Repression of Irs2 by let-7 miRNAs is essential for homeostasis of the telencephalic neuroepithelium.

Structural integrity and cellular homeostasis of the embryonic stem cell niche are critical for normal tissue development. In the telencephalic neuroepithelium, this is controlled in part by cell adhesion molecules and regulators of progenitor cell lineage, but the specific orchestration of these processes remains unknown. Here, we studied the role of microRNAs in the embryonic telencephalon as key regulators of gene expression. By using the early recombiner Rx-Cre mouse, we identify novel and critical roles of miRNAs in early brain development, demonstrating they are essential to preserve the cellular homeostasis and structural integrity of the telencephalic neuroepithelium. We show that Rx-Cre;DicerF/F mouse embryos have a severe disruption of the telencephalic apical junction belt, followed by invagination of the ventricular surface and formation of hyperproliferative rosettes. Transcriptome analyses and functional experiments in vivo show that these defects result from upregulation of Irs2 upon loss of let-7 miRNAs in an apoptosis-independent manner. Our results reveal an unprecedented relevance of miRNAs in early forebrain development, with potential mechanistic implications in pediatric brain cancer.

UCO Physical Rehabilitation: New Dataset and Study of Human Pose Estimation Methods on Physical Rehabilitation Exercises.

Physical rehabilitation plays a crucial role in restoring motor function following injuries or surgeries. However, the challenge of overcrowded waiting lists often hampers doctors' ability to monitor patients' recovery progress in person. Deep Learning methods offer a solution by enabling doctors to optimize their time with each patient and distinguish between those requiring specific attention and those making positive progress. Doctors use the flexion angle of limbs as a cue to assess a patient's mobility level during rehabilitation. From a Computer Vision perspective, this task can be framed as automatically estimating the pose of the target body limbs in an image. The objectives of this study can be summarized as follows: (i) evaluating and comparing multiple pose estimation methods; (ii) analyzing how the subject's position and camera viewpoint impact the estimation; and (iii) determining whether 3D estimation methods are necessary or if 2D estimation suffices for this purpose. To conduct this technical study, and due to the limited availability of public datasets related to physical rehabilitation exercises, we introduced a new dataset featuring 27 individuals performing eight diverse physical rehabilitation exercises focusing on various limbs and body positions. Each exercise was recorded using five RGB cameras capturing different viewpoints of the person. An infrared tracking system named OptiTrack was utilized to establish the ground truth positions of the joints in the limbs under study. The results, supported by statistical tests, show that not all state-of-the-art pose estimators perform equally in the presented situations (e.g., patient lying on the stretcher vs. standing). Statistical differences exist between camera viewpoints, with the frontal view being the most convenient. Additionally, the study concludes that 2D pose estimators are adequate for estimating joint angles given the selected camera viewpoints.

Predictors of perfusion computed tomography alterations in stroke mimics attended as stroke code.

BACKGROUND AND PURPOSE: Stroke mimics (SMs) account for a significant number of patients attended as stroke code (SC) with an increasing number over the years. Recent studies show perfusion computed tomography (PCT) alterations in some SMs, especially in seizures. The objective of our study was to evaluate the clinical characteristics and PCT alterations in SMs attended as SC in order to identify potential predictors of PCT alterations in SMs. METHODS: A retrospective study was performed including all SC activations undergoing a multimodal CT study including non-enhanced computed tomography (CT), CT angiography and PCT, as part of our SC protocol, over 39 months. Patients with a final diagnosis of SM after complete diagnosis work-up were therefore selected. Clinical variables, diagnosis, PCT alteration patterns and type of map affected (Tmax or time to peak, cerebral blood flow and cerebral blood volume) were registered. RESULTS: Stroke mimics represent up to 16% (284/1761) of SCs with a complete multimodal study according to our series. Amongst SMs, 26% (74/284) showed PCT alterations. PCT abnormalities are more prevalent in seizures and status epilepticus and the main pattern is alteration of the time to peak map, of unilateral hemispheric distribution or of non-vascular territory. In our series, the independent predictors of alteration in PCT in SMs are aphasia, female sex and older age. CONCLUSIONS: Perfusion computed tomography alterations can be found amongst almost a third of SMs attended as SC, especially older women presenting with aphasia with a final diagnosis of epileptic seizures and status epilepticus.

Microscale Fracture Behavior of Single Crystal Silicon Beams at Elevated Temperatures.

The micromechanical fracture behavior of Si [100] was investigated as a function of temperature in the scanning electron microscope with a nanoindenter. A gradual increase in KC was observed with temperature, in contrast to sharp transitions reported earlier for macro-Si. A transition in cracking mechanism via crack branching occurs at ∼300 °C accompanied by multiple load drops. This reveals that onset of small-scale plasticity plays an important role in the brittle-to-ductile transition of miniaturized Si.

Gene regulatory landscape of cerebral cortex folding.

Folding of the cerebral cortex is a key aspect of mammalian brain development and evolution, and defects are linked to severe neurological disorders. Primary folding occurs in highly stereotyped patterns that are predefined in the cortical germinal zones by a transcriptomic protomap. The gene regulatory landscape governing the emergence of this folding protomap remains unknown. We characterized the spatiotemporal dynamics of gene expression and active epigenetic landscape (H3K27ac) across prospective folds and fissures in ferret. Our results show that the transcriptomic protomap begins to emerge at early embryonic stages, and it involves cell-fate signaling pathways. The H3K27ac landscape reveals developmental cell-fate restriction and engages known developmental regulators, including the transcription factor Cux2. Manipulating Cux2 expression in cortical progenitors changed their proliferation and the folding pattern in ferret, caused by selective transcriptional changes as revealed by single-cell RNA sequencing analyses. Our findings highlight the key relevance of epigenetic mechanisms in defining the patterns of cerebral cortex folding.

Secondary loss of miR-3607 reduced cortical progenitor amplification during rodent evolution.

The evolutionary expansion and folding of the mammalian cerebral cortex resulted from amplification of progenitor cells during embryonic development. This process was reversed in the rodent lineage after splitting from primates, leading to smaller and smooth brains. Genetic mechanisms underlying this secondary loss in rodent evolution remain unknown. We show that microRNA miR-3607 is expressed embryonically in the large cortex of primates and ferret, distant from the primate-rodent lineage, but not in mouse. Experimental expression of miR-3607 in embryonic mouse cortex led to increased Wnt/ß-catenin signaling, amplification of radial glia cells (RGCs), and expansion of the ventricular zone (VZ), via blocking the ß-catenin inhibitor APC (adenomatous polyposis coli). Accordingly, loss of endogenous miR-3607 in ferret reduced RGC proliferation, while overexpression in human cerebral organoids promoted VZ expansion. Our results identify a gene selected for secondary loss during mammalian evolution to limit RGC amplification and, potentially, cortex size in rodents.

Language hemispheric dominance analyzed with magnetic resonance DTI: correlation with the Wada test.

OBJECTIVE: Language lateralization is a major concern in some patients with pharmacoresistant epilepsy who will face surgery; in these patients, hemispheric dominance testing is essential to avoid further complications. The Wada test is considered the gold standard examination for language localization, but is invasive and requires many human and material resources. Functional MRI and tractography with diffusion tensor imaging (DTI) have demonstrated that they could be useful for locating language in epilepsy surgery, but there is no evidence of the correlation between the Wada test and DTI MRI in language dominance. METHODS: The authors performed a retrospective review of patients who underwent a Wada test before epilepsy surgery at their institution from 2012 to 2017. The authors retrospectively analyzed fractional anisotropy (FA), number and length of fibers, and volume of the arcuate fasciculus and uncinate fasciculus, comparing dominant and nondominant hemispheres. RESULTS: Ten patients with temporal lobe epilepsy were reviewed. Statistical analysis showed that the mean FA of the arcuate fasciculus in the dominant hemisphere was higher than in the nondominant hemisphere (0.369 vs 0.329, p = 0.049). Also, the number of fibers in the arcuate fasciculus was greater in the dominant hemisphere (881.5 vs 305.4, p = 0.003). However, no differences were found in the FA of the uncinate fasciculus or number of fibers between hemispheres. The length of fibers of the uncinate fasciculus was longer in the dominant side (74.4 vs 50.1 mm, p = 0.05). Volume in both bundles was more prominent in the dominant hemisphere (12.12 vs 6.48 cm3, p = 0.004, in the arcuate fasciculus, and 8.41 vs 4.16 cm3, p = 0.018, in the uncinate fasciculus). Finally, these parameters were compared in patients in whom the seizure focus was situated in the dominant hemisphere: FA (0.37 vs 0.30, p = 0.05), number of fibers (114.4 vs 315.6, p = 0.014), and volume (12.58 vs 5.88 cm3, p = 0.035) in the arcuate fasciculus were found to be statistically significantly higher in the dominant hemispheres. Linear discriminant analysis of FA, number of fibers, and volume of the arcuate fasciculus showed a correct discrimination in 80% of patients (p = 0.024). CONCLUSIONS: The analysis of the arcuate fasciculus and other tract bundles by DTI could be a useful tool for language location testing in the preoperative study of patients with refractory epilepsy.

Prediction of Laterality in Temporal Lobe Epilepsy Using White Matter Diffusion Metrics.

BACKGROUND: Diagnostic methods of the epileptogenic area continue to be a challenge in epilepsy surgery research. We hypothesized that temporal lobe epilepsy (TLE) will result in white matter changes that can be detected using diffusion tensor imaging. Measurement of white matter diffusivity will therefore be useful for presurgical assessment. METHODS: Twelve patients with TLE who had undergone temporal lobectomy and amygdalohippocampectomy were included. In 6 patients, magnetic resonance imaging (MRI) showed evidence of mesial temporal sclerosis (m-TLE), whereas the 6 remaining MRI studies were informed without any abnormality (nl-TLE). All had excellent outcomes from surgery. Patients were compared with 12 age- and sex-matched controls. Five pairs of white matter fiber tracts were traced, and fiber tract fractional anisotropy and mean diffusivity were calculated. RESULTS: There were several alterations in diffusion parameters in white matter tracts, both ipsilateral and on the contralateral side, these alterations were more pronounced in the hemisphere ipsilateral to the epileptogenic focus. m-TLE patients had more alterations on ipsilateral side than nl-TLE patients, but similar alterations on contralateral side and bilateral fornix. The discriminant function analysis successfully lateralized all the patients with left TLE, 83.3% of the patients with right TLE, and all nl-TLE. CONCLUSIONS: Our results suggest that there are alterations in diffusion parameters in white matter tracts both in m-TLE and nl-TLE patients. Diffusion tensor imaging could be a useful presurgical tool to help establish the laterality of TLE, including patients with "normal" MRI. Further studies with a larger number of patients would be necessary to confirm these results.

Guía de Práctica Clínica en atresia esofágica / Clinical practice guide on esophageal atresia

La atresia esofágica es una de las anomalías congénitas más frecuentes en la práctica quirúrgica neonatal. Se estima que tiene una incidencia de 1 por cada 3500 recién nacidos vivos a nivel mundial. La preparación de guías de actuación y protocolos asistenciales es tendencia en la práctica médica actual. Esta Guía de Práctica Clínica se elaboró respondiendo a la necesidad de protocolizar la atención médico-quirúrgica de la atresia esofágica. En el Centro Territorial de Cirugía Neonatal de Holguín, donde se regionaliza la atención a neonatos de las cinco provincias orientales del país con afecciones congénitas y quirúrgicas de alta complejidad, la atresia esofágica fue la afección quirúrgica más frecuente en los últimos diez años, con una supervivencia ascendente que alcanzó 94,4 por ciento en 2019. La guía que se presenta se aprobó en el Primer Consenso Nacional de Guías de Prácticas Clínicas en Cirugía Pediátrica, en Varadero, Matanzas en 2019. Incluye las principales pautas para el diagnóstico, tratamiento y seguimiento de los pacientes afectados y se considera una herramienta eficiente para mejorar los resultados en la asistencia médica y quirúrgica neonatal(AU)

Esophageal atresia is one of the most common congenital anomalies in neonatal surgical practice. It is estimated to have an incidence of 1 per 3500 live newborns globally. The preparation of action guides and care protocols is a trend in current medical practice. This Clinical Practice Guide was prepared in response to the need to protocolize the medical-surgical care of esophageal atresia. In the Territorial Center for Neonatal Surgery of Holguín, where the care of neonates from the five eastern provinces of the country with congenital and surgical conditions of high complexity is regionalized, esophageal atresia was the most frequent surgical condition in the last ten years, with an ascending survival that reached 94.4 percent in 2019. The guideline presented was approved in the First National Consensus of Clinical Practice Guidelines in Pediatric Surgery, in Varadero, Matanzas in 2019. It includes the main guidelines for the diagnosis, treatment and follow-up of affected patients and is considered an efficient tool to improve outcomes in neonatal medical and surgical care(AU)

Infratentorial angioleiomyoma: case report and review of the literature.

INTRODUCTION: Intracranial angioleiomyomas are extremely rare lesions. Only 22 intracranial angioleiomyomas have been described in the literature and only three were infratentorial. CASE REPORT: We report a case of an infratentorial angioleiomyoma in a 43 year-old-man, who underwent a brain computer tomography because of hearing loss. The MRI showed a 1.4 cm tumor, initially described as a meningioma, with progressive enhancement after gadolinium injection, an augmented apparent diffusion coefficient and a generalized metabolite decreased in the spectroscopy. The lesion was surgically removed through a suboccipital approach with a good evolution and without postoperative complications. In the immunohistological study, the lesion was mainly composed of multiple vessels and the immunohistochemistry was positive for actin and caldesmon. Two years after surgery, no recurrence has been found in the MRI. CONCLUSION: Angioleiomyomas diagnostic may be complex, but some radiological features could help in the differential diagnostic. Angioleiomyomas are benign tumors associated with favorable outcomes after total resection, that in our case, did not show a significant bleeding risk.

TITLE: Angioleiomioma infratentorial: caso clinico y revision de la bibliografia.Introduccion. Los angioleiomiomas intracraneales son lesiones muy poco frecuentes. Solo se han descrito 22 casos en la bibliografia hasta la actualidad, unicamente tres de ellos de localizacion infratentorial. Caso clinico. Varon de 43 años con un angioleiomioma infratentorial descubierto de forma incidental tras la realizacion de una tomografia computarizada por hipoacusia. La resonancia magnetica mostro una tumoracion de 1,4 cm, descrita inicialmente como un meningioma, con un realce progresivo tras la administracion de gadolinio, un aumento del coeficiente de difusion aparente y un descenso generalizado de metabolitos en la espectroscopia. La lesion se reseco quirurgicamente mediante un abordaje suboccipital con buena evolucion y sin complicaciones postoperatorias. En el estudio histologico, la lesion presentaba un abundante componente vascular, y en la tincion inmunohistoquimica era positiva para actina y caldesmona. Dos años despues de la cirugia, el paciente no presentaba recurrencia en la resonancia magnetica de control. Conclusion. El diagnostico de los angioleiomiomas puede ser complejo, pero algunas de sus caracteristicas radiologicas pueden facilitarlo. Los angioleiomiomas son tumores benignos asociados con un resultado funcional favorable tras su reseccion completa, que en nuestro caso no presento un alto riesgo de sangrado.

Evolución histórica del proceso de atención al neonato quirúrgico en Cirugía Pediátrica / Historical evolution of the caring process to the surgical newborn in Pediatric Surgery

RESUMEN Introducción: la evolución histórica del proceso de atención al neonato quirúrgico en Cirugía Pediátrica se realizó teniendo como hito la creación de la Unidad de Cuidados Intensivos Neonatales por primera vez en Cuba, lo que significó un cambio en la calidad de la atención al recién nacido. Objetivo: determinar las características que definieron la evolución histórica del proceso de atención al neonato quirúrgico en Cirugía Pediátrica desde 1970 hasta la actualidad. Método: se utilizaron los métodos histórico lógico y enfoque sistémico para el estudio y la caracterización de las etapas del proceso de atención. Resultados: a partir de la década de 1970 se identificaron cuatro etapas del proceso de atención al neonato quirúrgico en Cuba: inicio (1970), fortalecimiento (1980), nuevas estrategias (1990), así como actualidad y proyecciones desde el año 2000, además se determinó el movimiento tendencial del mismo. Conclusiones: las etapas propuestas caracterizan el proceso de atención al neonato quirúrgico en Cirugía Pediátrica en Cuba y son: I Etapa: 1970 a 1979. Inicio de la atención médica al neonato quirúrgico, II Etapa: 1980 a 1989. Fortalecimiento de la atención médica al neonato quirúrgico. III Etapa: 1990 a 1999. Nuevas estrategias en la atención médica al neonato quirúrgico y IV Etapa: 2000 - 2018. Actualidad y proyecciones en la atención médica al neonato quirúrgico.

ABSTRACT Introduction: in Cuba, surgical newborn intensive care evolution has come up with the creation of Neonatal Intensive Care Unit. For the first time, newborns´ quality care has made a meaningful change in our health service quality. Objective: to figure out historical evolution characteristics of newborns´ surgical attention in Pediatric Surgery, since 1970 until nowadays. Method: we used the logical historical method, sistemic approach and stages of neonatal caring process. Results: in Cuba, since the 1970's four surgical newborn caring process stages were identified: beginning (1970), strenghening (1980), new strategics (1990). Since 2000, the updates and visualizations also determined this trend. Conclusions: previous stages define the surgical process newborns´ medical care inside Pediatric Surgery, in Cuba. We identified the following: Stage 1: 1970-1979 Beginning, stage 2: 1980-1989 Strenghening of medical attention to surgical newborn. Stage 3: 1990-1999 new medical attention strategics to the surgical newborn. Stage 4: 2000-2018 Current proyections of medical attention to the surgical newborn.

La supervivencia en neonatos con afecciones complejas en Cirugía Pediátrica / Neonates' survival from complex conditions after Pediatric Surgery

RESUMEN Introducción: el análisis de supervivencia es uno de los métodos estadísticos más empleado en los estudios clínicos. Objetivo: determinar la influencia de factores de riesgo en la supervivencia de los neonatos con afecciones complejas en Cirugía Pediátrica. Método: en este trabajo se estudiaron los pacientes atendidos en el Centro Regional de Cirugía Neonatal de Holguín, desde el 1ro de enero de 1994 hasta el 31 de diciembre del 2015. El universo estuvo constituido por 382 niños y la muestra por 295, con un tiempo de seguimiento de hasta 28 días. Se analizaron las variables como: peso al nacer, edad gestacional y anomalías asociadas; según los factores de riesgo como: bajo peso al nacer, neonato pretérmino y anomalías asociadas; así como su influencia en la supervivencia. Con la ayuda del paquete estadístico SPSS versión 15 sobre Windows XP, se efectuó el análisis de las curvas de supervivencia por el método Kaplan-Meier, y los factores de riesgo por el método Log-Rank. Resultados: del total de la muestra (n=295), se obtienen 66 neonatos con nacimiento pretérmino (22,4%), 82 con bajo peso al nacer (27,8%) y 154 con anomalías asociadas (52,2%); siendo este el factor de riesgo predominante. En dichos pacientes, se aprecia un alto número de eventos con influencia en su supervivencia, durante el seguimiento del período neonatal. El evento final ocurrió en 61 neonatos (20,7%). La media en el tiempo de supervivencia de los neonatos con afecciones complejas en Cirugía Pediátrica fue de 23 días. Conclusiones: en la supervivencia de los pacientes influyeron los factores de riesgo estudiados. La sepsis severa fue la causa directa de muerte que predominó.

ABSTRACT Introduction: survival study is one of the most used statistical methods in the clinical field. Objective: to determine the influence of risk factors over neonates´ survival form complex conditions after Pediatric Surgery. Method: in this work we studied patients attended in the Regional Center of Neonatal Surgery of Holguín, from January 1st, 1994 to December 31st, 2015. Universe was 382 neonates and the sample was 295, monitored during 28 days. Analyzed variables were: birth weight, gestational age and associated anomalies; according to its risk factors like: low birth weight, preterm neonates and associated anomalies, as well as their influence on neonates 'survival. By the use of statistical package SPSS version 15 on Windows XP, survival curves were analyzed by the Kaplan-Meier method and the risk factors by Log-Rank. Results: of the total sample (n=295) 66 neonates were preterm births (22.4%); 82 low birth weight (27.8%) and 154 showed associated anomalies (52.2%) as the main risk factor. Patients studied showed high numbers of events and their influence on survival during neonatal period. The final event occurred to 61 neonates (20.7%). Conclusions: risk factors studied were influenced by patients´ survival and the direct cause of death was severe sepsis.

Regionalización de la cirugía neonatal en las provincias orientales de Cuba / Regionalization of neonatal surgery in Eastern provinces of Cuba

INTRODUCCIÓN: el recién nacido quirúrgico es un paciente complejo, que necesita de equipos interdisciplinarios y centros altamente especializados para su atención médica. OBJETIVO: exponer los resultados obtenidos al ejecutar un grupo de acciones para mejorar los resultados del recién nacido quirúrgico con afecciones de mayor mortalidad, en el primer Centro Regional de Cirugía Neonatal del país. MÉTODOS: se realizó un estudio cuasi experimental, de los recién nacidos quirúrgicos de las provincias de Holguín y Santiago de Cuba, en el período comprendido entre el 1º de enero de 1999 y el 31 de diciembre de 2013. Para validar la regionalización se hizo una comparación de los resultados en las afecciones de mayor mortalidad.RESULTADOS: en el plano teórico se logró el concepto de regionalización. Se fundamentaron y elaboraron un conjunto de acciones para lograr mejores indicadores de supervivencia. En las 3 comparaciones se demostró su validez. En el período 2009-2013, a pesar de la atención de mayor número de casos complejos, se mantuvo el logro de la supervivencia (92 %). CONCLUSIONES: la regionalización de la cirugía neonatal ha tenido un efecto favorable en la supervivencia de los neonatos quirúrgicos en la región oriental de Cuba, y permitió la creación del primer centro regional del país.

INTRODUCTION: the surgical newborn is a complex patient who needs interdisciplinary teams and highly specialized centers for medical care. OBJECTIVE: to present the results of the implementation of a set of actions to improve the outcomes of the surgical newborn with high mortality conditions in the Regional Center of Neonatal Surgery. METHODS: quasi-experimental study of surgical newborns from Holguin and Santiago de Cuba provinces in the period of January 1st through December 31st, 2013. For validating the regionalization, the results were compared in the high mortality diseases. RESULTS: at the theoretical level, the concept of regionalization was devised. A set of actions were prepared and substantiated to achieve better survival rates. The three comparisons showed the validity of this concept. Despite the care to a higher number of complex cases in the 2009-2013 period, the survival rate was kept (92 %). CONCLUSIONS: regionalization of neonatal surgery has favorable effect on survival of surgical neonates in the Eastern region of Cuba and allowed the foundation of the first regional center in the country.

Evaluación de los resultados del traslado del recién nacido con tratamiento quirúrgico / Evaluation of the Transfer Results of the Newborn with Surgical Treatment

Introducción: el neonato quirúrgico requiere con relativa frecuencia de un traslado especializado hacia los centros que regionalizan su atención específica. Objetivo: evaluar la influencia del traslado en el pronóstico de los recién nacidos quirúrgicos intervenidos en el Centro Regional de Cirugía Neonatal del Hospital Pediátrico Provincial Octavio de la Concepción de la Pedraja de Holguín. Método: se realizó un estudio descriptivo desde enero de 2010 a diciembre de 2012 y se creó un protocolo para perfeccionar el traslado. Resultados: se trasladaron 139 recién nacidos con afecciones quirúrgicas provenientes de las provincias orientales y Holguín; predominó la edad de 1 a 7 días para ambos sexos (56,1 %), de forma general se alcanzó una supervivencia del 87,0 % y una mortalidad en las primeras 48 h del 5,0 %. La provincia que más casos trasladó fue Holguín. No existieron grandes diferencias entre las condiciones clínicas en que se trasladó y en las que se recibió el paciente, sin embargo, los registros de las mismas fueron insuficientes. Respecto a la correlación diagnóstica entre centro emisor y receptor las incongruencias predominaron en las malformaciones complejas del síndrome oclusivo, con diagnóstico incorrecto en 16 pacientes. La causa directa de muerte en la mayoría de los casos fue la sepsis. Conclusiones: los resultados del traslado fueron evaluados de aceptables, aunque el traslado dañino y deficiente influyó en la mortalidad en las primeras 48 horas.

Introduction: the surgical newborn often requires to be sent to specialized centers incharge of this specific care in the region. Objective: to assess the transfer influence on prognosis of neonates undergoing surgery at the Regional Surgical Newborn Center of Octavio de la Concepcion de la Pedraja Pediatric Hospital of Holguin. Method: a descriptive study was done from January 2010 to December 2012 and a protocol was set up to improve the surgical newborns’ movement. Results: one hundred and thirty nine (139) newborns with surgical diseases were sent to the above mentioned center, which were from eastern provinces and from Holguin. The predominant age was from one to seven days of both sexes (56.1 %) and in general the survival achieved was of 87.0 % and 5.0 % of mortality during the first 48 hours. The province which predominated regarding the transfer was Holguin. There were no great differences in clinical conditions between the cases which were transferred to and the ones that received the patients; nevertheless, the records were insufficient. Regarding the diagnostic correlation between the emissor and the receiver center, the irregularities prevailed on the complex malformations of occlusive syndrome, with wrong diagnosis in 16 patients. Sepsis was the direct cause of death in most of cases. Conclusions: the results of transfer were assessed as acceptable, although the harmful and deficient transfer influenced on the mortality in the first 48 hours.

Eventración diafragmática en el recién nacido / Diaphragmatic Hernia in the Newborn

La eventración diafragmática es una anomalía relativamente rara, se presenta con una elevación anormal de todo o una parte del hemidiafragma intacto. Se presentó un recién nacido de 41 semanas de edad gestacional, sin diagnóstico prenatal de malformación congénita, parto por cesárea por sufrimiento fetal agudo que se realizó en el Hospital Provincial Docente Carlos Manuel de Céspedes de Bayamo, Granma, nació deprimido, y en su evolución requirió intubación endotraqueal y ventilación mecánica. Se realizó radiografía de tórax, se interpretó como una hernia diafragmática derecha, se adoptaron las medidas correspondientes para su estabilización y se trasladó al Centro Regional de Cirugía Neonatal en Holguín. Se realizó ultrasonido toracoabdominal, y toracotomía derecha, se encontró una eventración diafragmática. Se realizó plicatura del músculo diafragma. A las 48 h presentó trastornos en la perfusión y la radiografía de tórax mostró imagen de neumoperitoneo, se reintervino y se encontró perforación puntiforme en el yeyuno que se suturó. La evolución postoperatoria fue satisfactoria.

A diaphragmatic hernia is a relatively rare condition with an abnormal elevation of the intact diaphragm. A newborn, of 41 weeks of gestation, without prenatal diagnosis of congenital malformation was presented; cesarean delivery was done in “Carlos M. de Céspedes” provincial hospital. Endotraqueal intubation and mechanical ventilation were required. Chest radiography showed diaphragmatic hernia, the patient was stabilized and referred to the Regional Neonatal Surgery Center. Sonographic scan was done and right thoracotomy, diaphragmatic hernia was found. The patient was operated on again in the next 48 hours; a yeyunal perforation was done and closed. The post operatory evolution was satisfactory.

Sustitución esofágica temprana en un paciente con atresia esofágica de brecha larga / Early Esophageal Replacement in a Patient with Long Gap Esophageal Atresia

La atresia esofágica de brecha larga es la falta congénita de continuidad del esófago con o sin comunicación a la vía aérea, donde una extensa separación entre los cabos esofágicos (mayor de cuatro centímetros) impide una anastomosis primaria. Para su tratamiento existen varias técnicas, pero ninguna es ideal. Se reportó un paciente transicional masculino, de un año y tres meses de edad, de raza blanca, procedente de Manzanillo, Granma, con diagnóstico de atresia esofágica de brecha larga en la etapa neonatal, al cual se le realizó sustitución esofágica temprana con estómago. Se revisó el expediente clínico del paciente, y se extrajeron los datos relacionados con los antecedentes, el cuadro clínico, los exámenes complementarios y el tratamiento.

Long gap esophageal atresia is the congenital lack of the continuity of esophagus with or without communication to the air way, where an extensive separation between the esophageal pouches (more than four centimeters) inhibit the primary anastomosis. For its treatment there are several techniques, but none of them is ideal. A male patient, with diagnosis of long gap esophageal atresia in the neonatal stage was reported, to whom the early esophageal replacement with stomach was performed. The patient's clinical record was revised, of which the data related with the history, the clinical manifestations, the complementary exams and the treatment were taken.

Alteraciones neuropsicológicas en pacientes con hipertensión portal prehepática / Neuropsychological alterations found in patients with prehepatic portal hypertension

INTRODUCCIÓN: en la hipertensión portal prehepática secundaria a obstrucción trombótica, pueden aparecer trastornos neurocognoscitivos, similares a la encefalopatía sistémica vista en afecciones hepáticas crónicas, la que puede estar bien definida clínicamente o en una forma subaguda, actualmente llamada encefalopatía hepática mínima. Esta consiste en la detección de déficits neuropsicológicos en pacientes sin alteraciones en la exploración neurológica rutinaria. En los niños, es difícil su estudio debido a la falta de pruebas neuropsicológicas estandarizadas para todas las edades. OBJETIVO: identificar la presencia de alteraciones neuropsicológicas en los pacientes pediátricos con hipertensión portal prehepática. MÉTODOS: se estudiaron 12 pacientes con hipertensión portal prehepática secundaria a cateterismo umbilical, mediante diferentes técnicas psicológicas. Las variables fueron: edad, tiempo de evolución de la enfermedad, forma clínica de inicio, alteraciones neuropsicológicas y procedimiento quirúrgico empleado. RESULTADOS: predominaron los pacientes entre 10 y 14 años de edad, y con un tiempo de evolución de la enfermedad entre 6 y 10 años. El sangrado digestivo alto fue la forma clínica de inicio más frecuente. En 11 casos se detectaron déficits en la atención involuntaria, concentración de la atención, memoria inmediata y dinámica de la actividad de la memoria. En 8 pacientes se afectó el pensamiento en su aspecto operacional, y en 6 la dinámica de la actividad del pensamiento. La mayoría de los niños con alteraciones neuropsicológicas no estaban intervenidos quirúrgicamente. CONCLUSIONES: existen trastornos predictivos de encefalopatía hepática mínima en pacientes con hipertensión portal prehepática. No utilizar el tratamiento quirúrgico puede estar relacionado con la aparición de las alteraciones neuropsicológicas. Teóricamente la solución sería derivaciones portoportales realizadas precozmente, o procedimientos que eliminen la obstrucción portal. Se requiere profundizar y generalizar esta investigación al resto de los pacientes con el diagnóstico de esta enfermedad

INTRODUCTION: in the prehepatic portal hypertension secondary to thrombotic obstruction, there may appear neurocognitive disorders similar to systemic encephalopathy seen in chronic hepatic illnesses. This portal hypertension may be clinically well-defined or occurs in a subacute form currently known as minimal hepatic encephalopathy. This consists of neurophyshcological deficits in patients without alterations in the routine neurological exploration. It is difficult to study it in children due to lack of standardized neuropsychological tests for all ages. OBJECTIVES: to identify the presence of neupsychological alterations in pediatric patients with prehepatic portal hypertension. METHODS: twelve patients with prehepatic portal hypertension secondary to umbilical catheterism were studied through different psychological techniques. The study variables were age, time of progression of disease, initial clinical form, neuropsychological alterations and surgical procedure used. RESULTS: predominance of patients aged 10 to 14 years and time of progression ranging 6 to 10 years. The upper digestive bleeding was the most common initial clinical form. Eleven patients showed deficit in involuntary attention, focused attention, immediate memory and dynamics of the memorizing activity. Eight patients suffered problems in the operational aspect of their thinking and 6 had the dynamics of their thinking affected. Most of children with neuropsychological alterations were not operated on. CONCLUSIONS: there are predictive disorders of minimal hepatic encephalopathy in patients suffering prehepatic portal hypertension. The failure to use the surgical treatment may be related to occurrence of neuropsychological alterations. Theoretically speaking, the solution would lie in performing early portoportal shunts or procedures eliminating the portal obstruction. It is necessary to delve into this research and generalize the results to the rest of patients diagnosed with this disease.

Tratamiento intraparto para la gastrosquisis / Intrapartum treatment for gastroschisis

El tratamiento intraparto de la gastrosquisis es una técnica quirúrgica llevada a cabo durante el nacimiento, bajo el principio de la preservación de la circulación fetoplacentaria, que permite reducir las vísceras herniadas antes de la primera respiración, evita la deglución del aire durante el llanto y minimiza la demora entre el nacimiento y la resolución quirúrgica de esta compleja afección. Se presenta la utilización de esta técnica, por primera vez, en el Centro Territorial de Cirugía Neonatal de Holguín, en un neonato femenino, procedente de la provincia de Santiago de Cuba, nacido por cesárea a las 36 semanas de gestación, e intervenido por un equipo multidisciplinario en la maternidad provincial de Holguín. La corrección del defecto se realizó en los primeros 15 minutos después del nacimiento, antes de cortar el cordón umbilical. La necesidad de apoyo ventilatorio fue menor de 24 horas, y se inició la vía oral en los primeros 3 días después del procedimiento. Al quinto día se observó el cierre total del defecto sin necesidad de suturas. Con la práctica del procedimiento Simil EXIT (similar al extra utero intrapartum treatment de sus siglas en inglés) para gastrosquisis, se logró la corrección inmediata de un defecto complejo de la pared abdominal, que es de alta morbilidad y mortalidad, y permitió la incorporación temprana de la paciente a una vida normal, sin huella quirúrgica y sin complicaciones.

Intrapartum treatment of gastroschisis is a surgical technique applied during birth under the principle of preservation of fetal-placental circulation. It allows reducing herniated viscera before first breathing, prevents air swallowing during crying and minimizes delay between birth and surgical resolution of this complex condition. This report presented the use of this technique for the first time in the territorial center of neonatal surgery in Holguin province. The patient was a female neonate from Santiago de Cuba province, she was born from cesarean section at 36 weeks of gestation and was operated on by a multidisciplinary team in the provincial maternal hospital of the province. The defect was corrected during the first 15 minutes after birth and before cutting the umbilical cord. The ventilation support was necessary for less than 24 hours, and three days after the procedure, the oral route started functioning. On the fifth day, there was observed the total closure of the defect without any suture. With the application of the Simil EXIT procedure (similar to extrauterine intrapartum treatment) for gastroschisis, it was possible to correct immediately the complex defect of the abdominal wall, which is of high mortality and morbidity, and to early incorporate the patient to her normal life with no trace of surgery and no complication.

Fibromatosis agresiva en edad pediátrica / Aggressive Fibromatosis in Pediatric Age

Introducción: la fibromatosis agresiva es rara, benigna, con alto grado de agresividad y recidivante. Objetivo: caracterizar la fibromatosis en pacientes atendidos en el Hospital Pediátrico Provincial de Holguín. Método: se realizó un estudio, transversal y retrospectivo de los pacientes, en edad pediátrica, con diagnósticos de fibromatosis profunda que incluyó los años 2002-2012. Resultados: la prevalencia de la fibromatosis fue de 2,1 por cada 100 000 habitantes. Predominó el sexo femenino. La localización glútea fue más frecuente seguida del cuello y la pared abdominal. La etiología fue desconocida en el 75 %. La biopsia por aspiración con aguja fina no fue útil para el diagnóstico. El tratamiento inicial fue quirúrgico e incompleto. La regresión espontánea ocurrió en el 50 % de la recidiva. La quimioterapia y la radioterapia se emplearon sin resultados satisfactorios. Conclusiones: debe considerarse el tratamiento inicial expectante, pues la regresión espontánea se ve en muchos casos y la cirugía no previene la recidiva independientemente del grado de resección tumoral.

Introduction: aggressive fibromatosis is an uncommon, benign and recurrent disease. Objective: to characterize fibromatosis in patients attended al Provincial Pediatric Hospital of Holguín. Method: a retrospective cross-sectional study on aggressive fibromatosis in children younger than 19 years old was done during 2002 to 2012. Results: fibromatosis prevalence was 2.1 per 100 000 inhabitants. Females prevailed over males. The tumor was mainly localized in the gluteal region followed by the neck and the abdominal wall. The etiology was unknown in 75 % of cases. Fine needle aspiration biopsy was not useful for diagnosis. The initial treatment was 100 % surgical but incomplete; nevertheless, spontaneous regression of recurrence was seen in 50 % of cases. Chemotherapy and radiotherapy were applied to recurrence without successful results. Conclusions: the procedure, as the first line of treatment, should be considered because of tumor spontaneous regression. Surgical resection degree of the tumor does not prevent recurrence.

Cirugía Pediátrica en Cuba. Etapas de su desarrollo / Pediatric surgery in Cuba. Stages of its development

La historia de la Cirugía Pediátrica cubana está pendiente de ser documentada científicamente. Se estableció como objetivo definir las etapas de desarrollo de la especialidad en Cuba, para lo cual se hizo un análisis histórico y se identificó cuatro períodos fundamentales. Este artículo tiene una significación práctica puesto que permite conocer en qué momento se encuentra la especialidad para modelar el futuro de la misma.

The history of Cuban pediatric surgery is pending of being scientifically documented. It was established as an objective to define the stages of the development of the specialty in Cuba. For this purpose a historical analysis was carried out and four main periods were identified. This study has a practical importance because it allows knowing the stage in which the specialty is to model its future.