with rearrangement of EWS gene confirmed by FISH. Both children were treated as patients with localized tumor and are currently in remission. These reports underline that, in Ewing sarcoma patients, retransplantation of cryopreserved ovarian tissue is not harmless and could lead to cancer relapse. These observations question also on the significance of ovarian dissemination on Ewing sarcoma prognosis and therapy

Oral treatment of febrile urinary tract infection in children: Feasibility and follow-up

To assess the feasibility of oral treatment protocol with available oral antibiotics in Belgium in children admitted in a emergency unit in a single center for a febrile urinary tract infectio