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Introduction: Invasive mechanical ventilation contributes to ventilator-induced diaphragmatic dysfunction, delaying extubation and increasing mortality in adults. Despite the possibility of having a higher impact in paediatrics, this dysfunction is not routinely monitored. Diaphragm ultrasound has been proposed as a safe and non-invasive technique for this purpose. The aim of this study was to describe the evolution of diaphragmatic morphology and functional measurements by ultrasound in ventilated children., Material and Methods: Prospective exploratory study. Children admitted to Paediatric Intensive Care Unit requiring mechanical ventilation > 48 hours were included. The diaphragmatic thickness, excursion and the thickening fraction were assessed by ultrasound., Results: Seventeen cases were included, with a median age of 42 months. Ten were male, seven had comorbidities and three in seventeen had malnutrition at admission. The median time under mechanical ventilation was seven days. The median of the initial and minimum diaphragmatic thickness was 2.3 mm and 1.9 mm, respectively, with a median decrease in thickness of 13% under pressure-regulated volume control. Diaphragmatic atrophy was observed in 14/17 cases. Differences in the median thickness variation were found between patients with sepsis and without (0.70 vs 0.25 mm; p = 0.019). During pressure support ventilation there was a tendency to increase diaphragmatic thickness and excursion. Extubation failure occurred for diaphragmatic thickening fraction ≤ 35%., Discussion: Under pressure-regulated volume control there was a tendency for a decrease in diaphragmatic thickness. In the pre-extubation stage under pressure support, there was a tendency for it to increase. These results suggest that, by titrating ventilation using physiological levels of inspiratory effort, we can reduce the diaphragmatic morphological changes associated with ventilation., Conclusion: The early recognition of diaphragmatic changes may encourage a targeted approach, namely titration of ventilation, in order to reduce ventilator-induced diaphragmatic dysfunction and its clinical repercussions.

Introduction: Acute liver failure is a rare disorder associated to high morbidity and mortality despite survival improvement through liver transplantation. The importance of a multidisciplinary approach and early referral to a pediatric liver transplantation center were important conclusions of a national meeting in 2008, from which resulted an actuation consensus., Objectives: To characterize acute liver failure admissions in a Pediatric Intensive Care Unit of the portuguese pediatric livertransplantation center. To compare results before (A) and after (B) 2008., Material and Methods: Observational, retrospective study during a 20 year period (1994-2014)., Inclusion Criteria: age < 18 years old and acute liver failure (INR ≥ 2 without vitamin K response and hepatocellular necrosis). Children with previous liver disease were excluded., Results: Fifty children were included, with median age of 24.5 months. The most common etiology under 2 years old was metabolic (34.6%) and above that age was infectious (29.2%). Forty six percent were submitted to liver transplantation and 78% of them survived. Overall mortality was 34%. Median referral time was 7 days in period A (n = 35) and 2 days in period B (n = 15; p = 0.006). Pediatric risk of mortality's median was 14.7 in period A and 6.5 in B (p = 0.019). Mortality was 37% vs 26% in periods A and B, respectively (p = 0.474)., Discussion and Conclusions: Overall mortality was similar to the observed in other European centers. Liver transplantation is in fact the most effective therapeutic option. After 2008, there was a reduction in referral time and cases severity on admission; however, mortality has not reduced so far.

Introduction: Meningococcal infection has a high mortality and morbidity. Recently a new prognostic scoring system was developed for paediatric invasive meningococcal disease, based on platelet count and base excess â base excess and platelets score. The main objective of this study was to evaluate the accuracy of base excess and platelets score to predict mortality in children admitted to intensive care due to invasive meningococcal disease., Material and Methods: Observational study, with retrospective data collection, during a 13.5 years period (01/2000 to 06/2013). Mortality by invasive meningococcal disease and related factors (organ dysfunction and multi-organ failure) were analysed. The base excess and platelets score was calculated retrospectively, to evaluate its accuracy in predicting mortality and compared with Paediatric Risk of Mortality and Paediatric Index of Mortality2., Results: Were admitted 76 children with invasive meningococcal disease. The most frequent type of dysfunction was cardiovascular (92%), followed by hematologic (55%). Of the total, 47 patients (62%) had criteria for multi-organ failure. The global mortality was 16%. Neurologic and renal dysfunction showed the strongest association with mortality, adjusted odds ratio 315 (26 - 3 804) and 155 (20 - 1 299). After application of receiver operating characteristic curves, Base Excess and Platelets score had an area under curve of 0.81, Paediatric Index of Mortality2 of 0.91 and Paediatric Risk of Mortality of 0.96., Discussion: The Base Excess and Platelets score showed good accuracy, although not as high as Paediatric Risk of Mortality or Paediatric Index of Mortality2., Conclusions: The Base Excess and Platelets score may be useful tool in invasive meningococcal disease because is highly sensitive and specific and is objectively measurable and readily available at presentation.

Introduction: Meningococcal infection has a high mortality and morbidity in children. Aggressive initial shock approach, early referral, secondary transport and vaccination are potential factors with impact in reducing its mortality. Objectives were to characterize children admitted to intensive care due to invasive meningococcal disease, to evaluate their prognostic scores and mortality., Material and Methods: Observational study, with retrospective data collection. Two periods were created according to the year of admission (A: 2000-2005 and B: 2006-2011). Prognostic parameters, organ failure and mortality rates were compared in these groups., Results: 70 children were admitted with invasive meningococcal disease. When compared with other causes of admission, a decrease in the number of admissions due to invasive meningococcal disease was observed (period A: 3.4%; period B: 1.5%; p = 0.001). The presence of meningitis was 41% in period A and 29% in period B (p = 0.461). Rapidly progressive purpura occurred in 78% in period A and 50% in period B (p = 0.032). Children from period A had multi-organ failure (80%), disseminated intravascular coagulation (76%) and coma (22%) more frequently than children from period B (29%, 29%, 0%; p < 0.05). Mortality was 26% in period A and 0% in period B (p = 0.006) and standardized mortality by PRISM was 1.3 and 0 in period A and B respectively., Discussion: The decrease in the number of admissions due to invasive meningococcal disease can be explained by the introduction of anti-meningococcal C vaccine in 2006. Mortality decline can be possibly explained by an improvement in the initial patient stabilization and to secondary transport., Conclusion: A decrease in the number of admissions due to invasive meningococcal disease and in mortality was observed.