Trasplante pediátrico pulmonar / Pediatric lung transplantation

El trasplante pulmonar constituye la última alternativa para los niños con una enfermedad pulmonar terminal cuando se han agotado todo los recursos terapéuticos. En las dos últimas décadas se ha experimentado una vance consideable en la supervivencia de lso niños trasplantados de pulmón, por las mejoras en la técnica del trasplante, la preervación de los órganos, el manejo perioperatorio y la inmunosupresión. El programa de trasplatne pulmonar pediátrico en el Hospital Universitari Vall d´Hebron se inició en el año 1996. En nuestro hospital se han realizado 672 trasplantes entre los años 1990 y 2012, de los que 51 (7,6%) corresponde a trasplantes pediátricos (menores de 18 años). La indicación más frecuente para el trasplante en los iños es la fibrosis quística. En nuestro centro, la fibrosis quística ha representado solo el 8,7% de los traplantes realizados enmenores de 11 años y el 86% de los realizados entre 12 y 17 años. Los otros grupos importantes de indicaciones los constituyen las enfermedades vasculares pulmonares (hipertensión pulmonar) y las neumopatías intersticiales crónicas. La superviviencia de los niños trasplantados en nuestro entro es similar a la reflejada en el registro internacional: 55% a los 5 años y 47% a los 7 años (AU)

Lung trasplantation has become an accepted therapeutic option for children with end-stage lung disease. Advances have been made over the last 2 decades in lung transplantation concerning the procurement and presevation of donor lung, surgical techniques, early post-operative care and immunosuppression. The pediatric lung transplant program at Hospital Universitari Vall d´Heborn was started in 1996. A total of 672 lung tansplants have been performed in our hospital since 1990, with 51 (7.6%) corresponding to children (younger than 18 years). Cystic fibrosis is the leading cause for pediatric patients to requere lung transplantation. In our hospital cystic fibrosis represent only 8.7% of lung transplants in children less tha 11 years-old, but 86% in children 12-17 years old. Other important indications for lung trnasplantation in children are pediatric forms of interstitial lung dieases and pulmonary vascular disorders (mainly pulmonary hypertension). Actuarial survival is our center (55% at 5 years and 47% at 7 years) is similar to survival published in the International Registry (AU)

[Socio-cultural, obstetric and anthropometric characteristics of newborn children of mothers who smoke in Spain]. / Características socioculturales, obstétricas y antropométricas de los recién nacidos hijos de madre fumadora.

INTRODUCTION: One in every four women smoke at the beginning of the pregnancy in Spain; of these, 25-50% give up smoking during gestation and most of them smoke again after childbirth. Maternal smoking has harmful effects on babies, such as low birth-weight with its resulting morbidity. The objective of this study is to compare the anthropometric and socio-cultural characteristics between newborns from smoking mothers and those from non- smoking ones. MATERIALS AND METHODS: Smoking habits and the socio-cultural characteristics were assessed from a sample of 1499 pregnant women, as well as their newborn anthropometric variables of their newborn babies during 2009 in the Aragon region of Spain. RESULTS: In the sample of 1499 pregnant women the prevalence of smoking during pregnancy was 19.6%; among the Spanish women, 23% of them smoked during the gestation compared with only 11% in women from other countries. The smoker mothers were younger and they had a lower educational level than the non smoker ones; however, there were no statistical differences between their anthropometric characteristics. Newborns from smoker women in Aragon had a lower weigh at delivery (3155 vs. 3295 grams: p<.001) and a relative risk of low weight at birth for their gestational age of 2.1 (95% CI: 1.35 - 2.97). The length and the rest of newborn anthropometric variables were also affected. CONCLUSION: The prevalence of smoking during gestation is high in Aragon. The mothers who smoke are younger and have different socio-cultural characteristics compared with non-smoker mothers. Maternal smoking habits have a harmful effect on newborn nutritional status, representing an avoidable risk of low weight at birth.

Síndrome de Gilbert: estudio de 12 observaciones y revisión de la bibliografía médica / Gilbert’s syndrome: a study of 12 observations and review of the literature

El síndrome de Gilbert es el hallazgo de hiperbilirrubinemia indirecta leve-moderada con pruebas de función hepática normales y sin signos de hemólisis. Es un trastorno hereditario del metabolismo de la bilirrubina, benigno, con una prevalencia mundial cercana al 10%. Tiene un patrón de herencia variable, con polimorfismo genético. Se diagnostica mediante pruebas confirmatorias de provocación, como la prueba del ayuno, aunque el diagnóstico definitivo es genético. Su pronóstico es bueno y actualmente se discute sobre los diversos efectos de la hiperbilirrubinemia. En este artículo, se revisan los casos diagnosticados de síndrome de Gilbert en el servicio de pediatría de un hospital universitario en los últimos años, y se describen las principales características halladas

Gilbert’s syndrome is characterized by a mild or moderate elevation of unconjugated bilirubin, with normal liver function and no evidence of hemolysis. It is a benign inherited disorder of bilirubin metabolism, with a worldwide prevalence of nearly 10%. It has a variable pattern of inheritance, with genetic polymorphism. Diagnosis is based on a confirmatory provocation test, such as the fasting test, although the definitive diagnosis requires a genetic study. The prognosis is good and, at the present time, the varied effects of hyperbilirubinemia are a matter of debate. The cases of Gilbert’s syndrome diagnosed in the pediatric service of a university hospital in recent years were reviewed and the main characteristics are described