Incidence and prognostic impact of U2AF1 mutations and other gene alterations in myelodysplastic neoplasms with isolated 20q deletion.

BACKGROUND: In myelodysplastic neoplasms (MDS), the 20q deletion [del(20q)] is a recurrent chromosomal abnormality that it has a high co-occurrence with U2AF1 mutations. Nevertheless, the prognostic impact of U2AF1 in these MDS patients is uncertain and the possible clinical and/or prognostic differences between the mutation type and the mutational burden are also unknown. METHODS: Our study analyzes different molecular variables in 100 MDS patients with isolated del(20q). RESULTS & CONCLUSIONS: We describe the high incidence and negative prognostic impact of U2AF1 mutations and other alterations such as in ASXL1 gene to identify prognostic markers that would benefit patients to receive earlier treatment.

Response to azacitidine in patients with chronic myelomonocytic leukemia according to overlap myelodysplastic/myeloproliferative neoplasms criteria.

BACKGROUND: Azacitidine (AZA) is approved for the treatment of high-risk chronic myelomonocytic leukemia (CMML) of myelodysplastic (MD) subtype. Data of response rates using the specific response criteria for this disease are scarce. The aim of this study was to evaluate the response to AZA in patients diagnosed with CMML from the Spanish Registry of Myelodysplastic Syndromes (MDS) applying the overlap myelodysplastic/myeloproliferative neoplasms (MDS/MPN) response criteria. METHODS: We retrospectively studied 91 patients with CMML treated with at least one cycle of AZA from the Spanish Registry of MDS. As it was a real-world study, the response rate was evaluated between cycle 4 and 6, applying the MDS/MPN response criteria FINDINGS: The overall response rate at cycle 4-6 was 58%. Almost half of the patients achieved transfusion independence and one quarter showed clinical benefit, regardless of the CMML French-American-British (FAB) and World Health Organization (WHO) subtypes and CMML Specific Prognosis Scoring (CPSS) risk groups. Toxicity was higher in the MD-CMML subtype. INTERPRETATION: In our series, most CMML patients achieved an overall response rate with AZA according to the overlap-MDS/MPN response criteria regardless of the CMML FAB and WHO subtypes and CPSS risk groups. Thus, AZA may also be a treatment option for patients with the myeloproliferative CMML subtype and those with a lower-risk CPSS, but symptomatic.

Delphi consensus on the diagnosis and treatment of patients with short stature in Spain: GROW-SENS study.

PURPOSE: To identify consensus aspects related to the diagnosis, monitoring, and treatment of short stature in children to promote excellence in clinical practice. METHODS: Delphi consensus organised in three rounds completed by 36 paediatric endocrinologists. The questionnaire consisted of 26 topics grouped into: (1) diagnosis; (2) monitoring of the small-for-gestational-age (SGA) patient; (3) growth hormone treatment; and (4) treatment adherence. For each topic, different questions or statements were proposed. RESULTS: After three rounds, consensus was reached on 16 of the 26 topics. The main agreements were: (1) diagnosis tests considered as a priority in Primary Care were complete blood count, biochemistry, thyroid profile, and coeliac disease screening. The genetic test with the greatest diagnostic value was karyotyping. The main criterion for initiating a diagnostic study was prediction of adult stature 2 standard deviations below the target height; (2) the main criterion for initiating treatment in SGA patients was the previous growth pattern and mean parental stature; (3) the main criterion for response to treatment was a significant increase in growth velocity and the most important parameter to monitor adverse events was carbohydrate metabolism; (4) the main attitude towards non-responding patients is to check their treatment adherence with recording devices. The most important criterion for choosing the delivery device was its technical characteristics. CONCLUSIONS: This study shows the different degrees of consensus among paediatric endocrinologists in Spain concerning the diagnosis and treatment of short stature, which enables the identification of research areas to optimise the management of such patients.

Cólico renal como forma de expresión de adenoma paratiroideo / Renal colic as a form of presentation of parathyroid adenoma

El hiperparatiroidismo primario es una entidad muy poco frecuente en la edad pediátrica. La mayoría de los casos son esporádicos y debidos a adenomas. Debe sospecharse ante una hipercalcemia con parathormona elevada. La resección quirúrgica es el tratamiento de elección, con una evolución favorable de los casos en general. Presentamos el caso de una niña de 9 años de edad con adenoma paratiroideo, iniciado en forma de cólico renal e hipercalcemia. La gammagrafía tiroidea con tecnecio-99 sestamibi confirmó el diagnóstico. Tras la intervención quirúrgica presentó una buena evolución, con normalización de las concentraciones de calcio. En el diagnóstico diferencial del cólico renal en niños, hay que tener en cuenta la posibilidad subyacente de enfermedades raras con tratamiento específico, como el adenoma paratiroideo

Primary hyperparathyroidism in children is a rare disorder. It is usually sporadic and due to adenoma. It should be suspected when hypercalcemia with elevated parathyroid hormone is detected. Surgical resection is the treatment of choice with a favorable outcome. We present the case of a 9-year-old girl with a parathyroid adenoma, whose primary manifestations were renal colic and hypercalcemia. The thyroid scan with technetium-99 sestamibi confirmed the diagnosis. After surgery blood calcium levels returned to normal. In the differential diagnosis of renal colic in children, the underlying possibility of rare diseases with specific treatment, such as parathyroid adenoma, should also be considered

Oral versus transdermal oestrogen delivery for endometrial preparation before embryo transfer: a prospective, comparative, randomized clinical trial.

RESEARCH QUESTION: To determine whether the transdermal route is equal or superior to the oral route, when preparing the endometrium with oestrogens for embryo transfer. DESIGN: Prospective, randomized controlled trial; 140 patients randomized; the pills group followed a protocol with oestradiol valerate pills and the patches group followed a protocol with oestradiol hemihydrate patches. The primary variable was endometrial thickness on day 10 ± 1 of treatment. Secondary variables were endometrial thickness on day 15 ± 1 of treatment, patient satisfaction, plasma levels of oestradiol, rates of pregnancy, miscarriage and delivery. Endometrial thickness was measured on day 10 ± 1 of the cycle, if the lining was 7 mm or less in thickness, another measurement was made on day 15 ± 1. Blood oestradiol levels were analysed on the day the endometrial lining was greater than 7 mm (day 10 ± 1 or day 15 ± 1). Patients completed a survey to evaluate comfort and side-effects. RESULTS: The patches group achieved significantly thicker endometrium by the first check-up on day 10 ± 1 (7.6 mm versus 7.0 mm; P = 0.026), with lower blood levels of oestradiol (159.2 pg/ml versus 237.1 pg/ml; P < 0.001) when the endometrial thickness was over 7mm. The pills group considered the treatment more comfortable, with less side-effects. No significant differences in the rates of pregnancy, miscarriage or live birth were found. CONCLUSIONS: Transdermal oestrogen treatment allows patients to reach a higher endometrial thickness after 10 days of treatment, with lower plasma levels of oestradiol, although it is not tolerated as well.

Study of the efficacy of a Streptococcus uberis mastitis vaccine against an experimental intramammary infection with a heterologous strain in dairy cows.

Streptococcus uberis is a worldwide pathogen that causes intramammary infections in dairy cattle. Nevertheless, commercial vaccines are currently not available and measures to control S. uberis mastitis are limited to the implementation of good management practices. The aim of the present study was to evaluate the efficacy of an S. uberis subunit vaccine against bovine mastitis (Laboratorios Hipra S.A., Amer, Spain) administered precalving against an experimental intramammary challenge with a heterologous S. uberis strain in dairy cows postcalving. With this objective, 25 gestating Holstein-Friesian heifers were randomly assigned to 1 of 2 groups: group 1 (n = 13), vaccinated by intramuscular route with the vaccine, and group 2 (n = 12), vaccinated by intramuscular route with phosphate-buffered saline as a control group. Both groups were immunized 60 and 21 d before the expected parturition date (75 and 36 d before challenge). Fourteen days after calving all cows were challenged by intramammary infusion of 100 colony-forming units of a heterologous S. uberis strain in 2 quarters per cow. Then, challenged quarters were monitored for clinical signs of mastitis, bacterial count, and somatic cell count for the following 21 d. Rectal temperature and daily milk yield per cow were also assessed. Results showed that all challenged quarters developed clinical mastitis. Nevertheless, vaccination significantly reduced the clinical signs of mastitis, bacterial count, rectal temperature, and daily milk yield losses after the intramammary infection and significantly increased the number of quarters with no bacterial isolation and somatic cell count <200,000 cells/mL at the end of the study (d 19, 20, and 21 after challenge). To confirm the efficacy of this vaccine, further studies under field conditions are needed.

Efecto de una acción formativa en cuidados intensivos sobre la tasa de contaminación de hemocultivos / Effect of a training programme on blood culture contamination rate in critical care

La contaminación de hemocultivos puede ocurrir desde la extracción al procesamiento, y su tasa no debería exceder del 3%. Objetivo: Evaluar el impacto de una acción formativa sobre la tasa de hemocultivos contaminados tras la instauración de recomendaciones de extracción de muestras basadas en la mejor evidencia. Método: Estudio prospectivo antes-después en una unidad de cuidados intensivos polivalente de 18 camas. Se establecieron dos fases (enero-junio 2012, octubre 2012-octubre 2015) con un período formativo entre ellas. Principales recomendaciones: técnica estéril, mascarilla quirúrgica, doble desinfección de piel (alcohol 70° y clorhexidina alcohólica 2%), desinfección con alcohol 70° de tapones de frascos de cultivo e inyección de muestras sin cambiar aguja. Incluidos todos los hemocultivos de pacientes con solicitud facultativa de extracción. Variables: demográficas, gravedad, patología, motivo de ingreso, estancia y resultados de hemocultivos (negativo, positivo y contaminado). Estadística descriptiva básica: media (desviación estándar), mediana (rango intercuartílico) o porcentaje (intervalo de confianza del 95%). Calculadas tasas de contaminación por 100 hemocultivos extraídos. Análisis bivariado entre períodos. Resultados: Incluidos 458 pacientes. Extraídos 841 hemocultivos, 33 de ellos contaminados. En las variables demográficas, gravedad, diagnóstico y estancia en pacientes con contaminación de la muestra, no se observaron diferencias con no contaminados. Tasas de contaminación pre-formación vs post-formación: 14 vs 5,6 por 100 hemocultivos extraídos (p = 0,00003). Conclusión: Una acción formativa basada en la evidencia ha reducido la contaminación de las muestras. Es necesario seguir trabajando en la planificación de actividades y cuidados para mejorar la detección de contaminantes y prevenir la contaminación de las mismas

Blood culture contamination can occur from extraction to processing; its rate should not exceed 3%. Objective: To evaluate the impact of a training programme on the rate of contaminated blood cultures after the implementation of sample extraction recommendations based on the best evidence. Method: Prospective before-after study in a polyvalent intensive care unit with 18 beds. Two phases were established (January-June 2012, October 2012-October 2015) with a training period between them. Main recommendations: sterile technique, surgical mask, double skin disinfection (70° alcohol and 2% alcoholic chlorhexidine), 70° alcohol disinfection of culture flasks and injection of samples without changing needles. Including all blood cultures of patients with extraction request. Variables: demographic, severity, pathology, reason for admission, stay and results of blood cultures (negative, positive and contaminated). Basic descriptive statistics: mean (standard deviation), median (interquartile range) and percentage (95% confidence interval). Calculated contamination rates per 100 blood cultures extracted. Bivariate analysis between periods. Results: Four hundred and eight patients were included. Eight hundred and forty-one blood cultures were taken, 33 of which were contaminated. In the demographic variables, severity, diagnosis and stay of patients with contaminated samples, no differences were observed from those with uncontaminated samples. Pre-training vs post-training contamination rates: 14 vs 5.6 per 100 blood cultures extracted (P = .00003). Conclusion: An evidence-based training programme reduced the contamination of samples. It is necessary to continue working on the planning of activities and care to improve the detection of pollutants and prevent contamination of samples

Effect of a training programme on blood culture contamination rate in critical care. / Efecto de una acción formativa en cuidados intensivos sobre la tasa de contaminación de hemocultivos.

Blood culture contamination can occur from extraction to processing; its rate should not exceed 3%. OBJECTIVE: To evaluate the impact of a training programme on the rate of contaminated blood cultures after the implementation of sample extraction recommendations based on the best evidence. METHOD: Prospective before-after study in a polyvalent intensive care unit with 18 beds. Two phases were established (January-June 2012, October 2012-October 2015) with a training period between them. Main recommendations: sterile technique, surgical mask, double skin disinfection (70° alcohol and 2% alcoholic chlorhexidine), 70° alcohol disinfection of culture flasks and injection of samples without changing needles. Including all blood cultures of patients with extraction request. VARIABLES: demographic, severity, pathology, reason for admission, stay and results of blood cultures (negative, positive and contaminated). Basic descriptive statistics: mean (standard deviation), median (interquartile range) and percentage (95% confidence interval). Calculated contamination rates per 100 blood cultures extracted. Bivariate analysis between periods. RESULTS: Four hundred and eight patients were included. Eight hundred and forty-one blood cultures were taken, 33 of which were contaminated. In the demographic variables, severity, diagnosis and stay of patients with contaminated samples, no differences were observed from those with uncontaminated samples. Pre-training vs post-training contamination rates: 14 vs 5.6 per 100 blood cultures extracted (P=.00003). CONCLUSION: An evidence-based training programme reduced the contamination of samples. It is necessary to continue working on the planning of activities and care to improve the detection of pollutants and prevent contamination of samples.

Formación de equimosis y/o hematoma tras la administración profiláctica de enoxaparina subcutánea en abdomen o brazo en pacientes críticos / Ecchymosis and/or haematoma formation after prophylactic administration of subcutaneous enoxaparin in the abdomen or arm of the critically ill patient

Introducción: Los eventos adversos más frecuentes de la administración subcutánea de heparina de bajo peso molecular son la equimosis y/o el hematoma. No existe una fuerte recomendación sobre la zona de punción. Objetivo: Evaluar los eventos adversos, equimosis y/o hematoma, tras administración de enoxaparina subcutánea profiláctica en abdomen vs. brazo, en pacientes críticos. Metodología: Ensayo clínico aleatorizado en dos ramas (inyección abdomen vs. brazo), entre julio de 2014 y enero de 2017, en una unidad de cuidados intensivos polivalente de 18 camas. Incluidos pacientes con enoxaparina profiláctica, ingreso >72h, sin hepatopatías o enfermedades hematológicas, con índice de masa corporal (IMC)>18,5, no embarazadas, mayores de edad y sin lesiones cutáneas que impidan la valoración. Excluidos fallecimientos o traslados de hospital antes de finalizar la valoración. Recogidas variables demográficas, clínicas y aparición de equimosis y/o hematoma en lugar de inyección a las 12, 24, 48 y 72h. Análisis descriptivo, comparación de grupos y regresión logística. Aprobado por la comité de ética, con consentimiento firmado de pacientes/familiares. Resultados: Un total de 301 casos (11 excluidos): 149 en abdomen vs. 141 en brazo. Sin diferencias significativas en variables demográficas, clínicas, IMC, dosis de enoxaparina y administración de antiagregantes. Equimosis en el 48% de los pacientes y hematoma en el 8%, sin diferencias estadísticas abdomen vs. brazo [equimosis, abdomen vs. brazo, n(%): 66(44) vs. 72(51), p=0,25] [hematoma abdomen vs. brazo, n(%):9(6) vs. 14(10), p=0,2]. Se halla significación estadística en el tamaño del hematoma a las 72h: [área de hematoma (mm2) abdomen vs. brazo, mediana (RIC): 2(1-5,25) vs. 20(5,25-156), p=0,027]. Conclusiones: En nuestra cohorte de pacientes, la enoxaparina subcutánea profiláctica administrada en el abdomen produce menos hematomas, a las 72h, que administrada en el brazo. La tasa de incidencia de equimosis y hematomas es menor a la publicada en pacientes críticos, advirtiéndose que pacientes con antiagregantes presentan mayor riesgo de presentar lesiones, no observándose relación de su aparición con el IMC (AU)

Introduction: Ecchymosis and/or haematoma are the most common adverse events after subcutaneous administration of low molecular weight heparin. There is no strong recommendation as to the puncture site. Objective: To evaluate the adverse events, ecchymosis and/or haematoma after the administration of prophylactic subcutaneous enoxaparin in the abdomen vs the arm in the critically ill patient. Methodology: A randomised, two-arm clinical trial (injection in the abdomen vs the arm), performed between July 2014 and January 2017, in an 18-bed, polyvalent intensive care unit. Patients receiving prophylactic enoxaparin, admitted >72h, with no liver or haematological disorders, a body mass index (BMI) >18.5, not pregnant, of legal age and with no skin lesions which would impede assessment were included. We excluded patients who died or who were transferred to another hospital before completing the evaluation. We gathered demographic and clinical variables, and the onset of ecchymosis and/or haematomas at the injection site after 12, 24, 48 and 72hours. A descriptive analysis was undertaken, with group comparison and logistic regression. The study was approved by the ethics committee with the signed consent of patients/families. Results: 301 cases (11 excluded): 149 were injected in the abdomen vs 141 in the arm. There were no significant differences in demographic and clinical variables, BMI, enoxaparin dose or antiplatelet administration [ecchymosis, abdomen vs arm, n(%): 66(44) vs 72(51), P=.25] [haematoma abdomen vs arm, n(%): 9(6) vs 14(10), P=.2]. Statistical significance was found in the size of the haematomas after 72h: [area of haematoma (mm2) abdomen vs arm, median (IQR): 2(1-5.25) vs 20(5.25-156), P=.027]. Conclusions: In our patient cohort, prophylactic subcutaneous enoxaparin administered in the abdomen causes fewer haematomas after 72hours, than when administered in the arm. The incidence rate of ecchymosis and haematoma was lower than the published incidence in critically ill patients, although patients receiving anti-platelet agents present a higher risk of injury. No relationship was observed in relation to BMI (AU)

Ecchymosis and/or haematoma formation after prophylactic administration of subcutaneous enoxaparin in the abdomen or arm of the critically ill patient. / Formación de equimosis y/o hematoma tras la administración profiláctica de enoxaparina subcutánea en abdomen o brazo en pacientes críticos.

INTRODUCTION: Ecchymosis and/or haematoma are the most common adverse events after subcutaneous administration of low molecular weight heparin. There is no strong recommendation as to the puncture site. OBJECTIVE: To evaluate the adverse events, ecchymosis and/or haematoma after the administration of prophylactic subcutaneous enoxaparin in the abdomen vs the arm in the critically ill patient. METHODOLOGY: A randomised, two-arm clinical trial (injection in the abdomen vs the arm), performed between July 2014 and January 2017, in an 18-bed, polyvalent intensive care unit. Patients receiving prophylactic enoxaparin, admitted >72h, with no liver or haematological disorders, a body mass index (BMI) >18.5, not pregnant, of legal age and with no skin lesions which would impede assessment were included. We excluded patients who died or who were transferred to another hospital before completing the evaluation. We gathered demographic and clinical variables, and the onset of ecchymosis and/or haematomas at the injection site after 12, 24, 48 and 72hours. A descriptive analysis was undertaken, with group comparison and logistic regression. The study was approved by the ethics committee with the signed consent of patients/families. RESULTS: 301 cases (11 excluded): 149 were injected in the abdomen vs 141 in the arm. There were no significant differences in demographic and clinical variables, BMI, enoxaparin dose or antiplatelet administration [ecchymosis, abdomen vs arm, n(%): 66(44) vs 72(51), P=.25] [haematoma abdomen vs arm, n(%): 9(6) vs 14(10), P=.2]. Statistical significance was found in the size of the haematomas after 72h: [area of haematoma (mm2) abdomen vs arm, median (IQR): 2(1-5.25) vs 20(5.25-156), P=.027]. CONCLUSIONS: In our patient cohort, prophylactic subcutaneous enoxaparin administered in the abdomen causes fewer haematomas after 72hours, than when administered in the arm. The incidence rate of ecchymosis and haematoma was lower than the published incidence in critically ill patients, although patients receiving anti-platelet agents present a higher risk of injury. No relationship was observed in relation to BMI.

Síndrome de Turner: mosaicismo 45, X/46, XX y sus implicaciones clínicas / Turner's Syndrome: mosaicism 45, X / 46, XX and its clinical implications

El objetivo de este manuscrito es conocer cuál sería la actitud a seguir frente a un mosaico para monosomía X y sus repercusiones clínicas a partir de un caso de mosaicismo residual diagnosticado en nuestro centro. El síndrome de Turner o monosomía X es la aneuploidía más prevalente de los cromosomas sexuales, presenta gran variedad fenotípica condicionada por múltiples genotipos, entre los que destaca el mosaicismo, presente en hasta la mitad de los casos. Un correcto diagnóstico genético es fundamental para un adecuado consejo clínico y para optimizar el manejo de cada caso ya que las repercusiones clínicas y su evolución son muy variables (AU)

The aim of this article is to know what should be the attitude we should continue with a mosaic monosomy X and its clinical consequences on the basis of a case diagnosed in our hospital. Turner syndrome or monosomy X is the most common sexual chromosomes' aneuploidy, can make a lot of phenotypic variations conditioned by multiple genotypes that may occur, especially mosaicism, present in up to half of cases. Appropriate genetic diagnosis is essential for proper and appropriate genetic counseling to optimize the management of each case, as the clinical implications and evolution are highly variable (AU)

Síndrome de Collet-Sicard metastásico / Collet-Sicard syndrome caused by metastasis

No disponible

A randomized phase II/III study of cabazitaxel versus vinflunine in metastatic or locally advanced transitional cell carcinoma of the urothelium (SECAVIN).

BACKGROUND: Despite the advent of immunotherapy in urothelial cancer, there is still a need to find effective cytotoxic agents beyond first and second lines. Vinflunine is the only treatment approved in this setting by the European Medicines Agency and taxanes are also widely used in second line. Cabazitaxel is a taxane with activity in docetaxel-refractory cancers. A randomized study was conducted to compare its efficacy versus vinflunine. PATIENTS AND METHODS: This is a multicenter, randomized, open-label, phase II/III study, following a Simon's optimal method with stopping rules based on an interim futility analysis and a formal efficacy analysis at the end of the phase II. ECOG Performance Status, anaemia and liver metastases were stratification factors. Primary objectives were overall response rate for the phase II and overall survival for the phase III. RESULTS: Seventy patients were included in the phase II across 19 institutions in Europe. Baseline characteristics were well balanced between the two arms. Three patients (13%) obtained a partial response on cabazitaxel (95% CI 2.7-32.4) and six patients (30%) in the vinflunine arm (95% CI 11.9-54.3). Median progression-free survival for cabazitaxel was 1.9 versus 2.9 months for vinflunine (P = 0.039). The study did not proceed to phase III since the futility analysis showed a lack of efficacy of cabazitaxel. A trend for overall survival benefit was found favouring vinflunine (median 7.6 versus 5.5 months). Grade 3- to 4-related adverse events were seen in 41% patients with no difference between the two arms. CONCLUSION: This phase II/III second line bladder study comparing cabazitaxel with vinflunine was closed when the phase II showed a lack of efficacy of the cabazitaxel arm. Vinflunine results were consistent with those known previously. TRIAL NUMBER: NCT01830231.

Long-term survival in advanced non-squamous NSCLC patients treated with first-line bevacizumab-based therapy

Background/Aim. First-line bevacizumab-based therapies have been shown to improve clinical outcomes in patients with non-squamous non-small-cell lung cancer (NSCLC). We aimed to descriptively analyse patients with non-squamous NSCLC who received a long-term period of maintenance bevacizumab. Patients and methods. This retrospective study included 104 patients who had already reached a progression-free survival (PFS) of at least 9 months. Results. Median overall survival and PFS were 30.7 and 15.1 months, respectively. The overall response rate was 83 %. Weight loss ≤5 %, ECOG PS = 0, or low number of metastatic sites seem to be predictive factors of good evolution. The incidence of bevacizumab-related adverse events appeared to be similar as the previous studies. Conclusion. Our findings show that there is a long-term survivor group whom the administration of bevacizumab resulted in a relevant prolongation of response without new safety signals. Due to the population heterogeneity, it was not possible to identify the standardised predictive factors (AU)

No disponible

Long-term survival in advanced non-squamous NSCLC patients treated with first-line bevacizumab-based therapy.

BACKGROUND/AIM: First-line bevacizumab-based therapies have been shown to improve clinical outcomes in patients with non-squamous non-small-cell lung cancer (NSCLC). We aimed to descriptively analyse patients with non-squamous NSCLC who received a long-term period of maintenance bevacizumab. PATIENTS AND METHODS: This retrospective study included 104 patients who had already reached a progression-free survival (PFS) of at least 9 months. RESULTS: Median overall survival and PFS were 30.7 and 15.1 months, respectively. The overall response rate was 83 %. Weight loss ≤5 %, ECOG PS = 0, or low number of metastatic sites seem to be predictive factors of good evolution. The incidence of bevacizumab-related adverse events appeared to be similar as the previous studies. CONCLUSION: Our findings show that there is a long-term survivor group whom the administration of bevacizumab resulted in a relevant prolongation of response without new safety signals. Due to the population heterogeneity, it was not possible to identify the standardised predictive factors.

Masa ventricular izquierda, espirometría basal forzada y perfil de adipocitocinas en niños obesos con y sin síndrome metabólico / Left ventricular mass, forced baseline spirometry and adipocytokine profiles in obese children with and without metabolic syndrome

Introducción: En los últimos años, se ha constatado un aumento de las alteraciones de la función cardiaca y pulmonar entre los pacientes obesos. Asimismo, también se ha demostrado que la obesidad es un estado de inflamación crónica. Por ello, hipotetizamos que los niños obesos con síndrome metabólico presentan un porcentaje superior de hipertrofia de ventrículo izquierdo y una espirometría alterada, secundaria a un mayor grado de inflamación. Pacientes y métodos: Estudio de la masa ventricular izquierda mediante ecografía, análisis de espirometría basal forzada mediante espirómetro (FlowScreen) y determinación de perfil de adipocitocinas (adiponectina, IL-6, leptina, MCP-1, PCR-hs, RBP-4, TNF-alfa y visfatina) a niños peripuberales obesos con y sin síndrome metabólico. Resultados: Se incluyó en el estudio a 41 pacientes (20 niñas y 21 niños), 20 de los cuales (10 niños y 10 niñas) presentaban síndrome metabólico. De las adipocitocinas estudiadas, leptina, PCR-hs y MCP-1, y el cociente leptina/adiponectina mostraron valores sustancialmente superiores en el grupo de síndrome metabólico (p<0,01). El análisis de la masa ventricular izquierda y la espirometría no evidenciaron diferencias entre los 2 grupos estudiados. Sin embargo, considerando la muestra completa, el 9,5% de los sujetos tenían ya una hipertrofia ventricular izquierda. Por otro lado, no se encontraron correlaciones significativas entre los datos antropométricos y el perfil de adipocitocinas, ni tampoco con masa ventricular izquierda ni con la espirometría. Conclusión: En el momento del estudio, la masa ventricular izquierda y la espirometría parecen no relacionarse con parámetros inflamatorios en el niño obeso(AU)

Introduction: Recent reports have shown an increase in changes in cardiac and pulmonary function among obese patients. Furthermore, it has also been demonstrated that obesity is a state of chronic inflammation. We hypothesized that obese children with metabolic syndrome exhibit a higher percentage of left ventricular hypertrophy and altered spirometry values due to higher levels of inflammation. Patients and methods: Left ventricular mass was studied using echocardiography, baseline forced spirometry by spirometer (FlowScreen) and adipocytokine profiles (adiponectin, IL-6, leptin, MCP-1, PCR-Hs, RBP-4, TNF-alpha and visfatin) were evaluated in peripubertal obese children with and without metabolic syndrome. Results: Forty-one patients (20 girls and 21 boys) were included in the study, 20 of whom (10 boys and 10 girls) were subjects with metabolic syndrome. Of the adipocytokines studied, only leptin, hs-CRP, MCP-1, and the leptin/adiponectin ratio yielded values that were substantially greater in the group with metabolic syndrome (P<0.01). An analysis of left ventricular mass index and baseline spirometry showed no differences between the groups studied. However, of the entire cohort, 9.5% had left ventricular hypertrophy. No significant relationship was found between anthropometric data and adipocytokines and the parameters used to study left ventricular mass and spirometry values on the other. Conclusion: At the time the study was performed, left ventricular mass and baseline forced spirometry did not appear to be influenced by inflammatory mechanisms(AU)

[Left ventricular mass, forced baseline spirometry and adipocytokine profiles in obese children with and without metabolic syndrome]. / Masa ventricular izquierda, espirometría basal forzada y perfil de adipocitocinas en niños obesos con y sin síndrome metabólico.

INTRODUCTION: Recent reports have shown an increase in changes in cardiac and pulmonary function among obese patients. Furthermore, it has also been demonstrated that obesity is a state of chronic inflammation. We hypothesized that obese children with metabolic syndrome exhibit a higher percentage of left ventricular hypertrophy and altered spirometry values due to higher levels of inflammation. PATIENTS AND METHODS: Left ventricular mass was studied using echocardiography, baseline forced spirometry by spirometer (FlowScreen) and adipocytokine profiles (adiponectin, IL-6, leptin, MCP-1, PCR-Hs, RBP-4, TNF-( and visfatin) were evaluated in peripubertal obese children with and without metabolic syndrome. RESULTS: Forty-one patients (20 girls and 21 boys) were included in the study, 20 of whom (10 boys and 10 girls) were subjects with metabolic syndrome. Of the adipocytokines studied, only leptin, hs-CRP, MCP-1, and the leptin/adiponectin ratio yielded values that were substantially greater in the group with metabolic syndrome (P<.01). An analysis of left ventricular mass index and baseline spirometry showed no differences between the groups studied. However, of the entire cohort, 9.5% had left ventricular hypertrophy. No significant relationship was found between anthropometric data and adipocytokines and the parameters used to study left ventricular mass and spirometry values on the other. CONCLUSION: At the time the study was performed, left ventricular mass and baseline forced spirometry did not appear to be influenced by inflammatory mechanisms.

[Anterior circulation embolic stroke secondary to thrombotic occlusion of the brachiocephalic trunk: the usefulness of neurosonological studies]. / Ictus embolico de circulacion anterior secundario a una oclusion trombotica del tronco braquiocefalico: utilidad del estudio neurosonologico.

INTRODUCTION. Atherosclerotic occlusion of the brachiocephalic trunk (CBT) is a rare clinical entity and its presentation in the form of arterio-arterial embolism is uncommon. Early identification of patients with CBT occlusion may have important therapeutic implications. CASE REPORT. A man aged 49 presents with sudden onset symptoms involving the territory of the right middle cerebral artery. Emergent transcranial doppler evaluation showed a flow pattern of proximal right M1 occlusion. After intravenous recombinant tissue plasminogen activator administration, partial recanalization of the vessel was found and the patient improved clinically. Cervical and transcranial duplex sonography demonstrated an occlusion in CBT, which was later confirmed on CT angiography and digital angiography of supraaortic vessels. After aorto-innominate bypass, pathological analysis confirmed the atherosclerotic origin. CONCLUSIONS. The most common clinical presentation of CBT occlusion are transient ischemic symptoms related to steal phenomenon in the vertebro-basilar territory. Symptoms of carotid circulation stroke usually result from arterio-arterial embolic mechanism. Early recognition is important for its therapeutic implications, therefore thrombolytic therapy should be indicated. Neurosonologic study allows rapid and reliable examination on hemodynamic status and the presence of distal embolic phenomena.

Impact of adjunct cytogenetic abnormalities for prognostic stratification in patients with myelodysplastic syndrome and deletion 5q.

This cooperative study assessed prognostic factors for overall survival (OS) and risk of transformation to acute myeloid leukemia (AML) in 541 patients with de novo myelodysplastic syndrome (MDS) and deletion 5q. Additional chromosomal abnormalities were strongly related to different patients' characteristics. In multivariate analysis, the most important predictors of both OS and AML transformation risk were number of chromosomal abnormalities (P<0.001 for both outcomes), platelet count (P<0.001 and P=0.001, respectively) and proportion of bone marrow blasts (P<0.001 and P=0.016, respectively). The number of chromosomal abnormalities defined three risk categories for AML transformation (del(5q), del(5q)+1 and del(5q)+ ≥ 2 abnormalities) and two for OS (one group: del(5q) and del(5q)+1; and del(5q)+ ≥ 2 abnormalities, as the other one); with a median survival time of 58.0 and 6.8 months, respectively. Platelet count (P=0.001) and age (P=0.034) predicted OS in patients with '5q-syndrome'. This study demonstrates the importance of additional chromosomal abnormalities in MDS patients with deletion 5q, challenges the current '5q-syndrome' definition and constitutes a useful reference series to properly analyze the results of clinical trials in these patients.