Correction to: Paediatric intensive care admission blood pressure and risk of death in 30,334 children.

The authors of the article entitled "Paediatric Intensive Care admission blood pressure and risk of death in 30,334 children" inform that due to an error in their database extraction, the following corrections to the data published should be notified.

Severe meningococcal disease is characterized by early neutrophil but not platelet activation and increased formation and consumption of platelet-neutrophil complexes.

Approximately 25% of polymorphonuclear leukocytes (PMNL) circulate in heterotypic complexes with one or more activated platelets. These platelet-neutrophil complexes (PNC) require platelet CD62P expression for their formation and represent activated subpopulations of both cell types. In this study, we have investigated the presence, time course, and mechanisms of PNC formation in 32 cases of severe pediatric meningococcal disease (MD) requiring intensive care. There were marked early increases in PMNL CD11b/CD18 expression and activation, and reduced CD62L expression compared with intensive care unit control cases. Minimal platelet expression of the active form of alphaIIbbeta3 (GpIIb/IIIa) was seen. PNC were reduced on presentation and fell to very low levels after 24 h. Immunostaining of skin biopsies demonstrated that PNC appear outside the circulation in MD. In vitro studies of anticoagulated whole blood inoculated with Neisseria meningitidis supported these clinical findings with marked increases in PMNL CD11b/CD18 expression and activation but no detectable changes in platelet-activated alphaIIbbeta3 or CD62P expression. In vitro PMNL activation with N. meningitidis (or other agonists) potentiated the formation of PNC in response to platelet activation with adenine diphosphate. Therefore, in severe MD, PMNL activation is likely to promote PNC formation, and we suggest that the reduced levels of PNC seen in established MD reflect rapid loss of PNC from the circulation rather than reduced formation.

Current management and outcome of tracheobronchial malacia and stenosis presenting to the paediatric intensive care unit.

OBJECTIVE: To identify factors associated with mortality and prolonged ventilatory requirements in patients admitted to our paediatric intensive care unit (PICU) with tracheobronchial malacia and stenosis diagnosed by dynamic contrast bronchograms. DESIGN: Retrospective review. SETTING: Tertiary paediatric intensive care unit. PATIENTS: Forty-eight cases admitted to our PICU over a 5-year period in whom a diagnosis of tracheobronchial malacia or stenosis was made by dynamic contrast bronchography (1994-1999). INTERVENTIONS: Conservative management, tracheostomy and long-term ventilation, surgical correction, internal or external airway stenting. MEASUREMENTS AND RESULTS: Recording of clinical details, length of invasive ventilation and appearance at contrast bronchography. Five groups of patients were defined: isolated primary airway pathology (n = 7), ex-premature infants (n = 11), vascular rings (n = 9), complex cardiac and/or syndromic pathology (n = 17) and tracheo-oesophageal fistulae (n = 4). The overall mortality was 29%. Median length of invasive ventilation in survivors was 38 days and in patients who died 45. Mortality was highest in the patients with complex cardiac and/or syndromic pathology (p = 0.039 Cox regression analysis) but was not related to any other factor. Patients with stenosis required a significantly longer period of ventilatory support (median length of ventilation 59 days) than patients with malacia (39 days). CONCLUSIONS: Length of ventilation and bronchographic diagnosis did not predict survival. The only factor found to contribute significantly to mortality was the presence of complex cardiac and/or syndromic pathology. However, patients with stenosis required longer ventilatory support than patients with malacia.

Congenital primitive neuroectodermal tumour presenting as obstructed labour.

Brain tumours presenting at delivery are extremely rare. A case of primitive neuroectodermal tumour (PNET) that presented intrapartum with failure to progress due to hydrocephalus is reported. Diagnosis required imaging with magnetic resonance and computed tomography in addition to open biopsy.

Enterovirus myocarditis as a cause of neonatal collapse.

Seven neonates required intensive care at our institution with enterovirus myocarditis, 2001-2003. Presentation was at a median age of 9 days. All had ischaemic electrocardiograms, poor ventricular function, raised creatine kinase, and enterovirus RNA detected by reverse transcriptase polymerase chain reaction. Four survived. Enterovirus myocarditis may be an under recognised cause of neonatal collapse.

The deep breath vasoconstriction reflex--a new tool for autonomic assessment in infancy?

We describe the presence of the deep breath vasoconstriction reflex in the neonatal and postneonatal period in a group of 24 infants, of whom 11 were term and 13 were preterm. Our results suggest that the development of this reflex correlates to postnatal rather than postconceptional age. The reflex is suggested for future use in autonomic assessment of babies to define normal and abnormal development and in the assessment of infants thought to be at risk of Sudden Infant Death Syndrome (SIDS).

Emergency management of children with severe sepsis in the United Kingdom: the results of the Paediatric Intensive Care Society sepsis audit.

OBJECTIVE: To audit current UK practice of the management of severe sepsis in children against the 2002 American College of Critical Care Medicine/Pediatric Advanced Life Support (ACCM-PALS) guideline. DESIGN: Prospective observational study. SETTING: 17 UK paediatric intensive care units (PICUs) and two UK PICU transport services. PARTICIPANTS: 200 children accepted for PICU admission within 12 h of arrival in hospital, whether or not successfully transported to a PICU, with a discharge diagnosis of sepsis or suspected sepsis. MAIN OUTCOME MEASURES: Medical interventions, physiological and laboratory data to determine the presence or absence of shock, inter-hospital transfer times, predicted mortality (using the Paediatric Index of Mortality, version 2 (PIM2) scoring system) and observed mortality. RESULTS: 34/200 (17%) children died following referral. Although children defined as being in shock received significantly more fluid (p<0.001) than those who were not in shock, overall fluid and inotrope management suggested by the 2002 ACCM-PALS guideline was not followed in 62% of shocked children. Binary logistic regression analysis demonstrated that the odds ratio for death, if shock was present at PICU admission, was 3.8 (95% CI 1.4 to 10.2, p = 0.008). CONCLUSIONS: The presence of shock at PICU admission is associated with an increased risk of death. Despite clear consensus guidelines for the emergency management of children with severe sepsis and septic shock, most children received inadequate fluid resuscitation and inotropic support in the crucial few hours following presentation.

The best interests test at the end of life on PICU: a plea for a family centred approach.

Approximately 80% of deaths in paediatric intensive care units occur as a result of a decision for limitation or withdrawal of life sustaining treatment, which in English law must be made with reference to the best interests of the child. However, as factors included in the best interests assessment depend on the subjective view of the decision maker, the test is not objective. Furthermore, the test is individualistic, in that only the child's interests are considered, ignoring the interests of others. Additionally, there is an element of absurd as only those who are alive can be said to possess interests and any decision resulting in death will result in loss of all interests along with loss of life. These criticisms could be largely addressed by taking into account the interests of others, particularly the interests of close family members.

Demystified...adhesion molecule deficiencies.

The basic physiology of leucocyte emigration from the intravascular space into the tissues is now known to be dependent on a class of cell surface molecules that have come to be known as adhesion molecules. Many cell-cell interactions are dependent on adhesion and signal transduction via the various adhesion molecules, particularly the integrins. The study of the functions of these molecules has been enhanced by the development of blocking and activating monoclonal antibodies, knockout mice, and by the rare "experiments of nature" in the human population, in whom there is absence or dysfunction of one of the adhesion molecules. This review describes these leucocyte adhesion defects and discusses how they have provided important insights into the function of these molecules.

Open lung biopsy in neonatal and paediatric patients referred for extracorporeal membrane oxygenation (ECMO).

BACKGROUND: This study was undertaken to determine the usefulness, safety, and most appropriate timing of open lung biopsy in infants and children considered for and on extracorporeal membrane oxygenation (ECMO) for respiratory failure. METHODS: A retrospective review of children referred for consideration of and placed on ECMO in our institution in the period 1996-2002. RESULTS: 506 patients were referred, 15 (3%) of whom underwent antemortem open lung biopsy (eight neonatal, four paediatric, and three cardiac patients). In the neonatal group open lung biopsy contributed to clinical decision making in all patients. Four neonates had a fatal lung dysplasia (three alveolar capillary dysplasia and one surfactant protein B deficiency) and treatment was withdrawn. Of the other four neonates, two had pulmonary hypoplasia, one had pulmonary lymphangiectasia, and one had meconium aspiration with mild barotrauma. Treatment was continued in these four patients and two survived. In the paediatric group the biopsies were of clinical relevance in two infants with pertussis who had lung infarction on biopsy in whom treatment was withdrawn. In the other two paediatric patients the biopsies were equivocal, treatment was continued, but both patients died. In the cardiac group, who presented perioperatively with pulmonary hypertension, the biopsies excluded a fatal lung dysplasia and severe pulmonary vascular disease but all three infants died. One patient had non-fatal bleeding complications. CONCLUSION: Open lung biopsy is clinically most useful when performed to diagnose fatal lung dysplasias in neonates and to confirm the presence of viable lung tissue in patients with acute lung injury due to pertussis infection.

Thermal entrainment of heart rate in the preterm infant.

Using spectral analysis we have studied changes in the heart rate during periodic thermal stimulation of one foot of infants during quiet sleep. Twenty-two appropriately grown preterm infants were studied in the first 15 d after birth to quantify responses in comparison with previously reported term infants. Babies were stimulated at 0.05, 0.10, and 0.15 Hz. Spectral power was calculated at the stimulus frequency +/-0.01 Hz and +/-0.02 Hz and over the low frequency range 0.03 Hz to 0.17 Hz. The data show that 1) there is an increase in power around the frequency of stimulation for each frequency studied (p < 0.002); and 2) there is an increase in the ratio of local to low frequency power at 0.05 Hz (p = 0.002) and 0.10 Hz (p = 0.001), but not at 0.15 Hz (p = 0.109). These data confirm the concept of entrainment in the appropriately grown preterm infant but demonstrate that it occurs over a wider frequency range than previously reported. The wider range is the same as that of the term infant, although there are differences in the patterns of entrainment between the two groups. Further work is required to map out the maturation of the autonomic nervous system in both the term and the preterm infant with respect to the low frequency components of the heart rate variability power spectrum.

The effect of early inhaled budesonide on pulmonary inflammation in infants with respiratory distress syndrome.

UNLABELLED: The aim of this pilot study was to determine the effect of early inhaled budesonide on clinical and inflammatory parameters in preterm infants ventilated for respiratory distress syndrome. CONCLUSION: Neither the inflammatory process associated with respiratory distress syndrome nor the progression to bronchopulmonary dysplasia appeared to be altered by treatment with inhaled budesonide.

Absence of platelet CD40L identifies patients with X-linked hyper IgM syndrome.

CD40 ligand (CD40L), a membrane protein expressed on activated T cells, plays a pivotal role in B cell proliferation and differentiation. Mutations in the CD40L gene are associated with a rare immunodeficiency state, X-linked hyper IgM syndrome (XLHIGM). Recently, platelets have been described as capable of expressing CD40L within minutes of stimulation. We have developed a rapid technique to determine expression of CD40L on activated platelets by flow cytometry in whole blood. We have demonstrated that this technique is useful in neonatal screening, in rapid diagnosis and in determining reconstitution by donor bone marrow post-transplantation.