Uso del soporte respiratorio no invasivo en pacientes con distrés moderado-severo por SARS-CoV-2. Experiencia en un hospital de segundo nivel / Use of non-invasive respiratory support in patients with moderate-severe distress due to sars-cov-2. Experience in a second level hospital

Objetivo: Describir las características de los pacientes ingresados en una unidad de cuidados respiratorios intermedios (UCRI) creada a raíz de la pandemia, analizar los factores asociados a la supervivencia y techo terapéutico. Material y método: Estudio descriptivo observacional, incluye todos los casos con neumonía por SARS-CoV-2 que requirieron ingreso en UCRI. Variables analizadas: clínicas, analíticas, terapéuticas, supervivencia, soporte respiratorio y evolución oximétrica. Se realizó un análisis univariante para las diferencias según techo terapéutico y supervivencia a 90 días, para la evolución de la SpO2/FiO2 en el tiempo se ajustaron modelos lineales mixtos. Resultados: Participaron 37 pacientes. El 46% no eran candidatos a cuidados críticos. Características asociadas a orden de no intubación de manera significativa: edad más avanzada (p=0,001), mayor índice de Charlson (p=0,003), EPOC (p=0,014) o cáncer (p=0,033). Supervivencia global en UCRI del 58%, según techo terapéutico en no intubables la supervivencia fue del 41% mientras en intubables asciende al 89%. Las variables asociadas a mortalidad fueron mayor edad (p=0,012), mayor índice de Charlson (p=0,030), mayor valor de proteína C reativa (p=0,045), menor nº de linfocitos (p=0,019) y tratamiento con lopinovir/ritonavir (p=0,006). La SpO2/FiO2 media al inicio del soporte respiratorio no invasivo (SRNI) fue 112 (DE:23), el 81% padecían distrés grado moderado-severo. A menor SpO2/FiO2 inicial peor pronóstico (p<0,001), el uso de SRNI mejora de manera progresiva la SpO2/FiO2 a mayor número de horas de uso (p=0,006). Conclusiones: El SRNI en UCRI es seguro, mejora la oxigenación y ofrece opciones terapéuticas en pacientes no intubables.(AU)

Objective: To describe the characteristics of the patients admitted to an intermediate respiratory care unit (UCRI) created as a result of the pandemic, to analyze the factors associated with survival and therapeutic ceiling. Material and metho: Descriptive observational study, includes all cases with SARS-CoV-2 pneumonia that required admission to UCRI. Variables analyzed: clinical, analytical, therapeutic, survival, respiratory support and oximetric evolution. A univariate analysis was performed for the differences according to therapeutic ceiling and survival at 90 days, for the evolution of SpO2/FiO2 over time, mixed linear models were adjusted. Results: 37 patients participated. The 46% of them were not candidates for critical care. Characteristics significantly associated with the order of non-intubation: older age (p = 0.001), higher Charlson index (p = 0.003), COPD (p = 0.014) or cancer (p = 0.033). Overall survival in UCRI 58%, according to the therapeutic ceiling in non-intubable patients, survival was 41%, while in intubable it was 89%. The variables associated with mortality were older age (p = 0.012), higher charlson index (p = 0.030), higher value of reactive protein C (p = 0.045), lower number of lymphocytes (p = 0.019) and treatment with lopinovir/ritonavir (p = 0.006). The mean SpO2/FiO2 at the beginning of non-invasive respiratory support (SRNI) was 112 (SD: 23), 81% suffered from moderate-severe distress. The lower the initial SpO2/FiO2, the worse the prognosis (p <0.001), the use of NIRS progressively improves the SpO2/FiO2 with the greater number of hours of use (p = 0.006). Conclusions: SRNI in UCRI is safe, improves oxygenation and offers therapeutic options in non-intubable patients.(AU)

Neonatal appendicitis: how many sides does this coin have? / Apendicitis neonatal, ¿cuántas caras tiene esta moneda?

Neonatal appendicitis is a very rare entity associated with immune, vascular, hypoxic, and obstructive factors. We present two cases of neonatal appendicitis with different characteristics. The first case is a 15-day-old newborn with congenital hypothyroidism who had abdominal distension and sepsis data. An appendicular plastron was found. Hirschsprung's disease was ruled out through biopsies. The second case is a 27-week-old preterm newborn with history of necrotizing enterocolitis who presented an incarcerated inguinal hernia consistent with Amyand's hernia. The prognosis of neonatal appendicitis varies according to how fast diagnosis is achieved, since it is associated with high mortality rates in the first hours. Although appendicitis is the condition best known to the pediatric surgeon, it represents a diagnostic challenge in newborns as a result of its extreme rarity and how likely it is to present comorbidities.

La apendicitis neonatal es una entidad infrecuente que asocia factores inmunes, vasculares, hipóxicos y obstructivos. Presentamos dos casos de apendicitis neonatal con diferentes características. El primer caso es un neonato de 15 días de vida con hipotiroidismo congénito. Presentaba distensión abdominal y datos de sepsis. Se halló un plastrón apendicular y se descartó enfermedad de Hirschsprung mediante biopsias. El segundo caso es un prematuro de 27 semanas, con antecedente de enterocolitis necrotizante, que a los 53 días de vida presentó una hernia inguinal incarcerada compatible con hernia de Amyand. El pronóstico de la apendicitis neonatal va unido a la celeridad del diagnóstico, ya que supone una importante mortalidad en las primeras horas. Aunque la apendicitis es el cuadro más conocido por el cirujano pediátrico, a edad neonatal es un reto diagnóstico por su extrema rareza y probable comorbilidad.

Apendicitis neonatal, ¿cuántas caras tiene esta moneda? / Neonatal appendicitis: how many sides does this coin have?

La apendicitis neonatal es una entidad infrecuente que asocia fac-tores inmunes, vasculares, hipóxicos y obstructivos. Presentamos doscasos de apendicitis neonatal con diferentes características.El primer caso es un neonato de 15 días de vida con hipotiroidismocongénito. Presentaba distensión abdominal y datos de sepsis. Se hallóun plastrón apendicular y se descartó enfermedad de Hirschsprung me-diante biopsias. El segundo caso es un prematuro de 27 semanas, conantecedente de enterocolitis necrotizante, que a los 53 días de vida pre-sentó una hernia inguinal incarcerada compatible con hernia de Amyand.El pronóstico de la apendicitis neonatal va unido a la celeridad deldiagnóstico, ya que supone una importante mortalidad en las primerashoras. Aunque la apendicitis es el cuadro más conocido por el cirujanopediátrico, a edad neonatal es un reto diagnóstico por su extrema rarezay probable comorbilidad.(AU)

Neonatal appendicitis is a very rare entity associated with immune,vascular, hypoxic, and obstructive factors. We present two cases of neo-natal appendicitis with different characteristics.The first case is a 15-day-old newborn with congenital hypothy-roidism who had abdominal distension and sepsis data. An appendicularplastron was found. Hirschsprung’s disease was ruled out through biop-sies. The second case is a 27-week-old preterm newborn with history ofnecrotizing enterocolitis who presented an incarcerated inguinal herniaconsistent with Amyand’s hernia.The prognosis of neonatal appendicitis varies according to how fastdiagnosis is achieved, since it is associated with high mortality rates inthe first hours. Although appendicitis is the condition best known to thepediatric surgeon, it represents a diagnostic challenge in newborns as aresult of its extreme rarity and how likely it is to present comorbidities.(AU)

Carotid glomus in childhood: presentation of a clinical case. / Glomus carotídeo en la infancia. Presentación de un caso clínico.

INTRODUCTION: Carotid glomus is an exceptional extra-adrenal paraganglioma in childhood originating at the carotid body. Only 3% of paragangliomas occur in the head and the neck. Familial forms, associated with Succinate Dehydrogenase (SDH) gene mutations, account for 10% of cases, the proportion being higher in childhood. They are benign in 95% of patients, but they can extend to both carotids. Treatment is surgical with or without previous embolization. Metastasis is rare and associated with malignant cases, which are limited. CLINICAL CASE: 8-year-old patient with a cervical mass originating 4 months ago and normal serum levels. Regarding family history, she had an aunt who underwent cervical surgery. Ultrasound examination demonstrated a greatly vascularized hypoechoic mass most likely related to carotid glomus. Full surgical resection without embolization was decided upon, which proved uneventful. The genetic study was positive for SDH gene mutation. CONCLUSION: Carotid glomus in childhood should be considered as a differential diagnosis in cervical masses. Surgical treatment without previous embolization represents a safe therapeutic option in selected cases.

INTRODUCCION: El glomus carotídeo es un paraganglioma extraadrenal, excepcional en la infancia, cuyo origen es el cuerpo carotídeo. Solo el 3% de los paragangliomas se presentan en cabeza y cuello. Existe un 10% de formas familiares asociadas a mutaciones en el gen de la succinato deshidrogenasa (SDH), porcentaje que es mayor en la infancia. Son tumores benignos en un 95% de los pacientes, pero pueden afectar por extensión a ambas carótidas. Su tratamiento es quirúrgico con o sin embolización previa. Las metástasis son raras y están asociadas a los escasos casos de malignidad. CASO CLINICO: Paciente de 8 años de edad con masa cervical de 4 meses de evolución, serologías normales. Como antecedente destaca una tía sometida a cirugía cervical. Ecográficamente se objetiva masa hipoecoica muy vascularizada en probable relación con glomus carotídeo. Se decide resección quirúrgica sin embolización que resulta completa y sin incidencias. Estudio genético positivo para la mutación en el gen de la SDH. CONCLUSION: El glomus carotídeo en la infancia ha de ser tenido en cuenta como diagnóstico diferencial en masas cervicales. El tratamiento quirúrgico sin embolización previa es una opción terapéutica segura en casos seleccionados.

Glomus carotídeo en la infancia. Presentación de un caso clínico / Carotid glomus in childhood: presentation of a clinical case

Introducción: El glomus carotídeo es un paraganglioma extraadre-nal, excepcional en la infancia, cuyo origen es el cuerpo carotídeo. Soloel 3% de los paragangliomas se presentan en cabeza y cuello. Existeun 10% de formas familiares asociadas a mutaciones en el gen de lasuccinato deshidrogenasa (SDH), porcentaje que es mayor en la infancia.Son tumores benignos en un 95% de los pacientes, pero pueden afectarpor extensión a ambas carótidas. Su tratamiento es quirúrgico con o sinembolización previa. Las metástasis son raras y están asociadas a losescasos casos de malignidad. Caso clínico: Paciente de 8 años de edad con masa cervical de 4meses de evolución, serologías normales. Como antecedente destacauna tía sometida a cirugía cervical. Ecográficamente se objetiva masahipoecoica muy vascularizada en probable relación con glomus ca-rotídeo. Se decide resección quirúrgica sin embolización que resultacompleta y sin incidencias. Estudio genético positivo para la mutaciónen el gen de la SDH. Conclusión: El glomus carotídeo en la infancia ha de ser tenido encuenta como diagnóstico diferencial en masas cervicales. El tratamientoquirúrgico sin embolización previa es una opción terapéutica segura encasos seleccionados.(AU)

Introduction: Carotid glomus is an exceptional extra-adrenal para-ganglioma in childhood originating at the carotid body. Only 3% ofparagangliomas occur in the head and the neck. Familial forms, associ-ated with Succinate Dehydrogenase (SDH) gene mutations, account for10% of cases, the proportion being higher in childhood. They are benignin 95% of patients, but they can extend to both carotids. Treatment issurgical with or without previous embolization. Metastasis is rare andassociated with malignant cases, which are limited. Clinical case. 8-year-old patient with a cervical mass originating4 months ago and normal serum levels. Regarding family history, shehad an aunt who underwent cervical surgery. Ultrasound examinationdemonstrated a greatly vascularized hypoechoic mass most likely relatedto carotid glomus. Full surgical resection without embolization wasdecided upon, which proved uneventful. The genetic study was positivefor SDH gene mutation.Conclusion: Carotid glomus in childhood should be considered as adifferential diagnosis in cervical masses. Surgical treatment without pre-vious embolization represents a safe therapeutic option in selected cases.(AU)

Descenso vaginal vía perineal asistido por laparoscopia en paciente con atresia vaginal distal / Laparoscopically assisted pull-through surgery in a patient with distal vaginal atresia

La atresia vaginal distal es una anomalía infrecuente que deriva de la falta de desarrollo de los 2/3 distales de la vagina a partir del seno urogenital. Presentamos una paciente de 14 años con amenorrea primaria, abdominalgia, masa palpable en hipogastrio y ausencia de orificio vaginal. La ecografía abdominal y la RMN evidenciaron hematometrocolpos secundario a agenesia vaginal distal, con una distancia al periné de 5cm, sin otras malformaciones. Se realiza un drenaje vaginal transuretral y descenso vía perineal asistido por laparoscopia. Evolución favorable con calibre vaginal adecuado y normalización de la menstruación a los 6 meses

Distal vaginal atresia is an uncommon disorder that results from the lack of development of the distal 2/3 of the vagina from the urogenital sinus. The case is presented on a 14 year-old patient with primary amenorrhoea, abdominal pain, a palpable mass in the hypogastrium, and an absent vaginal opening. The abdominal ultrasound and magnetic resonance imaging showed haematometrocolpos secondary to distal vaginal agenesis, with a distance of 5cm to the perineum, with no other malformations. A transurethral vaginal drainage and laparoscopic assisted pull-through were performed. Her follow-up was favourable with an adequate vaginal calibre and normal menstruation after 6 months

Consideraciones técnicas en el tratamiento endoscópico del reflujo vesicoureteral tras reimplante tipo Cohen / Technical challenges of endoscopic treatment for vesicoureteral reflux after Cohen reimplantation

Introducción: El abanico de indicaciones del tratamiento endoscópico del reflujo vesicoureteral (RVU) se abre cada vez más hasta incluir la corrección del reflujo secundario tras cirugía de reimplante ureteral. No obstante, este escenario supone un reto técnico debido a los cambios posquirúrgicos. El objetivo de este trabajo es presentar nuestra experiencia en el tratamiento endoscópico del RVU en unidades ureterales con reimplante tipo Cohen, con especial interés en las particularidades técnicas del procedimiento. Material y métodos: Se ha realizado un estudio retrospectivo de casos de RVU secundario tras cirugía de reimplante tratados mediante inyección subureteral. Técnica: Se coloca la aguja perpendicular al trayecto submucoso y se inyecta medial al orificio, formándose un habón en la cara anterior que ocluya el meato. Resultados: En el periodo comprendido entre 1993 y 2016 se realizaron 21 inyecciones sobre 15 unidades ureterales. La afección ureteral incluía el RVU primario (4), sistema dúplex con RVU al pielón inferior (4), megauréter (3) y ureterocele (2). La edad media de los pacientes fue de 5,7 años (rango: 2-12 años). El éxito se logró en 10 unidades ureterales (66,67%), disminución del grado de RVU en 4 (26,67%) y persistencia/no resolución en un caso (6,67%). Discusión: El mecanismo antirreflujo del reimplante depende de optimizar el trayecto submucoso. Este subgrupo de pacientes es pequeño y los estudios son escasos lo que dificulta el acuerdo sobre la técnica más adecuada. Conclusión: El tratamiento endoscópico del reflujo secundario tras cirugía de reimplante transtrigonal es un procedimiento con cierta particularidad técnica, pero seguro y eficaz que ofrece una alternativa previa a la reintervención quirúrgica

Introduction: The range of indications for endoscopic treatment of vesicoureteral reflux opens more and more until including correction of secondary reflux (VUR) after ureteral reimplantation. However these cases suppose a technical challenge due to postoperative changes. The aim of this work is to present our experience on endoscopic treatment for VUR in ureteral units with Cohen reimplantation surgery, with special interest in the technical peculiarities of the procedure. Material and methods: A retrospective study of cases of secondary VUR after reimplantation surgery treated by subureteral injection. Technique: We put the needle perpendicular to submucous tunnel and inject medially to hole forming a wheal on the anterior face that occludes the meatus. Results: During the 1993-2016 period 21 injections were performed in 15 ureteral units. The ureteral pathology included primary VUR (4), duplex system with lower pole reflux (4), megaureter (3) and ureterocele (2). Average patient age was 5.7 years old (2-12). Succesful outcome had been got in 10 ureteral units (66.67%), a decrease of VUR grade in 4 (26.67%) and perseverance/no resolution of grade IV VUR in 1 (6.67%). Discussion: The anti-reflux mechanism of reimplantation depends on optimizing the submucosous tunnel. This subgroup of pacients is small and there are few studies, hindering the agreement on the most appropiate technique. Conclusion: Endoscopic treatment of secondary reflux after reimplantation surgery is a procedure with certain technical feature, but safe and effective offering an alternative prior to surgical reoperation

Technical challenges of endoscopic treatment for vesicoureteral reflux after Cohen reimplantation. / Consideraciones técnicas en el tratamiento endoscópico del reflujo vesicoureteral tras reimplante tipo Cohen.

INTRODUCTION: The range of indications for endoscopic treatment of vesicoureteral reflux opens more and more until including correction of secondary reflux (VUR) after ureteral reimplantation. However these cases suppose a technical challenge due to postoperative changes. The aim of this work is to present our experience on endoscopic treatment for VUR in ureteral units with Cohen reimplantation surgery, with special interest in the technical peculiarities of the procedure. MATERIAL AND METHODS: A retrospective study of cases of secondary VUR after reimplantation surgery treated by subureteral injection. TECHNIQUE: We put the needle perpendicular to submucous tunnel and inject medially to hole forming a wheal on the anterior face that occludes the meatus RESULTS: During the 1993-2016 period 21 injections were performed in 15 ureteral units. The ureteral pathology included primary VUR (4), duplex system with lower pole reflux (4), megaureter (3) and ureterocele (2). Average patient age was 5.7 years old (2-12). Succesful outcome had been got in 10 ureteral units (66.67%), a decrease of VUR grade in 4 (26.67%) and perseverance/no resolution of grade IV VUR in 1 (6.67%) DISCUSSION: The anti-reflux mechanism of reimplantation depends on optimizing the submucosous tunnel. This subgroup of pacients is small and there are few studies, hindering the agreement on the most appropiate technique. CONCLUSION: Endoscopic treatment of secondary reflux after reimplantation surgery is a procedure with certain technical feature, but safe and effective offering an alternative prior to surgical reoperation.

The cytokine profile of women with severe anxiety and depression during pregnancy.

BACKGROUND: Controversial findings regarding the association between pro-inflammatory cytokines and depression have been reported in pregnant subjects. Scarce data about anxiety and its relationships with cytokines are available in pregnant women. To understand the association between anxiety and cytokines during pregnancy, we conducted the present study in women with or without depression. METHODS: Women exhibiting severe depression (SD) and severe anxiety (SA) during the 3rd trimester of pregnancy (n = 139) and control subjects exhibiting neither depression nor anxiety (n = 40) were assessed through the Hamilton Depression Rating Scale (HDRS) and the Hamilton Anxiety Rating Scale (HARS). Serum cytokines were measured by a multiplex bead-based assay. Correlation tests were used to analyze the data and comparisons between groups were performed. A general linear model of analysis of variance was constructed using the group as a dependent variable, interleukin concentrations as independent variables, and HDRS/HARS scores and gestational weeks as covariables. RESULTS: The highest levels of Th1- (IL-6, TNF-α, IL-2, IFN-γ), Th17- (IL-17A, IL-22), and Th2- (IL-9, IL-10, and IL-13) related cytokines were observed in women with SD + SA. The SA group showed higher concentrations of Th1- (IL-6, TNF-α, IL-2, IFN-γ) and Th2- (IL-4, and IL-10) related cytokines than the controls. Positive correlations were found between HDRS and IL-2, IL-6, and TNF-α in the SA group (p < 0.03), and between HDRS and Th1- (IL-2, IL-6, TNF-α), Th2- (IL-9, IL-10, IL-13) and Th17- (IL-17A) cytokines (p < 0.05) in the SD + SA group. After controlling the correlation analysis by gestational weeks, the correlations that remained significant were: HDRS and IL-2, IL-6, IL-9, and IL-17A in the SD + SA group (p < 0.03). HARS scores correlated with IL-17A in the SA group and with IL-17A, IL-17F, and IL-2 in the SD + SA group (p < 0.02). The linear model of analysis of variance showed that HDRS and HARS scores influenced cytokine concentrations; only IL-6 and TNF-α could be explained by the group. CONCLUSIONS: We found that the cytokine profiles differ when comparing pregnant subjects exhibiting SA with comorbid SD against those showing only SA without depression.

Actualización en anomalías renales y de vías urinarias altas en pediatría / Update in renal and upper urinary tract anomalies in pediatrics

La urología pediátrica comprende una extensa serie de patologías de complejidad variable, que afectan al sistema urogenital, y que suponen aproximadamente el 50% de las intervenciones quirúrgicas en cirugía pediátrica. describiremos aquellas de mayor impacto y repercusión clínica desde el punto de vista quirúrgico, destacando las denominadas CaKUt o anomalías congénitas del riñón y del tracto urinario. estas últimas tienen su origen en la alteración de alguno de los procesos que conforman el desarrollo renal: a) Malformación del parénquima renal: hipoplasia, displasia, agenesia renal y riñón multiquístico (drMQ). b) anomalías relacionadas con la migración de los riñones: ectopia renal y anomalías de fusión. c) anomalías en el desarrollo del sistema colector: estenosis pieloureteral (epU), duplicidades ureterales, megauréter primario, uréter ectópico, ureterocele y válvulas de uretra posterior (VUp). El objetivo de este artículo es determinar en qué casos es necesaria la cirugía para resolver un problema urológico en el niño y cuándo es el mejor momento para realizar la intervención

Pediatric Urology comprises an extensive series of pathologies of variable complexity, which affect the urogenital system, and account for approximately 50% of surgical interventions in pediatric surgery. We will describe those ones of greater impact and clinical repercussion from the surgical point of view, highlighting the so-called CaKUt or congenital anomalies of the kidney and urinary tract. the latter have their origin in the alteration of some of the processes during the renal development: a) Malformation of the renal parenchyma: hypoplasia, dysplasia, renal agenesis and multicystic kidney (MCdK). b) abnormalities related to the migration of the kidneys: renal ectopia and fusion anomalies. c) abnormalities in the development of the collecting system: pyelo-ureteral stenosis, ureteral duplicities, primary megaureter, ectopic ureter, ureterocele and posterior uretral valves. R The objective of this article is to inform about the cases in which surgery is necessary to solve a urological problem in the child, and the best time to perform the intervention

Oncología quirúrgica pediátrica / Pediatric surgical oncology

Las neoplasias suponen la primera causa de muerte en niños mayores de 1 año en países desarrollados. la cirugía pediátrica juega un importante papel terapéutico en el cáncer infantil a distintos niveles: extirpación tumoral, implantación de accesos vasculares, manejo de metástasis y complicaciones. La oncología pediátrica es una especialidad muy dinámica con actualizaciones y cambios en protocolos constantemente. el objetivo de este artículo es la revisión actualizada de los tumores sólidos más frecuentes y con características específicas en relación con la cirugía pediátrica: neuroblastoma, tumor de Wilms, hepatoblastoma, tumores ováricos, testiculares y de partes blandas. El neuroblastoma es el tumor sólido extracraneal más común en la infancia. tanto el neuroblastoma como el tumor de Wilms suelen presentarse como una masa abdominal asintomática. la combinación del grupo de riesgo, edad, factores biológicos y resultados histológicos permite asignar a cada paciente un estadio de riesgo con valor pronóstico, y establecer una estrategia terapéutica específica en cada caso de neuroblastoma. el nefroblastoma o tumor de Wilms es el tumor renal maligno más frecuente en niños. en nuestro medio el nuevo protocolo establecido por la Siop se conoce como UMBrella que se basa en la quimioterapia preoperatoria con el objetivo de reducir masa tumoral seguido de cirugía. En los casos de hepatoblastoma es importante la clasificación preteXt (Pretreatment Extent of Disease) porque define la extensión de parénquima hepático afectada y enfermedad extrahepática dando información sobre la resecabilidad del tumor y su respuesta a la quimioterapia. Ante una masa de tejido blando debe plantearse en primer lugar que se trate de procesos reactivos y tumores benignos, los tumores malignos de partes blandas son raros. dentro de estos, el rabdomiosarcoma se caracteriza por su buena respuesta a quimioterapia, por lo que está en desuso la cirugía con resecciones agresivas o mutilantes. Respecto a los tumores gonadales en la infancia, a diferencia de los adultos, predominan los germinales, en concreto el teratoma. la cirugia tiende a ser conservadora para respetar la fertilidad futura. en las últimas décadas, los avances en tratamientos oncológicos han logrado un aumento de la supervivencia en la mayoría de tumores infantiles, en parte gracias al enfoque multidisciplinar necesario desde el diagnóstico de cada caso

Malignant neoplasms constitute the first cause of death in children over 1 year of age in developed countries. Pediatric surgery plays an important therapeutic role in childhood cancer on different sides: removing tumors, placing vascular access devices, metastasis management and complications. Pediatric oncology is a dinamyc specialty with constant updates and changes in protocols. the principal aim of this report is an updated review of more common solid tumors and their specific surgical aspects: neuroblastoma, Wilms' tumor, hepatoblastoma, ovarian tumor, testicular tumor and rhabdomyosarcoma. Neuroblastoma is the most common extracranial solid tumor in chidhood. Wilms' tumor and neuroblastoma usually appears as an asyntomatic abdominal mass. the risk group, age, biological factors and histologic analysis allow to assign a risk stage to each patient, that has prognostic value and determines the specific treatment for each case of neuroblastoma. Nephroblastoma or Wilms' tumor is the most frequent malignant kidney tumor in children. UMBrella is the new protocol of Siop based on preoperative chemotherapy to reduce the size of the tumor and surgery after. The classification PRETEXT (Pretreatment Extent of Disease) is important in cases of hepatoblastoma because defines the extent of hepatic infiltrated parenchyma, so we can know about its resectability and the response to chemotherapy. A soft-tissue mass is probably a reactive process or a benign tumor, since malignant soft-tissue tumors are rare. rhabdomyosarcoma has a good response to chemotherapy, so wide resections and radical surgery are not current techniques. With regard to gonadal tumors in childhood, unlike adults, germ tumors predominate, in particular teratoma. Sparing surgery is the current treatment to preserve future fertility. over the last several decades, the advances in cancer treatment have achieved an increased survival in most of childhood tumors, thanks to multidisciplinary approach from diagnosis

Implication of non-coding PAX6 mutations in aniridia.

There is an increasing implication of non-coding regions in pathological processes of genetic origin. This is partly due to the emergence of sophisticated techniques that have transformed research into gene expression by allowing a more global understanding of the genome, both at the genomic, epigenomic and chromatin levels. Here, we implemented the analysis of PAX6, whose coding loss-of-function variants are mainly implied in aniridia, by studying its non-coding regions (untranslated regions, introns and cis-regulatory sequences). In particular, we have taken advantage of the development of high-throughput approaches to screen the upstream and downstream regulatory regions of PAX6 in 47 aniridia patients without identified mutation in the coding sequence. This was made possible through the use of custom targeted resequencing and/or CGH array to analyze the entire PAX6 locus on 11p13. We found candidate variants in 30 of the 47 patients. 9/30 correspond to the well-known described 3' deletions encompassing SIMO and other enhancer elements. In addition, we identified numerous different variants in various non-coding regions, in particular untranslated regions. Among these latter, most of them demonstrated an in vitro functional effect using a minigene strategy, and 12/21 are thus considered as causative mutations or very likely to explain the phenotypes. This new analysis strategy brings molecular diagnosis to more than 90% of our aniridia patients. This study revealed an outstanding mutation pattern in non-coding PAX6 regions confirming that PAX6 remains the major gene for aniridia.

Factores implicados en el fracaso tardío del tratamiento endoscópico del reflujo vesicoureteral / Factors involved in the late failure of endoscopic treatment of vesicoureteral reflux

Introducción: Los resultados del tratamiento endoscópico del reflujo vesicoureteral (RVU) a corto plazo son excelentes. No obstante, con el paso de los años se ha identificado un número de pacientes en quienes el RVU que fue resuelto mediante esta técnica vuelve a aparecer. El objetivo de este trabajo es analizar los factores relacionados con este evento. Material y métodos: Se ha realizado un estudio analítico retrospectivo tipo caso-control incluyendo 395 unidades ureterales con RVU primario tratadas con éxito en nuestro centro, con seguimiento mínimo de 3 años. Se han identificado los casos en los que el RVU reapareció y se han analizado variables demográficas, variables relativas al RVU (grado, lateralidad, estudio inicial) y a la intervención (material utilizado). Resultados: Se identificaron 77 unidades ureterales con recidiva de las 395 incluidas (19,5%). La incidencia de recidiva fue del 29,7% en los pacientes tratados con dextranómero/ácido hialurónico (Dx/HA), del 20,2% en los tratados con polidimetilxilosano (MP) y del 12,2% en el caso de politetrafluoroetileno (PTFE). La aparición de recidiva se eleva hasta el 35% en el caso de pacientes tratados antes del año de edad y aquellos con RVU de gradoV. La clínica de disfunción miccional también eleva la incidencia de recidiva al 34,9%. Conclusión: El uso del material reabsorbible Dx/HA está relacionado con la recidiva del tratamiento endoscópico del RVU. Los reflujos de alto grado, junto con el tratamiento en edades precoces, así como la presencia de disfunción miccional, también son factores asociados a la recurrencia

Introduction: The short-term results of endoscopic treatment of vesicoureteral reflux (VUR) are excellent. Over time, however, a number of patients have been identified for whom VUR reappeared after being resolved with this technique. The aim of this study was to analyse the factors related to this event. Material and methods: A retrospective, analytical, case-control study included 395 ureteral units with primary VUR treated successfully at our centre, with a minimum follow-up of 3 years. We identified cases in which VUR reappeared and analysed the demographic variables, those related to VUR (grade, laterality, initial study) and those related to the operation (materials used). Results: We identified 77 ureteral units with recurrence in the 395 included uni:ts (19.5%). The recurrence rate was 29.7% for the patients treated with dextranomer/hyaluronic acid (Dx/HA), 20.2% for those treated with polydimethylsiloxane (MP) and 12.2% for polytetrafluoroethylene (PTFE). The onset of recurrence rose to 35% for patients treated before 1 year of age and those with gradeV VUR. Urinary dysfunction symptoms also increased the recurrence rate to 34.9%. Conclusion: The use of resorbable dextranomer/hyaluronic acid material was related to recurrence in the endoscopic treatment of VUR. The high-grade reflux and treatment at an early age, as well as the presence of urinary dysfunction, are also factors associated with recurrence

Factors involved in the late failure of endoscopic treatment of vesicoureteral reflux. / Factores implicados en el fracaso tardío del tratamiento endoscópico del reflujo vesicoureteral.

INTRODUCTION: The short-term results of endoscopic treatment of vesicoureteral reflux (VUR) are excellent. Over time, however, a number of patients have been identified for whom VUR reappeared after being resolved with this technique. The aim of this study was to analyse the factors related to this event. MATERIAL AND METHODS: A retrospective, analytical, case-control study included 395 ureteral units with primary VUR treated successfully at our centre, with a minimum follow-up of 3 years. We identified cases in which VUR reappeared and analysed the demographic variables, those related to VUR (grade, laterality, initial study) and those related to the operation (materials used). RESULTS: We identified 77 ureteral units with recurrence in the 395 included units (19.5%). The recurrence rate was 29.7% for the patients treated with dextranomer/hyaluronic acid (Dx/HA), 20.2% for those treated with polydimethylsiloxane (MP) and 12.2% for polytetrafluoroethylene (PTFE). The onset of recurrence rose to 35% for patients treated before 1 year of age and those with gradeV VUR. Urinary dysfunction symptoms also increased the recurrence rate to 34.9%. CONCLUSION: The use of resorbable dextranomer/hyaluronic acid material was related to recurrence in the endoscopic treatment of VUR. The high-grade reflux and treatment at an early age, as well as the presence of urinary dysfunction, are also factors associated with recurrence.

Patología quirúrgica hepatobiliar en la infancia / Pediatric hepatobiliary surgery pathology

La patología quirúrgica hepatobiliar en la infancia posee una gran relevancia debido a la repercusión que presenta en los niños afectados, razón por la que el correcto conocimiento de la misma se correlaciona con un diagnóstico temprano y un manejo oportuno que redunda en beneficio para nuestros pacientes. la presente revisión es una actualización centrada en las cuatro principales patologías hepatobiliares pediátricas. en primer lugar, se considera la atresia de vías biliares (aVB), que es la causa quirúrgica más común de enfermedad colestásica neonatal y la primera indicación de trasplante hepático infantil. es de resaltar la evolución en el concepto de la aVB en la última década, que recientemente se ha definido como un fenotipo caracterizado por la obliteración o ausencia de las vías biliares extrahepáticas, asociado a una alteración en la formación de los conductos biliares intrahepáticos, que puede deberse a múltiples etiologías. en segundo lugar, se revisa la dilatación de la vía biliar extrahepática y/o intrahepática, que afecta con mayor frecuencia al colédoco, conocida como quiste de colédoco. Se hace énfasis en su clasificación anatómica y etiológica, en el papel relevante de la colangiorresonancia en la evaluación preoperatoria de la lesión y en el tratamiento oportuno en función del tipo de dilatación y de la condición clínica del niño. en tercer lugar, se analiza el incremento de la presión del sistema venoso portal, denominado como hipertensión portal. dicho aumento de presión puede encontrar su origen en una obstrucción venosa a nivel prehepático, hepático y posthepático. Se destaca la singularidad de la hipertensión portal en la infancia que, a diferencia de los adultos, muestran una gran proporción de pacientes con una causa prehepática, los cuales desarrollan hiperesplenismo y hemorragia secundaria a várices esofagogástricas con mayor frecuencia, pero con una baja mortalidad atribuible a dicha hemorragia y ausencia de progresión a cirrosis hepática. Finalmente, se habla de la litiasis biliar en la infancia, destacando el amplio espectro clínico que presentan los niños afectados y la variedad en la composición de los cálculos biliares en pediatría, remarcando las estrategias terapéuticas a seguir en cada caso

Pediatric hepatobiliary surgery pathology is of great relevance due to its repercussion in the affected children. For this reason, correct knowledge about it is correlated with an early diagnosis and pertinent management that results in a benefit for our patients. This review is an update focused on the four main pediatric hepatobiliary pathologies. Bile duct atresia (BVA) is considered in the first place, this being the most common surgical cause of neonatal cholestatic disease and the first indication of child liver transplant. the evolution in the concept of BVa in the last decade should be emphasized, which has recently been defined as a phenotype characterized by the obliteration or absence of the extrahepatic bile ducts, associated to an alteration in the formation of the intrahepatic bile ducts, which can be due to multiple etiologies. in the second place, dilatation of the extrahepatic and/or intrahepatic bile ducts, that with greater frequency affects the choledoch, known as choledochal cyst, is reviewed. emphasis is placed on their anatomical and etiological classification, on the relevant role of the cholangioresonance in the pre-operative evaluation of the lesion and on the pertinent treatment based on the type of dilatation and on the clinical condition of the child. in the third place, an analysis is made of the increase of the pressure of the portal venous system, called portal hypertension. Said increase in pressure can originate in a venous obstruction on the prehepatic, hepatic or post-hepatic level. the singularity of portal hypertension in children stands out which, on the contrary to in adults, shows a large proportion of patients with a prehepatic cause, these developing hypersplenism and hemorrhaging secondary to esophagogastric varices more frequently. However, they have a low mortality attributable to said hemorrhaging and absence of progression to hepatic cirrhosis. Finally, mention is made of gallstones in children, stressing the wide clinical spectrum that the affected children have and the variety in the composition of the gallstones in pediatrics, emphasizing the therapeutic strategies to follow in each case

Metabolism Regulation by Estrogens and Their Receptors in the Central Nervous System Before and After Menopause.

Estrogens through their intracellular receptors regulate various aspects of glucose and lipid metabolism. The effects of estrogens in metabolism can be mediated by their receptors located in different areas of the brain such as the hypothalamus, which is involved in the control of food intake, energy expenditure, and body weight homeostasis. Alterations in the metabolic regulation by estrogens participate in the pathogenesis of the metabolic syndrome and cardiovascular diseases in women. The metabolic syndrome is an important disease around the world, consisting in a combination of characteristics including abdominal obesity, dyslipidemia, hypertension, and insulin resistance. It increases the risk of cardiovascular disease and type 2 diabetes. It has been suggested that there is an increase in the incidence of metabolic syndrome during menopause due to estrogens deficiency. Estrogens replacement improves insulin sensitivity and reduces the risk of diabetes in rats. In the brain, estrogens through the interaction with their receptors regulate the activity of neurons involved in energy homeostasis, including appetite and satiety. Thus, estradiol and their receptors in the hypothalamus play a key role in metabolic syndrome development during menopause.

Anquiloglosia neonatal. ¿Existe un exceso de indicación intervencionista? / Neonatal ankyloglossia. Is there an excess of interventionist indication?

Introducción: El objetivo de este estudio es analizar a los pacientes remitidos a cirugía para practicar una frenotomía en el periodo neonatal y revisar su correcta indicación, ya que en la actualidad no existe consenso en el diagnóstico y tratamiento de la anquiloglosia. Material y métodos: Se realizó un estudio retrospectivo de 136 pacientes con anquiloglosia derivados a la consulta de cirugía plástica pediátrica. Se recogieron datos sobre la remisión a la consulta, problemas de lactancia y experiencia anterior y posterior a la intervención. Resultados: Los principales motivos de consulta se relacionaron con problemas en la técnica de lactancia materna (69 pacientes [65,7%]). El dolor durante la toma era el síntoma más habitual, aunque 33 pacientes (31,4%) no presentaban síntomas. En la mayoría de los casos, la remisión a cirugía la realizó el pediatra de zona (85 pacientes [80%]). El tipo más frecuente de anquiloglosia en la muestra fue el tipo II. Sólo se detectó 1 caso de recidiva. La ansiedad debida a la separación durante la frenotomía fue la experiencia negativa más frecuente entre las madres. Discusión: La falta de indicaciones establecidas para la frenotomía, así como la relativa facilidad de la aplicación de la técnica en la edad neonatal y el auge de las campañas de lactancia materna, está derivando en un exceso de indicación quirúrgica como tratamiento de la anquiloglosia. Debe promoverse la creación de guías que definan unos criterios adecuados de tratamiento, así como favorecer la remisión a la consulta de lactancia como un paso previo a la cirugía (AU)

Introduction: The aim of this study is to analyze patients referred to frenotomy surgery during neonatal period and to review their correct indication, as nowadays there is no consensus on the diagnosis and treatment of ankyloglossia. Material and methods: A retrospective study of 136 patients with ankyloglossia referred to pediatric plastic surgery. Data collection was based on the consultation, breastfeeding problems and experiences before and after intervention. Results: The main reasons to attend consultation were related to breastfeeding technique (69 patients [65.7%]), and pain in between takes the more frequent symptom, nevertheless 33 patients (31.4%) had no symptoms. In most cases, referral to surgery was done by the pediatrician (85 patients [80%]). The most common type of ankyloglossia in the sample was type II. Only one case of recurrence was detected. Separation anxiety during frenotomy was the most frequent negative experience among mothers. Discussion: The lack of guidelines established for frenotomy and the relative ease of application of the technique in the neonatal age and the rise of breastfeeding campaigns, is leading to an excess of surgical indication as treatment of ankyloglossia. To create guidelines defining an appropriate criteria of treatment should be promoted, and to facilitate referrals to lactation consultation as a step prior to surgery (AU)

I Jornada de expertos en ictiosis / First Symposium of Ichthyosis Experts

El día 22 de junio de 2012 se celebró en el Hospital Niño Jesús la I Jornada de expertos en ictiosis, una jornada monográfica dirigida a dermatólogos, pediatras y médicos en formación interesados en esta enfermedad, así como al resto de profesionales sanitarios que participan en su atención. El objetivo de la l Jornada de expertos en ictiosis fue intentar estructurar la atención de los pacientes con ictiosis en España. Como ocurre con el resto de las enfermedades raras, su escasa prevalencia y la ausencia de centros de referencia formales diluyen el número de pacientes atendidos en cada centro, y pocos dermatólogos tienen verdadera experiencia clínica o conocen la manera de solicitar diagnóstico genético. En este artículo se resumen las ponencias expuestas en la Jornada para consulta de aquellas personas interesadas en el tema (AU)

On June 22, 2012 the First Symposium of Ichthyosis Experts in Spain was held at the Hospital Niño de Jesús in Madrid. It was a one-day symposium for dermatologists, pediatricians, and physicians-in-training interested in this disease, as well as for other health care professionals involved in the care of patients with ichthyosis. The aim of the meeting was to try to structure the care of ichthyosis patients in Spain. As happens in other rare diseases, because of the low prevalence of ichthyosis and the absence of designated referral centers, the number of patients treated in each center is very low and few dermatologists have any real clinical experience with this condition or know how to order diagnostic genetic tests. This article summarizes the presentations given at the symposium and is intended as a reference for anyone interested in the subject (AU)

First symposium of ichthyosis experts.

On June 22, 2012 the First Symposium of Ichthyosis Experts in Spain was held at the Hospital Niño de Jesús in Madrid. It was a one-day symposium for dermatologists, pediatricians, and physicians-in-training interested in this disease, as well as for other health care professionals involved in the care of patients with ichthyosis. The aim of the meeting was to try to structure the care of ichthyosis patients in Spain. As happens in other rare diseases, because of the low prevalence of ichthyosis and the absence of designated referral centers, the number of patients treated in each center is very low and few dermatologists have any real clinical experience with this condition or know how to order diagnostic genetic tests. This article summarizes the presentations given at the symposium and is intended as a reference for anyone interested in the subject.

Lactation reduces glial activation induced by excitotoxicity in the rat hippocampus.

Motherhood induces a series of adaptations in the physiology of the female, including an increase of maternal brain plasticity and a reduction of cell damage in the hippocampus caused by kainic acid (KA) excitotoxicity. We analysed the role of lactation in glial activation in the hippocampal fields of virgin and lactating rats after i.c.v. application of 100 ng of KA. Immunohistochemical analysis for glial fibrillary acidic protein (GFAP) and ionised calcium binding adaptor molecule 1 (Iba-1), which are markers for astrocytes and microglial cell-surface proteins, respectively, revealed differential cellular responses to KA in lactating and virgin rats. A significant astrocyte and microglial response in hippocampal areas of virgin rats was observed 24 h and 72 h after KA. By contrast, no increase in either GFAP- or Iba-1-positive cells was observed in response to KA in the hippocampus of lactating rats. Western blot analysis of GFAP showed an initial decrease at 24 h after KA treatment, with an increase at 72 h in the whole hippocampus of virgin but not of lactating rats. The number of GFAP-positive cells was increased by lactation in the dentate gyrus of the hippocampus but not in CA1 and CA3 areas. The present results indicate that lactating rats exhibit diminished responses of astrocyte and microglial cells in the hippocampus to damage induced by KA, supporting the notion that the maternal hippocampus is resistant to excitotoxic insults.