Reporte de caso: Quiste neuroentérico intradural/extramedular en paciente joven / Case report: Intradural/extramedullary neurenteric cyst in a young patient

Introducción: Los quistes neuroentéricos son lesiones congénitas, benignas, solitarias e infrecuentes del eje espinal, compuestas de tejido endodérmico heterotópico, productos del cierre incompleto del tubo neural. Se presentan, principalmente, en hombres durante la primera y segunda década de vida. Se localizan mayoritariamente en el espacio intradural/extramedular, ventralmente a la médula espinal, a nivel cervical bajo o torácico superior. La sintomatología neurológica depende de la localización del quiste. El diagnóstico presuntivo se realiza con una resonancia magnética, y el definitivo con un estudio histopatológico, llevado a cabo posterior a la exéresis completa o parcial de la muestra, con abordaje posterior, en la mayoría de los casos. Caso: Se describe el caso de una paciente de 35 años, quien tiene antecedente de haber presentado a los 14 años un quiste neuroentérico intradural/extramedular, a nivel cervial bajo. Había debutado con cervicalgia irradiada a miembros superiores e inferiores, radiculopatía, pérdida de la fuerza muscular, e hiperreflexia. Se realizó en ese momento una resonancia magnética, evidenciando una lesión ocupante de espacio en C5 y C6, la cual fue intervenida quirúrgicamente a través de una laminectomía en C5-C6 con abordaje posterior. Se realizó exéresis completa de la misma. El estudio anatomopatológico reportó quiste neuroentérico intradural/extramedular, sin atipias celulares. La evolución posoperatoria de la paciente resultó satisfactoria. Conclusión: Se describe este caso clínico, resaltando su importancia, al tratarse de lesiones sumamente infrecuentes en la literatura médica, con sintomatología poco específica, pudiendo confundirse con otras patologías, y recidivar, incluso después de haberse extraído completamente

Introduction: Neuroenteric cysts are congenital, benign, solitary, and infrequent lesions of the spinal axis, composed of heterotopic endodermic tissue, resulting from an incomplete closure of the neural tube. They mainly occur in men, during the first or second decade of life. Most of these cysts are located in the intradural/extramedullary compartment, ventrally to the spinal cord, especially at the lower cervical or upper thoracic spine. The neurological symptomatology varies depending on the location of the cyst. The presumptive diagnosis is made with magnetic resonance imaging, and the definitive diagnosis is made with a histopathological assessment, which is done after a complete or partial resection of the mass, generally with a posterior approach. Case presentation: We describe the case of a 35-year-old female patient, with the medical history of presenting an intradural/extramedullary neuroenteric cyst, located at the lower cervical level, at the age of 14. She presented cervical pain irradiated to upper and lower limbs, radicular pain, loss of muscular strength, and hyperreflexia. A magnetic resonance imaging was indicated, showing a space-occupying lesion at the C5 and C6 levels, which was surgically intervened through a posterior cervical (C5-C6) laminectomy. A complete resection of this mass was performed. The histopathological assessment reported an intradural/extramedullary neuroenteric cyst, with no cellular atypia. The patient's postoperative progress and development were satisfactory. Conclusion: The objective is to describe this case, highlighting its importance, since these lesions are extremely infrequent in the medical literature, with a non-specific symptomatology, which is why they can be confused with other pathologies, and recur, even after their complete resection.

The role of folic acid in preventing neural tube defects in Iraqi pregnant women: A cross sectional study

Folate deficiency in pregnant has been linked to neural tube defects in a substantial to neural tube defects in a substantial amount of medical literatures and has become a well-known fact among doctors; however, the knowledge of pregnant ladies about this association is poorly evaluated in our community. Vitamin and mineral deficiency is common among people in developing countries;however, the awareness of those people about such deficits and their associating compications is lacking. This study was conducted to evaluate the knowledge, attitude and practice of a cohort of pregnant ladies in Al-Diwaniyiah province in Mid-Euphrates region in Iraq. The present cross sectional study included 30 pregnant ladies. The study started on November 2018 and ended on January 2019. The study was carried out inAl-Diwaniyah maternity and child teaching hospital in Al-Diwaniyah province in Mid-Euphrates region, Iraq. The knowledge, attitude and practice of women regardin folie acid supplementation during early pregnancy was assessed according to 7 knowledge questions, 5 attitude questions and 5 practice questions. These questions and the demopgraphic data concerning women enrolled in the current study were obtained. The results of current study revealed poor knowledge about the advantage of folic acid in preventing congenital abnormalities was very obvious since ost of responses to the 7 questions concerning knowledge domain were within strongy disagreeing, disagree and neutral scores. Majority of response within attitude domain were in the form of disagree and strongly disagree. No correlation was found between any of the demain and the demographic characteristies of the study sample. Current study concluded that in Iraqi community, the knowledge and attitude of women toward the benefit of folie acid during early pregnancy in preventing neural tube defect iis poor; however, they practice well with this regard not due to their knowledge but because of the policy adopted by governmental antenatal care clinics and institutes (AU)

A modified open primary laparoscopic surgery port placement through umbilical tube

Background:A safe, reliable technique for primary trocar introduction is important for laparoscopic surgery. In resource-constrained settings where there is paucity of needed equipment and cost is prohibitive, a method utilizing fewer instruments will be useful.Aim:This study aims to describe a method of primary trocar introduction that utilizes any available port.Methods:A supra- or infra-umbilical incision is made into an everted tubular umbilicus. The linear alba is incised and the resultant opening bluntly developed, after which any available port is inserted using the trocar as a guide. The trocar is withdrawn while the sleeve is pushed in.Results:One hundred and three successful insertions were affected in 107 patients with age range of 1­75 years, with no significant gas leaks.Conclusion:This modified open approach is a simple and reliable way of primary port insertion. Access is gained easily in different age groups and umbilicus types

Tratamento cirúrgico de úlcera por pressão na unidade de Pediatria de hospital de reabilitação / Pressure ulcer treatment in the Pediatric Unit of a Rehabilitation Hospital

Introdução: A úlcera por pressão (UPP) é uma das complicações mais comuns em portadores de lesão medular, dos mais graves e frequentes problemas destes pacientes. A incidência e evolução depende dos grupos estudados, tendo as crianças e adolescentes características específicas durante o crescimento. A maior parte das descrições na literatura referem-se a adultos que desenvolveram UPP durante internação. O objetivo é descrever os resultados de pacientes operados para fechamento de úlcera por pressão na unidade de Pediatria do Hospital Sarah Brasília. Métodos: Estudo descritivo, retrospectivo de pacientes operados de 2005 a 2010 devido à lesão por pressão e análise estatística. Resultados: 116 pacientes foram admitidos para tratamento cirúrgico de UPP no Hospital Sarah, sendo 20 (17,2%) internados na Pediatria; 15 (75%) homens e 5 (25%) mulheres, todos provenientes de ambiente comunitário; 60% localizadas na região isquiática; com seguimento de 15 anos; 70% eram portadores de malformação do tubo neural. Os procedimentos cirúrgicos foram indicados para úlcera por pressão grau III e IV. Utilizou-se retalho do músculo glúteo (3), em ilha (8) ou península (3), fechamento primário e coccigectomia; retalho do músculo tensor da fáscia lata. Três casos apresentaram complicações, 65% evoluíram com recidiva e 15% com surgimento de novas lesões na região pélvica. Conclusão: A principal causa de lesão por pressão em Pediatria foi devido à malformação do tubo neural (70%), adquiridas na comunidade, localizadas na região isquiática. Embora estivessem em atendimento no programa de reabilitação, ainda foi observado alto índice de recidivas (65%).

Introduction: Pressure ulcers (PUs), or pressure sores, are among the most common serious complications in patients with spinal cord injury. The incidence and evolution in children and adolescents have specific characteristics. Most prior reports evaluated adults who developed PUs in the hospital. The objective is to describe the outcomes of surgical PU closure in children and adolescents in the Hospital Sarah Brasília pediatric unit. Methods: This was a retrospective statistical analysis of patients who underwent surgery for PUs. Statistical analysis of the incidence of PUs was performed using Epi Info version 3.2.2. Results: Of 116 patients who underwent surgery for PUs at the Hospital Sarah between 2005 and 2010, 20 (17.2%) were admitted to the Pediatric Unit. All 15 (75%) male and 5 (25%) female patients were admitted from a community setting. PUs were located in the ischial region in 60% of patients. The followup period was 15 years. Neural tube malformations were present in 70% of patients. We performed surgery for grade III and IV PUs, using gluteal flaps, primary closure and coccygectomy, and tensor fascia lata flaps. Three cases had complications, 65% developed recurrences, and 15% developed new ulcers in the pelvic region. Conclusion: The main cause of PUs in pediatric cases admitted to the rehabilitation hospital was a neural tube malformation (70%) in the ischial region. Despite admission to the rehabilitation program, a high recurrence rate (65%) was observed.

Quiste epidermoide frontotemporal en un hombre de 78 años: reporte de un caso y revision de la literatura / Cyst epidermoid cyst in a 78-year-old man: case report and literature review

Introducción: Los quistes epidermoides cerebrales son tumores de origen embrionario presentes desde la formación del tubo neural. Caso clínico: Hombre de 78 años con focalización en hemicuerpoizquierdo TAC cerebral reportó masa frontotemporal que se extirpó quirúrgicamente y el estudio histopatológico confirma diagnóstico de quiste epidermoide. Discusión: Los quistes epidermoides son tumores poco frecuentes y representan menos del 2% de los tumores intracraneales, el angulo cerebelopontino es la ubicación más frecuente y la resección completa junto con toda la cápsula es el único tratamiento efectivo.

Introduction: Epidermoid cysts are tumors of embryonic origin present since the formation of the neural tube. Case report: A 78-year-old male with a left hemisphere, brain CT scan reported frontotemporal mass that was surgically removed and the histopathological study confirmed a diagnosis of epidermoid cyst. Discussion: Epidermoid cysts are rare tumors and account for less than 2% of intracranial tumors, cerebellar angle is the most frequent site, and complete resection with the entire capsule is the only effective treatment.

Cirugía precoz en la prevención de la sepsis del recién nacido con mielomeningocele / Precocious surgery in preventing the sepsis of newborn with meningomyelocele

El mielomeningocele es una malformación congénita por defecto del cierre del tubo neural, se produce en las primeras semanas de crecimiento intrauterino. Consiste en una masa quística que incluye tejido nervioso y meninges, acompañadas de una fusión incompleta de los arcos vertebrales. Produce severos daños neurales y puede asociarse a otras malformaciones. Su reparación, en las primeras horas de vida, es necesaria para evitar complicaciones que pueden comprometer la vida del niño o causar mayor discapacidad. Se reportó un caso de un recién nacido con diagnóstico de mielomeningocele fisurado, a nivel lumbar. Se intervino quirúrgicamente en las primeras 6 h de vida; se disecó el saco dural y se separaron las raíces, logrando el cierre completo de la duramadre. Se reparó la piel con afrontamiento de los bordes y adecuada cicatrización de la herida. Fue aplicada antibioticoterapia profiláctica con cefalosporina de tercera generación. El infante egresó a los 10 días de operado sin complicaciones neuroquirúgicas asociadas (AU).

Meningomyelocele is a congenital malformation by defect of neural tube closing, produced in the first weeks of intrauterine grow. It is a cystic mass that includes nervous tissues and meninges, together with an incomplete fusion of the vertebral arches. It produces severe neural damages and could be associated to other malformations. It is necessary to repair it during the first hours after birth to avoid complications that could compromise the child’s life or cause more disability. The case of a new-born child diagnosed with fissured meningomyelocele at the lumbar level is presented. He was operated in the first six hours after birth; the dural sac was dissected and the roots separated, reaching the complete dura mater closure. The skin was repaired with edges affronting and adequate wound healing. Prophylactic antibiotic therapy with third generation cephalosporin was applied. The child was discharged 10 days alter the surgery without associated neurosurgical complications (AU).

Embriología del sistema ventricular cerebral / Cerebral ventricular system embryology

El Sistema Ventricular Cerebral se desarrolla de forma paralela al resto del Sistema Nervioso Central, facilitando la circulación del Líquido Cefalorraquídeo, desde su separación del líquido amniótico a nivel embrionario. Este desarrollo es necesario para entender correctamente la anatomía ventricular y facilitar el abordaje para patologías intraventriculares. El objetivo de esta revisión es reconocer los puntos más importantes en la embriología ventricular para facilitar el aprendizaje de la anatomía quirúrgica ventricular.

The cerebral ventricular system is developed in parallel with the rest of the central nervous system, facilitating the circulation of cerebrospinal fluid, from the amniotic fluid separation in the embryonic phases. This development is necessary to correctly understand the ventricular anatomy and facilitate approach to intraventricular pathologies. The objective of this review is to recognize the most important points in the ventricular embryology and in the intraventricular endoscopic vision to facilitate learning of the ventricular surgical anatomy.

Rol de la vitamina E en el tubo neural de embriones y fetos de ratón (Mus musculus) tratados con ácido valproico: estudio inmunohistoquímico de sonic hedgehog / Role of vitamin E in neural tube of mouse (Mus musculus) embryos and fetuses treated with valproic acid: immunohistochemical study of sonic hedgehog

Sonic hedgehog (SHH) es un morfógeno esencial para el desarrollo del tubo neural, miembros y somitos. Variaciones en su expresión pueden ocasionar alteraciones en el sistema nervioso. Esto lo producen teratógenos, como el ácido valproico (VPA), el cual aumenta las especies reactivas de oxígeno, pudiendo contrarrestarse con la administración de vitamina E (VE). Se buscó determinar la expresión de SHH en tubo neural y médula espinal en embriones y fetos de ratones expuestos a VPA, VPA + VE y VE. Se conformaron 8 grupos de ratones hembra (Mus musculus). A los 8 días post-coito (p.c.) se les administró a los grupos 1 y 5 suero fisiológico 0,3 ml; grupos 2 y 6 VPA 600 mg/kg; grupos 3 y 7 VPA 600 mg/kg + VE 200 UI/kg; grupos 4 y 8 VE 200 UI/kg, todos los tratamientos vía oral. A los 12 días p.c., se sacrificaron los grupos 1, 2, 3 y 4, y a los 17 días los restantes. Fueron fijados en solución Bouin e incluidos en paraplast. Se realizaron cortes transversales a nivel torácico. Se utilizó anticuerpo policlonal anti-SHH (Santa Cruz, H-160, conejo), dilución 1:100. Se describió la morfología de las muestras marcadas positivamente, se midió la densidad óptica integrada y porcentaje de área inmunoreactiva. La expresión de SHH fue inmunopositiva en notocorda y placa del piso del tubo neural solo en embriones de 12 días p.c. Los grupos tratados con VPA+VE y VE presentaron mayor intensidad inmunohistoquímica y porcentaje de área inmunoreactiva en comparación al grupo tratado con VPA (p 0,0001) en la placa del piso, siendo similar al grupo control. En la notocorda, la intensidad de inmunoreacción fue similar a lo demostrado en la placa del piso, con diferencias significativas (p 0,0001), pero el porcentaje de área no arrojó diferencias. Los grupos de 17 días de gestación resultaron negativos a la expresión de SHH. La vitamina E regula la expresión de SHH en tubo neural, atenuando así los efectos del VPA.

Sonic hedgehog (SHH) is an essential morphogen for the development of neural tube, members and somites. Variations in expression can cause abnormalities in the nervous system. This will produce teratogens, such as valproic acid (VPA), which increases the reactive oxygen species and can be counteracted with the administration of vitamin E (VE). We sought to determine the expression of SHH in the neural tube and spinal cord in mice embryos and fetuses exposed to VPA, VPA + VE and VE. For the study we used 8 groups of female mice (Mus musculus). At day 8 post-coitus (p.c.) the groups were administered as follows: groups 1 and 5,0.3ml saline; groups 2 and 6, VPA 600 mg/kg; groups 3 and 7, VPA 600 mg / kg + VE 200 IU/kg; groups 4 and 8, VE 200 IU/kg, all treatments were given orally. On the 12th day p.c., groups 1, 2, 3 and 4 were euthanized and the remaining groups at day 17. They were fixed in Bouin solution and included in paraplast; thoracic cross sections were performed, anti-SHH polyclonal antibody (Santa Cruz, H-160, rabbit) dilution 1:100 was used. We described morphology of the positively labeled samples and measured integrated optic density and percentage of immunoreactivearea.SHH expression was immunopositive in notochord and floor plate of the neural tube in embryos only 12 day p.c. In the groups treated with VPA + VE and VE immunohistochemistry showed greater intensity and percentage of immunoreactive area compared to those in the group treated with VPA (p0.0001) in the floor plate, being similar to the control group. In the notochord, immunoreaction intensity was similar to that shown in the floor plate, with significant differences (p 0.0001), but the percentage of area showed no differences. The groups at day 17 of gestation were negative for the expression of SHH. VE regulates expression of SHH in neural tube, thus attenuating the effects of VPA.

Efectos del tratamiento con vitamina E en el tubo neural y médula espinal en embriones y fetos de ratones Mus musculus expuestos al uso de ácido valproico / Effects of treatment with vitamin E in the neural tube and spinal cord Mus musculus mouse embryos exposed to the use of valproic acid

El ácido valproico (VPA) es el principal anticonvulsivante utilizado contra la epilepsia durante la gestación. Sin embargo, en etapas iniciales del embarazo actúa como teratógeno y ocasiona malformaciones como fisura labio-palatina, alteraciones en el desarrollo genital y espina bífida, siendo esta última la más frecuente. Esto se produce debido al aumento de especies reactivas de oxígeno, pudiendo contrarrestarse administrando vitamina E. El objetivo fue determinar si la vitamina E disminuye el daño en tubo neural y médula espinal de embriones y fetos de ratonas expuestas a VPA. Se conformaron 8 grupos de animales. A los 8 días post-fecundación se les administró a los grupos 1 y 5 suero fisiológico 0,3 mL; grupos 2 y 6 VPA 600 mg/Kg; grupos 3 y 7 VPA 600 mg/Kg y vitamina E 200 UI/Kg; grupos 4 y 8 vitamina E 200 UI/kg. A los 12 días post-fecundación, se sacrificaron los grupos 1, 2, 3 y 4, y a los 17 días los restantes grupos. Los embriones fueron procesados y teñidos con cresil violeta, observándose cortes histológicos a nivel cervical, torácico y lumbar. Los grupos tratados con vitamina E presentaron menor cantidad de neuroblastos y motoneuronas, pero de tamaño mayor en comparación al grupo tratado con VPA (p<0,05), siendo similares a los grupos controles. Al comparar el tubo neural y médula espinal en los distintos niveles (cervical, torácico y lumbar), no hubo diferencias estadísticamente significativas. La administración prenatal de vitamina E disminuye los defectos en tubo neural y médula espinal de embriones de 12 y 17 días de gestación sometidos a VPA.

Valproic Acid (VPA) is the main anticonvulsant used for epilepsy throughout the gestation period. However, when used at early stages of pregnancy, it acts as a tetarogenic agent, causing congenital malformations such as cleft-lip and/or cleft palate, abnormal genital development and spina bifida, being the latter the most frequent. This is the result of the increase of reactive oxygen species, which can be countered with the supplementation of vitamin E. The aim was determine if vitamin E minimizes the damage to the neural tube and spinal cord of mice embryos and fetuses previously exposed to VPA. Eight groups of mice were constituted. Eight days post fertilization, groups 1 and 5 were administered 0,3 ml of saline solution; groups 2 and 6 600mg/Kg of VPA, groups 3 and 7 600mg/Kg of VPA and 200UI/Kg of Vitamin E; groups 4 and 8 200 UI/Kg of Vitamin E. 12 days after fertilization, groups 1, 2, 3 and 4 were euthanized, whereas in the case of the remaining groups, the same process was performed 17 days after fertilization. The embryos were stained with cresyl violet, thus enabling the observation of histological sections at cervical, thoracic and lumbar levels. Groups supplied with vitamin E presented a lower amount of neuroblasts and motoneurons. However, these elements were bigger in size compared to the group treated with VPA (p<0,05), being these results similar to those obtained with the control groups. When comparing the neural tube and spinal cord at different levels (cervical, thoracic and lumbar), no statistically significant differences were found. It was determined that prenatal administration of vitamin E lessens the damage to the neural tube and spinal cord of mice embryos of 12 and 17 days of gestation previously exposed to VPA.

Caso clínico de enfermería: mujer embarazada y recién nacido con defecto del tubo neural / Nurse Case: pregnant women and newborn with neural tube defect

Resumen:

La contribución del profesional de enfermería en la promoción y difusión de los defectos del tubo neural, es relevante debido a que la prevalencia de malformaciones congénitas en México es de 4.9 por 10 000 defectos y el 75% de los casos corresponden a mielomeningocele. El propósito fue proporcionar cuidado integral de enfermería y de colaboración al binomio madre e hijo desde el ingreso de la madre hasta el egreso del neonato a su domicilio.

El caso se trata de una mujer embarazada de 33.6 semanas de gestación con trabajo de parto que ingresa al servicio de Gineco-Obstetricia con el fin de mejorar el pronóstico fetal e interrumpir el embarazo por vía abdominal. A las 19:08 horas, se obtiene producto masculino de 2,200g, talla 48 cm, perímetro cefálico 30 cm, Apgar 4/8, Capurro 33.5 semanas y presencia de mielomeningocele en región lumbar de aproximadamente 5x4 cm y extremidades inferiores sin movilidad. Los datos se registraron en la hoja de enfermería, estructurada con las etapas del proceso de enfermería y con base en los requisitos de desarrollo, desviación de la salud y universales del modelo conceptual de Dorothea Orem. El plan de cuidados de enfermería permitió integrar la atención al binomio madre-hijo, en la madre su evolución clínica es favorable y el lactante egresa reactivo a estímulos externos, reflejos de succión y deglución presentes, tolerancia a la vía oral y cicatriz quirúrgica en región lumbar.

Abstract:

The contribution of the nurse in the promotion and dissemination of neural tube defects, is relevant because the prevalence of congenital malformations in Mexico is 4.9 per 10 000 defects and 75% of the cases correspond to myelomeningocele. The purpose was to provide comprehensive nursing care and collaboration to the binomial mother and son from the income of the mother of the baby until discharge to home.

The case of a pregnant woman is 33.6 weeks gestation with labor entering the service of Gynecology and Obstetrics in order to improve the fetal prognosis and terminate the pregnancy through the abdomen. At 19:08 hours, masculine product 2,200g, height 48 cm, 30 cm head circumference, Apgar 4/8, Capurro and 33.5 weeks in the presence of lumbar myelomeningocele about 5x4 cm and lower extremities without mobility is obtained. Data were recorded on the sheet of nursing structured stages of the nursing process and based on the requirements of development, health deviation and universal conceptual model of Dorothea Orem. The plan of nursing care possible to integrate mother-son, mother in their clinical evolution is favorable and the infant withdraws reactive to external stimuli, sucking and swallowing reflexes present oral tolerance and surgical scar in region lumbar.

Minimally Invasive Lateral Lumbar Interbody Fusion: Surgical Technique and Review

STUDY DESIGN: Review of the current surgical technique and literature. OBJECTIVES: The aim of this study was to review the surgical technique and the current evidence on minimally invasive lateral lumbar interbody fusion (LLIF). SUMMARY OF LITERATURE REVIEW: Spinal fusion is a useful method in the treatment of various degenerative lumbar diseases. Recently, minimally invasive LLIF has been developed, enabling spine surgeons to perform anterior interbody fusion in a minimally invasive manner. MATERIALS AND METHODS: Review of the surgical technique and the literature. RESULTS: Minimally invasive LLIF may reduce the incidence of complications of anterior lumbar interbody fusion. LLIF may restore disc height more effectively than posterior lumbar interbody fusion and indirectly decompress the neural canal without nerve root or dural retraction or perineural scaring. The current indications for LLIF are almost equivalent to those of anterior and posterior lumbar interbody fusion. Recent studies have reported no differences in the fusion rate or clinical outcomes between LLIF and the conventional anterior or posterior interbody fusion techniques. However, LLIF has nonspecific complications, such as anterior thigh pain and hip flexor weakness. CONCLUSIONS: Minimally invasive LLIF is a promising surgical alternative to the conventional anterior or posterior fusion techniques. LLIF has the advantages of less intraoperative bleeding and soft tissue injury, and a faster return to work. However, postoperative nonspecific complications are problems that need to be addressed.

Change in Neural Tube Size for a Lumbar Spinal Stenosis Patient on Axial Loading Magnetic Resonance Imaging / 대한정형외과학회잡지

PURPOSE: A conventional magnetic resonance imaging (MRI) was conducted in supine position, showing a slight different from that conducted in upright position. Therefore, we simulated the upright position by applying the axial load on a lumbar spinal stenosis patient and measured the change of neural tube size in axial load and standardized the data. MATERIALS AND METHODS: We compared the axial loading MRI obtained from spinal stenosis patients who visited Wonkwang University Hospital outpatient clinic between October 2010 and May 2011 showing radiologic and physical symptoms. RESULTS: Neural tube sizes by conventional MRI were as follows: 195.57 mm² and 203.20 mm² on average between the left and right sides in L3/4; 194.64 mm² and 211.43 mm² on average in L4/5; and 199.38 mm² and 203.04 mm² on average in L5/S1. Neural tube sizes by axial loading MRI were as follows: 166.43 mm² and 174.27 mm² on average between the left and right sides in L3/4; 154.81 mm² and 158.67 mm² on average in L4/5; and 148.48 mm² and 157.19 mm² on average in L5/S1. Changes of neural tube sizes in L3/4, L4/5, and L5/S1 had a significant correlation (p<0.05). CONCLUSION: The axial loading device was an excellent tool in simulating the upright position for spinal stenosis patients, and the change of neural tube sizes reproduced for the upright position was statistically significant. This is thought to be meaningful for clinical applicability.

Knowledge of supplemental folic acid during pregnancy / Conocimiento de la suplementación de ácido fólico en la gestación / Conhecimento da suplementação de ácido fólico na gestação

Objective.To identify the use of folic acid during pregnancy, as well as the new mothers´ knowledge about folic acid. Methodology. Quantitative, descriptive exploratory, and prospective study. A total of 198 mothers were interviewed in the pediatric outpatient service of Hospital de Base Sao José do Rio Preto, Brazil. They have taken their children for neonatal screening and formally consented to participating in the study. The research project was approved by the Research Ethics Committee (350,287). A specific instrument was used for data collection. The data were entered into an appropriate spreadsheet and later statistically analyzed. Pearson´s chi-squared test, p <0.15, was used. Results. On average, the interviewed mothers were 25 years old and received less than two minimum wages. Most had prenatal in the first quartile and a mean of seven appointments, starting the use of folic acid from the 7th to the 9th week of gestational age. However, when asked about the importance of folic acid and its action, almost the majority was not able to answer. Conclusion. Although daily acid supplementation is recommended in prenatal care, this study found that consumption is inadequate, contributing to the increased risk of fetal malformation. Healthcare professionals, especially nurses, should develop educational activities for women about the use of folic acid in the pre-gestation period and in the first pregnancy trimester.

Objetivo. IdIdentificar el conocimiento y el uso durante el embarazo del ácido fólico. Metodología.Cuantitativo, exploratorio descriptivo y prospectivo. Se entrevistaron un total de 198 madres en el servicio de consulta externa de pediatría del Hospital de Base de Sao José do Rio Preto, Brasil quienes se habían llevado a sus hijos para el cribado neonatal y aceptado participar del estudio por consentimiento formal. El proyecto de investigación fue aprobado por el Comité Ético de Investigación (350.287). Se utilizó un instrumento específico para la recolección de datos. Asimismo, una hoja de cálculo para la transcripción de datos; después, se analizaron estadísticamente. Prueba Person's Chi-cuadrado se utilizó p <0,15. Resultados. Las puérperas entrevistadas tuvieron una media de 25 años, el 61.5% ganaba menos de dos salarios mínimos. El 96.5% realizó control prenatal; el 18.2% de las gestantes inició control tardíamente, entre el segundo y tercer trimestre de embarazo. El 81.3% de las mujeres consumió ácido fólico en la gestación; cuatro de cada cinco en los tres primeros meses de embarazo. Cuando se preguntó sobre la importancia del ácido fólico y de su acción, la mayoría de las mujeres no supo responder. Conclusión. Aunque la suplementación diaria con ácidofólico sea recomendada en el control prenatal, se verificó en este estudio que su consumo es inadecuado, lo que contribuye al aumento del riesgo de malformación fetal. El profesional del área de la salud y, especialmente, el enfermero deben desarrollar actividades educativas para las mujeres en cuanto al uso de ácido fólico en el período pregestacionacional y durante el primer trimestre del embarazo.

Objetivo.Identificar o uso do ácido fólico durante a gestação e o conhecimento de puérperas sobre o ácido fólico. Metodologia. Estudo quantitativo, descritivo exploratório e prospectivo. Entrevistou 198 puérperas, no ambulatório da pediatria do Hospital de Base, São José do Rio Preto, Brasil, que trouxerem seus filhos para triagem neonatal e aceitaram por escrito participar da pesquisa. O projeto de pesquisa foi aprovado pelo comitê de ética em pesquisa (350.287). A coleta de dados foi realizada com um instrumento específico. Os dados foram transcritos para uma planilha e analisados estatisticamente. Utilizou o teste de Qui-Quadrado de Person, p<0,15. Resultados. Em média, as puérperas entrevistadas possuem 25 anos e ganham menos de dois salários mínimos. A maioria realizou o pré-natal, no primeiro trimestre e tiveram uma media de sete consultas, com o início do uso de ácido fólico na idade gestacional de sete a nove semanas. Contudo quando questionadas sobre a importância do ácido fólico e sua ação, quase que a maioria significativa não soube responder. Conclusão. Embora a suplementação com ácido fólico diária seja recomendado no pré-natal, verificou-se que seu consumo foi inadequado, contribuindo para o aumento do risco de malformação fetal. O profissional da área da saúde, especialmente os enfermeiros, devem desenvolver atividades educativas para as mulheres quanto ao uso de ácido fólico de durante o período pré-gestacional e primeiro trimestre da gravidez.

Síndrome de Wiskott-Aldrich: Caso clínico / Wiskott-Aldrich syndrome: Case report

El síndrome de Wiskott-Aldrich es una inmunodeficiencia primaria; con una incidencia de 3,5 a 5,2 por cada millón de recién nacidos masculinos. Se caracteriza por tener un patrón de herencia recesiva ligada al cromosoma X. En estos pacientes; se ha descrito la tríada clásica de inmunodeficiencia; microtrombocitopenia y eczema. Presentamos un paciente de 5 años de edad; hispánico; con antecedentes de numerosas infecciones desde el primer año de vida. Actualmente; presenta desnutrición crónica; talla baja secundaria y retraso en el desarrollo del lenguaje. Se diagnosticó una mutación poco frecuente del gen asociado al síndrome de Wiskott-Aldrich.

The Wiskott-Aldrich syndrome is a rare X-linked recessive immunodeficiency, with an estimated incidence of 3.5 to 5.2 cases per million males. It is characterizedby immunodeficiency, microthrombocytopenia and eczema. We present a 5-year-old Hispanic male, with a medical history of numerous infectious diseases, compromised health, chronic malnutrition, language delay and failure to thrive. An infrequent mutation in the Wiskott-Aldrich syndrome gene was found.

Los niveles de ácido fólico en la población venezolana y su impacto en el área de salud pública / Folic acid levels in the venezuelan population and its impact on public health

Los folatos, constituyen cofactores fundamentales para los procesos bioquímicos que conducen a la sintesis y eventual modificación epigénetica de ácidos nucleicos e histonas. Además, su metabolismo está intimamente relacionado al metabolismo de la homocisteina. Se han descrito numerosas causas de mal funcionamiento del ciclo metabólico de folatos, la causa más frecuente y quizas la más relevante desde el punto de vista de salud pública es la deficiencia nutricional. El papel central del folato en la sintesis y posteriores modificaciones epigenéticas del material genético, por una parte, así como su relación con la homeostasis de la homocisteina, lo han asociado a dos tipos de patologías de gran importancia en salud pública: a) Enfermedades cardiovasculares y b) Defectos del desarrollo embrionario. En la actualidad, su papel en el riesgo cardiovascular es sujeto de controversia ya que estudios clínicos poblacionales no lo respaldan, sin embargo, si existe evidencia clara de su papel en la aparición de defectos del desarrollo embrionario, especificamente aquellos centrados en el tubo neural y el sistema cardio-circulatorio. Estas evidencias, llevaron a proponer diversos modelos programáticos en salud pública para prevenir y tratar a nivel poblacional el deficit nutricional de folatos. De los varios modelos ensayados, la fortificación obligatoria de ciertos alimentos de consumo masivo ha demostrado ser el mas exitoso y es actualmente implementado en 54 paises a nivel mundial. En Venezuela, desde la decada del 2000 se ha demostrado experimentalmente una seria deficiencia nutricional de folato, que afecta a más del 70% de la población femenina en edad fertil. A pesar de los múltiples llamados hechos a los responsables de las politicas de salud en el país, Venezuela sigue siendo uno de los pocos paises en la región que no ha implementado un programa de fortificación. Su implementación no requiere de una fuerte inversión oficial, pero si debe ser acompañado de un programa eficiente de seguimiento que permita a) evaluar los beneficios y b) las posibles modificaciones en su implementación(AU)

The folate cofactors are essential for the biochemical processes that lead to the eventual synthesis and epigenetic modification of histones and nucleic acids. In addition, their metabolism is closely related to homocysteine metabolism. While there have been described numerous causes malfunction folate metabolic cycle, the most common and perhaps the most important cause, from the point of view of public health, is nutritional deficiency. The central role of folate in the synthesis and subsequent epigenetic modifications of genetic material, on the one hand, and their relationship with the homeostasis of homocysteine, have been associated with two types of diseases of great public health importance: a) Cardiovascular Diseases b) Defects of embryonic development. Currently, its role in cardiovascular risk is the subject of controversy since this have not received support from population trials , however, there is clear evidence of their role in the onset of embryonic development defects, specifically those focused on the neural tube and the cardio-circulatory system. This evidence, led to propose different program models in public health, at the population level, to attack the nutritional deficit of folates. Of the several models tested, mandatory fortification of certain foods for mass consumption has proved the most successful and is currently implemented in 54 countries worldwide. In Venezuela, since the 2000s it has been shown, experimentally, a serious nutritional folate deficiency, which affects over 70% of the female population of childbearing age. Despite numerous appeals made to those responsible for health policies in the country, Venezuela remains one of the few countries in the region that has not implemented a program of fortification. Its implementation does not requires a strong government investment, but must also be accompanied by an effective monitoring program that allows a) evaluation of the potential benefits and b) support rational implementation of any necessary changes(AU)

Associação de mielomeningocele e mielocistocele: relato de caso / Association of myelomeningocele and myelocystocele: case report

Relatamos um caso de mielomeningocele dorsolombar associada à mielocistocele na região dorsal, interescapular em recém-nascido do sexo feminino. As correções cirúrgicas dos defeitos foram realizadas nas primeiras 24 horas. São revisadas as teorias de fechamento do tubo neural visando a uma justificativa para a associação dessas malformações.

We report a case of dorsal-lumbar myelomeningocele associated with a dorsal myelocistocele in a female newborn. The defects were surgically corrected in the first 24 hours. Revision of the neural tube closure theory was done, to justify the association of these malformation.

Morfógenos durante el desarrollo embrionario de vertebrados / Morphogens during embryonic development of vertebrates

Durante el desarrollo embrionario, las células de muchos tejidos se diferencian de acuerdo con la información de posición que se establece por las gradientes de concentración de morfógenos. Estas son moléculas de señalización secretadas en una región restringida de un tejido y se difunden lejos de su fuente para formar una gradiente de concentración. La molécula de un mismo morfógeno actúa generalmente en distintas etapas de desarrollo de un organismo y puede provocar reacciones muy diferentes en las células en función de su historia de diferenciación. Los morfógenos más conocidos son miembros del factor de crecimiento beta (TGF-b), Hedgehog (Hh), familias Wnt y los microRNAs.

During embryonic development, cells in many tissues differ according to the positional information that is set by the concentration of morphogen gradients. These are signaling molecules that are secreted in a restricted region of a tissue and diffuse away from their source forming a concentration gradient. Morphogens generally act at different development stages in an organism and cause different reactions in cells depending on their history of differentiation. The best known example of morphogens are members of growth factor beta (TGF-beta), Hedgehog(Hh), and Wnt families or microRNAs.

Temporal and Spatial Expression Patterns of miR-302 and miR-367 During Early Embryonic Chick Development

The microRNAs (miRNAs) are small, non-coding RNAs that modulate protein expression by interfering with target mRNA translation or stability. miRNAs play crucial roles in various functions such as cellular, developmental, and physiological processes. The spatial expression patterns of miRNAs are very essential for identifying their functions. The expressions of miR-302 and miR-367 are critical in maintaining stemness of pluripotent stem cells, including embryonic stem cells (ESCs) and induced pluripotent stem cells (iPSCs) but their functions in early development are not fully elucidated. So, we used Locked Nucleic Acid (LNA) probes to perform in situ hybridization and confirmed the temporal and spatial distribution patterns during early chick development. As a result, we found that miR-302 and miR-367 were expressed in various tissues such as primitive steak, neural ectoderm, neural plate, neural fold, neural tube, notochord, and oral cavity. Specially, we confirmed that miR-302 and miR-367 were strongly expressed in neural folds in HH8 to HH10. miR-302 was expressed on dorsal part of the neural tube but miR-367 was expressed on lateral and ventral parts of the neural tube. And also we performed quantitative stem-loop real-time PCR to analyze global expression level of miR-302 and miR-367. miR-302 and miR-367 expression was sustained before Hamburger and Hamilton stage (HH) 14. Thus, the temporal and spatial expression patterns of miR-302 and miR-367 may provide us information of the role of these miRNAs on tissue formation during early chick development.