We assessed the spectrum of airway disorders in children with congenital cardiac anomalies, and reviewed our experience in using flexible bronchoscopy for assessment of airway problems in this patient group. The clinical records, flexible bronchoscopic findings, and cardiac imaging results of pediatric cardiac patients who presented with either clinical or radiological signs of airway obstruction between 1992-1999 were reviewed. Flexible bronchoscopic assessment was performed with the patients under sedation and topical anesthesia, using one of two bronchoscopes, i.e., an Olympus BFN20 or Olympus BF3C20. Of a total of 52 patients, 33 had acyanotic cardiovascular lesions, the commonest being left-to-right shunts (61%), while 19 had cyanotic heart lesions, with right ventricular outflow obstruction being the commonest (63%). Twenty-seven patients had undergone either surgical or transcatheter interventions. The median age at bronchoscopic assessment was 6 months (range, 4 days to 6 years). None of the patients developed significant procedural complications. A definitive diagnosis was made in 48 (92%) patients, 8 of whom had abnormalities involving only the upper airways, 35 only the lower airways, and 5 both. Abnormalities of the upper airway included laryngomalacia (n = 6), subglottic stenosis (n = 3), pharyngeal collapse (n = 2), and 1 each of choanal stenosis and supraglottitis. Extrinsic compression was the commonest lower airway abnormality that was found in 27/40 patients (67%), with a predilection for the left main bronchus (18/27, 67%). The structures that caused extrinsic compression included dilated pulmonary arteries with or without left atrial dilation (n = 20), an anomalous aortic or pulmonary arterial course (n = 3), a dilated aorta (n = 1), and a shunt (n = 1), but were not obvious in 2 patients. Intrinsic lower airway abnormalities included bronchomalacia (n = 4), tracheal stenosis (n = 4), and one each of variant bronchial bifurcation and a pouch arising from the tracheal wall. Intraluminal mucus plugging of the lower airways occurred in the remaining 3 patients. Children with congenital heart disease are at risk of airway obstruction both before and after surgery. Flexible bronchoscopy, being safe and effective in diagnosing airway disorders in this patient group, should be considered as the first line of investigation.

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We report the use of continuous arteriovenous hemodiafiltration (CAVHD) in a neonate with severe hyperammonemia due to a urea cycle disorder. We compared the ammonia clearance (C(NH3)) for peritoneal dialysis (PD) and CAVHD. C(NH3) for CAVHD was 7.45 ml/min per m2 at a dialysate flow of 300 ml/h and was 10.55 ml/min per m2 at a dialysate flow rate of 600 ml/h. The mean PD clearance was 2.15 ml/min per m2. Our data suggest that CAVHD is superior to PD for the removal of plasma ammonia. We conclude that CAVHD should be considered a reasonable alternative in the treatment of neonatal hyperammonemia in urea cycle disorders when medical treatment fails.

The objectives of this study are to identify prognostic factors of survival to discharge in pediatric hematopoietic stem cell transplant (HSCT) recipients requiring intensive care unit (ICU) admission, and to determine the utility of the Oncological Pediatric Risk of Mortality (O-PRISM) in predicting death of these patients. A retrospective cohort of 125 pediatric HSCT recipients from October 1992 to September 2002 was analysed to evaluate risk factors of mortality in those admitted to ICU after HSCT. Nineteen patients (median age 7.8 years, 14 boys) required 24 ICU admissions post-HSCT. The most frequent underlying diseases were acute myeloid leukemia (n=5). The survival rate on discharge from ICU was 54%. In univariate analysis, risk factors of mortality included earlier requirement of ICU admission post-HSCT (median 34 versus 166 days, p=0.002), a longer delay before ICU admission (median 12 versus 5 h, p=0.02), lack of neutrophil (p=0.011) or platelet engraftment (p=0.008), macroscopic hemorrhage (p<0.001), tachypnoea (p=0.033), hypoxemia (p=0.031), renal impairment (p=0.011), coagulopathy (p=0.012), mechanical ventilation (p<0.001), and an increasing number of organ failures (p=0.003). Macroscopic hemorrhage and mechanical ventilation remained significant in multivariate analysis. Both PRISM and O-PRISM scores were significant composite prognosticators. It was concluded that mortality of post-HSCT children requiring ICU admission is high, especially in those with poor prognosticators.