Inter-rater agreement in the evaluation of abdominal radiographs for necrotizing enterocolitis

Purpose: Abdominal radiographs are frequently employed in the surveillance of patients with necrotizing enterocolitis (NEC), with typical findings well described. Clinicians interpret and act upon these films at different intervals, however, and inter-rater agreement has not been evaluated to date. Methods: Thirty abdominal radiographs of premature infants were distributed to attending radiologists (4), pediatric surgeons (4), and trainee

Hyperammonemic encephalopathy as a manifestation of Reye syndrome in a previously-healthy 14-year-old girl: A case report

Reye syndrome is seldom considered in the older child presenting with acute encephalopathy. The paucity of literature on Reye syndrome in the past 30 years highlights the importance of sharing new observations of this less-commonly considered disease. This case details an atypical presentation of Reye syndrome in an adolescent girl whose course was unexpectedly complicated by dialysis disequilibrium syndrome, occurring as a result of rapid metabolic shifts in the setting of urgent hemodialysis. A 14-year-old, previously healthy girl born to non-consanguineous Pakistani parents presented with acute-onset encephalopathy characterized by aggressive behaviour and altered level of consciousness. This was preceded by frequent vomiting and weeks of disordered eating. Laboratory findings included high anion-gap [30 (normal range, \u3c14)] metabolic acidosis [pH 7.23 (normal range, 7.35-7.45), bicarbonate 10 mmol/L (normal range, 22-29 mmol/L)] with markedly elevated peak levels of lactate [13.6 mmol/L (normal range, 0.5-2.2 mmol/L)] and ammonia [573 μmol/L (normal range, 11-48 μmol/L)]. Emergent hemodialysis was undertaken for management of severe hyperammonemia, with subsequent worsening of cerebral edema, elevated intracranial pressure, and consequent uncal and tonsillar herniation, in keeping with the development of dialysis disequilibrium syndrome. The patient was ultimately diagnosed with Reye syndrome after extensive biochemical and molecular testing including exome sequencing were non-contributory. This diagnosis would be consistent with her poor nutritional state, depleted carnitine levels, mild liver dysfunction and hyperammonemia. Two weeks of intensive care were required. The patient was ultimately discharged to a rehabilitation facility with ongoing fatigue and regression in motor and cognitive domains. Hyperammonemic encephalopathy should prompt consideration for Reye syndrome. Hyperammonemia is a medical emergency; the extremely time-sensitive nature of hyperammonemia management mandates that ammonia level testing be undertaken for any unexplained encephalopathy. Severe elevations can result in neurological impairment, cerebral edema, and are acutely life-threatening. Hemodialysis may be indicated for levels \u3e150 μmol/L; the urgency of ammonia clearance must be balanced against the risk of developing dialysis disequilibrium syndrome

Enteroviral and herpes simplex virus central nervous system infections in infants < 90 days old: a Paediatric Investigators’ Collaborative Network on Infections in Canada (PICNIC) study

Background: The relative contribution of viruses to central nervous system (CNS) infections in young infants is not clear. For viral CNS infections, there are limited data on features that suggest HSV etiology or on predictors of unfavorable outcome. Methods: In this cross-sectional retrospective study, seven centers from the Pediatric Investigators Collaborative Network on Infections in Canada identified infants < 90 days of age with CNS infection proven to be due to enterovirus (EV) or herpes simplex virus (HSV) January 1, 2013 through December 31, 2014. Results: Of 174 CNS infections with a proven etiology, EV accounted for 103 (59%) and HSV for 7 (4%). All HSV cases and 41 (40%) EV cases presented before 21 days of age. Four HSV cases (57%) and 5 EV cases (5%) had seizures. Three (43%) HSV and 23 (23%) EV cases lacked cerebrospinal fluid (CSF) pleocytosis. HSV cases were more likely to require ICU admission (p = 0.010), present with seizures (p = 0.031) and have extra-CNS disease (p < 0.001). Unfavorable outcome occurred in 12 cases (11% of all EV and HSV infections) but was more likely following HSV than EV infection (4 (57%) versus 8 (8%); p = 0.002). Conclusions: Viruses accounted for approximately two-thirds of proven CNS infections in the first 90 days of life. Empiric therapy for HSV should be considered in suspected CNS infections in the first 21 days even in the absence of CSF pleocytosis unless CSF parameters are suggestive of bacterial meningitis. Neurodevelopmental follow-up should be considered in infants whose course of illness is complicated by seizures.Medicine, Faculty ofNon UBCPediatrics, Department ofReviewedFacult