Early Identification and Treatment of Trochlear Knee Dysplasia.

A shallow sulcus characterizes trochlear dysplasia (TD) of the femoral trochlea, which can lead to chronic pain or instability of the patellofemoral joint. Breech presentation at birth has been identified as a risk factor for developing this condition, which an ultrasound can identify early. Early treatment could be considered at this stage, given the potential for remodelling in these skeletally immature patients. Newborns with breech presentation at birth who meet the inclusion criteria will be enrolled and randomised in equal proportions between treatment with the Pavlik harness and observation. The primary objective is to determine the difference in the means of the sulcus angle between the two treatment arms at two months. Ours is the first study protocol to evaluate an early non-invasive treatment for TD in the newborn with breech presentation at birth using a Pavlik harness. We hypothesised that trochlear dysplasia could be reverted when identified and treated early in life with a simple harness, as it is done with developmental dysplasia of the hip.

Coledocolitiasis con resolución espontánea en lactante / Choledocholithiasis with spontaneous resolution in infants

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Aproximación a la validación de un juguete inteligente como apoyo en la detección precoz de problemas del desarrollo / Approach to the validation of a smart toy to support the early detection of developmental problems

Este artículo presenta un acercamiento a la validación de un juguete inteligente (desarrollado en el marco del proyecto EDUCERE), que aproxime a la detección no invasiva de trastornos de desarrollo del niño. El juguete consiste en una torre de 5 cubos en los que se han insertado sensores para medir una serie de parámetros. Para la recogida de datos, se contó con una muestra de 68 niños entre 24 y 48 meses. Además, a una submuestra de 27 niños se aplicó la escala de desarrollo Merrill Palmer-R. Los resultados parecen mostrar que el nº de cubos utilizados en la construcción de la torre, el nº de movimientos que realizan y la velocidad y aceleración de los movimientos son las variables más relevantes en la elaboración de la torre. El nivel de ejecución de la torre, evaluada a través de los sensores, se relaciona con diversas áreas del desarrollo infantil; siendo la motricidad gruesa el área de desarrollo que más diferencia entre los niños que construyeron bien la torre y los que la hicieron mal. Estos resultados apoyan la adecuación de los cubos inteligentes y sirven para futuras investigaciones que validen de forma consistente su utilidad para la detección de problemas de desarrollo.

This article is an approach to the validation of a smart toy (developed within the framework of a larger project called EDUCERE), with the aim of detecting developmental disorders in children, in an early, non-invasive manner. The toy consists of a tower of 5 cubes in which sensors have been inserted to measure a series of parameters. The sample was made up of 68 children between the ages of 24 and 48 months. The Merrill Palmer-R Scales of Development were applied to a sub-sample of 27 children. The results seem to show that the number of cubes used by the children in the construction of a tower, the number of movements they perform, as well as the speed and acceleration of their movements, are the most relevant variables in the construction of the tower. The execution level in the task of building of the tower ­measured by the sensors- is related to various areas of child development, such as gross and fine motor skills, social emotional and cognitive aspects, and the general intelligence level. Of these, gross motor skills are the area of development that differentiates more the children who built the tower correctly from those who did not. These results support the use of the smart cubes and serve as a precedent for future researches that aim to validate consistently their use in the detection of developmental problems

Enfermedad de Hirayama en pediatría: aportación de un caso clínico y revisión de la bibliografía / Hirayama disease in paediatrics: a clinical case report and review of the literature

Introducción. La enfermedad de Hirayama es una rara atrofia muscular juvenil que afecta a varones jóvenes de origen asiático, con atrofia muscular habitualmente de una de las extremidades superiores de progresión lenta con estabilización posterior. Se diagnostica por estudios electromiográficos/electroneurográficos con velocidad de conducción (EMG/ENG-VC), y por resonancia magnética (RM) medular en posición neutra y en flexión cervical. El tratamiento se basa en el collarín cervical y cirugía (casos graves). Son muy pocos los estudios realizados en edad pediátrica. Caso clínico. Niña de 7 años, con atrofia de la musculatura de la mano y el antebrazo izquierdos, de dos años de evolución. En EMG/ENG-VC presenta signos de denervación crónica muy grave en los miótomos correspondientes a C7, C8 y D1 izquierdos, con conservación de amplitudes de potenciales sensitivos evocados, congruentes con mielopatía cervical. La RM medular cervical en posición neutra muestra un resultado normal en ese nivel. Posteriormente, por la sospecha dirigida de enfermedad de Hirayama, se realiza una nueva RM medular cervical en posición neutra y en flexión, que muestra asimetría en el tamaño y morfología de los cordones anteriores medulares en C6/C7, hiperseñal en el asta anterior homolateral e ingurgitación del plexo venoso epidural posterior. Con el diagnóstico de enfermedad de Hirayama se inicia tratamiento con collarín cervical para evitar la progresión del daño. Conclusiones. Se presenta un caso de enfermedad de Hirayama peculiar por las características epidemiológicas, con la finalidad de difundir esta entidad en nuestro medio, cuyo diagnóstico precoz permite un tratamiento eficaz, y se revisan los estudios realizados en edad pediátrica (AU)

Introduction. Hirayama disease is a rare children’s muscular atrophy that affects young Asian males, with muscular atrophy usually in one of the upper limbs that progresses slowly and later stabilises. It is diagnosed by means of electromyographic/ electroneurographic with conduction speed studies (EMG/ENG-CS) and by magnetic resonance imaging (MRI) of the spinal cord in a neutral position and with cervical flexion. Treatment is based on the cervical collar and surgery (severe cases). Very few studies have been conducted on patients at the paediatric age. Case report. We report the case of a 7-year-old girl with atrophy of the muscles of the left hand and forearm, and a disease history of two years. The EMG/ENG-CS scans presented signs of very severe chronic denervation in the myotomes of C7, C8 and T1 on the left side, with conservation of the amplitudes of sensory evoked potentials, consistent with cervical myelopathy. Results of an MRI scan of the cervical spinal cord in a neutral position were normal at that level. Later, owing to suspicions pointing towards Hirayama disease, a new MRI scan of the cervical spinal cord was performed in a neutral position and in flexion. This second scan showed asymmetry in the size and morphology of the anterior funiculi of the spinal cord at C6/C7, hypersignal in the homolateral anterior horn and ingurgitation of the posterior epidural venous plexus. With a diagnosis of Hirayama disease, treatment is started with a cervical collar in order to prevent the damage from getting worse. Conclusions. This case of Hirayama disease is peculiar due to its epidemiological characteristics and is presented here with the aim of making this entity more widely known in our milieu. If diagnosed at an early stage, treatment is effective, and the studies conducted on children at the paediatric age are reviewed (AU)

[Hirayama disease in paediatrics: a clinical case report and review of the literature]. / Enfermedad de Hirayama en pediatría: aportación de un caso clínico y revisión de la bibliografía.

INTRODUCTION: Hirayama disease is a rare children's muscular atrophy that affects young Asian males, with muscular atrophy usually in one of the upper limbs that progresses slowly and later stabilises. It is diagnosed by means of electromyographic/electroneurographic with conduction speed studies (EMG/ENG-CS) and by magnetic resonance imaging (MRI) of the spinal cord in a neutral position and with cervical flexion. Treatment is based on the cervical collar and surgery (severe cases). Very few studies have been conducted on patients at the paediatric age. CASE REPORT: We report the case of a 7-year-old girl with atrophy of the muscles of the left hand and forearm, and a disease history of two years. The EMG/ENG-CS scans presented signs of very severe chronic denervation in the myotomes of C7, C8 and T1 on the left side, with conservation of the amplitudes of sensory evoked potentials, consistent with cervical myelopathy. Results of an MRI scan of the cervical spinal cord in a neutral position were normal at that level. Later, owing to suspicions pointing towards Hirayama disease, a new MRI scan of the cervical spinal cord was performed in a neutral position and in flexion. This second scan showed asymmetry in the size and morphology of the anterior funiculi of the spinal cord at C6/C7, hypersignal in the homolateral anterior horn and ingurgitation of the posterior epidural venous plexus. With a diagnosis of Hirayama disease, treatment is started with a cervical collar in order to prevent the damage from getting worse. CONCLUSIONS: This case of Hirayama disease is peculiar due to its epidemiological characteristics and is presented here with the aim of making this entity more widely known in our milieu. If diagnosed at an early stage, treatment is effective, and the studies conducted on children at the paediatric age are reviewed.

TITLE: Enfermedad de Hirayama en pediatria: aportacion de un caso clinico y revision de la bibliografia.Introduccion. La enfermedad de Hirayama es una rara atrofia muscular juvenil que afecta a varones jovenes de origen asiatico, con atrofia muscular habitualmente de una de las extremidades superiores de progresion lenta con estabilizacion posterior. Se diagnostica por estudios electromiograficos/electroneurograficos con velocidad de conduccion (EMG/ENG-VC), y por resonancia magnetica (RM) medular en posicion neutra y en flexion cervical. El tratamiento se basa en el collarin cervical y cirugia (casos graves). Son muy pocos los estudios realizados en edad pediatrica. Caso clinico. Niña de 7 años, con atrofia de la musculatura de la mano y el antebrazo izquierdos, de dos años de evolucion. En EMG/ENG-VC presenta signos de denervacion cronica muy grave en los miotomos correspondientes a C7, C8 y D1 izquierdos, con conservacion de amplitudes de potenciales sensitivos evocados, congruentes con mielopatia cervical. La RM medular cervical en posicion neutra muestra un resultado normal en ese nivel. Posteriormente, por la sospecha dirigida de enfermedad de Hirayama, se realiza una nueva RM medular cervical en posicion neutra y en flexion, que muestra asimetria en el tamaño y morfologia de los cordones anteriores medulares en C6/C7, hiperseñal en el asta anterior homolateral e ingurgitacion del plexo venoso epidural posterior. Con el diagnostico de enfermedad de Hirayama se inicia tratamiento con collarin cervical para evitar la progresion del daño. Conclusiones. Se presenta un caso de enfermedad de Hirayama peculiar por las caracteristicas epidemiologicas, con la finalidad de difundir esta entidad en nuestro medio, cuyo diagnostico precoz permite un tratamiento eficaz, y se revisan los estudios realizados en edad pediatrica.