Short-term outcomes after an enhanced recovery after surgery protocol in abdominal hysterectomies for leiomyomas in a teaching hospital.

OBJECTIVE: To compare the perioperative outcomes of patients undergoing abdominal hysterectomies for leiomyomas before and after the implementation of an enhanced recovery after surgery (ERAS) protocol in a teaching hospital. METHODS: This prospective cohort study compared a patient group from a historical series (pre-ERAS) with another group after ERAS implementation. Fasting time, length of hospital stay, complications, readmission rates, and procedure-related hospital costs were analyzed. RESULTS: Altogether, 187 patients were included in the analysis: 92 (49.2%) and 95 (50.8%) in the pre-ERAS and ERAS groups, respectively. Both groups had similar clinical characteristics. We observed reductions in surgical outcome findings: fasting time (13.9 to 6.7 h, P < 0.001), bladder catheter usage (21.1 to 10.9 h, P < 0.001), infection rates (20.7% to 5.3%, P = 0.002), length of stay (57.5 to 37.6 h), and 38.4% of the total estimated mean cost per procedure (USD $1570.8 to USD $967.2, P < 0.001) in the pre-ERAS and ERAS groups, respectively. Hospital readmission rates (P > 0.99) did not increase. CONCLUSION: ERAS protocol implementation for hysterectomies involving uterine leiomyomas reduced the length of hospital stay, surgical site infection rates, and hospital costs. A mean savings of USD $603.6 per procedure would allow 62.4% more hysterectomies to be performed.

A Pilot Study on the Use of Low Doses of CBD to Control Seizures in Rare and Severe Forms of Drug-Resistant Epilepsy.

Due to its anticonvulsant properties, cannabidiol can be supportive as an adjuvant therapy in the management of drug resistant epilepsy. This retrospective observational study evaluates the intensity and frequency of the seizures of patients with drug-resistant epilepsy that have been treated with antiepileptic medication associated with CBD in low doses for at least 12 months. Thirty-four patients were included in the study. The most frequent diagnosis of epilepsy was focal symptomatic epilepsy and Lennox−Gastaut syndrome (35.2%). During the follow-up, there was a statistically significant decrease in the seizure frequency (t student p < 0.001). A high proportion of patients, 16, concluded the study with a total control of the seizures reaching a 100% improvement, 12 reported ≥ 75% improvement, 3 ≥ 50%, and 2 ≥ 25%; only 1 patient had an improvement of less than 25%. This is the first Latin American study that demonstrates that long-term CBD added to the usual drugs significantly reduces the frequency, duration, and type of seizures in the different etiologies of epilepsy, being especially effective on the seizures that are the most incapacitating, improving the quality of life of the individual and their family.

Sindrome de Sjögren: una revisión de la literatura / Sjögren's syndrome: A review of the literature

El síndrome de Sjögren es un trastorno crónico autoinmune que afecta a las glándulas secretoras, principalmente salivales y lagrimales; además, puede presentar manifestaciones sistémicas extraglandulares. El objetivo de esta revisión fue revisar la literatura sobre los aspectos generales del síndrome de Sjögren, para lo cual se realizó una búsqueda en bases de datos entre el 15 de enero y el 15 de marzo del 2020, en donde se obtuvieron 29 artículos sobre los cuales se hizo la revisión. El síndrome de Sjögren tiene una importante prevalencia entre las enfermedades autoinmunes más comunes, caracterizada por presentar xerostomía y xeroftalmia. Los criterios diagnósticos tienen alta sensibilidad y especificidad y su tratamiento es sintomático.

Sjögren's syndrome is a chronic autoimmune disorder that affects the secretory glands, mainly salivary and lacrimal glands; and also can present extraglandular systemic manifestations. The objective of this review was to check the literature about the general aspects of Sjorgen's syndrome, for which a search of the literature was carried out between January 15 to March 15, 2020, 29 articles were obtained on which did the review. Sjögren's syndrome is highly prevalent among the most common autoimmune diseases, characterized by xerostomia and xerophthalmia. The diagnostic criteria have high sensitivity and specificity, and their treatment is symptomatic.

Cannabidiol: uso en epilepsias refractarias / Cannabidiol: its use in refractory epilepsies

Introducción. Algunos síndromes epilépticos se caracterizan por crisis de difícil control y asocian un retraso en el desarrollo neuropsicomotor, lo que conlleva un deterioro en la calidad de vida del paciente y su familia. Objetivo. Evaluar el uso del cannabidiol como tratamiento adyuvante en pacientes con epilepsias refractarias. Pacientes y métodos. Se realizó un estudio observacional por medio de una encuesta dirigida a la persona cuidadora del paciente. Se valoró la información sobre el paciente y el cuidador, cambios observados sobre las crisis, efectos neuropsicológicos, efectos adversos y percepción global de la familia tras el uso del cannabidiol. Resultados. Se evaluó a 15 pacientes con epilepsias refractarias, quienes recibieron cannabidiol durante un período de un mes a un año. En el 40% de los pacientes hubo una disminución en la frecuencia de las crisis, en el 60% de los pacientes se observó un control de más del 50% de las crisis y en el 27% las crisis desaparecieron totalmente. También se comunicaron cambios neurocognitivos: en el 73% hubo una mejoría del comportamiento; el 60% notificó una mejoría en el lenguaje; el 50%, en el sueño; el 43%, en la alimentación; y el 100%, en el estado de ánimo. La percepción global sobre la enfermedad notificó una mejoría en el 73%. Los efectos adversos más frecuentes fueron somnolencia y fatiga. Conclusiones. Estos resultados sugieren un posible efecto beneficioso del cannabidiol sobre el control de las crisis y en la mejoría de ciertos aspectos neurocognitivos en pacientes con epilepsias refractarias (AU)

Introduction. Some epileptic syndromes are characterised by seizures that are difficult to control and are associated to delayed neuropsychomotor development, which results in a deterioration in the patient’s quality of life as well as in that of his or her family. Aim. To evaluate the use of cannabidiol as adjuvant therapy in patients with refractory epilepsies. Patients and methods. An observational study was conducted by means of a survey addressed to the patient’s caregiver. Data collected included information about the patient and the caregiver, changes observed in the seizures, neuropsychological effects, side effects and the family’s overall perception following the use of cannabidiol. Results. The evaluation examined 15 patients with refractory epilepsies, who received cannabidiol over a period ranging from one month to one year. The frequency of seizures decreased in 40% of the patients, 60% of the patients were seen to have control over 50% of their seizures and in 27% of them the seizures disappeared completely. Neurocognitive changes were also reported: behaviour improved in 73%; 60% reported an improvement in language; in 50% sleep improved; 43% reported improvements in eating habits; and 100% said their mood had improved. The overall perception of the illness was that there had been improvements in 73% of respondents. The most common side effects were drowsiness and fatigue. Conclusions. These results suggest a possible beneficial effect of cannabidiol on the control of seizures and on the improvement of certain neurocognitive aspects in patients with refractory epilepsies (AU)

Emergencias neuropediatricas en niños atendidos en el Hospital Universitario de Maracaibo / Neuropaediatric emergencies in children admitted to the Hospital Universitario, Maracaibo, Venezuela

Durante los últimos años se ha observado un incremento en el número de consultas en los servicios de emergencias pediátricas de forma proporcional al incremento de las consultas por motivos neurológicos. Objetivos: Analizar la demanda de la actividad asistencial neuropediátrica y conocer los motivos de consultas más frecuentes y su distribución epidemiológica. Métodos: Estudio observacional de cohorte histórica mediante el registro prospectivo durante un año en el servicio de Emergencia Pediátrica del Hospital Universitario de Maracaibo. Resultados: Se solicitó evaluación neuropediátrica a 172 pacientes constituyendo el 0,17 % de todas las emergencias entre 0 y 15 años de edad, predominando los lactantes (39%) y niños varones (53,5%). Las crisis convulsivas son el motivo de consulta más frecuente (45%), seguidas del retraso psicomotor y la sospecha de encefalopatías estáticas. La epilepsia fue el principal diagnóstico de ingreso (23%) y egreso (25%) seguido de meningitis bacteriana aguda. Conclusiones: Las urgencias neurológicas constituyen un porcentaje significativo del total de las urgencias pediátricas. Son muchos los motivos de consulta y es rol del pediatra de emergencias solicitar la valoración especializada. Las crisis convulsivas, retraso psicomotor, infecciones del sistema nervioso central, síncope y cefalea constituyen los cinco principales motivos de consulta. Sin embargo la demora psicomotora no constituye una urgencia, por lo que el pediatra debe estar en capacidad de tomar la decisión acertada a quien debe derivar y solicitar la consulta por el especialista de forma inmediata.

In recent years there has been an increase in the number of consultations in pediatric emergency services in proportion to the increase in consultations for neurological reasons.Objectives: To analyze the demand of neuropaediatric care, and to determine the most frequent reasons for neuropediatric consultations. Methods: Historical cohort observational study by means of a prospective registry during one year in the Pediatric Emergency Service of the University Hospital of Maracaibo, Venezuela. Results: Neuropediatric evaluation was requested for 172 patients, 0.17% of all emergencies, ages from 0 to 15 years, predominantly infants (39%) and boys (53.5%). Seizures were the most frequent reason for consultation (45%), followed by psychomotor retardation and suspected static encephalopathies. Epilepsy was the most frequent diagnosis at admission (23%) and discharge (25%), followed by acute bacterial meningitis. Conclusions: Neurological emergencies constitute a significant percentage of all pediatric emergencies. There are many reasons for consultation and the pediatrician's role is to decide who deserves specialized neurologic care or who may be derived for an outpatient emergency assessment. Seizures, psychomotor retardation, central nervous system infections, syncope and headache, were the five main reasons for consultation; however psychomotor delay does not constitute an emergency, so the pediatrician should be able to decide which patient should be derived to the neuropediatrician.

Las alteraciones neuropsicológicas son frecuentes en la epilepsia rolándica y sus evoluciones atípicas / Neuropsychological alterations are frequent in rolandic epilepsy and its atypical developments

Introducción. La epilepsia rolándica o epilepsia benigna de la infancia con puntas centrotemporales se denomina benigna debido a lo favorables que suelen ser sus crisis y a la espontánea normalización del electroencefalograma al llegar a la pubertad, aunque se ha demostrado el impacto sobre el desarrollo cognitivo con la presencia de déficits cognitivos heterogéneos, relacionados especialmente con las descargas intercríticas persistentes durante el sueño no REM. El objetivo de este trabajo es estudiar las redes epileptógenas involucradas en los trastornos neuropsicológicos de esta patología. Desarrollo. Las evoluciones atípicas tienen en común una actividad epiléptica persistente durante el sueño lento, que desempeña un papel importante en el desarrollo de los déficits neurocognitivos que se asocian a esta patología. Factores como la edad de inicio de la epilepsia, el inicio de la evolución atípica, la localización de las descargas interictales y la actividad epiléptica continua durante el sueño que persista durante más de dos años pueden provocar cambios en el funcionamiento de las redes neurocognitivas, con los consecuentes déficits en las funciones neuropsicológicas, que incluso pueden resultar irreversibles. Conclusiones. Es necesario un seguimiento cercano tanto clínico como electroencefalográfico; además, deben realizarse estudios neuropsicológicos formales desde el inicio de la epilepsia benigna de la infancia con puntas centrotemporales y más en los casos que es evidente una evolución atípica para detectar y prevenir los déficits neuropsicológicos antes de que se instauren definitivamente (AU)

Introduction. Rolandic epilepsy or benign childhood epilepsy with centrotemporal spikes is called benign because its seizures are usually favourable and due to the spontaneous normalisation of the electroencephalogram on reaching puberty. Nevertheless, evidence has been found of the impact on cognitive development with the presence of heterogeneous cognitive deficits, especially related to persistent intercritical discharges during non-REM sleep. The aim of this study is to examine the epileptogenic networks involved in the neuropsychological disorders of this pathology. Development. A common feature of the atypical developments is persistent epileptic activity during slow sleep, which plays an important role in the development of the neurocognitive deficits that are associated to this pathology. Factors such as the age at onset of the epilepsy, the onset of the atypical development, the location of the interictal discharges and the continuous epileptic activity during sleep that persists for more than two years can trigger changes in the functioning of the neurocognitive networks. This may result in deficits in the neuropsychological functions, which may even be irreversible. Conclusions. A close clinical and electroencephalographic follow-up is necessary. Moreover, formal neuropsychological studies must be conducted as of the onset of benign childhood epilepsy with centrotemporal spikes. This is even more necessary in cases in which there is an obvious atypical development in order to detect and prevent the neuropsychological deficits before they establish themselves on a definitive basis (AU)

[Association between the degree of implementation of the Brazilian Breastfeeding Network and breastfeeding indicators]. / Associação entre o grau de implantação da Rede Amamenta Brasil e indicadores de amamentação.

This study aimed to evaluate the implementation of the Brazilian Breastfeeding Network and its impact on breastfeeding indicators. Implementation was analyzed according to type, including 56 primary healthcare units from three Brazilian municipalities. For evaluation of the degree of implementation, a score was created for each unit based on compliance with Ministry of Health certification criteria. Effects of implementation were analyzed according to exclusive and overall breastfeeding rates. Eighteen (32.1%) of the units met the four criteria for certification. The study sample included 1,052 children less than one year of age, of whom 563 were less than six months old. Units that met the four criteria for certification showed higher rates of exclusive breastfeeding (44%) when compared to the other units. Difficulties in implementing the Brazilian Breastfeeding Network were identified, and breastfeeding indicators varied according to the number of certification criteria met by the primary healthcare unit.

Associação entre o grau de implantação da Rede Amamenta Brasil e indicadores de amamentação / Association between the degree of implementation of the Brazilian Breastfeeding Network and breastfeeding indicators / Asociación entre el grado de implantación de la Red Amamanta Brasil e indicadores de lactancia materna

O objetivo foi avaliar a implantação da Rede Amamenta Brasil e seu impacto sobre indicadores de aleitamento materno (AM). Realizou-se análise de implantação de segundo tipo, incluindo 56 unidades básicas de saúde (UBS) de três municípios brasileiros. Para a avaliação do grau de implantação, criou-se um escore para cada UBS baseado no cumprimento dos critérios de certificação do Ministério da Saúde; para a análise dos efeitos da implantação, foram pesquisadas as prevalências de amamentação exclusiva e de amamentação. Verificou-se que 18 (32,1%) UBS cumpriam os quatro critérios preconizados para certificação. Foram incluídas no estudo 1.052 crianças menores de um ano, sendo 563 menores de seis meses. Verificou-se que as UBS que cumpriam os quatro critérios de certificação tiveram maior prevalência de amamentação exclusiva (44%), quando comparadas às demais UBS. Dificuldades para a implantação da Rede Amamenta Brasil foram identificadas, e os indicadores de AM variaram de acordo com o número de critérios de certificação cumpridos pelas UBS.

This study aimed to evaluate the implementation of the Brazilian Breastfeeding Network and its impact on breastfeeding indicators. Implementation was analyzed according to type, including 56 primary healthcare units from three Brazilian municipalities. For evaluation of the degree of implementation, a score was created for each unit based on compliance with Ministry of Health certification criteria. Effects of implementation were analyzed according to exclusive and overall breastfeeding rates. Eighteen (32.1%) of the units met the four criteria for certification. The study sample included 1,052 children less than one year of age, of whom 563 were less than six months old. Units that met the four criteria for certification showed higher rates of exclusive breastfeeding (44%) when compared to the other units. Difficulties in implementing the Brazilian Breastfeeding Network were identified, and breastfeeding indicators varied according to the number of certification criteria met by the primary healthcare unit.

El objetivo de este artículo fue evaluar la implantación de la Red Amamanta Brasil y su impacto sobre indicadores de lactancia materna (AM). Se realizó un análisis de implantación de segundo tipo, incluyendo 56 unidades básicas de salud (UBS) de tres municipios brasileños. Para la evaluación del grado de implantación se creó un marcador para cada UBS, basado en el cumplimento de los criterios de certificación del Ministerio de Salud; y para el análisis de los efectos de la implantación fueron investigadas las prevalencias de lactancia materna exclusiva y lactancia. Se verificó que 18 (32,1%) UBS cumplían los cuatro criterios preconizados para certificación. Se incluyeron en el estudio 1.052 niños menores de un año, siendo 563 menores de seis meses. Se verificó que las UBS que cumplían los cuatro criterios de certificación tuvieron mayor prevalencia de lactancia materna exclusiva (44%) cuando se comparan con las demás UBS. Dificultades para la implantación de la Red Amamanta Brasil fueron identificadas y los indicadores de AM variaron de acuerdo con el número de criterios de certificación cumplidos por las UBS.

Espectro de enfermedades desmielinizantes en pediatria experiencia Hospital Universitario de Maracaibo / Spectrum of pediatric experience demyelinating diseases Hospital Universitario de Maracaibo

El espectro de enfermedades desmielinizantes constituye un grupo de entidades clínicas e imagenológicas que presentan una base inmunológica autoinmune donde encontramos más frecuentemente en niños la Encefalomielitis Aguda Diseminada (EMAD), seguida de Neuritis Óptica (NO), Mielitis Transversa (MT), Neuromielitis Óptica (NMO), y Esclerosis Múltiple (EM). Objetivo: Describir el perfil clínico y epidemiológico de las enfermedades desmielinizantes en la edad pediátrica. Métodos: Estudio observacional, descriptivo, incluyendo todos los pacientes menores de 18 años, valorados en el servicio de Neurología Pediátrica del Hospital Universitario de Maracaibo, entre enero 2014 a marzo 2015 con diagnòstico confirmado de enfermedad desmielinizante. Resultados: 11 pacientes entre 1 y 14 años presentaron diagnóstico de enfermedades desmielinizantes predominando el género femenino (64%), la mayoría del Municipio Maracaibo del Estado Zulia. La entidad más frecuente fue EMAD (64%); la clínica predominante fue alteración del estado de conciencia tipo somnolencia (55%). Se presentó un pico en el mes de febrero para la presentación con predominio en el grupo etario adolescentes (37%). Cien porciento de los pacientes presentaron imágenes sugestivas de lesión de sustancia blanca. Conclusión: Las enfermedades desmielinizantes son una realidad en pediatría, siendo en este grupo etario la EMAD la entidad más frecuente. Dada la variedad clínica se hace necesario el conocimiento de estas entidades a fin de poder indicar tratamientos oportunos y adecuados.

The spectrum of demyelinating disease is a group of clinical and imaging entities that have an autoimmune immunological basis, among which the most often described in children is Acute Disseminated Encephalomyelitis (ADEM), followed by optic neuritis (NO), transverse myelitis (MT), optical neuromyelitis (NMO) and Multiple Sclerosis (MS). Objective: To describe the clinical and epidemiological profile of demyelinating diseases in children. Methods: Observational descriptive study including all patients less than 18 years of age, who attended the department of Pediatric Neurology, University Hospital of Maracaibo, between January 2014 and March 2015 with the diagnosis of demyelinating disease. Results: 11 patients between 1 and 14 years (64% females) had a diagnosis of demyelinating diseases. The most common condition was EMAD (64%). The predominant symptoms were altered state of consciousness, type drowsiness (55%). A peak in frequency occurred during February for the age group of adolescents (37%). All patients had images suggestive of white matter injury. Conclusion: Demyelinating diseases are a reality in pediatrics, with EMAD as the most frequent entity. Because of the clinical variety of these pathologies, an adequate knowledge of their presentation is necessary in order to prescribe timely and appropriate treatment.

Las evoluciones atípicas de la epilepsia rolándica son complicaciones predecibles / The atypical developments of rolandic epilepsy are predictable complications

Introducción. Las evoluciones atípicas de la epilepsia rolándica son parte de un espectro clínico de fenotipos variables, idiopáticos, dependientes de la edad y con una predisposición genéticamente determinada. Objetivo. Estudiar las características electroclínicas sugestivas de una evolución atípica en la epilepsia rolándica. Pacientes y métodos. Se realizó una búsqueda retrospectiva de 133 niños diagnosticados de epilepsia focal benigna atípica (EFBA), síndrome de Landau-Kleffner y epilepsia de punta-onda continua durante el sueño (POCS). Se seleccionaron nueve pacientes que, en el trascurso de su epilepsia rolándica, presentaron un cuadro clínico atípico y un patrón electroencefalográfico (EEG) de estado epiléptico eléctrico durante el sueño (ESES). Resultados. El inicio de la epilepsia rolándica fue, en promedio, a los 5 años. Los pacientes presentaron un empeoramiento clínico y del EEG año y medio más tarde en promedio. En tres pacientes se observaron características de EFBA, y en seis, de POCS. No se encontraron casos de síndrome de Landau-Kleffner. El EEG en vigilia mostró una focalidad centrotemporal izquierda en seis pacientes, y derecha, en tres. Todos los pacientes presentaron un ESES en el EEG de sueño. En tres de ellos se observó un patrón atípico de ESES regional. Además, se detectaron alteraciones cognitivas y conductuales por déficits en áreas específicas del aprendizaje, como lenguaje, memoria, atención e inquietud. Conclusiones. El inicio precoz de la epilepsia rolándica, la aparición de nuevas crisis con un incremento en su frecuencia y una focalidad frontocentrotemporal en el EEG, que aumenta en frecuencia, tanto en vigilia como en sueño, son características electroclínicas sugerentes de una evolución atípica (AU)

Introduction. The development of atypical features in rolandic epilepsy is part of a clinical spectrum of phenotypes that are variable, idiopathic and age-dependent, as well as having a genetically determined predisposition. Aim. To study the electroclinical characteristics suggesting an atypical development in rolandic epilepsy Patients and methods. A retrospective search was performed in 133 children diagnosed with atypical benign focal epilepsy (ABFE), Landau-Kleffner syndrome and continuous spike-wave during sleep (CSWS). Nine patients were selected, all of whom presented atypical clinical features and an electroencephalogram (EEG) pattern of electrical status epilepticus during sleep (ESES) in the course of their rolandic epilepsy. Results. The average age at onset of rolandic epilepsy was 5 years. Patients showed a deterioration of both their clinical features and their EEG recording one and a half years later, on average. ABFE was observed in three of them and CSWS in six. No cases of Landau-Kleffner syndrome were found. The EEG in wakefulness showed the focus to be in the left centrotemporal region in six patients and in three of them it was on the right-hand side. All the patients presented ESES in the EEG during sleep. An atypical pattern was observed in the regional ESES in three of the patients. Moreover, cognitive and behavioural disorders were detected due to deficits in specific learning areas, such as language, memory, attention and restlessness. Conclusions. The early onset of rolandic epilepsy, the appearance of new seizures with an increased frequency and the frontocentrotemporal focus in the EEG, which increases in frequency, both in wakefulness and in sleep, are all electroclinical characteristics of an atypical development (AU)

[The atypical developments of rolandic epilepsy are predictable complications]. / Las evoluciones atipicas de la epilepsia rolandica son complicaciones predecibles.

INTRODUCTION: The development of atypical features in rolandic epilepsy is part of a clinical spectrum of phenotypes that are variable, idiopathic and age-dependent, as well as having a genetically determined predisposition. AIM: To study the electroclinical characteristics suggesting an atypical development in rolandic epilepsy. PATIENTS AND METHODS: A retrospective search was performed in 133 children diagnosed with atypical benign focal epilepsy (ABFE), Landau-Kleffner syndrome and continuous spike-wave during sleep (CSWS). Nine patients were selected, all of whom presented atypical clinical features and an electroencephalogram (EEG) pattern of electrical status epilepticus during sleep (ESES) in the course of their rolandic epilepsy. RESULTS: The average age at onset of rolandic epilepsy was 5 years. Patients showed a deterioration of both their clinical features and their EEG recording one and a half years later, on average. ABFE was observed in three of them and CSWS in six. No cases of Landau-Kleffner syndrome were found. The EEG in wakefulness showed the focus to be in the left centrotemporal region in six patients and in three of them it was on the right-hand side. All the patients presented ESES in the EEG during sleep. An atypical pattern was observed in the regional ESES in three of the patients. Moreover, cognitive and behavioural disorders were detected due to deficits in specific learning areas, such as language, memory, attention and restlessness. CONCLUSIONS: The early onset of rolandic epilepsy, the appearance of new seizures with an increased frequency and the frontocentrotemporal focus in the EEG, which increases in frequency, both in wakefulness and in sleep, are all electroclinical characteristics of an atypical development.

TITLE: Las evoluciones atipicas de la epilepsia rolandica son complicaciones predecibles.Introduccion. Las evoluciones atipicas de la epilepsia rolandica son parte de un espectro clinico de fenotipos variables, idiopaticos, dependientes de la edad y con una predisposicion geneticamente determinada. Objetivo. Estudiar las caracteristicas electroclinicas sugestivas de una evolucion atipica en la epilepsia rolandica. Pacientes y metodos. Se realizo una busqueda retrospectiva de 133 niños diagnosticados de epilepsia focal benigna atipica (EFBA), sindrome de Landau-Kleffner y epilepsia de punta-onda continua durante el sueño (POCS). Se seleccionaron nueve pacientes que, en el trascurso de su epilepsia rolandica, presentaron un cuadro clinico atipico y un patron electroencefalografico (EEG) de estado epileptico electrico durante el sueño (ESES). Resultados. El inicio de la epilepsia rolandica fue, en promedio, a los 5 años. Los pacientes presentaron un empeoramiento clinico y del EEG año y medio mas tarde en promedio. En tres pacientes se observaron caracteristicas de EFBA, y en seis, de POCS. No se encontraron casos de sindrome de Landau-Kleffner. El EEG en vigilia mostro una focalidad centrotemporal izquierda en seis pacientes, y derecha, en tres. Todos los pacientes presentaron un ESES en el EEG de sueño. En tres de ellos se observo un patron atipico de ESES regional. Ademas, se detectaron alteraciones cognitivas y conductuales por deficits en areas especificas del aprendizaje, como lenguaje, memoria, atencion e inquietud. Conclusiones. El inicio precoz de la epilepsia rolandica, la aparicion de nuevas crisis con un incremento en su frecuencia y una focalidad frontocentrotemporal en el EEG, que aumenta en frecuencia, tanto en vigilia como en sueño, son caracteristicas electroclinicas sugerentes de una evolucion atipica.

Dois pares de irmãos com artrite idiopática juvenil (AIJ): relato de casos / Two pairs of brothers with juvenile idiopathic arthritis (JIA): case reports

Relato de casos de ocorrência de Artrite Idiopática Juvenil (AIJ) em dois pares de irmãos acompanhados no serviço de reumatologia pediátrica da Universidade Federal da Bahia. O envolvimento genético na patogênese da AIJ está claro e o risco de recorrência entre irmãos corrobora esta contribuição. Um importante marco dessa descoberta envolve a confirmação da contribuição dos polimorfismos do complexo principal de histocompatibilidade (MHC) na susceptibilidade ao desenvolvimento da AIJ. Apesar de muitos progressos, os inúmeros estudos existentes ainda não são capazes de explicar diversos mecanismos implícitos na patogênese da AIJ.

This is a case report of juvenile idiopathic arthritis in two pairs of brothers followed in the Department of Pediatric Rheumatology, Universidade Federal da Bahia. Genetic involvement in juvenile idiopathic arthritis pathogenesis is clear and the risk of recurrence among siblings supports this contribution. An important landmark of this discovery involves the acknowledgment of major histocompatibility complex polymorphism contribution to juvenile idiopathic arthritis development susceptibility. Despite many advances, the numerousavailable studies cannot explain several implicit mechanisms in juvenile idiopathic arthritispathogenesis yet.

[Two pairs of brothers with juvenile idiopathic arthritis (JIA): case reports]. / Dois pares de irmãos com artrite idiopática juvenil (AIJ): relato de casos.

This is a case report of juvenile idiopathic arthritis (JIA) in two pairs of brothers followed in the department of pediatric rheumatology, Universidade Federal da Bahia. Genetic involvement in JIA pathogenesis is clear and the risk of recurrence among siblings supports this contribution. An important landmark of this discovery involves the acknowledgment of major histocompatibility complex (MHC) polymorphism contribution to JIA development susceptibility. Despite many advances, the numerous available studies cannot explain several implicit mechanisms in JIA pathogenesis yet.

Análisis clínico y experiencia terapéutica de la corea en pediatría: Hospital Universitario de Maracaibo / Clinical analysis and therapeutic experience in childhood chorea: Hospital Universitario de Maracaibo

Las causas más frecuentes de corea adquirida en la infancia son la reumática y la lúpica, siendo muchas veces un diagnóstico de exclusión frente a muchas posibles enfermedades OBJETIVO: Conocer las características clínicas, y la evolución posterior al tratamiento de niños con corea. MÉTODOS: Estudio observacional, descriptivo, tipo serie de casos que incluyó 9 niños entre 0 y 15 años con diagnóstico de corea, que consultaron al servicio de Neuropediatría del Hospital Universitario de Maracaibo entre Enero 2009 y Enero 2013. RESULTADOS: La corea predominó en niñas (proporción 2:1), restringidas al grupo de escolares y adolescentes, 44% corea reumática, 22% puras, 11% lúpicas, 11% asociadas a parálisis cerebral infantil coreo- atetósica y un caso 11% de enfermedad de Huntington infantil. Todos los pacientes presentaron manifestaciones psiquiátricas en grado leve a moderado, 44% pacientes recibieron valproato, 22% carbamazepina en monoterapia, el resto tratamiento combinado 22% valproato y carbamazepina, 22% carbamazepina y haloperidol; la duración del tratamiento entre 3 y 14 meses, con evolución favorable en la mayoría de los casos. CONCLUSIONES: La presentación clínica de los casos no difiere de los documentados en la literatura. La corea reumática es la más frecuente siendo precedida por infección faríngea; en forma general responden bien al tratamiento convencional siendo más eficiente el valproato con menos efectos secundarios en monoterapia o combinado. La enfermedad de Huntington es extremadamente infrecuente en forma general y aún más en la infancia, sin embargo, el estado Zulia es una zona endémica de la patología por lo que debe considerarse ésta posibilidad diagnóstica.

The most common causes of acquired chorea in childhood are rheumatic pathologies and systemic lupus eritematosus, often being a diagnosis of exclusion against many possible diseases. OBJECTIVE: To determine clinical characteristics and post-treatment evolution of children with chorea. METHODS: An observational, descriptive study, case series type, which includes 9 children between 0 and 15 years with chorea, which attended the department of pediatric neurology at the University Hospital in Maracaibo between January 2009 and January 2013. RESULTS: Chorea predominated in girls (ratio 2; 1), restricted to the group of schoolchildren and adolescents, 44 % rheumatic chorea, 22 % pure, 11% lupic, 11% associated with choreo-athetoid cerebral palsy, in one case (11%) Huntington's disease; all presented mild to moderate psychiatric manifestations, 44 % patients received valproate or carbamazepine: 22 % as monotherapy, the rest (22 %) as combination therapy, 22 % carbamazepine and haloperidol; the duration of the treatment was between 3 and 14 months with a very favorable response in most cases. CONCLUSIONS: The clinical presentation of the cases is not different to those documented in the literature. Rheumatic chorea is the most frequent, preceded by pharyngeal infection. It generally responds well to conventional treatment. The most efficient and with fewer side effects drug is valproate. Huntington's disease is extremely rare in general and even more in childhood; however, the state of Zulia is an endemic area for this disease so this diagnostic possibility should be considered.

Epilepsia y enfermedades neurocutáneas: Abordaje en neuropediatría / Epilepsy and neurocutaneous diseases. Approach in neuropediatrícs

Introducción: La epilepsia es generalmente la primera manifestación que obliga a la consulta con neurología pediátrica pasando por alto las lesiones de la piel encontradas en la mayoría de las Enfermedades Neurocutáneas (EN), las cuales son trastornos determinados genéticamente que afectan selectivamente a órganos y tejidos derivados del ectodermo embrionario. Objetivo: Conocer las características de la epilepsia en los pacientes pediátricos con EN que asisten a la consulta de neuropediatría del Hospital Universitario de Maracaibo entre 2010 y 2013 Método: estudio observacional tipo serie de casos Resultados: Veintiún pacientes cumplieron los criterios de EN, 17 presentaron crisis epilépticas, 9 niñas y 8 niños. La Esclerosis Tuberosa (ET) fue la causa más frecuente seguida del síndrome de Sturge Weber. Se encontró significación estadística al correlacionar el inicio precoz de las crisis convulsivas antes de los 24 meses de edad y el diagnóstico de ET. Las crisis focales fueron más frecuentes que las generalizadas. El valproato y la oxcarbazepina en monoterapia o combinados son los medicamentos de elección. El retardo mental fue la comorbilidad más frecuentemente encontrada. Conclusiones: La ET fue la principal EN asociada a epilepsia con un mayor riesgo de inicio de crisis antes de los 24 meses, seguida del Síndrome de Sturge Weber. No existe una asociación significativa entre el sexo, el grupo etario y el riesgo de epilepsia. La severidad variable de las crisis amerita en muchos casos politerapia farmacológica para su control.

Epilepsy is usually the first sign that requires neurological consultation often overlooking the visible skin lesions so common in most neurocutaneous diseases, genetically determined disorders that selectively affect organs and tissues derived from the embryonic ectoderm Objective: To determine the characteristics of epilepsy in pediatric patients with neurocutaneous diseases attending the neuropaediatric clinic at the University Hospital of Maracaibo between 2010 and 2013 Methods: Observational case series Results: Twenty one patients met the criteria for neurocutaneous disorders. Epilepsy was seen in 17 patients, 9 were female and 8 were male. Tuberous sclerosis was the most frequent cause, followed by Sturge Weber syndrome. Statistical significance was found for the association of early onset epileptic crisis before 24 months of age and tuberous sclerosis. Focal seizures were more frequent than generalized seizures. Valproate and oxcarbazepine as monotherapy or in combination are the drugs of choice. Mental retardation was the most frequent comorbidity found Conclusions: Tuberous sclerosis was the major neurocutaneous disease associated with epilepsy with an increased risk of onset of crisis before 24 months of age, followed by Sturge Weber syndrome. There is no significant association between gender and age group and risk of epilepsy. Variable severity of epileptic crises requires polytherapy for adequate control in many cases.

[Deployment analysis of the Brazilian Breastfeeding Network: challenges and prospects for promoting breastfeeding in primary care]. / Análise de implantação da Rede Amamenta Brasil: desafios e perspectivas da promoção do aleitamento materno na atenção básica.

The article evaluates the implementation of the Brazilian Breastfeeding Network, a strategy developed by the Ministry of Health to encourage breastfeeding in primary health care. We performed an evaluation study on the implementation analysis of component 1, considering three levels (Federal, State, and Municipal). A log-frame model and matrix of indicators for the Breastfeeding Network were developed. To understand the organizational context, applying the categories analyzed by Matus Triangles and the degree of implementation of the Breastfeeding Network, we used document analysis, interviews, and focus groups. The degree of implementation was obtained by assessing the proposed activities at all three levels; municipalities were evaluated according to Ministry of Health criteria for certification of primary health care services. We identified different organizational contexts and different degrees of deployment, ranging from 18 to 100%. The study showed the influence of context on the deployment strategy, such that more favorable contexts were associated with more advanced levels of deployment.

Análise de implantação da Rede Amamenta Brasil: desafios e perspectivas da promoção do aleitamento materno na atenção básica / Deployment analysis of the Brazilian Breastfeeding Network: challenges and prospects for promoting breastfeeding in primary care / Análisis sobre la implementación de la Red Amamanta Brasil: desafíos y perspectivas de la promoción de la lactancia materna en la atención primaria

Avaliou-se a implantação da Rede Amamenta Brasil, estratégia elaborada pelo Ministério da Saúde para o incentivo à amamentação na atenção básica. Realizou-se pesquisa avaliativa do tipo análise de implantação do componente 1, considerando-se três níveis de análise (federal, estadual e municipal) e adotando-se o estudo de casos múltiplos. Elaboraram-se o modelo lógico e a matriz de indicadores da Rede Amamenta Brasil. Para apreensão do contexto organizacional, analisado por meio das categorias do Triângulo de Matus e do grau de implantação, foram utilizadas a análise documental, entrevistas e grupos focais. O grau de implantação foi obtido pela avaliação do cumprimento das atividades propostas nos três níveis; nos municípios, foram avaliados os critérios estabelecidos pelo Ministério da Saúde para certificação das unidades básicas. Identificaram-se diferentes contextos organizacionais e graus de implantação, variando, nos municípios, de 18% a 100%. Foi possível observar a influência do contexto sobre a implantação da estratégia, de modo que se evidenciou relação entre contextos mais favoráveis e graus de implantação mais avançados.

The article evaluates the implementation of the Brazilian Breastfeeding Network, a strategy developed by the Ministry of Health to encourage breastfeeding in primary health care. We performed an evaluation study on the implementation analysis of component 1, considering three levels (Federal, State, and Municipal). A log-frame model and matrix of indicators for the Breastfeeding Network were developed. To understand the organizational context, applying the categories analyzed by Matus Triangles and the degree of implementation of the Breastfeeding Network, we used document analysis, interviews, and focus groups. The degree of implementation was obtained by assessing the proposed activities at all three levels; municipalities were evaluated according to Ministry of Health criteria for certification of primary health care services. We identified different organizational contexts and different degrees of deployment, ranging from 18 to 100%. The study showed the influence of context on the deployment strategy, such that more favorable contexts were associated with more advanced levels of deployment.

Se evaluó la implementación de la Red de Lactancia Materna en Brasil, estrategia desarrollada por el Ministerio de Salud para fomentar la lactancia materna en la atención primaria. Se realizó un estudio evaluativo sobre el análisis de la implementación del componente 1, teniendo en cuenta tres niveles (federal, estatal y municipal) y adoptando un estudio de caso múltiple. Hemos desarrollado el modelo lógico y la matriz de indicadores de red de lactancia materna en Brasil. Para comprender el contexto de la organización, las categorías fueron analizadas por el Triángulo de Matus y para el grado de implementación se utilizaron análisis de documentos, entrevistas y grupos focales. El grado de ejecución se obtuvo mediante la evaluación de las actividades propuestas en los tres niveles y los municipios fueron evaluados por los criterios de certificación de unidades básicas del Ministerio de Salud. Se identificaron contextos organizacionales y diferentes grados de implantación en las ciudades que van de 18 a un 100%. Se puede observar la influencia del contexto en la estrategia de implementación.

Análise do desenvolvimento da empatia aos dois anos de idade: contexto de criação e presença de depressão pós-parto / Analysis of the development of empathy at two years of age: Context creation and presence of Postpartum Depression

A empatia consiste em compartilhar uma emoção percebida de outra pessoa, e, em certa medida, sentir a mesma emoção que a outra pessoa está sentindo (Eisenberg e Strayer; 1992). Aos dois anos, as crianças têm a capacidade de atribuir significado emocional às expressões dos outros, além de responder a estas expressões de modo adequado; verifica-se neste contexto de compartilhamentos emocionais precoces o aparecimento de comportamentos de consolo e de ajuda, ainda que de maneira incipiente (Harris, 1996; Strayer, 1993). O presente estudo faz parte do Projeto Temático (FAPESP) Depressão Pós-Parto (DPP) como fator de risco no desenvolvimento: Estudo interdisciplinar dos fatores envolvidos na gênese do quadro e em suas consequências, que acompanha díades mãe-bebê da gestação ao terceiro ano da criança. Dentro deste projeto, este estudo objetivou avaliar relações entre variáveis do contexto socioafetivo da criança sobre o desenvolvimento da empatia na criança, aos dois anos de idade. Recebeu atenção especial a presença ou não de indicadores de DPP, bem como de outros fatores contextuais associados ao sexo da criança, apoio social, ao fato de a criança ter outros cuidadores, frequentar a creche, desfrutar da presença do pai, considerando-se também as percepções da mãe quanto ao temperamento de suas crianças, a impaciência e a satisfação em ser mãe. Métodos: A amostra foi composta por 69 díades (mãe-bebê) com a presença ou não de indicadores de DPP. Esta foi avaliada no puerpério, aos 8 meses e aos 24 meses, pela Escala de Depressão Pós-parto de Edimburgh (EDPE). Para analisar o desenvolvimento da empatia, utilizou-se aos 24 meses das crianças o teste do Teddy Bear, segundo protocolo desenvolvido por Bischof Kohler (1991), que visa avaliar ações e emoções indicativas de empatia. As reações apresentadas pelas crianças foram analisadas quanto à presença de indicadores de empatia, conforme protocolo do teste.(AU)

Empathy is to share a perceived emotion of another person, and to some extent, feel the same emotion that the other person is feeling (Eisenberg e Strayer; 1992). At two years the children have the ability to assign meaning to the emotional expressions of others, and respond to these expressions appropriately: there is in this context shares the early emergence of emotional behaviors consolation and help, albeit in a incipient (Harris, 1996; Strayer, 1993). This study is part of the Thematic Project (FAPESP) "Postpartum Depression (DPP) as a risk factor in development: Interdisciplinary study of the factors involved in the genesis of the framework and its consequences ", accompanying mother-infant dyads from pregnancy to the child's third year. Within this project, this study aimed to evaluate relationships between variables in the context of socio-affective child on the development of empathy in children at two years of age. Received special attention to the presence or absence of indicators of DPP, as well as other contextual factors associated with the sex of the child, social support, the fact that the child has other caregivers, attending daycare, enjoy the presence of the father, considering also the mother's perceptions regarding the temperament of your children, impatience and satisfaction in being a mother. Methods: The sample consisted of 69 dyads (mother-baby) with the presence or absence of indicators of postpartum depression; this was evaluated postpartum, 8 months and 24 months, the Depression Scale of Postpartum Edimburgh (EDPE). To analyze the development of empathy, we used 24 months of testing children's "Teddy Bear", a protocol developed by Bischof - Kohler (1991), which aims to evaluate actions and emotions indicative of empathy. The reactions presented by the children were analyzed for the presence of indicators of empathy as the test protocol.(AU)

Amamentação em presídio: estudo das condições e práticas no Estado de São Paulo, Brasil / Breast feeding in prison: a study about its conditions and practices in the State of São Paulo, Brazil

Este artigo tem o objetivo de analisar o processo de amamentação em presídio feminino no Estado do São Paulo (Brasil). Para tanto, foi feita uma pesquisa qualitativa do tipo exploratório-analítica. O estudo ocorreu no Centro de Atendimento Hospitalar à Mulher Presa, na cidade de São Paulo. Entrevistaram-se vinte mães em amamentação exclusiva e três profissionais envolvidos em práticas de promoção da amamentação. Observadas as questões éticas, gravaram-se entrevistas semiestruturadas que, transcritas, foram analisadas empregando-se a análise de conteúdo temática. Como resultados, as mães apontaram: o valor do leite materno como fonte de vitaminas e proteção contra doenças; a importância do vínculo criado durante a amamentação; e o sofrimento causado pela separação entre a mãe e o bebê, já que as mulheres permanecem com seus filhos por apenas quatro meses. Os profissionais focaram: a importância da amamentação para a criança nos primeiros meses de vida; o fato de não estarem preparados para receber e abrigar mães que amamentam; e a relevância das pesquisas científicas, como estímulo para outros profissionais no aprimoramento da qualidade deste serviço. Conclui-se que a amamentação foi vista como relevante, ressaltando que toda criança tem o direito à proteção, à vida e à saúde. Identificou-se que a amamentação em presídios femininos é realidade, embora haja impedimentos de se cumprir o que está previsto pela legislação, tornando-se necessária a criação de disposições que possam garantir efetivamente a prática de amamentação em presídios femininos.