Eficacia y seguridad de un compuesto de ácidos grasos hiperoxigenados en la mejora de la microcirculación de la púrpura fulminante en pacientes pediátricos con sepsis: estudio piloto / Efficacy and safety of a hyperoxygenated fatty acid compound in improving the microcirculation of purpura fulminans in paediatric patients with sepsis: a pilot study

Introducción La púrpura fulminante (PF) es una complicación grave de la sepsis resultado de un conjunto de alteraciones caracterizadas por el desarrollo de lesiones hemorrágicas equimóticas y necrosis de la piel. Objetivo Analizar la eficacia y la seguridad de la aplicación tópica de un compuesto de ácidos grasos hiperoxigenados (AGHO) en la microcirculación de la PF en pacientes pediátricos afectados de sepsis. Material y métodos Se llevó a cabo un estudio prospectivo cuasiexperimental pretest-posttest de un solo grupo en una Unidad de Cuidados Intensivos Pediátrica (UCIP). Se incluyeron en el estudio pacientes con PF de 0 a 18 años. Para evaluar la efectividad del producto objeto de evaluación en la microcirculación de las lesiones por PF se determinaron los valores de oximetría somática antes y después de la aplicación de AGHO cada 4h durante los 3 primeros días de ingreso de los pacientes. Resultados Se reclutaron 4 pacientes, con una mediana de edad de 98 meses. Las lesiones purpúricas que se midieron estaban ubicadas, sobre todo, en ambos pies y manos y, en 2 pacientes, también en los maléolos laterales y gemelos de ambas extremidades inferiores. Se obtuvieron un total de 225 mediciones, observando unas puntuaciones medias preintervención de 71,17±15,65% versus los 73,68±14,83% postintervención. Se objetivó significación estadística (p<0,001) al comparar las mediciones pre- y postintervención. Conclusiones La aplicación precoz y continuada de AGHO en el manejo de la PF por sepsis es una práctica eficaz y segura en los casos de población pediátrica analizada. En más de la mitad de los episodios analizados se objetivó un aumento de microcirculación tisular tras la aplicación de los AGHO, sin eventos adversos. (AU)

Introduction Purpura fulminans (PF) is a serious complication of sepsis resulting from a set of alterations characterised by the development of ecchymotic haemorrhagic lesions and skin necrosis. Aim To analyse the efficacy and safety of the topical application of HOFA compound, in the cutaneous microcirculation of PF lesions in paediatric patients affected by sepsis. Material and methods A prospective quasi-experimental pre-test/post-test single-group conducted in a Paediatric Intensive Care Unit of a third level hospital was performed. Paediatric patients aged 0-18 years with sepsis were included. Somatic oximetry values were measured before and after application of HOFAs every 4hours over the first three days of the patients’ hospitalisation. Patient's socio-demographic and clinical variables and somatic oximetry by placing a sensor for measuring tissue perfusion on the area with PF were determined. Results Four patients were recruited, with a median age of 98 months. The purpuric lesions measured were mainly located on both feet and hands and, in two patients, also on the lateral malleoli and calves of both lower extremities. A total of 225 measurements were obtained, with mean pre-intervention scores of 71.17±15.65% versus 73.68±14.83% post-intervention. Statistical significance (p<0.001) was observed upon comparison of the pre- and post-intervention measurements. Conclusions Early and continued application of HOFAs in the management of sepsis-induced PF is an effective and safe practice in the cases analysed. In more than half of the episodes analysed, an increase in tissue microcirculation was observed after the application of HOFAs, with no adverse events. (AU)

Eficacia y seguridad de un compuesto de ácidos grasos hiperoxigenados en la mejora de la microcirculación de la púrpura fulminante en pacientes pediátricos con sepsis: estudio piloto / Efficacy and safety of a hyperoxygenated fatty acid compound in improving the microcirculation of purpura fulminans in paediatric patients with sepsis: a pilot study

Introducción La púrpura fulminante (PF) es una complicación grave de la sepsis resultado de un conjunto de alteraciones caracterizadas por el desarrollo de lesiones hemorrágicas equimóticas y necrosis de la piel. Objetivo Analizar la eficacia y la seguridad de la aplicación tópica de un compuesto de ácidos grasos hiperoxigenados (AGHO) en la microcirculación de la PF en pacientes pediátricos afectados de sepsis. Material y métodos Se llevó a cabo un estudio prospectivo cuasiexperimental pretest-posttest de un solo grupo en una Unidad de Cuidados Intensivos Pediátrica (UCIP). Se incluyeron en el estudio pacientes con PF de 0 a 18 años. Para evaluar la efectividad del producto objeto de evaluación en la microcirculación de las lesiones por PF se determinaron los valores de oximetría somática antes y después de la aplicación de AGHO cada 4h durante los 3 primeros días de ingreso de los pacientes. Resultados Se reclutaron 4 pacientes, con una mediana de edad de 98 meses. Las lesiones purpúricas que se midieron estaban ubicadas, sobre todo, en ambos pies y manos y, en 2 pacientes, también en los maléolos laterales y gemelos de ambas extremidades inferiores. Se obtuvieron un total de 225 mediciones, observando unas puntuaciones medias preintervención de 71,17±15,65% versus los 73,68±14,83% postintervención. Se objetivó significación estadística (p<0,001) al comparar las mediciones pre- y postintervención. Conclusiones La aplicación precoz y continuada de AGHO en el manejo de la PF por sepsis es una práctica eficaz y segura en los casos de población pediátrica analizada. En más de la mitad de los episodios analizados se objetivó un aumento de microcirculación tisular tras la aplicación de los AGHO, sin eventos adversos. (AU)

Introduction Purpura fulminans (PF) is a serious complication of sepsis resulting from a set of alterations characterised by the development of ecchymotic haemorrhagic lesions and skin necrosis. Aim To analyse the efficacy and safety of the topical application of HOFA compound, in the cutaneous microcirculation of PF lesions in paediatric patients affected by sepsis. Material and methods A prospective quasi-experimental pre-test/post-test single-group conducted in a Paediatric Intensive Care Unit of a third level hospital was performed. Paediatric patients aged 0-18 years with sepsis were included. Somatic oximetry values were measured before and after application of HOFAs every 4hours over the first three days of the patients’ hospitalisation. Patient's socio-demographic and clinical variables and somatic oximetry by placing a sensor for measuring tissue perfusion on the area with PF were determined. Results Four patients were recruited, with a median age of 98 months. The purpuric lesions measured were mainly located on both feet and hands and, in two patients, also on the lateral malleoli and calves of both lower extremities. A total of 225 measurements were obtained, with mean pre-intervention scores of 71.17±15.65% versus 73.68±14.83% post-intervention. Statistical significance (p<0.001) was observed upon comparison of the pre- and post-intervention measurements. Conclusions Early and continued application of HOFAs in the management of sepsis-induced PF is an effective and safe practice in the cases analysed. In more than half of the episodes analysed, an increase in tissue microcirculation was observed after the application of HOFAs, with no adverse events. (AU)

Efficacy and safety of a hyperoxygenated fatty acid compound in improving the microcirculation of purpura fulminans in paediatric patients with sepsis: a pilot study.

INTRODUCTION: Purpura fulminans (PF) is a serious complication of sepsis resulting from a set of alterations characterised by the development of ecchymotic haemorrhagic lesions and skin necrosis. AIM: To analyse the efficacy and safety of the topical application of HOFA compound, in the cutaneous microcirculation of PF lesions in paediatric patients affected by sepsis. MATERIAL AND METHODS: A prospective quasi-experimental pre-test/post-test single-group conducted in a Paediatric Intensive Care Unit of a third level hospital was performed. Paediatric patients aged 0-18 years with sepsis were included. Somatic oximetry values were measured before and after application of HOFAs every 4h over the first three days of the patients' hospitalisation. Patient's socio-demographic and clinical variables and somatic oximetry by placing a sensor for measuring tissue perfusion on the area with PF were determined. RESULTS: Four patients were recruited, with a median age of 98 months. The purpuric lesions measured were mainly located on both feet and hands and, in two patients, also on the lateral malleoli and calves of both lower extremities. A total of 225 measurements were obtained, with mean pre-intervention scores of 71.17±15.65% versus 73.68±14.83% post-intervention. Statistical significance (p<0.001) was observed upon comparison of the pre- and post-intervention measurements. CONCLUSIONS: Early and continued application of HOFAs in the management of sepsis-induced PF is an effective and safe practice in the cases analysed. In more than half of the episodes analysed, an increase in tissue microcirculation was observed after the application of HOFAs, with no adverse events.

Asignatura sobre el cuidado de las personas con lesiones cutáneas en el grado de enfermería: competencias y propuesta de contenidos / A course on care for people with skin lesions for the undergraduate nursing programme: competencies and proposed contents

El presente artículo pretende desarrollar una propuesta marco sobre una asignatura sobre el cuidado de las personas con lesiones cutáneas en el grado de enfermería. Esta ha sido realizada por consenso de expertos, profesores con experiencia en el cuidado de heridas y fue sometida a modificaciones en el marco del Segundo Encuentro Iberolatinoamericano de Facultades y Escuelas Universitarias con Programas Formativos en Heridas. Con ello se presenta una propuesta de competencias sobre el cuidado de las personas con lesiones cutáneas para la enfermera graduada, así como la propuesta curricular, con su estructura pedagógica basada en unidades didácticas y la propuesta de carga docente y ubicación de la asignatura de cuidados de las personas con lesiones cutáneas en el grado en enfermería. Finalmente se presentan documentos de apoyo al profesorado y estudiantado para la creación de la asignatura de cuidados de las personas con lesiones cutáneas para el grado en enfermería (AU)

This article aims to develop a framework proposal for a course on the care of people with skin lesions in the nursing degree. It has been developed by consensus of experts, professors with experience in wound care and was subject to modifications in the framework of the Segundo Encuentro Iberolatinoamericano de Facultades y Escuelas Universitarias con Programas Formativos en Heridas. This includes a proposal of competencies on the care of people with skin lesions for the graduate nurse, as well as the Curricular proposal, with its pedagogical structure based on didactic units and the proposed teaching load and location of the subject of care for people with skin lesions in the nursing degree. Finally, support documents are presented for the teaching staff and students for the creation of the subject of care of people with skin lesions for the degree in nursing (AU)

Estudio comparativo de efectividad de un apósitode plata nanocristalina frente a sulfadiazinaargéntica en el tratamiento de pacientes quemados / Comparative study on the effectiveness of a dressing of nanocrystalline silver opposite to the use of silver sulfadiazine in burned patients treatment

La plata es un producto utilizado como agente antimicrobiano desde la antigüedad y ya a finales del siglo XIX se empezaron a utilizar compuestos de plata con finalidades médicas. La utilización tópica de la crema de sulfadiazina argéntica al 1% se considera mayoritariamente como tratamiento de elección para las quemaduras a fin de tratar o prevenir la infección local. El objetivo de este artículo es comparar el tratamiento con Acticoat® (plata nanocristalina 15 nanómetros) frente al tratamiento con sulfadiazina argéntica, ambos regímenes en igualdad de condiciones y un mismo centro de quemados. También se realiza una revisión de la literatura reciente sobre los factores de riesgo para la infección en quemaduras, las medidas que se utilizan para prevenirla y su importancia en la disminución de la morbi-mortalidad (AU)

Silver is a product that has been used as antimicrobialagent from ancient times; at the end of the XIXth century silver compounds were used with medical purposes. The use of silver sulfadiazine to 1 % cream is considered as gold standard for local treatment of burns in order to prevent or to treat local infection We compare treatment with Acticoat ® (nanocrystalline silver 15 nanometers) and silver sulfadiazine, both systems on equal terms and in the same medical burned enter. We review recent literature of risk factors for burn infection, methods to prevent it and to diminish morbimortality in burned patients (AU)

[Intratumoral pseudoaneurysm: atypical radiologic manifestation of bleeding from an angiomyolipoma]. / Pseudoaneurisma intratumoral: manifestación radiológica atípica del sangrado de un angiomiolipoma.

We present the case of a patient with tuberous sclerosis and multiple bilateral renal angiomyolipomas (AML) who developed an intralesional pseudoaneurysm in the context of bleeding from an AML. The diagnosis was reached by ultrasonography and computed tomography, and the histological diagnosis of a contained hematoma confirmed the bleeding.

Pseudoaneurisma intratumoral: manifestación radiológica atípica del sangrado de un angiomiolipoma / Intratumoral pseudoaneurysm: atypical radiologic manifestation of bleeding from an angiomyolipoma

Presentamos el caso de una paciente afecta de esclerosis tuberosa con múltiples angiomiolipomas (AML) renales bilaterales, que en el contexto del sangrado de uno de ellos desarrolla un pseudoaneurisma intralesional, cuyo diagnóstico se realiza mediante ecografía y tomografía computarizada (TC), existiendo confirmación histológica del mismo como hematoma contenido, que traduce sangrado (AU)

We present the case of a patient with tuberous sclerosis and multiple bilateral renal angiomyolipomas (AML) who developed an intralesional pseudoaneurysm in the context of bleeding from an AML. The diagnosis was reached by ultrasonography and computed tomography, and the histological diagnosis of a contained hematoma confirmed the bleeding (AU)

Economics of pressure-ulcer care: review of the literature on modern versus traditional dressings.

Published evidence suggests that some of the benefits of modern dressings--longer wear times and less frequent dressing changes--make them more cost-effective than traditional gauze dressings in pressure ulcer management.

Estudio "Auriga-04" sobre la utilización de la gama de apósitos hidrocelulares Allevyn® en el tratamiento de heridas agudas por parte de profesionales de Atención Primaria / The use of Allevyn(R) hydrocellular dressings range in acute wounds– AURIGA-04 study in primary care

Aunque los apósitos de cura en ambiente (CAH) húmedo se han utilizado predominantemente en heridas crónicas, ello no es óbice para que su uso en heridas agudas permita solucionar algunos problemas, como el conseguir un ambiente óptimo que facilite la migración epitelial, así como una adecuada protección de las heridas y una correcta gestión del exudado. Es por ello que, dentro del marco del estudio AURIGA-04, nos planteamos la realización de un estudio prospectivo observacional, abierto y multicéntrico, de medidas repetidas en una cohorte de pacientes que presentan heridas agudas de diversa etiología en el que se incluyeron pacientes con heridas traumáticas, quirúrgicas o quemaduras tratados por profesionales de Atención Primaria, con el objetivo de generar evidencias acerca de la utilización de apósitos de CAH, en concreto, de la gama de apósitos hidrocelulares Allevyn®, en el tratamiento de heridas agudas. Se consideraron como criterios de exclusión heridas con signos clínicos de infección. Solo se incluyó una lesión por paciente. En el caso de los pacientes con heridas agudas, la muestra a estudio quedó compuesta por 61 pacientes con una edad media de 71,1 años; 36 casos corresponden a mujeres (60%). El estado general de salud de la muestra era bueno en un 49,1% de los casos y prácticamente la totalidad de los pacientes presentaba pluripatología. Un 10% de los pacientes consumía fármacos que podían interferir en la cicatrización y un 6% presentaba malnutrición. El 67,2% de las lesiones eran heridas traumáticas, el 24,6% quirúrgicas y un 8,2% quemaduras. Un 37% de las lesiones fueron clasificadas como superficiales y el 63% restante como profundas. Presentaban 64 días de evolución previa a su inclusión en el estudio y una superficie media de 23,34 cm2. Los pacientes permanecieron en el estudio un promedio de 43,6 días, con una cadencia de cambios de apósito cada 2,7 días. Durante el estudio cicatrizaron el 63,9% de las heridas, en un promedio de 40,9 días. La superficie inicial de las lesiones mostró una reducción significativa a lo largo del seguimiento (inicial: 23,34 cm2, final: 4,27 cm2, p< 0,001). En los modelos de regresión de Cox, sólo resultó estadísticamente significativa la edad del paciente, en el grupo de lesiones traumáticas (RR: -0,03, p= 0,039). Durante el período de seguimiento, un 80,3% de los pacientes fue tratado en combinación con Intrasite Conformable®, Iruxol Mono® o la combinación de ambos productos. Los profesionales que realizaron las curas con Allevyn® valoraron muy favorablemente su manejo durante todo el estudio; así, en más del 96% de los cambios se consideró fácil de colocar, en el 100% indoloro y también en el 100% de fácil retirada. A la vista de los resultados, los apósitos de CAH y, en concreto, la gama de apósitos Allevyn®, tienen una clara aplicación en el caso de las heridas agudas, permitiendo un óptimo abordaje local de las lesiones, proporcionando unos niveles óptimos de humedad, pH y temperatura para las células implicadas en el proceso de cicatrización. Así, se promueve la formación de tejido de granulación y facilita la epitelización, además de proteger la herida ante traumatismos y gérmenes

Although moist environment dressings have mainly been used for chronic wounds, they can also be used on acute wounds in order to achieve an optimum atmosphere that facilitates epithelial migration, providing adequate protection for the wounds as well as good exudate management. Hence, as part of the AURIGA-04 study, an observational study was carried out at different centres, involving repeated measures on a group of patients who presented diverse acute wounds, including patients with traumatic wounds, surgical wounds or burns treated by primary care professionals. The aim of this study was to find evidence about the use of moist environment dressings, specifically the Allevyn® range of hydrocellular dressings, in the treatment of acute wounds. The exclusion criterion considered was if the wounds had clinical signs of infection. Only one wound was included per patient. In the case of patients with acute wounds, the study sample was made up of 61 patients with an average age of 71.1; 36 cases were women (60%). The patient’s general state of health was good in 49.1% of cases and practically all the patients presented multiple pathologies. 10% of the patients were taking medication that could interfere with the healing process and 6% of the patients presented signs of malnutrition. 67.2% of the wounds were traumatic in origin, 24.6% were surgical and 8.2% were burns. 37% of the wounds were classified as superficial and the remaining 63% were deep. The wounds had been present for 64 days before the patient’s inclusion in the study and the average surface area was 23.34 cm2. The patients remained in the study programme for an average of 43.6 days and their dressings were changed every 2.7 days. During the study, 63.9% of the wounds healed in an average of 40.9 days. The initial surface area of the wounds was reduced significantly over the monitoring period (initial: 23.34 cm2, final: 4.27 cm2, p ≤ 0.001). When Cox regression models were applied, the age of the patient was only statistically significant in the group with traumatic wounds (RR: –0.03, p= 0.039). During the monitoring period, 80.3% of the patients were also treated with Intrasite Conformable®, Iruxol Mono® or a combination of both products. The professionals that treated the wounds with Allevyn® rated the product very highly throughout the study; in over 96% of dressing changes, they were assessed as very easy to apply, 100% painless and also 100% easy to remove. Given these results, moist environment dressings, particularly the Allevyn® range of dressings, have a clear application in the case of acute wounds, permitting optimum local treatment of the wounds, providing optimum levels of moisture, pH and temperature for the cells involved in the healing process. Thus the formation of granulation tissue is promoted and epithelisation is facilitated. Furthermore, the wound is also protected from traumas and germs

2º Estudio Nacional de Prevalencia de Úlceras por Presión en España, 2005. Epidemiología y variables definitorias de las lesiones y pacientes / 2nd National Study of Pressure Ulcers Prevalence in Spain, 2005. Epidemiology and defining variables of lesions and patients

Desde el año 1999 el GNEAUPP consideró de vital interés para rescatar y mantener el valor real del problema de las úlceras por presión (UPP), la constitución de un observatorio permanente de la evolución epidemiológica de estos procesos, cuyo siguiente paso ha sido el desarrollo de este 2º Estudio Nacional de Prevalencia que presentamos. Se definieron como objetivos de la investigación obtener indicadores epidemiológicos que permitiesen dimensionar el problema de las UPPen España, así como algunos aspectos relacionados con las tendencias de prevención de las mismas en instituciones de Atención Primaria, atención hospitalaria y asistencia sociosanitaria. Elaboramos un cuestionario postal para estimar la prevalencia puntual y media de UPP a partir de una muestra no aleatoria. En estos cuestionarios se solicitaba a los profesionales de Atención Primaria que informasen acerca de su lista de usuarios mayores de 14 años (Unidades básicas asistenciales de Medicina General) y a los profesionales de unidades de hospitalización o de centros sociosanitarios que informasen acerca de los pacientes de sus respectivas unidades. Se definió la prevalencia como “prevalencia puntual” (información en un solo momento del tiempo). Se calculó la prevalencia cruda (PC) y media (PM). Los cuestionarios contenían preguntas para obtener datos sobre los pacientes con UPP (numerador del indicador), sobre el total de pacientes de esa unidad a estudio (denominador del indicador), sobre las lesiones mismas, así como información de las tendencias de prevención de UPP en las instituciones de los receptores de los cuestionarios. Se reportaron 1.791 pacientes con UPP (39% en hospital, 27% en Atención Primaria y 34% en centros sociosanitarios). En Atención Primaria la PC fue de 3,73% y la PM de 9,11% ± 10,9% para los pacientes mayores de 14 años incluidos en el programa de atención domiciliaria; en las unidades de hospital, la PC fue de 8,24% y la PM de 8,91% ± 12,20%, y en los centros sociosanitarios la PC fue de 6,10% y la PM de 10,9% ± 11,9%. En todos los niveles asistenciales, el grupo de población mayoritariamente afecto era el de los mayores de 65 años (84,7%). Los cuestionarios recibidos proporcionaron información sobre 2.837 lesiones: 23,2% de estadio I, 37,5% de estadio II, 27,0% de estadio III, 11,8% de estadio IV y un 0,5% sin estadiar. La localización más frecuente fue el sacro seguido de los talones, en todos los niveles asistenciales. Puede concluirse que las UPP siguen constituyendo, todavía, un importante problema de salud pública que afecta de manera primordial a personas mayores

Since 1999, GNEAUPP considered it vital to create a permanent observatory of the epidemiological evolution of these processes, in order to rescue and maintain the real value of the problem of Pressure Ulcers, which has given way to the next step, the 2nd National Prevalence Study we now present. The objectives set forth in this study were to obtain epidemiological indicators that would allow us to find the extent of the pressure ulcer problem in Spain, as well as some aspects related to the prevention of such in primary care centers, hospitals and socio-sanitary institutions. We sent out a questionnaire in order to estimate the punctual and average prevalence of PU (initials UPP in Spanish) using non-random sampling. Primary Care professionals were asked to give information about their patients who were over 14 years of age (General Practitioner Surgeries) and professionals from Hospital Units and socio-sanitary centers were asked about their patients respectively. Prevalence was defined as ‘punctual prevalence’ (information in a specific moment in time). The crude prevalence (initials PC in Spanish) and average prevalence (PM) were calculated. The questionnaires only included questions on patients with PU (numerator of the indicator) over the total of patients of the unit being studied (denominator of the indicator) about the wounds caused as well as information on PU prevention in the institutions where each respondant worked. 1,791 patients were reported to have pressure ulcers (39% in hospital, 27% in primary care centers and 34% in socio-sanitary centers). In Primary Care, the PC was 3.73% and the PM was 9.11% ± 10.9% for patients over 14 years who were included in the home care program. In the hospital units, the PC was 8.24% and the PM was 8.91% ± 12.20% and in the socio-sanitary centers, the PC was 6.10% and the PM was 10.9% ± 11.9%. The group most affected of each health care level was the one over 65 years of age (84.7%). The completed questionnaires provided information about 2,837 wounds: 23.2% were stage I, 37.5% stage II, 27.0% stage III, 11.8% stage IV and 0.5% did not state the stage. PU most frequently affected the sacral region, followed by the heels, in all health care levels. It can be concluded that PU are still an important public health problem that affect mainly elderly people

Valosin-containing protein gene mutations: clinical and neuropathologic features.

BACKGROUND: Hereditary inclusion body myopathy (IBMPFD) with Paget disease of bone (PDB) and frontotemporal dementia (FTD) is a rare multisystem disorder with autosomal dominant inheritance. Recently, missense mutations in the gene encoding valosin-containing protein (VCP) have been found in individuals with IBMPFD. VCP/P97, which exerts a variety of cellular functions, plays a key role in the ubiquitin-proteasome dependent degradation of cytosolic proteins and in the retrotranslocation of misfolded proteins from the endoplasmic reticulum into the cytoplasm. METHODS: The authors describe the clinical features of two kindreds in which VCP R93C and R155C missense mutations segregate and perform a histopathologic examination of brain, muscle, bone, and liver of three subjects harboring the R155C mutation. RESULTS: Frontotemporal dementia was present in 100% of affected subjects in Family F1 and 70% in Family F2, as compared with an average of 30% in previously described IBMPFD families. In contrast, PDB was a more inconstant clinical feature. Biochemical and histopathologic data are consistent with the hypothesis that VCP R155C mutation disrupts normal VCP function, leading to diffuse accumulation of ubiquitinated proteins within the cells. CONCLUSIONS: VCP mutations are present in two families in which FTD is the most prominent symptom. The histopathologic study performed in patients harboring the R155C mutation supports the hypothesis that this mutation disrupts normal VCP function, leading to diffuse accumulation of ubiquitinated proteins within the cells. IBMPFD belongs to a class of genetic diseases associated with an alteration of the ubiquitin-proteasome system.

Metrorragia postmenopáusica en una paciente tratada con sintrom / Postmenopausal metrorrhagia in a patient trated with Sintrom

Paciente postmenopausica en tratamiento con Sintrom que comienza con un pequeño sangrado genital y que debido a la cleisis vaginal que presenta, se hace imposible hacer un diagnóstico de certeza por los métodos habituales, pues se hace imposible acceder al endometrio. Por ello, se realiza una histerectomía con doble anexectomía, diagnosticándose un adenocarcinoma de endometrio (AU)

Postmenopausal patient being treated with Sintrom beginning with a small genital bleeding and, due to a vaginal cleisis that she present, is impossible to asevere a diagnosis by the usual methods, because the Access to endometrium is not possible. This is why and hysterectomy with double anexectomy is practiced, discovering and endometrial adenocarcinoma (AU)

Efectividad de un compuesto de ácidos grasos hiperoxigenados en la prevención de las úlceras por presión / Efficiency of a hyperoxigenated fatty acid compound in the prevention of pressure

Objetivo: Comparar los efectos de Mepentol®, un compuesto de ácidosgrasos hiperoxigenados, frente a placebo en la prevención del desarrollode úlceras por presión. Material y métodos: El estudio de investigaciónconsistió en un ensayo clínico aleatorizado, multicéntrico y dobleciego. Se calculó: la incidencia de úlceras por presión, riesgo relativo(RR), fracción prevenible (FP) y número necesario a tratar (NNT).Además, se aplicaron curvas de supervivencia de Kaplan-Meier conpruebas de log-rank y modelos de riesgos proporcionales de regresión deCox para comparar ambos grupos. Resultados: Completaron el estudioun total de 331 pacientes: 167 en el grupo control y 164 en el grupo aestudio. La incidencia de las úlceras por presión durante el estudio fuede 7,32% en el grupo de intervención frente a 17,37% en el grupo placebo(p= 0,006). Estos resultados muestran que por cada 10 pacientestratados con Mepentol® se previene la aparición de una úlcera por presión(NNT= 9,95). Las curvas de supervivencia y los modelos de regresiónmuestran una diferencia estadísticamente significativa para ambosgrupos (p≤ 0,001). El coste medio del uso de Mepentol® durante elestudio fue de 7,74 €. Conclusión: Mepentol® es una medida efectivapara la prevención de úlceras por presión. Fue más efectiva que un placebograso y demostró ser coste-efectiva. Declaración de interés: elestudio fue esponsorizado por Laboratorios Bama-Geve SA, Barcelona, España

Objective: To compare the effects of Mepentol®, a hyperoxygenated fatty acidpreparation, with a placebo treatment in preventing the development of pressureulcers. Method: The research study consisted of a multicentre double-blindrandomised clinical trial. The incidence of pressure ulcers, relative risk (RR), preventable fraction and number necessary to treat (NNT) were calculated. Inaddition, Kaplan-Meier survival curves, with log-rank test, and Cox’s proportional hazards regression model were used to compare both groups. Results: Atotal of 331 patients completed the study: 167 in the control group and 164in the study group. Pressure-ulcer incidence during the study was 7.32% inthe intervention group versus 17.37% in the placebo group (p= 0,006). Theseresults show that for each 10 patients treated with Mepentol® one pressure ulcerwas prevented (NNT= 9,95). Survival curves and the regression model showeda significant statistical difference for both groups (p≤ 0,001). The averagecost of Mepentol® during the study was € 7,74. Conclusion: Mepentol® is aneffective measure for pressure ulcer prevention. It was more effective than agreasy placebo product, and was found to be cost-effective.Declaration ofinterest: This study was sponsored by Laboratorios Bama-Geve SA,Barcelona, Spain

Optimal maintenance of constructed wetlands using an environmental decision support system.

Constructed wetlands (CWs) are artificial wastewater treatment systems appropriate for small communities because of their affordability, operability and reliability. These qualities are true whenever CWs are designed and constructed properly, and as long as the necessary operation and maintenance procedures are carried out correctly. Experience shows that the operation and maintenance procedures, and the frequencies with which these procedures are carried out, differ from one CW to another. With this in mind, and along with a projected increase in CWs in Catalonia, the Catalan Water Agency (Agència Catalana de l'Aigua) has developed an Environmental Decision Support System (EDSS) which proposes guidelines for monitoring and maintenance, according to the characteristics of each CW. This EDSS was developed following a methodology based on five steps: (i) problem analysis; (ii) collecting data and knowledge acquisition; (iii) model selection; (iv) model implementation and (v) validation. This paper describes the methodology followed to build the decision support system and presents some examples of the information provided by this EDSS.

Evaluating the application of a decision support system in identifying adequate wastewater treatment for small communities. A case study: the Fluvia River Basin.

The identification of adequate wastewater treatment for small communities is a complex problem since it demands a combination of data from different sources, such as aspects of the community and landscape, the receiving environment and the available wastewater treatment technologies. The Catalan Water Agency (Agència Catalana de l'Aigua) considered using an EDSS (Environmental Decision Support System) as a tool to help water managers select the most adequate treatment for the urban wastewater of nearly 3,500 small communities in Catalonia (Spain). From that moment, EDSS was applied to all the river basins in Catalonia. In this paper the authors present the results obtained for the 76 small communities located in one of these river basins: the Fluvia River Basin. The characteristics of the community used in the reasoning process of the EDSS, the list of selected wastewater treatment alternatives, the technical environmental justification for the selected treatments and the reasons for discarding, favouring or disadvantaging them are presented. Finally, some results for the Fluvia River Basin are compared with those obtained in other Catalan river basins with different characteristics in order to evaluate which are the significant features in identifying adequate wastewater treatments.

Molecular diagnosis of autosomal dominant early onset Alzheimer's disease: an update.

BACKGROUND: Autosomal dominant early onset Alzheimer's disease (ADEOAD) is genetically heterogeneous. Mutations of the amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2) genes have been identified. OBJECTIVE: To further clarify the respective contribution of these genes to ADEOAD. METHODS: 31 novel families were investigated. They were ascertained using stringent criteria (the occurrence of probable or definite cases of Alzheimer's disease with onset before 60 years of age in three generations). All cases fulfilled the NINCDS-ADRDA criteria for probable or definite Alzheimer's disease. The entire coding regions of PSEN1 and PSEN2 genes and exons 16 and 17 of APP gene were sequenced from genomic DNA RESULTS: PSEN1 mutations, including eight previously unreported mutations, were detected in 24 of the 31 families, and APP mutations were found in five families. In this sample, the mean ages of disease onset in PSEN1 and APP mutation carriers were 41.7 and 51.2 years, respectively. CONCLUSIONS: Combining these data with previously published data, yielding 65 ADEOAD families, 66% of the cases were attributable to PSEN1 mutations and 16% to APP mutations, while 18% remained unexplained.

The effectiveness of a hyperoxygenated fatty acid compound in preventing pressure ulcers.

OBJECTIVE: To compare the effects of Mepentol, a hyperoxygenated fatty acid preparation, with a placebo treatment in preventing the development of pressure ulcers. METHOD: The research study consisted of a multicentre double-blind randomised clinical trial. The incidence of pressure ulcers, relative risk (RR), preventable fraction and number necessary to treat (NNT) were calculated. In addition, Kaplan-Meier survival curves, with log-rank test, and Cox's proportional hazards regression model were used to compare both groups. RESULTS: A total of 331 patients completed the study: 167 in the control group and 164 in the study group. Pressure-ulcer incidence during the study was 7.32% in the intervention group versus 17.37% in the placebo group (p 0.006). These results show that for each 10 patients treated with Mepentol one pressure ulcer was prevented (NNT = 9.95). Survival curves and the regression model showed a significant statistical difference for both groups (p < or = 0.001). The average cost of Mepentol during the study was 7.74 Euro. CONCLUSION: Mepentol is an effective measure for pressure ulcer prevention. It was more effective than a greasy placebo product, and was found to be cost-effective.

Hyperprolinemia is a risk factor for schizoaffective disorder.

DNA sequence variations within the 22q11 DiGeorge chromosomal region are likely to confer susceptibility to psychotic disorders. In a previous report, we identified several heterozygous alterations, including a complete deletion, of the proline dehydrogenase (PRODH) gene, which were associated with moderate hyperprolinemia in a subset of DSM III schizophrenic patients. Our objective was (i) to determine whether hyperprolinemia is associated with increased susceptibility for any of three psychiatric conditions (schizophrenia, schizoaffective disorder and bipolar disorder) and (ii) to establish a correlation between hyperprolinemia and PRODH genotypes. We have conducted a case-control study including 114 control subjects, 188 patients with schizophrenia, 63 with schizoaffective disorder and 69 with bipolar disorder. We report that, taking into account a confounding effect due to valproate treatment, hyperprolinemia is a risk factor for DSM IIIR schizoaffective disorder (P=0.02, Odds ratio=4.6, 95% confidence interval 1.3-16.3). We did not detect 22q11 interstitial deletions associated with the DiGeorge syndrome among the 320 patients of our sample and we found no association between common PRODH polymorphisms and any of the psychotic disorders. In contrast, we found that five rare PRODH alterations (including a complete PRODH deletion and four missense substitutions) were associated with hyperprolinemia. In several cases, two variations were present simultaneously, either in cis or trans in the same subject. A total of 11 from 30 hyperprolinemic subjects bore at least one genetic variation associated with hyperprolinemia. This study demonstrates that moderate hyperprolinemia is an intermediate phenotype associated with certain forms of psychosis.