tailieunhanh - Paediatric interstitial lung disease

The frequency of pain crises was reported as an outcome in five pediatric studies, with a reduction in frequency reported in three. In one retrospective cohort study in a resource-poor environment, the frequency of pain crises declined from a median of 3 per year to a median of per year during treatment, with a median followup time of 24 months. Of note is the fact that these results were obtained using a fixed dose of hydroxyurea (15 mg/kg/day). A small, high- quality prospective study found a decrease in pain events, from per year in the year prior to. | CHAPTER 17 Paediatric interstitial lung disease A. Bush . Nicholson Imperial College and Royal Brompton Hospital London UK. Correspondence A. Bush Dept of Paediatric Respiratory Medicine Royal Brompton Hospital Sydney Street London SW3 6NP UK. E-mail Interstitial lung disease ILD in children chILD is very different in many aspects to the adult disease. First chILD is rare estimated at per 100 000 compared with 6080 per 100 000 for ILD in adults 1 . Secondly the spectrum of conditions in particular in infancy is much wider than in adults. The conditions encompass growth and developmental issues as well as immunological problems. The consequence is that paediatricians are even less advanced than adult physicians when it comes to making diagnoses by radiology and bronchoalveolar lavage BAL and this combined with the rarity of the conditions means that there have been no randomised controlled trials of treatment. Thus chILD is very much work in progress. However chILD is a really important topic for adult chest physicians some of the paediatric diseases may in fact present in adult life and if diagnostic awareness is not heightened patients may disappear into a dustbin category such as usual interstitial pneumonia UIP . Furthermore rare genetic abnormalities may lead to an understanding of modifier genes important in adult ILD. In this regard it is a pity that recent guidelines saw fit to ignore chILD altogether 2 . This chapter will first review recent advances in the classification of ILD in children and then discuss presentation diagnosis and differential diagnosis as well as what little is known about treatment options. Classification of ILD in children There are two published classifications 3 4 and a third is still only reported as an abstract 5 . The definitive classification in the 0-2-yr age range is from North America 4 and this is recommended for adoption. The European Respiratory Society ERS Task Force also contained data in .