[Interindividual variability in patients with visuospatial neglect: a retrospective study]. / Variabilidad interindividual en pacientes con heminegligencia visuoespacial: estudio retrospectivo.

INTRODUCTION: Patients who have suffered a stroke may present with visuospatial neglect (VSN). In clinical practice, different degrees of impairment can be observed among patients with VSN; however, there is no consensus regarding the criteria and tests used to determine them. AIM: This study aims to classify patients with VSH based on their level of impairment and to study their response to computerized cognitive training. PATIENTS AND METHODS: The sample consisted of 34 patients (19 men and 15 women) with a mean age of 47.59 ± 8.39 years. All patients underwent a neuropsychological exploration protocol composed of specific tests that assess visuospatial attention and others to evaluate multiple cognitive domains. All participants underwent computerized cognitive training consisting of 15 one-hour sessions. RESULTS: A cluster analysis was performed that divided the sample into three groups: group 1: mildly affected VSN (n = 17), group 2: moderately affected VSN (n = 11), and group 3: severely affected VSN (n = 6). Statistically significant differences were found in all tests of the visuospatial attention protocol, both in the pre-treatment and post-treatment evaluation. CONCLUSIONS: There are different levels of impairment among patients with VSN, differences that persist after applying computerized cognitive training. These results suggest that the evolution of VSN follows a homogeneous pattern linked to the initial level of impairment. These findings, although preliminary, may be relevant to neurorehabilitation professionals.

TITLE: Variabilidad interindividual en pacientes con heminegligencia visuoespacial: estudio retrospectivo.Introducción. Los pacientes que han sufrido un ictus pueden presentar heminegligencia visuoespacial (HVE). En la práctica clínica es posible observar diferentes grados de afectación entre los pacientes con HVE; sin embargo, no existe consenso respecto a los criterios y pruebas utilizadas para determinarlos. Objetivo. Este estudio tiene como objetivo clasificar a los pacientes que presentan HVE en función de su nivel de afectación y estudiar su respuesta a un entrenamiento cognitivo computarizado. Pacientes y métodos. La muestra estaba formada por 34 pacientes (19 hombres y 15 mujeres) con una edad media de 47,59 ± 8,39 años. A todos los pacientes se les administró un protocolo de exploración neuropsicológico compuesto por pruebas específicas que evalúan la atención visuoespacial y otro para evaluar múltiples dominios cognitivos. Todos los participantes realizaron un entrenamiento cognitivo computarizado consistente en 15 sesiones de una hora de duración. Resultados. Se realizó un análisis de conglomerados que dividió la muestra en tres grupos: grupo 1, HVE con afectación leve (n = 17); grupo 2, HVE con afectación moderada (n = 11); y grupo 3, HVE con afectación grave (n = 6). Se obtuvieron diferencias estadísticamente significativas en todas las pruebas del protocolo de atención visuoespacial, tanto en la evaluación pretratamiento como en la postratamiento. Conclusiones. Existen diferentes niveles de afectación entre los pacientes con HVE, diferencias que se mantienen después de aplicar un entrenamiento cognitivo computarizado. Estos resultados sugieren que la evolución de la HVE sigue un patrón homogéneo vinculado al nivel de afectación inicial. Estos hallazgos, aunque preliminares, pueden ser relevantes para los profesionales de la neurorrehabilitación.

Computerized social cognitive training in the subacute phase after traumatic brain injury: A quasi-randomized controlled trial.

OBJECTIVES: To assess the usefulness of a computerized tasks module designed for the rehabilitation of social cognition (SC) in acquired brain injury. METHODS: Quasi-randomized controlled trial (ClinicalTrials.gov:NCT03479970) involving 45 patients with moderate-severe traumatic brain injury (TBI) in a subacute inpatient rehabilitation hospital. The experimental group (n = 28) received treatment with a computerized SC module in combination with a non-SC module. The control group (n = 26) only received a treatment with non-SC module. RESULTS: Intragroup comparisons showed that the experimental group had better results for all SC measures, except for International Affective Picture System (IAPS). The control group improved for Facial Expressions of cEmotion-Stimuli and Tests (FEEST) and Moving Shapes Paradigm (MSP), showing no changes with respect to pretreatment in IAPS, MSP and Reading the Mind in the Eyes Test (RMET). Intergroup comparisons did not present differences between the two groups for pretreatment measures. Post-treatment comparison showed that the experimental group obtained better results for RMET than the control group. CONCLUSION: The computerized SC module was useful for the rehabilitation of SC in patients with moderate-severe TBI in the subacute phase. The group that received combined rehabilitative treatment (SC + non-SC) obtained better results for SC than the group that received treatment intended only for non-SC.

Social and nonsocial cognition: Are they linked? A study on patients with moderate-to-severe traumatic brain injury.

OBJECTIVES: The first aim was to study the relationship between Social Cognition (SC) and nonsocial Cognition (n-SC) measures in a group of patients with moderate or severe traumatic brain injury (TBI) to assess the dependence or independence of both types of cognition. The second aim was to explore the relationships between SC measures and generate a model based on the results of these relationships. METHODS AND PROCEDURES: Forty-three subacute patients with TBI were included in the study. They were administered a SC battery and n-SC battery. SC battery included the following measures: International Affective Picture System (IAPS); Facial Expressions of Emotion-Stimuli Test (FEEST); Moving Shapes Paradigm (MSP); Reading the Mind in the Eyes Test- Revised Version (RMET); Social Decision Making Task (SDMT). n-SC battery included Digit Span Forwards and Backwards; Trail Making Test (Part A); Rey's Auditory Verbal Learning Test; Letter-Number Sequencing; and verbal fluency test (PMR). RESULTS: FEEST, MSP and RMET were related to n-SC measures. The exploratory factor analysis shows a two-factor SC structure: Factor 1: Emotional recognition and mentalization (FEEST, MSP and RMET) and Factor 2: Acquisition and contextualization (IAPS and SDMT). CONCLUSION: The performance of subjects with moderate-to-severe TBI in the SC measures is related, at least partially, by the performance in the n-SC measures. Our SC model shows a two-factor structure characterized by a first factor that brings together SC measures that are highly related to n-SC domains and a second factor that brings together measures whose performance is not influenced by n-SC domains.

Complejo esclerosis tuberosa: análisis de los ámbitos de afectación, progreso en el tratamiento y traslación a la práctica clínica habitual en una cohorte de pacientes pediátricos / Tuberous sclerosis complex: analysis of areas of involvement, treatment progress and translation to routine clinical practice in a cohort of paediatric patients

Introducción: El complejo esclerosis tuberosa (CET) presenta gran variabilidad fenotípica. El diagnóstico cada vez más precoz, incluyendo la identificación prenatal, conlleva la necesidad de establecer una sospecha e identificación temprana, por parte del pediatra y del neuropediatra, de factores que pueden influir en su pronóstico y tratamiento. Objetivo: Determinar los criterios clínicos de un diagnóstico precoz, las pruebas complementarias iniciales, las actuaciones y los tratamientos que prevengan diferentes comorbilidades, mejorando el pronóstico de estos pacientes. Pacientes y métodos: Estudio descriptivo, retrospectivo de = 18 años con diagnóstico definitivo de CET en un hospital terciario desde 1998 hasta 2019. Se recogieron variables epidemiológicas, de afectación multisistémica, pruebas complementarias y genética. Resultados: Se analizó a 94 pacientes. Los principales motivos diagnósticos fueron la epilepsia y los rabdomiomas. Se determinó la frecuencia de aparición de los criterios clínicos, y los hallazgos neuropatológicos fueron los principales, seguidos de los estigmas cutáneos, los rabdomiomas y las lesiones renales. Se comprobaron relaciones estadísticas entre aspectos clínicos, radiológicos, genéticos, la influencia de las actividades preventivas sobre la aparición de epilepsia y la relevancia del uso de everolimús. Conclusiones: Los rabdomiomas y los estigmas cutáneos en pacientes y progenitores constituyen signos diagnósticos principales en lactantes. Los túberes y los nódulos subependimarios tienen asociación estadística con el desarrollo de epilepsia. Los espasmos epilépticos en edades precoces, refractarios a tratamiento en los primeros meses, incrementan el riesgo de déficit cognitivo y trastorno del espectro autista...(AU)

Introduction: Tuberous sclerosis complex (TSC) displays great phenotypic variability. Increasingly early diagnosis, including prenatal identification, entails the need for the paediatrician and neuropaediatrician to establish early suspicion and identification of factors that may influence prognosis and treatment. Aim: To determine the clinical criteria for early diagnosis, initial complementary tests, actions and treatments to prevent different comorbidities, so as to improve the prognosis of these patients. Patients and methods: Descriptive, retrospective study of ≤ 18-year-olds with a definitive diagnosis of TSC in a tertiary hospital from 1998 to 2019. We collected variables referring to epidemiological data, multisystem involvement, complementary tests and genetics. Results: Ninety-four patients were analysed. The main diagnostic reasons were epilepsy and rhabdomyomas. The frequency of occurrence of clinical criteria was determined, and neuropathological findings were the main findings, followed by cutaneous stigmata, rhabdomyomas and renal lesions. Statistical relationships were found between clinical, radiological and genetic aspects, the influence of preventive activities on the occurrence of epilepsy and the relevance of everolimus use were tested. Conclusions: Rhabdomyomas and skin stigmata in patients and parents are major diagnostic signs in infants. Tubers and subependymal nodules are statistically associated with the development of epilepsy. Early epileptic spasms, refractory to treatment in the first months, increase the risk of cognitive deficits and autism spectrum disorder. Epileptic abnormalities need to be closely monitored in the first year of life. Everolimus is an alternative treatment for several comorbidities, but its early use (< 3 years) requires further study.(AU)

[Tuberous sclerosis complex: analysis of areas of involvement, treatment progress and translation to routine clinical practice in a cohort of paediatric patients]. / Complejo esclerosis tuberosa: análisis de los ámbitos de afectación, progreso en el tratamiento y traslación a la práctica clínica habitual en una cohorte de pacientes pediátricos.

INTRODUCTION: Tuberous sclerosis complex (TSC) displays great phenotypic variability. Increasingly early diagnosis, including prenatal identification, entails the need for the paediatrician and neuropaediatrician to establish early suspicion and identification of factors that may influence prognosis and treatment. AIM: To determine the clinical criteria for early diagnosis, initial complementary tests, actions and treatments to prevent different comorbidities, so as to improve the prognosis of these patients. PATIENTS AND METHODS: Descriptive, retrospective study of = 18-year-olds with a definitive diagnosis of TSC in a tertiary hospital from 1998 to 2019. We collected variables referring to epidemiological data, multisystem involvement, complementary tests and genetics. RESULTS: Ninety-four patients were analysed. The main diagnostic reasons were epilepsy and rhabdomyomas. The frequency of occurrence of clinical criteria was determined, and neuropathological findings were the main findings, followed by cutaneous stigmata, rhabdomyomas and renal lesions. Statistical relationships were found between clinical, radiological and genetic aspects, the influence of preventive activities on the occurrence of epilepsy and the relevance of everolimus use were tested. CONCLUSIONS: Rhabdomyomas and skin stigmata in patients and parents are major diagnostic signs in infants. Tubers and subependymal nodules are statistically associated with the development of epilepsy. Early epileptic spasms, refractory to treatment in the first months, increase the risk of cognitive deficits and autism spectrum disorder. Epileptic abnormalities need to be closely monitored in the first year of life. Everolimus is an alternative treatment for several comorbidities, but its early use (< 3 years) requires further study.

TITLE: Complejo esclerosis tuberosa: análisis de los ámbitos de afectación, progreso en el tratamiento y traslación a la práctica clínica habitual en una cohorte de pacientes pediátricos.Introducción. El complejo esclerosis tuberosa (CET) presenta gran variabilidad fenotípica. El diagnóstico cada vez más precoz, incluyendo la identificación prenatal, conlleva la necesidad de establecer una sospecha e identificación temprana, por parte del pediatra y del neuropediatra, de factores que pueden influir en su pronóstico y tratamiento. Objetivo. Determinar los criterios clínicos de un diagnóstico precoz, las pruebas complementarias iniciales, las actuaciones y los tratamientos que prevengan diferentes comorbilidades, mejorando el pronóstico de estos pacientes. Pacientes y métodos. Estudio descriptivo, retrospectivo de = 18 años con diagnóstico definitivo de CET en un hospital terciario desde 1998 hasta 2019. Se recogieron variables epidemiológicas, de afectación multisistémica, pruebas complementarias y genética. Resultados. Se analizó a 94 pacientes. Los principales motivos diagnósticos fueron la epilepsia y los rabdomiomas. Se determinó la frecuencia de aparición de los criterios clínicos, y los hallazgos neuropatológicos fueron los principales, seguidos de los estigmas cutáneos, los rabdomiomas y las lesiones renales. Se comprobaron relaciones estadísticas entre aspectos clínicos, radiológicos, genéticos, la influencia de las actividades preventivas sobre la aparición de epilepsia y la relevancia del uso de everolimús. Conclusiones. Los rabdomiomas y los estigmas cutáneos en pacientes y progenitores constituyen signos diagnósticos principales en lactantes. Los túberes y los nódulos subependimarios tienen asociación estadística con el desarrollo de epilepsia. Los espasmos epilépticos en edades precoces, refractarios a tratamiento en los primeros meses, incrementan el riesgo de déficit cognitivo y trastorno del espectro autista. Es necesario monitorizar estrechamente las anomalías epilépticas en el primer año de vida. El everolimús supone una alternativa de tratamiento en varias comorbilidades, pero su uso precoz (menor de 3 años) precisa más estudios.

[Rehabilitation of anosognosia in patients with unilateral visuospatial neglect]. / Rehabilitacion de la anosognosia en pacientes con heminegligencia visuoespacial.

INTRODUCTION: Patients with unilateral visuospatial neglect secondary to a stroke are usually unaware of the fact that their perception and exploration of contralesional space are deficient. This clinical phenomenon, know as anosognosia, directly conditions the rehabilitation process and prolongs its duration to a significant extent, while also making it more difficult for the patient to adhere to it. AIM: To assess the efficacy of a specific rehabilitation programme for the treatment of anosognosia in patients presenting with unilateral visuospatial neglect. PATIENTS AND METHODS: Twelve patients with a stroke in the right hemisphere were divided into two groups. The experimental group received 15 sessions of computerised cognitive therapy along with 15 sessions of specific rehabilitation for anosognosia. The control group underwent 15 sessions of computerised cognitive treatment. All of them were administered, before and after treatment, a battery of tests to evaluate visuospatial attention. The level of functionality was evaluated by means of the Catherine Bergego Scale. RESULTS: After the intervention, the control group showed statistically significant psychometric differences. The same did not occur with the experimental group. No differences were obtained in the pre- and post-treatment intergroup comparisons, or in the psychometric measures or on the functional scale. CONCLUSIONS: Further research is needed to help us improve the treatment of anosognosia in patients with unilateral visuospatial neglect. Some methodological recommendations emerge from the limitations identified in this study.

TITLE: Rehabilitacion de la anosognosia en pacientes con heminegligencia visuoespacial.Introduccion. Habitualmente, el paciente con heminegligencia visuoespacial secundaria a un ictus no es consciente de que su percepcion y exploracion del espacio contralesional son defectuosas. Este fenomeno clinico, conocido como anosognosia, condiciona directamente el proceso rehabilitador y amplia sensiblemente su duracion, al tiempo que dificulta la adhesion del paciente a dicho proceso. Objetivo. Valorar la eficacia de un programa de rehabilitacion especifico para el tratamiento de la anosognosia en pacientes que presentan heminegligencia visuoespacial. Pacientes y metodos. Se distribuyo a 12 pacientes con ictus hemisferico derecho en dos grupos. El grupo experimental recibio 15 sesiones de tratamiento cognitivo informatizado junto con 15 sesiones de rehabilitacion especificas para la anosognosia. El grupo control realizo 15 sesiones de tratamiento cognitivo informatizado. A todos ellos se les administro, antes y despues del tratamiento, una bateria de test para evaluar la atencion visuoespacial. El nivel de funcionalidad se valoro mediante la Catherine Bergego Scale. Resultados. Tras la intervencion, el grupo control mostro diferencias psicometricas estadisticamente significativas. No sucedio lo mismo con el grupo experimental. No se obtuvieron diferencias en las comparaciones intergrupales pre y postratamiento, ni en las medidas psicometricas ni en la escala funcional. Conclusiones. Es necesario seguir realizando investigaciones que nos ayuden a mejorar el tratamiento de la anosognosia en pacientes que presentan heminegligencia visuoespacial. Se plantean algunas recomendaciones metodologicas surgidas de las limitaciones identificadas en el presente estudio.

¿Es posible potenciar la recuperación espontánea después de un ictus? Ensayo controlado aleatorizado con pacientes que presentan heminegligencia espacial / Can spontaneous neurological recovery of visuospatial neglect be maximised in patients with a right hemispheric stroke?

Introducción. Aproximadamente el 25-30% de los pacientes que presentan un ictus muestran signos de heminegligencia espacial. Los objetivos del presente trabajo fueron dos: 1) valorar cómo el tiempo transcurrido entre el ictus y el inicio de tratamiento repercute (o puede llegar a repercutir) en la mejora del paciente y 2) analizar la respuesta de los pacientes a dos tipos de tratamientos (tratamiento único: estimulación cognitiva informatizada vs. tratamiento combinado: estimulación cognitiva informatizada junto con la técnica del right hemifield eye-patching). Material y método. Ensayo clínico aleatorizado realizado con 31 pacientes. Dos grupos experimentales: grupo tratamiento único (n=18) y grupo tratamiento combinado (n=13). Cada grupo de tratamiento fue dividido en función del tiempo transcurrido entre el ictus y el inicio del tratamiento (≤ 12 semanas vs.>12 semanas). Todos ellos recibieron una media de 15 sesiones de rehabilitación cognitiva informatizada de una hora de duración mediante la plataforma de telerrehabilitación Guttmann, NeuroPersonalTrainer®, con la diferencia de que el grupo que recibió el tratamiento combinado ejecutó los ejercicios de rehabilitación con un dispositivo visual que llevaba el hemicampo derecho de cada ojo ocluido. Resultados. Los resultados obtenidos tras aplicar los tratamientos indican cambios a nivel intragrupal en los cuatro grupos; sin embargo, no se obtienen diferencias intergrupales. Conclusiones. Ni el tiempo transcurrido entre el ictus y el inicio del tratamiento, ni recibir un tratamiento combinado respecto a uno único, parecen ser variables concluyentes que influyan en la mejora de estos pacientes (AU)

Introduction. Approximately 25-30% of patients with stroke show signs of visuospatial neglect. The objectives of the present study were two-fold: 1) to assess how the time between stroke and the start of treatment impacts (or can impact) patient improvement, and 2) to analyse patient response to two treatments (single treatment: computerised cognitive stimulation vs. combination treatment: computerised cognitive stimulation with right hemifield eye-patching). Material and method. A randomised clinical trial was conducted in 31 patients. These were grouped into two experimental groups: a single treatment group (n=18) and a combined treatment group (n=13). Each treatment group was divided according to the period that elapsed between the stroke and the start of treatment (≤ 12 weeks vs.>12 weeks). All received an average of 15 one-hour sessions of computerised cognitive rehabilitation using the Guttmann, NeuroPersonalTrainer(R) telerehabilitation platform. Patients in the combined treatment group performed the sessions wearing a visual device that occluded the right hemifield of each eye. Results. The results obtained after application of these treatments indicated changes at the intragroup level in the four groups analysed. However, no between-group differences were found. Conclusions. Neither the time between the stroke and the beginning of treatment nor the type of treatment received are conclusive variables influencing improvement in these patients (AU)

Cyclosporine A for severe atopic dermatitis in children. efficacy and safety in a retrospective study of 63 patients.

BACKGROUND: Cyclosporine A (CSA) is an immunosuppressant agent widely used in severe atopic dermatitis (AD). However, experience in children is limited. OBJECTIVES: To assess the efficacy and adverse events of CSA therapy in children. METHODS: Retrospective study of children with severe AD treated with CSA between January 2009 and December 2015. RESULTS: Data from 63 patients were collected. Mean age at the beginning of treatment was 8.4 years (±3.6). The median starting dose was 4.27 (±0.61) mg/kg/day. After 4 weeks of treatment, the outcome was excellent in 35% of cases, good in 29% and poor in 36% of the patients. The response was better in patients without eosinophilia (P < 0.05). The median duration of treatment was 4.6 months (range 1.5-21.6). Side-effects were frequent but mild, being more common in patients after longer treatment periods (P < 0.05). Mean time of follow-up was 19.4 months (±12.7). Prolonged remission (>6 months) was observed in 13 patients (20%). LIMITS: This is a retrospective review. The follow-up period is limited. CONCLUSIONS: Our data confirm that CSA is efficacious and acts rapidly in the majority of children with severe AD. CSA therapy can provide sustained remission in some patients. CSA seems to be well tolerated in children, but strict monitoring is mandatory.

Heminegligencia espacial secundaria a traumatismo craneoencefálico / Hemispatial neglect secondary to a traumatic brain injury

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[Relationship between executive functioning and behaviour in children with cerebral palsy]. / Relacion entre el funcionamiento ejecutivo y la conducta en niños con paralisis cerebral.

INTRODUCTION: Cerebral palsy is defined as a group of developmental disorders of movement and posture that causes social and cognitive deficits, emotional, and behavior disturbances. AIM: To study the relationship between executive functioning and behavior in children with cerebral palsy from the answers given by parents and teachers on the Behavior Rating Inventory of Executive Function (BRIEF) and on the System Assessment Adaptive Behavior (ABAS-II). PATIENTS AND METHODS: The sample consisted on 46 children with CP with a mean age of 10.26 ± 2.95 years. Forty-four of the 46 children were distributed in Gross Motor Function Classification System (GMFCS) into level I (n = 16), level II (n = 3), level III (n = 11), level IV (n = 10) and level V (n = 4). RESULTS: The results showed a relationship between BRIEF and ABAS-II. Furthermore, discrepancies between the responses from parents and teachers, both in the ABAS-II and in the BRIEF, were obtained. CONCLUSIONS: We found a significant relationship between executive functioning in children with cerebral palsy and adaptive behavior. We found discrepancies in the answers given by parents and teachers. Finally, the data showed that the higher motor impairment increases difficulties at home.

TITLE: Relacion entre el funcionamiento ejecutivo y la conducta en niños con paralisis cerebral.Introduccion. La paralisis cerebral se define como un grupo de trastornos del desarrollo del movimiento y la postura que cursa con deficits cognitivos, alteraciones emocionales, de conducta y sociales. Objetivo. Estudiar la relacion entre el funcionamiento ejecutivo y la conducta en niños con paralisis cerebral a partir de las respuestas aportadas por padres y profesores en el Behavior Rating Inventory of Executive Function (BRIEF) y el sistema de evaluacion de la conducta adaptativa (ABAS-II). Pacientes y metodos. La muestra quedo formada por 46 niños con paralisis cerebral, con una edad media de 10,26 ± 2,95 años. Del total, 44 niños se distribuyeron en el Gross Motor Function Classification System (GMFCS) en nivel I (n = 16), nivel II (n = 3), nivel III (n = 11), nivel IV (n = 10) y nivel V (n = 4). Resultados. Los resultados mostraron relacion entre el BRIEF y el ABAS-II; ademas, se obtuvieron discrepancias entre las respuestas aportadas por padres y profesores, tanto en el ABAS-II como en el BRIEF. Asimismo, se hallo relacion entre el GMFCS y los subindices de vida en el hogar, indice practico y autocuidado del ABAS-II. Conclusiones. Se encontro relacion entre el funcionamiento ejecutivo y la conducta adaptativa en niños con paralisis cerebral. Se hallaron discrepancias en las respuestas aportadas por padres y profesores. Por ultimo, los datos muestran que, a mayor afectacion motora, mayores dificultades en el hogar, en el indice practico y en el autocuidado.

Estancamiento ponderal, vómitos y diarrea. Algo más que patología digestiva / Failure to thrive, vomiting and diarrhea. More than gastrointestinal diseases

Las acidosis tubulares renales son un grupo de trastornos que cursan con acidosis metabólica, en las que la función glomerular permanece conservada y es la función tubular la que está alterada. Son producidas por un defecto en la reabsorción tubular renal de bicarbonato y/o en la excreción urinaria de ion hidrógeno. Su etiología es diversa. Se debe pensar en ellas ante un lactante con escasa ganancia ponderal, vómitos, poliuria y/o deshidratación. El pronóstico es variable, según corresponda a formas primarias o secundarias y a la rapidez con la que se instaure el tratamiento. Presentamos un paciente afecto de acidosis tubular distal primaria diagnosticado a los tres meses de edad, con clínica de estancamiento ponderal (AU)

Renal tubular acidosis is a group of disorders with metabolic acidosis, in which tubular function is impaired while glomerular function is preserved. They are caused by an impairment in the bicarbonate’s renal tubular reabsorption and/or in the urine excretion of hydrogen. The etiology of tubular acidosis is diverse. We should think of it in an infant with vomiting, polyuria, dehydration and poor weight gain. The prognosis is variable, it depends on whether it is a primary or a secondary form and on how prompt the treatment is instituted. We present a patient with primary distal tubular acidosis diagnosed at 3 months of age with poor weight gain (AU)

[Cognitive stimulation in children with cerebral palsy]. / Estimulacion cognitiva en niños con paralisis cerebral.

INTRODUCTION: Cerebral palsy is often accompanied by cognitive impairment affecting attention, visuoperception, executive functions and working memory. AIMS. To analyse the effect of cognitive stimulation treatment on the cognitive capabilities in children with cerebral palsy. PATIENTS AND METHODS: Our sample consisted of 15 children with cerebral palsy, with a mean age of 8.80 ± 2.51 years, who were classified with the aid of the Gross Motor Function Classification System (GMFCS) on level I (n = 6), level II (n = 4), level III (n = 2) and level V (n = 3). Cognitive impairment was evaluated by means of the Wechsler Intelligence Scale for Children (WISC-IV) and the Continuous Performance Test (CPT-II). Both the questionnaires for parents and teachers from the Behavior Rating Inventory of Executive Function (BRIEF) and the Conners rating scales (CPRS-48 and CTRS-28) were administered. A cognitive stimulation programme was carried out at a rate of two hours a week for a total of eight weeks. RESULTS: Statistically significant differences were observed after applying the cognitive stimulation treatment in the perceptive reasoning index of the WISC-IV. No differences were obtained on the Conners' and the BRIEF scores before and after the treatment. Neither were any differences found in the results on the WISC-IV according to sex or on the GMFCS. CONCLUSIONS: The cognitive performance of children with cerebral palsy improves after applying a cognitive rehabilitation programme.

TITLE: Estimulacion cognitiva en niños con paralisis cerebral.Introduccion. La paralisis cerebral a menudo cursa con deficits cognitivos de atencion, visuopercepcion, funciones ejecutivas y memoria de trabajo. Objetivo. Analizar el efecto de un tratamiento de estimulacion cognitiva sobre las capacidades cognitivas en niños con pa­ralisis cerebral. Pacientes y metodos. Muestra de 15 niños con paralisis cerebral, con una edad media de 8,80 ± 2,51 años, clasificados mediante el Gross Motor Function Classification System (GMFCS) en nivel I (n = 6), nivel II (n = 4), nivel III (n = 2) y nivel V (n = 3). Los deficits cognitivos se evaluaron mediante la escala de inteligencia de Wechsler para niños (WISC-IV) y el Continuous Performance Test (CPT-II). Se administraron los cuestionarios para padres y profesores del Behavior Rating Inventory of Executive Function (BRIEF) y las escalas de evaluacion de Conners (CPRS-48 y CTRS-28). Se realizo un programa de estimulacion cognitiva dos horas semanales durante ocho semanas. Resultados. Se observaron diferencias estadisticamente significativas tras aplicar el tratamiento de estimulacion cognitivo en el indice de razonamiento perceptivo de la WISC-IV. No se obtuvieron diferencias antes y despues del tratamiento en las puntuaciones del Conners y del BRIEF. Tampoco se hallaron diferencias en los resultados de la WISC-IV en funcion del sexo ni en el GMFCS. Conclusion. El rendimiento cognitivo de los niños con paralisis cerebral mejora tras la aplicacion de un programa de rehabilitacion cognitiva.

Síndrome de Imerslund-Gräsbeck: anemia megaloblástica con proteinuria / Imerslund-Gräsbeck syndrome: Megaloblastic anaemia with proteinuria

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[Measurement of health-related quality of life in children with chronic kidney disease using a specific test. Influence of treatment]. / Medida mediante un test específico de la calidad de vida relacionada con la salud en niños con enfermedad renal crónica. Influencia del tratamiento.

INTRODUCTION: Chronic kidney disease (CKD) affects the daily life of the child especially during the stage where their personal development takes place. Adult renal patients have a demonstrated worse health related quality of life (HRQL) mainly under hemodialysis (HD), however there are few published data about HRQL in children with CKD, most of them obtained after patient s childhood or with generic tests that do not discriminate changes in a specific disease. OBJECTIVE: To assess how our patients perceive their health by measuring the HRQL and its most affected domains. To determine how the different therapies affect the child with CKD and the agreement on the opinion between children and their parents. MATERIAL AND METHODS: We included 71 CKD children and their parents in a cross-sectional study (33 transplanted, 11 peritoneal dialysis [PD], 5 HD, 22 conservative treatment). We used a specific quality of life test for CKD children that we had previously developed (TECAVNER). If the child was younger than 9 years, only their parents completed the survey. RESULTS: children on HD refer a worse HRQL followed by those who underwent PD and those transplanted. The best HRQL was obtained in children with conservative treatment. Both parents and children agree that the domains more frequently affected are physical activity and school attendance especially those on HD. The way the adolescents 15 years and older perceived their health was similar to that of their parents. This was not the case in the younger group, 9 through 15 ears.

Desarrollo de un cuestionario en español de medida de calidad de la vida en pacientes pediátricos con enfermedad renal crónica / Design of a quality of life questionnaire in Spanish for children with chronic kidney disease

El tratamiento actual de la enfermedad renal crónica en la infancia debe incluir los aspectos sociales y psicológicos implicados en la calidad de vida del niño y de su familia. Nuestro objetivo ha sido desarrollar un instrumento de medida específico de la calidad de vida de los pacientes pediátricos con enfermedad renal crónica en español, ya que no existe actualmente ninguno validado para niños. Hemos desarrollado un cuestionario en español específico para enfermedad renal en niños basado en el test de calidad de vida para adultos con enfermedad renal (KDQOL-SFTM) y en el test de calidad de vida para niños con epilepsia (CAVE) adaptándolos a niños con enfermedad renal, al que denominamos TECAVNER (Test de Calidad de Vida en Niños con Enfermedad Renal). La fiabilidad de dicho cuestionario, determinada por el coeficiente alfa Cronbach, fue de 0,92. Las limitaciones del estudio consisten en que no se ha realizado validez de constructo ni test-retest. En conclusión, este trabajo constituye un primer intento para diseñar un cuestionario específico de calidad de vida relacionada con la salud en español para niños con enfermedad renal crónica (AU)

Treatment of chronic kidney disease in childhood must include assessment of social and psychological aspects involved in the perceived quality of life of the child and its family. Our objective has been to design a specific tool in Spanish for measuring quality of life in pediatric patients with chronic kidney disease, since there is not a validated test for children at the moment. Results: We designed a specific questionnaire for kidney disease in children based on the test of quality of life for adults with kidney disease (KDQOL-SFTM) and on the test of quality of life for children with epilepsy (CAVE)adapting them to children with kidney disease, denominating TECAVNER (Test of Quality of Life in Children with Kidney Disease). Reliability of this questionnaire determined by alfa Cronbach coefficient was 0,92. Limitations: questionnaire determined by Test-retest reliability and construct validity were not conducted. In conclusion, this is a first approach for design a specific health related quality of life test in Spanish for children with chronic kidney disease (AU)

Medida mediante un test específico de la calidad de vida relacionada con la salud en niños con enfermedad renal crónica. Influencia del tratamiento / Measurement of health related quality of life in children with chronic kidney disease using a specific test. Influence of treatment

Introducción: La enfermedad renal crónica (ERC) modifica la vida del paciente y afecta de manera especial a los niños en su etapa de desarrollo personal. En adultos se ha demostrado peor calidad de vida relacionada con la salud(CVRS), sobre todo en hemodiálisis (HD); sin embargo, hay muy pocos datos objetivos sobre CVRS en niños con ERC, la mayoría obtenidos una vez superada la infancia de estos enfermos o con cuestionarios genéricos que no permiten discriminar cambios en una enfermedad específica. Objetivos: Conocer cómo perciben nuestros pacientes su estado de salud midiendo la CVRS y sus áreas más afectadas. Determinar la influencia de los distintos tratamientos y la concordancia entre la opinión de los niños y de sus padres. Material y métodos: Estudio transversal en 71 niños(33 sometidos a trasplante, 11 en diálisis peritoneal, cinco en HD y 22 en tratamiento conservador) y en sus padres. Utilizamos un cuestionario específico desarrollado por nosotros para la medida de CVRS en niños con ERC. En los menores de 9 años sólo contestaron los padres. Resultados: Los niños en HD refieren una peor CVRS, seguidos de los pacientes en diálisis peritoneal (DP) y de los sometidos a trasplante (TX), y la mejor CVRS se obtiene en los pacientes sometidos a tratamiento conservador. Los aspectos que más se ven alterados son la actividad física y la asistencia escolar, sobre todo en HD, hecho en el que coinciden padres e hijos. La concordancia entre padres e hijos fue buena en los mayores de 15 años, existiendo discordancia en los niños de 9 a 15 años (AU)

Introduction: Chronic kidney disease (CKD) affects the daily life of the child especially during the stage where their personal development takes place. Adult renal patients have a demonstrated worse health related quality of life (HRQL)mainly under hemodialysis (HD), however there are few published data about HRQL in children with CKD, most of them obtained after patient´s childhood or with generic tests that do not discriminate changes in a specific disease. Objetive: To assess how our patients perceive their health by measuring the HRQL and its most affected domains. To determine how the different therapies affect the child with CKD and the agreement on the opinion between children and their parents. Material and methods: We included 71CKD children and their parents in a cross-sectional study ( 33transplanted, 11 peritoneal dialysis [PD], 5 HD, 22conservative treatment). We used a specific quality of life test for CKD children that we had previously developed(TECAVNER). If the child was younger than 9 years, only their parents completed the survey. Results: Children on HD refer a worse HRQL followed those who underwent PD and those transplanted. The best HRQL was obtained in children with conservative treatment. Both parents and children agree that the domains more frequently affected are physicalactivity and school attendance especially those on HD. The way the adolescents 15 years and older perceived their health was similar to that of their parents. This was not the case in the younger group, 9 through 15 years (AU)

Calidad de vida percibida por niños con enfermedad renal crónica y por sus padres / Perceived quality of life in children with chronic renal disease and in their parents

Introducción: La enfermedad renal crónica (ERC) afecta al bienestar físico, psíquico y social del niño. En adultos y adolescentes se ha descrito una peor calidad de vida (CV)en enfermos renales que en población sana, pero hay pocos estudios con instrumentos de medida de CV en niños con enfermedad renal. Objetivo: Estudiar la CV de los niños con ERC y compararla con un grupo control de niños sanos. Material y métodos: Estudio transversal en 71 niños con ERC y 57 sanos utilizando el cuestionario de salud generalMOS-SF-20 en mayores de 9 años y sus padres o sólo en éstos en los menores de 9 años. Resultados: Los niños con ERC tienen peor CV que los niños sanos con diferencia significativa en la percepción de la función física, del rol físico y del estado general de la salud y no significativa en la socialización. Los niños enfermos refieren menos dolor y mejor bienestar emocional que los niños sanos. La calidad percibida por los padres es también peor en la población de niños enfermos que en la de niños sanos en todos los dominios excepto en el dolor. La CV percibida por los padres coincide en todos los dominios con la de los niños de 9 a 12 años e infravalora la función social y el bienestar emocional en los niños mayores de 12 años. Conclusiones: La CV en niños con ERC es peor que en niños sanos y afecta sobre todo al ámbito físico y a la salud general, en lo que coinciden niños y padres (AU)

Introduction: Chronic renal disease (CRD) affects physical, emotional and social wellbeing of children. Renal adult and adolescent patients have a poorer quality of life (QL) than healthy population but few studies have been performed in children with CRD and appropriate QL measurement tools. Objectives: To assess QL in children with CRD comparing it with healthy children. Material and methods: Cross-sectional study in 71 children with CRD and 57 healthy children with the generic health status tool MOSF-SF-20 answered by children older than 9 and their parents and only by parents when children were younger than 9. Results: Children with CRD have a poorer QL than healthy children with significant differences in general self-esteem, physical performance and physical activity and no significant difference in socialization. On the contrary they refer less pain and emotional discomfort than healthy population. Perceived QL of children by parents is also worse in CRD population affecting all but pain dominions. 9-12 years old children and their parents agree in all QL dominions while parents underestimate social function and emotional well-being when their children were older than 12. Conclusions: QL in children with CRD is worse than in healthy children mainly in physical function and general self esteem and agree with parents perceptions (AU)

[Design of a quality of life questionnaire in Spanish for children with chronic kidney disease]. / Desarrollo de un cuestionario en español de medida de calidad de la vida en pacientes pediátricos con enfermedad renal crónica.

UNLABELLED: Treatment of chronic kidney disease in childhood must include assessment of social and psychological aspects involved in the perceived quality of life of the child and its family. Our objective has been to design a specific tool in Spanish for measuring quality of life in pediatric patients with chronic kidney disease, since there is not a validated test for children at the moment. RESULTS: We designed a specific questionnaire for kidney disease in children based on the test of quality of life for adults with kidney disease (KDQOL-SFTM) and on the test of quality of life for children with epilepsy (CAVE) adapting them to children with kidney disease, denominating TECAVNER (Test of Quality of Life in Children with Kidney Disease). Reliability of this questionnaire determined be alfa Cronbach coefficient was 0,92. LIMITATIONS: Questionnaire determined by Test-retest reliability and construct validity were not conducted. In conclusion, this is a first approach for design a specific health related quality of life test in Spanish for children with chronic kidney disease.