Cuantificación absoluta de la captación miocárdica de 99mTc-DPD en pacientes con amiloidosis cardiaca por depósitos de transtiretina (ATTR) / Absolute quantification of myocardial uptake of 99mTc-DPD in patients with cardiac amyloidosis due to transthyretin deposits (ATTR)

Objetivo Determinar la contribución diagnóstica de la cuantificación absoluta del depósito miocárdico de 99mTc-DPD en pacientes con amiloidosis cardiaca por depósitos de transtiretina (ATTR). Materiales y métodos Se realizó SPECT/TC a 41 pacientes con resultado gammagráfico positivo para amiloidosis cardiaca ATTR. Se dividió a los pacientes en dos grupos (grados2 y3 de Perugini) y se calcularon los SUVmax a nivel del hueso y de ambos ventrículos y el porcentaje de dosis calculado en estas áreas. Se empleó la prueba t de Student para comparar resultados y se calculó el área bajo la curva (AUC) para evaluar la eficacia diferencial y establecer unos puntos de corte discriminatorios entre ambos grupos de pacientes. Resultados Se observaron diferencias estadísticamente significativas en todas las variables a estudio, a excepción del SUVmax hueso. Las diferencias con mayor potencia estadística se observaron en las variables SUVmaxVD y el porcentaje de dosis en ambos ventrículos (p<0,001). El punto de corte obtenido para la variable SUVmaxVI fue de 8,620 (sensibilidad del 87,9% y especificidad del 100%; AUC: 0,966), mientras que el de la variable SUVmaxVD fue de 6,195 (sensibilidad del 81,8% y especificidad del 100%; AUC: 0,955). Conclusiones La cuantificación absoluta de la captación miocárdica de 99mTc-DPD en las imágenes SPECT/TC de pacientes con sospecha de amiloidosis cardiaca por depósitos de transtiretina supone una nueva herramienta diagnóstica que permite una adecuada clasificación de los pacientes, acorde a la escala de gradación visual de Perugini (AU)

Purpose To determine the diagnostic contribution of the absolute quantification of the myocardial deposit of 99mTc-DPD in patients with cardiac amyloidosis due to transthyretin deposits (ATTR). Materials and methods SPECT/CT was performed on 41 patients with positive scintigraphic results for ATTR cardiac amyloidosis. The patients were divided into two groups (Perugini grades2 and3) and the SUVmax at the level of the bone and both ventricles and the percentage of dose calculated in these areas were calculated. Student's t-test was used to compare results and the area under the curve (AUC) was calculated to assess differential efficacy and establish discriminatory cut-off points between both groups of patients. Results Statistically significant differences were observed in all the study variables, with the exception of SUVmax bone. The differences with the greatest statistical power were observed in the variables SUVmaxRV and the percentage of dose in both ventricles (P<.001). The cut-off point obtained for the variable SUVmaxLV was 8.620 (sensitivity 87.9% and specificity 100%; AUC: 0.966), while that of the variable SUVmaxRV was 6.195 (sensitivity 81.8% and specificity 100%; AUC: 0.955). Conclusions The absolute quantification of myocardial uptake of 99mTc-DPD in the SPECT/CT images of patients with suspected cardiac amyloidosis due to transthyretin deposits represents a new diagnostic tool that allows an adequate classification of patients, according to the visual grading scale of Perugini (AU)

Vía clínica de recuperación intensificada en cirugía cardiaca. Documento de consenso de la Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor (SEDAR), la Sociedad Española de Cirugía Cardiovascular y Endovascular (SECCE) y la Asociación Española de Perfusionistas (AEP) / Guidelines for enhanced recovery after cardiac surgery. Consensus document of Spanish Societies of Anesthesia (SEDAR), Cardiovascular Surgery (SECCE) and Perfusionists (AEP)

La vía clínica de recuperación intensificada en cirugía cardiaca (RICC) pretende identificar, difundir y favorecer la implementación de las mejores actuaciones basadas en la evidencia científica para disminuir la variabilidad en la práctica clínica. La puesta en marcha de estas prácticas en el proceso clínico global favorecerá la obtención de mejores resultados, acortamiento de estancias hospitalarias y en la Unidad de Cuidados Críticos, lo que permitirá una reducción de costes y una mayor eficiencia. Tras realizar una revisión sistemática en cada uno de los puntos del proceso perioperatorio en cirugía cardiaca, se han redactado recomendaciones basadas en la mejor evidencia científica disponible en la actualidad con el consenso de las sociedades científicas implicadas.(AU)

The ERAS guidelines are intended to identify, disseminate and promote the implementation of the best, scientific evidence-based actions to decrease variability in clinical practice. The implementation of these practices in the global clinical process will promote better outcomes and the shortening of hospital and critical care unit stays, thereby resulting in a reduction in costs and in greater efficiency. After completing a systematic review at each of the points of the perioperative process in cardiac surgery, recommendations have been developed based on the best scientific evidence currently available with the consensus of the scientific societies involved.(AU)

Guidelines for enhanced recovery after cardiac surgery. Consensus document of Spanish Societies of Anesthesia (SEDAR), Cardiovascular Surgery (SECCE) and Perfusionists (AEP). / Vía clínica de recuperación intensificada en cirugía cardiaca. Documento de consenso de la Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor (SEDAR), la Sociedad Española de Cirugía Cardiovascular y Endovascular (SECCE) y la Asociación Española de Perfusionistas (AEP).

The ERAS guidelines are intended to identify, disseminate and promote the implementation of the best, scientific evidence-based actions to decrease variability in clinical practice. The implementation of these practices in the global clinical process will promote better outcomes and the shortening of hospital and critical care unit stays, thereby resulting in a reduction in costs and in greater efficiency. After completing a systematic review at each of the points of the perioperative process in cardiac surgery, recommendations have been developed based on the best scientific evidence currently available with the consensus of the scientific societies involved.

Etiopathogenesis, prediction, and prevention of preeclampsia.

Hypertensive disorders are a major cause of maternal death. Preeclampsia (PE) affects about 5% of pregnancies and is associated to high cardiovascular death risk. Understanding of its origin and cause is difficult and many etiologies have been proposed. So far, little can be done for real prevention and the only treatment is pregnancy interruption, increasing the child's risk for prematurity complications. Early markers of disease are a promising path for understanding the pathogenesis and developing new strategies for prediction and eventually disease prevention.

Nail-patella syndrome: report of 11 pediatric cases.

BACKGROUND: Nail-patella syndrome (NPS) is an inherited disease produced by mutations in the LMX1B gene. It is characterized by fingernail dysplasia, hypoplastic or absent patella, dysplasia of the elbows and iliac horns on X-ray. It is useful to know this syndrome since some patients develop nephropathy and eye abnormalities. There are very few accurate descriptions related to this syndrome in the literature. OBJECTIVE: Describe the features of 11 patients with NPS in a paediatric hospital. METHODS: We retrospectively reviewed our clinical database of 11 patients with proven diagnosis of NPS from 1977 to 2014. Clinical and radiological features were assessed. RESULTS: Eleven children (seven male/four female) were included in the study. Mean age at the time of diagnosis was 6.54 years (range 0-11 years). Five patients had a family history of NPS. All patients had nail abnormalities (100%), the most frequent finding being hyponychia. Triangular lunulae were observed in four patients. The knee was the most commonly affected joint, aplasia or hypoplasia of the patella being the most usual findings. Only one patient presented renal involvement. The genetic study revealed three different LMX1B mutations. CONCLUSION: Nail-patella syndrome is a rare disorder. The aim of the present study is to highlight the importance of nail examination in children with skeletal dysplasias, in order to diagnose the NPS.

Increase in socioeconomic inequalities in mortality in a Southern European region: a small-area ecological study.

BACKGROUND: This study sought to describe the total mortality trend by socioeconomic deprivation (SED) in the Madrid Autonomous Region, by sex and age group. METHODS: Cross-sectional ecological study by census tract, in two periods: 1994-2000 (P1) with SED of 1996 census and 2001-07 (P2) with SED of 2001 census. We calculated the relative risks (RRs) and their 95% credibility intervals (95% CIs) by SED quintile (Q), taking the quintile of least deprivation as reference. Besag-York-Mollié ecological regression models and the Integrated Nested Laplace Approximation procedure were applied. The absolute differences in age-standardized rates were compared by SED quintile. RESULTS: Inequalities decreased in young adults: among men aged 20-39 years, the RR in Q5 versus Q1 ranged from 2.73 (95% CI, 2.51-3.02) in P1 to 1.93 (95% CI, 1.76-2.15) in P2, due to the greater improvement in the most underprivileged groups. In contrast, there was an increase in SED-related mortality in the 40-79 age group. Among men aged 40-59 years, the RR in Q5 versus Q1 rose from 1.88 (95% CI, 1.76-2.02) in P1 to 2.29 (95% CI, 2.17-2.43) in P2; the improvement was greater in the most privileged groups. CONCLUSION: In a context of an economic boom, inequalities were observed to increase among adults by a greater improvement in the most privileged groups.

Evidence-based guidelines of the spanish psoriasis group on the use of biologic therapy in patients with psoriasis in difficult-to-treat sites (nails, scalp, palms, and soles).

Psoriatic lesions affecting the scalp, nails, palms, and the soles of the feet are described as difficult-to-treat psoriasis and require specific management. Involvement of these sites often has a significant physical and emotional impact on the patient and the lesions are difficult to control with topical treatments owing to inadequate penetration of active ingredients and the poor cosmetic characteristics of the vehicles used. Consequently, when difficult-to-treat sites are involved, psoriasis can be considered severe even though the lesions are not extensive. Scant information is available about the use of biologic therapy in this setting, and published data generally comes from clinical trials of patients who also had moderate to severe extensive lesions or from small case series and isolated case reports. In this article we review the quality of the scientific evidence for the 4 biologic agents currently available in Spain (infliximab, etanercept, adalimumab, and ustekinumab) and report level i evidence for the use of biologics to treat nail psoriasis (level of recommendation A) and a somewhat lower level of evidence in the case of scalp involvement and palmoplantar psoriasis.

Value of the bone scintigraphy in multiple osteochrondromatosis with sarcomatous degeneration.

Multiple osteochondromatosis can become malignant in 20% of the cases, this being more common when the lesion is multiple than when it is solitary. A male patient with multiple osteochondromatosis who had several local recurrences of secondary chondrosarcoma and who is still under follow-up by the Nuclear Medicine Department is presented. The bone scintigraphy findings were compared with the histopathologic results, and the importance of the patient's symptoms was verified when a sarcomatous transformation is suspected. The bone scintigraphy has the potential to detect malignization of the benign bone lesions. It also makes it possible to obtain whole-body images in a single examination, this being very useful to detect the presence of new bone lesions.

Valor de la gammagrafía ósea en la osteocondromatosis múltiple con degeneración sarcomatosa / Value of the bone scintigraphy in multiple osteochrondromatosis with sarcomatous degeneration

La osteocondromatosis múltiple puede malignizar hasta en un 20% de los casos, siendo mucho más frecuente que cuando la lesión es solitaria. Presentamos el caso de un paciente con osteocondromatosis múltiple que ha presentado varias recidivas locales de un condrosarcoma secundario en años sucesivos y que sigue controles gammagráficos en nuestro servicio. Los hallazgos de la gammagrafía ósea se compararon con los resultados anatomopatológicos y se comprobó la importancia de la sintomatología del paciente ante la sospecha de transformación sarcomatosa. La gammagrafía ósea puede aportar datos sobre la posible malignización de las lesiones benignas y permite obtener imágenes de cuerpo completo en una sola exploración, siendo muy útil ante la aparición de nuevas lesiones(AU)

Multiple osteochondromatosis can become malignant in 20% of the cases, this being more common when the lesion is multiple than when it is solitary. A male patient with multiple osteochondromatosis who had several local recurrences of secondary chondrosarcoma and who is still under follow-up by the Nuclear Medicine Department is presented. The bone scintigraphy findings were compared with the histopathologic results, and the importance of the patient‘s symptoms was verified when a sarcomatous transformation is suspected. The bone scintigraphy has the potential to detect malignization of the benign bone lesions. It also makes it possible to obtain whole-body images in a single examination, this being very useful to detect the presence of new bone lesions(AU)

Kinetic study of the thermal hydrolysis of Agave salmiana for mezcal production.

The kinetics of the thermal hydrolysis of the fructans of Agave salmiana were determined during the cooking step of mezcal production in a pilot autoclave. Thermal hydrolysis was achieved at different temperatures and cooking times, ranging from 96 to 116 °C and from 20 to 80 h. A simple kinetic model of the depolymerization of fructans to monomers and other reducing sugars and of the degradation of reducing sugars to furans [principally 5-(hydroxymethyl)furfural, HMF] was developed. From this model, the rate constants of the reactions were calculated, as well as the pre-exponential factors and activation energies of the Arrhenius equation. The model was found to fit the experimental data well. The tradeoff between a maximum fructan hydrolysis and a critical furan concentration in allowing for the best ethanol yield during fermentation was investigated. The results indicated that the thermal hydrolysis of agave was optimal, from the point of view of ethanol yield in the ensuing fermentation, in the temperature range of 106-116 °C and the cooking range time of 6-14 h. The optimal conditions corresponded to a fructan hydrolysis of 80%, producing syrups with furan and reducing sugar concentrations of 1 ± 0.1 and 110 ± 10 g/L, respectively.

Chronic liver disease and cirrhosis mortality and social deprivation: a spatial analysis in small areas of Madrid region.

OBJECTIVES: To study census-tract distribution of chronic liver disease and cirrhosis mortality in the Madrid Region and its association with socio-economic deprivation. METHODS: Cross-sectional, ecological (3906 census-tract) study, using mortality data for 1996-2003 and a deprivation index drawn up on the basis of 2001 census data. Standardised mortality ratios were calculated taking Spanish rates for 2001 as reference. Smoothed census-tract relative risks were computed using the Besag-York-Mollie model. Relative risks (RRs) of dying and their 95% credibility intervals (95% CIs) were calculated according to quartiles of the deprivation index (with the fourth quartile -Q- of the indicator being the most unfavourable situation). Maps were plotted depicting the distribution of the posterior probability of RR > 1. RESULTS: Census tracts with a high risk of mortality were detected, mostly located in the centre and on the eastern, south-eastern and south-western fringes of the city of Madrid. Mortality increased with deprivation. RRs of mortality according to quartíles of the deprivation index were: Q2 = 1.5 (CI: 1.3-1.6), Q3 = 1.9 (CI:1.7-2.2) and Q4 = 2.5 (CI:2.2-2.8) for men; and Q2 = 1.3 (CI:1.1-1.5), Q3 = 1.5 (CI:1.3-1.7) and Q4 = 1.6 (CI:1.3-1.8) for women. CONCLUSIONS: This small-area study enabled census tracts with excess mortality eligible for a special public health intervention to be identified, and their association with socio-economic deprivation to be confirmed.

Doble injerto microvascularizado de peroné para reconstrucción bilateral mandibular por osteorradionecrosis / Double-barrel microvascularised fibular graft for bilateral mandibular reconstruction due to osteoradionecrosis

La osteorradionecrosis mandibular supone una complicación grave del tratamiento radioterápico, que a menudo origina una seria deformidad facial. La dificultad para la masticación, la articulación y la deglución son también frecuentes. A pesar de los avances realizados en la última década en radioterapia para tumores de la cabeza y el cuello, las complicaciones por osteorradionecrosis aún se producen. La indicación para una cirugía radical no está claramente definida, pero esta modalidad de tratamiento debe instaurarse cuando las medidas conservadoras han fracasado o cuando prevalece la necrosis ósea y de partes blandas. Las fracturas patológicas o fístulas persistentes son claros indicadores para un abordaje radical. En este artículo se presenta un caso de osteorradionecrosis bilateral mandibular tratada con amplia extirpación quirúrgica y reconstrucción en dos tiempos con dos injertos microvascularizados de peroné(AU)

Osteoradionecrosis of the mandible is a serious complication of radiotherapy that often leads to severe facial deformity. Difficulties in mastication, articulation, and swallowing are also common. Despite major improvements in radiation therapy of head and neck cancers during the last decade, osteoradionecrosis complications still occur. The indication for radical surgery is not clearly defined, but this kind of treatment should only be instituted when conservative methods fail or when severe bone and soft-tissue necrosis prevails. Pathological fractures or persistent fistulas are strong indications for a more radical surgical approach. This article reports a case of bilateral osteoradionecrosis of the mandible treated with radical escision and reconstruction in two stages with two fibular osteoseptocutaneous free flaps(AU)

Mandibular osteoradionecrosis: use of sequential fibula free flaps for a remote sequence of complications.

Mandibular osteoradionecrosis is a serious complication of radiotherapy that often leads to severe facial deformity, intolerable pain, fracture, sequestration of devitalized bone and fistulas. The prophylaxis of this severe complication is a major goal in modern combined tumor therapy, but once the pathology is established, conservative treatment modalities are used in almost all patients in an effort to control progression. Radical surgery should only be instituted when conservative methods fail or when severe bone and soft-tissue necrosis prevails. Pathologic fractures and chronic fistulae are clear indicators for radical surgical management. This paper document a case report of severe bilateral mandibular osteoradionecrosis reconstructed with two sequencial osteocutaneous fibular free flap. The authors discuss the different alternatives of treatment and the most important steps for a successful management of this challenge.

Census tract socioeconomic and physical environment and cardiovascular mortality in the Region of Madrid (Spain).

BACKGROUND: Features of the area might contribute to differences in cardiovascular mortality. The census tract distribution of ischaemic heart disease (IHD) and cerebrovascular disease mortality in the Region of Madrid and its association with deprivation and environmental variables were examined in this study. METHODS: Cross-sectional, ecological study covering 3906 census tracts (median of around 1000 inhabitants), using mortality data (population aged <75 years) for 1996-2003, as well as socioeconomic deprivation and other environmental indicators (subjective perceptions of pollution, background noise, lack of green spaces and delinquency) drawn from the 2001 census. Standardised mortality ratios were calculated. Smoothed census tract relative risks were calculated using the Besag-York-Mollié model. Relative risks (RRs) of dying and their 95% credibility intervals (95% CI) were calculated according to the indicators considered (with the fourth quartile, Q, being the most unfavourable situation). Maps were plotted depicting the distribution of the posterior probability of RR>1. RESULTS: Census tracts with excess mortality were mostly located in the city of Madrid. Mortality increased with deprivation: RRs of IHD and stroke mortality in Q4 with respect to Q1 were 1.42 (95% CI 1.31 to 1.54) and 1.66 (95% CI 1.45 to 1.88) for men, and 1.54 (95% CI 1.33 to 1.79) and 1.52 (95% CI 1.29 to 1.76) for women respectively. Associations with deprivation decreased only slightly when perceived lack of green spaces and delinquency were included in the model. In men, subjective perceptions of areas remained associated with cardiovascular mortality after adjustment for deprivation. CONCLUSION: Deprivation and subjective perceptions of physical environmental characteristics are ecologically associated with cardiovascular disease mortality.

Health-related quality of life in patients with primary Sjögren's syndrome: relationship with serum levels of proinflammatory cytokines.

OBJECTIVE: A cross-sectional study of 30 patients with primary Sjögren's syndrome (pSS) was performed to analyse the health-related quality of life and its relationship with serum levels of macrophage- and lymphocyte-derived cytokines. PATIENTS AND METHODS: Health-related quality of life was evaluated using the 36-item Short Form Health Survey (SF-36). Serum levels of interleukin (IL)-1beta, IL-6, IL-10, tumour necrosis factor (TNF)-alpha, and gamma-interferon (gamma-INF) were analysed by a sandwich immunoassay-based protein array system. RESULTS: Each of the eight scales of the SF-36 evaluating quality of life, as well as the physical composite score (PCS) and the mental composite score (MCS), showed a decrease in pSS patients. Similarly, patients with pSS showed significantly increased concentrations of each of the five cytokines analysed, when compared with the healthy control group (n = 20). In pSS patients, a significant negative correlation was detected between serum levels of IL-6 and the PCS of the SF-36. Those patients with concentrations of IL-6 higher than those of the healthy controls showed a significantly lower score in the dimensions of bodily pain and physical functioning, and in the PCS. CONCLUSIONS: Patients with pSS showed increased levels of several macrophage- and lymphocyte-derived cytokines, indicating the existence of an immune activation state. Serum levels of one of these cytokines, IL-6, were correlated with poor quality of life in these individuals.

Rituximab in systemic lupus erythematosus: A systematic review of off-label use in 188 cases.

The complexity of the therapeutic approach in systemic lupus erythematosus (SLE) is increased by the large number of patients who do not respond to the first-line therapies and by relapses after initial clinical remission. In these patients, second-line drugs are often prescribed according to individual clinical decisions. The emergence of biological therapies has increased the therapeutic armamentarium available in these complex situations, but their use is limited by the lack of licensing. Available data on the use of rituximab in SLE rely on a large number of case reports and some observational studies. We analyzed current evidence on the therapeutic use of rituximab in adult SLE patients by a systematic review of reports included in the PubMed database between 2002 and 2007. A total of 188 SLE patients treated with rituximab were identified; 171 (91%) patients showed a significant improvement in one or more of the systemic SLE manifestations. There were 103 patients with lupus nephritis, with an overall rate of therapeutic response of renal involvement of 91%. Adverse events were reported in 44 (23%) patients; the most frequent were infections (19%). Although it is not yet possible to make definite recommendations, the global analysis of all cases reported to date support the off-label use of rituximab in severe, refractory SLE cases, whereas its use as a first-line therapy or in patients with a predominantly mild form of the disease is not advised.

Implications of immunomodulatory interleukins for the hyperimmunoglobulinemia of Sjögren's syndrome.

A prospective study of 37 patients with pSS and 20 healthy controls was performed to analyze the differences in circulating levels of macrophage-derived and Th1/Th2 cytokines which could explain the hyperimmunoglobulinemia, characteristic of primary Sjögren's syndrome (pSS). Serum levels of interleukin (IL)-6, IL-10, IL-12, gamma-interferon (gamma-INF) and IL-4 were analyzed by a sandwich immunoassay-based protein array system. When compared with the control group, higher levels of IL-6, IL-12 and IL-10 and a lower Th1/Th2 ratio, as demonstrated by the gamma-INF/IL-4 ratio, were detected in patients. The levels of IL-4 were notably higher in pSS patients with monoclonal gammopathy. Serum IL-4 and IL-10 levels and immunoglobulin G concentrations were significantly correlated. In conclusion, patients with pSS show a state of macrophage and T-lymphocyte activation with increased concentrations of cytokines implicated in the differentiation of B cells and secretion of immunoglobulins.

Queratoquiste odontogénico mandibular. Presentación como trismus de larga evolución / Mandibular odontogenic keratocyst. Presentation as gradually developing trismus

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[Renal failure and insulin resistance: effect of the dialysis dose]. / Insuficiencia renal y resistencia insulínica: efecto de la dosis de diálisis.

BACKGROUND: The main cause of death in patients undergoing hemodialysis are cardiovascular events. We have analyzed the association between intradialysis fractional clearance of urea or Kt/V index in patients with chronic renal failure in hemodialysis as an indicator of adequate dialysis and the classical factors of cardiovascular risk. PATIENTS AND METHODS: A total of 47 patients with chronic renal failure on hemodialysis were included. Diabetes mellitus was considered an exclusion criteria. Optimization of dialysis was analyzed by Kt/v index in accordance with the Daugirdas formula. The cardiovascular risk factors evaluated were: insulin resistance, calculated according to the HOMA method, total cholesterol, LDL-cholesterol, triglycerides, arterial hypertension, obesity and metabolic syndrome. The relationship between cardiovascular risk factors and Kt/V index was analyzed with the variant and multivariant analysis. RESULTS: The HOMA median (interquartile range) of the patients was 1.16 (0.53-5.77). HOMA was correlated with triglycerides and HDL-cholesterol levels. HOMA was significantly greater in those who had less adapted dialysis (Kt/V < 1.4) (1.9 +/- 1.3 vs 1.0 +/- 0.3, p = 0.02). Furthermore, a negative correlation was found between HOMA and Kt/V. The multivariant analysis of the factors associated to HOMA demonstrated that the only associated parameters were Kt/V greater than 1.4, body mass index and age. CONCLUSIONS: In patients with chronic renal failure, adapted dialysis, determined by the Kt/V index, correlated negatively with insulin resistance. Based on these data, we suggest the need for longitudinal studies that consider this index as a predictive variable of cardiovascular events in this type of patients.