Fragile X Syndrome in children

Fragile X syndrome is caused by the expansion of CGG triplets in the FMR1 gene, which generates epigenetic changes that silence its expression. The absence of the protein coded by this gene, FMRP, causes cellular dysfunction, leading to impaired brain development and functional abnormalities. The physical and neurologic manifestations of the disease appear early in life and may suggest the diagnosis. However, it must be confirmed by molecular tests. It affects multiple areas of daily living and greatly burdens the affected individuals and their families. Fragile X syndrome is the most common monogenic cause of intellectual disability and autism spectrum disorder; the diagnosis should be suspected in every patient with neurodevelopmental delay. Early interventions could improve the functional prognosis of patients with Fragile X syndrome, significantly impacting their quality of life and daily functioning. Therefore, healthcare for children with Fragile X syndrome should include a multidisciplinary approach.

El síndrome de X frágil es causado por la expansión de tripletas CGG en el gen FMR1, el cual genera cambios epigenéticos que silencian su expresión. La ausencia de la proteína codificada por este gen, la FMRP, causa disfunción celular, llevando a deficiencia en el desarrollo cerebral y anormalidades funcionales. Las manifestaciones físicas y neurológicas de la enfermedad aparecen en edades tempranas y pueden sugerir el diagnóstico. Sin embargo, este debe ser confirmado por pruebas moleculares. El síndrome afecta múltiples aspectos de la vida diaria y representa una alta carga para los individuos afectados y para sus familias. El síndrome de C frágil es la causa monogénica más común de discapacidad intelectual y trastornos del espectro autista; por ende, el diagnóstico debe sospecharse en todo paciente con retraso del neurodesarrollo. Intervenciones tempranas podrían mejorar el pronóstico funcional de pacientes con síndrome de X frágil, impactando significativamente su calidad de vida y funcionamiento. Por lo tanto, la atención en salud de niños con síndrome de X frágil debe incluir un abordaje multidisciplinario.

Effect of Combining EGFR Tyrosine Kinase Inhibitors and Cytotoxic Agents on Cholangiocarcinoma Cells / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#The potential of members of the epidermal growth factor receptor (ErbB) family as drug targets in cholangiocarcinoma (CCA) has not been extensively addressed. Although phase III clinical trials showed no survival benefits of erlotinib in patients with advanced CCA, the outcome of the standard-of-care chemotherapy treatment for CCA, gemcitabine/cisplatin, is discouraging so we determined the effect of other ErbB receptor inhibitors alone or in conjunction with chemotherapy in CCA cells. Materials and Methods ErbB receptor expression was determined in CCA patient tissues by immunohistochemistry and digital-droplet polymerase chain reaction, and in primary cells and cell lines by immunoblot. Effects on cell viability and cell cycle distribution of combination therapy using ErbB inhibitors with chemotherapeutic drugs was carried out in CCA cell lines. 3D culture of primary CCA cells was then adopted to evaluate the drug effect in a setting that more closely resembles in vivo cell environments. @*Results@#CCA tumors showed higher expression of all ErbB receptors compared with resection margins. Primary and CCA cell lines had variable expression of erbB receptors. CCA cell lines showed decreased cell viability when treated with chemotherapeutic drugs (gemcitabine and 5-fluorouracil) but also with ErbB inhibitors, particularly afatinib, and with a combination. Sequential treatment of gemcitabine with afatinib was particularly effective. Co-culture of CCA primary cells with cancer-associated fibroblasts decreased sensitivity to chemotherapies, but sensitized to afatinib. Conclusion Afatinib is a potential epidermal growth factor receptor targeted drug for CCA treatment and sequential treatment schedule of gemcitabine and afatinib could be explored in CCA patients.

Effect of Combining EGFR Tyrosine Kinase Inhibitors and Cytotoxic Agents on Cholangiocarcinoma Cells / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#The potential of members of the epidermal growth factor receptor (ErbB) family as drug targets in cholangiocarcinoma (CCA) has not been extensively addressed. Although phase III clinical trials showed no survival benefits of erlotinib in patients with advanced CCA, the outcome of the standard-of-care chemotherapy treatment for CCA, gemcitabine/cisplatin, is discouraging so we determined the effect of other ErbB receptor inhibitors alone or in conjunction with chemotherapy in CCA cells. Materials and Methods ErbB receptor expression was determined in CCA patient tissues by immunohistochemistry and digital-droplet polymerase chain reaction, and in primary cells and cell lines by immunoblot. Effects on cell viability and cell cycle distribution of combination therapy using ErbB inhibitors with chemotherapeutic drugs was carried out in CCA cell lines. 3D culture of primary CCA cells was then adopted to evaluate the drug effect in a setting that more closely resembles in vivo cell environments. @*Results@#CCA tumors showed higher expression of all ErbB receptors compared with resection margins. Primary and CCA cell lines had variable expression of erbB receptors. CCA cell lines showed decreased cell viability when treated with chemotherapeutic drugs (gemcitabine and 5-fluorouracil) but also with ErbB inhibitors, particularly afatinib, and with a combination. Sequential treatment of gemcitabine with afatinib was particularly effective. Co-culture of CCA primary cells with cancer-associated fibroblasts decreased sensitivity to chemotherapies, but sensitized to afatinib. Conclusion Afatinib is a potential epidermal growth factor receptor targeted drug for CCA treatment and sequential treatment schedule of gemcitabine and afatinib could be explored in CCA patients.

Cáncer avanzado de orofaringe. Nuevo paradigma de tratamiento / ADVANCED OROPHARYNGEAL CANCER TREATED WITH SEQUENTIAL CHEMORADIATION PRESERVING THE ORGAN

Antecedentes: Hasta la década del 80 los pacientes con esta patología eran intervenidos quirúrgicamente. A partir de 1997 la Cleveland Clinic publica un estudio basado en la quimioradioterapia en el cáncer avanzado de orofaringe con resultados similares a los de la época anterior, pero con preservación del órgano. En nuestro medio no existe bibliografía que analice estadísticamente los resultados y los factores que lo condicionaron. Objetivo: Evaluar los resultados con quimioradioterapia secuencial en el cáncer avanzado de la región orofaríngea. Lugar de aplicación: Hospital de atención terciaria Diseño: Prospectivo no randomizado Material y metodo: Desde Enero del 2002 al 30 de diciembre del 2004 se estudiaron 60 pacientes, 55 pertenecían al sexo masculino, con edad media de 60 años, el 76.7% eran T III y los restantes T IV con tumores malignos de la región orofaríngea mediante quimioradioterapia secuencial. Resultados: La supervivencia a 152 meses fue del 30.1%. El análisis univariado, demostró que el estadío, con p<0,001, la reducción con p=0,010 y la cirugía de rescate con p=0,028 fueron las únicas variables estadísticamente significativas, mientras la edad, el sexo y la localización no tuvieron los mismos resultados. En el análisis multivariado, el estadío con una p< 0.001, el sexo con una p=0.016 y la reducción con una p=0.024 resultaron ser estadisticamente significativos. Conclusiones: El tratamiento de los tumores de la región orofaríngea, en estadíos III y IV, mediante quimioradioterapia secuencial alcanzaron resultados similares a los tratados con cirugía seguida de radioterapia, pero con preservación del órgano

Background: Until the 80th decade, patients with advanced oropharyngeal cancer were surgically treated. In 1997 the Cleveland Clinic published similar results with chemoradiotherapy but preserving the organ. In our country there are not papers analyzing those results and conditional factors. Objective: Evaluation of sequential chemoradiation results in advanced oropharyngeal cancer Setting: Public tertiary care Hospital of tumours Design: Prospective not randomized. Population and methods: From January 2002 to December 2008, 60 patients were analysed, 55 of them were male with a mean age of 60, 76.7% TIII/IV oropharyngeal carcinomas treated with sequential chemoradiation. Results: The overall 152 months of survival was 30.1%. Univariate analize showed stage p<001, reduce of lesion p:0.010 and rescue surgery p:0.028 were statiscally significative. Age, gender and subsite had not similar results. In multivariate analize only stage p:0.001, gender p: 0.016 and reduce of lesion p:0.024 resulted statiscally significative. Conclusions: Sequential chemoradiation of III and IV stage tumours of oropharyngeal area have similar long terms results than those treated with surgery plus radiation but with organ preservation

Identificación de marcadores moleculares (SNP) y genes candidatos asociados a la tolerancia genética al complejo mancha de asfalto en variedades nativas de maíz (Zea mays L) / Identification of molecular markers (SNP) and candidate genes associated to genetic tolerance to tar spot complex disease in maize (Zeamays L) landraces

El complejo mancha de asfalto (CMA) en maíz (ZeamaysL.), causado por los hongos Phyllachora maydis Maubl. Y Monographella maydis Müller & Samuels, es una enfermedad de importancia económica en Guatemala, que ha causado pérdida en el rendimiento entre 30 a 50%, inclusive del 100% si las condiciones son favorables. El objetivo de esta investigación fue identificar marcadores de un solo nucleótido o SNP (Single Nucleotide Polymorphism, por sus siglas en inglés) y genes candidatos asociados a la tolerancia genética al CMA. Para ello se analizaron 463 poblaciones nativas y 329,692 SNP, y se compararon dos modelos genómicos, single markery BayesB, para la identificación de regiones asociadas a la tolerancia genética al CMA. Se identificaron 40 marcadores SNP asociados significativamente a la tolerancia genética al CMA con ambos modelos. La proporción de variación fenotípica total explicada (PVE) por los 40 SNPs fue de 56%, atribuida a efectos genéticos aditivos. Múltiples genes de resistencia a enfermedades fueron identificados en las regiones señaladas por los marcadores SNP. Sus funciones principales son receptores y transductores de señal, factores de transcripción que regulan positivamente la expresión de genes de tolerancia y genes de la familia kinasa, por lo que potencialmente están involucrados en el mecanismo de defensa al CMA.

The tar spot complex (TSC) diseasein maize (ZeamaysL.), caused by the fungi Phyllachora maydis Maubl. And Monographella maydis Müller & Samuels, is an economic important disease in Guatemala, producing yield losses between 30 to 50%, inclusive of 100% if the conditionsare favorable. The objective of this researchwasto identify single nucleoti depolymorphism markers (SNP) and candidate genes associated withgenetictoleranceto TSC. Asetof 463 native populations and 329,692 SNP were analyzed with two genomic models, single marker and BayesB, for the identification of regions associated with genetic tolerance to TSC. Forty SNP markers were significantly associated with the genetic tolerance to TSC with both models. The proportion of total phenotypic variation explained (PVE) by the 40 SNPs was 56%, attributed to additive genetice ffects. Multiple candidate genes for disease resistance were identified in the región sindicated by the SNP markers. Their main functionsare signal transducersand receptors, transcription factors that positively regulatethe expression of tolerance genes and family kinase genes, there fore, they are potentially involved in the defense mechanism to TSC.

Cáncer palatosinusal t4a/b: extensión de las resecciones oncológicas / Palato-sinusal cancer: Extention of oncological resections

Antecedentes: la cirugía reconstructiva del tercio medio de la cara es compleja y variada. La vecindad anatómica con la órbita, la base del cráneo y el seno maxilar favorece la extensión tumoral del paladar a dichas estructuras, desafiando al cirujano que debe realizar una resección con intención curativa. Objetivo: obtener conclusiones sobre la supervivencia y el intervalo libre de enfermedad en cánceres palatosinusales T4a/b, sucesivamente operados durante un período de 30 años. Material y métodos: la cirugía se extendió a la órbita en el 85,2%, al cráneo en el 8,3%, al cuello en el 18,7% y a la glándula parótida en el 7,3%. La reconstrucción de partes blandas se realizó con colgajos libres en el 32,5%, musculares en el 21,6%, de vecindad en el 20,2%, musculocutáneos en el 14,2% y con piel en el 11,3%. Resultados: se produjeron complicaciones locales y generales. Estas últimas llevaron a la muerte de 4/203 ‒2%‒ pacientes. La supervivencia global a 5 años fue del 62,5% y la libre de enfermedad, del 53%, El análisis multivariado para recurrencia fue significativo en los vírgenes de tratamiento previo y para supervivencia a favor de los escamosos frente a otras estirpes histológicas. Conclusiones: en presencia de oftalmoplejía o compromiso del contenido orbitario o de ambos, la exenteración tiene indicación absoluta. La supervivencia a 5 años resulta aceptable si se tiene en cuenta que fueron solo estadios avanzados de la enfermedad. Los tratamientos previos con que concurrieron algunos pacientes fueron negativos para su evolución. La cirugía primaria desempeñó un papel esencial en la supervivencia libre de enfermedad.

Background: the problems of reconstructive surgery for the midface are variable and can be very complex. The anatomical proximity of the midface to the orbit, base of the skull and maxillary sinuses is a challenge for the surgeon who must perform a curative resection. Objective: The aim of this presentation is to report the survival rate and disease-free interval in T4a and T4b neoplasms of the palate and paranasal sinuses consecutively resected over a 30-year period. Material and methods: Surgery was extended to the orbit in 85.2%, the skull in 8.3%, the neck in 18.7% and the parotid gland in 7.3%. Soft tissue reconstruction was performed using free flaps in 32.5%, muscle flaps in 21.6%, local flaps in 20.2%, musculocutaneous flaps in 14.7% and skin flaps in 11.3%. Results: Local and general complications were reported, and 4/203 patients (2%) died. At 5 years, overall survival was 62.5% and disease-free survival was 53%. Univariate analysis revealed that lack of previous treatment was significantly associated with recurrence and squamous cell carcinoma was a predictor of survival. Conclusions: The indication of exenteration is mandatory in the presence of ophthalmoplegia or involvement of the orbital content. Survival at 5 years is acceptable, considering the advanced stages of the disease. In some patients, previous treatments were associated with adverse outcome. Primary surgery plays an essential role for disease-free survival.

Comparative outcomes of subcutaneous and transvenous cardioverter-defibrillators / 中华医学杂志(英文版)

BACKGROUND@#The comparative outcomes of subcutaneous implantable cardioverter-defibrillator (S-ICD) and transvenous ICD (T-ICD) have not been well studied. The aim of this study was to evaluate the safety and efficacy of currently available S-ICD and T-ICD.@*METHODS@#The study included 86 patients who received an S-ICD and 1:1 matched to those who received single-chamber T-ICD by gender, age, diagnosis, left ventricular ejection fraction (LVEF), and implant year. The clinical outcomes and implant complications were compared between the two groups.@*RESULTS@#The mean age of the 172 patients was 45 years, and 129 (75%) were male. The most common cardiac condition was hypertrophic cardiomyopathy (HCM, 37.8%). The mean LVEF was 50%. At a mean follow-up of 23 months, the appropriate and inappropriate ICD therapy rate were 1.2% vs. 4.7% (χ = 1.854, P = 0.368) and 9.3% vs. 3.5% (χ = 2.428, P = 0.211) in S-ICD and T-ICD groups respectively. There were no significant differences in device-related major and minor complications between the two groups (7.0% vs. 3.5%, χ = 1.055, P = 0.496). The S-ICD group had higher T-wave oversensing than T-ICD group (9.3% vs. 0%, χ = 8.390, P = 0.007). Sixty-five patients had HCM (32 in S-ICD and 33 in T-ICD). The incidence of major complications was not significantly different between the two groups.@*CONCLUSIONS@#The efficacy of an S-ICD is comparable to that of T-ICD, especially in a dominantly HCM patient population. The S-ICD is associated with fewer major complications demanding reoperation.

Carcinoma adenoquístico de las glándulas salivales / Adeno cystic carcinoma of salivary glands

Antecedentes: el carcinoma adenoquístico es el tumor maligno más frecuente de las glándulas sub-maxilar y menores. A pesar de su lento crecimiento y diseminación regional, muestra un pronóstico desfavorable debido a su tardía diseminación a distancia. Objetivo: analizar la serie propia a la luz de la bibliografia. Material y métodos: 44 pacientes con una edad media de 52,47 años y predominio del género femenino. De ellos, el 61,3% fueron tumores de glándula salival menor, 20,4% de submaxilar y 18,2% de parótida. En todos se realizó resección completa del tumor, seguida de vaciamiento cervical en el 34%. En el 43,2%, la cirugía fue seguida de Co60 radioterapia. El seguimiento medio de la muestra fue 52,5 (1-120) meses. Resultados: la curva de recurrencia acumulada fue del 36,8% a 5 años. La supervivencia global fue del 60 y 36% a 5 y 10 años, respectivamente, y se vio afectada con significación estadística por los márgenes insuficientes de la resección, el requerimiento de radioterapia adyuvante y el estadio. El análisis multivariado demostró que solo los márgenes insuficientes conservaron su significación estadística para la supervivencia y una tendencia desfavorable para el intervalo libre de enfermedad. Conclusiones: la extensión de la cirugía al cuello y la radioterapia adyuvante dependieron del estadio, los márgenes quirúrgicos, el compromiso nervioso, la presencia de adenopatas positivas y el subtpo histológico.

Background: adenoid cystic carcinoma is the most frequent malignant tumor of submaxillary and mi-nor salivary glands. Despite its slow growth, it shows an unfavorable prognosis because of its distant disseminaton. Objective: to analyse our series of patents in relaton to the literature. Materials and methods: 44 patents with mean age 52.47 years female gender. Of them, 61.3% were of minor salivary glands, 20.4% of submaxillary gland, and 18.2% the parotid gland. All patents underwent complete resecton of the tumor followed by neck dissecton in 34%. Co60 radiotherapy was administered to 43.2%. Mean follow up was 52.5 (range, 1-120) months. Results: 5-year recurrence rate was 36.8%. At 5 and 10 years, overall survival was 60 and 36%,respect-vely. Close surgical margins, requirement of adjuvant radiotherapy and stage significantly afected survival rate. The multivariate analysis showed that close margins was statstically significant for survival and associated with an unfavorable tendency for disease-free interval. Conclusions: extenton of surgery to the neck and adyuvant radiotherapy was dependent of stage, clear surgical margins, neural invasion, positive nodes in the neck and hystological subtype.

Impact of Single Dose Praziquantel Treatment on Schistosoma haematobium Infection among School Children in an Endemic Nigerian Community

Schistosomiasis is prevalent in Nigeria, and the foremost pathogen is Schistosoma haematobium, which affects about 29 million people. Single dose of the drug praziquantel is often recommended for treatment but the efficacy has not been documented in certain regions. Therefore, this study was designed to assess the impact of single dose praziquantel treatment on S. haematobium infection among school children in an endemic community of South-Western Nigeria. Urine samples were collected from 434 school children and 10 ml was filtered through Nucleopore filter paper before examination for egg outputs by microscopy. The prevalence was 24.9% at pre-treatment. There was no statistically significant difference for the prevalence of infection between males (14.7%) and females (10.2%), although the mean egg count for the females (9.87) was significantly more (P < 0.05) than the males (6.06). At 6 and 12 months post-treatment there was 74.4% and 86.4% reduction in the mean egg count, respectively. Interestingly, an increased prevalence of infection from 2.1% at 6 months to 7.7% at 12 months post-treatment was observed, nonetheless the mean egg count was reduced to 0.27 at 12th month from 1.98 at 6 months post-treatment. Resurgence in the prevalence rate between 6 and 12 months post-treatment with praziquantel is herein reported and the need for a follow-up treatment in endemic areas for adequate impact on schistosomiasis control is discussed.

Colgajos pediculados: Una alternativa no descartable en grandes defectos de la cabeza y el cuello / Pedicled faps: An alternative worth to be considered for large defects of the head and neck

Antecedentes: la cirugía de cabeza y cuello requiere la reconstrucción de los defectos que crea la resección de lesiones neoplásicas de dicha área. Para ello, se necesita el aporte de tejidos vecinos o tomados a distancia. Los colgajos libres cumplen a la perfección con tales principios. Sin embargo, los colgajos pediculados podrían suplir en gran medida las falencias que la aparatología y el personal entrenado ocasionan en un servicio de la especialidad. Objetivo: analizar la aplicabilidad, las ventajas y complicaciones de los colgajos pediculados, sin que signifquen la primera opción cuando hay que reconstruir un paciente. Resultados: los colgajos pediculados resultaron muy nobles en su aplicación tanto como cobertura como para reconstruir distintos sitos de la vía aerodigestiva superior. Solo se requirió una técnica depurada y la atenta preservación de su pedículo arteriovenoso. En general, se les reconocen ventajas y desventajas a todos. Sobresale entre ellos, el de trapecio lateral por la implícita posibilidad de incorporar hueso. La mayoría reveló reducida curva de aprendizaje, tempo operatorio comparado con el de los colgajos libres y baja tasa de complicaciones. Conclusiones: los colgajos pediculados no son cosa del pasado. En todos los servicios que no cuenten con un microcirujano entrenado, los colgajos pediculados deben formar parte del menú de opciones reconstructivas en la cirugía de cabeza y cuello.

Background: head and neck surgery requires reconstructon of defectis afer resecton of neoplastic lesions. Although free faps from close or distant locatons may largely satisfy this need, pedicled faps could replace the lack of aparatology and trained personnel in a specilized unit. Objective: to analyze applicability, advantages and complicatons of pedicled faps. Results: pedicled faps resulted a good opton for coverage or reconstructon of diferent areas of the aerodigestive tract. A neat technique and a careful preservaton of the arteriovenous pedicle were required. Although all of the pedicles used have advantages and disadvantages, the best of them resulted the lateral trapezius fap for it allows the associaton of bone to the fap. All pedicled faps showed shorter learning curve , operative tme and lower complicaton rate as compared to free faps. Conclusions: pedicled faps should not be considered a past issue. Any head and neck surgery unit without a trained microsurgeon should include pedicled faps as an opton for reconstructive procedures.

Liver Transplantation for Hepatocellular Carcinoma: Impact of Wait Time at a Single Center

ABSTRACT Introduction and aim. Liver transplantation (LT) provides durable survival for hepatocellular carcinoma (HCC). However, there is continuing debate concerning the impact of wait time and acceptable tumor burden on outcomes after LT. We sought to review outcomes of LT for HCC at a single, large U.S. center, examining the influence of wait time on post-LT outcomes. Material and methods. We reviewed LT for HCC at Mayo Clinic in Florida from 1/1/2003 until 6/30/2014. Follow up was updated through 8/1/ 2015. Results. From 2003-2014,978 patients were referred for management of HCC. 376 patients were transplanted for presumed HCC within Milan criteria, and the results of these 376 cases were analyzed. The median diagnosis to LT time was 183 days (8 - 4,337), and median transplant list wait time was 62 days (0 -1815). There was no statistical difference in recurrence-free or overall survival for those with wait time of less than or greater than 180 days from diagnosis of HCC to LT. The most important predictor of long term survival after LT was HCC recurrence (HR: 18.61, p < 0.001). Recurrences of HCC as well as survival were predicted by factors related to tumor biology, including histopathological grade, vascular invasion, and pre-LT serum alpha-fetoprotein levels. Disease recurrence occurred in 13%. The overall 5-year patient survival was 65.8%, while the probability of 5-year recurrence-free survival was 62.2%. Conclusions. In this large, single-center experience with long-term data, factors of tumor biology, but not a longer wait time, were associated with recurrence-free and overall survival.

Establishing national noncommunicable disease surveillance in a developing country: a model for small island nations / Introducción de la vigilancia nacional de las enfermedades no transmisibles en un país en desarrollo: un modelo para las pequeñas naciones insulares

ABSTRACT Objective To describe the surveillance model used to develop the first national, population-based, multiple noncommunicable disease (NCD) registry in the Caribbean (one of the first of its kind worldwide); registry implementation; lessons learned; and incidence and mortality rates from the first years of operation. Methods Driven by limited national resources, this initiative of the Barbados Ministry of Health (MoH), in collaboration with The University of the West Indies, was designed to collect prospective data on incident stroke and acute myocardial infarction (MI) (heart attack) cases from all health care facilities in this small island developing state (SIDS) in the Eastern Caribbean. Emphasis is on tertiary and emergency health care data sources. Incident cancer cases are obtained retrospectively, primarily from laboratories. Deaths are collected from the national death register. Results Phased introduction of the Barbados National Registry for Chronic NCDs (“the BNR”) began with the stroke component (“BNR–Stroke,” 2008), followed by the acute MI component (“BNR–Heart,” 2009) and the cancer component (“BNR–Cancer,” 2010). Expected case numbers projected from prior studies estimated an average of 378 first-ever stroke, 900 stroke, and 372 acute MI patients annually, and registry data showed an annual average of about 238, 593, and 349 patients respectively. There were 1 204 tumors registered in 2008, versus the expected 1 395. Registry data were used to identify public health training themes. Success required building support from local health care professionals and creating island-wide registry awareness. With spending of approximately US$ 148 per event for 2 200 events per year, the program costs the MoH about US$ 1 per capita annually. Conclusions Given the limited absolute health resources available to SIDS, combined surveillance should be considered for building a national NCD evidence base. With prevalence expected to increase further worldwide, Barbados’ experiences are offered as a “road map” for other limited-resource countries considering national NCD surveillance.

RESUMEN Objetivo Describir el modelo de vigilancia que se utilizó para crear el primer registro poblacional nacional de múltiples enfermedades no transmisibles en el Caribe (uno de los primeros registros de esta clase en el mundo), la ejecución del registro, las lecciones aprendidas y las tasas de incidencia y mortalidad desde sus primeros años de funcionamiento. Métodos Esta iniciativa del Ministerio de Salud de Barbados, realizada en colaboración con la Universidad de las Indias Occidentales e impulsada por la limitación de los recursos nacionales, tuvo por finalidad recoger datos prospectivos sobre los casos nuevos de accidente cerebrovascular e infarto agudo de miocardio en todos los establecimientos de atención de salud de este pequeño estado insular en desarrollo del Caribe oriental. El análisis se centró en las fuentes de datos sobre la atención de salud terciaria y de urgencia. La información sobre los casos nuevos de cáncer se obtuvo de manera retrospectiva, principalmente de los laboratorios. Los datos sobre las defunciones se tomaron del registro nacional de mortalidad. Resultados La introducción progresiva del Registro Nacional de Enfermedades Crónicas no Transmisibles de Barbados se inició con el componente de los accidentes cerebrovasculares en 2008, seguido del componente de infarto agudo de miocardio en 2009 y el componente de cáncer en 2010. Las estimaciones previstas con base en los estudios anteriores fueron en promedio de 378 casos de un primer accidente cerebrovascular, 900 casos de accidente cerebrovascular y 372 pacientes con infarto agudo de miocardio cada año; los datos del registro mostraron un promedio anual cercano a 238, 593 y 349 casos respectivamente. En el 2008, se registraron 1204 casos de cáncer, frente a los 1395 previstos. En función de los datos del registro se definieron los temas de capacitación en salud pública. El éxito de la iniciativa exigió fomentar el apoyo de los profesionales de salud a nivel local y dar a conocer la existencia del registro en toda la isla. Con un gasto cercano a 148 dólares por episodio y 2200 episodios por año, el programa cuesta al Ministerio de Salud alrededor de un dólar por habitante cada año. Conclusiones Dada la limitación de los recursos absolutos destinados a la salud en los pequeños estados insulares en desarrollo, es preciso analizar la posibilidad de realizar una vigilancia combinada, con el objeto de crear una base nacional de datos fidedignos sobre las enfermedades no transmisibles. Ante la perspectiva de un aumento continuo de la prevalencia mundial, la experiencia en Barbados se ofrece como una “hoja de ruta” destinada a otros países con recursos limitados que planean introducir la vigilancia nacional de las enfermedades no transmisibles.

Probiotic Properties and Antibiotic Resistance Pattern of Bacillus spp. Isolated from Two Types of Fermented Locust Bean (iru).

Bacillus spp. associated with two types of fermented African locust beans iru woro and iru pete were isolated and screened for probiotic potentials using standard microbiological techniques. The total bacterial counts for iru woro (pH 8.4) and iru pete (with pH 8.1) were 6.4314 and 6.4771 log10CFU/g respectively. In the two samples, the load of aerobic sporeformers were 6.2068 and 6.2553 log10CFU/g. In the samples Bacillus subtilis had the highest occurrence (44%), followed by B. lichenliformis (28%) and B. megaterium (24%) while B. coagulans had the least (4%). Only 28% of Bacillus isolates produced caseinase, while 28% produced haemolysin. Majority of these isolates showed tolerance to salt at concentrations less than 5% and also grew fairly at pH tending to neutral. Bacillus subtilis P14, Bacillus lichenliformis P12 and Bacillus megaterium P6 grew at 3.0% bile. Percentage hydophobicity, auto-aggregation and co-aggregation of the isolates ranged from - 49.00 to 65.00%, -53.00 to 84.00% and -69.44 to 36.08% respectively. High level of antibiotic resistance (especially to first line antibiotics) was recorded among isolates. Most of the Bacillus species isolated from the iru samples had very poor probiotic properties. Molecular and in vitro probiotic properties of promising candidates are still open to investigation.

Association of virulence genes with mecA gene in Staphylococcus aureus isolates from Tertiary Hospitals in Nigeria.

Introduction: Staphylococcus aureus is the etiological agent for a wide range of human infections, and its pathogenicity largely depends on various virulence factors associated with adherence, evasion of the immune system and damage of the host. This study determined the prevalence of methicillin-resistant S. aureus (MRSA) and some selected virulence genes in clinical isolates of S. aureus from South-Western Nigeria. Materials and Methods: The antibiotic susceptibility of 156 S. aureus isolates to various antibiotics was determined. Moreover, polymerase chain reaction detection of the mecA gene was performed including SCCmec typing, and the isolates were screened for selected genes (alpha hemolysin [hla], intracellular adhesion A [icaA], Panton-Valentine leukocidin [PVL], fibronectin binding protein A [fnbA], bone sialoprotein binding protein [bbp], exfoliative toxin A [eta], exfoliative toxin B [etb], and collagen binding adhesion [cna]) associated with virulence. Results: The prevalence of mecA gene was 42.3% (66 out of 156 S. aureus), and SCCmec typing showed that 24 (36.4%) carried the SCCmec II element, 4 (6.1%) with type III, 10 (15.2%) with SCCmec IV, and 28 (42.4%) harbored type V. The proportion of S. aureus with the following genes was ascertained: Hla (55.1%), icaA (42.3%), PVL (34.6%), fnbA (8.3%), bbp (4.5%), and eta (3.8%). All the isolates were etb and cna negative. The prevalence of the PVL gene in methicillin susceptible Staphylococcus aureus (MSSA) was 53.3% compared with 9.1% of MRSA. An association between virulence genes (eta and icaA) and mecA positive S. aureus; and significant difference in the distribution of virulence genes in in-patients and out-patients were found. The MRSA strains in South-Western Nigeria were dominated by SCCmec II and SCCmec V. Conclusion: The study concluded that there is a high prevalence of MRSA in Nigeria with association of eta and icaA genes with mecA gene in S. aureus isolates.

Trígono retromolar: Temible subsito del cáncer bucal / Retromolar trigone: A dreadful subsite of oral cancer

Antecedentes: el trígono retromolar es el sector de mucosa bucal situado detrás de los arcos dentarios superior e inferior. Los resultados de su tratamiento y las tasas de supervivencia han sido esporádicamente publicados solo en series retrospectivas. Objetivo: determinar los factores que afectaron la supervivencia y el intervalo libre de enfermedad de una serie de pacientes tratados con un protocolo preestablecido. Material y métodos: estudio retrospectivo, con revisión de historias clínicas e informes de anatomía patológica. Resultados: la extensión de la cirugía en 90 pacientes operados de inicio fue resección simple en 11, asociada a resección marginal de mandíbula en 23, segmentaria de mandíbula en 40, de las cuales se reconstruyeron solo 7, y parcial de maxilar superior en 21. En el 12,2% (11/90) se agregó un vaciamiento supraomohioideo y en 87,8% (79/90) vaciamiento de los 5 niveles. El 80,5% (83/103) recibieron radioterapia posoperatoria. Solo en el 12,6% (13/103) de los pacientes se aplicó un tratamiento de preservación con quimioterapia/radioterapia secuencial. La morbilidad resultó 81,1% y la mortalidad 5,5%.El 64% de los pacientes desarrolló recurrencias, con una media del 58,3% a los 5 años. En el análisis multivariado solo los márgenes de resección tuvieron significación estadística. La supervivencia global a 5 años fue del 46,7% y libre de enfermedad del 39,1%. En el análisis mul-tivariado solo la invasión perineural, los márgenes insuficientes y las adenopatas positivas fueron estadísticamente significativos. Conclusiones: el alto número de enfermos no pasibles de un tratamiento con intención curativa, que tenen infltración ósea, invasión perineural y extensión al cuello, hacen del trígono retromolar un temible subsito del cáncer bucal.

Background: the retromolar trigone is an area of the oral mouth located behind the superior and inferior dental archs. Results of its treatment and overall survival has been sporadically published in retrospective series. Objective: to discover factors which afect survival and free of disease interval in a series of patents under a preset protocol. Methods: retrospective study, review of medical records and pathologic reports. Results: The type of surgery in 90 patents included was: simple resecton, 11; combined with marginal resecton of mandible, 23; segmentary resecton, 40 (7 of them reconstructed with fbula); and partal maxillectomy, 21. A supraomohyoid neck dissecton was addedin 11/90 (12.2%) and a complete neck dissecton of fve levels, in 79/90 (97.8%). In 80.5% of the cases (83/103) received postoperative radiotherapy. In only 12.6% of the patents (13/103), preservaton treatment with sequental chemo/radiotherapy was applied. Morbidity was 81.1 % and mortality 5.5%. Sixty four per cent of patents relapsed, with a 5 years average of 58.3%. In the multivariate analysis for recurrence, only margin resecton was statstically significant. Five years overall survival was 46.7% and free of disease 39.1%. In multivariate analysis for survival, perineural invasion, margin resecton and node metastases were statstically significant. Conclusions: The high number of patents not suitable por curative surgery usually afected by bone infltraton, perineural invasion or extension to the neck suggests that the retromolar trigone is a dreadful subsite for oral cancer.

Temporal distribution of baseline characteristics and association with early mortality among HIV-positive patients at University College Hospital, Ibadan, Nigeria

The first six months of HIV care and treatment are very important for long-term outcome. Early mortality (within 6 months of care initiation) undermines care and treatment goals. This study assessed the temporal distribution in baseline characteristics and early mortality among HIV patients at the University College Hospital; Ibadan; Nigeria from 2006-2013. Factors associated with early mortality were also investigated. This was a retrospective analysis of data from 14 857 patients enrolled for care and treatment at the adult antiretroviral clinic of the University College Hospital; Ibadan; Nigeria. Effects of factors associated with early mortality were summarised using a hazard ratio with a 95% confidence interval obtained from Cox proportional hazard regression models. The mean age of the subjects was 36.4 (SD=10.2) years with females being in the majority (68.1%). While patients' demographic characteristics remained virtually the same over time; there was significant decline in the prevalence of baseline opportunistic infections (2006-2007=55.2%; 2011-2013=38.0%). Overall; 460 (3.1%) patients were known to have died within 6 months of enrollment in care/treatment. There was no significant trend in incidence of early mortality. Factors associated with early mortality include: male sex; HIV encephalopathy; low CD4 count ( 50 cells); and anaemia. To reduce early mortality; community education should be promoted; timely access to care and treatment should be facilitated and the health system further strengthened to care for high risk patients

Association of Enterococcus faecalis with Different Forms of Dental Diseases among Patients Visiting a Tertiary Hospital in Ekiti State, Nigeria.

Aims: The implication of Enterococcus faecalis in dental infections is rising and resistance to common antibiotics continues to rise globally as well. In this study, the association of E. faecalis with different forms of dental infections was investigated and the antibiotic resistance profile of the isolates was determined. Place and Duration of Study: The samples were collected from a tertiary health institution in Ekiti State, transferred to the Department of Microbiology, Ekiti State University, Ado-Ekiti, Nigeria and processed immediately. This study was carried out between June, 2009 and March, 2010. Methodology: Oral interview was conducted among the patients in order to collect relevant data while sterile culturette was used to collect the samples after proper diagnoses, plated and incubated appropriately using standard microbiological techniques. The susceptibility of the isolates to commonly and frequently used antibiotics was determined by the disc diffusion method on Müller-Hinton agar. Results: Overall, 46.0% of the subjects had dental infections The subjects with dental infections were 46.60% males and 53.40% females. Students accounted for 58.25% of the total subjects followed by traders with 16.50%. Enterococcus faecalis was isolated from 52.08% of the students, 22.92% traders and 4.17% of the farmers. The highest occurrence (37.86%) of dental infection was recorded among patients within the age bracket 21 and 30 years while dento-aveola abscess (DAA) followed by dental caries (DC) were the predominant dental infections recorded in that order. A total of 46.60% of the dental infections were colonized by E. faecalis that were mostly resistant to amoxicillin (93.90%) and cloxacillin (92.68%). Resistance to the fluoroquinolones tested was relatively low, ranging from 8.54 to 25.61% in spafloxacin and perfloxacin, respectively whilst it was 1.22% to vancomycin among the isolates. Conclusion: The isolates were resistant to common antibiotics tested, however, vancomycin proved to be the most effective in the inhibition of the isolates.

In vitro Antibacterial Activity of Aqueous Extracts of Cashew (Anacardium occidentale L.) Fruit Peels Using Bioautography Method.

Aims: Bark, leaves and gum of cashew (Anacardium occidentale L.) have been reported to be effective in curtailing the growing problems of resistance of bacterial pathogens. The in vitro activity of aqueous extracts of cashew apple peels was determined in this study against two clinically important pathogens. Place and Duration of Study: The work was conducted at the Department of Microbiology, Ekiti State University, Ado-Ekiti, Nigeria and processed immediately. This study was carried out between June, 2009 and January, 2010. Methodology: Bioautographic method was used to test the antibacterial activity of aqueous (cold and hot water) extracts of cashew apple peels on Escherichia coli O157:H7 and methicillin-resistant Staphylococcus aureus (MRSA). The zones of inhibition of the extracts were compared. Results: The activity of the fifth hour hot water extract was highest with zones of inhibition of 415.48 and 346.30 sq. mm against E. coli O157:H7 and MRSA respectively. E. coli O157:H7 was more susceptible to the extract with the zone of inhibition ranging between 176.79 and 283.53 sq. mm while that of MRSA was153.94 - 346.30 sq. mm. The 5 h extract of cold water was more potent on the test organisms with 615.75 and 490.87 sq. mm diameters of inhibition on E. coli O157:H7 and MRSA respectively. Cold water extracts produced more active compounds (13 biologically active spots) that inhibited the growth of the test organisms than the hot water extracts, with six spots. Conclusion: The extracts of the peels of the cashew apple against the test organisms are promising. However, the nature and mechanisms of action of the biologically active compounds in the extracts are still open to investigation.

Paragangliomas cervicales. Resultados actuales comparados con los de la serie histórica / Cervical paragangliomas. Comparison of current results with historical series

Antecedentes: los paragangliomas cervicales son tumores poco frecuentes, que reciben diferentes denominaciones según su ubicación. Los que asientan en la bifurcación carotdea son llamados ca-rotdeos; en el oído medio, tmpánicos; en el bulbo yugular, yugulares y en el nervio vagal, vagales. Lugar de aplicación: hospital público de atención terciaria de tumores. Población: veintséis pacientes, 22 de ellos operados. La edad media fue 48,6 años, el 77% del sexo femenino. Método: revisión de historias clínicas e informes de anatomía patológica. Resultados: doce eran izquierdos, 11 derechos, 2 bilaterales y 1 múltple. Se llegó al diagnóstico por la semiología, por angiorresonancia y por tomografa computarizada. Fueron divididos según la clasificación de Shamblin en grado 1-2 en 16 y grado 3 en 10. La extensión de la cirugía demandó en todos la resección del tumor que implicó en 2 la reparación vascular, en 2 la resección de los pares craneales XI y XII y en los 4 restantes por ser asintomáticos solo la observación. La morbilidad fue del 22,7%, las secuelas del 31,8% y la mortalidad del 4,5% distnta a la presentada en la serie histórica de 44,4%, 55,5% y 5,4%, respectvamente. Conclusiones: 1) Los tumores del cuerpo carotdeo son infrecuentes, benignos en su mayoría y de crecimiento lento. 2) Los recursos diagnósticos más utlizados fueron: el ecodoppler, la tomografa computarizada con contraste y la angiorresonancia, con resultados positvos en el 100% de los casos, a diferencia de la serie histórica donde se utlizó la angiografa con éxito en el diagnóstico en el 70%. 3) Los pacientes con tumores I, II y III sintomáticos de la clasificación de Shamblin son quirúrgicos con un porcentaje de secuelas del 31,8 % a diferencia de la serie histórica del 55,5 %. 4) En los tumores tpo III asintomáticos la selección de los pacientes debe ser cuidadosamente considerada para recomendar una cirugía agresiva y con alta morbilidad. Esta conclusión difere de la presentada en la serie histórica, donde todos los pacientes fueron intervenidos quirúrgicamente.

Background: cervical paragangliomas are very rare tumours and receive diferent names according to the site of origin. Carotd for those localized in the carotd bifurcaton, tympanic for those in the middle ear, yugular for those in the yugular bulb and vagal for those in the vagal nerve. Setng: tertary care oncologic public hospita. Populaton: 26 patents, 22 of them surgically treated. Mean age was 48.6 years, 77% female. Methods: review of clinical records and pathologic reports. Results: twelve were at the lef side, 11 at the right, 2 bilateral and 1 multple. Diagnosis was done by semiology, magnetic resonance and computed tomography. The tumors were classifed according to Shamblin classificaton in 1, 2 grades in 16 patents and 3 in 10. Surgery included tumor resecton with vascular graf in 2, XI and XII craneal nerve also in 2, while 4 patents remained in observaton because they were asymptomatic. Morbidity was 22.7%, sequels 31.8% and mortality 4.5%, diferent to the historic series with 44.4%, 55.5% and 5.4% respectvely. Conclusions: tumors of carotd body are infrequent, benign and of slow growth. Diagnostic techniques frequently used were ecodopler, CT scan and MRI, with positve results in about 100% of cases. It was diferent to historic series in which angiographic studies had positve results in only 70% of them. Symptomatic Shamblin 1, 2 and 3 were surgically treated with 31.8% of sequels, diferent to historic sample with 55.5%, but in asymptomatic grade 3, careful selecton of patents must be taken into ac-count for avoiding an aggressive surgery with high morbidity. This conclusion is diferent of the historic series in which all patents were surgically treated.

Técnica de preservación del volumen alveolar post-extracción utilizando hueso liofilizado y lámina ósea cortical / Preservation techniques of alveolar post-extraction volume using liophilisate bone and cortical bone sheets

Se evaluó la eficacia del hueso liofilizado humano (Matriz Ósea UNC en Polvo) injertado en cavidades alveolares post-extracción, recubierto por una lámina ósea cortical (Matriz Ósea UNC en membrana), en el tratamiento de preservación del perfil volumétrico del reborde alveolar.La metodología de trabajo se fundamentó en: 1) El estudio de una casuística de 27 casos clínicos en pacientes de ambos sexos que poseían elementos dentarios unirradiculares con indicación de extracción. Se injertó en las cavidades óseas resultantes hueso liofilizado, contenido in situ mediante una lámina ósea cortical parcialmente desmineralizada. Los pacientes fueron evaluados clínica y radiográficamente, mediante modelos de estudio, Rx convencional y radiovisiografía que permitieron mensurar las modificaciones producidas por resorción durante un año. Se realizaron controles pre y post-operatorios, a los 7, 15, 30, 60, 90, 120, 180 y 360 días. A los modelos de yeso preliminares y a los obtenidos a los 120 y 360 días se les efectuaron cortes transversales en las zonas de estudio y se los escaneó. Las imágenes obtenidas se procesaron mediante un analizador de imágenes (Image Pro-Plus). Los datos se analizaron estadísticamente con software específico (SPSS). El estudio demostró que las mayores modificaciones dimensionales del reborde se observaron sobre el área superficial del alvéolo y en los primeros 4 meses post-extracción. La lámina cortical presentó características físicas, estructurales y biológicas que le permitieron actuar como barrera física oclusiva, minimizando los fenómenos de inhibición celular heterotípica y favoreciendo los procesos osteogénicos por el mecanismo de osteopromoción...

Human efficacy lyophilized bone (UNC Bone Matrix Powder) grafted post-extraction alveolar sacs, covered by a cortical bone plate (UNC Bone Matrix membrane), in the treatment volume preservation ridge profile was evaluated. The working methodology was based on: 1) The study of a case series of 27 clinical cases in patients of both sexes who had single-rooted tooth elements indicating extraction. It was grafted bone cavities in the resulting lyophilized bone content in situ by a partially demineralized cortical bone plate. Patients were evaluated clinically and radiographically, using study models, and conventional Rx radiovisiography that allowed mensurar resorption induced changes for a year. Pre and post-operative controls at 7, 15, 30, 60, 90, 120, 180 and 360 days were performed. A preliminary plaster models and those obtained at 120 and 360 days transects were conducted in the study areas and were scanned. The images obtained were processed by an image analyzer (Image Pro-Plus). The data were statistically analyzed with software (SPSS) .The study showed that older flange dimensional changes were observed on the surface area of the alveoli and in the first 4 months post-extraction. The cortical sheet submitted physical, structural, and biological characteristics that allowed him to act as occlusive physical barrier, minimizing heterotypic cellular inhibition phenomena and processes favoring osteogenic mechanism osteopromoción...