David Schell

Improved outcomes of children with malignancy admitted to a pediatric intensive care unit.

ObjectiveTo assess the acute and long-term outcomes of children admitted to the intensive care unit with cancer or complications after bone marrow transplantation. DesignRetrospective analysis of databases from a prospective pediatric intensive care unit (PICU) database supplemented by case notes review. SettingA PICU in a tertiary pediatric hospital. PatientsAll children with malignancy admitted to the PICU between May 1, 1987, and April 30, 1996. InterventionsNone. Measurements and Main ResultsThere were 206 admissions to the PICU during a 9-yr study period of 150 children with malignancies or complications after bone marrow transplantation. Forty patients died in the PICU (27% mortality rate). The most frequent indications for PICU admission were shock and respiratory disease. Of 56 children admitted with shock, there were 16 deaths (29% mortality rate). In 24 episodes of sepsis, inotropic and ventilatory support were required and 13 patients (54%) survived. Analysis of long-term survival gave estimates of 50% survival for all oncology patients admitted to the PICU and 42% for those admitted for shock. ConclusionsA high proportion of oncology patients admitted to the PICU requiring intensive intervention survive and go on to be cured of their malignancy. Our study suggests the PICU outcome for these patients has improved.

Continuous venovenous haemofiltration in the acute treatment of inborn errors of metabolism

The accumulation of toxic metabolites in children with inborn errors of metabolism may cause acute metabolic crises and result in long-term neurological dysfunction or death. Peritoneal dialysis often provides insufficient clearance to protect against these complications, while intermittent haemodialysis cannot prevent reaccumulation of metabolites between dialysis sessions. We describe the use of continuous venovenous haemofiltration (CVVH) or haemodiafiltration (CVVHD) in three infants with maple syrup urine disease (MSUD) and one child with carbamyl phosphate synthetase (CPS) deficiency. All children with MSUD had a satisfactory reduction in branchedchain amino acids within 24 h of onset of haemofiltration, and are now neurologically normal. The child with CPS deficiency had an ammonia level of <100 μmol/l within 24 h of onset of therapy, but died 3 days later from unrelated cardiovascular complications. Complications of the therapy included the clotting of one haemofilter and the replacement of two vascular access catheters per patient on average per therapy. Two patients required blood transfusion. We report the successful use of CVVH and CVVHD in the acute management of metabolic crises associated with inborn errors of metabolism, and believe that these may be the optimal techniques for the acute clearance of toxic metabolites.

Indirect calorimetry in mechanically ventilated children: a new technique that overcomes the problem of endotracheal tube leak.

OBJECTIVES To develop indirect calorimetry to enable measurement of energy expenditure in mechanically ventilated children and to assess the effect of endotracheal tube leak on the accuracy of indirect calorimetry measurements. DESIGN Prospective, observational study, using a convenience sample. SETTING Tertiary pediatric intensive care unit in a university-associated childrens hospital. PATIENTS Eighteen patients, 3 months to 10 yrs of age, with various diagnoses, and requiring mechanical ventilation. INTERVENTIONS Patients were intubated and received routine intensive care treatment. MEASUREMENTS AND MAIN RESULTS Energy expenditure and respiratory quotient were measured using a new modification to the technique of indirect calorimetry, which includes an assessment of any expired gas lost around the endotracheal tube. Mean energy expenditure was 97% of predicted energy expenditure, but there was great variability between patients, and energy expenditure could not be estimated reliably from predictive equations. The amount of expired gas lost because of gas leak around the endotracheal tube was often a clinically important proportion of total expired gas, and this lost gas could not be predicted by audible endotracheal tube leak. CONCLUSIONS Measurement of energy expenditure by indirect calorimetry may be useful in the nutritional management of critically ill children. Results may be inaccurate if the gas lost because of leak around uncuffed endotracheal tubes is not taken into account.

The Capability of Static and Dynamic Features to Distinguish Competent From Genuinely Expert Practitioners in Pediatric Diagnosis

Children’s Hospital at Westmead, Sydney, Australia Objective: The authors describe the development of a new, more objective method of distinguishing experienced competent nonexpert from expert practitioners within pediatric intensive care. Background: Expert performance involves the acquisition and use of refined feature-event associations (cues) in the operational environment. Competent nonexperts, although experienced, possess rudimentary cue associations in memory. Thus, they cannot respond as efficiently or as reliably as their expert counterparts, particularly when key diagnostic information is unavail- able, such as that provided by dynamic cues. Method: This study involved the application of four distinct tasks in which the use of relevant cues could be expected to increase both the accuracy and the efficiency of diagnostic performance. These tasks included both static and dynamic stimuli that were varied systematically. A total of 50 experienced pediatric intensive staff took part in the study. Results: The sample clustered into two levels across the tasks: Participants who performed at a consistently high level throughout the four tasks were labeled experts, and participants who performed at a lower level throughout the tasks were labeled competent nonexperts. The groups differed in their responses to the diagnostic scenarios presented in two of the tasks and their ability to maintain performance in the absence of dynamic features. Conclusion: Experienced pediatricians can be decomposed into two groups on the basis of their capacity to acquire and use cues; these groups differ in their diagnostic accuracy and in their ability to maintain performance in the absence of dynamic features. Application: The tasks may be used to identify practitioners who are failing to acquire expertise at a rate consistent with their experience, position, or training. This information may be used to guide targeted training efforts.

Giant hemangioma of the arm associated with cardiac failure and the Kasabach-Merritt syndrome in a neonate.

We report a neonate who presented within hours of birth with severe congenital cardiac failure, thrombocytopenia, and consumption coagulopathy, caused by a massive hemangioma of the left arm. Initial treatment with glucocorticoids, platelet and clotting factor replacements, and cardiovascular support failed to control these hemangioma effects and amputation was avoided only when axillary artery ligation and an intermittent pneumatic compression device, manufactured in this hospital, achieved control of this lesion and hastened its subsequent resolution. The treatment of such lesions is reviewed, emphasizing individualized treatment protocols, and stressing that such lesions and their effects, cannot be regarded as variants of a single disease entity when planning management.

Pattern Recognition as an Indicator of Diagnostic Expertise

Expertise is typically associated with high levels of experience in a domain. However, high levels of experience do not necessarily mean that operators are capable of performing at the level of expertise. Based on evidence that pattern-recognition is the foundation of expert diagnostic performance, two studies investigated the utility of distinguishing competent from expert practitioners using measures of the component tasks of pattern-recognition. In two dissimilar domains, performance across the tasks clustered into two levels, reflecting competence and expertise. Performance on the tasks was only weakly correlated with years of experience in the domain. The significance of these results is discussed in relation to assessment and training evaluation.

Humidified high-flow nasal cannula oxygen in bronchiolitis reduces need for invasive ventilation but not intensive care admission

To describe the changes to paediatric intensive care unit (PICU) admission patterns and ventilation requirements for children with bronchiolitis following the introduction of humidified high‐flow nasal cannula oxygen outside the PICU.

Traumatic bleeding at birth treated with Factor VII

We report 36 week gestation twins born following a traumatic delivery. Twin 2 had profuse haemorrhage where haemostasis was achieved with recombinant Factor VIIa (rFVIIa – NovoSeven®;Novo Nordisk A/S, Bagsværd, Denmark)