AAV gene therapy for Tay-Sachs disease.

Tay-Sachs disease (TSD) is an inherited neurological disorder caused by deficiency of hexosaminidase A (HexA). Here, we describe an adeno-associated virus (AAV) gene therapy expanded-access trial in two patients with infantile TSD (IND 18225) with safety as the primary endpoint and no secondary endpoints. Patient TSD-001 was treated at 30 months with an equimolar mix of AAVrh8-HEXA and AAVrh8-HEXB administered intrathecally (i.

Disclosure after medical error.

Purpose Of Review: Disclosure after medical error is a complex process that goes beyond a single provider and the patient. The types and severity of errors occurring in the practice of anesthesiology may have more implications for patients, requiring a thoughtful approach to the patient, their families and the healthcare system as a whole.

Recent Findings: Tenants of the disclosure have been established.

Restrictive Intraoperative Fluid Therapy is Associated with Decreased Morbidity and Length of Stay Following Hyperthermic Intraperitoneal Chemoperfusion.

Background: Recent data have demonstrated multiple benefits of intra- and postoperative fluid restriction in major abdominal surgery; however, data regarding the outcomes of fluid restriction in cytoreductive surgery and hyperthermic intraperitoneal chemoperfusion (CRS/HIPEC) are limited. This study evaluates the safety and short-term clinical outcomes of restricted intraoperative fluid therapy in CRS/HIPEC.

Methods: This was a single-institution, retrospective review of all CRS/HIPEC procedures performed at the University of Massachusetts Medical School between January 2009 and July 2017.

Extubation of the difficult airway.

Managing the airway in the intensive care unit (ICU) is complicated by a wide array of physiologic factors. Difficult airway may be a consequence of patient’s anatomy or airway edema developed during the ICU stay and mechanical ventilation. The incidence of failed airways and of cardiac arrest related to airway instrumentation in the ICU is much higher than that of elective intubations performed in the operating room.