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Kansas City, MO, United States

Penn E.,Childrens Memorial Hospital | Yasso S.F.,Childrens Mercy Hospital and Clinics | Wei J.L.,University of Kansas Medical Center
Otolaryngology - Head and Neck Surgery (United States) | Year: 2012

Large amounts of waste in hospitals are generated in the operating rooms from disposable surgical supplies. Tonsillectomy/adenotonsillectomy (T&A) cases use many disposable supplies that are not recyclable. It is critical to reduce disposable waste, as such waste directly affects the environment and increases health care costs. The authors noticed a difference between the number of disposable items prepared, available, but almost never used, for each tonsillectomy case between a children's hospital setting and a university ambulatory surgery center setting. The aims were the following: (1) identify what disposable medical supplies were unnecessarily opened for each case, (2) eliminate all disposable medical waste that was not critical to the case in both settings, and (3) determine the cost reduction at both hospital and surgery center facilities by revising the current disposable instruments/supplies pulled for tonsillectomy cases. The authors report projected cost savings and reduction in waste for one children's hospital and nationally based on their waste reduction. © 2012 American Academy of Otolaryngology - Head and Neck Surgery Foundation.

Kauffman J.,Childrens Mercy Hospital and Clinics
Journal of Infusion Nursing | Year: 2011

Hemophilia is a genetic condition that causes prolonged bleeding in those affected after surgeries, dental procedures, and injuries. It has been treated with a variety of products in the modern era, with some of the treatments causing serious viral infections. The development of recombinant-factor products has led to longer life expectancy and better quality of life for those affected. The objectives of this article are to review the epidemiology and pathophysiology of hemophilia A and B and von Willebrand's disease and to outline the development of treatment products, along with the advantages and disadvantages of each product. Research on newer products is progressing at a rapid pace. The article will also discuss some of the newer products currently in development. © Copyright 2011 by the Infusion Nurses Society.

Hicks J.K.,St Jude Childrens Research Hospital | Hicks J.K.,Cleveland Clinic | Swen J.J.,Leiden University | Gaedigk A.,Childrens Mercy Hospital and Clinics | Gaedigk A.,University of Missouri - Kansas City
Current Drug Metabolism | Year: 2014

The cytochrome P450 2D6 (CYP2D6) enzyme contributes to the metabolism and/or bioactivation of approximately 25% of clinically used drugs. The CYP2D6 gene locus is highly polymorphic and complex, and variants within this gene locus affect CYP2D6 enzymatic function resulting in a wide range of metabolic activity from little to no activity to ultrarapid metabolism. For many of the drugs metabolized by CYP2D6, the variation in metabolic activity is one of the most important factors responsible for interindividual drug response. Therefore, determining an individual's CYP2D6 phenotype, or metabolic status, will help identify individuals that may benefit from a change in drug or drug dosage. Genotype analysis has become the method of choice to predict a person's metabolic status. Numerous reference laboratories now offer CYP2D6 genotyping; however, there can be substantial differences in the number of genetic variants interrogated as well as test interpretation. Furthermore, there is no standardized process of how a CYP2D6 genotype result is translated into a phenotype assignment. This review summarizes the complexity of CYP2D6 genotyping and highlights the major challenges for phenotype classification. We call for the implementation of a universally accepted system for CYP2D6 phenotype assignment to promote consistency of test interpretation among reference laboratories and medical institutions. We propose a system that utilizes the CYP2D6 activity score system to place individuals into a continuum of activity scores - rather than using the traditional poor, intermediate, extensive and ultra-rapid metabolizer categorizations - and directly translating activity scores into clinically actionable recommendations. © 2014 Bentham Science Publishers.

Goldstein S.L.,University of Cincinnati | Morris D.,WebbWrites LLC | Warady B.A.,Childrens Mercy Hospital and Clinics
American Journal of Kidney Diseases | Year: 2013

Background: Iron deficiency is a common cause of anemia in young persons with chronic kidney disease (CKD). Iron repletion with intravenous (IV) iron formulations has been studied in children; maintenance IV iron regimens have not been reported extensively. Study Design: A multicenter randomized trial of IV iron sucrose. Setting & Participants: 145 children, adolescents, and young adults with CKD receiving erythropoiesis-stimulating agent (ESA) therapy were stratified by dialysis category (hemodialysis, peritoneal dialysis, or non-dialysis dependent) and weight (<50 and ≥50 kg). Intervention: Patients were randomly assigned to 1 of 3 dosing arms: 0.5, 1.0, or 2.0 mg/kg (maximum single dose, 100 mg), stratified into hemodialysis versus nonhemodialysis (peritoneal dialysis or non-dialysis-dependent CKD) groups. Patients treated with hemodialysis received study medication once every other week for 6 doses. Patients in the nonhemodialysis group received study medication once every 4 weeks for 3 doses. Outcomes: We assessed adverse event rates between dosing groups. The main clinical end point was a composite of hemoglobin level ≥10.5-14.0 g/dL, inclusive; transferrin saturation ≥20%-50%, inclusive; and stable ESA dosing (±25% of baseline dose). Results: Between-group difference for composite clinical end point rate attainment was -3.9% (95% CI, -21.4% to 13.7%) for the 1.0-mg/kg group versus 0.5-mg/kg group, +3.9% (95% CI, -15.1% to 23.0%) for the 2-mg/kg group versus 0.5-mg/kg group, and +7.8% (95% CI, -10.9% to 26.5%) for the 2-mg/kg group versus 1-mg/kg group. No differences were noted between regimens in reported adverse effects, which were all minor. Limitations: Absence of a control group receiving no IV iron. Short duration of intervention and observation. A small proportion of patients having achieved the primary clinical outcome. Conclusions: IV iron sucrose at a dose of 0.5 mg/kg at the intervals prescribed is noninferior to higher doses in maintaining hemoglobin levels >10.5 g/dL in children, adolescents, and young adults receiving ESA therapy. © 2013 National Kidney Foundation, Inc.

Mann A.,University of Toronto | Miksys S.L.,University of Toronto | Gaedigk A.,Childrens Mercy Hospital and Clinics | Kish S.J.,University of Toronto | And 2 more authors.
Neurobiology of Aging | Year: 2012

Cytochrome P450 2D6 (CYP2D6) is a drug-metabolizing enzyme expressed in the brain that also metabolizes endogenous neural compounds (e.g., catecholamines) and inactivates neurotoxins (e.g., 1-methyl-4-thenyl-1,2,3,6-tetrahydropyridine; MPTP). Genetically poor CYP2D6 metabolizers are at higher risk for developing Parkinson's disease (PD), a risk that increases with exposure to pesticides. As age is a risk factor for PD we measured the ontogenic expression of CYP2D6 in human brain, and compared brain CYP2D6 levels in PD cases with age-matched controls. CYP2D6 increased from fetal to 80 years of age (n = 76), exhibiting 3 distinct phases of change. Compared with PD controls, PD cases had approximately 40% lower CYP2D6 levels in the frontal cortex, cerebellum, and the hippocampus, even when controlling for CYP2D6 genotype. In contrast, CYP2D6 levels in cases were similar to controls in PD-affected brain areas, the substantia nigra, and caudate, consistent with higher astrocytic and cellular CYP2D6 staining observed in PD cases. In summary, the lower CYP2D6 levels in PD cases may have reduced their ability to inactivate PD-causing neurotoxins contributing to their disease risk. © 2012 Elsevier Inc.

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