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Chapter 17 - Disorders of Intermediaries of Metabolism and Malignant Hyperthermia
- Edited by David R. Gambling, University of California, San Diego, M. Joanne Douglas, University of British Columbia, Vancouver, Grace Lim, University of Pittsburgh
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- Book:
- Obstetric Anesthesia and Uncommon Disorders
- Published online:
- 26 January 2024
- Print publication:
- 01 February 2024, pp 273-289
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Summary
Many inherited conditions result from disorders of intermediary metabolism. Many more are discovered annually using advanced gene sequencing and other tools. These diseases cause symptoms because of the accumulation of precursors, absence of the final product, excessive toxic intermediaries, or a combination of all three mechanisms. Many are fatal in childhood, but some are compatible with adult life and pregnancy. A better understanding of the enzymatic deficiencies and new technologies have made recombinant enzyme replacement therapy possible. Along with early diet manipulation, current management allows many patients to live relatively normal lives. Because fertility may not be affected, some of these conditions will be encountered by the anesthesiologist. This chapter describes diseases caused by certain enzyme deficiencies and the by-products that cause symptoms. Some are exacerbated by pregnancy and the stress of labor and delivery. The anesthesiologist plays an essential role in reducing physiologic stress and avoiding triggering agents and routines that cause severe metabolic derangements or cardiopulmonary decompensation. The final portion of the chapter describes the most recent advances in the prevention and treatment of malignant hyperthermia in pregnancy, discussing the impact on mother and baby.
SARS-CoV-2 outbreak in a synagogue community: longevity and strength of anti-SARS-CoV-2 IgG responses
- Yael Gozlan, Stephen Reingold, Ravit Koren, Osnat Halpern, Gili Regev-Yochay, Carmit Cohen, Asaf Biber, Orit Picard, Ella Mendelson, Yaniv Lustig, Orna Mor
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- Journal:
- Epidemiology & Infection / Volume 149 / 2021
- Published online by Cambridge University Press:
- 24 June 2021, e153
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Severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) pandemic is still ongoing along with the global vaccination efforts against it. Here, we aimed to understand the longevity and strength of anti-SARS-CoV-2 IgG responses in a small community (n = 283) six months following local SARS-COV-2 outbreak in March 2020. Three serological assays were compared and neutralisation capability was also determined. Overall 16.6% (47/283) of the participants were seropositive and 89.4% (42/47) of the IgG positives had neutralising antibodies. Most of the symptomatic individuals confirmed as polymerase chain reaction (PCR) positive during the outbreak were seropositive (30/32, 93.8%) and 33.3% of the individuals who quarantined with a PCR confirmed patient had antibodies. Serological assays comparison revealed that Architect (Abbott) targeting the N protein LIASON® (DiaSorin) targeting the S protein and enzyme-linked immunosorbent assay (ELISA) targeting receptor binding domain detected 9.5% (27/283), 17.3% (49/283) and 17% (48/283), respectively, as IgG positives. The latter two assays highly agreed (kappa = 0.89) between each other. In addition, 95%, (19/20, by ELISA) and 90.9% (20/22, with LIASON) and only 71.4% (15/21, by Architect) of individuals that were seropositive in May 2020 were found positive also in September. The unexpected low rate of overall immunity indicates the absence of un-noticed, asymptomatic infections. Lack of overall high correlation between the assays is attributed mainly to target-mediated antibody responses and suggests that using a single serological assay may be misleading.
Contributors
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- By Brittany L. Anderson-Montoya, Heather R. Bailey, Carryl L. Baldwin, Daphne Bavelier, Jameson D. Beach, Jeffrey S. Bedwell, Kevin B. Bennett, Richard A. Block, Deborah A. Boehm-Davis, Corey J. Bohil, David B. Boles, Avinoam Borowsky, Jessica Bramlett, Allison A. Brennan, J. Christopher Brill, Matthew S. Cain, Meredith Carroll, Roberto Champney, Kait Clark, Nancy J. Cooke, Lori M. Curtindale, Clare Davies, Patricia R. DeLucia, Andrew E. Deptula, Michael B. Dillard, Colin D. Drury, Christopher Edman, James T. Enns, Sara Irina Fabrikant, Victor S. Finomore, Arthur D. Fisk, John M. Flach, Matthew E. Funke, Andre Garcia, Adam Gazzaley, Douglas J. Gillan, Rebecca A. Grier, Simen Hagen, Kelly Hale, Diane F. Halpern, Peter A. Hancock, Deborah L. Harm, Mary Hegarty, Laurie M. Heller, Nicole D. Helton, William S. Helton, Robert R. Hoffman, Jerred Holt, Xiaogang Hu, Richard J. Jagacinski, Keith S. Jones, Astrid M. L. Kappers, Simon Kemp, Robert C. Kennedy, Robert S. Kennedy, Alan Kingstone, Ioana Koglbauer, Norman E. Lane, Robert D. Latzman, Cynthia Laurie-Rose, Patricia Lee, Richard Lowe, Valerie Lugo, Poornima Madhavan, Leonard S. Mark, Gerald Matthews, Jyoti Mishra, Stephen R. Mitroff, Tracy L. Mitzner, Alexander M. Morison, Taylor Murphy, Takamichi Nakamoto, John G. Neuhoff, Karl M. Newell, Tal Oron-Gilad, Raja Parasuraman, Tiffany A. Pempek, Robert W. Proctor, Katie A. Ragsdale, Anil K. Raj, Millard F. Reschke, Evan F. Risko, Matthew Rizzo, Wendy A. Rogers, Jesse Q. Sargent, Mark W. Scerbo, Natasha B. Schwartz, F. Jacob Seagull, Cory-Ann Smarr, L. James Smart, Kay Stanney, James Staszewski, Clayton L. Stephenson, Mary E. Stuart, Breanna E. Studenka, Joel Suss, Leedjia Svec, James L. Szalma, James Tanaka, James Thompson, Wouter M. Bergmann Tiest, Lauren A. Vassiliades, Michael A. Vidulich, Paul Ward, Joel S. Warm, David A. Washburn, Christopher D. Wickens, Scott J. Wood, David D. Woods, Motonori Yamaguchi, Lin Ye, Jeffrey M. Zacks
- Edited by Robert R. Hoffman, Peter A. Hancock, University of Central Florida, Mark W. Scerbo, Old Dominion University, Virginia, Raja Parasuraman, George Mason University, Virginia, James L. Szalma, University of Central Florida
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- Book:
- The Cambridge Handbook of Applied Perception Research
- Published online:
- 05 July 2015
- Print publication:
- 26 January 2015, pp xi-xiv
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Notes on Contributors
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- By Charles Altieri, Faith Barrett, Alfred Bendixen, David Bergman, Edward Brunner, Stephen Burt, Susan Castillo Street, Michael C. Cohen, Robert Daly, Betty Booth Donohue, Jim Egan, Richard Flynn, Ed Folsom, Stephen Fredman, Frank Gado, Roger Gilbert, Rigoberto González, Nick Halpern, Jeffrey A. Hammond, Kevin J. Hayes, Matthew Hofer, Tyler Hoffman, Christoph Irmscher, Virginia Jackson, Joseph Jonghyun Jeon, John D. Kerkering, George S. Lensing, Mary Loeffelholz, Wendy Martin, Cristanne Miller, David Chioni Moore, Walton Muyumba, John Timberman Newcomb, Bob Perelman, Siobhan Phillips, Brian M. Reed, Elizabeth Renker, Eliza Richards, Reena Sastri, Robin G. Schulze, Mark Scroggins, David E. E. Sloane, Angela Sorby, Juliana Spahr, Willard Spiegelman, Lisa M. Steinman, Ernest Suarez, Joseph T. Thomas, Lesley Wheeler, David Wojahn
- Edited by Alfred Bendixen, Princeton University, New Jersey, Stephen Burt, Harvard University, Massachusetts
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- The Cambridge History of American Poetry
- Published online:
- 05 December 2014
- Print publication:
- 27 October 2014, pp xi-xviii
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Contributors
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- By Giustino Albanese, Andrew Amaranto, Brandon H. Backlund, Alexander Baxter, Abraham Berger, Mark Bernstein, Marian E. Betz, Omar Bholat, Suzanne Bigelow, Carl Bonnett, Elizabeth Borock, Christopher B. Colwell, Alasdair Conn, Moira Davenport, David Dreitlein, Aaron Eberhardt, Ugo A. Ezenkwele, Diana Felton, Spiros G. Frangos, John E. Frank, Jonathan S. Gates, Lewis Goldfrank, Pinchas Halpern, Jean Hammel, Kristin E. Harkin, Jason S. Haukoos, E. Parker Hays, Aaron Hexdall, James F. Holmes, Debra Houry, Jennifer Isenhour, Andy Jagoda, John L. Kendall, Erica Kreisman, Nancy Kwon, Eric Legome, Matthew R. Levine, Phillip D. Levy, Charles Little, Marion Machado, Heather Mahoney, Vincent J. Markovchick, Nancy Martin, John Marx, Julie Mayglothling, Ron Medzon, Maurizio A. Miglietta, Elizabeth L. Mitchell, Ernest Moore, Maria E. Moreira, Sassan Naderi, Salvatore Pardo, Sajan Patel, David Peak, Christine Preblick, Niels K. Rathlev, Charles Ray, Phillip L. Rice, Carlo L. Rosen, Peter Rosen, Livia Santiago-Rosado, Tamara A. Scerpella, David Schwartz, Fred Severyn, Kaushal Shah, Lee W. Shockley, Mari Siegel, Matthew Simons, Michael Stern, D. Matthew Sullivan, Carrie D. Tibbles, Knox H. Todd, Shawn Ulrich, Neil Waldman, Kurt Whitaker, Stephen J. Wolf, Daniel Zlogar
- Edited by Eric Legome, Lee W. Shockley
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- Trauma
- Published online:
- 07 September 2011
- Print publication:
- 16 June 2011, pp ix-xiv
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- By Phillip L. Ackerman, Soon Ang, Susan M. Barnett, G. David Batty, Anna S. Beninger, Jillian Brass, Meghan M. Burke, Nancy Cantor, Priyanka B. Carr, David R. Caruso, Stephen J. Ceci, Lillia Cherkasskiy, Joanna Christodoulou, Andrew R. A. Conway, Christine E. Daley, Janet E. Davidson, Jim Davies, Katie Davis, Ian J. Deary, Colin G. DeYoung, Ron Dumont, Carol S. Dweck, Linn Van Dyne, Pascale M. J. Engel de Abreu, Joseph F. Fagan, David Henry Feldman, Kurt W. Fischer, Marisa H. Fisher, James R. Flynn, Liane Gabora, Howard Gardner, Glenn Geher, Sarah J. Getz, Judith Glück, Ashok K. Goel, Megan M. Griffin, Elena L. Grigorenko, Richard J. Haier, Diane F. Halpern, Christopher Hertzog, Robert M. Hodapp, Earl Hunt, Alan S. Kaufman, James C. Kaufman, Scott Barry Kaufman, Iris A. Kemp, John F. Kihlstrom, Joni M. Lakin, Christina S. Lee, David F. Lohman, N. J. Mackintosh, Brooke Macnamara, Samuel D. Mandelman, John D. Mayer, Richard E. Mayer, Martha J. Morelock, Ted Nettelbeck, Raymond S. Nickerson, Weihua Niu, Anthony J. Onwuegbuzie, Jonathan A. Plucker, Sally M. Reis, Joseph S. Renzulli, Heiner Rindermann, L. Todd Rose, Anne Russon, Peter Salovey, Scott Seider, Ellen L. Short, Keith E. Stanovich, Ursula M. Staudinger, Robert J. Sternberg, Carli A. Straight, Lisa A. Suzuki, Mei Ling Tan, Maggie E. Toplak, Susana Urbina, Richard K. Wagner, Richard F. West, Wendy M. Williams, John O. Willis, Thomas R. Zentall
- Edited by Robert J. Sternberg, Oklahoma State University, Scott Barry Kaufman, New York University
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- Book:
- The Cambridge Handbook of Intelligence
- Published online:
- 05 June 2012
- Print publication:
- 30 May 2011, pp xi-xiv
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13 - Disorders of intermediary metabolism
- from Section 4 - Metabolic disorders
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- By Stephen Halpern, Professor, Department of Anesthesia, University of Toronto; Director of Obstetrical Anesthesia, Sunnybrook and Women's College, Health Sciences Centre, Toronto, Ontario, Canada, Bhadresh Shah, Fellow in Obstetrical Anesthesia, Sunnybrook and Women's College, Health Sciences Centre, Toronto, Ontario, Canada
- Edited by David R. Gambling, University of California, San Diego, M. Joanne Douglas, University of British Columbia, Vancouver, Robert S. F. McKay, University of Kansas
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- Book:
- Obstetric Anesthesia and Uncommon Disorders
- Published online:
- 19 October 2009
- Print publication:
- 20 March 2008, pp 239-248
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Summary
Introduction
There are a large number of inherited conditions that result from disorders of intermediary metabolism. These cause symptoms because of the build up of precursors, the absence of the final product, an excess of toxic intermediaries, or a combination of these mechanisms. Many are fatal in childhood, but some are compatible with adult life and pregnancy. These disorders may be encountered by an obstetric anesthesiologist (either because of their prevalence or, for some rare conditions, because modern management confers an improved chance of fertility). Some examples of these disorders can be found in Table 13.1. Malignant hyperthermia, plasma pseudocholinesterase deficiency, and inherited hematological, endocrine, connective tissue, or bone disorders are discussed elsewhere.
In all these rare disorders, a basic tenet of management is to refer to a specialist in the relevant field of medicine, to coordinate a multidisciplinary team approach and to make a thorough and early antenatal assessment, with documentation of a plan of management.
Glycogen storage diseases
Glucose metabolism plays a fundamental role in supplying energy for most cellular metabolic processes. Glycogen, the storage form of glucose, is composed of a branched polymer of glucose residues and can be found in muscle and liver. Defects in glycogen metabolism typically cause accumulation of glycogen in the tissues. Historically, the glycogen storage diseases have been classified numerically according to the specific enzyme defect. Since the main manifestations are either primarily related to liver or muscle, it may be more useful to categorize them according to the primary organ involved.
18 - Obstetrical anaesthesia
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- By Stephen Halpern, University of Toronto, Obstetrical Anaesthesia, Sunnybrook and Women's College Health Sciences Centre, Toronto, ON, Canada
- Edited by Ann Møller, KAS Herlev, Copenhagen, Tom Pedersen, Rigshospitalet, Copenhagen
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- Book:
- Evidence-based Anaesthesia and Intensive Care
- Published online:
- 05 September 2009
- Print publication:
- 09 October 2006, pp 205-222
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Summary
This chapter discusses the effect of neuraxial analgesia on caesarean section rates, instrumental delivery rates and the duration of labour. Neuraxial analgesia effectively relieves labour pain and is often chosen by parturients because of the known efficacy of the technique. Although in most cases, randomised controlled trials (RCTs) are the strongest study design in the sense that, when properly performed, they result in the least amount of bias, there are a number of barriers to consider when studying labour analgesia. The duration of first and second stage of labour has been compared in RCTs in patients who received neuraxial analgesia and opioid analgesia controls. Conversely, neuraxial analgesia may cause an increase in the incidence of instrumental vaginal delivery. When deciding which type of analgesia to offer a parturient, the benefits and risks must be assessed. Neuraxial analgesia provides the most complete analgesia when compared to any other mode of treatment.