S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200001 Caricaturas de Médicos Ginebrinos Jiménez-Bonilla Rafael ,Comité Editorial AMC 06 2010 2010 52 68 70
S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200002 Malformaciones anorectales a través del tiempo Arrea Baixench Carlos ,Academia Nacional de Medicina Comité Editorial AMC 06 2010 2010 52 71 72
S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200003 Dr. Solón Núñez Frutos Jiménez Bonilla Rafael ,Comité Editorial AMC 06 2010 2010 52 73 75 Schilling Paulo El Imperio Rockefeller: América Latina El Imperio Rockefeller: América Latina 1970 Tierra Nueva Homenaje al Dr. Solón Núñez Frutos Homenaje al Dr. Solón Núñez Frutos 1963 San José Homenaje al doctor Solón Núñez Frutos Homenaje al doctor Solón Núñez Frutos 1958 Murillo Gabriela Galván Brayan Solano Karen Seminario de Realidad Nacional I Seminario de Realidad Nacional I 2001 Universidad de Costa Rica Frutos Verdesia Juan Bautista Doctor Solón Núñez Frutos Doctor Solón Núñez Frutos 1979 San José Departamento de Publicaciones del Ministerio de Cultura Juventud y Deportes
S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200004 Elementos básicos de bioética en investigación Arguedas-Arguedas Olga ,Caja Costarricense de Seguro Social Hospital Nacional de Niños Dr. Carlos Sáenz Herrera 06 2010 2010 52 76 78
S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200005 Regulación del peso corporal y del apetito Body Weight and Appetite Regulation Vásquez-Machado Moisés Ulate-Montero Guido ,Universidad de Costa Rica Departamento de Fisiología Escuela de Medicina 06 2010 2010 52 79 89

Debido a que la epidemia mundial de obesidad no muestra signos de disminución, se ha hecho necesaria una mejor comprensión de los mecanismos fisiológicos subyacentes a la homeostasis energética, el proceso mediante el cual el consumo energético se iguala al gasto energético a lo largo del tiempo, de manera que los almacenes energéticos corporales, en la forma de tejido adiposo, se mantienen constantes a pesar de las variaciones diarias en la ingesta calórica. El sistema que controla el balance energético posee, a su vez, dos componentes: uno en el corto y otro en el largo plazo. El sistema, en el corto plazo, se encarga de regular el apetito o inicio y finalización de comidas individuales, y responde, fundamentalmente, a hormonas gastrointestinales o señales de saciedad que se acumulan durante la alimentación y contribuyen a terminar la ingesta. Los factores de adiposidad, como la leptina e insulina, son señales que se liberan en proporción a los depósitos energéticos del organismo, y su función involucra la regulación del balance energético por periodos prolongados, por lo que se encargan de la estabilidad del peso corporal. La vía central de las melanocortinas representa un punto crucial de integración de estas señales. Los ligandos de los receptores de melanocortina son sintetizados en poblaciones neuronales discretas dentro del núcleo arcuado del hipotálamo, y ejercen acciones en los dos componentes del balance energético. Sumado a su capacidad de respuesta frente a los niveles de hormonas circulantes, el cerebro también responde directamente a los niveles circulantes de nutrientes. Se han identificado dos proteincinasas que sensan los niveles de nutrientes y funcionan como reguladoras del peso corporal y consumo energético en el hipotálamo: mTOR y AMPK. Además de estos circuitos homeostáticos, los mecanismos hedónicos de alimentación son importantes en la regulación del consumo energético, superando la capacidad reguladora del sistema de balance energético. El objetivo de este artículo es revisar avances recientes en la comprensión de los mecanismos reguladores del peso corporal y el apetito, los cuales han ampliado la visión de la fisiopatología de la obesidad, al tiempo que ofrecen diversas perspectivas para su tratamiento.

Due to the fact that the obesity epidemic shows no signs of diminishing, a better understanding of the physiological mechanisms underlying energy homeostasis has become necessary. During this process energy intake is matched to energy expenditure over time, in such a way that body fuel stored in the form of adipose tissue is held constant despite daily fluctuations in caloric intake. The system that controls energy balance possesses 2 components: a short and a long-term. The short-term system is in charge of appetite regulation or the initiation and termination of individual meals. It responds basically to gut hormones or satiety signals that accumulate during eating and ultimately contribute to meal termination. Adiposity factors are circulating signals generated in proportion to the body energy storages, such as insulin and leptin, levels of which are involved in the regulation of energy balance over long intervals thereby promoting body weight stability. The central melanocortin pathway represents a crucial integration point for these signals. Melanocortin receptor ligands are synthesized by discrete neuronal populations within the arcuate nucleus of the hypothalamus and exert actions in both components of the energy balance equation. In addition to hormones, the brain also responds directly to the nutrient circulating levels. Two fuel-sensing protein kinases functioning as main regulators of body weight and food intake in the hypothalamus have been identified: mTOR and AMPK. Besides these basic homeostatic circuits, the hedonic mechanisms of feeding are important in the regulation of energy intake since they can override the energy balance system regulatory capability. The aim of this article is to review recent advances in the comprehension of the mechanisms underlying body weight and appetite regulation. Those mechanisms not only allow a new understanding about obesity pathophysiology but also provide new perspectives for its treatment.

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S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200006 Carcinomas invasores triples negativosde la glándula mamaria: incidencia y características clínico-patológicas Triple-Negative Invasive Breast Carcinoma: Incidence and Clinical - Pathological Characteristics Quirós-Alpízar José L Jiménez-Rodríguez Yendry Jiménez-Montero Ernesto Monge-Víquez Melissa Salazar-Sánchez Lizbeth ,Universidad de Costa Rica Escuela de Medicina Departamento de Anatomía ,Universidad de Costa Rica Escuela de Medicina ,Hospital San Juan de Dios Servicio de Anatomía Patológica ,Universidad de Costa Rica Centro de Investigación en Hematología y Trastornos Afines (CIHATA) 06 2010 2010 52 90 95

Justificación y objetivo: El comportamiento epidemiológico de los tumores malignos del país ha presentado un cambio en los últimos años; el cáncer de mama ha aumentado su incidencia hasta colocarse como el segundo tumor maligno más frecuente en la mujer y el primero en mortalidad. Este tipo de tumor tiene características que se pueden estudiar con las técnicas de inmunohistoquímica, como son la expresión de receptores en las células neoplásicas (estrógenos, progesterona y el HER2 o receptor 2 del factor de crecimiento epidérmico humano). Los carcinomas que no expresan ninguno de estos tres receptores tienen un peor pronóstico. El estudio tiene como objetivos conocer las principales características de este grupo de tumores denominados triples negativos, y determinar su relevancia dentro del grupo total del carcinoma de mama. Materiales y métodos: Se recolectaron los casos diagnosticados de cáncer de mama en mujeres, en el periodo comprendido entre el 1 de enero y el 31 de diciembre de 2006, en el Hospital San Juan de Dios, en el Servicio de Patología, que dispusieran de estudios inmunohistoquímicos. Se consideró la información acerca de la edad de las pacientes, así como el tipo histológico y grado de diferenciación de cada uno de los tumores. Se separó el grupo correspondiente a los tumores triple negativo y se realizaron los análisis estadísticos por medio del programa Epi Info 3.3.4. Resultados: De una población de 221 pacientes diagnosticadas con cáncer de mama en el periodo mencionado, se presentaron 40 pacientes con cáncer de mama triple negativo, resultando en una incidencia de un 18% (I.C. 95%, 12,79 - 23,40) y una edad promedio de 54 años (I.C. 95%, 50 - 59), con una mediana de 52 y una moda de 48. El cáncer de mama triple negativo tuvo una relación mayor con el tipo histológico ductal infiltrante, con un 67,5% (I.C. 95%, 51,7383,26); seguido por el lobulillar infiltrante, con un 12,5% (I.C.95%, 4,18 - 26,80); el medular con un 5% (I.C. 95%, 0,61 - 16,92), y con menos relación el tipo papilar y el metaplásico fusocelular, ambos con un 2,5% (I.C. 95%, 0,06 - 13,16). Con respecto al grado de diferenciación, hubo 23 casos con grado 3, 8 con grado 2 y 3 con grado 1. Conclusiones: Los cánceres de mama triple negativo son un grupo de tumores agresivos, que se manifiestan a edad más temprana, con mayor tamaño, grado histológico alto, principalmente son carcinomas ductales infiltrantes. Debido a que el estudio se basó solo en pruebas de receptores hormonales y HER-2, se podría realizar otras pruebas como tinción de CK5/6 y 17, para comprobar los casos de carcinomas basales. Además, se observa que los carcinomas lobulillares infiltrantes muestran una mayor prevalencia que en otros estudios, de manera que se podrían efectuar pruebas como la e-cadherina, para comprobar los casos de carcinomas lobulillares y aumentar la fidelidad de este estudio.

Aim: The epidemiological behavior of malignant tumors in our country has undergone an important change during the last years, Breast carcinoma has increased its incidence, occupying the second place in malignancies in women and the first one in mortality. This type of tumor has characteristic features from the immunohistochemistry, standpoint since they can express different receptors such as estrogens, progesterone and HER2 or receptor 2 of epidermal human growth factor. It is known that carcinomas that do not express any of these 3 receptors have worse prognosis. Our objective is to define the main characteristics of the so called triple negative tumors and determine their rol within the total group of breast carcinomas. Methodology: All cases of breast cancer seen at the Pathology Department of the Hospital San Juan de Dios, from January 1 to December 31, of 2006 were included if they had immunohistochemistry studies. The age of the patient;s, and the histological type and degree of differentiation of each one of the tumors was recorded. The triple negative group was separated and an statistical analyses was undertaken utilizing the Epi Info 3.3.4 program. Results: Of 221 patients diagnosed with breast carcinomas, 40 patients were identified as triple-negative cancer, giving an incidence of 18% (I.C. 95%, 12,79 - 23,40), they had an average age of 54 years (I.C. 95%, 50 - 58 ) with a median of 52 and mode of 48. Likewise, triple-negative cancer individuals were more related with ductal infiltratiing: 67,5% (I.C. 95%, 51,73-83,26), followed by unfiltrating lobular:12,5% (I.C.95%, 4,18 - 26,80), medular, 5 % and with other such as papillary Regarding the degree of differentiation, 23 cases were III degree, 8 were II degree , and 3 were I degree. Conclusion: Triple-negative breast cancers affect young women, are of higher histological grade, have larger tumor size and have a more aggressive clinical behavior, and preferentially they are of infiltrating ductal histological type. The incidence in our study seems to be higher than the one reported in the world literature. Due to the fact that this was based only on determination of receptors, other tests might be necessary such as CK5/6 and 17 stains to verify the cases. In addition, in this study a high proportion of tumors were of lobular type, other tests might be indicated such as e-cadherin to verify the cases of lobular carcinomas to increase in this way the reliability of results.

 cáncer de mama triple negativo inmunohistoquímica receptores de estrógenos y progesterona receptor 2 del factor humano de crecimiento epidermal (HER-2) breast cancer triple-negative immunohistochemistry progesterone and estrogen receptors HER-2 Incidencia de tumores malignos más frecuentes en mujeres en Costa Rica 1995-2006 Incidencia de tumores malignos más frecuentes en mujeres en Costa Rica 1995-2006 2008 Unidad de Estadística, Registro Nacional de Tumores Rosen P Patología mamaria de Rosen Patología mamaria de Rosen 2005 Seg Editorial AMOLCA Sørlie T Perou CM Tibshirani R Aas T Geisler S Johnsen Gene expression patterns of breast carcinomas distinguish tumor subclasses with clinical implications Proc Natl Acad Sci 2001 98 Hayes DF Thor AD c-erbB-2 in breast cancer: development of a clinically useful marker Semin Oncol 2002 29 Alejandro C Gene expression profiles for therapeutic decisions in breast cancer Rev. méd Chile 2007 135 Mocellin S Provenzano M Rossi CR Pilati P Nitti D Lise M DNA array-based gene profiling: from surgical specimen to the molecular portrait of cancer Ann Surg 2005 241 van’t Veer L Dai H van de Vijver M He Y Hart A Mao M Gene expression profiling predicts clinical outcome of breast cancer Nature 2002 415 Cleator S Heller W Coombes C Triple negative breast cancer: therapeutic options Lancet Oncol 2007 8 Bauer K Brown M Cress R Parise C Caggiano V Descriptive analysis of estrogen receptor (ER)-negative, progesterone receptor (PR)-negative and HER2-negative invasive breast cancer, the so-called triple-negative phenotype Cancer 2007 109 Nelson HD Fuman LH Fu R Harris EL Genetic risk assessment and BRCA Mutation testing for breast and ovarian cancer susceptibility: systematic evidence review for the U.S. preventive services task force Ann Intern Med 2005 143 Análisis Epidemiológico de Datos Tabulados (EPIDAT): Organización Mundial de la Salud, Organización Panamericana de la Salud Análisis Epidemiológico de Datos Tabulados (EPIDAT): Organización Mundial de la Salud, Organización Panamericana de la Salud Jiménez G Villalobos M Jiménez E Palma W Determinación de la efectividad de cinco protocolos de extracción de ADN a partir de material parafinado para estudios moleculares Rev med UCR 2007 1 Rakha E.A Morphology and immunophenotypic analysis of breast carcinoma with basal and myoepithelial differentiation J Pathology 2006 208 Jung Kim Mi Jae Y Ro Sei-Hyun Ahn Hak Hee Kim Sung Bae Kim Gyungyub Gong Clinicopathologic significance of the basal-like subtype of breast cancer: a comparison with hormone receptor and Her2/neu-overexpressing phenotypes Human Pathology 2006 37 Spitale A Mazzola P Soldini D Mazzuccheli L Bordoni A Breast cancer classification according to immunohistochemical markers: clinicopathologic features and short-term survival analysis in a population-based study from the South of Switzerland Ann Oncol 2008
S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200007 Determinación de arsénico en abastecimientos de agua para consumo humano de la provincia de Cartago, Costa Rica Arsenic Determination in Water Supplies for Human Consumption of the Province of Cartago, Costa Rica Montero-Campos Virginia Quesada-Kimsey Jaime Ledezma-Espinoza Aura Sandoval-Mora José A ,Instituto Tecnológico de Costa Rica Escuela de Química Centro de Investigación y de Servicios Químicos y Microbiológicos- CEQIATEC 06 2010 2010 52 96 101

Objetivos: Divulgar conocimiento científico sobre el hidroarsenicismo en América Latina, y determinar la presencia de arsénico en agua utilizada para consumo humano en lugares de riesgo en Costa Rica, específicamente en la provincia de Cartago, en los cantones de Oreamuno, Central, Paraíso y Alvarado. Métodos: Cuantificación de arsénico en su forma reducida trivalente, mediante generación de hidruros metálicos volátiles por absorción atómica de llama, metodología 7062 de la Agencia de Protección Ambiental de Estados Unidos. Resultados: Ninguna de las muestras analizadas superó el límite máximo permitido por la legislación actual costarricense, de 10 µg/L. Conclusión: A pesar de que las áreas de investigación correspondieron a zonas de alto riesgo por su naturaleza volcánica, la amplia oferta hídrica de los cantones marco del estudio, con áreas de relativa protección circundando las zonas de recarga de las nacientes, hace que la población consuma agua de origen subterráneo, pero de influjo directo superficial, con menor influencia de rocas volcánicas que se encuentran a mayor profundidad.

Aim: To disclose scientific knowledge about hydroarsenicism in Latin America, as well as to determine the presence of arsenic in water used for human consumption in risk areas of Costa Rica, specifically at the Central, Oreamuno, Paraíso and Alvarado counties of the province of Cartago. Methods: Quantification of inorganic arsenic by hydride generation and flame atomic absorption, according to method 7062 of the US Agency of Environmental Protection. Results: None of the analyzed samples surpassed the maximum limit of 10 µg/L allowed by the present Costa Rican legislation. Conclusion: Even though sampled areas are of volcanic nature and therefore of arsenic risk, the population consumes water of direct underground origin with a great superficial influence and little or no contact to deeper volcanic rock, thus not contaminated with arsenic.

 arsénico agua potable hidroarsenicismo crónico arsenic drinking water chronic hidroarsenicism Resumen de Salud Pública Arsénico CAS#: 7440-38-2 Resumen de Salud Pública Arsénico CAS#: 7440-38-2 2007 A go Agencia para las Sustancias Tóxicas y el Registro de Enfermedades (ATSDR) División de Toxicología y Medicina Ambiental Arsenic: GUIDELINES FOR DRINKINGWATER QUALITY Arsenic: GUIDELINES FOR DRINKINGWATER QUALITY 2003 Arsénico en agua de pozos domésticos Arsénico en agua de pozos domésticos 2003 Guidelines for Canadian Drinking Water Quality Supporting Guidelines for Canadian Drinking Water Quality Supporting 2007 Castro M Presencia de arsénico en el agua de bebida en América Latina y su efecto en la salud pública Presencia de arsénico en el agua de bebida en América Latina y su efecto en la salud pública Congreso International Ciudad de México 20-24 June 2006 Ryker S Mapping Arsenic in Groundwater in United States Geotimes 2001 Jornadas Iberoamericanas del impacto del arsénico sobre la salud de poblaciones iberoamericanas: Herramientas para la evaluación y manejo de riesgos, 2006 Jornadas Iberoamericanas del impacto del arsénico sobre la salud de poblaciones iberoamericanas: Herramientas para la evaluación y manejo de riesgos, 2006 Moya R Leandro L Monge F Preservación de postes de melina mediante el método vacío-presión con sales de CCA-C. Kurú Revista Forestal 2004 1 Campos J León J C Bravo S Contenido de arsénico III en agua de la comunidad de Santa Bárbara de Heredia Memoria Jornadas de Investigación 1999 Universidad de Costa Rica Gómez A Monitoreo y atención de intoxicados con arsénico en el Zapote, Municipio de San Isidro, Departamento de Matagalpa, Nicaragua 1994-2002: El arsénico y metales pesados en aguas de Nicaragua Monitoreo y atención de intoxicados con arsénico en el Zapote, Municipio de San Isidro, Departamento de Matagalpa, Nicaragua 1994-2002: El arsénico y metales pesados en aguas de Nicaragua 2003 UNICEF El arsénico y metales pesados en aguas de Nicaragua: Puntos de abastecimiento de agua contaminada por arsénico y plomo identificados en Nicaragua en julio de 2002 El arsénico y metales pesados en aguas de Nicaragua: Puntos de abastecimiento de agua contaminada por arsénico y plomo identificados en Nicaragua en julio de 2002 PIDMA/UNICEF Arsenic Guidelines for Canadian Drinking Quality Ottawa 1992 Frederick P Kenneth B Chien-Jen C Health Implications of Arsenic in Drinking Water Journal AWWA 1994 86 Cantor K Arsenic in Drinking Water: How Much Is Too Much? Epidemiology 1996 7 Cebrián M Albores A García G Del Razo L Chronic Arsenic Poisoning In Humans: The Case of Mexico. Arsenic in the Environment. Part II: Human Health and Ecosystem Effects 1994 Bates M Rey O Biggs M Hopenhayn C Moore L Kalman D Case-Control Study of Bladder Cancer and Exposure to Arsenic in Argentina Am J Epidemiol 2004 159 Miller J Miller J Estadística y quimiometría para química analítica: Errores en análisis instrumental: Regresión y correlación Estadística y quimiometría para química analítica: Errores en análisis instrumental: Regresión y correlación 2002 Madrid Prentice Hall
S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200008 Caracterización de pacientes pediátricos con neutropenia enviados a un hospital de referencia Characterization of neutropenic pediatric patients sent to a referral centre Adolfo Lazo-Páez Gustavo Porras Oscar ,Caja Costarricense de Seguro Social Hospital Nacional de Niños Dr. Carlos Sáenz Herrera Servicio de Inmunología y Reumatología Pediátrica San José 06 2010 2010 52 102 108

Objetivo: La neutropenia es un motivo relativamente frecuente de referencia al Servicio de Inmunología y Reumatología Pediátrica del Hospital Nacional de Niños; el estudio pretende caracterizar los casos de neutropenia referidos a este Servicio en el periodo comprendido entre noviembre de 1988 y junio de 2008. Métodos: Se estudiaron 84 pacientes entre 0 y 12 años de edad, referidos entre el 6 de noviembre de 1988 y el 1 de junio de 2008. Se efectuó un análisis descriptivo global de las características presentadas por estos pacientes en términos de evolución clínica, patrón de infección, gérmenes más frecuentes causantes de infección, complicaciones y tratamiento. Resultados: El 52.2% de los pacientes analizados resolvieron su neutropenia espontáneamente, por lo que fueron catalogados como neutropenia transitoria; el 21.7% de los casos evolucionó como neutropenia cíclica; el 13% de los pacientes fueron catalogados como neutropenia crónica benigna; el 7.2% evolucionaron como neutropenia crónica grave sintomática; el 2.9% tuvieron neutropenia asociada a glucogenosis tipo 1B, y el 2.9% de los casos no fueron clasificables en las categorías propuestas. El 56.5% de los casos se asoció a un patrón de infección anormal, sea por incremento en la frecuencia, mayor gravedad, compromiso multisistémico o presencia de microorganismos oportunistas. El sistema más afectado por infección fue la vía respiratoria superior. El 39.1% de los casos de neutropenia evaluados ameritaron uso de antibióticos profilácticos, y el 11.6% de los casos requirieron usar factor estimulante de colonias granulocíticas, en algún momento de su evolución. Los gérmenes más frecuentemente involucrados en infección fueron Pseudomona aeruginosa, Staphylococcus spp y E. coli. Conclusión: La gran mayoría de los pacientes neutropénicos estudiados tiene un curso clínico benigno caracterizado por pocas hospitalizaciones y una intervención farmacológica relativamente simple. Las infecciones documentadas en los casos analizados suelen ser causadas por los mismos gérmenes descritos en otras series. El conteo absoluto de neutrófilos al diagnóstico no incide sobre la frecuencia de infección recurrente, pero el tipo o curso clínico de la neutropenia sí lo hace.

Aim: Neutropenia is a relatively common cause of patient referral to the Immunology and Pediatric Rheumatology Department of the National Children’s Hospital. The present study characterizes the cases of neutropenia referred to this department between November 1988 and June 2008. Methods: Eighty four patients between 0 and 12 years of age, were referred from November 6th, 1988 and June 1st, 2008. We performed a comprehensive descriptive analysis of the characteristics exhibited by these patients in terms of clinical course, pattern of infection, most common causative germs, complications and treatment applied. Results: Neutropenia resolved spontaneously in 52.2% of the patients, and they were classified as transient neutropenia, 21.7% of the cases developed cyclic neutropenia, 13% of were categorized as benign chronic neutropenia, 7.2% developed severe chronic symptomatic neutropenia, 2.9% had neutropenia associated with type 1B glycogenosis and 2.9% of the cases were not classifiable in any of the proposed categories. More than 50% of the cases were associated with an abnormal pattern of infection in terms of frequency, severity, multiplicity of systems involved, or the presence of opportunistic microorganisms. The upper respiratory tract was the most commonly affected system with infection. Prophylactic antibiotics were used in 39.1% of the cases and granulocyte colony stimulating factor was required in 11.6% at some stage of the course. The most frequently involved pathogens in infection were Pseudomonas aeruginosa, Staphylococcus sp and E. coli. Conclusion: The vast majority of neutropenic patients had a benign clinical course. The same germs described in other series, caused infections in our patients. The absolute neutrophil count at diagnosis has no effect on the incidence of recurrent infection, but the type or clinical course of neutropenia does.

 neutropenia factor estimulante de colonias de granulocitos infección recurrente inmunodeficiencia población pediátrica neutropenia granulocyte colony stimulation factor recurrent infection immunodeficiency children Lakshman R Finn A Neutrophil disorders and their management J Clin Pathol 2001 54 Bernini JC Diagnosis and management of chronic neutropenia during childhood Pediatr Clin North Am 1996 43 Berliner N Horwitz M Loughran T Congenital and adquired neutropenia Hematology 2004 Boxer LA Blackwood RA Leukocyte disorders: Quantitative and qualitative disorders of the neutrophil, Part 2 Pediatr Rev 1996 17 James RM Kinsey SE The investigation and management of chronic neutropenia in children Arch Dis Child 2006 91 Capsoni F Sarzi-Puttini P Zanella A Primary and secondary autoimmune neutropenia Arthritis Res Ther 2005 7 Horwitz M Duan Z Korkmaz B Lee HH Meallife ME Salipante SJ Neutrophil elastase in cyclic and severe congenital neutropenia Blood 2007 109 Slatter MA Gennery AR Clinical Immunology Review Series: An approach to the patient with recurrent infections in childhood Clin Exp Immunol 2008 152 Rosensweig S Holland S Phagocyte immunodeficiencies and their infections J Allergy Clin Immunol 2004 113 Roskos RR Boxer LA Clinical disorders of neutropenia Pediatr Rev 1991 12 Dale D Person RE Bolyard AA Aprikyan AG Bos C Bonilla MA Mutations in the gene encoding neurophil elastase in congenital and cyclic neutropenia Blood 2000 93 Elastase 2 Elastase 2 Ancliff PJ Congenital neutropenia Blood Rev 2003 17 Klein C Grudzien M Appaswamy G Germeshausen M Sandrock I Schäffer AA HAX1 deficiency causes autosomal recessive severe congenital neutropenia (Kostmann disease) Nat Genet 2007 39 Boxer LA Neutrophil abnormalities Pediatr Rev 2008 24 Zaromb A Chamberlain D Schoor R Almas K Blei F Periodontitis as a manifestation of chronic benign neutropenia J Periodontol 2006 77 Kuritzkes DR Neutropenia, neutrophil dysfunction, and bacterial infection in patients with human immunodeficiency virus disease: the role of granulocyte colony-stimulating factor Clin Infect Dis 2000 30 Newburger PE Disorders of neutrophil number and function Hematology 2006 7 Segel GB Halterman JS Neutropenia in pediatric practice Pediatr Rev 2008 29 Boxer LA Blackwood RA Leukocyte disorders: Quantitative and qualitative disorders of the neutrophil, Part 1 Pediatr Rev 1996 17 Lehrnbecher T Welte K Haematopoietic growth factors in children with neutropenia Br J Haematol 2002 116 Lima C Paula E Takahashi T Saad ST Lorand-Metze S Costa FFl Causes of incidental neutropenia in adulthood Ann Hematol 2006 85 Palmblad J Papadaki HA Chronic idiopathic neutropenias and severe congenital neutropenia Curr Opin Hematol 2008 15 Papadaki H Eliopoulos A Kosteas T Gemetzic C Damianaki A Kontala H Impaired granulocytopoiesis in patients with chronic idiopathic neutropenia is associated with increased apoptosis of bone marrow progenitor cells Blood 2003 101
S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200009 Análisis de 200 casos pediátricos de malformaciones anorectales Analysis of 200 cases of Pediatric Anorectal Malformation Ceciliano-Romero Norma Beauchamp-Carvajal Debora Chavez-Fernández Laura Cortés-Ojeda Jaime ,Hospital Nacional de Niños Dr. Carlos Sáenz Herrera 06 2010 2010 52 109 117

Objetivo:Las malformaciones anorectales son una patología frecuente en el Servicio de Cirugía General del Hospital Nacional de Niños, sin embargo, en Costa Rica las publicaciones sobre el tema han sido pocas. El objetivo de este trabajo es analizar los pacientes operados entre 1998 y 2008, para conocer el detalle de los tratamientos usados y determinar cuál ha sido la experiencia, algo muy recomendable para que, con base en los hallazgos, se procesen las sugerencias para mejorar. Material y métodos: Se revisaron los expedientes y se examinaron los pacientes con malformaciones anorectales operados entre 1990 y 2008, controlados en la sección especializada de la Consulta Externa del Servicio de Cirugía General del Hospital Nacional de Niños. Los pacientes fueron agrupados de acuerdo con su tipo de anomalía, y fueron examinados para determinar: malformaciones congénitas asociadas, tipo de malformación anorectal, tipo de cirugía, complicaciones, y resultados de los siguientes exámenes: Ultrasonido de abdomen, CUMS, colografía distal y sus resultados funcionales, después de los tres años de edad. Los datos de los pacientes se digitaron en una base de datos del programa Excel, para analizarlos y tener una clara visión de los procedimientos empleados con cada grupo y de los resultados obtenidos. Resultados: Se controlaron 200 casos operados entre 1998 y 2008; en todos se estudiaron las malformaciones congénitas asociadas y su malformación anal, y se encontraron distintos tipos que fueron clasificados de la siguiente forma: los que tenían fístula, que se agruparon por el sitio donde la fístula desembocaba: a cuello vesical (12 casos), uretra prostática (12 casos), uretra bulbar (18 casos), vestibular (26 casos), perineal (92 casos), estenosis anal (12 casos); los que no tenían fístula: altos (16 casos) o bajos (3 casos), y finalmente, las malformaciones cloacales, tanto las altas (2 casos) como las bajas (7 casos). Todos los pacientes fueron operados con la técnica de Peña11 con las modificaciones para cada tipo. No se intervino ningún paciente con técnica laparoscópica. Conclusión: Se concluye que estos pacientes son muy complejos y deben estudiarse como un todo, ya que las malformaciones asociadas tienen mucha importancia en los resultados y la morbimortalidad. En la mayoría de los casos se obtienen buenos resultados. Lo importante en ellos es controlarlos por un tiempo prolongado, y usar los procedimientos necesarios para que se les permita llevar una vida normal, lo cual es posible.

Aim: Despite the high frequency of anorectal malformations treated at the General Surgery Service of the National Children’s Hospital, there exists a dearth of national medical publications related to this pathology. The aim of this investigation is to verify the treatment details of 200 patients who underwent anorectal surgery between 1998 and 2008 as well as to determine our experience. Any recommendations will be based on the findings of this study. Materials and methods: Two hundred patients of the Dr. Carlos Sáenz National Children’s Hospital were studied. This group presented anorectal malformations, were treated between 1998 and 2008 and received follow up care at the General Surgery outpatient clinic.The patients were grouped according to their type of anomaly and were examined for the following: associated congenital malformations, type of anorectal anomaly, type of surgery, complications and the results of the following tests: abdominal ultrasound, CUMS, distal colography and the functional results after three years of age. All of the data was collected from the patients’ medical files. This information was then transferred to an Excel data sheet for further analysis and in order to have a clear picture of the conduct that was followed with each group of patients. Results:The groups were made using the following classification: those patients with a fistula were grouped according to the location of the opening: bladder neck (12 cases), prostate (12 cases), bulbar (18 cases), vestibular (26 cases), perineal (92 cases), anal stenosis (12 cases). Those who had no fistula: high (16 cases) or low (3 cases). Finally, the cloacae malformations, both high cases (2 cases) and low cases (7cases). All of the patients underwent surgery using the Peña technique with the corresponding modifications. No patients were treated with the laparoscopic technique. Conclusion: Because we consider these cases to be highly complex, they must be studied holistically due to the fact that associated malformations bear much weight in the final results and in the morbimortality rate. In the majority of cases, satisfactory results are obtained. Long term follow up care that includes all necessary procedures, is essential in order for the patient to lead a normal life.

 malformación anorectal cloaca colostomía fístula laparoscopía colografía anorectal malformation cloaca colostomy fistula laparoscopy colography DeVries PA Pena A Posterior sagittal anorectoplasty J Pediatr Surg 1982 17 Peña A Anorectal malformations Semin Pediatr Surg 1995 4910 Beals RK Robbins JR Rolfe B Anomalies associated with vertebral malformations Spine 1993 18 Keith E Georgeson Inge Thomas H Albanese Craig T Laparoscopically assisted anorectal pull-through for high imperforate anus-A new technique J Pediatric Surg 2000 35 6 Gross GW Wolfson PJ Peña A Augmented-pressure colostogram in imperforate anus with fistula Pediatr Radiol 1991 21 Pena A Management of anorectal malformations during the newborn period World J Surg 1993 17 Hendren WH Cloaca, the most severe degree of imperforate anus: experience with 195 cases Ann Surg 1998 228 Hendren WH Cloacal malformations: experience with 105 cases J Pediatr Surg 1992 27 Hendren WH Pediatric rectal and perineal problems Pediatr Clin North Am 1998 45 Levitt MA Stein DM Pena A Gynecologic concerns in the treatment of teenagers with cloaca J Pediatr Surg 1998 33 Levitt MA Pena A Pitfalls in the management of newborn cloacas Pediatr Surg Int 2005 21 Levitt MA Stein DM Pena A Gynecologic concerns in the treatment of teenagers with cloaca J Pediatr Surg 1998 33 Rich MA Brock WA Pena A Spectrum of genitourinary malformations in patients with imperforate anus Pediatric Surg Intl 1988 3 Tovilla-Mercado J M Peña A Pediatr Mex 2008 29 Pena A Migotto-Krieger M Levitt MA Colostomy in anorectal malformations: a procedure with serious but preventable complications J Pediatr Surg 2006 41
S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200010 Cistitis enfisematosa Emphysematous Cistitis Orlich-Castelán Claudio Loyola-Castro Eduardo ,Hospital CIMA Servicio de Urología ,Hospital CIMA Servicio de Emergencias 06 2010 2010 52 118 120

Se reporta el caso de un marinero con diabetes mellitus descompensada e insuficiencia renal con una cistitis enfisematosa, evidente en un ultrasonido de la vejiga que se presentó con hematuria, disuria, dolor suprapúbico y síntomas urinarios obstructivos, tratada inicialmente en altamar por 48 horas, con antibióticos. Se mantuvo en excelente estado general y afebril, sin poder aislar una bacteria causal, por haberse iniciado antibioticoterapia temprana; fue tratado además con cirugía conservadora, con debridamiento y lavado de tejidos afectados con agua oxigenada y solución yodada, y con derivación de la orina con una cistostomía suprapúbica, para una excelente evolución y resultado final.

We herein report an uncommon case of a diabetic sailor with renal failure and emphysematous cystitis, who presented with hematuria, dysuria, suprapubic pain and obstructive urinary symptoms, initially treated at sea with antibiotics, with an ultrasound showing the presence of gas. This man remained in good general condition with no fever, but it was not possible to isolate bacteria in the cultures because of early use of antibiotics. He was also treated conservatively with surgical debridement of superficial tissues and bladder mucosa, washings with peroxide and iodine solutions and with a suprapubic tube for urinary diversion. The outcome was excellent with bladder preservation and full recovery of his health.

 vejiga cistitis gas diabetes mellitus bladder cystitis gas diabetes mellitus Gillenwater J Grayhack Emphysematous cistitis Emphysematous cistitis 2002 Fourth Lippincott Williams and Wilkins Bobba R Arsura E Sama P Sawh A Emphysematous cystitis: an unusal disease of the Genito-Urinary system suspected on imaging Ann Clin Microbiol Antimicrob 2004 3 O'Connor L Guzman J Emphysematous cystitis: a radiographic diagnosis Am J Emerg Med 2001 19 Leclercq P What's your call CMAJ 2008 178 Bor Fuh Shen Images in Emergency Medicine Ann Emerg Med 2008 51 Tsao J Diagnostic dilema: Emphysematous cystitis Am J Med 2001 110
S0001-600220100002 Acta Médica Costarricense Acta méd. costarric 0001-6002 Colegio de Médicos y Cirujanos de Costa Rica S0001-60022010000200011 Investigación Costarricense en Revistas Extranjeras , 06 2010 2010 52 121 121