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The Nature of Disease: Pathology for

the Health Professions 2nd Edition,


(Ebook PDF)
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Reviewers

We gratefully acknowledge the generous assistance of Mark Lafferty


the reviewers whose names appear in the list that fol- Science Department Chair
lows. These individuals were kind enough to provide Delaware Technical and Community College
input on different aspects of this text; their comments Wilmington, DE
helped shape its final form.
Rene Lapierre
Margy Blankenship Coordinator, “Soins Paramédicaux”
Chair, Health Division Collège Boréal
Kentucky Community & Technical College System Ontario, Quebec, Canada
Somerset, KY
Susan Leftwich Sale
Gerald Callahan Level Coordinator, Faculty
Associate Professor Riverside School of Professional Nursing, Riverside
Microbiology, Immunology, and Pathology School of Health Careers
Colorado State University Newport News, VA
Fort Collins, CO
Steve Moon
David Derrico Instructor, Allied Medicine
Assistant Clinical Professor, Nursing College of Medicine, The Ohio State University
University of Florida Columbus, OH
Gainesville, FL
John Olson
Bertha C. Escobar-Poni Lecturer
Associate Professor, Pathology and Human Anatomy Arizona State University
School of Medicine Phoenix, AZ
Loma Linda University
Loma Linda, CA Alisa Petree
Instructor/Clinical Coordinator
Jacquelyn Harris Medical Laboratory Technician
Medical Department Chair McLennan Community College
Bryan College Waco, TX
Springfield, MO
Christine Recktenwald
Lisa Hight Assistant Teaching Professor
Associate Profesor, Biology College of Nursing at University of Missouri—
Baptist College of Health Sciences St. Louis
Memphis, TN St. Louis, MO

Jody LaCourt Vickie Roettger


Senior Teaching Specialist Profesor
University of Minnesota Missouri Southern State University
Minneapolis, MN Joplin, MO

vi
Reviewers vii

Sandra A. Sieck Wanda Thuma-McDermond


Program Director Associate Professor of Nursing
UW-L-Gunderson-Mayo PA Program Messiah College
LaCrosse, WI Mechanicsburg, PA

J. Steve Smith Karen Tombs-Harling


Biology Department Dean, Academic Affairs
Universityof West Florida Harrisburg Area Community College
Pensacola, FL Harrisburg, PA

Becky J. Socha, Sheila Trahan


Adjunct Faculty Academic Department Chair, Allied Health
Merrimack College Lamar Institute of Technology
North Andover, MA Beaumont, TX

Gina Stephens
Program Chair, Medical Business Administrative
Technologies
Georgia Northwester Technical Colleage
Rome, GA
Preface

This Second Edition of The Nature of Disease (TNOD) paper, I know that brevity, manner, and style are the
is, like the first edition, written for a particular audi- essence of written communication. TNOD adopts a
ence: students in the health professions. deliberately casual narrative style, which served me well
In this edition, I combine three important features to in medical practice. It makes reading easier, holds the
bring students a unique learning experience. reader’s attention, and enhances understanding and
recall of important points without sacrificing scientific
• First, my writing style is deliberately casual. It is a nar-
relevance.
rative (storyteller) style, which is less formal than the
TNOD focuses on answering the most important
stiff prose that populates similar textbooks. My expe-
questions that students have about every disease—
rience shows that it makes reading and learning easier.
definition of the condition, its cause, how the anatomy
• Second, each chapter opens with a review of normal
and physiology change and evolve, how it is diagnosed
anatomy and physiology. Given that pathology and
and treated, and the outlook. Along the way, the text
pathophysiology are nothing more than normal
uses a number of devices to deepen understanding,
anatomy and physiology gone wrong, a brief review
retain interest, and enhance recall:
prepares the reader for the disease discussions that
follow. • Much of the molecular and microscopic detail typically
• Third, each chapter focuses on one or more case stud- found in similar textbooks has been eliminated. Each
ies, which bind the material together and make it chapter focuses on the essentials necessary to build a
more memorable. broad, fundamental understanding, with supporting
detail where relevant.
Classroom Vetted • New terms are boldfaced and defined at their first use
in the narrative. This practice alerts the reader to the
importance of the new term, which is defined in the
TNOD literally grew out of a classroom. When I joined
same sentence, or the one immediately following.
the academic community in 1997 after a career in the
Terms of secondary importance are italicized.
laboratory business, the classroom was an alien place to
• Selected important phrases are italicized for emphasis.
me. I puzzled over the fact that the students I taught,
For example, in Chapter 7, Disorders of Blood Cells,
who were of the very highest quality, still had trouble
the following italicized phrase emphasizes the threat
grasping the material. I began to pay more attention
of colon cancer: . . . until proven otherwise, the cause
to the textbooks available, and learned the student
of iron deficiency anemia in adult men or postmeno-
perspective of most pathology texts: they are difficult
pausal women is occult (undetected) bleeding from the
to read.
gastrointestinal tract.
Much of the difficulty springs from the fact that
• The narrative is sprinkled with quotations—serious,
most pathology books are compilations written by mul-
whimsical, or humorous—to humanize the material
tiple authors, each with a certain writing style and with
and make the subject matter more memorable. For
differing views about the relative importance of things.
example, Chapter 9, Disorders of the Heart, begins
Their style is generally formal. The text doesn’t flow,
with a line from country and western singer Tim
and reading is bare of enjoyment. I avoid these prob-
McGraw’s tune, “Where the Green Grass Grows”:
lems by bringing a single point of view and a natural
“. . . another supper from a sack, a ninety-nine cent
writing style that is easy to read and remember.
heart attack. . . .” This snippet of lyric speaks vol-
umes about the American diet and heart disease, and
Approach students invariably enjoy and remember it.
• History of Medicine boxes further humanize the nar-
Having spent much of my professional life commu- rative by presenting historical anecdotes that put in its
nicating with busy physicians buried in a blizzard of historical perspective. For example, in Chapter 23,

viii
Preface ix

Disorders of Daily Life, the box titled French Food, twist to some of them, which imagines a better outcome
Fast Food, Fat Food discusses the history of res- for the patient had the case unfolded in a different way.
taurants, the development of fast food in America, For example, in Chapter 8, Disorders of Blood Vessels,
and the devastating rise of obesity in America since the case is that of a man found dead in his office. He
World War II. Study of the history of medicine had a history of high blood pressure, obesity, lack of
makes the scienctific points memorable in a way not exercise and tended not to take his antihypertension
achievable otherwise. prescription drugs. The alternative scenario imagines
the patient behaving differently—losing weight, taking
Cases his blood pressure medicine regularly, exercising, and
watching his diet—and living happily ever after.
Each chapter is built around one or more real-life cases.
Learning about disease, its development, and its effects Organization
is an academic exercise, which requires orderly study
using textbooks, lectures, seminars, journals, lab ex- Although this textbook is unique in many ways, it
periments, and so on. is organized in a familiar fashion: it presents general
Another important tool in understanding this sub- pathology and pathophysiology first and follows with
ject is case studies. The case-study method for learning discussions of disorders of organ systems.
medicine is as old as medical science. A case study is Part 1, Mechanisms of Health and Disease, opens with
the “story” of a particular patient and the course of a chapter titled Health and Disease, which discusses the
their condition over a period of time. Cases human- nature of d ­ isease—that is, the intimate relationship be-
ize and particularize medicine in a way that no other tween form and function in health and in illness. This
method can. Diseases occur in people, and people vary chapter also emphasizes the difference between the dis-
greatly from one to another. Every disease occurs in ease itself and the signs and symptoms it produces. The
someone of a certain age, sex, and ethnicity; someone failure of healthcare professionals and their patients to
who lives a certain lifestyle, and who, for good or ill, appreciate this distinction accounts for a great deal of
has found a certain niche in life. Every one of these medical misdirection and misunderstanding. The re-
characteristics relates in some way to the condition maining chapters in Part 1 deal with pathologic forces
from which they are suffering when we meet them in that can affect any part of the body: the life and death
the chapters. of cells, inflammation and repair, immune disorders,
Cases are chosen to illustrate a point, usually about infections, neoplasia, and aberrations of fluid balance
disease behavior, diagnosis, or treatment. Typically they and blood flow.
are written in the past tense, do not use the patient’s Part 2, Dis­orders of Organ Systems, expands on the
real name, and follow a certain form, (discussed in understanding established in Part 1 by discussing con-
detail below). Individualizing disease by presenting it ditions of the various organs and organ systems. Along
in living, breathing, flesh-and-blood form enhances the way, the narrative is stitched together with liberal
learning. use of cross-references to other material. In early chap-
The idea is to make the story memorable, the better ters cross-references steer the reader to more detailed
from which to learn, by telling the story as it actually discussion in later chapters. In later chapters they r­ ecall
happened and by including the unadorned facts, some earlier discussion of basic concepts. For ­example, in
of which may not be flattering to patients or c­ aregivers. Chapter 17, Disorders of the Female ­Genital Tract and
In this book, we have condensed cases to their essence, Breast, the discussion of dysplasia of the cervix calls
which means I have eliminated unimportant detail. on the reader to understand the concept of metaplasia,
And in some cases, I have added a bit of fiction to the which was defined and discussed initially in Chapter 2.
illness to add teaching value. I am confident you will Part 3, Disorders of the Stages and States of Life, fuses
find these memorable, because all of them are real knowledge gained in Parts 1 and 2 into discussions
people in some disguise. of disorders associated with states of being. We suffer
Many TNOD cases are supplemented by “What ­differently as we progress from fetus, to neonate, to
if . . .?” alternative scenarios that are posted online child, to adult, and to old age. Foremost we are prison-
at thePoint.com. This feature is entitled The Road Not ers of our genes—some grant partial exemption from
Taken—An Alternative Scenario. Because most of the risk, others are the outright cause of disease. To a lesser
cases are derived from autopsy material, I have added a extent we are prisoners of environment and habit.
x Preface

We benefit from careful diet, exercise and helpful stress. Instructor Resources
Or we suffer from harmful stress malnutrition, obesity, in-
dolence, tobacco use or illicit drug abuse. We are always In addition to the student resources (see Digital Con-
at risk of trauma and the problem of pain is universal. nections on page xiii) instructors also have access to the
following teaching tools:
Art Program
• PowerPoint slides with accompanying lecture notes
• Image bank of figures from the text
No textbook of pathology can succeed without a first-
• Answers to Chapter Challenge review questions in
rate art program. Line art simplifies the structures and
the text
concepts depicted by distilling them to their basic,
• Test generator with more than 2,000 questions
most easily recognizable forms, while photographs
show anatomic structures as they appear in real life.
The Nature of Disease is richly illustrated with both. Summary
Text discussions are augmented by more than 560
full-color figures. In keeping with the core notion that I trust you will learn by study of the material. But more
anatomic form and function go hand in hand, this text- than that, I hope you will enjoy reading it. I have spent
book contains more gross clinical photographs of pa- a great deal of time and energy to make the science
tients and pathologic photographs of organs, tissues and clear and the reading easy. This book is larded with hu-
cells than comparable texts. Each photograph illustrates manity in order to make the science easy to grasp and
a critical point and is intended to speak for itself. The retain. My hope is that this distinctive approach will
guiding principle in developing medical line art is that entertain and will give disease a human face.
good art should be understandable at a glance, or with So, here it is; judge for yourself. And after you have
minimal study. Our line drawings have been designed to judged, I want you to tell me what you think. This is
be esthetically pleasing and to guide the reader’s thought no idle invitation—please send your comments, sug-
without refering to the text or to read a lengthy legend. gestions, praise, or criticism to me at thmiii@gmail.com
A full description of each of the text’s features as
well as the online resources can be found in Chapter Thomas H. McConnell, MD, FCAP
Features: A Guided Tour, which begins on the next page. Dallas, Texas
Chapter Features: A Guided Tour

Each chapter content begins with a discussion of normal anatomy and physiology, provid-
ing just the right amount of information to support the disease discussions that follow.
The disease discussions are the core of each chapter and consist of a narrative examina-
tion of the many disorders that may arise.
The discussion of normal anatomy and physiology and of diseases and disorders is
enhanced by the following features:

The Contents list outlines major


headings and subheadings—
providing an at-a-glance look
at the material covered and its
organization.

Chapter Objectives follow the


chapter outline and are sorted
and displayed according to the
headings under which they
are discussed.

The Case Study that opens each chapter’s content


(some chapters contain more than one) is pre-
sented in the usual clinical fashion:
• Chief Complaint. The problem that stimulated
the patient to seek care.
• Clinical History. The timeline of signs and
symptoms relating to the current illness.
• Physical Examination and Othe Data. Physical,
laboratory and imaging data.
• Clinical Course. The story of treatment, further
diagnosis, and recovery or death.

Brief Quotations help to illustrate


the main idea of each chapter in an
entertaining and informative way.

xi
xii Chapter Features: A Guided Tour

Case Study Revisited. The case and its outcome is re-


viewed discussed at the end of the chapter to reinforce
the science discussed in the chapter.

Chapter Challenge. Finally, each chapter ends with the


Chapter Challenge, a comprehensive list of noncase
questions and tasks that covers all of the chapter mate-
rial. Answers are provided in the instructor’s material.

Chapters are sprinkled with two types of questions, visual reminders of key points, and
special boxed features.

Case notes. These are case-related questions that pertain to the re-
lationship between the case and the topic at hand. For example, in
Chapter 9, Disorders of the Heart, there is a discussion on the prin-
ciple mechanisms of heart disease (e.g., pump failure, obstructed
flow, etc.). The patient, ­Willard, suffered from stenosis of the aortic
valve. Case Note 9.3 is “Which of the five principle mechanisms of
heart disease did Willard have?” Answers are posted online.

Pop Quiz. At the end of each major chapter heading is a list of


questions related to the material covered under the heading.
These are short, straightforward queries designed to solidify
knowledge while it is fresh and readily available by quick restudy.
Answers are posted online.

Remember This. Within narrative sections,these highlighted state-


ments emphasize the most important ideas, or suggest other ways
to hep you remember key facts.

The Clinical Side presents supplemental information designed to high-


light the patient side of a topic. For example, in Chapter 9, Disorders of
the Heart, the box title is Lifestyle and Coronary ­Artery Disease. The box
asks, What Can the Average Joe or Jane Do to Prevent a Heart A ­ ttack?, then
briefly provides an answer, elements of which are sprinkled throughout
the chapter narrative.
Chapter Features: A Guided Tour xiii

Molecular Medicine presents additional insight into disease at the mo-


lecular level. Topics are simple molecular mechanisms that are easy to
grasp and supplment understanding of chapter material. For example, in
Chapter 8, Disorders of Blood Vessels, the box title is A Tale of Two Sources
of Cholesterol. The box briefly discusses the sources of blood cholesterol
and the molecular mechanisms of cholesterol-lowering drugs.

History of Medicine boxes present interesting stories about the de-


velopment of medical science. For example, it is well-­established
today that coronary artery disease is common and fatal and is
accompanied by clinical signs and symptoms known even to the
average person on the street. It is easy to assume this has always
been the case, but the History of Medicine box in Chapter 9,
Disorders of the Heart, points out that chest pain and death were
attributed to coronary artery disease only 100 years ago.

Digital Connections: Reinforcing and Enhancing Learning


Visit thePoint.lww.com and reinforce your Chapter 4:
learning with the following:
• Answers to Case Note questions • Animation: The Chain of Infection
• Answers to Case Note and Pop Quiz questions • The Road Not Taken: Case Study Alternative Scenario
• Glossary of Key Terms • Supplemental Case Study: “A spider bit me.” The
Animations, supplemental box content, supplemental case of D.W.
case studies, and “The Road Not Taken” (an alternative • History of Medicine: Who was Syphilus?
ending for selected chapter case studies) enhance your • History of Medicine: The Tuskegee Syphilis Experiment
learning and expand your understanding. In addition • Lab Tools: How Do I Know If a Patient with a Genital
to the resources above, you can access the following on Ulcer Has Syphilis?
thePoint.com • Lab Tools: Serologic Tests for Syphilis (STS)
• History of Medicine: The Discovery of Antibiotics
Chapter 1: • History of Medicine: The History of Tuberculosis

• Animation: Acute Inflammation Chapter 5:


• Animation: The Cell Cycle
• Animation: Wound Healing • The Road Not Taken: Case Study Alternative Scenario
• Supplemental Case Study: “I have a chest cold that
Chapter 3: won’t go away.” The Case of Tina D.
• History of Medicine: Where There’s Smoke, There’s Cancer
• Animation: The Immune Response
• The Road Not Taken: Case Study Alternative Scenario Chapter 6:
• Molecular Medicine: Detecting Autoimmune
Antibodies • Animation: Hemostatis
• The Clinical Side: Coombs Test • The Road Not Taken: Case Study Alternative Scenario
xiv Chapter Features: A Guided Tour

• Supplemental Case Study: “She’s gone.” The case of Rita B. • The Clinical Side: Measurement of Glomerular Fil-
• Lab Tools: Mixing Blood and Water tration Rate

Chapter 7: Chapter 17:


• Animation: Oxygen Transport • The Road Not Taken: Case Study Alternative Scenario
• The Road Not Taken: Case Study Alternative Scenario • The Clinical Side: The Difference Between Absolute
• Lab Tools: Measurement of Total Red Cell Mass and Relative Risk
• History of Medicine: “The Royal Disease” • The Clinical Side: Long-Term Estrogen Replacement
Therapy
Chapter 8:
Chapter 19:
• Animation: Hypertension
• The Road Not Taken: Case Study Alternative Scenario • Animation: Action Potential
• Animation: Nerve Synapse, Stroke
Chapter 9: • History of Medicine: Strokes in United States
Presidents
• Animation: The Cardia Cycle
• Animation: Congestive Heart Failure (CHF) Chapter 20:
Chapter 10: • History of Medicine: Braille
• The Clinical Side: Diagnosing Glaucoma
• Animation: Asthma, Gas Exchange
• The Road Not Taken: Case Study Alternative Scenario
Chapter 21:
Chapter 11:
• Animation: The Immune Response
• The Road Not Taken: Case Study Alternative Scenario
• Animation: Digestion of CHO
• History of Medicine: John D. Rockefeller, Sr.’s Hair
• Animation: General Digestion

Chapter 12: Chapter 22:

• Animation: Cirrhosis • The Road Not Taken: Case Study Alternative Scenario
• History of Medicine: The History of DNA
Chapter 13: • Lab Tools: Laboratory Diagnosis in Genetic Disease
• Molecular Medicine: Meiosis—From 46 Chromo-
somes to 23 and Back Again
• Animations: Diabetes, Hormone Control (Insulin
• The Clinical Side: Diagnosis of Cytogenetic Defects
and Glucose Metabolism)
• The Road Not Taken: Case Study Alternative Scenario
• Supplemental Case Study: “He drinks; I don’t.” The Chapter 23:
case of Charisa M.
• The Road Not Taken: Case Study Alternative Scenario
Chapter 14: • History of Medicine: Benjamin Franklin and Lead
Toxicity
• History of Medicine: President John F. Kennedy and • The Clinical Side: Metabolic Rate and Aging
Addison Disease
Chapter 24:
Chapter 15:
• The Clinical Side: Eat Less, Live Longer?
• Animation: Renal Function
Acknowledgments

There was a time when I paid little attention to Ac- executive acquisition editor, oversaw the first edition
knowledgments pages in books. That was before I and continued his role with this second edition.
became an author and realized how critical are the con- In publishing a medical textbook many people are
tributions of people whose names are not on the cover. working independently on pieces of the whole. There
The first edition of TNOD would not have occurred are the text documents (in successive versions), the art
but for a chain of unlikely events that led me into aca- and photographs (in separate successive versions), the
demia after a career as a practicing pathologist. It began design team and its work products, the compositors
in June 1997 when I answered the phone to hear the who assembled all into the final layout you are now
voice of Lynn Little, a former employee I’d not heard examing, and the printers. Eve Klein, Senior Product
from in years. He was calling in his capacity as Chair- Development Editor, kept us and our work organized
man of the Medical Laboratory Sciences department and on time. This was no mean feat.
in the UT Southwestern Allied Health Sciences School I fancied myself good with words until I got into
(now the School of Health Professions). Lynn asked if I the hands of professional editors. To an extent that
would be interested in teaching the required pathology would surprise those not familiar with the editorial
course. Being somewhat at loose ends at the time, and process, this second edition is the product of the superb
having narrowly chosen private practice over academia editorial skills of Development Editor Laura Bonaz-
30 years earlier, I leapt at the chance. zoli. Laura collected and codified critiques of the first
Then came the task of assembling course materials edition and suggested structural reorganization of the
from the archives in the pathology department of UT content of this second edition. On a smaller scale she
Southwestern Medical School. Beni Stewart, guru in made chapter-by-chapter detailed recommendations
the photography lab, and Beverly Shackelford, Supervi- about sequence, organization, and emphasis. Later, as
sor of Education Programs, guided me through a huge draft chapters emerged, her medical knowledge added
collection of microscopic slides and photo images and materially to chapter scientific accuracy and complete-
helped me assemble the rudiments of a course. ness, and her skill with words and grammar greatly
Next I created an outline for students, which after improved text flow and comprehension.
a few years evolved into a ring-bound textbook. Soon Finally, a few words about Vera Paulson, MD, PhD,
word spread, other institutions began wanting to use and Mark Valasek, MD, PhD, products of the MD/PhD
my materials and before long I found myself in the program at UT Southwestern. Their work is embed-
publishing business. This was time-consuming, so I ded on every page. I wrote the first and final drafts
decided to mail copies to about two dozen publishers. of each chapter, but they independently combed the
One landed at Lippincott Williams and Wilkins. Several intermediate drafts for scientific completeness and
other publishers were interested, but it didn’t take long accuracy. Each also has knack for organizing thoughts
for Lippincott to rise to the top of the heap by virtue of and a way with words that greatly improved the final
plainly evident professionalism. product.
Then came the formal editorial process, completely
new to me, which proved to be one of the best educa- Thomas H. McConnell, MD, FCAP
tional experiences in a lifetime of learning. David Troy, Dallas, Texas

xv
Contents

Dedication v 11 Disorders of the Gastrointestinal Tract 305


Reviewers vi
12 Disorders of the Liver and Biliary Tract 351
Preface viii
Chapter Features: A Guided Tour xi
13 Disorders of the Pancreas 384

Acknowledgments xv 14 Disorders of the Endocrine Glands 409

Part 1 15 Disorders of the Urinary Tract 445

Mechanisms of Health and Disease 16 Disorders of the Male Genitalia 488

1 Health and Disease 2 17 Disorders of the Female Genitalia


and Breast 507
2 Cellular Pathology: Injury, Inflammation,
and Repair 16 18 Disorders of Bones, Joints, and Skeletal
Muscle 556
3 Disorders of the Immune System 43
19 Disorders of the Nervous System 595
4 Infectious Disease 79
20 Disorders of the Senses 638
5 Neoplasia 114
21 Disorders of the Skin 671
6 Disorders of Fluid, Electrolyte and Acid-Base
Balance, and Blood Flow 140
Part 3
Part 2 Disorders of the Stages
and States of Life
Disorders of the Organ Systems
22 Congenital and Childhood Disorders 706
7 Disorders of Blood Cells 177
23 Disorders of Daily Life 741
8 Disorders of Blood Vessels 210
24 Aging, Stress, Exercise, and Pain 768
9 Disorders of the Heart 237
Index 788
10 Disorders of the Respiratory Tract 276

xvi
Expanded Contents

Dedication v Fungus Infections 101


Parasite Infections 102
Reviewers vi Sexually Transmitted Infections 105
Laboratory Tools 110

Preface viii
5 Neoplasia 114
Characteristics and Definitions of Neoplasia 116
Chapter Features: A Guided Tour xi
The Causes of Cancer 118
The Molecular Basis of Neoplasia 120
Acknowledgments xv The Biology of Neoplastic Growth 121
Clinical Manifestations 127
Part 1 Clinical and Laboratory Assessment
of Neoplasms 129
Mechanisms of Health and Disease Cancer Treatment 134
Early Detection and Prevention of Cancer 136
1 Health and Disease 2
What Is Disease? 3 6 Disorders of Fluid, Electrolyte and Acid-Base
How Do Scientists Study Disease? 5 Balance, and Blood Flow 140
What Causes and Influences Disease? 6 Pressure and the Movement of Body Fluids 142
How Is Disease Expressed? 7 Fluid Flow in Blood Vessels and Lymphatics 145
How Are Medical Tests Interpreted? 9 Water and Fluid Compartments 145
Fluid Imbalance 148
2 Cellular Pathology: Injury, Inflammation, Electrolyte Imbalances 152
and Repair 16 Acid-Base Imbalance 154
Cell Reproduction and Differentiation 17 Hyperemia and Congestion 157
Cell Injury, Disease, and Death 20 Hemostasis 158
The Inflammatory Response to Injury 26 Hemorrhage 160
Repair 35 Thrombosis 163
Disseminated Intravascular Coagulation 166
3 Disorders of the Immune System 43 Embolism 166
Non-Immune Defense Mechanisms 46 Infarction 167
Lymphoid Organs and the Lymphatic System 48 The Collapse of Circulation: Shock 169
Innate and Adaptive Immunity 48
Cells of the Immune System 51
B Lymphocyte (Antibody)-Mediated Immunity 51
Part 2
T Lymphocyte (Delayed)-Mediated Immunity 53 Disorders of the Organ Systems
Hypersensitivity Reactions 54
Allergic Disorders and Atopy 57 7 Disorders of Blood Cells 177
Autoimmune Disorders 60 The Formed Elements of Blood 179
Amyloidosis 64 Laboratory Assessment of Formed
Immunity in Tissue Transplantation and Blood Elements 183
Transfusion 65 Anemia 183
Immunodeficiency Disorders 69 Polycythemia 194
Leukopenia, Leukocytosis, and Lymphadenopathy 194
4 Infectious Disease 79 Overview of Malignancies of White Blood Cells 195
The Biology of Infectious Disease 82 Myeloid Malignancies 197
Virus Infections 89 Lymphoid Malignancies 200
Bacterial Infections 92 Disorders of the Spleen and Thymus 206

xvii
xviii Expanded Contents

8 Disorders of Blood Vessels 210 Viral Hepatitis 363


Non-Viral Inflammatory Liver Disease 370
Overview of Vascular Structure
and Functioning 212 Toxic Liver Injury 370
Hypertensive Vascular Disease 218 Metabolic Liver Disease 373
Atherosclerosis 222 Disease of Intrahepatic Bile Ducts 375
Aneurysms and Dissections 227 Circulatory Disorders 376
Vasculitis 230 Tumors of the Liver 377
Raynaud Syndrome 231 Disorders of the Gallbladder and Extrahepatic Bile
Ducts 378
Diseases of Veins 231
Tumors of Blood and Lymphatic Vessels 232
13 Disorders of the Pancreas 384
9 Disorders of the Heart 237 Normal Pancreatic Physiology 387
Pancreatitis 388
The Uniqueness of the Heart 239
Diabetes 394
Heart Failure 244
Pancreatic Neoplasms 403
Coronary Artery Disease 248
Valvular Heart Disease 255
Diseases of the Myocardium 260 14 Disorders of the Endocrine Glands 409
Pericardial Disease 262 The Normal Endocrine System 411
Congenital Heart Disease 263 Disorders of the Pituitary Gland 417
Tumors of the Heart 266 Disorders of the Thyroid Gland 422
Cardiac Arrhythmias 267 Disorders of the Adrenal Cortex 431
Disorders of the Adrenal Medulla 438

10 Disorders of the Respiratory Tract 276 Disorders of the Parathyroid Glands 439
Multiple Endocrine Neoplasia Syndromes (MEN) 441
The Normal Respiratory Tract 278
Lung Volume, Air Flow, and Gas Exchange 281
Diseases of the Upper Respiratory Tract 283 15 Disorders of the Urinary Tract 445
Atelectasis 284 The Normal Urinary Tract 447
Pulmonary Edema 285 Urine 453
Acute Respiratory Distress Syndrome 285 Urinary Obstruction 457
Obstructive Lung Diseases 286 Urolithiasis 460
Restrictive Lung Diseases (Diffuse Interstitial Disease) 291 Neoplasms of the Urinary Tract 461
Vascular and Circulatory Lung Disease 293 Congenital Anatomic Abnormalities 465
Pneumonia 294 Infection and Inflammation 466
Lung Neoplasms 298 Voiding Disorders 466
Diseases of the Pleura 301 Clinical Presentations of Renal Disorder 470
Inherited, Congenital, and Developmental Disorder 471

11 Disorders of the Gastrointestinal Tract 305 Glomerular Disorders 472


Tubular and Interstitial Disorders 479
The Normal Gastrointestinal Tract 307
Pyelonephritis 481
Signs and Symptoms of Gastrointestinal Disorder 314
Vascular Disorder 483
Diseases of the Oral Cavity 318
Diseases of the Esophagus 322
Diseases of the Stomach 323 16 Disorders of the Male Genitalia 488
Congenital Anomalies of the Small and Large Bowel 328 The Normal Male Genital System 490
Vascular Diseases of the Small and Large Bowel 329 Disorders of Reproductive Function 493
Infectious Diseases Affecting the Small and Large Disorders of the Penis, Scrotum, and Groin 494
Bowel 331 Disorders of the Epididymis and Testis 496
Malabsorption Syndromes 333 Disorders of the Prostate 499
Inflammatory Bowel Disease 335
Neoplasms of the Large and Small Bowel 339 17 Disorders of the Female Genitalia and
Colonic Diverticulosis and Anorectal Conditions 345 Breast 507
Diseases of the Appendix and Peritoneum 347 The Normal Female Genitalia 510
The Pituitary-Ovarian-Endometrial Cycle 512
12 Disorders of the Liver and Biliary Tract 351 Pregnancy 515
The Normal Liver 353 Infertility 521
The Liver Response to Injury 357 Vulvar Disorder and Vaginitis 522
Expanded Contents xix

Disorders of the Cervix 523 Disorders of the Inner Ear 665


Disorders of the Endometrium and Myometrium 532 Normal Taste and Smell 666
Disorders of the Ovary 538 Disorders of Taste and Smell 667
The Normal Breast 543 Normal Somatic Senses 667
Evaluation of Breast Disorders 544 Disorders of Somatic Senses 668
Benign Breast Conditions 545
Breast Cancer 547 21 Disorders of the Skin 671
Normal Skin 675
18 Disorders of Bones, Joints, and Skeletal General Conditions of Skin 677
Muscle 556 Infections, Infestations, Bites, and Stings 680
The Normal Skeleton 557 Disorders of Hair Follicles and Sebaceous Glands 683
Normal Joints 561 Dermatitis 684
Normal Skeletal Muscle 562 Diseases of the Dermis and Subcutis 689
Disorders of Bone Growth, Maturation, Modeling, Blistering Diseases 689
and Maintenance 565 Disorders of Pigmentation and Melanocytes 690
Fractures 570 Neoplasms of Skin Not Including Melanoma 696
Bone Infarction and Infection 572 Disorders of Hair and Nails 700
Bone Tumors and Tumor-Like Lesions 573
Arthritis 577
Injuries to Joints and Periarticular Tissues 583 Part 3
Periarticular Pain Syndromes 584 Disorders of the Stages
Tumors and Tumor-Like Lesions of Joints and Soft and States of Life
Tissues 586
Pathologic Reactions of Muscle 588
Myopathies 589
22 Congenital and Childhood Disorders 706
Normal Pregnancy and Gestation 708
Myasthenia Gravis 592
Overview of Congenital Defects 711
Defects Caused by Environmental Factors 712
19 Disorders of the Nervous System 595 Genetic Disorders An Introduction 716
The Normal Nervous System 597
Single-Gene Defects Transmitted According to Mendel’s
Increased Intracranial Pressure 610 Rules 719
CNS Congenital and Perinatal Disorder 613 Single-Gene Defects Transmitted According to Non-­
CNS Trauma 613 Mendelian Rules 725
Cerebrovascular Disease 617 Disorders Influenced by Multiple Genes 726
CNS Infections 622 Disorders Caused by Large-Scale Chromosome
CNS Demyelinating Diseases 624 Abnormalities 726
CNS Metabolic Disorders 625 Genetic Diagnosis 729
CNS Degenerative Disorders 627 Perinatal and Neonatal Disease 731
CNS Neoplasms 630 Infections of Infants and Children 735
Diseases of Peripheral Nerves 632 Sudden Infant Death Syndrome (SIDS) 737
Tumors and Tumor-Like Conditions in Children 737
20 Disorders of the Senses 638
The Normal Eye and Orbit 641 23 Disorders of Daily Life 741
Disorders of Alignment and Movement 646 Injury from Physical Agents 743
Trauma 646 Toxic Exposures 748
Disorders of Refraction 647 Tobacco Use 752
Disorders of the Eyelid, Conjunctiva, Sclera, and Lacrimal Alcohol Use and Abuse 754
Apparatus 648 Illicit Drug Abuse 756
Disorders of the Cornea 649 Nutritional Disease 759
Disorders of the Lens 651
Disorders of the Uveal Tract 652 24 Aging, Stress, Exercise, and Pain 768
Disorders of the Vitreous Humor and Retina 653 Aging and the Decline of Body Functions 770
Disorders of the Optic Nerve 656 Stress 775
Ocular Neoplasms 659 Exercise 778
The Normal Ear 661 Pain 783
Disorders of the External Ear 663
Disorders of the Middle Ear 664 Index 788
PART

Mechanisms of Health and Disease 1


These chapters discuss basic disease processes and pathophysiology that can affect any tissue,
organ, or system of organs.

Chapter 1 Health and Disease • Leukocytosis, lymphocytosis, eosinophilia, and other


• Pathology, epidemiology, signs, symptoms, syndromes, and characteristics of infections by particular agents
other concepts of disease • Gonorrhea, Chlamydia, syphilis, hepatitis, and other
• The effects of genetics and environment transmitted infections
• The meaning of “normal” and “abnormal”; test sensitivity
Chapter 5 Neoplasia
and specificity; false-positive and false-negative tests; the
• Definitions of adenoma, sarcoma, carcinoma, lymphoma,
effect of prevalence on test interpretation
and other types of neoplasms
Chapter 2 Cellular Pathology: Injury, Inflammation, • DNA mutations, proto-oncogenes, tumor suppressor
and Repair genes, the importance of apoptosis
• Labile, stable, and permanent tissues; the role of stem cells • Premalignant states, malignant clones, growth fraction,
• Necrosis, apoptosis, and other cell changes in health and degrees of differentiation, tumor blood supply, invasion
diseaase and metastasis, immune surveillance
• Acute and chronic inflammation and the body’s response • The importance of clinical history; grading, staging, biopsy,
to injury cytology, cell markers, paraneoplastic syndromes, other
• Regeneration, scarring, and repair in the recovery from aspects of clinical behavior and assessment
injury • Surgery, radiation, chemotherapy, vaccination, and other
Chapter 3 Disorders of the Immune System immune treatments
• Epithelial barriers and other nonimmune protection; alien Chapter 6 Disorders of Fluid, Electrolyte and Acid–Base
antigens and the reactions of the immune system Balance, and Blood Flow
• Cells and organs of the lymphoid and immune systems • Hydrodynamic pressure, osmotic pressure, and the
• Anaphylaxis, delayed immunity, and other immune reactions movement of fluid and blood
• Allergy and autoimmune disease • Intracellulular and extracellular fluid, plasma and blood
• AIDS and other immunodeficiencies; avian tuberculosis and volume, other body fluid compartments
other opportunistic infections • Edema, acidosis, dehydration, electrolyte imbalances
Chapter 4 Infectious Disease • Hemostasis, hemorrhage, congestion, thrombosis
• Prions, viruses, bacteria, worms, ticks, and other varieties • Thromboembolism and infarction
of infectious agents • Hypovolemic, cardiac, and septic shock; collapse of blood
• Leukocytosis, fever, and other effects of infection circulation

1
CHAPTER

1 Health and Disease

Contents

Case Study “My daughter has a fever and an earache.” HOW IS DISEASE EXPRESSED?
The case of Anne M. Symptoms Are Subjective, and Signs Are Objective
Medical Tests Provide Data about Disease
WHAT IS DISEASE? A Syndrome Is a Collection of Symptoms, Signs, and Data
HOW DO SCIENTISTS STUDY DISEASE? HOW ARE MEDICAL TESTS INTERPRETED?
Pathology Is the Study of Disease in Individuals The Terms Normal and Abnormal Describe Observations
Epidemiology Is the Study of Disease in Populations and Measurements
Test Sensitivity and Specificity Are Key Considerations
WHAT CAUSES AND INFLUENCES DISEASE?
Tests Vary in Their Predictive Value
All Disease Is Due to Environmental Injuries and/or
Disease Prevalence Influences a Test’s Usefulness
Genetic Defects
Determinants of Health Can Indirectly Influence Case Study Revisited “My daughter has a fever and an
Disease earache.” The case of Anne M.

Chapter Objectives

After studying this chapter, you should be able to WHAT CAUSES AND INFLUENCES DISEASE?
complete the following tasks: 6. Discuss the roles of environmental factors, genetic
factors, and determinants of health in the disease
WHAT IS DISEASE? process.
1. Define disease, and compare and contrast acute and
HOW IS DISEASE EXPRESSED?
chronic disease.
7. Compare and contrast symptoms and signs.
2. Describe the relationship between structure and
8. List the types of tests that are used to study disease
function.
(consider anatomical and clinical pathology).
3. Discuss disease progression from latent period to
complications/sequelae. HOW ARE MEDICAL TESTS INTERPRETED?
9. Explain the meaning of the terms “mean,” “normal
HOW DO SCIENTISTS STUDY DISEASE? range,” and “standard deviation” as they relate
4. Compare and contrast the terms “etiology,” to medical tests and the concepts of normal and
“pathogenesis,” and “pathophysiology.” Also abnormal.
compare and contrast the terms “idiopathic,” 10. List the factors that influence the use of diagnostic
“iatrogenic,” and “nosocomial.” tests. How does disease prevalence and incidence af-
5. Define “epidemiology,” “incidence,” and fect a diagnostic test? How should these tests be ad-
“prevalence.” ministered (e.g., why administer a sensitive test first)?

2
Case Study
“My daughter has a fever and an earache.” The case of Anne M.

Chief Complaint: Fever and earache Clinical Course: The next evening Anne’s mother
returned her to the clinic. She said Anne was still
Clinical History: Anne M. was a 21-month-old girl feverish but seemed strangely sleepy and “jumpy”
sitting in her mother’s lap. Her mother told the nurse at the same time. The nurse practitioner called in a
practitioner that Anne had had a runny nose for sev- pediatrician for consultation who found that Anne’s
eral days but no fever. She became feverish, however, neck was now stiff and she cried when her head was
during the afternoon and had been crying and tug- moved. The anterior fontanel had become tense and
ging at her left ear. This is when her mother brought bulging. The pediatrician inserted a needle between
her to the emergency room. two lumbar vertebrae to collect a sample of spinal
fluid (a spinal tap). The cerebrospinal fluid was milky.
Physical Examination and Other Data: The nurse Lab microscopic examination revealed that it con-
practitioner found that Anne had a temperature of tained many white blood cells and a few rod-shaped
103°F and a perforated left eardrum with pus in the bacteria. A call to the laboratory revealed that the ear
external auditory canal. Crusted mucus was present in culture obtained the day before was growing a pure
and around the nostrils. The remainder of the exam growth of the bacterium Haemophilus influenzae. The
was unremarkable—there was no skin rash, the chest diagnosis became acute bacterial meningitis. Anne was
was clear, the neck was flexible and moving the head admitted to the hospital and placed on high doses of
produced no reaction from the child, and the anterior intravenous antibiotics. She made a prompt recovery.
cranial fontanel was flat and soft. The practitioner After you have read this chapter, you should be
made a diagnosis of acute rhinitis (a “cold”) and acute able to discuss this case in proper scientific terms
otitis media (a middle ear infection), swabbed the pus and explain why the physical examination did not
for culture by the lab, and wrote a prescription for an detect meningitis on the first visit, and why diagnosis
antibiotic. became certain the next day.

Be careful about reading health books. You may die of a misprint.


MARK TWAIN (SAMUEL LANGHORNE CLEMENS), 1835–1910, AMERICAN NOVELIST AND HUMORIST

In this chapter we are going to introduce you to disease, according to whether or not disease is actually present. So
how it develops from beginning to end, and how it affects what, precisely, is disease?
our anatomy and the functioning of our organs. But what Disease is really nothing more than healthy anatomy
exactly is disease? How does it differ from health? What’s (structure) and physiology (function) gone wrong. Put
more, how can you know if someone is unhealthy, and if another way, disease is a condition resulting from ana-
unhealthy, how can you discover the cause? Then, once tomical distortion or physiologic dysfunction. This defi-
you know the cause, what can you do to correct the situ- nition holds whether or not the distortion or dysfunction
ation safely; what can you say about the patient’s future? is perceptible. Sometimes disease will cause no obvious
dysfunction or “dis-ease,” especially in the early stages,
as we saw with the young man above. Moreover, some
What Is Disease? distortions occur at the molecular level and may not be
detectable even under a microscope. Another example is
A young man visits his physician for a routine checkup. high blood pressure, which is a famously silent killer.
He feels fine and has a completely normal physical Structure and function are inseparably locked together.
exam—despite the fact that an undetected malignant tu- For example, bacterial infection of the mitral heart valve
mor is growing in his lungs. Although this young man and may erode a hole (a structural abnormality) in the valve
his physician perceive him as healthy, he is certainly sick. (Fig. 1.1). With each ventricular contraction, the hole
That’s because the terms sickness and health refer to a state allows backflow of blood (a dysfunction) into the left
characterized, not according to how a person feels, but atrium. This inefficiency causes the heart to perform extra

3
4 Part 1 • Mechanisms of Health and Disease

Holes in mitral valve

Normal Thickened
thickness heart muscle
Figure 1.2 Initial functional disorder. High blood pressure is the initial
functional disorder. Pumping against abnormally high pressure puts ex-
cess strain on the left ventricle. The result is thickening of heart muscle—a
structural disorder.

example, have brain tissue that malfunctions in ways that


are largely invisible to science. This is also true of certain
other disorders. Fibromyalgia, for example, is a condition
characterized by muscle and periarticular pain, tenderness,
and stiffness that is not associated with any objective abnor-
Figure 1.1 Initial structural disorder. Holes eaten into the mitral mality on medical imaging, blood analyses, or other inves-
valve by bacteria are the initial structural defect. The result is regurgita- tigations. Irritable bowel syndrome is another condition that
tion (backflow) of blood into the atrium—a functional disorder. illustrates the point. Patients suffer from diarrhea or consti-
pation, abdominal pain, and bloating, but do not have any
work to move the required amount of blood. This extra of the objective abnormalities associated with disease; labs,
labor can lead to heart muscle exhaustion (heart failure), imaging studies, and physical findings are normal.
a functional disorder discussed in Chapter 9. Apart from these exceptions, diseases present them-
Likewise, a functional disorder may lead to structural selves by causing observable and measurable changes in
change. For example, high blood pressure is a functional the appearance (form) or performance (function) of cells,
disorder that puts excessive strain on heart muscle as tissues, and organs. Alterations of form (such as a mass
it struggles to eject blood against the elevated pressure in the neck) and function (such as difficulty breathing)
in the arterial tree. This stress causes the left ventricu- are assessed by collecting a medical history, performing
lar muscle to enlarge just like the skeletal muscles of a a physical examination, and gathering objective data by
weightlifter doing gym exercises. The abnormally en- laboratory tests, X-rays, and other means. We discuss this
larged heart muscle is a structural disorder that has arisen process later in this chapter, but you’ve already seen it
from a functional disorder (Fig. 1.2). reflected in the opening case study. Notice that the nurse
practitioner first took a clinical history, and then per-
formed a physical examination. She also sent a sample of
Case Notes tissue fluid to the laboratory for analysis.
All disease is either acute or chronic. Acute disease
1.1 Is the hole in Anne’s eardrum a functional arises rapidly, is accompanied by distinctive clinical mani-
or a structural disorder? festations, and lasts a short time. For example, the bacterial
infection in Anne’s middle ear, acute otitis media, begins
suddenly, is accompanied by characteristic ear pain and
Occasionally, medical science is unable to demonstrate fever, and lasts a few days. Chronic disease usually begins
a distortion or dysfunction responsible for a particular slowly, with manifestations that are difficult to interpret. It
disease. When this occurs, it does not necessarily reflect persists for a long time, and generally cannot be prevented
the actual state of things in the body, but rather the lim- by vaccines or cured by medication. For example, the onset
its of our technology. Patients with mental disorders, for of wear and tear arthritis (called osteoarthritis) begins with
Chapter 1 • Health and Disease 5

vague stiffness or aches in certain joints, progresses slowly,


cannot be cured (but can be treated), and lasts a lifetime. 1.3 What is the scientific name for a period of
The beginning of a disease is its onset, which may be vague, early manifestations that herald the
facilitated by certain predisposing factors. These factors coming of more pronounced disease?
can be genetic or environmental. For example, heart disease 1.4 What is the name for a short period of
may be promoted by certain genes inherited from an ances-
increased intensity of disease?
tor or by exposure to environmental toxins such as those
in tobacco smoke. As discussed earlier, disease may be pres-
ent but cause no apparent problems. This subclinical state
may also be called the latent period. In infectious diseases, How Do Scientists Study Disease?
it is called the incubation period to reflect the fact that,
although the person feels well, the infecting microorgan-
Two branches of medicine study disease as it occurs in
ism is rapidly reproducing within the body. Some disease,
individuals and in populations. Study of individuals elu-
especially infectious disease, begins with a period of minor,
cidates functional and anatomic detail. Study of popu-
nonspecific aches, dizziness, or other indications called the
lations elucidates broad ethnic and geographic trends,
prodromal period that heralds the coming of more intense,
modes of transmission, the influences of habits such as
specific indications of disease. For example, viral hepatitis
smoking, and the effects of age and sex, little of which
often presents with loss of appetite, malaise, and mild fever
can be gained by the study of individuals.
that may persist for several days or longer before jaundice
and other findings reveal the true nature of the problem. Pathology Is the Study of Disease
After revealing itself, the condition may resolve with or
in Individuals
without treatment. Alternatively, the condition may linger
as chronic disease, which may wax and wane. When the Pathology is the scientific study of changes in bodily
disease is quiet, it is in remission; when it reappears, it structure and function that occur as a result of disease.
is a recurrence. For example, after apparently successful The term is derived from the Greek pathos meaning suf-
treatment, breast cancer may disappear clinically only to fering, and logos indicating word or reason. The discipline
reappear years, or decades, after initial treatment. A period of pathology has four main goals:
of increased intensity of disease is an exacerbation. After • To describe the lesion (the anatomic abnormality pro-
the main illness has subsided, the patient enters a period duced by the disease)
of recovery during which health improves. • To discover the etiology (cause) of the disease
Sometimes a disease may quickly give rise to adverse • To understand the pathogenesis (natural history and
consequences, which are called complications. For ex- development) of the disease process
ample, severe skin burns are often complicated by bacte- • To explain the pathophysiology (the manner in which
rial infections. In like manner, a disease may be associated
the incorrect function is expressed)
with adverse outcomes at a later time, which are called
sequelae. For example, repeated head trauma may lead to If the etiology is unknown, the disease is said to be
dementia later in life. idiopathic (from Greek idio meaning personal, thus of a
personal, not commonly known, cause). For example, if
a patient has a failing heart because of weak heart muscle
and the cause of the weakness cannot be identified, the
Case Notes patient can be said to have idiopathic cardiomyopathy. In
1.2 Is Anne’s meningitis an exacerbation or a contrast, if the disease is a byproduct of medical diagnosis
complication of otitis media? or treatment, it is said to be iatrogenic (from Greek iatros
meaning physician). For example, if a patient develops
a bladder infection after a catheter is inserted into the
urinary bladder, the patient can be said to have iatrogenic
cystitis. Finally, if a disease—especially an infection—
originates while a patient is hospitalized, it is described as
Pop Quiz nosocomial, from Greek words meaning “to take care of
1.1 True or false? A functional disorder disease.” For example, a form of pneumonia caused by the
can lead to a structural change. Staphylococcus bacterium is commonly hospital acquired.
As an example of how these concepts fit together, con-
1.2 True or false? Acute disease typically begins sider an ordinary sunburn. The lesion is red, swollen, hot,
with manifestations that are difficult to painful skin. The etiology is excessive exposure to sun-
interpret. light. The pathogenesis is absorption of high-energy ultra-
violet (UV) rays, which injure skin. The pathophysiology
6 Part 1 • Mechanisms of Health and Disease

is characterized by skin pain, swelling, redness, and


warmth due to blood vessel dilation and increased blood 1.7 True or false? The incidence of a disease
flow, all of which are part of the reaction to the injury. is the number of new cases of a particular
disease that appear in a year.

Case Notes
1.3 Is Anne’s disease idiopathic? What Causes and Influences
Disease?

Epidemiology Is the Study of Disease When considering the origin of disease, it’s important to
in Populations distinguish between two types of factors: those that are ca-
pable of directly causing disease, and those that indirectly
Epidemiology is a discipline of medicine that studies
influence the initiation and progression of disease.
the broad behavior of disease in large populations. One
goal of epidemiology is to determine the incidence of a All Disease Is Due to Environmental
disease, which is the number of new cases of a particular
Injuries and/or Genetic Defects
disease that appear in a year, as well as the prevalence,
which is the number of people with a certain disease at The causes of disease can be conceived of as a continuum.
a given moment. For example, in 2009 the incidence of At one end of the continuum are diseases caused solely
new prostate cancers in American men was approximately by environmental injury. At the opposite end are those
192,000 cases. The prevalence of prostate cancer in Ameri- caused solely by our genetic makeup. In the middle are
can men was about 1.5%; that is, somewhat more than 1 the majority of diseases, those resulting from some com-
in every 100 men had a history of prostate cancer. The bination of the two.
morbidity rate is the number of people with an illness
or complication of an illness and can be stated as either Environmental Injuries
incidence or prevalence. The mortality rate is the number The term injuries typically brings to mind physical trauma
of people dying from a particular disease in a particular (burns, broken bones, etc.). But toxic molecules––from
period of time. chemical poisonings to molecules released by infectious
Epidemiological methods are also used to identify organisms––also commonly cause injuries. Cancer is also
factors that may increase an individual’s likelihood of due to molecular injury: all cancers originate from dam-
developing a specific disease. These factors are known as aged DNA. Such injurious forces are environmental fac-
risk factors. For example, careful population studies have tors; that is, they arise from the world in which we live.
revealed that cigarette smoking is a risk factor for heart As we discuss below, our genetic makeup, age, gender,
disease, and use of oral contraceptives is a risk factor for nutrition, and other factors can play a role in how we
cervical cancer after five years of use. In contrast, protec- respond to injurious forces.
tive factors are those that decrease risk. Engaging in regu-
lar physical activity is a protective factor in heart disease.
In addition, an epidemiologist may study a group of pa- Genetic Defects
tients with a particular disease to determine what happens A genetic defect can be the sole cause of disease, such as
to them over time. This type of study helps epidemiolo- cystic fibrosis, hemophilia, or sickle cell anemia. One of
gists establish a prognosis—the probability of recovery, the most common genetic disorders is red–green color-
death, or another outcome, for a disease. Probabilities are blindness, which affects fully 7% of all males and is due
statistical likelihoods, and are often expressed as percent- to a defect in a clearly identified single gene. But most
ages. For example, the prognosis for many cancers is the single gene (monogenic) diseases are rare. Diseases
percentage of patients expected to survive for a period of caused by the interaction of multiple genes (polygenic)
five, ten, or twenty years. are much more common, much less visible, and much
more difficult to study. Not only certain diseases, but
most human characteristics (traits) are polygenic. Hair
and eye color, height, weight, intelligence, and facial
Pop Quiz features are examples. Important though they are, it is
1.5 What is the scientific name for the very difficult to identify the individual genes that make
cause of a disease? up the combination influencing, for example, intel-
ligence. We don’t know if it is closer to 100 or 1,000
1.6 What is the scientific name for a structural genes, much less which genes are responsible and what
abnormality of disease? role each plays. (See The Clinical Side, “The New Age of
Personal Genomics.”)
Chapter 1 • Health and Disease 7

Recall Anne, from our case study, whose mother sought


The Clinical Side care for Anne’s fever and ear pain. How do you think
Anne’s disease process might have been affected if her
THE NEW AGE OF PERSONAL GENOMICS mother did not have access to the clinic—if, for example,
she were a single mother working a minimum-wage job
In recent years, genetic technology companies have with no health insurance, and had waited a day or two to
begun offering consumers an analysis of their personal see if Anne’s condition might resolve on its own, without
genome that can identify genes that may predispose the expense of medical care?
them to certain diseases. Even though anyone can Access to quality healthcare is just one of many factors
purchase these genetic screenings, doing so may or may known to influence the development and progression of
not be a good idea. In some ways it’s like playing with disease. Called determinants of health, these include per-
dynamite. There is a lesson to be learned among families sonal, social, economic, and environmental factors that
with Huntington chorea, an invariably fatal monogenic may not directly cause disease, but certainly play a role
disease that begins to manifest in early to middle adult- in its behavior. For instance, having social support—such
hood. Statistically speaking, the children of affected as a loving family member who encourages you to see
patients have a 50% chance of inheriting the disorder. the doctor about that nagging cough—is a protective
Although these children could learn their fate from ge- factor against disease, whereas living in a high-crime
netic analysis, many prefer not to know. For them there area—with limited options for safely engaging in walk-
is wisdom in the saying, “Ignorance is bliss.” ing, jogging, biking, and other types of outdoor physical
Consumers who do opt for genetic screening should activity—may promote obesity.
have a thorough understanding of the benefits and draw-
backs. It is advisable to speak with a doctor or genetic
counselor before purchase of such tests and especially af- Pop Quiz
ter results become available. Consumers should also have
realistic expectations: studies show that most people who
1.8 True or false? The majority of
purchase personal gene tests do so with the expectation disease is monogenic.
that they will change their evil ways if they are found to 1.9 True or false? A patient’s ability to read and
have a tendency to develop a certain disease. But the comprehend prescription-drug information
same studies show they don’t change. is a determinant of health.

Multifactorial Diseases How Is Disease Expressed?


Although genes are solely responsible for a few diseases
and the environment accounts solely for many others, The nature of a disease is expressed by its symptoms, which
on the whole, both genetics and environment play a role are subjective and described by the patient during the med-
in most. That is, the majority of disease is multifactorial. ical history, and its signs, which are objective and are re-
For example, some cancers develop because of inherited vealed during the physical, lab, X-ray, or other examination.
genetic mutations that predispose the patient to develop The assembled facts then suggest the diagnosis, which is a
cancer, provided that environmental factors—like expo- name for the cause of the patient’s problem. In considering
sure to cigarette smoke—injure the patient’s DNA. The the diagnostic process, it’s important to keep in mind that
fact that most disease is multifactorial means that most although symptoms, signs, and test results may suggest a
disease is not completely preventable in any individual. diagnosis, they may or may not suggest the correct diagno-
Again, cigarette smoking and lung cancer provide an ex- sis. It is helpful to think of such data as a roadmap, and the
cellent illustration of this tricky concept. Although 85% disease as the actual road. The roadmap may be incorrect,
of all lung cancer deaths occur in smokers, 15% occur in and the road may differ from what the map suggests.
lifelong nonsmokers, some of whom have had no signifi-
cant exposure to secondhand smoke. So although elimi- Symptoms Are Subjective, and Signs
nating all smoking would dramatically reduce lung cancer Are Objective
mortality within a population, avoiding smoking is not Symptoms are complaints reported by the patient or by
guaranteed to prevent lung cancer in any one individual. someone else on behalf of the patient. They therefore
reflect a subjective experience of the disease. One of the
Determinants of Health Can Indirectly most commonly reported symptoms is, of course, pain.
Influence Disease Typically, the examiner asks the patient to describe the
In addition to direct environmental and genetic causes, onset, duration, quality, and intensity of the pain, as well as
literally hundreds of factors in an individual’s life can in- what seems to exacerbate it and relieve it. Other common
directly influence the initiation and progression of disease. symptoms include fatigue, nausea, sensory impairment,
8 Part 1 • Mechanisms of Health and Disease

and bowel dysfunction. All symptoms become part of the


medical history. medical professional for examination and diagnosis.
Signs are objective data: observations by an examiner Typically the specimen is placed in a fixative solution
(e.g., registered nurse, nurse practitioner, physician assis- (usually formaldehyde) to prevent degeneration or bacte-
tant, or physician), lab data, imaging studies, electrocar- rial growth and to ready the specimen for further study.
diogram, and so on. For example, diarrhea reported by the Examination occurs in two stages: gross and micro-
patient is a symptom, but diarrhea observed by the examiner scopic. The gross examination is study of the specimen
is a sign. Similarly, hearing loss reported by the patient is a with the unaided eye and includes the weight, size,
symptom, but hearing loss demonstrated upon examination shape, texture, color, and other features. The gross exam
is a sign. Many signs can be detected only by the examiner. is followed by a microscopic study of all or carefully se-
For instance, auscultation with a stethoscope may reveal lected small pieces of the specimen.
heart, lung, or bowel sounds not detectible by the patient. In microscopic study of a biopsy, light shines upward
Notice that both symptoms and signs are detectible from below the specimen and through it to the micros-
manifestations of disease. For example, a person may have copist’s eye. Microscopic study, therefore, requires slices
a liver tumor that produces no symptoms and is too small of tissue thin enough to be transparent, usually less than
to be palpated or seen. As noted earlier, in such cases the one cell thick. But, just as a glass of water from the deep
disease is said to be latent (or subclinical). blue sea is almost colorless, in such thin slices there is not
enough natural color present to make cells clearly visible.
To solve this problem, dyes (stains) are added to color
Case Notes the cells. The finished result is somewhat like looking at
a flag with the sun shining through from the backside.
1.4 Name some symptoms and signs present on
Consider a specimen from a breast biopsy. The surgeon
Anne’s second visit. puts the raw lump of tissue in formaldehyde to preserve
it and kill any bacteria that might cause decay during lab
processing. A small sample is selected by the pathologist
Medical Tests Provide Data about Disease for further processing and is placed in a series of chemicals
Assessment of body tissues, fluids, and other components to soak out the fat and water, both of which render tissue
is a third way in which disease may make itself known. fuzzy and blurry under the microscope. Next, the piece is
These studies are either anatomic or clinical. immersed in hot paraffin wax, which soaks into the speci-
men to take the place of the missing fat and water. The
Anatomic Pathology paraffinized piece is chilled and becomes hard enough for
Anatomic pathology is the study of structural changes very thin slicing by a highly precise instrument. A slice is
caused by disease. Assessment of tissue specimens by the laid flat on a slide and dipped in a series of chemicals to
unaided eye is gross examination; assessment of magni- remove the paraffin, leaving behind on the slide surface
fied images of small structures is microscopic examina- an exceedingly thin layer of waterless, fat-free tissue; all
tion. The most basic and extensive gross examination is an that remains is protein, carbohydrate, and minerals. This
autopsy, an after-death (postmortem) dissection of a body is then dipped in a series of dyes that stain cell nuclei
to determine the cause of death and other facts about the blue and cytoplasm red. Collagen, calcium, and other
condition of the patient at the time of death. On a smaller interstitial materials stain red or blue or a mixture of the
scale, a biopsy is examination of living tissue, usually via two colors depending on individual characteristics. Places
microscope. For example, the study of tissues and cells in where fat and water used to be are empty and colorless.
a breast biopsy or a Pap smear is an anatomic pathology Pathologists, or other specialists with microscopic ex-
procedure. Refer to The Clinical Side, “What Happens to pertise, study the tissue searching for patterns of disease—
a Biopsy Specimen,” to see how tissue specimens are pre- inflammation, degeneration, peculiar-looking cells, and so on.
pared for study. In addition to ordinary microscopic study, special
techniques can highlight certain cell characteristics and
make them microscopically visible. An example is detec-
tion of estrogen-receptor molecules in breast cancer
The Clinical Side
cells. The presence or absence of estrogen receptors is
important in crafting the best therapy for breast can-
WHAT HAPPENS TO A BIOPSY SPECIMEN?
cer. The technique (called “immunohistochemistry”)
The word biopsy derives from Greek bios = life + opsis = requires treating a thin slice of raw tumor tissue with
sight. It is the obtaining of a piece of living tissue to antibodies and chemicals, the combination of which
discover the presence, cause, or extent of disease. The causes a colored precipitate to accumulate in breast
biopsy specimen is submitted to a pathologist or other cancer cells if estrogen receptors are present in them.
Chapter 1 • Health and Disease 9

Clinical Pathology The Terms Normal and Abnormal Describe


Clinical pathology is the study of the functional aspects of Observations and Measurements
disease by laboratory study of tissue, blood, urine, or other In everyday conversation, we may refer to cancer or de-
body fluids. Examples include blood glucose measurement mentia as “abnormal,” but clinicians do not use the terms
to diagnose diabetes, or a culture of urine to detect bacterial normal and abnormal to describe health and disease. Rather,
infection. Clinical pathology extends from the lab to the bed- they use them to characterize observations and measure-
side, too. A pathologist is practicing clinical pathology when ments. That’s because medical test results vary greatly
he or she supervises the performance of a laboratory test, among healthy people, just as do height, weight, and other
such as a blood aldosterone assay, and consults with another physical features. For example, a shoe size 15 EEE might be
physician about the results. normal for a 6′10′, 350-lb man, but for a 5′2′, 105-lb woman
it would be abnormal in the extreme. Neveretheless, even in
A Syndrome Is a Collection of Symptoms, a small woman, such a foot, though of a very abnormal size,
Signs, and Data would not necessarily indicate disease—it may function
A distinctive collection of symptoms, signs, and data normally and be perfectly healthy. In the same way, healthy
(anatomic or clinical) is a syndrome. For example, ac- people may have unusually low, high, or otherwise abnor-
quired immunodeficiency syndrome (AIDS) commonly mal test results even though they do not have a disease—the
includes profound fatigue (a symptom), and abnormally abnormal results merely reflect variation among individuals.
low counts of a particular type of T cell (a sign). These variations of normal require that clinicians use
Sometimes, a particular syndrome may be caused by an established definition of normal. For these purposes,
any of several different diseases. For example, Cushing normal means the usual result in healthy people. Like-
syndrome (Chapter14) is a collection of symptoms, signs, wise, abnormal means not the usual result in healthy
and data attributable to chronic adrenocortical hormone people. It is true that most sick patients have abnormal
(steroid hormone) excess. It is characterized by truncal test results, and most healthy patients have normal results.
obesity, a moon face, excess facial hair, easy bruising, skin Nevertheless, sometimes sick patients have normal test
striae (stretch marks), brittle bones, high blood glucose, results and sometimes healthy patients have abnormal test
and high blood cortisol, among many other features. results; thus, applying the terms normal and abnormal only
Cushing syndrome is often due to medical treatment, but to observations and measurements is essential. Figure 1.3
it can be due to adrenal, pituitary, or other disease. depicts these concepts.

Qualitative versus Quantitative Tests


Remember This! A syndrome is a distinctive
Qualitative tests are used to describe qualities, most com-
collection of symptoms, signs, and test data.
monly the presence, absence, or characteristics of a com-
ponent, such as the shape of the heart as seen in a chest
X-ray. For qualitative tests, the result is either normal
Pop Quiz (the expected result in healthy people) or abnormal (not
the expected result in healthy people). For example, if a
1.10 Is an abnormality observed by a
patient is suspected of having intestinal bleeding, stool
nurse a sign or a symptom?
can be tested for the presence or absence of blood. Either
1.11 True or false? A biopsy is an examination of blood is present (abnormal) or it is not present (normal)
a tissue specimen to determine the cause of and decisions can be made accordingly.
death. More commonly, a determination of normal or ab-
1.12 What is the name for a distinctive collection normal must be made for the results of quantitative tests,
which measure quantities (amounts or numbers) of a
of symptoms, signs, and data?
component. For example, a quantitative test of kidney
function might measure the amount of nitrogen in a pa-
tient’s blood. This numerical data must then be compared
to a standard. That is, for quantitative tests, a normal
How Are Medical Tests Interpreted? range must be established. Recall from above that normal
is defined as the usual result in healthy people. But how
We’ve said that one way disease expresses itself is by caus- do clinicians know what is usual?
ing alterations in body tissues and chemicals that can be
detected by medical tests. But when test results are in, Establishing Normal for Quantitative Tests
how do clinicians interpret them in relation to disease? To To establish normal for any particular quantity, epidemi-
answer that question, we first need to understand how to ologists perform statistical analysis of many results in a
distinguish between normal and abnormal. large number of presumably healthy people. These results
10 Part 1 • Mechanisms of Health and Disease

Sick with Sick with


normal tests abnormal tests

Healthy with Healthy with


normal tests abnormal tests
A B C

Healthy Normal
test
Sick Abnormal
test
Figure 1.3 Healthy or sick, normal or abnormal, and how they combine. A. All patients are either healthy or sick. B. All measurements (tests)
are either normal or abnormal. C. Some healthy patients have abnormal test results, and some sick patients have normal test results.

are averaged to determine the mean (average). Statistical (average) and standard deviations are calculated for the
formulas are also applied to the data to determine the group. If the average glucose in our group is 90 mg/dL, and
standard deviation, a measure of the degree of natural one standard deviation (SD) is 10 mg/dL, then the normal
variability of results; that is, the degree of variation from range for fasting blood glucose levels would be from 90
one normal person to another. When test results cluster minus 20 to 90 plus 20, or 70 to 110 mg/dL, as shown in
tightly around the mean, the standard deviation is small. Figure 1.4.
The test results for blood calcium levels, for example,
have a small standard deviation because the body tightly Positive versus Negative Results
controls blood calcium, and levels vary little from one When referring to tests for a particular disease, results
person to another. On the other hand, when test results are often referred to as positive if abnormal and negative if
are widely scattered above and below the mean, as they are normal. The presumption is that positive suggests disease
with blood glucose levels, the standard deviation is large. may be present, while negative suggests it is not. Presum-
To accommodate the natural variability of test results, ing we know by other methods whether the patient is sick
epidemiologists use the mean and standard deviation or well, test results for a particular disease are referred to
to establish a normal range. By widespread agreement, as true positive if the test is positive and the patient actu-
the lower limit of the normal range is always set at two ally has the disease. Conversely, the test is referred to as
standard deviations below the mean, and the upper false positive if the test is positive but the patient does
limit is set at two standard deviations above the mean. not have the disease. That is to say, a true positive test
A graphic display of a hypothetical normal range study
for blood glucose is shown in Figure 1.4. When normal
is defined this way, the lowest 2.5% and highest 2.5% of
results in presumably healthy persons are so far from the Mean
particular blood glucose level
Number of people with a

average that they are considered abnormal even though


by definition the patient is healthy. Thus, by definition, One standard
deviation
5% of presumably healthy people will have an abnormal
test result.
Abnormally Abnormally
Remember This! Healthy is not the same as low high
normal; sick is not the same as abnormal. Normal = mean ± 2
standard deviations
As an example, let’s presume we want to establish a
normal range for blood glucose. We therefore ask 100 pre-
sumably healthy young adults to volunteer to have a blood 60 70 80 90 100 110 120
glucose test. Those with signs or symptoms that suggest Blood glucose levels (mg/dL) in presumably healthy people
diabetes or those with a family history of diabetes are
Figure 1.4 The normal distribution curve. Among healthy people
rejected. Those who are accepted are instructed not to eat
who do not have diabetes, the greatest numbers of blood glucose
or drink anything for four hours before the test. A blood levels are near the mean (90 mg/dL). A few people will have a blood
glucose test is performed on each person, and the mean glucose level below 70 mg/dL or greater than 110 mg/dL.
Chapter 1 • Health and Disease 11

Table 1.1 Test Results: True and False Positive; True and False Negative
Normal Test Abnormal Test

HEALTHY Healthy patient with normal test result: Healthy patient with abnormal test result:
True negative False positive
Example: People without Normal fasting blood glucose level: High fasting blood glucose level: Perhaps
diabetes Diagnosis—no diabetes patient not really fasting
SICK Sick patient with normal test result: Sick patient with abnormal test result:
False negative True positive
Example: People with untreated Normal fasting blood glucose level: High fasting blood glucose level:
diabetes Perhaps lab error Diagnosis—diabetes

correctly indicates that disease is present, whereas a false


positive test incorrectly suggests disease is present when,
in fact, it is not. Likewise, negative results are referred to Case Notes
as true negative or false negative, depending on whether 1.6 Presuming Anne had mild meningitis on the
the test result correctly or incorrectly indicates that disease first visit, as a test for meningitis did the neck
is absent. These combinations are depicted in grid form in manipulation test lack sensitivity or specificity?
Table 1.1.

Case Notes There is a trade-off between sensitivity and specificity.


Highly sensitive tests are likely to be positive in patients
1.5 Presuming that Anne’s meningitis was with the condition or disease (truly positive), but they
present but not severe on the first visit, was also have a tendency to be positive (falsely positive) in
Anne’s soft, flat fontanel a true positive, false some healthy people, too. That is to say, if you screen for
positive, true negative, or false negative test for a certain condition using a highly sensitive test, the group
meningitis? with positive results will include most of the patients with
the condition (you won’t miss many), but mixed in will be
a fairly large number of healthy patients who do not have
The Extent of Abnormality the condition (their tests are falsely positive). Although
this is less than ideal, the flip side is that you can be confi-
If a test is abnormal, the degree of abnormality is dent that those who had negative results are healthy (truly
important—markedly abnormal results are more signifi- negative). That is to say, a negative result using a highly
cant than are mildly abnormal ones. Disease is a contin- sensitive test is a very reliable indicator that the condition
uum from mildly ailing to desperately ill, and test results for which you are testing is not present. In the group with
vary accordingly. The greater the degree of abnormality, positive tests, you can sort out the false positives from the
the more likely it is that the result means disease is present true positives by doing additional tests.
(the test is truly positive). For example, if the upper limit The opposite is true for highly specific tests—the test
of normal blood glucose levels is 110 mg/dL, a patient with is likely to be negative in healthy patients who, of course,
a fasting blood glucose level of 190 mg dL is much more do not have the condition for which you are testing. The
likely to have diabetes than is a patient with a fasting blood test may be negative, however, in some patients with the
glucose level of 120 mg/dL. condition (their test is falsely negative). It follows that if
you screen a group of patients using a highly specific test,
Test Sensitivity and Specificity you can be confident that those with positive tests have
Are Key Considerations the condition (their test is likely to be a true positive, not
In addition to interpreting the values of test results as nor- a false positive). Nevertheless, the group with negative
mal and abnormal, clinicians must be able to appreciate a results will include some patients with disease, whom you
test’s sensitivity and specificity. The ability of a test to be can identify by further testing later.
positive in the presence of disease is test sensitivity. For Again, as a rule, highly sensitive tests are not very spe-
example, a test is 99% sensitive if it is positive in 99 of cific, and highly specific tests are not very sensitive. By
100 patients known to have the disease. Similarly, speci- way of example, consider home burglar alarms as a test
ficity is the ability of a test to be negative in the absence of for burglars. Alarms are very sensitive but not very spe-
the disease. A test is said to be 99% specific if it is negative cific—so although they do not miss many burglars, there
in 99 of 100 persons known not to have the disease. are lots of false alarms. That is to say, burglar alarms have
12 Part 1 • Mechanisms of Health and Disease

many false positives but few false negatives. By contrast, presumably healthy women; it is, however, very useful to
having a personal observer at home is much more specific distinguish the true-positive smears (women with cancer)
but it is less sensitive. Rarely would an observer in the from the false-positive smears (women without cancer).
house falsely accuse someone of being a burglar unless
they were unknown or unwelcome, but if the observer is Remember This! Test first with highly sensitive
out working in the back garden, then a burglar might not tests; retest positives with highly specific tests.
be detected.
Given that both sensitive and specific tests have draw-
Tests Vary in Their Predictive Value
backs, which type of test does a clinician choose, and
why? In the diagnostic process, the most effective strategy The purpose of testing is to determine who has disease and
is this: first use a very sensitive test, and then follow up on who does not. The best test has high predictive value; that
patients who test positive by administering a very specific is, it accurately predicts who has and who does not have
test. This is precisely the strategy used in many types of disease. Highly sensitive tests tend to have a lot of false
cancer screening. For example, sexually active women are positives, but very few false negatives. Therefore, a nega-
routinely screened for cervical cancer using a Pap smear, tive result in a highly sensitive test has high predictive value.
which is a highly sensitive test; that is, it misses very few Highly specific tests tend to have a lot of false negatives, but
cases of cervical cancer (Fig. 1.5). A Pap smear is inex- few false positives. Therefore, a positive result in a highly spe-
pensive, painless, and minimally invasive. The clinician cific test has high predictive value. Another way to say this is
collects a sample of cervical cells in a matter of seconds that if a test has many true positives and few false positives,
during a woman’s routine pelvic exam. Nevertheless, the predictive value of a positive test is high. Likewise, if a
because it is highly sensitive, Pap smear screening will test has a great number of true negatives and few false nega-
result in false positives. So a diagnosis of—and treatment tives, the predictive value of a negative test is high.
for—cervical cancer is not yet warranted for those women For example, cardiac troponin I, a heart muscle protein
who test positive. Instead, these women undergo a sec- that increases in blood as a result of a heart attack, nor-
ond, highly specific test, a tissue biopsy. This second test mally circulates in blood in small amounts. Therefore, in a
is more invasive, more painful, more time consuming, and patient with chest pain and possible heart attack, increased
more expensive, so it is not practical as a screening test for cardiac troponin I is considered a positive test for cardiac

Highly sensitive test Highly specific test

Population False positive Diagnosis:


Cancer
Positive
test Biopsy
Diagnosis:
Pap No cancer
smear

Biopsy
Negative Diagnosis:
test Cancer
Resmear
in one
year

= Cervical cancer

= No cancer
False negative

Figure 1.5 Test sensitivity and specificity in the search for cancer of the cervix. To detect cancer of the cervix, first use a highly sensitive test,
the Pap smear, which is not likely to miss many cancers. Those who test positive by Pap smear are further investigated by cervical biopsy, a more
specific test. Those who tested negative by Pap smear are retested by Pap smear the next year, which will likely identify false negatives missed on
the first smear.
Chapter 1 • Health and Disease 13

muscle damage and a reliable sign of a heart attack. Normal because among such patients there are many having a
levels of cardiac troponin I suggest no cardiac muscle dam- heart attack. Therefore, in an emergency room population,
age has occurred and the cause of the pain must be found a positive result is much more likely to be truly positive.
elsewhere. Diagnostic use of cardiac troponin I as a tool to In medical diagnostic terms, a positive test is more likely
predict the presence or absence of heart muscle damage to be truly positive (to have a high predictive value; to be a
has shown that most patients with abnormally high cardiac correct indication of disease) if there are a lot of people in
troponin I have heart muscle damage. Conversely, the great the tested population who have the disease; that is, if the
majority of patients with normal cardiac troponin I do not prevalence of disease is high in the tested population.
have heart muscle damage. Thus, the predictive value of
cardiac troponin I as an indicator of the presence or ab-
sence of heart muscle damage is high for both positive and
negative tests, making cardiac troponin I a very widely used Case Notes
diagnostic test when heart muscle damage is suspected. 1.7 Is the prevalence of meningitis likely to be
As discussed above, the degree of test abnormality is low or high in a group of infants with a tense,
important—the greater the abnormality, the more likely is bulging fontanel?
it that the result correctly suggests that disease is present.
This means that a patient with very high cardiac troponin
I is much more likely to have heart muscle damage (and
more extensive damage) than is a patient with mildly el-
evated cardiac troponin I. Pop Quiz
Disease Prevalence Influences a Test’s 1.13 True or false? All sick patients will
Usefulness have at least one abnormal test.

How well a test performs (whether it has high or low 1.14 What percentage of the test results in
predictive value) depends to a surprising degree on how healthy people fall within the mean plus
many cases exist (the prevalence) in the group being and minus two standard deviations?
tested. For example, consider the cardiac troponin I test 1.15 The ability of a test to be positive in the
just mentioned. The number of people having an acute presence of disease is __________.
heart attack is near zero among asymptomatic persons
entering a shopping mall. Any positive test in such a 1.16 True or false? The best testing strategy is to
group is very likely a false positive. On the other hand, start with highly sensitive tests and follow
the same test will be much more useful if performed in pa- with highly specific ones.
tients who present with chest pain to an emergency room

Case Study Revisited


“My daughter has a fever and an earache.” The case of Anne M.

Reviewing this case gives us an opportunity to review can be a complication of ear infection, the nurse also
many of the terms and concepts covered in this checked the flexibility of Anne’s neck and the softness
chapter. of her anterior fontanel. Both were normal; that
Anne’s primary symptom on her first visit was is, they were negative tests. After examination the
pain, which she “reported” (through her mother) by nurse practitioner concluded that the diagnosis was
crying and tugging on her ear. The nurse practitioner acute bacterial infection of the left middle ear (otitis
examined Anne and found the following signs, media) with perforation of the tympanic membrane.
each of which can be thought of as a positive test: The initial etiology was acute viral upper respiratory
elevated temperature, runny nose and crusted infection. The pathogenesis was swelling and mucus
nostrils, perforated left eardrum, and pus in the obstruction of the eustachian tube (abnormal form),
external auditory canal. Knowing that meningitis which caused accumulation of fluid in the left middle

(continued)
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DANCE ON STILTS AT THE GIRLS’ UNYAGO, NIUCHI

Newala, too, suffers from the distance of its water-supply—at least


the Newala of to-day does; there was once another Newala in a lovely
valley at the foot of the plateau. I visited it and found scarcely a trace
of houses, only a Christian cemetery, with the graves of several
missionaries and their converts, remaining as a monument of its
former glories. But the surroundings are wonderfully beautiful. A
thick grove of splendid mango-trees closes in the weather-worn
crosses and headstones; behind them, combining the useful and the
agreeable, is a whole plantation of lemon-trees covered with ripe
fruit; not the small African kind, but a much larger and also juicier
imported variety, which drops into the hands of the passing traveller,
without calling for any exertion on his part. Old Newala is now under
the jurisdiction of the native pastor, Daudi, at Chingulungulu, who,
as I am on very friendly terms with him, allows me, as a matter of
course, the use of this lemon-grove during my stay at Newala.
FEET MUTILATED BY THE RAVAGES OF THE “JIGGER”
(Sarcopsylla penetrans)

The water-supply of New Newala is in the bottom of the valley,


some 1,600 feet lower down. The way is not only long and fatiguing,
but the water, when we get it, is thoroughly bad. We are suffering not
only from this, but from the fact that the arrangements at Newala are
nothing short of luxurious. We have a separate kitchen—a hut built
against the boma palisade on the right of the baraza, the interior of
which is not visible from our usual position. Our two cooks were not
long in finding this out, and they consequently do—or rather neglect
to do—what they please. In any case they do not seem to be very
particular about the boiling of our drinking-water—at least I can
attribute to no other cause certain attacks of a dysenteric nature,
from which both Knudsen and I have suffered for some time. If a
man like Omari has to be left unwatched for a moment, he is capable
of anything. Besides this complaint, we are inconvenienced by the
state of our nails, which have become as hard as glass, and crack on
the slightest provocation, and I have the additional infliction of
pimples all over me. As if all this were not enough, we have also, for
the last week been waging war against the jigger, who has found his
Eldorado in the hot sand of the Makonde plateau. Our men are seen
all day long—whenever their chronic colds and the dysentery likewise
raging among them permit—occupied in removing this scourge of
Africa from their feet and trying to prevent the disastrous
consequences of its presence. It is quite common to see natives of
this place with one or two toes missing; many have lost all their toes,
or even the whole front part of the foot, so that a well-formed leg
ends in a shapeless stump. These ravages are caused by the female of
Sarcopsylla penetrans, which bores its way under the skin and there
develops an egg-sac the size of a pea. In all books on the subject, it is
stated that one’s attention is called to the presence of this parasite by
an intolerable itching. This agrees very well with my experience, so
far as the softer parts of the sole, the spaces between and under the
toes, and the side of the foot are concerned, but if the creature
penetrates through the harder parts of the heel or ball of the foot, it
may escape even the most careful search till it has reached maturity.
Then there is no time to be lost, if the horrible ulceration, of which
we see cases by the dozen every day, is to be prevented. It is much
easier, by the way, to discover the insect on the white skin of a
European than on that of a native, on which the dark speck scarcely
shows. The four or five jiggers which, in spite of the fact that I
constantly wore high laced boots, chose my feet to settle in, were
taken out for me by the all-accomplished Knudsen, after which I
thought it advisable to wash out the cavities with corrosive
sublimate. The natives have a different sort of disinfectant—they fill
the hole with scraped roots. In a tiny Makua village on the slope of
the plateau south of Newala, we saw an old woman who had filled all
the spaces under her toe-nails with powdered roots by way of
prophylactic treatment. What will be the result, if any, who can say?
The rest of the many trifling ills which trouble our existence are
really more comic than serious. In the absence of anything else to
smoke, Knudsen and I at last opened a box of cigars procured from
the Indian store-keeper at Lindi, and tried them, with the most
distressing results. Whether they contain opium or some other
narcotic, neither of us can say, but after the tenth puff we were both
“off,” three-quarters stupefied and unspeakably wretched. Slowly we
recovered—and what happened next? Half-an-hour later we were
once more smoking these poisonous concoctions—so insatiable is the
craving for tobacco in the tropics.
Even my present attacks of fever scarcely deserve to be taken
seriously. I have had no less than three here at Newala, all of which
have run their course in an incredibly short time. In the early
afternoon, I am busy with my old natives, asking questions and
making notes. The strong midday coffee has stimulated my spirits to
an extraordinary degree, the brain is active and vigorous, and work
progresses rapidly, while a pleasant warmth pervades the whole
body. Suddenly this gives place to a violent chill, forcing me to put on
my overcoat, though it is only half-past three and the afternoon sun
is at its hottest. Now the brain no longer works with such acuteness
and logical precision; more especially does it fail me in trying to
establish the syntax of the difficult Makua language on which I have
ventured, as if I had not enough to do without it. Under the
circumstances it seems advisable to take my temperature, and I do
so, to save trouble, without leaving my seat, and while going on with
my work. On examination, I find it to be 101·48°. My tutors are
abruptly dismissed and my bed set up in the baraza; a few minutes
later I am in it and treating myself internally with hot water and
lemon-juice.
Three hours later, the thermometer marks nearly 104°, and I make
them carry me back into the tent, bed and all, as I am now perspiring
heavily, and exposure to the cold wind just beginning to blow might
mean a fatal chill. I lie still for a little while, and then find, to my
great relief, that the temperature is not rising, but rather falling. This
is about 7.30 p.m. At 8 p.m. I find, to my unbounded astonishment,
that it has fallen below 98·6°, and I feel perfectly well. I read for an
hour or two, and could very well enjoy a smoke, if I had the
wherewithal—Indian cigars being out of the question.
Having no medical training, I am at a loss to account for this state
of things. It is impossible that these transitory attacks of high fever
should be malarial; it seems more probable that they are due to a
kind of sunstroke. On consulting my note-book, I become more and
more inclined to think this is the case, for these attacks regularly
follow extreme fatigue and long exposure to strong sunshine. They at
least have the advantage of being only short interruptions to my
work, as on the following morning I am always quite fresh and fit.
My treasure of a cook is suffering from an enormous hydrocele which
makes it difficult for him to get up, and Moritz is obliged to keep in
the dark on account of his inflamed eyes. Knudsen’s cook, a raw boy
from somewhere in the bush, knows still less of cooking than Omari;
consequently Nils Knudsen himself has been promoted to the vacant
post. Finding that we had come to the end of our supplies, he began
by sending to Chingulungulu for the four sucking-pigs which we had
bought from Matola and temporarily left in his charge; and when
they came up, neatly packed in a large crate, he callously slaughtered
the biggest of them. The first joint we were thoughtless enough to
entrust for roasting to Knudsen’s mshenzi cook, and it was
consequently uneatable; but we made the rest of the animal into a
jelly which we ate with great relish after weeks of underfeeding,
consuming incredible helpings of it at both midday and evening
meals. The only drawback is a certain want of variety in the tinned
vegetables. Dr. Jäger, to whom the Geographical Commission
entrusted the provisioning of the expeditions—mine as well as his
own—because he had more time on his hands than the rest of us,
seems to have laid in a huge stock of Teltow turnips,[46] an article of
food which is all very well for occasional use, but which quickly palls
when set before one every day; and we seem to have no other tins
left. There is no help for it—we must put up with the turnips; but I
am certain that, once I am home again, I shall not touch them for ten
years to come.
Amid all these minor evils, which, after all, go to make up the
genuine flavour of Africa, there is at least one cheering touch:
Knudsen has, with the dexterity of a skilled mechanic, repaired my 9
× 12 cm. camera, at least so far that I can use it with a little care.
How, in the absence of finger-nails, he was able to accomplish such a
ticklish piece of work, having no tool but a clumsy screw-driver for
taking to pieces and putting together again the complicated
mechanism of the instantaneous shutter, is still a mystery to me; but
he did it successfully. The loss of his finger-nails shows him in a light
contrasting curiously enough with the intelligence evinced by the
above operation; though, after all, it is scarcely surprising after his
ten years’ residence in the bush. One day, at Lindi, he had occasion
to wash a dog, which must have been in need of very thorough
cleansing, for the bottle handed to our friend for the purpose had an
extremely strong smell. Having performed his task in the most
conscientious manner, he perceived with some surprise that the dog
did not appear much the better for it, and was further surprised by
finding his own nails ulcerating away in the course of the next few
days. “How was I to know that carbolic acid has to be diluted?” he
mutters indignantly, from time to time, with a troubled gaze at his
mutilated finger-tips.
Since we came to Newala we have been making excursions in all
directions through the surrounding country, in accordance with old
habit, and also because the akida Sefu did not get together the tribal
elders from whom I wanted information so speedily as he had
promised. There is, however, no harm done, as, even if seen only
from the outside, the country and people are interesting enough.
The Makonde plateau is like a large rectangular table rounded off
at the corners. Measured from the Indian Ocean to Newala, it is
about seventy-five miles long, and between the Rovuma and the
Lukuledi it averages fifty miles in breadth, so that its superficial area
is about two-thirds of that of the kingdom of Saxony. The surface,
however, is not level, but uniformly inclined from its south-western
edge to the ocean. From the upper edge, on which Newala lies, the
eye ranges for many miles east and north-east, without encountering
any obstacle, over the Makonde bush. It is a green sea, from which
here and there thick clouds of smoke rise, to show that it, too, is
inhabited by men who carry on their tillage like so many other
primitive peoples, by cutting down and burning the bush, and
manuring with the ashes. Even in the radiant light of a tropical day
such a fire is a grand sight.
Much less effective is the impression produced just now by the
great western plain as seen from the edge of the plateau. As often as
time permits, I stroll along this edge, sometimes in one direction,
sometimes in another, in the hope of finding the air clear enough to
let me enjoy the view; but I have always been disappointed.
Wherever one looks, clouds of smoke rise from the burning bush,
and the air is full of smoke and vapour. It is a pity, for under more
favourable circumstances the panorama of the whole country up to
the distant Majeje hills must be truly magnificent. It is of little use
taking photographs now, and an outline sketch gives a very poor idea
of the scenery. In one of these excursions I went out of my way to
make a personal attempt on the Makonde bush. The present edge of
the plateau is the result of a far-reaching process of destruction
through erosion and denudation. The Makonde strata are
everywhere cut into by ravines, which, though short, are hundreds of
yards in depth. In consequence of the loose stratification of these
beds, not only are the walls of these ravines nearly vertical, but their
upper end is closed by an equally steep escarpment, so that the
western edge of the Makonde plateau is hemmed in by a series of
deep, basin-like valleys. In order to get from one side of such a ravine
to the other, I cut my way through the bush with a dozen of my men.
It was a very open part, with more grass than scrub, but even so the
short stretch of less than two hundred yards was very hard work; at
the end of it the men’s calicoes were in rags and they themselves
bleeding from hundreds of scratches, while even our strong khaki
suits had not escaped scatheless.

NATIVE PATH THROUGH THE MAKONDE BUSH, NEAR


MAHUTA

I see increasing reason to believe that the view formed some time
back as to the origin of the Makonde bush is the correct one. I have
no doubt that it is not a natural product, but the result of human
occupation. Those parts of the high country where man—as a very
slight amount of practice enables the eye to perceive at once—has not
yet penetrated with axe and hoe, are still occupied by a splendid
timber forest quite able to sustain a comparison with our mixed
forests in Germany. But wherever man has once built his hut or tilled
his field, this horrible bush springs up. Every phase of this process
may be seen in the course of a couple of hours’ walk along the main
road. From the bush to right or left, one hears the sound of the axe—
not from one spot only, but from several directions at once. A few
steps further on, we can see what is taking place. The brush has been
cut down and piled up in heaps to the height of a yard or more,
between which the trunks of the large trees stand up like the last
pillars of a magnificent ruined building. These, too, present a
melancholy spectacle: the destructive Makonde have ringed them—
cut a broad strip of bark all round to ensure their dying off—and also
piled up pyramids of brush round them. Father and son, mother and
son-in-law, are chopping away perseveringly in the background—too
busy, almost, to look round at the white stranger, who usually excites
so much interest. If you pass by the same place a week later, the piles
of brushwood have disappeared and a thick layer of ashes has taken
the place of the green forest. The large trees stretch their
smouldering trunks and branches in dumb accusation to heaven—if
they have not already fallen and been more or less reduced to ashes,
perhaps only showing as a white stripe on the dark ground.
This work of destruction is carried out by the Makonde alike on the
virgin forest and on the bush which has sprung up on sites already
cultivated and deserted. In the second case they are saved the trouble
of burning the large trees, these being entirely absent in the
secondary bush.
After burning this piece of forest ground and loosening it with the
hoe, the native sows his corn and plants his vegetables. All over the
country, he goes in for bed-culture, which requires, and, in fact,
receives, the most careful attention. Weeds are nowhere tolerated in
the south of German East Africa. The crops may fail on the plains,
where droughts are frequent, but never on the plateau with its
abundant rains and heavy dews. Its fortunate inhabitants even have
the satisfaction of seeing the proud Wayao and Wamakua working
for them as labourers, driven by hunger to serve where they were
accustomed to rule.
But the light, sandy soil is soon exhausted, and would yield no
harvest the second year if cultivated twice running. This fact has
been familiar to the native for ages; consequently he provides in
time, and, while his crop is growing, prepares the next plot with axe
and firebrand. Next year he plants this with his various crops and
lets the first piece lie fallow. For a short time it remains waste and
desolate; then nature steps in to repair the destruction wrought by
man; a thousand new growths spring out of the exhausted soil, and
even the old stumps put forth fresh shoots. Next year the new growth
is up to one’s knees, and in a few years more it is that terrible,
impenetrable bush, which maintains its position till the black
occupier of the land has made the round of all the available sites and
come back to his starting point.
The Makonde are, body and soul, so to speak, one with this bush.
According to my Yao informants, indeed, their name means nothing
else but “bush people.” Their own tradition says that they have been
settled up here for a very long time, but to my surprise they laid great
stress on an original immigration. Their old homes were in the
south-east, near Mikindani and the mouth of the Rovuma, whence
their peaceful forefathers were driven by the continual raids of the
Sakalavas from Madagascar and the warlike Shirazis[47] of the coast,
to take refuge on the almost inaccessible plateau. I have studied
African ethnology for twenty years, but the fact that changes of
population in this apparently quiet and peaceable corner of the earth
could have been occasioned by outside enterprises taking place on
the high seas, was completely new to me. It is, no doubt, however,
correct.
The charming tribal legend of the Makonde—besides informing us
of other interesting matters—explains why they have to live in the
thickest of the bush and a long way from the edge of the plateau,
instead of making their permanent homes beside the purling brooks
and springs of the low country.
“The place where the tribe originated is Mahuta, on the southern
side of the plateau towards the Rovuma, where of old time there was
nothing but thick bush. Out of this bush came a man who never
washed himself or shaved his head, and who ate and drank but little.
He went out and made a human figure from the wood of a tree
growing in the open country, which he took home to his abode in the
bush and there set it upright. In the night this image came to life and
was a woman. The man and woman went down together to the
Rovuma to wash themselves. Here the woman gave birth to a still-
born child. They left that place and passed over the high land into the
valley of the Mbemkuru, where the woman had another child, which
was also born dead. Then they returned to the high bush country of
Mahuta, where the third child was born, which lived and grew up. In
course of time, the couple had many more children, and called
themselves Wamatanda. These were the ancestral stock of the
Makonde, also called Wamakonde,[48] i.e., aborigines. Their
forefather, the man from the bush, gave his children the command to
bury their dead upright, in memory of the mother of their race who
was cut out of wood and awoke to life when standing upright. He also
warned them against settling in the valleys and near large streams,
for sickness and death dwelt there. They were to make it a rule to
have their huts at least an hour’s walk from the nearest watering-
place; then their children would thrive and escape illness.”
The explanation of the name Makonde given by my informants is
somewhat different from that contained in the above legend, which I
extract from a little book (small, but packed with information), by
Pater Adams, entitled Lindi und sein Hinterland. Otherwise, my
results agree exactly with the statements of the legend. Washing?
Hapana—there is no such thing. Why should they do so? As it is, the
supply of water scarcely suffices for cooking and drinking; other
people do not wash, so why should the Makonde distinguish himself
by such needless eccentricity? As for shaving the head, the short,
woolly crop scarcely needs it,[49] so the second ancestral precept is
likewise easy enough to follow. Beyond this, however, there is
nothing ridiculous in the ancestor’s advice. I have obtained from
various local artists a fairly large number of figures carved in wood,
ranging from fifteen to twenty-three inches in height, and
representing women belonging to the great group of the Mavia,
Makonde, and Matambwe tribes. The carving is remarkably well
done and renders the female type with great accuracy, especially the
keloid ornamentation, to be described later on. As to the object and
meaning of their works the sculptors either could or (more probably)
would tell me nothing, and I was forced to content myself with the
scanty information vouchsafed by one man, who said that the figures
were merely intended to represent the nembo—the artificial
deformations of pelele, ear-discs, and keloids. The legend recorded
by Pater Adams places these figures in a new light. They must surely
be more than mere dolls; and we may even venture to assume that
they are—though the majority of present-day Makonde are probably
unaware of the fact—representations of the tribal ancestress.
The references in the legend to the descent from Mahuta to the
Rovuma, and to a journey across the highlands into the Mbekuru
valley, undoubtedly indicate the previous history of the tribe, the
travels of the ancestral pair typifying the migrations of their
descendants. The descent to the neighbouring Rovuma valley, with
its extraordinary fertility and great abundance of game, is intelligible
at a glance—but the crossing of the Lukuledi depression, the ascent
to the Rondo Plateau and the descent to the Mbemkuru, also lie
within the bounds of probability, for all these districts have exactly
the same character as the extreme south. Now, however, comes a
point of especial interest for our bacteriological age. The primitive
Makonde did not enjoy their lives in the marshy river-valleys.
Disease raged among them, and many died. It was only after they
had returned to their original home near Mahuta, that the health
conditions of these people improved. We are very apt to think of the
African as a stupid person whose ignorance of nature is only equalled
by his fear of it, and who looks on all mishaps as caused by evil
spirits and malignant natural powers. It is much more correct to
assume in this case that the people very early learnt to distinguish
districts infested with malaria from those where it is absent.
This knowledge is crystallized in the
ancestral warning against settling in the
valleys and near the great waters, the
dwelling-places of disease and death. At the
same time, for security against the hostile
Mavia south of the Rovuma, it was enacted
that every settlement must be not less than a
certain distance from the southern edge of the
plateau. Such in fact is their mode of life at the
present day. It is not such a bad one, and
certainly they are both safer and more
comfortable than the Makua, the recent
intruders from the south, who have made USUAL METHOD OF
good their footing on the western edge of the CLOSING HUT-DOOR
plateau, extending over a fairly wide belt of
country. Neither Makua nor Makonde show in their dwellings
anything of the size and comeliness of the Yao houses in the plain,
especially at Masasi, Chingulungulu and Zuza’s. Jumbe Chauro, a
Makonde hamlet not far from Newala, on the road to Mahuta, is the
most important settlement of the tribe I have yet seen, and has fairly
spacious huts. But how slovenly is their construction compared with
the palatial residences of the elephant-hunters living in the plain.
The roofs are still more untidy than in the general run of huts during
the dry season, the walls show here and there the scanty beginnings
or the lamentable remains of the mud plastering, and the interior is a
veritable dog-kennel; dirt, dust and disorder everywhere. A few huts
only show any attempt at division into rooms, and this consists
merely of very roughly-made bamboo partitions. In one point alone
have I noticed any indication of progress—in the method of fastening
the door. Houses all over the south are secured in a simple but
ingenious manner. The door consists of a set of stout pieces of wood
or bamboo, tied with bark-string to two cross-pieces, and moving in
two grooves round one of the door-posts, so as to open inwards. If
the owner wishes to leave home, he takes two logs as thick as a man’s
upper arm and about a yard long. One of these is placed obliquely
against the middle of the door from the inside, so as to form an angle
of from 60° to 75° with the ground. He then places the second piece
horizontally across the first, pressing it downward with all his might.
It is kept in place by two strong posts planted in the ground a few
inches inside the door. This fastening is absolutely safe, but of course
cannot be applied to both doors at once, otherwise how could the
owner leave or enter his house? I have not yet succeeded in finding
out how the back door is fastened.

MAKONDE LOCK AND KEY AT JUMBE CHAURO


This is the general way of closing a house. The Makonde at Jumbe
Chauro, however, have a much more complicated, solid and original
one. Here, too, the door is as already described, except that there is
only one post on the inside, standing by itself about six inches from
one side of the doorway. Opposite this post is a hole in the wall just
large enough to admit a man’s arm. The door is closed inside by a
large wooden bolt passing through a hole in this post and pressing
with its free end against the door. The other end has three holes into
which fit three pegs running in vertical grooves inside the post. The
door is opened with a wooden key about a foot long, somewhat
curved and sloped off at the butt; the other end has three pegs
corresponding to the holes, in the bolt, so that, when it is thrust
through the hole in the wall and inserted into the rectangular
opening in the post, the pegs can be lifted and the bolt drawn out.[50]

MODE OF INSERTING THE KEY

With no small pride first one householder and then a second


showed me on the spot the action of this greatest invention of the
Makonde Highlands. To both with an admiring exclamation of
“Vizuri sana!” (“Very fine!”). I expressed the wish to take back these
marvels with me to Ulaya, to show the Wazungu what clever fellows
the Makonde are. Scarcely five minutes after my return to camp at
Newala, the two men came up sweating under the weight of two
heavy logs which they laid down at my feet, handing over at the same
time the keys of the fallen fortress. Arguing, logically enough, that if
the key was wanted, the lock would be wanted with it, they had taken
their axes and chopped down the posts—as it never occurred to them
to dig them out of the ground and so bring them intact. Thus I have
two badly damaged specimens, and the owners, instead of praise,
come in for a blowing-up.
The Makua huts in the environs of Newala are especially
miserable; their more than slovenly construction reminds one of the
temporary erections of the Makua at Hatia’s, though the people here
have not been concerned in a war. It must therefore be due to
congenital idleness, or else to the absence of a powerful chief. Even
the baraza at Mlipa’s, a short hour’s walk south-east of Newala,
shares in this general neglect. While public buildings in this country
are usually looked after more or less carefully, this is in evident
danger of being blown over by the first strong easterly gale. The only
attractive object in this whole district is the grave of the late chief
Mlipa. I visited it in the morning, while the sun was still trying with
partial success to break through the rolling mists, and the circular
grove of tall euphorbias, which, with a broken pot, is all that marks
the old king’s resting-place, impressed one with a touch of pathos.
Even my very materially-minded carriers seemed to feel something
of the sort, for instead of their usual ribald songs, they chanted
solemnly, as we marched on through the dense green of the Makonde
bush:—
“We shall arrive with the great master; we stand in a row and have
no fear about getting our food and our money from the Serkali (the
Government). We are not afraid; we are going along with the great
master, the lion; we are going down to the coast and back.”
With regard to the characteristic features of the various tribes here
on the western edge of the plateau, I can arrive at no other
conclusion than the one already come to in the plain, viz., that it is
impossible for anyone but a trained anthropologist to assign any
given individual at once to his proper tribe. In fact, I think that even
an anthropological specialist, after the most careful examination,
might find it a difficult task to decide. The whole congeries of peoples
collected in the region bounded on the west by the great Central
African rift, Tanganyika and Nyasa, and on the east by the Indian
Ocean, are closely related to each other—some of their languages are
only distinguished from one another as dialects of the same speech,
and no doubt all the tribes present the same shape of skull and
structure of skeleton. Thus, surely, there can be no very striking
differences in outward appearance.
Even did such exist, I should have no time
to concern myself with them, for day after day,
I have to see or hear, as the case may be—in
any case to grasp and record—an
extraordinary number of ethnographic
phenomena. I am almost disposed to think it
fortunate that some departments of inquiry, at
least, are barred by external circumstances.
Chief among these is the subject of iron-
working. We are apt to think of Africa as a
country where iron ore is everywhere, so to
speak, to be picked up by the roadside, and
where it would be quite surprising if the
inhabitants had not learnt to smelt the
material ready to their hand. In fact, the
knowledge of this art ranges all over the
continent, from the Kabyles in the north to the
Kafirs in the south. Here between the Rovuma
and the Lukuledi the conditions are not so
favourable. According to the statements of the
Makonde, neither ironstone nor any other
form of iron ore is known to them. They have
not therefore advanced to the art of smelting
the metal, but have hitherto bought all their
THE ANCESTRESS OF
THE MAKONDE
iron implements from neighbouring tribes.
Even in the plain the inhabitants are not much
better off. Only one man now living is said to
understand the art of smelting iron. This old fundi lives close to
Huwe, that isolated, steep-sided block of granite which rises out of
the green solitude between Masasi and Chingulungulu, and whose
jagged and splintered top meets the traveller’s eye everywhere. While
still at Masasi I wished to see this man at work, but was told that,
frightened by the rising, he had retired across the Rovuma, though
he would soon return. All subsequent inquiries as to whether the
fundi had come back met with the genuine African answer, “Bado”
(“Not yet”).
BRAZIER

Some consolation was afforded me by a brassfounder, whom I


came across in the bush near Akundonde’s. This man is the favourite
of women, and therefore no doubt of the gods; he welds the glittering
brass rods purchased at the coast into those massive, heavy rings
which, on the wrists and ankles of the local fair ones, continually give
me fresh food for admiration. Like every decent master-craftsman he
had all his tools with him, consisting of a pair of bellows, three
crucibles and a hammer—nothing more, apparently. He was quite
willing to show his skill, and in a twinkling had fixed his bellows on
the ground. They are simply two goat-skins, taken off whole, the four
legs being closed by knots, while the upper opening, intended to
admit the air, is kept stretched by two pieces of wood. At the lower
end of the skin a smaller opening is left into which a wooden tube is
stuck. The fundi has quickly borrowed a heap of wood-embers from
the nearest hut; he then fixes the free ends of the two tubes into an
earthen pipe, and clamps them to the ground by means of a bent
piece of wood. Now he fills one of his small clay crucibles, the dross
on which shows that they have been long in use, with the yellow
material, places it in the midst of the embers, which, at present are
only faintly glimmering, and begins his work. In quick alternation
the smith’s two hands move up and down with the open ends of the
bellows; as he raises his hand he holds the slit wide open, so as to let
the air enter the skin bag unhindered. In pressing it down he closes
the bag, and the air puffs through the bamboo tube and clay pipe into
the fire, which quickly burns up. The smith, however, does not keep
on with this work, but beckons to another man, who relieves him at
the bellows, while he takes some more tools out of a large skin pouch
carried on his back. I look on in wonder as, with a smooth round
stick about the thickness of a finger, he bores a few vertical holes into
the clean sand of the soil. This should not be difficult, yet the man
seems to be taking great pains over it. Then he fastens down to the
ground, with a couple of wooden clamps, a neat little trough made by
splitting a joint of bamboo in half, so that the ends are closed by the
two knots. At last the yellow metal has attained the right consistency,
and the fundi lifts the crucible from the fire by means of two sticks
split at the end to serve as tongs. A short swift turn to the left—a
tilting of the crucible—and the molten brass, hissing and giving forth
clouds of smoke, flows first into the bamboo mould and then into the
holes in the ground.
The technique of this backwoods craftsman may not be very far
advanced, but it cannot be denied that he knows how to obtain an
adequate result by the simplest means. The ladies of highest rank in
this country—that is to say, those who can afford it, wear two kinds
of these massive brass rings, one cylindrical, the other semicircular
in section. The latter are cast in the most ingenious way in the
bamboo mould, the former in the circular hole in the sand. It is quite
a simple matter for the fundi to fit these bars to the limbs of his fair
customers; with a few light strokes of his hammer he bends the
pliable brass round arm or ankle without further inconvenience to
the wearer.
SHAPING THE POT

SMOOTHING WITH MAIZE-COB

CUTTING THE EDGE


FINISHING THE BOTTOM

LAST SMOOTHING BEFORE


BURNING

FIRING THE BRUSH-PILE


LIGHTING THE FARTHER SIDE OF
THE PILE

TURNING THE RED-HOT VESSEL

NYASA WOMAN MAKING POTS AT MASASI


Pottery is an art which must always and everywhere excite the
interest of the student, just because it is so intimately connected with
the development of human culture, and because its relics are one of
the principal factors in the reconstruction of our own condition in
prehistoric times. I shall always remember with pleasure the two or
three afternoons at Masasi when Salim Matola’s mother, a slightly-
built, graceful, pleasant-looking woman, explained to me with
touching patience, by means of concrete illustrations, the ceramic art
of her people. The only implements for this primitive process were a
lump of clay in her left hand, and in the right a calabash containing
the following valuables: the fragment of a maize-cob stripped of all
its grains, a smooth, oval pebble, about the size of a pigeon’s egg, a
few chips of gourd-shell, a bamboo splinter about the length of one’s
hand, a small shell, and a bunch of some herb resembling spinach.
Nothing more. The woman scraped with the
shell a round, shallow hole in the soft, fine
sand of the soil, and, when an active young
girl had filled the calabash with water for her,
she began to knead the clay. As if by magic it
gradually assumed the shape of a rough but
already well-shaped vessel, which only wanted
a little touching up with the instruments
before mentioned. I looked out with the
MAKUA WOMAN closest attention for any indication of the use
MAKING A POT. of the potter’s wheel, in however rudimentary
SHOWS THE a form, but no—hapana (there is none). The
BEGINNINGS OF THE embryo pot stood firmly in its little
POTTER’S WHEEL
depression, and the woman walked round it in
a stooping posture, whether she was removing
small stones or similar foreign bodies with the maize-cob, smoothing
the inner or outer surface with the splinter of bamboo, or later, after
letting it dry for a day, pricking in the ornamentation with a pointed
bit of gourd-shell, or working out the bottom, or cutting the edge
with a sharp bamboo knife, or giving the last touches to the finished
vessel. This occupation of the women is infinitely toilsome, but it is
without doubt an accurate reproduction of the process in use among
our ancestors of the Neolithic and Bronze ages.
There is no doubt that the invention of pottery, an item in human
progress whose importance cannot be over-estimated, is due to
women. Rough, coarse and unfeeling, the men of the horde range
over the countryside. When the united cunning of the hunters has
succeeded in killing the game; not one of them thinks of carrying
home the spoil. A bright fire, kindled by a vigorous wielding of the
drill, is crackling beside them; the animal has been cleaned and cut
up secundum artem, and, after a slight singeing, will soon disappear
under their sharp teeth; no one all this time giving a single thought
to wife or child.
To what shifts, on the other hand, the primitive wife, and still more
the primitive mother, was put! Not even prehistoric stomachs could
endure an unvarying diet of raw food. Something or other suggested
the beneficial effect of hot water on the majority of approved but
indigestible dishes. Perhaps a neighbour had tried holding the hard
roots or tubers over the fire in a calabash filled with water—or maybe
an ostrich-egg-shell, or a hastily improvised vessel of bark. They
became much softer and more palatable than they had previously
been; but, unfortunately, the vessel could not stand the fire and got
charred on the outside. That can be remedied, thought our
ancestress, and plastered a layer of wet clay round a similar vessel.
This is an improvement; the cooking utensil remains uninjured, but
the heat of the fire has shrunk it, so that it is loose in its shell. The
next step is to detach it, so, with a firm grip and a jerk, shell and
kernel are separated, and pottery is invented. Perhaps, however, the
discovery which led to an intelligent use of the burnt-clay shell, was
made in a slightly different way. Ostrich-eggs and calabashes are not
to be found in every part of the world, but everywhere mankind has
arrived at the art of making baskets out of pliant materials, such as
bark, bast, strips of palm-leaf, supple twigs, etc. Our inventor has no
water-tight vessel provided by nature. “Never mind, let us line the
basket with clay.” This answers the purpose, but alas! the basket gets
burnt over the blazing fire, the woman watches the process of
cooking with increasing uneasiness, fearing a leak, but no leak
appears. The food, done to a turn, is eaten with peculiar relish; and
the cooking-vessel is examined, half in curiosity, half in satisfaction
at the result. The plastic clay is now hard as stone, and at the same
time looks exceedingly well, for the neat plaiting of the burnt basket
is traced all over it in a pretty pattern. Thus, simultaneously with
pottery, its ornamentation was invented.
Primitive woman has another claim to respect. It was the man,
roving abroad, who invented the art of producing fire at will, but the
woman, unable to imitate him in this, has been a Vestal from the
earliest times. Nothing gives so much trouble as the keeping alight of
the smouldering brand, and, above all, when all the men are absent
from the camp. Heavy rain-clouds gather, already the first large
drops are falling, the first gusts of the storm rage over the plain. The
little flame, a greater anxiety to the woman than her own children,
flickers unsteadily in the blast. What is to be done? A sudden thought
occurs to her, and in an instant she has constructed a primitive hut
out of strips of bark, to protect the flame against rain and wind.
This, or something very like it, was the way in which the principle
of the house was discovered; and even the most hardened misogynist
cannot fairly refuse a woman the credit of it. The protection of the
hearth-fire from the weather is the germ from which the human
dwelling was evolved. Men had little, if any share, in this forward
step, and that only at a late stage. Even at the present day, the
plastering of the housewall with clay and the manufacture of pottery
are exclusively the women’s business. These are two very significant
survivals. Our European kitchen-garden, too, is originally a woman’s
invention, and the hoe, the primitive instrument of agriculture, is,
characteristically enough, still used in this department. But the
noblest achievement which we owe to the other sex is unquestionably
the art of cookery. Roasting alone—the oldest process—is one for
which men took the hint (a very obvious one) from nature. It must
have been suggested by the scorched carcase of some animal
overtaken by the destructive forest-fires. But boiling—the process of
improving organic substances by the help of water heated to boiling-
point—is a much later discovery. It is so recent that it has not even
yet penetrated to all parts of the world. The Polynesians understand
how to steam food, that is, to cook it, neatly wrapped in leaves, in a
hole in the earth between hot stones, the air being excluded, and
(sometimes) a few drops of water sprinkled on the stones; but they
do not understand boiling.
To come back from this digression, we find that the slender Nyasa
woman has, after once more carefully examining the finished pot,
put it aside in the shade to dry. On the following day she sends me
word by her son, Salim Matola, who is always on hand, that she is
going to do the burning, and, on coming out of my house, I find her
already hard at work. She has spread on the ground a layer of very
dry sticks, about as thick as one’s thumb, has laid the pot (now of a
yellowish-grey colour) on them, and is piling brushwood round it.
My faithful Pesa mbili, the mnyampara, who has been standing by,
most obligingly, with a lighted stick, now hands it to her. Both of
them, blowing steadily, light the pile on the lee side, and, when the
flame begins to catch, on the weather side also. Soon the whole is in a
blaze, but the dry fuel is quickly consumed and the fire dies down, so
that we see the red-hot vessel rising from the ashes. The woman
turns it continually with a long stick, sometimes one way and
sometimes another, so that it may be evenly heated all over. In
twenty minutes she rolls it out of the ash-heap, takes up the bundle
of spinach, which has been lying for two days in a jar of water, and
sprinkles the red-hot clay with it. The places where the drops fall are
marked by black spots on the uniform reddish-brown surface. With a
sigh of relief, and with visible satisfaction, the woman rises to an
erect position; she is standing just in a line between me and the fire,
from which a cloud of smoke is just rising: I press the ball of my
camera, the shutter clicks—the apotheosis is achieved! Like a
priestess, representative of her inventive sex, the graceful woman
stands: at her feet the hearth-fire she has given us beside her the
invention she has devised for us, in the background the home she has
built for us.
At Newala, also, I have had the manufacture of pottery carried on
in my presence. Technically the process is better than that already
described, for here we find the beginnings of the potter’s wheel,
which does not seem to exist in the plains; at least I have seen
nothing of the sort. The artist, a frightfully stupid Makua woman, did
not make a depression in the ground to receive the pot she was about
to shape, but used instead a large potsherd. Otherwise, she went to
work in much the same way as Salim’s mother, except that she saved
herself the trouble of walking round and round her work by squatting
at her ease and letting the pot and potsherd rotate round her; this is
surely the first step towards a machine. But it does not follow that
the pot was improved by the process. It is true that it was beautifully
rounded and presented a very creditable appearance when finished,
but the numerous large and small vessels which I have seen, and, in
part, collected, in the “less advanced” districts, are no less so. We
moderns imagine that instruments of precision are necessary to
produce excellent results. Go to the prehistoric collections of our
museums and look at the pots, urns and bowls of our ancestors in the
dim ages of the past, and you will at once perceive your error.
MAKING LONGITUDINAL CUT IN
BARK

DRAWING THE BARK OFF THE LOG

REMOVING THE OUTER BARK


BEATING THE BARK

WORKING THE BARK-CLOTH AFTER BEATING, TO MAKE IT


SOFT

MANUFACTURE OF BARK-CLOTH AT NEWALA


To-day, nearly the whole population of German East Africa is
clothed in imported calico. This was not always the case; even now in
some parts of the north dressed skins are still the prevailing wear,
and in the north-western districts—east and north of Lake
Tanganyika—lies a zone where bark-cloth has not yet been
superseded. Probably not many generations have passed since such
bark fabrics and kilts of skins were the only clothing even in the
south. Even to-day, large quantities of this bright-red or drab
material are still to be found; but if we wish to see it, we must look in
the granaries and on the drying stages inside the native huts, where
it serves less ambitious uses as wrappings for those seeds and fruits
which require to be packed with special care. The salt produced at
Masasi, too, is packed for transport to a distance in large sheets of
bark-cloth. Wherever I found it in any degree possible, I studied the
process of making this cloth. The native requisitioned for the
purpose arrived, carrying a log between two and three yards long and
as thick as his thigh, and nothing else except a curiously-shaped
mallet and the usual long, sharp and pointed knife which all men and
boys wear in a belt at their backs without a sheath—horribile dictu!
[51]
Silently he squats down before me, and with two rapid cuts has
drawn a couple of circles round the log some two yards apart, and
slits the bark lengthwise between them with the point of his knife.
With evident care, he then scrapes off the outer rind all round the
log, so that in a quarter of an hour the inner red layer of the bark
shows up brightly-coloured between the two untouched ends. With
some trouble and much caution, he now loosens the bark at one end,
and opens the cylinder. He then stands up, takes hold of the free
edge with both hands, and turning it inside out, slowly but steadily
pulls it off in one piece. Now comes the troublesome work of
scraping all superfluous particles of outer bark from the outside of
the long, narrow piece of material, while the inner side is carefully
scrutinised for defective spots. At last it is ready for beating. Having
signalled to a friend, who immediately places a bowl of water beside
him, the artificer damps his sheet of bark all over, seizes his mallet,
lays one end of the stuff on the smoothest spot of the log, and
hammers away slowly but continuously. “Very simple!” I think to
myself. “Why, I could do that, too!”—but I am forced to change my
opinions a little later on; for the beating is quite an art, if the fabric is
not to be beaten to pieces. To prevent the breaking of the fibres, the
stuff is several times folded across, so as to interpose several
thicknesses between the mallet and the block. At last the required
state is reached, and the fundi seizes the sheet, still folded, by both
ends, and wrings it out, or calls an assistant to take one end while he
holds the other. The cloth produced in this way is not nearly so fine
and uniform in texture as the famous Uganda bark-cloth, but it is
quite soft, and, above all, cheap.
Now, too, I examine the mallet. My craftsman has been using the
simpler but better form of this implement, a conical block of some
hard wood, its base—the striking surface—being scored across and
across with more or less deeply-cut grooves, and the handle stuck
into a hole in the middle. The other and earlier form of mallet is
shaped in the same way, but the head is fastened by an ingenious
network of bark strips into the split bamboo serving as a handle. The
observation so often made, that ancient customs persist longest in
connection with religious ceremonies and in the life of children, here
finds confirmation. As we shall soon see, bark-cloth is still worn
during the unyago,[52] having been prepared with special solemn
ceremonies; and many a mother, if she has no other garment handy,
will still put her little one into a kilt of bark-cloth, which, after all,
looks better, besides being more in keeping with its African
surroundings, than the ridiculous bit of print from Ulaya.
MAKUA WOMEN

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