Homozygous mice carrying kd (kidney disease) mutations in the gene encoding prenyl diphosphate synthase subunit 2 (Pdss2kd/kd) develop interstitial nephritis and eventually die from end-stage renal disease. The PDSS2 polypeptide in concert with PDSS1 synthesizes the polyisoprenyl tail of coenzyme Q (Q or ubiquinone), a lipid quinone required for mitochondrial respiratory electron transport. We have shown that a deficiency in Q content is evident in Pdss2kd/kd mouse kidney lipid extracts by 40 days of age and thus precedes the onset of proteinuria and kidney disease by several weeks. The presence of the kd (V117M) mutation in PDSS2 does not prevent its association with PDSS1. However, heterologous expression of the kd mutant form of PDSS2 together with PDSS1 in Escherichia coli recapitulates the Q deficiency observed in the Pdss2kd/kd mouse. Dietary supplementation with Q10 provides a dramatic rescue of both proteinuria and interstitial nephritis in the Pdss2kd/kd mutant mice. The results presented suggest that Q may be acting as a potent lipid-soluble antioxidant, rather than by boosting kidney mitochondrial respiration. Such Q10 supplementation may have profound and beneficial effects in treatment of certain forms of focal segmental glomerulosclerosis that mirror the renal disease of the Pdss2kd/kd mouse.

Ambulatory surgery has gained tremendous popularity within the last 2–3 decades. More and more surgeries are performed on outpatient basis at ambulatory surgery centers. The understanding of how the postanesthesia care unit (PACU) functions is important in the appropriate care and discharge of the patients. Multiple phases of recovery exist and patients can be triaged based on their emergence from anesthesia. PACU discharge scoring systems have been implemented to progress a patient through the stages of recovery until discharge. The scoring systems initially developed by Aldrete, later modified by Chung and White, are still being used today. Discharge from ambulatory surgery centers has unique concerns when compared with discharge from PACU to hospital wards. This chapter will discuss the special considerations when discharging patients from PACU in ambulatory surgery centers.

No abstract

No abstract

This review focuses on the perioperative anesthetic management of patients having liver transplantation (LT) performed for several uncommon indications or in combination with rare pathology. Conditions discussed in the article include Alagille syndrome, hypertrophic cardiomyopathy, Gilbert's syndrome, porphyria, Wilson's disease, and Budd-Chiari syndrome. In comparison to other indications, LT in these settings is infrequent because of the low incidence of these pathologies. Most of these conditions (with the exception of Gilbert syndrome) are associated with a high probability of significant perioperative complications and increased mortality and morbidity. Experience in management of these unusual conditions is only gained over time. Developing clinical pathways for patients with these conditions should result in outcomes similar to LT performed for more common indications.