Editorial

Gentamicin is a frequently used antibiotic for neonates, yet there remains debate as to the most appropriate dose and the dosing intervals. In this issue, Dr. Pacifici reports a search of the literature to compare peak and trough concentrations following schedules of gentamicin 2.5 mg/kg twice daily, 4 mg/kg once daily and extended interval dosing (5 ml/kg every 48 hours). The literature search highlighted little was known about the peak and trough concentrations of extended interval dosing. Cystic fibrosis is estimated to affect 60,000 individuals worldwide. The gene responsive (CF Transmembrane Conductance Regulator), was discovered in 1989. In this issue, Conese and colleagues review studies of gene therapy vectors and strategies for delivering them more safely and efficiently. In addition, the results of novel interventions to improve mucociliary clearance including hypertonic saline mannitol and ENaC inhibitors are discussed. Deconinck and Stojkovic have described the typical clinical and biochemical findings of the collagenopathies - Ullrich congenital muscular dystrophy and Bethlem myopathy, to facilitate paediatricians identifying the collagenopathies within the complex diagnostic group of congenital dystrophies. In addition, they have outlined the current understanding of the pathogenesis, management and potential therapeutic avenues. Many chronic paediatric diseases arise from early life immune insults and postnatal immune dysfunction. As a consequence, to further improve the understanding of the nature of the immune dysfunction and the reported associated health risks, Dietert and Zelikoff have described paediatric immune dysfunctions produced by well studied immunotoxicants and provide a matrix of health risks which appear to be linked together via underlying paediatric immune dysfunction. Gisser and colleagues describe a rare case of upper gastrointestinal bleeding that is a Schwannoma, which is a rare gastrointestinal mesenchymal tumour occurring most commonly in the stomach as a nodular mass in the muscularis propria. They highlight that they are benign tumours with a good prognosis, even when treated with enucleation. Idopathic nephrotic syndrome has a reported incidence of between two and seven cases per 100,000 children. There are two distinct histological variants: minimal change nephrotic syndrome and focal segmental glomerulosclerosis. Kaneko in this issue discusses the pathogenesis of idiopathic nephrotic syndrome both by reviewing the literature and experimental data.