SignificanceMaintaining low CO2 levels in our bodies is critical for life and depends on neurons that generate the respiratory rhythm and monitor tissue gas levels. Inadequate response to increasing levels of CO2 is common in congenital hypoventilation diseases. Here, we identified a mutation in LBX1, a homeodomain transcription factor, that causes congenital hypoventilation in humans. The mutation alters the C terminus of the protein without disturbing its DNA-binding domain. Mouse models carrying an analogous mutation recapitulate the disease. The mutation spares most Lbx1 functions, but selectively affects development of a small group of neurons central in respiration. Our work reveals a very unusual pathomechanism, a mutation that hampers a small subset of functions carried out by a transcription factor.

Background: A study was undertaken to evaluate the efficacy of dexamethasone in patients mechanically ventilated for lower respiratory infection caused by respiratory syncytial virus (RSV-LRTI). Methods: In a multicentre randomised controlled trial patients were randomised to receive either intravenous dexamethasone (0.15 mg/kg 6 hourly for 48 hours) or placebo. End points were the duration of mechanical ventilation, length of stay (LOS) in the pediatric intensive care unit (PICU) and in hospital, and the duration of supplemental oxygen administration. Results: Thirty seven patients received dexamethasone and 45 received placebo. There was no significant difference in any of the end points between the two groups. In a post hoc analysis patients were stratified into those with mild gas exchange anomalies (PaO 2 /FiO 2 >200 mm Hg and/or mean airway pressure < 10 cm H 2 O, bronchiolitis group) and those with severe gas exchange anomalies (PaO 2 /FiO 2 <200 mm Hg and mean airway pressure >10 cm H 2 O, pneumonia group). In the 39 patients with bronchiolitis the duration of mechanical ventilation was 4.3 days shorter in the dexamethasone group than in the placebo group (4.9 v 9.2 days, 95% CI -7.8 to -0.8, p=0.02) and the duration of supplemental oxygen was 3.6 days shorter (7.7 v 11.3 days, 95% CI -8.0 to -0.1, p=0.048). No differences in end points were found in the pneumonia group. Conclusions: Dexamethasone had no beneficial effect in patients mechanically ventilated for RSV-LRTI but was found to have a beneficial effect in patients with bronchiolitis.

Parents', physicians', and nurses' perceptions of suffering overlap but also show important differences. Differences found seem to be rooted in the relation to and kind of responsibility (parental/professional) for the child. The child's illness, suffering, and hospital admission cause suffering in parents. Health-care professionals in PICUs need to be aware of these phenomena.

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Osteomyelitis due to Proteus mirabilis is rare. Spinal osteomyelitis caused by this organism has only been described in adults. This is the first paediatric case of P. mirabilis vertebral osteomyelitis.

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A ventilator-dependent child had been in the paediatric intensive care unit (PICU) ever since birth. As a result, she had fallen behind considerably in her development.After 18 months, continuous positive airway pressure was successfully administered via a tracheostomy tube with a novel lightweight device. This enabled her to walk in the PICU. With this device, the child was discharged home where she could walk with an action range of 10 m. Subsequently, her psychomotor development improved remarkably.To the authors' knowledge, this is the first case report of a patient, adult or paediatric, who could actually walk with a sufficient radius of action while receiving long-term respiratory support.

Results For 72 children (31 girls, 41 boys), 91 decision-making meetings were organised. We identified 27.7% (20/72) disagreements or conflicts: 4 simple disagreements, 12 continuing disagreements and 4 conflicts. Five children had acute disease and 15 children had chronic disease. Source of disagreements was continuing LST in 19 cases (families wanted to continue aggressive treatment). In 1 case, the family wanted to stop treatments despite medical opinion (refusal of tracheotomy). Consequences of theses disagreements were continuation of treatments despite LST decisions in 12 cases. For 3 cases a compromise solution was found. Conclusion Disagreements are frequent in decisions to forgo LST (27,7%) and most of the child undergo treatments that are medically futile. Background and aims In a previous study on suffering of children during admission to a paediatric intensive care unit (PICU), we found that parents described suffering of their child mainly in relation to physical symptoms. In this study we evaluated if these children still have signs of suffering four years after the PICU admission and if the symptoms of suffering, as perceived by the parents, are different compared to the PICU period. Methods A structured audio taped interview with 15 parents of children four years after admission to a 20 bed level III PICU of a university teaching hospital to assess whether their child perceived to suffer and to identify perceived aspects of suffering. Results About 50% of the parents experienced 4 years after PICU admission suffering in their child. Parents of 8 children did not perceive suffering in their child. Parents indicated that the suffering during the PICU admission was due to physical and psychosocial factors. Psychosocial factors were related to the disease causing the admission to the PICU, the treatment and the hospital stay. Four years later the signs of suffering are related to communication, physical and mental retardation and being different from mates. Conclusions A child's admission to a PICU and its suffering not only cause suffering in the child during admission, but often suffering is still present four years after admission. Caregivers in paediatrics need to be aware of these perceived symptoms. In long-term follow up of critically ill children this phenomena needs attention. PO-0319 IS THERE SUFFERING IN CHILDREN FOUR YEARS AFTER