Objectives:To compare the efficacy and performance of the pediatric Ambu AuraOnce (Ambu AO) mask (Ambu, Copenhagen, Denmark) and i-gel mask (Intersurgical Ltd., Wokingham, United Kingdom).Methods:From May 2015 to September 2016, 112 patients, 0-14 years old, underwent elective surgery at a tertiary university hospital (Riyadh, Saudi Arabia). They were randomly assigned to the Ambu AO or i-gel group. Three groups underwent a subgroup analysis: ≤5 kg (group 1), 5.1–10.0 kg (group 2), and >10 kg (group 3).Results:The oropharyngeal leak pressure was significantly higher for the i-gel (25.4±4.1 cm H2O) than for the Ambu AO (22.5±3.9 cm H2O, p<0.001). The Ambu AO had a slightly higher ease of insertion compared to the i-gel (100% versus 94%, p=0.08) and required less manipulation (2% versus 11%, p=0.07).The Ambu AO and i-gel showed non-significant differences in performance between weight groups. There were statistically significant differences for higher leak pressure in group 2 (p=0.01) and group 3 (p=0.002) in favor of the i-gel, and for less manipulation in the Ambu AO in group 1 (p=0.04). Fiberoptic viewing was superior in group 2 for the i-gel (p=0.03) and in group 3 for the Ambu AO (p=0.02).Conclusion:Both devices demonstrated equally good performance with low morbidity. The Ambu AO had a statistical tendency towards easier insertion and less manipulation. Confirming this finding will require large scale trials.
Johanson-Blizzard syndrome (JBS) is a rare autosomal recessive condition characterized by pathognomonic facies and a constellation of other features most notably exocrine pancreatic insufficiency, oligodontia, growth retardation, hearing loss, mental retardation, scalp defects, hypothyroidism and imperforate anus. We report on an infant with classical JBS who also has unusually severe neonatal cholestatic liver disease that progressed to liver fibrosis and portal hypertension. Sequencing of UBR1 revealed a previously unreported homozygous missense mutation in a consensus splice acceptor site (IVS12-1G > A). This report is the first to document severe liver involvement in JBS and raises the possibility that this could be a rare but genuine association.
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