Genetic basis of Sjögren's syndrome. How strong is the evidence?

Sjögren's syndrome (SS) is a late-onset chronic autoimmune disease (AID) affecting the exocrine glands, mainly the salivary and lachrymal. Genetic studies on twins with primary SS have not been performed, and only a few case reports describing twins have been published. The prevalence of primary SS in siblings has been estimated to be 0.09% while the reported general prevalence of the disease is approximately 0.1%. The observed aggregation of AIDs in families of patients with primary SS is nevertheless supportive for a genetic component in its etiology. In the absence of chromosomal regions identified by linkage studies, research has focused on candidate gene approaches (by biological plausibility) rather than on positional approaches. Ancestral haplotype 8.1 as well as TNF, IL10 and SSA1 loci have been consistently associated with the disease although they are not specific for SS. In this review, the genetic component of SS is discussed on the basis of three known observations: (a) age at onset and sex-dependent presentation, (b) familial clustering of the disease, and (c) dissection of the genetic component. Since there is no strong evidence for a specific genetic component in SS, a large international and collaborative study would be suitable to assess the genetics of this disorder.

Experience in the Surgical Management of Abdominal Compartment Syndrome in the Hospital Occidente de Kennedy

The abdominal compartment syndrome (ACS) is the result of various physiological alterations produced by an abnormal increase of the intra-abdominal pressure. Some of these patients will undergo a surgical procedure for its management. Methods: This is a retrospective case series of 28 patients with ACS who required surgical treatment at the Hospital Occidente de Kennedy between 1999 and 2003. We assessed retrospectively the behavior of McNelis’s equation for prediction of the development of the ACS. Results: The leading cause of ACS in our study was intraabadominal infection (n=6 21,4%). Time elapsed between diagnosis and surgical decompression was less than 4 hours in 75% (n=21) of the cases. The variables that improved significantly after the surgical decompression were CVP (T: 4,0 p: 0,0001), PIM (T: 2,7; p: 0,004), PIA (T1,8; p:0,034) and Urine Output (T:-2,4; p:0,02). The values of BUN, Creatinine and the cardiovascular instability did not show improvement. The ICU and hospital length of stay were 11 days (SD: 9) and 18 days (SD13) respectively. Global mortality was 67,9% (n=19) and mortality directly attributable to the syndrome was 30% (n=8). The behavior of the McNelis’s equation was erratic. Conclusions: The demographic characteristics as well as disease processes associated with ACS are consistent with the literature. The association between physiological variables and ACS is heterogeneous between patients. Mortality rates attributable to ACS in our institution are within the range described world-wide. The behavior of the McNelis’s equation seems to depend greatly upon fluid balance.

Anestesia regional y periférica guiada por ultrasonido en el paciente critico

El presente trabajo es un capítulo de libro titulado “Anestesia Regional y Periférica Guiada por Ultrasonido en el Paciente Crítico” que será incluido en la última edición del libro “Manual de Ultrasonido en Terapia Intensiva y Emergencias” cuyo editor es el Doctor José de Jesús Rincón Salas y que será publicado por la Editorial Prado de México para distribución latinoamericana desde dicho país. Por solicitud del editor y teniendo en cuenta el enfoque del libro, el presente trabajo está dirigido a estudiantes de formación, médicos graduados y especialistas en las áreas de cuidado intensivo, anestesiología, dolor, medicina interna y medicina de urgencias. Tiene como propósito empapar de conocimientos necesarios y prácticos en anestesia regional a personas que usualmente no han tenido contacto con la anestesia regional, pues desafortunadamente sólo en los últimos años ha sido posible que la anestesia regional haya comenzado a salir de las salas de cirugía, ámbito donde ha estado confinada tradicionalmente. El lenguaje utilizado es sencillo y el capítulo ha sido escrito para que sea fácil de leer y consultar, dejando así mensajes muy claros sobre la utilidad, viabilidad e implicaciones que tiene el uso de anestesia regional guiada por ultrasonido en cuidado intensivo. Los autores esperamos que de esta manera, el presente capítulo permita continuar superando los obstáculos que se interponen entre los invaluables beneficios de la anestesia regional y los pacientes de cuidado intensivo que necesitan de ella.