Use of Emergency Manuals to Treat Delayed Emergence After Robotic-Assisted Cholecystectomy.

Delayed emergence is defined as failure to regain consciousness 30 to 60 minutes after general anesthesia. Although incidence is low and difficult to accurately estimate, there is a wide differential diagnosis to be considered. Emergency manuals (EMs) are visual cognitive aids that can be used in the operating room to help manage intraoperative complications. They provide immediate access to evidence-based guidelines to optimize management of intraoperative complications. They are being increasingly implemented in the clinical setting and have been shown to improve patient safety. A case of a patient with delayed emergence after undergoing robotic-assisted cholecystectomy is described here. The delayed emergence section of the Stanford Anesthesia Emergency Model was referenced immediately and guided management of the patient. Utilization of an EM resulted in rapid return to baseline mental status. EMs allow health care providers to respond to intraoperative scenarios efficiently and effectively and ultimately improve patient care.

The Changing Face of Infantile Pompe Disease: A Report of Five Patients from the UAE.

OBJECTIVE: We aim to present our experience with infantile Pompe disease with focus on the impact of availability of treatment on awareness, diagnosis, and management of such patients. METHOD: Case - review study of patients diagnosed with infantile Pompe disease and literature search. RESULTS: We identified five cases of infantile Pompe disease. The first was diagnosed by muscle biopsy; all others were diagnosed by enzyme assay on peripheral blood lymphocytes or dried blood spot. There was no determination of the CRIM status on these patients. Two have died at a much later age than the reported median age of death for untreated cases. One died very early at 2 months of age with severe cardiomyopathy and had received only one dose of enzyme replacement therapy (ERT). The remaining two surviving patients are siblings: the younger was diagnosed by prenatal ultrasound screening and started on ERT at 24 h of age; she is the youngest treated patient in our case series. CONCLUSION: The natural history of infantile Pompe disease is changing, so are the challenges of managing these infants in the post- ERT era. Currently, increased awareness and early access to therapy provide the best outcomes and incur the least shift of burden from mortality to morbidity. Disclosure: Authors have received travel support to attend scientific symposiums by Genzyme. WF has also received consulting fees for speaking in a scientific symposium by Genzyme.

Gender variability in presentation with Alpers' syndrome: a report of eight patients from the UAE.

INTRODUCTION: Alpers' syndrome is a progressive and often fatal cerebral and hepatic degeneration caused by a mutation in the polymerase gamma (POLG) gene involved in mitochondrial DNA replication. OBJECTIVE: We report on eight successive cases from five families. METHODS: Our analysis consisted of case series reports and literature search. RESULTS: The eight patients were from five extended families, all with clinical manifestations of the syndrome. Seven were confirmed by POLG sequence analysis and one died before testing was possible. We observed that whereas the five females presented with advanced hepatic disease at the onset of neurological symptoms, the three males had normal hepatic function well after presentation, with progressive neurological disease. Two of the three males are distant relatives; two of the five females were sisters of two male patients. DISCUSSION: Most authors report the coexistence of both hepatic and cerebral disease at the onset of Alpers' syndrome. It is unusual that all three males in our series had no signs of liver disease but had advanced neurological signs. CONCLUSION: Initial manifestations in Alpers' syndrome may be gender specific. In males, the condition should be considered in patients with seizures and encephalopathy, even in the absence of hepatic disease.

Infant botulism type Ba: first culture-confirmed case in the United Arab Emirates.

We report on a 3-month-old girl with culture-confirmed infant botulism caused by a rare double toxin-producing Clostridium botulinum type Ba. This case was not related to honey-feeding. The clinical course was prolonged, with minimal spontaneous improvement at onset, and a period of fluctuating motor weakness and nasogastric feeding dependence afterward. Neurophysiologic studies produced normal results. Human botulism immune globulin was administered empirically on day 23 of presentation, with rapid full recovery. This case highlights the importance of pursuing diagnoses of infant botulism despite normal results of neurophysiologic testing and no history of honey-feeding. Our case also demonstrates a favorable response to human botulism immune globulin, despite the relatively late treatment.