International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia.

BACKGROUND: HHT is an autosomal dominant disease with an estimated prevalence of at least 1/5000 which can frequently be complicated by the presence of clinically significant arteriovenous malformations in the brain, lung, gastrointestinal tract and liver. HHT is under-diagnosed and families may be unaware of the available screening and treatment, leading to unnecessary stroke and life-threatening hemorrhage in children and adults. OBJECTIVE: The goal of this international HHT guidelines process was to develop evidence-informed consensus guidelines regarding the diagnosis of HHT and the prevention of HHT-related complications and treatment of symptomatic disease. METHODS: The overall guidelines process was developed using the AGREE framework, using a systematic search strategy and literature retrieval with incorporation of expert evidence in a structured consensus process where published literature was lacking. The Guidelines Working Group included experts (clinical and genetic) from eleven countries, in all aspects of HHT, guidelines methodologists, health care workers, health care administrators, HHT clinic staff, medical trainees, patient advocacy representatives and patients with HHT. The Working Group determined clinically relevant questions during the pre-conference process. The literature search was conducted using the OVID MEDLINE database, from 1966 to October 2006. The Working Group subsequently convened at the Guidelines Conference to partake in a structured consensus process using the evidence tables generated from the systematic searches. RESULTS: The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.

Graded contrast echocardiography in pulmonary arteriovenous malformations.

To compare the results of transthoracic contrast echocardiography (TTCE) adding a grading scale with the results of thoracic computed tomography (CT) in order to optimise the use of both techniques. 95 patients with hereditary haemorrhagic telangiectasia (HHT) were examined with TTCE and thoracic CT to detect pulmonary arteriovenous malformations (PAVMs). According to previous studies, TTCE was divided into a four grade scale depending on the degree of opacification of the left ventricle after the administration of a contrast agent. Of the 95 patients (50.5% female; mean age 46 yrs), none with normal or grade 1 TTCE had detectable PAVMs on thoracic CT. Shunts of grades 2, 3 and 4 were associated with PAVMs according to thoracic CT in 25, 80, and 100% of the cases. There was a statistically significant association between the TTCE grade and the detection of a PAVM by thoracic CT. There were also statistically significant associations between TTCE grade and the cardiac cycle when the contrast was first visible in the left atrium, and size of the feeding artery. Graded TTCE and timing of left atrium opacification may be useful techniques in selecting HHT patients for PAVM screening with thoracic CT scans.

Modelo de cirugía mayor ambulatoria invasive de gastrostomía laparotómica para alimentación y hospitalización a domicilio / Model of invasive ambulatory major surgery of laparotomic gastrostomy for feeding and home hospitalization

To evaluate the model of early hospital discharge offered by the home hospitalization unit (HAD), with safety and cost-efficiency criteria in patients undergoing minimally invasive ambulatory gastric bypass surgery. With this joint program, invasive surgery can be performed without admission and taking the hospital to the patient's home can be a reality

Evaluar el modelo asistencial de alta temprana hospitalaria que ofrece la unidad de hospitalización a domicilio (HAD), con criterios de seguridad y de costo-eficiencia en pacientes sometidos a cirugía mayor gástrica ambulatoria mínimamente invasiva. Con este programa conjunto se puede realizar la cirugía invasiva sin ingreso y llevar el hospital al domicilio del paciente puede ser una realidad

Multifactorial thrombotic-type microangiopathy with skin ulcers and hepatitis C infection.

The case of a patient with hepatitis C virus infection who presented with symmetric ulcers of the legs is reported. She was found to have type III mixed cryoglobulinemia, high titers of anticardiolipin antibodies, lupus anticoagulant, and a free protein S deficiency. To our knowledge, this is the first reported case of such an association. The role of these factors in the pathogenesis of skin lesions in the setting of hepatitis C virus infection is discussed.

Cambios y tendencias en Medicina Interna 1987-1996 / Changes and tendencies in internal medicine 1987-1996

Fundamento: En medios hospitalarios, los internistas, suponemos que los pacientes ingresados en los Servicios de Medicina Interna (MI) actualmente son de más edad, están afectados por más enfermedades y que estas son más crónicas e invalidantes que las que asistíamos pocos años antes. Material y Métodos: Con el objeto de mostrar que cambios ha habido, realizamos un análisis de algunas variables de epidemiología clínica y de gestión hospitalaria de los ingresos de una Unidad de MI. Los periodos del estudio son el último semestre de los años 1987 y 1988 y del año 1996. Analizamos las tendencias habidas en el intervalo y relacionamos la población hospitalaria con la demográfica de la provincia que atiende el hospital. Resultados: La media de la edad de los pacientes asistidos en la actualidad es avanzada (70 años), y más del 30 por ciento superan los 80 años. El promedio de la edad del primer periodo (64,5 años), se ha incrementado en 5,5 años, en el caso de las mujeres 5,8 años y de 4,5 años en el de los varones. En ambos periodos la media de la edad de las mujeres es 5,5 años superior al de ellos. El envejecimiento de la población de MI, es muy superior al demográfico de la región que atiende el hospital. El índice de diagnósticos por ingreso aumentó, de 3,2 a 3,8 y la proporción de enfermos sin enfermedad de base crónica disminuyó, del 9,4 por ciento al 16,5 por ciento. El índice de mortalidad no varió, en ambos periodos, (11,6 por ciento y 11,1 por ciento). Conclusiones: La complejidad de los enfermos que asistimos ha aumentado, a pesar de ello, algunos de los parámetros de gestión como la estancia media disminuyó, otros como "reingresos y adecuación de los ingresos" mantienen niveles parecidos (AU)