BRIEF CONTENTS Introduction to Pathophysiology, xv 25 A lterations of the Male Reproductive System, 885 26 S exually Transmitted Infections, 918 PART ONE: CENTRAL CONCEPTS UNIT VIII: THE HEMATOLOGIC SYSTEM OF PATHOPHYSIOLOGY: CELLS AND TISSUES 27 S tructure and Function of the Hematologic System, 945 28 A lterations of Erythrocyte Function, 982 UNIT I: THE CELL 29 A lterations of Leukocyte, Lymphoid, and Hemostatic 1 Cellular Biology, 1 Function, 1008 2 Altered Cellular and Tissue Biology, 49 30 Alterations of Hematologic Function in Children, 1055 3 The Cellular Environment: Fluids and Electrolytes, UNIT IX: THE CARDIOVASCULAR AND Acids and Bases, 103 LYMPHATIC SYSTEMS UNIT II: GENES AND GENE-ENVIRONMENT 31 S tructure and Function of the Cardiovascular and INTERACTION Lymphatic Systems, 1083 4 Genes and Genetic Diseases, 135 32 A lterations of Cardiovascular Function, 1129 5 Genes, Environment-Lifestyle, and Common 33 A lterations of Cardiovascular Function Diseases, 164 in Children, 1194 6 Epigenetics and Disease, 183 UNIT X: THE PULMONARY SYSTEM UNIT III: MECHANISMS OF SELF-DEFENSE 34 Structure and Function of the Pulmonary System, 1225 7 Innate Immunity: Inflammation, 191 35 A lterations of Pulmonary Function, 1248 8 Adaptive Immunity, 224 36 A lterations of Pulmonary Function in 9 Alterations in Immunity and Inflammation, 262 Children, 1290 10 Infection, 298 UNIT XI: THE RENAL AND UROLOGIC 11 Stress and Disease, 338 SYSTEMS UNIT IV: CELLULAR PROLIFERATION: CANCER 37 S tructure and Function of the Renal and Urologic 12 Cancer Biology, 363 Systems, 1319 13 Cancer Epidemiology, 402 38 A lterations of Renal and Urinary Tract Function, 1340 14 Cancer in Children, 442 39 A lterations of Renal and Urinary Tract Function in Children, 1376 PART TWO: PATHOPHYSIOLOGIC UNIT XII: THE DIGESTIVE SYSTEM ALTERATIONS: ORGANS AND SYSTEMS 40 S tructure and Function of the Digestive System, 1393 41 A lterations of Digestive Function, 1423 42 A lterations of Digestive Function in Children, 1486 UNIT V: THE NEUROLOGIC SYSTEM UNIT XIII: THE MUSCULOSKELETAL SYSTEM 15 Structure and Function of the Neurologic System, 447 16 Pain, Temperature Regulation, Sleep, and Sensory 43 S tructure and Function of the Musculoskeletal Function, 484 System, 1510 17 Alterations in Cognitive Systems, Cerebral 44 A lterations of Musculoskeletal Function, 1540 Hemodynamics, and Motor Function, 527 45 A lterations of Musculoskeletal Function in Children, 18 Disorders of the Central and Peripheral Nervous 1591 Systems and the Neuromuscular Junction, 581 UNIT XIV: THE INTEGUMENTARY SYSTEM 19 Neurobiology of Schizophrenia, Mood Disorders, and Anxiety Disorders, 641 46 S tructure, Function, and Disorders of the 20 Alterations of Neurologic Function in Children, 660 Integument, 1616 47 A lterations of the Integument in Children, 1653 UNIT VI: THE ENDOCRINE SYSTEM UNIT XV: MULTIPLE INTERACTING SYSTEMS 21 Mechanisms of Hormonal Regulation, 689 22 Alterations of Hormonal Regulation, 717 48 S hock, Multiple Organ Dysfunction Syndrome, and Burns in Adults, 1668 UNIT VII: THE REPRODUCTIVE SYSTEMS 49 S hock, Multiple Organ Dysfunction Syndrome, and 23 Structure and Function of the Reproductive Burns in Children, 1699 Systems, 768 24 Alterations of the Female Reproductive System, 800 Glossary, 1728 WHAT’S NEW? Endoplasmic Reticulum, Protein Folding, and ER Stress, 6 Aldosteronism and Metabolic Syndrome, 756 From the Fluid Mosaic Model to New Understandings about Human Papillomavirus (HPV) Vaccine and Cancer Prevention in Biologic Membranes, 12 Women and Men, 775 Myocardial Hypertrophy, Stem Cells, Regeneration Controversy, 52 Precocious Puberty, 803 Cardioprotection for Ischemia-Reperfusion Injury, 58 Early Programming for PCOS? 811 The Percentage of Child Medication–Related Poisoning Deaths Is Vaginal Mesh, 819 Increasing, 63 Cervical Cancer Screening in the Developing World, 826 Low Level Lead Exposure Harms Children: A Renewed Call for Breast-Feeding and a New Emerging Target―NSAIDs for the Primary Prevention, 65 Prevention of Pregnancy-Associated Breast Cancer? 846 Cold Water Immersion and Cold Shock Response and the Diving Summary of the American College of Physicians (ACP) Guidance Response, 76 Statement on Screening for Prostate Cancer, 910 Health Risks Related to Use of LED Lighting Systems, 81 Sexually Transmitted Infection (STI) Statistical Summary from Decline in Life Expectancy in Some U.S. Counties, 94 2010 CDC Report, 919 Hyponatremia and Hospitalization, 113 HPV Vaccine, 935 Maternal Microchimerism and Autoimmune Disease, 275 Clinical Trials of Thrombopoietin (TPO) Receptor Agonists for Avian Influenza, 321 Treatment of Idiopathic Thrombocytopenic Purpura (ITP), 1073 Drug-Resistant Tuberculosis, 331 The Potential for Using Epicardial Cells in Myocardial Repair, 1086 Acute Emotional Stress and Adverse Heart Effects, 342 New Roles for the Arterial Adventitia, 1108 Psychosocial Stress and Progression to Coronary Heart Disease, 354 Growth of New Lymphatic Vessels from Macrophages, 1119 Telomeres, Disease, and Childhood Stress, 355 The Renin-Angiotensin-Aldosterone System (RAAS) and Cardio- Partner’s Survival and Spouse’s Hospitalizations and/or Death, 357 vascular Disease, 1134 Mechanical Stress and Tumors, 388 Obesity and Hypertension, 1136 Screening Mammography: Why It Has Not Lived Up to Expecta- Controversies in the Use of Medications for the Primary Preven- tions, 393 tion of Cardiovascular Disease, 1151 Global Cancer Transitions, 409 Cardiovascular Risk Reduction with Bariatric Surgery, 1152 Preventing Cancer—Protecting the Health of Current and Future Medical Devices—Are They Safe and Effective? 1156 Generations, 412 The Broken Heart Syndrome, 1162 CT Scans and Issues, 426-427 Metabolic Changes in Heart Failure, 1178 Far Fewer Genetic Mutations in Pediatric Cancer, 444 Brain Natriuretic Peptide and Heart Failure, 1179 CT Scans in Childhood Appear to Increase Cancer Risk, 445 Gene Therapy for Heart Failure, 1180 Astrocytes, 450 Ambulatory Blood Pressure Monitoring in Children, 1221 Nucleus Accumbens, Dopamine, and Drug Addiction, 460 Update on Hypoxic Pulmonary Vasoconstriction, 1232 Pain and Gender, 496 Update on Surfactant and Immunity, 1236 The Frontotemporal Disorders, 550 Pharmacogenetics and Beta Agonists in the Treatment of Asthma, Tourette Syndrome, 562 1266 Bell Palsy, 572 Genetic and Immunologic Breakthroughs in Lung Cancer Treat- Progesterone for Treatment of Traumatic Brain Injury (TBI), 589 ment, 1280 Copy Number Variations Increase in Schizophrenia and in Maternal Smoking: Fetal Lung Development and Lung Disease, Offspring of Older Fathers, 642 1293 Controversy in Mental Health Research, 645 Asthma Phenotypes, 1309 Hippocampal Neurogenesis and the Glucocorticoid Receptor Play The Kidney and Glucose Regulation, 1320 Essential Roles in Deterring Depression, 649 Catheter-Associated Urinary Tract Infection, 1349 Comprehensive Treatment of Risk Factors May Promote Resil- Biomarkers and Acute Kidney Injury, 1363 ience and Rapid Recovery from Depression, 652 Kidney Stones in Children, 1387 Reduction of Risks for Neural Tube Defects (NTDs), 663 Microscopic Colitis, 1441 Thyronamines, 702 Refeeding Syndrome, 1451 Vitamin D, 703 Acetaminophen and Acute Liver Failure, 1452 Growth Hormone (GH) and Insulin-like Growth Factor (IGF) in Abdominal Compartment Syndrome, 1466 Aging, 712 Pediatric Inflammatory Bowel Disease, 1500 Thyroid Disease and Iodinated Contrast Media (ICM), 725 Progress in Rebuilding Cartilage, 1525 The Role of Viruses in the Etiology of Type 1 Diabetes, 736 Tissue Engineering for Cartilage and Tendon, 1568 Immunodilation in the Prevention and Treatment of Type 1 Hidradenitis Suppurativa (Inverse Acne), 1631 Diabetes, 738 Anaphylactic Shock, 1675 Incretin-Based Therapies for Diabetes Mellitus, 741 Prolactin Levels, 1679 Bariatric Surgery in the Treatment of Diabetes, 742 Advances in Burn Wound Care, 1722 PATHOPHYSIOLOGY THE BIOLOGIC BASIS FOR DISEASE IN ADULTS AND CHILDREN ABOUT THE COVER IMAGE The cover image represents a stylized interface between genetics and epigenetics. Technological advances have changed our understanding of the genome and the role of transcriptional programs in the determination of cell fate and the response to environmental factors. We are beginning to understand how epigenetic modifications affect gene expression and alter cellular states without changing genomic DNA sequences. One analogy compares genetics to computer hardware with epigenetics as the software, software that may determine how the hardware behaves. Just as software can be tinkered with and erased, epigenetic marks may also be erasable, giving hope that we can intervene to change the landscape of the epigenome and prevent or cure disease. There is much to be learned and, over time, translated into clinical practice. PATHOPHYSIOLOGY THE BIOLOGIC BASIS FOR DISEASE IN ADULTS AND CHILDREN S E V E N T H E D I T I O N KATHRYN L. McCANCE, RN, PhD Professor Emeritus College of Nursing University of Utah Salt Lake City, Utah SUE E. HUETHER, RN, PhD Professor Emeritus College of Nursing University of Utah Salt Lake City, Utah SECTION EDITORS Valentina L. Brashers, MD Professor of Nursing and Woodard Clinical Scholar Attending Physician in Internal Medicine University of Virginia Health System Charlottesville, Virginia Neal S. Rote, PhD Academic Vice-Chair and Director of Research Department of Obstetrics and Gynecology University Hospitals Case Medical Center William H. Weir, MD. Professor of Reproductive Biology and Pathology Case Western Reserve University School of Medicine Cleveland, Ohio with more than 1200 illustrations 3251 Riverport Lane St. Louis, Missouri 63043 PATHOPHYSIOLOGY: THE BIOLOGIC BASIS FOR DISEASE IN ADULTS AND CHILDREN, SEVENTH EDITION ISBN: 978-0-323-08854-1 Copyright © 2014 by Mosby, an imprint of Elsevier Inc. Copyright © 2010, 2006, 2002, 1998, 1994, 1990 by Mosby, an affiliate of Elsevier Inc. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechan- ical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Details on how to seek permission, further information about the Publisher’s permis- sions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions. This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein). Notices Knowledge and best practice in this field are constantly changing. As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary. Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein. In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility. With respect to any drug or pharmaceutical products identified, readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications. It is the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions. To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors assume any liability for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein. Library of Congress Cataloging-in-Publication Data Pathophysiology (McCance) Pathophysiology: the biologic basis for disease in adults and children /[edited by] Kathryn L. McCance, Sue E. Huether; section editors, Valentina L. Brashers, Neal S. Rote. -- Seventh edition. p. ; cm. Preceded by Pathophysiology: the biologic basis for disease in adults and children / [edited by] Kathryn L. McCance, Sue E. Huether. 6th ed. c2010. Includes bibliographical references. ISBN 978-0-323-08854-1 (hardcover : alk. paper) I. McCance, Kathryn L. II. Huether, Sue E. III. Title. [DNLM: 1. Pathology--Nurses’ Instruction. 2. Disease--Nurses’ Instruction. 3. Physiological Phenomena--Nurses’ Instruction. QZ 4] RB113 616.07--dc23 2013033059 Senior Content Strategist: Sandra Clark Senior Content Development Specialist: Karen C. Turner Senior Content Coordinator: Brooke Kannady Publishing Services Manager: Jeffrey Patterson Senior Project Manager: Jeanne Genz Design Direction: Amy Buxton Multimedia Producer: Lisa Godoski Printed in Canada Last digit is the print number: 9 8 7 6 5 4 3 2 1 CONTRIBUTORS* Rose Ann Urdiales Baker, PhD RN, Susanna G. Cunningham, BSN, MA, Gwen Latendresse, PhD, CNM PMHCNS-BC PhD, FAAN, FAHA Assistant Professor Assistant Professor Professor University of Utah College of Nursing College of Health Professions School of Nursing Salt Lake City, Utah The University of Akron University of Washington Akron, Ohio Seattle, Washington Linda L. Martin, DNP, RN, CFNP Assistant Professor of Professional Practice Barbara Boss, PhD, APRN, FNP-BC, Alexa K. Doig, PhD, RN Texas Christian University ANP-BC Associate Professor Harris College of Nursing and Health Professor of Nursing and Director of DNP College of Nursing Sciences Program University of Utah Fort Worth, Texas University of Mississippi Medical Center Salt Lake City, Utah Jackson, Mississippi Sue A. McCann, MSN, RN, DNC Todd C. Grey, MD Programmatic Nurse Specialist Valentina L. Brashers, MD, FACP, FNAP Chief Medical Examiner—State of Utah University of Pittsburgh Medical Center Professor of Nursing and Woodard Clinical Associate Clinical Professor Pittsburgh, Pennsylvania Scholar Department of Pathology Attending Physician in Internal Medicine University of Utah School of Medicine Nancy L. McDaniel, MD University of Virginia Health System Salt Lake City, Utah Associate Professor of Pediatrics Charlottesville, Virginia University of Virginia Mary Fran Hazinski, RN, MSN, FAAN, Charlottesville, Virginia Kristen Lee Carroll, MD FAHA, FERC Associate Professor, Orthopedics Professor, Vanderbilt University School of Mary A. Mondozzi, MSN, RN University of Utah Nursing Burn Center Education/Outreach Salt Lake City, Utah Assistant, Departments of Surgery and Coordinator Shriner’s Intermountain Unit Pediatrics, Vanderbilt University School Akron Children’s Hospital Shriner’s Hospital for Children of Medicine The Paul and Carol David Foundation Burn Salt Lake City, Utah Clinical Nurse Specialist Institute Monroe Carell, Jr. Children’s Hospital at Akron, Ohio Dennis Cheek, RN, PhD, FAHA Vanderbilt Abell-Hanger Professor of Gerontology Nashville, Tennessee Stephen E. Morris, MD Nursing Associate Professor of Surgery Harris College of Nursing and Health Robert E. Jones, MD, FACP, FACE Director, University of Utah Burn Center Sciences Professor of Medicine Salt Lake City, Utah Texas Christian University University of Utah School of Medicine Fort Worth, Texas Salt Lake City, Utah Noreen Heer Nicol, PhD, RN, FNP, NEA-ABC Margaret F. Clayton, PhD, APRN Lynn B. Jorde, PhD Associate Professor Associate Professor, College of Nursing H.A. and Edna Benning Presidential University of Colorado, College of Nursing University of Utah Professor and Chair Director, Children’s Hospital Colorado Salt Lake City, Utah Department of Human Genetics Denver, Colorado University of Utah School of Medicine Christy L. Crowther-Radulewicz, MS, Salt Lake City, Utah Julia Phillippi, PhD, CNM, FACNM CRNP Assistant Professor Nurse Practitioner Lynne M. Kerr, MD, PhD Vanderbilt University School of Nursing Anne Arundel Orthopedic Surgeons Associate Professor Nashville, Tennessee Annapolis, Maryland Departments of Pediatrics and Neurology Adjunct Faculty University of Utah Medical Center Patricia Ring, RN, PNP-BC Johns Hopkins University School of Nursing Salt Lake City, Utah Pediatric Nephrology Nurse Practitioner Baltimore, Maryland Children’s Hospital of Wisconsin Nancy E. Kline, PhD, RN, CPNP, FAAN Milwaukee, Wisconsin Director, Nursing Research, Medicine Patient Services/Emergency Department George W. Rodway, PhD, RN, ANP *The authors also would like to thank the Boston Children’s Hospital Assistant Professor previous edition contributors. Boston, Massachusetts Orvis School of Nursing University of Nevada—Reno Reno, Nevada v vi CONTRIBUTORS Neal S. Rote, PhD Anna L. Schwartz, PhD, FNP-BC, FAAN Lorey K. Takahashi, PhD Academic Vice-Chair and Director of Associate Professor Professor of Psychology Research School of Nursing University of Hawaii at Manoa Department of Obstetrics and Gynecology Northern Arizona University Honolulu, Hawaii University Hospitals Case Medical Center Flagstaff, Arizona William H. Weir, MD. Professor of Gillian Tufts, DNP, FNP-C Reproductive Biology and Professor of Richard A. Sugerman, PhD Associate Professor Pathology Professor of Anatomy, Emeritus Clinical College of Nursing Case Western Reserve University School of Director of Service Learning Projects University of Utah College of Nursing Medicine College of Osteopathic Medicine of the Salt Lake City, Utah Cleveland, Ohio Pacific Pomona, California REVIEWERS Nancy M. Burruss, PhD, RN, CNE Carol Anne Marchetti, PhD, RN, CNS, Abby Saunders, MS, PA-C Associate Professor NP Physician Assistant Bellin College Assistant Professor School of Health and Medical Sciences Green Bay, Wisconsin Bouve College of Health Sciences Seton Hall University School of Nursing South Orange, New Jersey David J. Derrico, RN, MSN Northeastern University Clinical Assistant Professor Boston, Massachusetts Lorna L. Schumann, PhD, NP-C, ACNS, Adult and Elderly Department BC, ACNP, BC, CCRN-R, FAANP University of Florida College of Nursing Denise Morita, MD Associate Professor Gainesville, Florida Assistant Professor College of Nursing Department of Pediatrics Washington State University Diane P. Genereux, PhD Division of Pediatric Neurology Spokane, Washington Assistant Professor University of Utah School of Medicine Department of Biology Salt Lake City, Utah Karin C. VanMeter, PhD Westfield State University Lecturer Westfield, Massachusetts Jason Mott, PhD, RN Department of Biomedical Sciences Instructor of Nursing College of Veterinary Medicine Sandra L. Kaminski, MS, PA-C Nursing Department Iowa State University Assistant Professor Bellin College Ames, Iowa School of Health and Medical Sciences Green Bay, Wisconsin Seton Hall University South Orange, New Jersey Jane Cross Norman, PhD, RN, CNE Masters of Science in Nursing Fei Li, PhD Program Director Assistant Professor Tennessee State University Department of Biology Nashville, Tennessee New York University New York, New York vii
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