[Hemophagocytic syndrome, current diagnostic and therapeutic approach]. / Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr.

Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a highly lethal hyper-inflammatory disorder that leads to a storm of cytokines, hemophagocytosis and multiple organ failure. It can be primary, which is inherited, or secondary. In the latter, virus infections are a frequent trigger, predominantly the family of herpes viruses, such as Epstein-Barr virus. An early treatment is recommended. Until recently there was no consensus about the management of secondary cases. The protocols for the treatment of primary HLH were used, which include cytotoxic agents and corticosteroids. We herein review the current diagnostic and therapeutic approach of HLH, based on a case associated with a reactivation of the Epstein-Barr virus in an immunocompetent adolescent. We highlight the importance of suspecting this disease in patients with a persistent inflammatory response state or with a fever of unknown origin, in order to carry out a timely treatment, with the least toxicity, and appropriate to the characteristics of each individual, which is the current therapeutic trend.

[Eosinophilic cystitis: review and two case reports]. / Cistitis eosinofílica: revisión y reporte de dos casos.

Eosinophilic cystitis is a low frequency disease, with less than 200 reported cases in the world. It is characterized by a bladder wall inflammation, mainly by eosinophils, with fibrosis and muscle necrosis areas. Its origin seems to be immunological, although the triggers are not well known. Several predispose factor have been described such as allergic diseases, bladder injuries, drugs, infections, etc. It affects patient of all ages, mainly adults. It presents with frecuency, haematuria and suprapubic pain. Other less frequent symptoms are disuria, urinary retention, nicturia, and enuresis. The laboratory study (urinalysis, urinalysis and haemogram) and radiology (ultrasound, intravenous pyelography, computed tomography and nuclear magnetic resonance) are non specific. The lesions observed in the cystoscopy could emulate other diseases, that why the proper diagnostic is the histological analysis. The management could be observation o antihistaminic, anti-inflammatory and corticoid treatment. In refractory cases, surgery is an alternative. In this work, two male adult cases are reported with their symptoms, studies and management.

[Intrauterine device migration to the bladder: four case reports]. / Migración de dispositivo intrauterino a vejiga: aportación de cuatro casos.

The intrauterine device has been commonly used in our population for many years as contraceptive method. One of the complications is the device migration out of the uterus. One of the most uncommon fait of this migration is the bladder, with few reports on the literature. In this work, four cases are reported with their clinical presentation, study and treatment.

Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr / Hemophagocytic syndrome, current diagnostic and therapeutic approach

Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a highly lethal hyper-inflammatory disorder that leads to a storm of cytokines, hemophagocytosis and multiple organ failure. It can be primary, which is inherited, or secondary. In the latter, virus infections are a frequent trigger, predominantly the family of herpes viruses, such as Epstein-Barr virus. An early treatment is recommended. Until recently there was no consensus about the management of secondary cases. The protocols for the treatment of primary HLH were used, which include cytotoxic agents and corticosteroids. We herein review the current diagnostic and therapeutic approach of HLH, based on a case associated with a reactivation of the Epstein-Barr virus in an immunocompetent adolescent. We highlight the importance of suspecting this disease in patients with a persistent inflammatory response state or with a fever of unknown origin, in order to carry out a timely treatment, with the least toxicity, and appropriate to the characteristics of each individual, which is the current therapeutic trend.

Allozyme variation in populations of Pleurodema thaul (Lesson, 1826) (Anura; Leptodactylidae).

Genetic variation of 18 loci detected in 13 Chilean and one Argentinian population of the toad Pleurodema thaul are described. Seven loci were polymorphic in at least one of the studied populations. The average heterozygosity values observed for locus went from H = 0.00 for the Las Cardas population to 0.114 in the Lautaro locality, with a global average of 5.2%. The genetic similarity values gave an average of 0.875 (range 0.726-1.00). Accordingly, similarity values were possible to distinguish three groups: north, central and Osorno. The Osorno population differed from the central group in Est-1 and Est-2, yet the Osorno population is only 20 km from a population of the central group.

Complicaciones en cirugía laparoscópica: como lo hago yo, para que usted no lo haga y le vaya bien / Complications in laparoscopic surgery: how I do it, so you won´t and do well

INTRODUCCIÓN: La cirugía laparoscópica urológica no esta exenta de complicaciones. Estas van a depender de la selección del paciente, así como de la experiencia del cirujano. MATERIAL Y MÉTODOS: Se presentan cuatro casos en los que ocurrieron complicaciones durante una cirugía laparoscópica renal. Se discute la complicación y se analizan los factores que la precipitaron, el manejo de esta y sugerencias para evitarla. RESULTADOS: Se presentan dos casos de lesión de pedículo renal durante nefrectomía parcial laparoscópica, 1 lesión de vena renal y una lesión de arteria renal. En ambos fue posible la resolución mediante sutura intracorpórea. Se presenta dos casos de lesión de pedículo durante nefrectomía radical laparoscópica. 1 lesión de arteria renal y una lesión de venal renal. En ambos casos fue necesaria la conversión a cirugía abierta para controlar la complicación. Se presenta un caso de lesión esplénica durante una nefrectomía parcial laparoscópica. La laceración fue controlada mediante compresión y electro coagulación. CONCLUSIONES: Las presencia de complicaciones durante la cirugía laparoscópica urológica es inherente al procedimiento. El uso de equipo e instrumental adecuados son fundamentales para minimizar el riesgo, así como también la selección del paciente es un factor importante. El dominio de la sutura intracorpórea es de suma importancia para solucionar la complicación una vez que esta ya está establecida. linfadenectomía aorto-ilíaca. Se realizó un abordaje transperitoneal con el sistema da Vinci S-HD® y técnica de 6 trócares. Nuestra plantilla de disección ganglionar tiene como límites el pubis hacia distal, la arteria mesentérica inferior hacia proximal, el nervio genito-femoral hacia lateral y la vejiga hacia medial. (AU)

Introduction: Laparoscopic urological surgery is not without complications. These will depend on the patient's selection as well as the surgeon's experience. Material and Methods: Four cases in which complications occurred during laparoscopic renal surgery are presented . The complication is discussed and the causes that produced it are analyzed. Results: Two cases of renal pedicle injury are presented during laparoscopic partial nephrectomy, 1 renal vein and 1 renal artery injuries. In both cases resolution was possible by intracorporeal suture. Two cases of pedicle injury are presented during laparoscopic radical nephrectomy. 1 renal artery injury and renal venal injury. In both cases, conversion to open surgery was necessary to control the complication. A case of splenic injury is presented during a laparoscopic partial nephrectomy. The laceration was controlled by compression and electrocoagulation. Conclusions: The presence of complications during laparoscopic urological surgery is inherent in the procedure. The use of appropriate equipment and instruments are essential to minimize risk, as well as patient selection is an important factor. The domain of intracorporeal suture is of paramount importance to solve the complication once it is already established. (AU)

[Treatment of massive spasms with synthetic ACTH]. / Tratamiento de espasmos masivos con ACTH sintético.

Ten infants with infantile spasms (IS) and hypoarrhythmic EEG, received 0.5 mg i.m. of synthetic ACTH, three times a week for 2 weeks. Six of them showed complete remission of seizures and dramatic EEG changes, and 3 cases resulted in a partial response. Spasms were controlled during the first week in 5/6 cases where treatment was successful. A significant relationship was observed between treatment precocity and favourable outcome (Fisher p < 0.02). Synthetic ACTH treatment did not produce severe side effects in any patient. During follow up (X 6.2 m) relapse occurred in one case, with 5 patients maintaining the initial positive response and a normal or mildly retarded psychomotor development. The other five patients show severe psychomotor retardation and intractable spasms and/or other seizures. Future research should focus on the efficacy of other treatment schedules, equilibrating success and side effects.

[Etiologic research in massive spasms]. / Pesquisa etiológica en espasmos masivos.

A systematic clinical protocol was applied in 16 infants that suffered from infantile spasms (IS) in order to identify etiologic factors. A positive family history was present in 2/16 patients and relevant perinatal or postnatal pathology in 5/16. Psychomotor retardation and other seizures anteceded IS in 10/16 and 8/16 infants respectively. Physical and neurologic examination revealed microcephalia (4/16), dysmorphic features (2/16), hypopigmented skin lesions (1/16) and pyramidal syndrome (8/16). Neuroimaging technics yielded positive findings in 9/16 patients, diffuse or localized atrophy (7/16), porencephalic cysts (3/16), periventricular calcifications (1/16), callosal agenesis (1/16). Laboratory examination allowed diagnosis of two metabolic diseases: congenital hyperlactatemia an maple syrup urine disease. Two patients were classified as cryptogenetic and fourteen as symptomatic. Within the latter an etiologic factor was identified in 12/14. This study underlines the value of etiologic search in IS, because it may contribute substantially to specific treatment and genetic counselling.

Nefrectomias parciales y tumorectomias: experiencia durante los ultimos 10 años en servicio de urología Hospital Clínico de Valdivia / Partial nephrectomy and Tumorectomy specimens: experience duringthe last 10 years in the Urology department of the Hospital the Valdivia

INTRODUCCIÓN Y OBJETIVO: El mayor uso de imágenes diagnósticas, ha implicado un aumento en el diagnóstico de lesionestumorales renales de menor tamaño, llevando al desarrollo de técnicas quirúrgicas conservadoras de parenquimarenal. Series internacionales han demostrado que los resultados oncológicos de este tipo de cirugía son similares a la nefrectomía radical. Nuestro objetivo es mostrar la experiencia y resultados oncológicos de la cirugía conservadora deparenquima renal. MATERIALES Y MÉTODOS: Estudio retrospectivo, de las nefrectomías parciales y tumorectomías realizadas entre Enero del año 2002 y Junio del año 2013, en el Hospital Clínico Regional de Valdivia. RESULTADOS: 50 pacientes fueron sometidos a cirugía renal conservadora, con 51 cirugías realizadas. 26 corresponden a sexo masculino y 24 a sexo femenino; promedio de edad de 58,2 años. De las cirugías realizadas veinte correspondieron a nefrectomías parciales, contamaño tumoral promedio de 4,3 cms. (2-7 cms.) y 31 a tumorectomías, con tamaño tumoral promedio de 2,5 cms. (1-6,1cms.). Del total de los tumores resecados, ocho (26%) fueron informados como tumores benignos y 43 (84%) corresponden a adenocarcinomas; 41 (95%) corresponden a la variante células claras y solo dos a variante de células cromofobas. Lasbiopsias rápidas están todas entre 1-2 mm de tejido sano como margen, y todas fueron negativas para tumor. La biopsiadiferida, informo cuatro márgenes positivos para tumor. De estas, tres fueron a re-cirugía cuyas biopsias fueron negativaspara tumor. El tiempo promedio de seguimiento fue de 51,1 meses, con una sobrevida actuarial a los diez años cercana al70% y una sobrevida específica a los diez años cercana al 95%...

INTRODUCTION AND OBJECTIVE: The increased use of diagnostic imaging, has involved an increase in the diagnosis ofsmaller tumor lesions, taking the development of conservative surgical techniques of renal parenchyma. Internationalseries have shown that cancer results from this type of surgery are similar to radical nephrectomy. Our goal is to show the experience and oncological results of renal parenchymal sparing surgery. MATERIALS AND METHODS: Retrospective studyof tumorectomies and partial nephrectomies performed between January 2002 and June 2013, at the Regional Hospital ofValdivia. RESULTS: 50 patients underwent renal sparing surgery, with 51 surgeries performed. 26 are male and 24 female,average age of 58.2 years old. Of the surgeries performed, twenty were partial nephrectomy, with average tumor size of4.3 cm. (2-7 cms.) and 31 tumorectomies, with average tumor size of 2.5 cm. (1-6.1 cms.). Of all resected tumors, eight (26%)were reported as benign and 43 (84%) were adenocarcinomas, 41 (95%) were clear cell variant and only two chromophobecell variant. Quick biopsies are all 1-2 mm of healthy tissue margin, and all were negative for tumor. The deferred biopsy,reported four positive tumor margins. Of these, three went to re-surgery, whose biopsies were negative for tumor. The average follow-up time was 51.1 months, with an actuarial survival at ten years about 70% and a specific survival at ten years was approximately 95%...

Cistitis eosinofílica: Revisión y reporte de dos casos / Eosinophilic cystitis: Review and two cases report

La cistitis eosinofílica es una enfermedad de baja frecuencia, con menos de 200 casos reportados en el mundo. Se caracteriza por la inflamación de toda la pared vesical, predominantemente por eosinófilos, con fibrosis y áreas de necrosis muscular. Su origen parece ser inmunológico aunque los desencadenantes no se conocen bien. Se han descrito varios factores predisponentes como enfermedades alérgicas, trauma vesical, fármacos, infecciones, etc. Afecta a pacientes de todas las edades, preferentemente adultos. Se presenta con polaquiuria, hematuria y dolor suprapúbico. Otros de menor frecuencia son disuria, retención urinaria, nicturia y enuresis. El estudio de laboratorio (sedimento de orina, urocultivo, hemograma)y radiológico (ecotomografía, pielografía de eliminación, tomografía computada y resonancia nuclear magnética) son inespecíficos. En la cistoscopía se observan lesiones que pueden simular otras enfermedades, por lo que el diagnóstico definitivo es histológico. El manejo puede ser expectante o con antihistamínicos, antiinflamatorios y corticoides. En los casos refractarios al tratamiento médico la cirugía puede ser una alternativa. Reportamos dos casos de pacientes hombres, adultos, con su cuadro de presentación, estudio y manejo (AU)

Eosinophilic cystitis is a low frequency disease, with less than 200 reported cases in the world. It is characterized by a bladder wall inflammation, mainly by eosinophils, with fibrosis and muscle necrosis areas. Its origin seems to be immunological, although the triggers are not well known. Several predispose factor have been described such as allergic diseases, bladder injuries, drugs, infections, etc. It affects patient of all ages, mainly adults. It presents with frecuency, haematuria and suprapubic pain. Other less frequent symptoms are disuria, urinary retention, nicturia, and enuresis. The laboratory study(urinalysis, urinalysis and haemogram) and radiology (ultrasound, intravenous pyelography, computed tomography and nuclear magnetic resonance) are non specific. The lesions observed in the cystoscopy could emulate other diseases, that why the proper diagnostic is the histological analysis. The management could be observation o antihistaminic, anti-inflammatory and corticoid treatment. In refractory cases, surgery is an alternative. In this work, two male adult cases are reported with their symptoms, studies and management (AU)

Migración de dispositivo intrauterino a vejiga: aportación de cuatro casos / Intrauterine device migration to the bladder: four cases report

El dispositivo intrauterino (DIU) ha sido utilizado en nuestro medio durante muchos años como método anticonceptivo. Una de las complicaciones es la migración fuera del útero, siendo la localización vesical inusual, con escasos casos en la literatura. Presentamos cuatro casos de migración del DIU a la vejiga. Se describe cuadro clínico, estudio y tratamiento (AU)

The intrauterine device has been commonly used in our population for many years as contraceptive method. One of the complications is the device migration out of the uterus. One of the most uncommon fait of this migrationis the bladder, with few reports on the literature. In this work, four cases are reported with their clinical presentation, study and treatment (AU)

Consenso chileno de manejo de fármacos antiepilépticos en algunos síndromes electro-clínicos y otras epilepsias en niños y adolescentes / Chilean consensus on the use of antiepileptic drugs in electro-clinical syndromes

Por iniciativa de tres instituciones: Liga Chilena contra la Epilepsia (LICHE), Sociedad de Epileptología de Chile (SOCEPCHI) y Sociedad de Psiquiatría y Neurología de la Infancia y Adolescencia (SOPNIA) de Chile, se constituye un comité de trabajo que convoca a un consenso de uso de fármacos antiepilépticos (FAEs) en un grupo de 16 Síndromes electro-clínicos y otras Epilepsias en niños y adolescentes. Cuarenta y dos médicos neuropediatras especialistas en Epilepsias de todas las regiones de Chile, participaron en la discusión y realizaron una propuesta de tratamiento farmacológico para cada cuadro. El comité de trabajo realizó un análisis exhaustivo y discusión de los documentos, para finalmente concluir en una recomendación de tratamiento para cada cuadro. Este consenso es una guía práctica de orientación para ayudar a las decisiones de tratamiento en situaciones clínicas concretas. Su objetivo final es ofrecer una mejor calidad de atención a los niños y adolescentes con epilepsias, a través de decisiones fundadas que contribuyan a disminuir la variabilidad de las decisiones terapéuticas.

Committed by three institutions: Liga Chilena contra la Epilepsia (LICHE), Sociedad de Epileptología de Chile (SOCEPCHI) y Sociedad de Psiquiatría y Neurología de la Infancia y Adolescencia (SOPNIA) de Chile, a 6-member working committee called for a meeting of 42 Chilean pediatric epileptologists from all over the country, with the aim of reaching a consensus on the use of antiepileptic drugs in 16 selected children and adolescents electro-clinical syndromes and epilepsies. These treatment proposals were analyzed and fully discussed by the working committee, ending in an antiepileptic drug treatment recommendation guideline for each condition. This consensus is a practical guideline to be used in specific clinical situations, which aims to support treatment decision making. Its main purpose is to offer the best evidence based treatments to our children and adolescents patients with epilepsy, thus contributing to diminish variability in therapeutic decisions.

Macrocefalias no quirurgicas. / Non-surgical macrocephaly

Se apresenta un grupo de 15 pacientes con macrocefalia de evolucion no hipertensiva pero de diferentes etiologias, evolucion y pronostico. Se analizan las macrocefalias de origen primario y las asociadas a sindromes conocidos y enfermedades degenerativas del sistema nervioso central. Todas tienen un rasgo genetico hereditario. Se hace enfasis en el grupo de megaencefalias benignas. Las tecnicas de laboratorio para diagnosticarlas y hacer seguimiento de la macrocefalia especialmente utilizando la ultrasonografia como metodo no invasivo y de alta eficiencia

Caso clínico: adenocarcinoma mesonefroide de la uretra femenina / Clinic case: mesonephroide adenocarcinoma of the female urethra

Se presenta un caso de adenocarcinoma mesonefroide de la uretra en un paciente de 63 años, tratada con exanteración pelviana anterior logrando una sobrevida de tres años. Se destaca la rareza de este tumor y se revisa la literatura