Effects of Enteral Nutrition Powder in Children with Phenylketonuria under One Year / 中国药房

OBJECTIVE:To explore the effects of enteral nutrition powder in children with phenylketonuria(PKU)under one year. METHODS:120 PKU children under one year were selected and given enteral nutrition powder(20 g/kg,qd)for consecutive 32 weeks. The level of benzene,height,weight,head circumference,IQ level,nutrition indicators and ADR were compared be-fore treatment,8 weeks after treatment,32 weeks after treatment. RESULTS:8 and 32 weeks after treatment,the level of benzene decreased gradually,and height,weight and head circumference increased gradually,with statistical significance(P0.05). 66 cases suffered from 181 times of ADR,mainly including 10 times diarrhea and 126 times upper respiratory tract infection,among which one children developed moderate gastroen-teritis. They were all recovered after symptomatic treatment. CONCLUSIONS:For PKU children under one year,enteral nutrition powder can effectively control the phenylalanine levels,prevent the decrease of IQ and improve the nutritional status so as to meet the normal growth and development of children nutritional requirements,with better safety and tolerance.

Patología vascular en pacientes menores de un año / Cerebrovascular pathology in infants

Objetivo. Analizar nuestra experiencia en patología neuroquirúrgica vascular en los pacientes menores de un año.Material y método. Entre el 1/1/89 y el 1/6/09 se trataron en el Servicio de Neurocirugía del Hospital Nacional de Pediatría Juan P. Garrahan 235 pacientes con malformación vascular del SNC. De ellos 19 (8%) eran menores de un año;11 fueron varones y 8 mujeres, con una edad media de 4,5 meses al diagnóstico, en un rango desde prenatal a 1 año. Se analizaron las historias clínicas y los estudios neurradiológicos de cada uno de ellos.Resultados. Todos los pacientes fueron estudiados con TAC inicial excepto las malformaciones aneurismáticas de la vena de Galeno (MAVG) que se diagnosticaron en un principio, con ecografía. En 16/19 pacientes se realizó angiografía digital cerebral, y en uno de los casos se efectuó angiografía espinal, hallándose la malformación a ese nivel. Los tipos de lesiones halladas fueron: 6 MAVG (4 coroideas y 2 murales), 6 malformaciones arteriovenosas (MAV), 2 fístulas arteriovenosas (A-V) piales (1 cerebral y 1 medular), 1 fístula A-Vmedular, 1 cavernoma, 1 aneurisma sacular y 2 malformaciones embrionarias en fosa posterior. Conclusión. Es remarcable la variabilidad y complejidad de las malformaciones vasculares del SNC en el primer año de vida. Es necesaria la integración del equipo endovascular al neuroquirúrgico, no sólo para el diagnóstico, sino también para completar o facilitar el tratamiento de alguna de estas lesiones. Las malformaciónes vasculares más frecuentes en este grupo son la MAVG y las MAV , seguidas de las fístulas A-V.

Objective. To analyze our experience in neurosurgical vascular disease in patients under one year. Materials and Methods. Between 1/1/89 and 1/6/09 we treated at the Department of Neurosurgery of the Hospital Nacional de Pediatría Juan P. Garrahan 235 patients with vascular malformation of the central nervous system. Of these19 (n = 19; 8%) were infants under one year, 11 were males and 8 females, mean age at diagnosis 4.5 months (range from prenatal to 1 year). We evaluated all charts and neuroradiologic studies of each patient. Results. All patients were studied with initial CT scan except one patient with Vein of Galen Aneurysmal Malformation (VGAM)who was diagnosed antenatal by ultrasound. The types of lesions were 6 VGAM (4 choroidal type and 2 mural type), 6 Arteriovenous Malformations (AVM), 2 Pial Arteriovenous Fistulas(1 brain and 1 spinal), 1 spinal Dural Arteriovenous Fistula, 1 Cavernoma, 1 Aneurysm and 2 Embryologic Dural Sinus Malformations at the posterior fossa. Conclusion. It is remarkable variability and complexity ofvascular malformations of the central nervous system in the first year of life. It is necessary to integrate the endovascular neurosurgical team, not only for diagnosis but also to complement or facilitate the treatment of any of theses vascular disease. The most frequent vascular malformations in our group were the VGAM and the AVM followed by AV Fistulas.

An Analysis on Legal Autopsy Under 1Year of Age / 대한법의학회지

This is a statistical analysis based on the data of legal autopsies under 1 year of age performed at the Headquarters of the National Institute Scientific Investigation, requested from law enforcement agencies from 1996 to 2000. This report aims to analyse various aspects of 530 unusual deaths under 1 year of age in Korea, chiefly Seoul and its surrounding area. Male was 55.8% and female was 44.2%. Unnatural deaths were 39.6%, natural 53.4%, and the unknown 7.0%. Homicide occupied 66.7% in unnatural death and asphyxia was 41.4%, starvation and neglect 35.0%, and trauma 20.7% in homicide. Smothering was the leading cause of asphyxial homicide and accidental death. Natural death including perinatal disorders, sudden infant death syndrome and congenital malformations was the main cause of death in this age group.