Atypical presentation of Best Disease. / Presentación atípica de la enfermedad de Best.

CLINICAL CASE: A 43-year-old man was treated for reduced visual acuity, initially attributed to strabismic amblyopia. On fundus examination, bilateral neurosensory detachments (NSD) were observed in posterior pole, surrounded by deposits of lipofuscin. His 3-year-old son was also examined and circumscribed NSD was observed with the presence of pseudohypopyon in OD and a fibrosis scar in OS. The Arden ratio were decreased in electrooculography (EOG) in both patients, and genetic studies revealed a single mutation of the BEST1 gene. DISCUSSION: The existence of extensive bilateral NSD may be an unusual form of presentation of Best disease. Family history, EOG, and genetic study supported this diagnosis.

[Use of pacifiers and breastfeeding]. / Uso del chupete y lactancia materna.

Pacifiers are widely used in developed societies. They are used for soothing infants, reducing their stress and pain during procedures and to help them sleep. The use of pacifiers has been associated, however, with a shorter duration and exclusivity of breastfeeding, with higher rates of otitis media and dental problems, as well as a higher risk of accidents during infancy. Recent studies have also described a relationship between pacifier use and SIDS, especially when used during infant́s sleep. Other described benefits are analgesia and stimulation of non-nutritive sucking in preterm and term infants. There is, at present, wide debate and controversy on whether or not to recommend its use; thus it seems important for professionals and parents to be aware of the risks and benefits associated to its use, particularly related to breastfeeding. Due to the existing controversy of scientific findings, the Committee on Breastfeeding of the Spanish Paediatrics Society, publishes this review, trying to summarise present evidence with the objective of, after analysing scientific results and recommendations, making recommendations regarding the use of the pacifier in the breastfed infant.

[Family impact of rotavirus gastroenteritis in children under two years]. / Impacto familiar de la gastroenteritis por rotavirus en menores de dos años.

INTRODUCTION: Acute gastroenteritis (AGE) in infants has a significant impact on the quality of life of their parents. MATERIAL AND METHODS: Cross-sectional study on the sociological family impact related to rotavirus AGE in children under 2 years. The study was carried out in 25 hospitals and 5 primary care centres in Spain. Sociodemographic, epidemiological and clinical data were recorded, as well as the symptomatology of AGE and its severity measured by the Clark scale. Stool samples were tested to determine rotavirus positive (RV+) or negative (RV-). The parents were asked to complete a a family impact questionnaire. RESULTS: Stool specimens were tested in 1087 AGE cases (584 RV+ vs 503 RV-). The 99.5 % of parents whose children were RV+ reported more worries vs. the 97.7 % of RV-, and RV+ had a higher importance score (p < 0.05). A higher percentage of RV+ parents and those with a high importance score reported more time dedicated to dehydration treatment (p < 0.05). The 82.5 % vs. 73.9 % had disruption of their household tasks, with more importance scores (p < 0.05). RV+ had a higher percentage and importance score than RV- ones in all aspects of their child's AGE symptoms, except loss of appetite. CONCLUSION: AGE produces important dysfunctional experiences in daily family life. According to parental perceptions, RV+ produces greater worries and dysfunctions in child behaviour.

Ethyleneglycol pulp from tagasaste.

In this work, we examined the influence of operational variables [viz. Ethyleneglycol concentration (50-70%), temperature (155-185 degrees C), time (30-90 min) and number of PFI beating revolutions (500-1500)] in the ethyleneglycol pulping of tagasaste (Chamaecytisus proliferus) on pulp yield and the breaking length, stretch, burst index, tear index and brightness of paper sheets formed from it. Application of a fuzzy neural network model in combination with an experimental factorial design allowed the results for the dependent variables to be predicted as a function of the operating conditions used with errors less than 15% in all cases. The operating conditions of choice provided pulp with a high yield (56.85%) and a low brightness (22.51%) which may thus be useful to obtain non-white paper.

Bleaching of soda pulp of fibres of Musa textilis nee (abaca) with peracetic acid.

In this work, we studied the influence of operational variables in the bleaching of soda pulp of Musa textilis nee (abaca) [viz. temperature (55-85 degrees C), bleaching time (30-150 min) and peracetic acid concentration oven dry pulp (0.5-4.5%)] on the kappa number and viscosity of the bleached pulp, as well as on the breaking length, burst index and brightness of paper sheets made from it. For this purpose, we used a central composite factorial design in order to identify the optimum operating conditions. In this way equations relating the dependent variables to the operational variables of the bleaching process were derived. These equations reproduce the dependent variables with errors less than 12% for all, except the viscosity which was predicted with errors less than 18%. Obtaining bleached pulp with the highest possible viscosity (1519 ml/g), and paper sheets with the maximum possible breaking length (6547 m) and burst index (5.00 kN/g), entails using a temperature of 55 degrees C, a peracetic acid concentration of 4.5% and a bleaching time of 150 min. This provides a brightness of 79.90%, which is only 6.53% lower than the maximum possible value (85.48%).

[Diffuse large B cell lymphoma presenting with a bilateral serous macular detachment]. / Desprendimiento seroso macular bilateral como forma de presentación de linfoma B difuso de célula grande.

CLINICAL CASE: A 42 year-old male was assessed for a subacute, bilateral and progressive visual loss. His medical history included chronic hepatitis C infection and Evans syndrome. On fundal examination, multiple areas of neurosensorial and retinal pigmented epithelium detachment were observed in the region of both macula. A few days later, his general health deteriorated and he was noted to have a high fever and adenopathy. Biopsy of an enlarged lateral cervical lymph node demonstrated the existence of a diffuse large B cell lymphoma. After several cycles of chemotherapy, he experienced a progressive and bilateral improvement of his vision, which was accompanied by a reattachment of the previously detached areas. DISCUSSION: A bilateral and serous macular detachment may be the initial manifestation of a diffuse large B cell lymphoma. The prognosis of these retinal lesions may be favourable if lymphoma remission is achieved.

Optimization of pulping conditions of abaca. An alternative raw material for producing cellulose pulp.

The influence of temperature (150-170 degrees C), pulping time (15-45 min) and soda concentration (5-10%) in the pulping of abaca on the yield, kappa, viscosity, breaking length, stretch and tear index of pulp and paper sheets, was studied. Using a factorial design to identify the optimum operating conditions, equations relating the dependent variables to the operational variables of the pulping process were derived that reproduced the former with errors lower than 25%. Using a high temperature, and a medium time and soda concentration, led to pulp that was difficult to bleach (kappa 28.34) but provided acceptable strength-related properties (breaking length 4728 m; stretch 4.76%; tear index 18.25 mN m2/g), with good yield (77.33%) and potential savings on capital equipment costs. Obtaining pulp amenable to bleaching would entail using more drastic conditions than those employed in this work.

Incidencia de meningitis neumocócica en niños de 0 a 23 meses en Cantabria. Estudio retrospectivo 1997-2001 / Incidence of pneumococcal meningitis in children from 0 to 23 months in Cantabria. Retrospective study 1997-2001

Objetivo: Contribuir al conocimiento de la epidemiología de enfermedad neumocócica en nuestro país, y conocer la incidencia actual de la meningitis neumocócica en población infantil menor de 2 años en Cantabria. Pacientes y métodos: Estudio retrospectivo (1 de enero 1997 a 31 diciembre 2001), de los pacientes menores de 2 años ingresados en nuestro Servicio en los que se aisló el S. Pneumoniae en sangre y /o LCR asociado a signos clínicos y analíticos de meningitis bacteriana. Se revisaron las historias clínicas y se calculó las tasas de incidencia con intervalo de confianza al 95 por ciento en los distintos grupos de edad (0 a 11 meses y 0 a 23 meses). El número de niños y su distribución por edades se obtuvo del Movimiento Natural de las Población de Cantabria, del Instituto Nacional de Estadística (www.ine.es/tempus/cgi-bin/itie). El cálculo estadístico se realizó por el método exacto binomial. Resultados: En el período estudiado se detectaron 4 pacientes con meningitis neumocócica, todos ellos de edad inferior a 12 meses. La tasa de incidencia en el primer año de vida es de 20,13 (intervalo de confianza al 95 por ciento: 5,49- 51,54) y en el niño de 0 a 24 meses de 10,19 (intervalo de confianza al 95 por ciento: 2,78-26,12). Conclusiones: Nuestro estudio demuestra una incidencia elevada de meningitis neumococica en Cantabria, particularmente en el primer año de vida (20,13 casos por 100.000 habitantes). La tasa de incidencia, es similar a la encontrada en otros comunidades de nuestro país (AU)

[Subconjunctival mitomycin C for the treatment of ocular cicatricial pemphigoid]. / Mitomicina C subconjuntival en el tratamiento del penfigoide ocular cicatricial.

PURPOSE: We study the efficacy of treating ocular cicatricial pemphigoid (OCP) with subconjuctival mitomycin C (MMC). METHODS: The patients were eligible for treatment under two criteria: 1. Significant complications of systemic immunosuppressant therapy. 2. Avanced stage of OCP. All patients received an injection of 0.25 ml of 0.2 mg/ml mitomycin C to both the upper and lower bulbar conjunctiva in both eyes. RESULTS: Four patients were treated with subconjunctival mitomycin C (both eyes) and were followed for an average of 17 months (range 9-26 months). Three of four patients showed progression of the conjunctival disease and required concomitant systemic immunosuppressive therapy after subconjuctival mitomycin C. CONCLUSION: No complications of subconjunctival mitomycin C treatment were noted but, contrary to what other authors had referred, its use was not effective in preventing progression of conjunctival cicatrization in our patients with OCP.

Influence of chronic treatment with the growth hormone secretagogue Ipamorelin, in young female rats: somatotroph response in vitro.

Growth hormone (GH) is secreted in the anterior pituitary gland by the somatotroph cells. Secretion is regulated by growth hormone releasing hormone (GHRH) and somatostatin. Morever, GH secretagogues (GHS) can exert a considerable effect on GH secretion. In order to determine the effects of chronic treatment with the GHS Ipamorelin on the composition of the somatotroph cell population and on somatotroph GH content, an in vitro analysis was performed of the percentage of somatotroph cells (% of total), the ratio of different GH cell types (strongly/weakly-staining) and individual GH content, in pituitary cell cultures obtained from young female rats receiving Ipamorelin over 21 days (Ipamorelin group) and the effects were compared with those of GHRH (GHRH group) or saline (saline group). The ultrastructure of somatotroph cells did not change, but the volume density of secretion granules was increased (P<0.05) by previous in vivo Ipamorelin or GHRH treatment. In 3-day basal pituitary cell monolayer cultures, the percentage of somatotroph cells showed no modifications between groups, nor was there any change in the ratio of strongly/weakly immunostaining GH cells. In the Ipamorelin group alone, in vitro treatment with Ipamorelin (10(-8) M), or GHRP 6 (10(-8) M), or GHRH (10(-8) M) for 4 hours, increased the percentage of somatotroph cells, without modifying the ratio of strongly/weakly immunostained GH cells. Basal intracellular GH content in somatotroph cells over 4 hours was lower in the Ipamorelin group and the GHRH group than in the saline group. Only in the Ipamorelin group did Ipamorelin (10(-8) M), GHRP 6 (10(-8) M) and GHRH (10(-8) M) prompt increased intracellular GH content. These data suggest that, at least in the young female rat, the GHS Ipamorelin is able to exert a dynamic control effect on the somatotroph population and on GH hormone content.

Estudio del estado nutricional de la población infantil de la zona rural de la costa ecuatoriana / Nutritional status in the pediatric population of a rural area on the Ecuadorian coast

Objetivos: Valorar la repercusión de una dieta fundamentada en hidratos de carbono en el estado nutricional de la población infantil según un estudio realizado en una zona rural de la provincia de Los Ríos (Ecuador).Pacientes y métodos: Se valoraron 871 niños ecuatorianos pertenecientes al ámbito rural en un consultorio médico ambulante habilitado para el estudio. Se tomaron medidas antropométricas básicas (peso, talla, perímetro cefálico y perímetro braquial) y datos clínicos procedentes de la exploración física. Las medidas antropométricas observadas se compararon con las tablas de crecimiento utilizadas para la población infantil española, que se tomaron como estándar. Se realizó además una encuesta nutricional a las madres. Resultados: Se incluyeron 871 niños (53% varones, 47% mujeres). Edad: rango, 0-14; media, 6,39; moda, 4. Comparando los datos observados con las tablas de crecimiento utilizadas para la población infantil española, tomada como estándar se encontraron los siguientes resultados: índice de masa corporal: diferencias significativas (p < 0,05) en varones de 5 a 14 años y mujeres de 3 a 12 años, diferencias altamente significativas (p < 0,01) en varones de 6 a 13 años y mujeres de 3 a 12 años; perímetro braquial: diferencias significativas (p < 0,05) en varones y mujeres de 1 a 14 años, diferencias altamente significativas (p < 0,01) en varones de 1 a 12 años y mujeres de 2 a 12 años. Perímetro braquial < 14 cm (malnutrición leve): 6,67% de varones y 9,15% de mujeres; perímetro braquial < 12,5 cm (malnutrición grave), 1,82% de varones y 2,82% de mujeres. Encuesta nutricional: la ingesta energética fue aproximadamente de 1.500 calorías/día, de las cuales el 80% eran hidratos de carbono, el 15% grasas y el 5% proteínas. Conclusiones: Una dieta pobre cualitativamente, aunque no cuantitativamente, se pone de manifiesto en el estado nutricional de la población infantil. El déficit nutricional lleva consigo la prevalencia de gran número de enfermedades infantiles que podrían ser evitadas con la alimentación y la higiene adecuadas. Los escasos recursos económicos y sanitarios hacen que la prevención sea el arma fundamental para preservar la salud de los países subdesarrollados. Sobre la nutrición infantil influyen tanto la alimentación, a su vez influida por factores ambientales de orden físico, climático, cultural y político, como el estrés provocado por las infecciones y la conducta de los padres. El estado nutricional infantil es un claro reflejo del estado socioeconómico de una población determinada (AU)

Inmunoprofilaxis activa después del trasplante alogénico de progenitores hemopoyéticos en pacientes pediátricos / Active immune-prophylaxis after allogeneic hematopoietic progenitor cell transplantation in pediatric patients

Existen diferentes evidencias de la desaparición de inmunidad frente a las enfermedades para las que existe inmunoprofilaxis activa, tras un trasplante alogénico de medula ósea. Estos hallazgos no constituyen sólo un problema epidemiológico. Desde un punto de vista estrictamente clínico, estos pacientes podrían beneficiarse de la administración de determinadas formas de vacunas frente a enfermedades a las que son especialmente susceptibles. Sin embargo, no existe en la actualidad una pauta unificada para la revacunación de estos pacientes. Más aún, diferentes encuestas sugieren que la inmunoprofilaxis activa es con frecuencia infravalorada en estos pacientes. En la presente revisión, se pretende ofrecer tanto una información actualizada sobre las actuales tendencias, como una guía práctica para la reinmunización de estos pacientes de acuerdo con las necesidades de nuestro medio (AU)

[Active immune-prophylaxis after allogeneic hematopoietic progenitor cell transplantation in pediatric patients]. / Inmunoprofilaxis activa después del trasplante alogénico de progenitores hemopoyéticos en pacientes pediátricos.

There is considerable evidence supporting the disappearance of immunity against diseases for which there is active immune-prophylaxis available after allogeneic hematopoietic progenitor cell transplantation. These findings do not constitute merely an epidemiologic problem. From a strictly clinical point of view, these patients might benefit from the administration of different forms of vaccines against diseases to which they are particularly susceptible. There are, however, no unified criteria for the revaccination of these patients. Moreover, several surveys suggest that active immune-prophylaxis is frequently undervalued in these patients. In the present review, we provide up-to-date information on current trends as well as practical guidelines for the re-immunization of these patients in our environment.

Breast-feeding in Spain.

Human milk is the best way to nurture the human infant. By breast-feeding their babies, mothers provide them with the best opportunities to wholly develop their potential, while protecting the infants and themselves from a whole range of diseases in the near future and in the years to come. Even though these benefits are widely known and there is ample scientific evidence on the topic, it seems from published data that Spanish women are not breast-feeding their babies as much and for as long as they should. Less than 90% start breast-feeding, at 1 month there is already an attrition of 30%, at 3 months more than half of the infants are taking artificial milk and by 6 months only 10% continue to breast-feed their infants. Low birth weight, Caesarean section and low study level are among the more significant factors that negatively affect breast-feeding. There is still work to do to improve this situation. Promotion of breast-feeding among the general population, mothers and health professionals is needed.

Parenteral nutrition and immature neonates. Comparative study of neonates weighing under 1000 and 1000-1250 g at birth.

UNLABELLED: We studied the nutritional requirements of 53 neonates with a birth-weight of 1250 g or less and analysed the parenteral and enteral nutrition provided, the weight-gain curves, the incidence of prior pathology and complications. We compared those weighing under 1000 g at birth (n=25) with those weighing 1001-1250 g (n=28). All neonates received central parenteral nutrition at an average age of 42.3 h. The liquid requirements of the lower birth-weight group were significantly greater. No differences were found in the supply of glucose, proteins, lipids and calories until after the first 15 days of life, when the <1000 g group required a greater liquid and caloric intake. Parenteral nutrition was suspended earlier for the >1000 g group (32.6 vs. 48.1 days). Maximum weight loss (12.56%) for the two groups occurred at 5.23 days. No differences in weight gain (g/kg/day) between the groups were observed. The >1000 g group began enteral nutrition significantly earlier and presented greater tolerance. The incidence of complications (bronchopulmonary dysplasia, enterocolitis, nosocomial sepsis, Candidas A sepsis, osteopenia) was greater in the lower birth-weight group, as was that of hyaline membrane disease and mechanically assisted respiration. There were no differences in the incidence of intracraneal haemorrhage, ductus arteriosus, early sepsis, delayed intrauterine growth or hypoglucemia. CONCLUSIONS: The severity of the initial pathology and the greater incidence of complications among the lower birth-weight neonates (<1000 g) influenced both the need for parenteral nutrition and the reduced tolerance to enteral nutrition. Although the rate of weight gain was similar for the two groups, the <1000 g group required a longer period of parenteral nutrition.