Thrombotic Microangiophatic in the ICU, postpartum hemolytic uremic syndrome: case report

Underlying injures produced by Hemolytic Uremic Syndrome, a clinical entity defined by the triad, inclu-de:  non-immune hemolytic anemia, thrombocytopenia, and organ involvement which are measured by the Trombotic Systemic Microangiopathy (TSM)  process. The atypical HUS (HUSa) is a subtype of HUS in which the phenomena of TSM are the result of the lack of regulation in alternative pathway of complements on cell surfaces, secondary to a genetic cause. The key role played by the deregulation of the complement system on the damaged endothelial layer in patients with HUSa has been established;   this system measures by multiple mutations and polymorphisms in the genes that encode certain regulatory proteins of the add-in has been established. Taking into account the great complement activity of physiological way during ges-tation, each day more cases of HUSa related to pregnancy, are described.  There is a monoclonal antibody, Eculizumab, which inhibits the terminal complement fraction (C5). Eculizumab blocks the formation of the attack complex of the membrane, with significant long-term improvement in morbidity and mortality asso-ciated with this disease, promoting long-term recovery of renal function and with a significant reduction in the need for dialysis or plasma therapy.A case of 17 year in puerperium, who developed icteric syndrome considered as HELLP syndrome, whose atypical evolution made us think about alternative diagnosis of thrombotic microangiopathy in pregnant woman HUSa type. Discard diagnosis between sepsis, HELLP syndrome and disseminated intravascular coagulation was made.  ADAMST13 measurement was taken, whose standard value allowed us to make the differential diagnosis with a Thrombotic Thrombocytopenic Purpura (TTP). In this way, we were able to achieve the clinical diagnosis of HUSa and begin treatment

Elaboración de procesos para adquisición y obsolencia de equipos médicos en el Hospital Marco Fidel Suárez

El presente trabajo tiene dos ejes principales, como primer eje la creación de una guía para la adquisición de tecnología médica brindando herramientas que ayuden en los procesos de preinversión, inversión y evaluación de tecnología médica en el E.S.E Hospital Marco Fidel Suárez, Teniendo como fase de preinversión, la etapa donde se realizan todos los estudios necesarios para tomar la decisión de adquirir o no un equipo de tecnología biomédica en el hospital. Esta fase consta de dos procesos: el primero consiste en la identificación del problema, la identificación de necesidades por servicio, el diagnóstico del equipo existente y el proceso de calificación y justificación de los requerimientos de equipo, en el segundo proceso, un estudio de costos comparativos para garantizar la factibilidad de la inversión. También la fase de Inversión la cual corresponde a todo el proceso en el que el Hospital empieza a destinar dineros en la adquisición de la tecnología. Este proceso abarca etapas como de negociación, contratación e importación. En la etapa final del proceso de adquisición se tiene la fase de evaluación, en esta fase se trata de evaluar que tan beneficioso ha sido la adquisición de la tecnología y como ha sido una solución a los problemas específicos del Hospital, esta evaluación se suele hacer mediante indicadores. El segundo eje de este proyecto está enfocado en la creación de un algoritmo para evaluar la obsolescencia, la cual es una fase del ciclo de vida de los equipos médicos, consecuencia de la influencia que ejerce el progreso tecnológico en el equipamiento médico, al producir equipos más baratos, de menor volumen, más silenciosos y construidos con materiales de mayor calidad, que eventualmente proporcionan mayor seguridad, eficacia y eficiencia en el tratamiento de los pacientes. No existe normatividad que obligue a la creación de un tipo específico de protocolo para el proceso de obsolescencia, por lo que es necesario que tanto el personal asistencial, técnico y administrativo logren un balance costo-beneficio que cumpla con los objetivos establecidos por el hospital. El algoritmo creado se implementó sobre 566 equipos del inventario del Hospital, con el fin de determinar que equipos médicos se deben reemplazar y cuáles equipos el algoritmo sugiere realizarles un seguimiento anual para garantizar las condiciones de seguridad y economía del Hospital. Este algoritmo toma como base 3 etapas para el análisis, una etapa técnica que da una idea sobre el estado en que se encuentran los equipos médicos, una etapa clínica que indica la importancia y porcentaje de uso del equipo, además, el grado de satisfacción del personal asistencial con el equipo. También se tiene una etapa tecnológica que da idea de económicamente que tan rentable es tener el equipo médico en funcionamiento. Palabras clave: Adquisición, equipos médicos, renovación, inversión, obsolescencia

Troca plasmática terapêutica em doenças reumáticas : a experiência de um hospital universitário

Introdução: Diariamente acumulam-se evidências relacionadas com a utilização da troca plasmática terapêutica (TPT) em pacientes com doenças reumáticas. O Hospital Universitário San Ignacio registrou todas as sessões de TPT realizadas pelo grupo de aférese desta instituição. Objetivo: Descrever a experiência do Hospital Universitário San Ignacio na TPT em pacientes com doenças reumatológicas. Métodos: Trata-se de uma análise observacional, retrospectiva, descritiva. Este estudo incluiu análises das sessões de TPT realizadas em pacientes com doenças reumáticas de novembro de 2009 a novembro de 2013. Resultados: O grupo de aférese realizou 136 sessões em 27 pacientes. A idade média dos pacientes foi de 43 anos (DP 18,5) e 59,3% dos pacientes eram do sexo feminino. Quanto ao diagnóstico, os mais frequentes foram: vasculite associada ao ANCA seguida de lúpus eritematoso sistêmico e síndrome antifosfolipídica catastrófica. A quantidade média de sessões por paciente foi de 5 (DP 1,8) e a média de troca plasmática por paciente foi de 1,3 unidades de substituição do volume de plasma. A solução de substituição mais utilizada foi o plasma fresco congelado (PFC, 63,2% das sessões). De todas as sessões, 4,4% apresentaram complicações, a maioria delas relacionadas com o acesso vascular. Quinze pacientes necessitaram de terapia de substituição renal (TSR) secundária à mesma causa que levou à necessidade de TPT; três pacientes necessitaram de TSR em decorrência de outras causas além da intervenção diagnóstica de TPT e um paciente tinha sido submetido à diálise crônica. Conclusões: A TPT é uma alternativa terapêutica que é necessária para o manejo de pacientes com doenc¸as reumáticas com envolvimento renal e daqueles que são refratários ao tratamento convencional. Os resultados clínicos do presente estudo estão de acordo com o que é encontrado na literatura global.Q3Artículo original397-402Introduction: Each day, evidence accumulates related to the use of therapeutic plasma exchange (TPE) in patients with rheumatic diseases. San Ignacio University Hospital has recorded all of the TPE sessions performed by the institution’s apheresis group. Objective: To describe the TPE experience of patients with rheumatologic diseases in a hospital setting. Methods: Descriptive, observational, retrospective analysis. This study included analyses of the TPE sessions that were performed in patients with rheumatic diseases from November 2009 to November 2013. Results: The apheresis group performed 136 sessions in 27 patients. The mean patient age was 43 years (SD 18.5), and 59.3% of the patients were female. Regarding the diagnosis, the most frequents ones where: ANCA-associated vasculitis followed by systemic lupus erythematosus and catastrophic antiphospholipid syndrome. The average number of sessions per patient was 5 (SD 1.8), and the average plasma exchange per patient was 1.3 plasma volume replacement units. The most used replacement solution was frozen fresh plasma (FFP; 63.2% of the sessions). Of all the sessions, 4.4% presented complications, and the majority of the complications were related to vascular access. Fifteen patients required renal replacement therapy (RRT) secondary to the same cause that created the need for TPE, 3 patients required RRT due to causes other than the TPE diagnostic intervention and 1 patient had undergone chronic dialysis. Conclusions: TPE is a therapeutic alternative that is needed for the management of patients with rheumatic diseases with renal involvement and those who are refractory to conventional management. Our clinical results were in agreement with the global literatur

Modelling genetic evaluation for dressage in Pura Raza Español horses with focus on the rider effect

The most popular use of the Pura Raza Espanol horse in sport is for dres- ~ sage competitions. Tests on young sport horses were first established in 2004 in Spain to collect data for the genetic evaluation of this breed’s suitability for dressage. The aim of this study was to compare eight different models to find out the most appropriate way to include the rider in the genetic evaluation of dressage. A progressive removal of systematic effects from model was also analysed. A total of 8867 performance records collected between 2004 and 2011 from 1234 horses aged between 4 and 6 years old were used. The final score in the dressage test was used as the performance trait. The pedigree matrix contained 8487 individuals. A BLUP animal model was applied using a Bayesian approach with TM software. The horse’s age, gender, travelling time, training level, stud of birth and event were included as systematic effects in all the models. Apart from the animal and residual effects that were present in all models, different models were compared combining random effects such as the rider, match (i.e. rider–horse interaction) and permanent environmental effects. A cross-validation approach was used to evaluate the models’ prediction ability. The best model included the permanent environmental, rider and match random effects. As far as systematic effects are concerned, the event or the stud of birth was essential effects needed to fit the data

Application of individual increase in inbreeding to estimate realized effective sizes from real pedigrees

The objective of this study was to test the performance of a recently proposed methodology for the estimation of realized effective size (N(e)) based on individual increase in inbreeding (DeltaF(i)) on several real pedigrees: (a) an experimental mice population; (b) a closed pedigree of fighting bulls; (c) the Spanish Purebred (SPB, Andalusian) horse pedigree; (d) the Carthusian strain of SPB pedigree; (e) the Spanish Arab horse pedigree; and (f) the Spanish Anglo-Arab horse pedigree. Several reference subpopulations were defined on the basis of generation length in order to consider only animals in the last generation, to assess the influence of the pedigree content on the estimates of N(e). The estimates of realized N(e) computed from DeltaF(i) (Ne) tended to be higher than those obtained from regression on equivalent generations. The new parameter Ne remained approximately stable when pedigree depth achieved about five equivalent generations. Estimates of take into account the genetic history of the populations, the size of their founder population, and the mating policy or bottlenecks caused by poor use of reproducing individuals. The usefulness of the realized N(e) computed from individual increase in inbreeding in real pedigrees is also discusse

Therapeutic plasmapheresis

Introducción: cada día hay más evidencia acerca de la utilidad de la plasmaféresis en diferentes entidades clínicas. Existen múltiples registros internacionales de plasmaféresis; sin embargo, la información en Colombia e incluso en Latinoamérica es limitada. Objetivo: realizar análisis descriptivo de la experiencia en plasmaféresis de una institución académica en Bogotá, Colombia. Analizar las características de la técnica, indicaciones, complicaciones y características demográficas de los pacientes. Material y métodos: se realiza un análisis descriptivo de las sesiones de plasmaféresis realizadas en el Hospital Universitario San Ignacio, en pacientes mayores de 18 años durante el periodo comprendido entre agosto de 2008 hasta agosto de 2011. Todas las sesiones se realizaron mediante la técnica de filtración transmembrana y no se usó anticoagulante en ninguna de ellas. Resultados: se analizaron 278 sesiones de plasmaféresis en 33 pacientes adultos durante un período de tres años. 69.7% de los pacientes eran mujeres, el promedio de edad era 42 años. 57% de las sesiones se realizaron en pacientes con diagnóstico de miastenia gravis y hemorragia alveolar. El volumen promedio de intercambios plasmáticos fue 1.28. El 9.3% de las sesiones presentaron al menos una complicación. Se documentó frecuentemente hipotensión arterial en las sesiones realizadas con albúmina al 3.5%. 12% de los registros de electrolitos tenían hipocalcemia y 47% hipermagnesemia. No hubo muertes relacionadas con la terapia y pese al no uso de anticoagulante, no se presentó coagulación del circuito en ninguna de las sesiones. Conclusiones: dada nuestra experiencia consideramos que la plasmaféresis es una terapia segura. Es necesario evaluar la necesidad de la administración de anticoagulante al circuito de plasmaféresis, dado que no se documentó coagulación de éste en ninguna sesión y su uso podría estar asociado a eventos adversos. El monitoreo continuo de los electrolitos es fundamental y la reposición de los mismos debe ser individualizada.Artículo original29-34Introduction: there is growing evidence for the usefulness of plasmapheresis in different clinical entities. There are many international records of plasmapheresis, but the information in Colombia and even in Latin America, is limited. Objective: to perform a descriptive analysis of the experience in plasmapheresis of an academic institution in Bogotá, Colombia. To analyze the characteristics of the technique, indications, complications and demographic characteristics of patients. Material and methods: descriptive analysis of plasmapheresis sessions performed in the Hospital Universitario San Ignacio in patients older than 18 years during the period from August 2008 to August 2011. All sessions were conducted by the technique of transmembrane filtration. No anticoagulation was used in any of them. Results: 278 sessions of plasmapheresis were analyzed in 33 adult patients over a period of three years. 69.7% of patients were female, with mean age of 42 years. 57% of the sessions were conducted in patients diagnosed with myasthenia gravis and alveolar hemorrhage. The average volume of plasma exchange was 1.28. 9.3% of the sessions presented at least one complication. Hypotension was frequently documented in the sessions performed with 3.5% albumin. 12% of electrolytes records hadhypocalcemia and 47% hypermagnesemia. There were no therapy-related deaths and despite the non-use of anticoagulant, no clotting of the circuit in any of the sessions was presented. Conclusions: Given our experience, we believe that plasmapheresis is a safe therapy. It is necessary to assess the need for administration of anticoagulant to the plasmapheresis circuit since no clotting in it in any session was documented and its use could be associated with adverse events. Continuous monitoring of electrolytes is essential and its replacement should be individualized

Individual increase in inbreeding allows estimating effective sizes from pedigrees

We present here a simple approach to obtain reliable estimates of the effective population size in real world populations via the computation of the increase in inbreeding for each individual (delta F-i) in a given population. The values of delta Fi are computed as t-root of 1 - (1 - F-i) where F-i is the inbreeding coefficient and t is the equivalent complete generations for each individual. The values of delta F computed for a pre-defined reference subset can be averaged and used to estimate effective size. A standard error of this estimate of N-e can be further computed from the standard deviation of the individual increase in inbreeding. The methodology is demonstrated by applying it to several simulated examples and to a real pedigree in which other methodologies fail when considering reference subpopulations. The main characteristics of the approach and its possible use are discussed both for predictive purposes and for analyzing genealogies

Síndrome hemolítico urémico atípico posterior a trasplante renal: presentación de un caso y revisión de la literatura

Haemolytic uremic syndrome (HUS) is a clinical entity characterized by the appearance of non immune hemolytic anemia, thrombocytopenia and acute renal failure. It is a disease belonging to the group of thrombotic microangiopathy (MAT) which are part of thrombotic thrombocytopenic purpura also (PTT) and some other MAT associated with other medical conditions formerly known as secondary MAT.Moreover, the variety known as atypical HUS (aHUS) is an ultra-orphan disease that frequently progresses to chronic renal failure (CRF) and is associated with high morbidity and mortality if not properly treated. If a patient presents its first clinical manifestation of aHUS later receive a cadaveric renal transplant which not only makes it an even more exotic case but involves more complexity in their management is presented.El síndrome hemolítico urémico (SHU) es una entidad clínica caracterizada por la aparición de anemia hemolítica no inmune, trombocitopenia e insuficiencia renal aguda. Se trata de una enfermedad perteneciente al grupo de las microangiopatías trombóticas (MAT) de la que hacen parte también la purpura trombocitopénica trombótica (PTT) y algunas otras MAT asociadas a otras condiciones médicas antes conocidas como MAT secundarias. Por otra parte, la variedad conocida como SHU atípico (SHUa) es una patología ultra-huérfana que frecuentemente evoluciona a insuficiencia renal crónica (IRC) y se asocia con elevada morbimortalidad si no recibe el tratamiento adecuado. Se examina el caso de un paciente que presenta su primera manifestación clínica de síndrome hemolítico urémico atípico después de trasplante renal cadavérico lo cual no solo lo hace un caso aún más exótico, sino que implica mayor complejidad en su manejo. &nbsp

Different types domains are present in complex I from immature seeds and of CA adult plants in Arabidopsis thaliana

Mitochondrial Nicotinamide adenine dinucleotide (NADH) dehydrogenase complex is the first complex of the mitochondrial electron transfer chain. In plants and in a variety of eukaryotes except Opisthokonta, complex I (CI) contains an extra spherical domain called carbonic anhydrase (CA) domain. This domain is thought to be composed of trimers of gamma type CA and CA-like subunits. In Arabidopsis, the CA gene family contains five members (CA1, CA2, CA3, CAL1 and CAL2). The CA domain appears to be crucial for CI assembly and is essential for normal embryogenesis. As CA and CA-like proteins are arranged in trimers to form the CA domain, it is possible for the complex to adopt different arrangements that might be tissue-specific or have specialized functions. In this work, we show that the proportion of specific CI changes in a tissue-specific manner. In immature seeds, CI assembly may be indistinctly dependent on CA1, CA2 or CA3. However, in adult plant tissues (or tissues derived from stem cells, as cell cultures), CA2-dependent CI is clearly the most abundant. This difference might account for specific physiological functions. We present evidence suggesting that CA3 does not interact with any other CA family member. As CA3 was found to interact with CI FRO1 (NDUFS4) subunit, which is located in the matrix arm, this suggests a role for CA3 in assembly and stability of CI.Fil: Córdoba, Juan Pablo. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Mar del Plata. Instituto de Investigaciones Biológicas. Universidad Nacional de Mar del Plata. Facultad de Ciencias Exactas y Naturales. Instituto de Investigaciones Biológicas; ArgentinaFil: Fassolari, Marisol. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Mar del Plata. Instituto de Investigaciones Biológicas. Universidad Nacional de Mar del Plata. Facultad de Ciencias Exactas y Naturales. Instituto de Investigaciones Biológicas; ArgentinaFil: Marchetti, Maria Fernanda. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Mar del Plata. Instituto de Investigaciones Biológicas. Universidad Nacional de Mar del Plata. Facultad de Ciencias Exactas y Naturales. Instituto de Investigaciones Biológicas; ArgentinaFil: Soto, Débora. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Mar del Plata. Instituto de Investigaciones Biológicas. Universidad Nacional de Mar del Plata. Facultad de Ciencias Exactas y Naturales. Instituto de Investigaciones Biológicas; ArgentinaFil: Pagnussat, Gabriela Carolina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Mar del Plata. Instituto de Investigaciones Biológicas. Universidad Nacional de Mar del Plata. Facultad de Ciencias Exactas y Naturales. Instituto de Investigaciones Biológicas; ArgentinaFil: Zabaleta, Eduardo Julian. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Mar del Plata. Instituto de Investigaciones Biológicas. Universidad Nacional de Mar del Plata. Facultad de Ciencias Exactas y Naturales. Instituto de Investigaciones Biológicas; Argentin

Population history and genetic variability in the Spanish Arab Horse assessed via pedigree analysis

The studbook of the Spanish Arab Horse was analysed in order to assess the available genetic variability in the population via genealogical analyses and to ascertain the influence of different countries in the formation of the population. The Spanish Arab horse studbook could be traced back to the late 19th century with a total of 18,880 animals. A 15.7% (2965) of the registered individuals were Arab horses imported from foreign populations. The total number of founders was 1626: from these, 1495 individuals were identified as imported from foreign countries. The sum of the genetic contributions of the founders to the population revealed that those imported from the ‘Middle East’, Poland, the United Kingdom and Egypt had a higher importance in the formation of the Spanish Arab Horse, contributing, respectively, 36.4%, 18.7%, 11.9% and 9.1% of the genetic variability. The effective number of founders was 38.6 and the effective number of ancestors was 19 thus characterising the abusive use of a little number of individuals for reproduction. This was due to a breeding policy that included mating between relatives, but also due to the limited availability of reproductive individuals after the Spanish Civil War. The average inbreeding of the population was 7.0% (9.8% for the individuals born during 1995–2004). Individuals with very high levels of inbreeding (≥12.5%) were 17.7% in the whole population and 26.7% of the individuals born in the last decade. Positive genealogical FIS near 2% characterised a breeding policy including matings between relatives. No clear within-population differentiation (inferred via genealogical FST) could be assessed between sire lines (FST = 0.01) and no differentiation between geographical areas existed. Overall, the Spanish Arab Horse can be described as a population without genetic subdivision and with a mating policy and historical constraints that reduced the available genetic variability regardless of the relatively large size of its founder population. The obtained information will be useful to implement a selection program in the Spanish Arab Horse