Síndrome de realimentación en niña con parálisis cerebral / Refeeding syndrome in a girl with cerebral palsy

El síndrome de realimentación es una complicacióngrave y potencialmente mortal asociada a la terapianutricional por vía oral, enteral o parenteral. Afecta apacientes con desnutrición severa o en riesgo de desnutrición, como aquellos con parálisis cerebral infantil. Presentamos el caso de una paciente de ocho añoscon parálisis cerebral que ingresó por hipoglucemiasevera y que, tras iniciar la alimentación enteral porsonda nasogástrica, desarrolló un síndrome de realimentación. En niños con parálisis cerebral es fundamental valorar la presencia de factores de riesgo paradesarrollar un síndrome de realimentación, iniciar laalimentación de manera progresiva y monitorizar losiones séricos.(AU)

Refeeding syndrome is a serious and life-threateningcomplication associated with oral, enteral and parenteral nutritional therapy. It appears in severely malnourished patients or in those at risk of malnutrition,such as persons with cerebral palsy. We present the case of an 8-year-old girl with cerebral palsy who was admitted with severe hypoglycemia.After starting enteral nutrition by nasogastric tube, shedeveloped refeeding syndrome. In children with cere-bral palsy, it is essential to assess the presence of riskfactors for refeeding syndrome before starting any nutritional support, and then start feeding progressivelyand monitor serum electrolytes.(AU)

[Refeeding syndrome in a girl with cerebral palsy].

Refeeding syndrome is a serious and life-threatening complication associated with oral, enteral and parenteral nutritional therapy. It appears in severely malnourished patients or in those at risk of malnutrition, such as persons with cerebral palsy. We present the case of an 8-year-old girl with cerebral palsy who was admitted with severe hypoglycemia. After starting enteral nutrition by nasogastric tube, she developed refeeding syndrome. In children with cerebral palsy, it is essential to assess the presence of risk factors for refeeding syndrome before starting any nutritional support, and then start feeding progressively and monitor serum electrolytes.

Soporte nutricional en el lactante y niño menor de 3 años / Nutritional support in the infant and child under 3 years of age

La alimentación y nutrición en los primeros años de la vida presentan aspectos particulares respecto a otras edades; algunos dependen de las habilidades y posibles enfermedades del niño y otros, de los conocimientos y de la interacción de los padres con sus hijos en el momento de la alimentación. La falta de apetito, el rechazo del alimento o la excesiva duración de las comidas, y la afectación ponderal son consultas habituales en estas edades. La valoración médica en estos casos debe partir de una historia clínica general, una valoración dietético-alimentaria y un examen físico, valorando la necesidad de exámenes complementarios posteriormente. Deberá detectarse déficit de ingesta de alimentos, enfermedad orgánica, problemas en la interacción cuidador-niño y problemas de crecimiento o malnutrición. Los pilares que constituyen la base del tratamiento de estos problemas son el soporte nutricional, el tratamiento de los trastornos de la alimentación y el tratamiento de las deficiencias nutricionales específicas. El soporte nutricional se inicia con una correcta evaluación clí- nica y consistirá en mejoras en el rendimiento de la ingesta oral, para evaluar la indicación de alimentación enteral mediante sonda nasogástrica o gastrostomía con dietoterápicos en forma de módulos o dietas enterales completas. El tratamiento de los trastornos de la alimentación se inicia valorando y fomentando los conocimientos de alimentación y nutrición en los cuidadores, instruyéndoles en las situaciones de conflicto. A pesar de ello, el tratamiento en ocasiones deberá ser multidisciplinario (AU)

Diet and nutrition early in life have particular aspects regarding other ages, some depend on infant, their skills and possible diseases, and other on parents, nutritional knowledge and interaction with their infant at feeding. Lack of appetite, food refusal, excessive duration of meals, and poor weight are common queries involvement in these ages. Medical assessment in these cases must start from a clinical history, a dietary assessment and physical examination considering the need for additional tests. Poor food intake, physical illness, problems in caregiver-child interaction and growth problems or malnutrition must be identified. The basis of the treatment of these problems are nutritional support, treatment of eating disorders and treatment of specific nutritional deficiencies. Nutritional support begins with a clinical assessment and will consist of improvements of oral intake, evaluating the indication for enteral feeding via nasogastric or gastrostomy and using nutritional supplements or complete enteral diets. The treatment of eating disorders starts valuing the knowledge about food and nutrition in caregivers, encouraging and instructing them in conflict situations. However, sometimes multidisciplinary approach will be necessary (AU)

[Childhood cancer in the Autonomous Community of Cantabria in Spain (1995-2000)]. / Cáncer infantil en la comunidad de Cantabria (1995-2000).

BACKGROUND: Since 1980, the epidemiology of childhood cancer in Spain has been registered through the National Registry of Childhood Cancer. However, this registry does not include patients from Autonomous Community of Cantabria because there is no reporting clinical center. The absence of data on childhood cancer in this region justifies this study. OBJECTIVES: To analyze the clinical presentation, diagnostic delay and incidence of childhood cancer in Cantabria. METHODS: We performed a retrospective analysis of 89 children (aged 0-15 years) diagnosed with cancer in Cantabria from 1995-2000. RESULTS: The annual incidence of childhood cancer in the region was 198.1 cases per million inhabitants with a predominance of males (53.9 %) and children aged less than 5 years (46.1 %). The most frequent cancers were leukemia (32.6 %) and brain tumors (23.6 %). The most frequent signs and symptoms were fever (29.2 %) and decreased appetite or fatigue (19.1 %). The mean delay in diagnosis for all tumors was 5.99 weeks and delays were longest for brain tumors. There was a direct statistical relationship between non-specific clinical presentation and diagnostic delay. A total of 20.2 % of all patients received treatment outside Cantabria. CONCLUSIONS: The incidence of all childhood cancers and especially that of neuroblastoma was higher in Cantabria than in other areas of Spain. In general, the major difficulties in the diagnosis of childhood cancer are its low incidence and non-specific presenting symptoms. Specialized pediatric oncology units near to patients' homes should be created to avoid problems due to treatment outside the area of residence.

Cáncer infantil en la comunidad de Cantabria (1995-2000) / Childhood cancer in the autonomous community of Cantabria in Spain (1995-2000)

Antecedentes: Desde el año 1980, el Registro Nacional de Tumores Infantiles recoge los datos estadísticos del cáncer infantil en España. Este registro no recoge los pacientes de la Comunidad de Cantabria debido a la ausencia de un centro informante y ello justifica la realización del presente trabajo. Objetivos Analizar la incidencia, demora diagnóstica y aspectos clínicos del cáncer infantil en Cantabria. Métodos Estudio retrospectivo mediante la revisión de historias clínicas de 89 pacientes menores de 15 años con cáncer, diagnosticados en Cantabria durante los años 1995-2000.Resultados La incidencia anual de cáncer infantil en Cantabria fue 198,1 casos por millón de habitantes, predominando en varones (53,9 per cent) y menores de 5 años (46,1 per cent). El tumor más frecuente fue la leucemia (32,6 per cent), seguido de los tumores cerebrales (23,6 per cent). Los síntomas de presentación más frecuentes fueron fiebre-febrícula (29,2 per cent) y astenia-anorexia (19,1 per cent). La demora diagnóstica media fue de 5,99 semanas, presentando la mayor demora los tumores cerebrales. Se observa una relación estadística directa entre la demora diagnóstica y la inespecificidad de los síntomas de presentación. El 20,2 per cent de los pacientes fueron seguidos fuera de Cantabria. Conclusiones En Cantabria, la incidencia global de cáncer infantil y, en concreto, de neuroblastomas resultó alta comparada con nuestro entorno. En general, las principales dificultades diagnósticas del cáncer infantil son su baja frecuencia y la inespecificidad de su clínica inicial. Se debe fomentar la creación de unidades de seguimiento del paciente oncológico infantil cercanos a sus lugares de residencia y evitar los trastornos derivados de su desplazamiento (AU)

Microcefalia, opacidad corneal bilateral y holoprosencefalia lobarcongénitas con desarrollo posterior de un rabdomio sarcoma en un paciente expuesto a radiación prenatal / Microcephaly, bilateral corneal opacity and congenital lobar holoprosencephaly with subsequent development of a rhabdomyosarcoma in a patient exposed to prenatal radiation

Introducción. La mayoría de las malformaciones congénitas de causa identificada se corresponden con causas genéticas, multifactoriales o secundarias a algún teratógeno. Muchas malformaciones congénitas tienen un origen desconocido, pero la asociación de diferentes malformaciones permite al menos definir el momento prenatal en que la noxa afectó el desarrollo embrionario o fetal. Caso clínico. Presentamos el caso de un recién nacido de una gestación de 40 semanas, con exposición prenatal a radiaciones ionizantes, hallazgos ecográficos prenatales de microcefalia y cariotipo normal. El neonato presenta opacidades corneales, microcefalia y malformación encefálica compleja. Las opacidades corneales, unidas al glaucoma congénito constituyen un síndrome de Peters que condiciona ceguera. El pacientes fue tratado con trabeculectomía y trasplante corneal bilateral. La microcefalia y la holoprosencefalia lobar con agenesia de cuerpo calloso determinaron un escaso desarrollo psicomotor, hipertonía y una epilepsia con registro electroencefalográfico de hemihipsarritmia, tratada sin éxito con valproato y vigabatrina. A los 21 meses el paciente desarrolla un rabdomiosarcoma embrionario de base de lengua, y fallece en el contexto de una infección sistémica tras la quimioterapia. Conclusiones. La asociación de malformaciones descrita no ha sido referida anteriormente en las bases de datos internacionales. Aunque la posible relación causal entre radioexposición prenatal y el cuadro clínico no puede demostrarse, la posibilidad teratogénica y carcinogénica de dicha exposición llevan a insistir en evitar que las mujeres gestantes o que desconocen su estado gestacional estén presentes en las salas de radiodiagnóstico (AU)

Consumo de tabaco, alcohol y drogas no legales entre adolescentes y relación con los hábitos de vida y el entorno / Tobacco, alcohol and illegal drug consumption among adolescents. Relationship with lifestyle and environment

Objetivos: Conocer la edad de contacto con tabaco, alcohol y drogas ilegales entre los adolescentes, su consumo y sus relaciones con el entorno y hábitos de vida. Método: Estudio transversal descriptivo, mediante encuesta a 2.178 adolescentes de 12 a 16 años representativos de Cantabria. Resultados: El 80,4% de los adolescentes considera el tabaco una droga. El 44% ha fumado un cigarrillo alguna vez y son fumadores el 19,3%. El 44,5% no cree que el alcohol sea una droga. El 69,2% ha probado el alcohol y el 37% son bebedores. La mayoría (92,9%) bebe los fines de semana y el 88% todas las bebidas encuestadas. Amigos (54,1%) y familiares (16,4%) son los iniciadores en este hábito. El 46% de los bebedores actuales se ha embriagado en los últimos 6 meses una o más veces. A los 15 años fuma el 29,4% y bebe el 61,8%. El contacto con drogas ilegales es menos frecuente y el 10,2% declara consumir hachís. El consumo de todas estas sustancias se asocia con una mayor edad, un entorno consumidor y determinado patrón de ocio. El análisis mediante regresión logística refleja que la consideración de tabaco y alcohol como drogas es un factor protector para su consumo y son factores de riesgo consumir otras drogas, embriagarse y tener un entorno consumidor de las mismas. Conclusiones: Los adolescentes presentan un contacto precoz y un consumo preocupante de tabaco, alcohol y drogas ilegales. El entorno y los hábitos de vida se relacionan marcadamente con estas conductas de riesgo (AU)

[Tobacco, alcohol and illegal drug consumption among adolescents and the relationship with lifestyle and environment]. / Consumo de tabaco, alcohol y drogas no legales entre adolescentes y relación con los hábitos de vida y el entorno.

OBJECTIVES: To identify the age at which adolescents first experiment with tobacco, alcohol and illegal drugs and to identify the relationship between consumption of these substances and lifestyle and environment. METHOD: Cross-sectional descriptive study based on a survey of 2,178 adolescents aged 12-16 years old from Cantabria (Spain). RESULTS: Tobacco was considered a drug by 80.4 % of the adolescents. Forty-four percent had smoked at some time and 19.3 % were smokers. Alcohol was not considered a drug by 44.5 %, 69.2 % had drunk alcohol at some time and 37 % drank regularly. Most of the adolescents (92.9 %) drank at weekends and 88 % drank all the spirits surveyed. The adolescents were introduced to this habit by friends (54.1 %) and relatives (16.4 %). Forty-six percent of those who drank regularly had got drunk once or more in the previous 6 months. At the age of 15 years, 29.4 % smoked and 61.8 % drank alcohol regularly. An association with illegal drugs was less common; 10.2 % smoked hashish. Consumption of all these substances was associated with greater age, living in an environment in which these substances were consumed and with certain forms of leisure activity. Logistic regression analysis revealed that considering tobacco and alcohol as drugs protected adolescents against their consumption and that consuming other drugs, getting drunk and being in a consumer environment were risk factors for consumption. CONCLUSIONS: Contact with illegal drugs, alcohol and tobacco starts early in adolescence. Levels of consumption are worrying. Environment and lifestyle are closely associated with risk factors for consumption.

[Microcephaly, bilateral corneal opacity and congenital lobar holoprosencephaly with subsequent development of a rhabdomyosarcoma in a patient exposed to prenatal radiation]. / Microcefalia, opacidad corneal bilateral y holoprosencefalia lobar congénitas con desarrollo posterior de un rabdomiosarcoma en un paciente expuesto a radiación prenatal.

INTRODUCTION: Most congenital malformations of which the cause is known are due to genetic or multifactorial causes or are secondary to a teratogen. Many congenital malformations are of unknown origin. However, the association of different malformations allows us to define the moment in which the noxous agent affected embryonic or foetal development. CLINICAL CASE: We present the case of a baby born after 40 weeks gestation, who had been exposed to ionising radiation before birth. Prenatal echography showed microcephaly and the karyotype was normal. The newborn baby had corneal opacities, microcephaly and complex encephalic malformations. The corneal opacity together with congenital glaucoma constitute Peters syndrome which leads to blindness, and is treated by trabeculectomy and bilateral corneal transplants. The microcephaly and lobar holoprosencephaly with agenesis of the corpus callosum led to reduced psychomotor development, hypertonia and epilepsy with an electroencephalogram recording of hemihypsarrythmia which was unsuccessfully treated with valproate and vigabatrine. At the age of 21 months the patient developed an embryonic rhabdomyosarcoma of the base of the tongue. He died with systemic infection whilst being treated with chemotherapy. CONCLUSIONS: The association of the malformations described has not previously been reported in the international data bases. Although it was not possible to prove that prenatal exposure to radiation caused the clinical condition described, the possibility of teratogenesis and carcinogenesis following such exposure means that pregnant women or those who may be pregnant should not be in places where radiodiagnosis is carried out.

Síndrome de Moebius / The Moebius syndrome

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Short-term effects of postnatal manipulation on central beta-adrenoceptor transmission.

Neonatal handling is known to induce long-lasting changes in behavioral and neuroendocrine responses to stress. Since the central noradrenergic system participates in the adaptive responses to stressful conditions we have analyzed the effects of postnatal handling on beta-adrenoceptor binding sites and isoprenaline- and forskolin-stimulated cyclic AMP accumulation in cerebral cortex, hippocampus and cerebellum of rats at 1 and 3 months of age. Handled animals showed reduced emotional reactivity and lower ACTH and corticosterone secretion after stress. Binding studies using [(3) H]CGP12-177 revealed increased beta-adrenoceptor binding sites in handled rats in cerebellum and cerebral cortex with no changes in hippocampus, and decreased affinity in all cerebral regions. Handling reduced basal levels of cyclic AMP in hippocampus and cerebellum but not in cerebral cortex. The concentration-response curves of cyclic AMP to isoprenaline were displaced to the right in cerebellum of handled rats without differences in Emax; however, Emax was significantly reduced in cerebral cortex and hippocampus. Direct stimulation of the catalytic subunit of adenylyl cyclase by forskolin reduced the efficiency in hippocampus and cerebellum, but not in cerebral cortex of handled animals. It is concluded that neonatal handling reduces the binding properties of beta-adrenoceptor and its primary biochemical responses in the young rat brain, which may account for the reduced responsiveness to stress attained in the handled rats, and may explain the persistence of the effect. The present study emphasizes the role of the central noradrenergic system in modulating the behavioral and neurendocrine responses to neonatal handling.

Alterations of central noradrenergic transmission in Ts65Dn mouse, a model for Down syndrome.

Mice with segmental trisomy 16 (Ts65Dn) which have triplication of a region of mouse chromosome 16 homologous to the Down syndrome critical region in human chromosome 21, are used as a model for Down syndrome. Functioning of the central beta-noradrenergic transmission was studied in Ts65Dn mice. Binding analysis in cerebral cortex revealed no change in the number of beta-adrenoceptors and a slight reduction of affinity. The beta-adrenoceptor transduction was assessed by analyzing cAMP formation in the cerebral cortex, hippocampus and cerebellar cortex under basal conditions and after stimulation with isoprenaline and forskolin. Basal production of cAMP was significantly reduced in hippocampus and cerebellar cortex of Ts65Dn mice compared to control, but not in cerebellum. After phosphodiesterase inhibition, net increments in cAMP accumulation were similar in both groups of mice. Stimulation of cAMP production by isoprenaline (10 microM) and forskolin (10 microM) was much higher in hippocampus than in cerebral cortex of either group. In both areas, but not in cerebellum, the stimulatory responses were consistently and significantly smaller in Ts65Dn than in control mice. Concentration-response curves for isoprenaline and forskolin were generated in the cerebral cortex. Emax responses were lower in trisomic than in control mice; however, in Ts65Dn mice the slope of the response curve to isoprenaline was markedly depressed whereas that to forskolin was similar to control. It is concluded that Ts65Dn mice show severe deficiencies in the synaptic transmission of the central beta-noradrenergic system, which are selective for specific brain areas.