European journal of pediatrics
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Chronic relentless lung infection and pancreatic insufficiency are the cardinal features of cystic fibrosis (CF), a life-shortening autosomal recessive disease. Mutations in the 'cystic fibrosis transmembrane conductance regulator' (CFTR) are currently classified into five groups according to their repercussion on CFTR protein synthesis and its chloride channel function. Stop codon mutations (class I) result in a truncated nonfunctional CFTR, class II mutations consist of aberrantly folded CFTR protein that is degraded by the cell quality control system, while class III mutations lead to defective regulation of the CFTR protein and, consequently, the absence of CFTR function. ⋯ The positive effect of nebulised hypertonic saline on mucociliary clearance is based on the restoration of ASL height. Recent advances in the current treatment of lung infection and inflammation are highlighted in this review. Lung transplantation should be considered in terminally ill patients, but the timing of the transplantation is crucial: transplanting too early shortens survival, while transplanting too late results in patients dying on the waiting list.
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Piritramide is a synthetic opioid commonly used in Germany and Austria for the analgesia of pediatric patients. Little pharmacokinetic and pharmacodynamic data for the pediatric population is available. The aim of this investigation was to gain pharmacodynamic data on postsurgical analgesia and the side effects of piritramide. ⋯ We conclude that dosages of more than 50-70 microg/kg are needed for sufficient analgesia in ventilated postsurgical infants. In spontaneously breathing patients, 50-70 microg/kg provides a 120-min period of analgesia for more than 50% of patients. Cardiovascular stability of the patients was good and, with one exception, there was no respiratory depression.