Treatment of autoinflammatory diseases: results from the Eurofever Registry and a literature review.

OBJECTIVE: To evaluate the response to treatment of autoinflammatory diseases from an international registry and an up-to-date literature review. METHODS: The response to treatment was studied in a web-based registry in which clinical information on anonymised patients with autoinflammatory diseases was collected retrospectively as part of the Eurofever initiative. Participating hospitals included paediatric rheumatology centres of the Paediatric Rheumatology International Trial Organisation network and adult centres with a specific interest in autoinflammatory diseases. The following diseases were included: familial Mediterranean fever (FMF), cryopyrin-associated periodic syndromes (CAPS), tumour necrosis factor (TNF)-receptor associated periodic syndrome (TRAPS), mevalonate kinase deficiency (MKD), pyogenic arthritis pustulosis acne (PAPA) syndrome, deficiency of interleukin-1 receptor antagonist (DIRA), NLRP12-related periodic fever and periodic fever aphthosis pharyngitis adenitis (PFAPA) syndrome. Cases were independently validated by experts for each disease. A literature search regarding treatment of the abovementioned diseases was also performed using Medline and Embase. RESULTS: 22 months from the beginning of the enrolment, complete information on 496 validated patients was available. Data from the registry in combination with evidence from the literature confirmed that colchicine is the treatment of choice for FMF and IL-1 blockade for DIRA and CAPS. Corticosteroids on demand probably represent a valid therapeutic strategy for PFAPA, but also for MKD and TRAPS. Patients with poorly controlled MKD, TRAPS, PAPA or FMF may benefit from IL-1 blockade; anti-TNF treatment may represent a possible valuable alternative. CONCLUSIONS: In the absence of high-grade evidence, these results could serve as a basis for therapeutic guidelines and to identify candidate drugs for future therapeutic trials.

Observational study on the consumption of recreational drugs and alcohol by Swiss travelers.

BACKGROUND: Studies carried out on specific travelers' groups such as students describe an increase in the consumption of alcohol and drugs during travel and vacation time. The present study investigates the risk behaviors (alcohol and drugs) in a general adult population in Switzerland travelling abroad who visited a travel clinic before departure. METHODS: This retrospective study was conducted in a travel clinic between January 2006 and December 2008. 14,496 patients came to the clinic for a pre-travel consultation. 3,537 of them answered a questionnaire about their life habits in Switzerland and during their last trip. The only exclusion criterion was an age inferior to 18 years old.The consumption habits of drugs and at-risk alcohol intake (8 standard drinks (SD) per week for women and 15 SD for men) was analyzed according to gender, sex, destination and profession. Predictors of adopting a risky behavior between habits in Switzerland and during their previous trip were also analyzed. RESULTS: 7% (229/3477) of participants declared having at-risk alcohol consumption in Switzerland and 14% (473/3275 [95% CI 13-16]) during their trip. 9% (332/3527) of the participants used drugs in Switzerland and 5% (178/3481) during their trip. Risk factors for at-risk alcohol consumption during a trip were: at-risk alcohol consumption in Switzerland (OR 31[95% CI 21-45]), smoking (1.7 [95% CI 1-2]), use of drugs in Switzerland (OR 2.2 [95% CI 2-3]), leisure travel (OR 1.6 [95% CI 1-2]) and managerial professions (OR 1.8 [95% CI 1-3]). Risk factors for the use of drugs during a trip were: alcohol consumption in Switzerland (OR 2.1 [95% CI 1-4]), smoking (OR 1.9 [95% CI 1-3]), and use of drugs in Switzerland (OR 29.7 [95% CI 19-45]). CONCLUSIONS: At-risk alcohol consumption and, to a lesser extent, use of drugs, affect a large number of travelers which expose them to health problems during a trip. Exploring the alcohol and drugs consumption patterns of people visiting a travel clinic should be part of the pre-travel routine consultation and would allow to identifying people who would benefit most from a specific prevention.

Carma1, a CARD-containing binding partner of Bcl10, induces Bcl10 phosphorylation and NF-kappaB activation.

Bcl10, a caspase recruitment domain (CARD)-containing protein identified from a breakpoint in mucosa-associated lymphoid tissue (MALT) B lymphomas, is essential for antigen-receptor-mediated nuclear factor kappaB (NF-kappaB) activation in lymphocytes. We have identified a novel CARD-containing protein and interaction partner of Bcl10, named Carma1. Carma1 is predominantly expressed in lymphocytes and represents a new member of the membrane-associated guanylate kinase family. Carma1 binds Bcl10 via its CARD motif and induces translocation of Bcl10 from the cytoplasm into perinuclear structures. Moreover, expression of Carma1 induces phosphorylation of Bcl10 and activation of the transcription factor NF-kappaB. We propose that Carma1 is a crucial component of a novel Bcl10-dependent signaling pathway in T-cells that leads to the activation of NF-kappaB.

Clinical Experience with Immune Monitoring for Cytomegalovirus in Solid-Organ Transplant Recipients.

Novel strategies are needed to further reduce the burden of cytomegalovirus (CMV) disease in solid-organ transplant (SOT) recipients. Measurement of the specific cell-mediated immunity against CMV can identify the actual risk for the development of CMV disease in a given patient. Thus, immune monitoring is an attractive strategy for individualizing the management of CMV after transplantation. A growing number of observational studies on immune monitoring for CMV have been published over recent years, although there is a lack of data coming from interventional trials. In high-risk patients, measurement of CMV-specific T-cell responses appropriately stratifies the risk of CMV disease after discontinuation of antiviral prophylaxis. Immune monitoring may also help to identify patients followed by the preemptive approach at low risk for progression to CMV disease. Pretransplant assessment of cell-mediated immunity in seropositive patients may predict the development of posttransplant CMV infection. Overall, these studies indicate that the use of cell-mediated immunity assays has the potential to improve the management of CMV disease in SOT recipients.

Prescription footwear for severe injuries of foot and ankle: effect on regularity and symmetry of the gait assessed by trunk accelerometry.

After foot and/or ankle fracture, the restoration of optimal gait symmetry is one of the criteria of recovery. Orthotic insoles and orthopaedic shoes improve gait symmetry and regularity by controlling joint motion and improving alignment. The aim of the present study was to assess the effect of prescription footwear on gait quality by using accelerometers attached to the lower back. Sixteen adult patients with persistent disability after ankle and/or foot fractures performed two 30-s walking trials with and without prescription footwear (insoles and stabilizing shoes). Sixteen control subjects were also tested for comparison. The autocorrelation function was computed from the acceleration signal and the first two dominant periods were assessed (d1 and d2). Two parameters were used: (1) Stride Regularity (SR) which expresses the similarity between strides over time (d2), and (2) Stride Symmetry (SS) a ratio (d1/d2) which expresses the left/right similarity of gait independently of repeatability in the successive movements of each limb. In control subjects, SR and SS were 0.86+/-0.05 (correlation coefficient) and 81+/-10%, respectively. In the patient group, the effect of footwear was significant (SR: 0.88+/-0.06 vs. 0.90+/-0.05, SS: 38+/-23% vs. 46+/-27%). Pain was also significantly reduced (-34%). By using a rapid and low-cost method, we objectively quantified gait quality improvement after footwear intervention, concomitant to pain reduction. Substantial inter-patient variability in the footwear outcome was observed. In conclusion, we believe that trunk accelerometry can be a useful tool in the field of gait rehabilitation.

Indagación edípica e ironía trágica: el modelo sofocleo en Cat on a Hot Tin Roof de Tennessee Williams

Dado que el referente clásico "Edipo en busca de su identidad" ha sido siempre reconocido para Suddenly Last Summer , el autor de este artículo, mediante un análisis minucioso del texto del dramaturgo americano, propone leer en este caso Cat on a Hot Tin Roof desde el modelo Edipo Rey de Sófocles y descubrir en él igualmente la tradicional ironía clásica tanto desde el punto de vista del espectador como de los mismos personajes principales, Brick y su padre, ambos en busca de su verdad, una verdad, claro está, contraria a la esperada.

Oscar Wilde. De profundis: un juicio severo de Sófocles como poeta

O. Wilde considera que Jesucristo es un verdadero poeta y que Shelley y Sófocles le acompañan. Sin embargo, el resultado de esta equiparación es en realidad muy desfavorable para el gran trágico griego, el cual, precisamente como poeta, jamás podrá alcanzar las cotas de sublimidad y sufrimiento trágico real que Jesucristo representa. Según Wilde, Jesucristo es poeta y poema trágico, mientras que Sófocles es un simple poeta, certificando así la imposibilidad de la equiparación.

Oscar Wilde. De profundis: a severe judgement of Sophocles as a poet

O. Wilde considers that Christ ranks with the poets and Shelley and Sophocles are of his company. Nevertheless, the result of this comparison becomes unfavourable as far Sophocles is concerned, who, precisely as a poet can reach neither the sublimity nor the real tragic sufferance which Christ symbolises. According to Wilde, Christ is both a poet and a tragic poem, while Sophocles is merely a poet, thus being certified the impossibility of the above mentioned comparison.

Oscar Wilde. De profundis: un judici sever de Sòfocles com a poeta

O. Wilde considera que Jesucrist és un veritable poeta i que Shelley i Sòfocles l'acompanyen. Tanmateix, el resultat d'aquesta equiparació és en realitat molt desfavorable per al gran tràgic grec, el qual, precisament com a poeta, mai no podrà assolir les fites de sublimitat i sofrença tràgica real que Jesucrist representa. Segons Wilde, Jesucrist és poeta i poema tràgic, mentre que Sòfocles és un simple poeta, certificant així la impossibilitat de l'equiparació.

New York versus la tragedia y Edipo. El legado sofocleo -y sofístico- en Crimes and Misdemeanors de W. Allen

Más allá de la referencia explícita a la tragedia griega y Edipo, el objetivo de este artículo es presentar la estrecha relación, en opinión del autor, entre lo que mantienen los protagonistas en Crimes and Misdemeanors y las teorías de los sofistas griegos sobre Dios, la ley, etc. Una confrontación minuciosa de sus textos con el guión de la película revela claramente unas raíces sofísticas que no pueden ser atribuidas, en este caso, a la presencia constante del legado judío en la obra de W. Allen.

Indagació edípica i ironia tràgica: el model sofocleu a Cat on a Hot Tin Roof de Tennessee Williams

Atès que el referent clàssic "Èdip en cerca de la seva identitat" ha estat sempre reconegut per a Suddenly Last Summer, l'autor d'aquest article, mitjançant una anàlisi acurada del text del dramaturg americà, proposa de llegir en aquest cas Can on a Hot tin Roof des del model Èdip Rei de Sòfocles i descobrir-hi igualment la tradicional ironia clàssica tant des del punt de vista de l'espectador com dels mateixos personatges principals, Brick i el seu pare, ambdós en cerca de la seva veritat, una veritat, és clar, contrària a la que esperaven.

New York versus Tragedy and Oedipus. The Legacy of Sophocles and the Sophists in Woody Allen's "Crimes and Misdemeanors"

Beyond the explicit reference to the Greek tragedy and Oedipus, the aim of this article is to show the clear relationship, in the author's opinion, between what the protagonists of the film maintain and the theories of the Greek Sophists about God, the law, etc. An accurate analysis both of their texts and the screenplay of Crimes and Misdemeanors reveals different sophistic roots, which, in this case, cannot be attributed to the constant presence of the Jewish legacy in W. Allen's work.

New York versus la tragedia y Edipo. El legado sofocleo -y sofístico- en Crimes and Misdemeanors de W. Allen

Más allá de la referencia explícita a la tragedia griega y Edipo, el objetivo de este artículo es presentar la estrecha relación, en opinión del autor, entre lo que mantienen los protagonistas en Crimes and Misdemeanors y las teorías de los sofistas griegos sobre Dios, la ley, etc. Una confrontación minuciosa de sus textos con el guión de la película revela claramente unas raíces sofísticas que no pueden ser atribuidas, en este caso, a la presencia constante del legado judío en la obra de W. Allen.

New York versus la tragèdia i Èdip. El llegat de Sòfocles -i dels sofistes- a Crimes and Misdemeanors de W. Allen

Més enllà de la referència explícita a la tragèdia grega i Èdip, l'objectiu d'aquest article és presentar l'estreta relació, en opinió del autor, entre allò que mantenen els protagonistes a Crimes and Misdemanors i les teories del sofistes grecs sobre Déu, la llei, etc. Una confrontació acurada dels seus textos amb el guió de la pel·lícula revela clarament unes arrels sofístiques que no poden ser atribuïdes, en aquest cas, a la constant presència del llegat jueu en l'obra de W. Allen.