Background: Giant cell Arteritis (GCA) is the most common systemic vasculitis in patients over 50 years. Diagnosis is based on clinical, laboratory, imaging and biopsy. Temporal artery biopsy (TAB) may be inconclusive in up to 40% of patients. Aim: To describe disease features of patients diagnosed with GCA. Material and Methods: Review of pathology reports of giant cell arteritis and clinical records of patients seen with the diagnosis between 2000 and 2019. Demographic, clinical, laboratory, histopathology, imaging, treatment and follow-up variables were analyzed. Results: We fetched 32 patients with a median age at diagnosis of 70.5 years (range 57-90), 81% women. Twenty eight percent had polymyalgia. 72% had only cranial symptoms, 12% had extracranial involvement and 13% exclusive extracranial involvement. The median time from onset of symptoms to diagnosis was two months (range 0.5-8). All had elevated erythrocyte sedimentation rate and c reactive protein. A TAB was performed in 27 patients and in 17 (65.4%) it confirmed the diagnosis. Transmural inflam- mation was the most frequent finding. All patients received steroids. Follow-up information was available from 25 patients and 92% received a steroid-spa- ring agent, usually methotrexate (74%). Ninety two percent achieved clinical remission in the first year and 59% had minor relapses during steroid tapering. Conclusions: Our patients showed frequent extracranial involvement and TAB was a useful diagnostic tool.

Primary immunodeficiency diseases (PID) are congenital disorders secondary to an impaired immune response. Infections, autoimmune disorders, atopy, and lymphoproliferative syndromes are commonly associated with this disorder. Objective: To present and discuss 3 infants diagnosed with PID. Clinical cases: The cases are presented of three patients with PID diagnosed during their first admission to a Paediatric Intensive Critical Care Unit. The first patient, a 4-month-old infant affected by a severe pneumonia, and was diagnosed as a Severe Combined Immunodeficiency Disease. The second patient was an 8-month-old infant with Candida lusitaniae mesenteric adenitis, and diagnosed with a Chronic Granulomatous Disease. The last patient, a 6-month-old infant presented with ecthyma gangrenosum and X-linked agammaglobulinaemia. Conclusion: PID should be suspected when an infectious disease does not responde to the appropriate therapy within the expected period. An update of each disease is presented.

Las inmunodeficiencias primarias (IDP) son enfermedades congénitas causadas por alteraciones cuantitativas o funcionales de la respuesta inmunitaria. Se caracterizan por predisposición a infecciones, autoinmunidad, alergia y enfermedades linfoproliferativas. Objetivo: Reportar 3 casos de lactantes menores con IDP que se manifestaron como infecciones graves de curso inhabitual. Casos clínicos: Se presentan 3 pacientes diagnosticados como IDP en su estadía en la Unidad de Paciente Crítico Pediátrico. El primero corresponde a un lactante de 4 meses con neumonía multifocal extensa a quien se diagnosticó un síndrome de inmunodeficiencia combinada severa ligada a X; el segundo es un lactante de 8 meses que se manifestó como una adenitis mesentérica por Candida lusitaniae y que correspondió a enfermedad granulomatosa crónica, y el tercero se trata de un lactante de 6 meses que se presentó con un ectima por Pseudomona y se diagnosticó una agammaglobulinemia ligada a X. Conclusión: El diagnóstico de IDP debe sospecharse en presencia de una infección de evolución arrastrada que no responde a tratamiento habitual. Se discuten los casos y se presenta una puesta al día de las patologías diagnosticadas.

OBJECTIVE: Synthesize and assess the available scientific evidence from the period 2008-2012 on interventions of demonstrated efficacy in the treatment and rehabilitation of adolescents and adults engaged in the problematic use of alcohol and other substances. METHODS: A systematic review was undertaken with search and analysis of national and international literature on the subject in Spanish and English in the main international databases: PubMed/MEDLINE, LILACS, Embase, PsycINFO, SciELO, the databases of the York University Centre for Reviews and Dissemination (DARE, ETS Database), the Cochrane Library, and other sources of gray literature. The search criteria included randomized clinical trials and systematic reviews but excluded observational studies, qualitative studies, and articles of poor methodological quality. RESULTS: The final sample consisted of 69 studies. The psychosocial interventions shown to be effective were cognitive behavioral therapy, family interventions, self-help interventions using the Internet, couples behavioral therapy, community strengthening and family training, telephone monitoring and support, and integrated therapy for substance abuse disorder with anxiety and depression comorbidity. Pharmacological interventions of demonstrated effectiveness were acamprosate, lysergic acid diethylamide (LSD), and benzodiazepines in problematic alcohol use, as well as maintenance therapy with high-dose opioids. CONCLUSIONS: The demonstrated effectiveness of psychosocial and pharmacological interventions is slight but significant. However, strongly multidisciplinary interventions that use a cognitive behavioral approach and the involvement of people close to the consumer, as well as some of the specific pharmacological interventions, have been shown to yield the best results in terms of indicators of abstinence and prevention of relapses.

OBJETIVO: Sintetizar y valorar la evidencia científica disponible durante el período 2008-2012, respecto de las intervenciones con eficacia demostrada en el tratamiento y rehabilitación de personas adolescentes y adultas con consumo problemático de alcohol y otras sustancias. MÉTODOS: Se realizó una revisión sistemática con búsqueda y análisis de la literatura nacional e internacional, en español e inglés, consultando las principales bases de datos internacionales, incluyendo PubMed/MEDLINE, LILACS, EMBASE, PsycINFO, SciELO, bases de datos del Centro para Revisión y Diseminación de la Universidad de York (DARE, HTA Database), Cochrane Library y otras fuentes de literatura gris. Se incluyeron ensayos clínicos aleatorizados, revisiones sistemáticas, y se excluyeron estudios observacionales, estudios cualitativos y artículos de baja calidad metodológica. RESULTADOS: La muestra final consistió en 69 estudios. Las intervenciones psicosociales con eficacia demostrada fueron terapia cognitivo conductual; intervenciones de tipo familiar; intervenciones de autoayuda vía Internet; terapia conductual de pareja; refuerzo comunitario y entrenamiento familiar; seguimiento y apoyo telefónico, y terapia integrada del trastorno por abuso de sustancia con comorbilidad ansiosa y depresiva. Las intervenciones farmacológicas con eficacia demostrada fueron acamprosato, dietilamida de ácido lisérgico (LSD) y benzodiacepinas en el consumo problemático de alcohol, y la terapia de mantención con opioides en altas dosis. CONCLUSIONES: La eficacia demostrada de las intervenciones psicosociales y farmacológicas es pequeña, pero significativa. Son las intervenciones marcadamente multidisciplinarias, con enfoque cognitivo conductual e involucramiento de personas significativas allegadas al consumidor, las que han demostrado tener mejores resultados en indicadores de abstinencia y prevención de recaídas, así como también algunas intervenciones farmacológicas específicas.