Containing state-of-the-art contributions on the various domains of European media policies, this Handbook deals with theoretical approaches to European media policy: its historical development; ...specific policies for film, television, radio and the Internet; and international aspects of the fragmented policy domain.
Harlequin phenomenon consists of facial flush and erythrosis with unilateral sweating and pallor, associated with contralateral anhidrosis. We present the case of a child in whom the syndrome was ...associated with Horner's syndrome, epilepsy, mental and psychomotor retardation.
A 9-year-old boy presented with right unilateral hemifacial erythema on effort, with normal colouring and Horner's syndrome on the left side of the face. His medical history revealed generalized myoclonic epilepsy, psychomotor delay and mental retardation. No underlying anomalies were identified. Harlequin phenomenon was diagnosed.
Despite its stereotypical clinical features, Harlequin phenomenon is a poorly known disease. However, clinicians must be aware of it in order to determine the diagnosis and investigate for causes and any associated abnormalities. The underlying mechanism is an autonomic neuropathy affecting the sympathetic vasodilator neurons. To our knowledge, there have been no previous reports of Harlequin phenomenon in association with Horner syndrome, psychomotor delay and mental retardation.
Summary
Background The interest of long‐term superpotent topical steroids (STS) in bullous pemphigoid (BP) has been supported by randomized controlled trials. However, inadequate compliance, poor ...cutaneous tolerance and nursing difficulties are potential drawbacks. Open‐label studies on limited series of patients suggested that low‐dose methotrexate (MTX) may be useful, permitting long‐term maintenance of a clinical remission obtained by initial, short‐term STS.
Objectives Open, clinical records‐based retrospective analysis of a multicentre series of patients receiving a combined regimen of initial, short‐term STS and MTX followed by long‐term MTX alone. The primary objective was evaluation of the clinical efficiency of this strategy based on initial clinical remission and subsequent clinical maintenance. The secondary objective was evaluation of the tolerance (type and rating of adverse events) of this combined regimen.
Methods Seventy patients with BP (mean age 82·7 years) were included. Treatment consisted of an initial combination of STS and MTX for a mean duration of 12·3 weeks followed by long‐term MTX alone for a mean duration of 8·48 months with a mean and median MTX dosage of 10 mg per week.
Results One hundred per cent of the patients showed an initial, complete clinical remission after a mean time interval of 21·9 days. The overall rate of long‐term disease control was 76%, whereas 24% of patients experienced at least one relapse during subsequent treatment with MTX alone. Drug‐related adverse effects were mainly haematological and gastrointestinal and resulted in treatment discontinuation in 11 patients (16%). Six patients (9%) died during the follow‐up period with one death (1%) most likely to be related to treatment.
Conclusions Long‐term low‐dose MTX combined with short‐term STS may result in protracted control of BP in carefully selected patients. These results should prompt randomized controlled trials comparing this treatment with the more usual regimen of long‐term STS alone.
Summary
Background Some cases of dermatofibrosarcoma protuberans (DFSP) do not protrude above the skin.
Objectives To assess the prevalence of these DFSPs and further to describe their presentation ...and course.
Methods One hundred and forty‐three patients were retrospectively collected. They were asked to complete a standardized questionnaire indicating the history and appearance of the DFSP from the first skin changes identified to the time of diagnosis.
Results Eighty‐one DFSPs were described as protuberant ab initio, and 62 as initially nonprotuberant (npDFSP). The latter remained at this stage for a mean period of 7·6 years. Twenty‐nine per cent of npDFSPs were ‘morphoea‐like’, 19% were ‘atrophoderma‐like’ and 42% were ‘angioma‐like’. Age at diagnosis was similar for both initial presentations. npDFSPs were most often misdiagnosed by physicians.
Conclusions Nearly half the patients first identified their early DFSP‐related skin changes as patches. Both this frequency and the long duration at this preprotuberant stage should prompt dermatologists to consider the diagnosis of DFSP earlier, in order to make surgical treatment easier.
Introduction. –
Les effets secondaires musculaires des statines (inhibiteurs de l'HMG-CoA réductase) comportent une myopathie, des myalgies, une myosite ou une authentique rhabdomyolyse. Plus ...récemment, la survenue de maladies auto-immunes de type lupus, vascularite, polymyosite ou dermatomyosite a été rapportée au cours d'un traitement par statines.
Exégèse. –
Nous rapportons un cas de dermatomyosite chez une patiente traitée par pravastatine (Elisor
®) depuis deux ans. Le diagnostic est établi devant les signes cliniques cutanés et musculaires et l'élévation des CPK (40 N) tandis que les examens immunologiques sont négatifs, avec un électromyogramme (EMG) et une biopsie musculaire quasiment normaux. Le traitement par pravastatine est arrêté. La patiente récupère en trois mois, sous traitement par corticothérapie locale. La recherche d'un cancer est négative. L'analyse de la littérature confirme la rareté de cette association en trouvant huit autres cas. L'élévation des CPK est constante. La biopsie musculaire et/ou l'électromyogramme ont conforté le diagnostic dans la moitié des cas. Le traitement a été arrêté chez tous les patients et six ont reçu une corticothérapie générale pour obtenir cette amélioration. Tous sauf un ont guéri, une patiente décédant d'une fibrose pulmonaire extensive malgré un traitement immunosuppresseur par cyclophosphamide (Endoxan
®).
Conclusion. –
Une dermatomyosite survenant chez un patient traité par statines doit faire discuter la responsabilité du traitement dans l'apparition de la maladie et impose son arrêt. L'évolution de la maladie, parfois spontanément favorable, peut aussi nécessiter le recours aux traitements usuels, corticothérapie générale en premier lieu.
Introduction. –
The toxic myopathy caused by statins (HMG-CoA reductase inhibitors) is well established. Recent reports add to these effects systemic immune diseases including systemic lupus erythematosus, vasculitis, polymyositis or dermatomyositis.
Exegesis. –
We report a case of dermatomyositis in a 69-year-old patient treated with pravastatin Elisor
®. She presented with typical features of dermatomyositis 2 years after she started a treatment with pravastatin. The treatment was discontinued and she slowly improved, with a transient dermocorticosteroid treatment. Eight other patients with dermatomyositis and chronic treatment with HMG-CoA reductase inhibitors are reported in the literature. All of them presented with classical features of dermatomyositis. The discontinuation of the treatment was followed by spontaneous clinical and biological improvement in 3/9 patients. The other patients received high doses of corticosteroids and improved, except one patient who died of respiratory failure (pulmonary fibrosis) despite the adjunction of oral cyclophosphamide Endoxan
®. In these patients, dermatomyositis can be considered as a severe adverse reaction to HMG-CoA reductase inhibitors although a distinct casual link cannot be definitely established.
Conclusion. –
The increasing prescription of statins has led to the parallel increment of reported side-effects, where autoimmune diseases are now described. Among them, our case of dermatomyositis in a patient receiving pravastatin adds to the eight reported cases in the literature and highlights the potential role of statins as triggers of immune systemic diseases.
ObjectiveThe prevalence of severe primary IGF1 deficiency (IGFD) is unclear. IGFD must be identified promptly as treatment with recombinant human IGF1 (rhIGF1) is now available. Our objective was to ...characterize and assess the prevalence of severe primary IGFD in a large cohort of patients evaluated for short stature at a pediatric endocrinology unit in France.DesignObservational study in a prospective cohort.MethodsConsecutive patients referred to our unit between 2004 and 2009 for suspected slow statural growth were included. Patients were classified into eight etiological categories. IGFD was defined by height ≤−3 SDS, serum IGF1 levels <2.5th percentile, GH sufficiency, and absence of causes of secondary IGFD.ResultsOut of 2546 patients included, 337 (13.5%) were born small for gestational age and 424 (16.9%) had idiopathic short stature. In these two categories, we identified 30 patients who met our criterion for IGFD (30/2546, 1.2%). In these 30 patients, we assessed the response to IGF1 generation test, time course of IGF1 levels, and efficiency of GH replacement therapy. The results indicated that only four of the 30 children were definite or possible candidates for rhIGF1 replacement therapy.ConclusionThe prevalence of severe primary IGFD defined using the standard criterion for rhIGF1 treatment was 1.2%, and only 0.2% of patients were eligible for rhIGF1 therapy.
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