[Neonatal outcome of triplets]. / Evolución neonatal de los recién nacidos trillizos (desde el nacimiento hasta el alta hospitalaria).

OBJECTIVE: To determine the neonatal morbidity and mortality in triplets. METHOD: Retrospective study of 189 triplets born between January'98 and December'04. RESULTS: Mean maternal age was 33 years. Of the pregnancies, 71.4 % were achieved using fertilization techniques, 84 % received antenatal steroids and 96.8 % of births were by caesarean section. The mean gestational age was 32 weeks (246-355), with a mean birth weight of 1,500 g (450-2,650). There 53.4 % were female neonates. Cardiopulmonary resuscitation (CPR) at birth was not required by 82 %, 13.5 % required bag-mask ventilation and 4.9 % required advanced CPR. Low/moderate respiratory distress syndrome was observed in 27 % of the infants (oxygen/CPAP), and 19 % required mechanical ventilation and surfactant. 12 % showed patent ductus arteriosus was seen in 12 %, necrotizing enterocolitis in 4.2 %, sepsis (vertical and nosocomial), 17 %, grade III retinopathy in 1.1 % and 7.4 % had bronchopulmonary dysplasia. Grade III/IV intraventricular haemorrhage was present in 4.2 %, and 3.2 % periventricular leukomalacia. Survival rate at discharge from hospital was 95.2 %. Of those, 10 % showed risk of serious sequelae defined as: grade III-IV intraventricular haemorrhage, periventricular leukomalacia, grade III retinopathy and bronchopulmonary dysplasia. There were no major complications in 64.5 % of the children. Despite a prematurity rate of 100 %, this large series of triplets shows an excellent survival and a relatively low serious associated morbidity. It is also important to point out the intensive perinatological follow-up to which these pregnancies are subject.

Evolución neonatal de los recién nacidos trillizos (desde el nacimiento hasta el alta hospitalaria) / Neonatal outcome of triplets

Objetivo: Determinar la morbilidad y la mortalidad neonatales de los trillizos. Método: Estudio retrospectivo de 189 trillizos nacidos entre enero de 1998 y diciembre de 2004. Resultados: La edad media materna fue de 33 años. El 71,4 % de las gestaciones se obtuvieron mediante técnicas de reproducción asistida. El 84 % recibió corticoides prenatales. El 96,8 % nació mediante cesárea. La edad gestacional media fue de 32 semanas (246-355), con peso medio de 1.500 g (450-2.650). El 53,4 % fueron niñas. El 82 % no necesitó reanimación al nacer, el 13,5 % requirió reanimación con bolsa autoinflable y el 4,9 %, reanimación avanzada. El 27 % de los neonatos presentaron síndrome de distrés respiratorio leve/moderado (oxígeno/CPAP) y el 19 %, grave (ventilación mecánica/agente tensiactivo). El 12 % presentó ductus arterioso persistente; el 4,2 %, enterocolitis; el 17 %, sepsis (vertical y nosocomial); el 1,1 %, retinopatía de grado III, y el 7,4 %, displasia broncopulmonar. El 4,2 % presentó hemorragia intraventricular (HIV) grado III-IV, y el 3,2 %, leucomalacia periventricular. La supervivencia al recibir el alta fue del 95,2 %, el 10 % mostraron signos de riesgo de secuelas graves, definidas como: HIV grado III-IV, leucomalacia periventricular, retinopatía grado III y displasia broncopulmonar. El 64,5 % de los neonatos no presentaron ninguna complicación destacable durante su ingreso. Aunque la incidencia de prematuridad es del 100 %, esta serie larga de trillizos muestra una excelente supervivencia y una morbilidad grave asociada relativamente baja. Destaca el elevado control perinatológico de estas gestaciones (AU)

Objective: To determine the neonatal morbidity and mortality in triplets. Method: Retrospective study of 189 triplets born between January'98 and December'04. Results: Mean maternal age was 33 years. Of the pregnancies, 71.4 % were achieved using fertilization techniques, 84 % received antenatal steroids and 96.8 % of births were by caesarean section. The mean gestational age was 32 weeks (246-355), with a mean birth weight of 1,500 g (450-2,650). There 53.4 % were female neonates. Cardiopulmonary resuscitation (CPR) at birth was not required by 82 %, 13.5 % required bag-mask ventilation and 4.9 % required advanced CPR. Low/moderate respiratory distress syndrome was observed in 27 % of the infants (oxygen/CPAP), and 19 % required mechanical ventilation and surfactant. 12 % showed patent ductus arteriosus was seen in 12 %, necrotizing enterocolitis in 4.2 %, sepsis (vertical and nosocomial), 17 %, grade III retinopathy in 1.1 % and 7.4 % had bronchopulmonary dysplasia. Grade III/IV intraventricular haemorrhage was present in 4.2 %, and 3.2 % periventricular leukomalacia. Survival rate at discharge from hospital was 95.2 %. Of those, 10 % showed risk of serious sequelae defined as: grade III-IV intraventricular haemorrhage, periventricular leukomalacia, grade III retinopathy and bronchopulmonary dysplasia. There were no major complications in 64.5 % of the children. Despite a prematurity rate of 100 %, this large series of triplets shows an excellent survival and a relatively low serious associated morbidity. It is also important to point out the intensive perinatological follow-up to which these pregnancies are subject (AU)

Cooperación internacional pediátrica en situación de catástrofes / International cooperation among pediatric agencies in disaster situations

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[Castleman disease]. / Enfermedad de Castleman.

Castleman disease or angiofollicular hyperplasia is a rare disorder included in the group of lymphoproliferative disorders. This entity was originally described by Castleman in 1956. The etiology remains unknown but it is postulated to be a reactive lymphoid hyperplasia due to chronic antigenic stimulation caused by a viral infection. The disease presents in young adults and is more frequent in women; it is exceptionally rare in the pediatric age group. It is classified into two clinical groups (localized disease and disseminated disease) and there are two histologic variants (hyaline-vascular and plasma cell Castleman disease). Localized disease is usually asymptomatic, has a good prognosis, and is the most common presentation in pediatric patients, usually corresponding to highly vascularized mediastinal masses. Resection of the mass, which is curative, is associated with a high risk of blood loss. Recently, preoperative arteriography with embolization has been used satisfactorily in the preoperative management of these tumors. We present a case of localized Castleman disease in a 12-year-old girl satisfactorily treated with embolization before curative resection.

Enfermedad de Castleman / Castleman disease

La enfermedad de Castleman o hiperplasia angiofolicular es una entidad poco frecuente enmarcada dentro de los trastornos linfoproliferativos. Esta enfermedad fue descrita inicialmente por Castleman en 1956. Su etiología es desconocida, pero se cree que responde a una estimulación antigénica crónica de probable etiología viral. Se presenta en adultos jóvenes, más frecuente en mujeres, y es excepcional en la edad pediátrica. Clínicamente se distinguen dos formas de enfermedad: una localizada y otra multicéntrica. La forma localizada, de buen pronóstico, asintomática al diagnóstico, es la forma de presentación más frecuente en la edad pediátrica y por lo general corresponde a masas mediastínicas muy vascularizadas. La resección de la lesión, que es curativa, se asocia a un alto riesgo de sangrado. Recientemente se ha introducido la arteriografía con embolización prequirúrgica para minimizar el riesgo de sangrado en el acto quirúrgico. Se presenta un caso de enfermedad de Castleman localizada en una paciente de 12 años en la que se utilizó con éxito la angioembolización prequirúrgica

Castleman disease or angiofollicular hyperplasia is a rare disorder included in the group of lymphoproliferative disorders. This entity was originally described by Castleman in 1956. The etiology remains unknown but it is postulated to be a reactive lymphoid hyperplasia due to chronic antigenic stimulation caused by a viral infection. The disease presents in young adults and is more frequent in women; it is exceptionally rare in the pediatric age group. It is classified into two clinical groups (localized disease and disseminated disease) and there are two histologic variants (hyaline-vascular and plasma cell Castleman disease). Localized disease is usually asymptomatic, has a good prognosis, and is the most common presentation in pediatric patients, usually corresponding to highly vascularized mediastinal masses. Resection of the mass, which is curative, is associated with a high risk of blood loss. Recently, preoperative arteriography with embolization has been used satisfactorily in the preoperative management of these tumors. We present a case of localized Castleman disease in a 12-year-old girl satisfactorily treated with embolization before curative resection

[Glycogen storage disease type IX presenting as abdominal distention, hepatomegaly and hypoglycemia during infancy]. / Hepatomegalia, distensión abdominal e hipoglucemia en un lactante: expresión clínica de la glucogenosis tipo IX.

Glycogen storage diseases are a rare group of disorders in daily pediatric practice but must be taken into account when a patient presents with poor physical growth, hepatomegaly, hypoglycemia, hypotonia and/or other metabolic disturbances. Early diagnosis allows treatment that might improve the patient's outcome to be started or, at the very least, genetic counseling to be given to the parents. We present a 10-month-old boy who presented with growth retardation, abdominal distention and hepatomegaly and who was finally diagnosed with glycogenosis type IX. Definitive diagnosis was obtained by demonstrating the enzyme defect (phosphorylase beta-kinase) in affected tissues. Enteral nutrition was started using a diurnal high-carbohydrate diet with frequent feedings and nocturnal nasogastric continuous feeding, achieving optimal growth parameters and clinical response.

Hepatomegalia, distensión abdominal e hipoglucemia en un lactante: expresión clínica de la glucogenosis tipo IX / Glycogen storage disease type IX presenting as abdominal distention, hepatomegaly and hypoglycemia during infancy

Las glucogenosis son entidades poco frecuentes en la práctica diaria de un pediatra, pero deben tenerse en cuenta ante todo paciente que se presente con retraso del crecimiento, hepatomegalia, hipoglucemia, alteración del tono muscular y/o alteraciones en el estudio metabólico. La importancia de su diagnóstico precoz puede permitir iniciar un tratamiento con el cual poder mejorar el pronóstico del paciente o, en el peor de los casos, ofrecer a la familia un consejo genético adecuado. Se comenta el caso de un lactante de sexo masculino de 10 meses de edad que presentaba un cuadro de estancamiento ponderal, distensión abdominal y hepatomegalia. El diagnóstico de glucogenosis tipo IX se confirmó mediante la demostración de la ausencia de actividad enzimática de la fosforilasa -cinasa, enzima alterada en esta entidad. Se inició alimentación enteral mediante gastroclisis continua nocturna y dieta diurna rica en hidratos de carbono con buena respuesta clínica y recuperación de los parámetros somatométricos (AU)