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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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Coencapsulation with Sertoli-enriched testicular cell fractions prolongs islet graft survival time compared with islet encapsulation alone in a highly discordant tilapia (fish)-to-mouse xenotransplantation model. Here we investigate whether Fas ligand (Fas-L) expression by testicular Sertoli cells is responsible for this additional protective effect. Sertoli-enriched testicular cell fractions (7 × 10 6 cells) harvested from either Fas-Ldefective (group I) or Fas-L-positive (group II) mice were coencapsulated in alginate gel spheres with fish islets and then transplanted into streptozotocin-diabetic Balb/c recipients. Group III mice received encapsulated islets without coencapsulated Sertoli cells. After transplantation, blood glucose levels were monitored three times per week. Mean graft survival times for the three groups were: group I = 35.6 ± 10.2 days (n = 9), group II = 31.3 ± 9.4 days (n = 7), and group III = 23.3 ± 2.2 days (n = 6) (ANOVA, p = 0.043). Coencapsulation, regardless of the Fas-L status of the Sertoli cell donors, modestly prolonged graft survival. There was no significant difference between Fas-L-deficient and Fas-L-positive donors. Our results suggest that Fas/Fas-L interaction is not responsible for the additional protection afforded to encapsulated discordant islet xenografts by coencapsulation with Sertoli cells.

Background:Umbilical hernia repair, a common day-case surgery procedure in children, is associated with a significant postoperative pain. The most popular peripheral nerve blocks used in umbilical hernia repair are rectus sheath infiltration and caudal block. The rectus sheath block may offer improved pain relief following umbilical hernia repair with no undesired effects such as lower limb motor weakness or urinary retention seen with caudal block which might delay discharge from the hospital. Ultrasound guidance of peripheral nerve blocks has reduced the number of complications and improved the quality of blocks. The aim of this case series is to assess the post rectus sheath block pain relief in pediatric patients coming for umbilical surgery.Methods:Twenty two (22) children (age range: 1.5-8 years) scheduled for umbilical hernia repair were included in the study. Following the induction of general anesthesia, the ultrasonographic anatomy of the umbilical region was studied with a 5-16 MHz 50 mm linear probe. An ultrasound-guided posterior rectus sheath block of both rectus abdominis muscles (RMs) was performed (total of 44 punctures). An in-plain technique using Stimuplex A insulated facet tip needle 22G 50mm. Surgical conditions, intraoperative hemodynamic parameters, and postoperative analgesia by means of the modified CHEOPS scale were evaluated.Results:ultrasonograghic visualization of the posterior sheath was possible in all patients. The ultrasound guided rectus sheath blockade provided sufficient analgesia in all children with no need for additional analgesia except for one patient who postoperatively required morphine 0.1 mg/kg intravenously. There were no complications.Conclusions:Ultrasound guidance enables performances of an effective rectus sheath block for umbilical hernia. Use of the Stimuplex A insulated facet tip needle 22G 50mm provides easy, less traumatic skin and rectus muscle penetration and satisfactory needle visualiza.

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