Book contents
- Frontmatter
- Introduction
- Part I Medicine and Disease: An Overview
- Part II Changing Concepts of Health and Disease
- Part III Medical Specialties and Disease Prevention
- Part IV Measuring Health
- Part V The History of Human Disease in the World Outside Asia
- Part VI The History of Human Disease in Asia
- Part VII The Geography of Human Disease
- Part VIII Major Human Diseases Past and Present
- VIII.1 Acquired Immune Deficiency Syndrome (AIDS)
- VIII.2 African Trypanosomiasis (Sleeping Sickness)
- VIII.3 Ainhum
- VIII.4 Alzheimer’s Disease
- VIII.5 Amebic Dysentery
- VIII.6 Anemia
- VIII.7 Anorexia Nervosa
- VIII.8 Anthrax
- VIII.9 Apoplexy and Stroke
- VIII.10 Arboviruses
- VIII.11 Arenaviruses
- VIII.12 Arthritis (Rheumatoid)
- VIII.13 Ascariasis
- VIII.14 Bacillary Dysentery
- VIII.15 Beriberi
- VIII.16 Black Death
- VIII.17 Black and Brown Lung Disease
- VIII.18 Bleeding Disorders
- VIII.19 Botulism
- VIII.20 Brucellosis
- VIII.21 Bubonic Plague
- VIII.22 Carrión’s Disease
- VIII.23 Catarrh
- VIII.24 Cestode Infections
- VIII.25 Chagas’ Disease
- VIII.26 Chlorosis
- VIII.27 Cholera
- VIII.28 Cirrhosis
- VIII.29 Clonorchiasis
- VIII.30 Croup
- VIII.31 Cystic Fibrosis
- VIII.32 Cytomegalovirus Infection
- VIII.33 Dengue
- VIII.34 Diabetes
- VIII.35 Diarrheal Diseases (Acute)
- VIII.36 Diphtheria
- VIII.37 Down Syndrome
- VIII.38 Dracunculiasis
- VIII.39 Dropsy
- VIII.40 Dysentery
- VIII.41 Dyspepsia
- VIII.42 Ebola Virus Disease
- VIII.43 Echinococcosis (Hydatidosis)
- VIII.44 Eclampsia
- VIII.45 Emphysema
- VIII.46 Encephalitis Lethargica
- VIII.47 Enterobiasis
- VIII.48 Epilepsy
- VIII.49 Ergotism
- VIII.50 Erysipelas
- VIII.51 Fascioliasis
- VIII.52 Fasciolopsiasis
- VIII.53 Favism
- VIII.54 Filariasis
- VIII.55 Fungus Infections (Mycoses)
- VIII.56 Fungus Poisoning
- VIII.57 Gallstones (Cholelithiasis)
- VIII.58 Gangrene
- VIII.59 Giardiasis
- VIII.60 Glomerulonephritis (Bright’s Disease)
- VIII.61 Goiter
- VIII.62 Gonorrhea
- VIII.63 Gout
- VIII.64 Herpes Simplex
- VIII.65 Herpesviruses
- VIII.66 Histoplasmosis
- VIII.67 Hookworm Disease
- VIII.68 Huntington’s Disease (Chorea)
- VIII.69 Hypertension
- VIII.70 Infectious Hepatitis
- VIII.71 Infectious Mononucleosis
- VIII.72 Inflammatory Bowel Disease
- VIII.73 Influenza
- VIII.74 Japanese B Encephalitis
- VIII.75 Lactose Intolerance and Malabsorption
- VIII.76 Lassa Fever
- VIII.77 Lead Poisoning
- VIII.78 Legionnaires’ Disease
- VIII.79 Leishmaniasis
- VIII.80 Leprosy
- VIII.81 Leptospirosis
- VIII.82 Leukemia
- VIII.83 Lupus Erythematosus
- VIII.84 Lyme Borreliosis (Lyme Disease)
- VIII.85 Malaria
- VIII.86 Marburg Virus Disease
- VIII.87 Mastoiditis
- VIII.88 Measles
- VIII.89 Meningitis
- VIII.90 Milk Sickness (Tremetol Poisoning)
- VIII.91 Multiple Sclerosis
- VIII.92 Mumps
- VIII.93 Muscular Dystrophy
- VIII.94 Myasthenia Gravis
- VIII.95 Nematode Infections
- VIII.96 Onchocerciasis
- VIII.97 Ophthalmia (Conjunctivitis and Trachoma)
- VIII.98 Osteoarthritis
- VIII.99 Osteoporosis
- VIII.100 Paget’s Disease of Bone
- VIII.101 Paragonimiasis
- VIII.102 Parkinson’s Disease
- VIII.103 Pellagra
- VIII.104 Periodontal Disease (Pyorrhea)
- VIII.105 Pica
- VIII.106 Pinta
- VIII.107 Plague of Athens
- VIII.108 Pneumocystis Pneumonia (Interstitial Plasma Cell Pneumonia, Pneumocystosis)
- VIII.109 Pneumonia
- VIII.110 Poliomyelitis
- VIII.111 Protein-Energy Malnutrition
- VIII.112 Protozoan Infections
- VIII.113 Puerperal Fever
- VIII.114 Q Fever
- VIII.115 Rabies
- VIII.116 Relapsing Fever
- VIII.117 Rheumatic Fever and Rheumatic Heart Disease
- VIII.118 Rickets and Osteomalacia
- VIII.119 Rickettsial Diseases
- VIII.120 Rocky Mountain Spotted Fever and the Spotted Fever Group Diseases
- VIII.121 Rubella
- VIII.122 St. Anthony’s Fire
- VIII.123 Scarlet Fever
- VIII.124 Schistosomiasis
- VIII.125 Scrofula (Scrophula)
- VIII.126 Scurvy
- VIII.127 Sickle-Cell Anemia
- VIII.128 Smallpox
- VIII.129 Streptococcal Diseases
- VIII.130 Strongyloidiasis
- VIII.131 Sudden Infant Death Syndrome
- VIII.132 Sudden Unexplained Death Syndrome (Asian)
- VIII.133 Sweating Sickness
- VIII.134 Syphilis
- VIII.135 Syphilis, Nonvenereal
- VIII.136 Tapeworm
- VIII.137 Tay-Sachs Disease
- VIII.138 Tetanus
- VIII.139 Tetanus, Neonatal
- VIII.140 Tetany
- VIII.141 Toxoplasmosis
- VIII.142 Trematode Infections
- VIII.143 Trench Fever
- VIII.144 The Treponematoses
- VIII.145 Trichinosis
- VIII.146 Trichuriasis
- VIII.147 Tuberculosis
- VIII.148 Tularemia
- VIII.149 Typhoid Fever
- VIII.150 Typhomalarial Fever
- VIII.151 Typhus, Epidemic
- VIII.152 Typhus, Murine
- VIII.153 Typhus, Scrub (Tsutsugamushi)
- VIII.154 Urolithiasis (Renal and Urinary Bladder Stone Disease)
- VIII.155 Varicella Zoster
- VIII.156 Whooping Cough
- VIII.157 Yaws
- VIII.158 Yellow Fever
- Indexes
- References
VIII.18 - Bleeding Disorders
from Part VIII - Major Human Diseases Past and Present
Published online by Cambridge University Press: 28 March 2008
- Frontmatter
- Introduction
- Part I Medicine and Disease: An Overview
- Part II Changing Concepts of Health and Disease
- Part III Medical Specialties and Disease Prevention
- Part IV Measuring Health
- Part V The History of Human Disease in the World Outside Asia
- Part VI The History of Human Disease in Asia
- Part VII The Geography of Human Disease
- Part VIII Major Human Diseases Past and Present
- VIII.1 Acquired Immune Deficiency Syndrome (AIDS)
- VIII.2 African Trypanosomiasis (Sleeping Sickness)
- VIII.3 Ainhum
- VIII.4 Alzheimer’s Disease
- VIII.5 Amebic Dysentery
- VIII.6 Anemia
- VIII.7 Anorexia Nervosa
- VIII.8 Anthrax
- VIII.9 Apoplexy and Stroke
- VIII.10 Arboviruses
- VIII.11 Arenaviruses
- VIII.12 Arthritis (Rheumatoid)
- VIII.13 Ascariasis
- VIII.14 Bacillary Dysentery
- VIII.15 Beriberi
- VIII.16 Black Death
- VIII.17 Black and Brown Lung Disease
- VIII.18 Bleeding Disorders
- VIII.19 Botulism
- VIII.20 Brucellosis
- VIII.21 Bubonic Plague
- VIII.22 Carrión’s Disease
- VIII.23 Catarrh
- VIII.24 Cestode Infections
- VIII.25 Chagas’ Disease
- VIII.26 Chlorosis
- VIII.27 Cholera
- VIII.28 Cirrhosis
- VIII.29 Clonorchiasis
- VIII.30 Croup
- VIII.31 Cystic Fibrosis
- VIII.32 Cytomegalovirus Infection
- VIII.33 Dengue
- VIII.34 Diabetes
- VIII.35 Diarrheal Diseases (Acute)
- VIII.36 Diphtheria
- VIII.37 Down Syndrome
- VIII.38 Dracunculiasis
- VIII.39 Dropsy
- VIII.40 Dysentery
- VIII.41 Dyspepsia
- VIII.42 Ebola Virus Disease
- VIII.43 Echinococcosis (Hydatidosis)
- VIII.44 Eclampsia
- VIII.45 Emphysema
- VIII.46 Encephalitis Lethargica
- VIII.47 Enterobiasis
- VIII.48 Epilepsy
- VIII.49 Ergotism
- VIII.50 Erysipelas
- VIII.51 Fascioliasis
- VIII.52 Fasciolopsiasis
- VIII.53 Favism
- VIII.54 Filariasis
- VIII.55 Fungus Infections (Mycoses)
- VIII.56 Fungus Poisoning
- VIII.57 Gallstones (Cholelithiasis)
- VIII.58 Gangrene
- VIII.59 Giardiasis
- VIII.60 Glomerulonephritis (Bright’s Disease)
- VIII.61 Goiter
- VIII.62 Gonorrhea
- VIII.63 Gout
- VIII.64 Herpes Simplex
- VIII.65 Herpesviruses
- VIII.66 Histoplasmosis
- VIII.67 Hookworm Disease
- VIII.68 Huntington’s Disease (Chorea)
- VIII.69 Hypertension
- VIII.70 Infectious Hepatitis
- VIII.71 Infectious Mononucleosis
- VIII.72 Inflammatory Bowel Disease
- VIII.73 Influenza
- VIII.74 Japanese B Encephalitis
- VIII.75 Lactose Intolerance and Malabsorption
- VIII.76 Lassa Fever
- VIII.77 Lead Poisoning
- VIII.78 Legionnaires’ Disease
- VIII.79 Leishmaniasis
- VIII.80 Leprosy
- VIII.81 Leptospirosis
- VIII.82 Leukemia
- VIII.83 Lupus Erythematosus
- VIII.84 Lyme Borreliosis (Lyme Disease)
- VIII.85 Malaria
- VIII.86 Marburg Virus Disease
- VIII.87 Mastoiditis
- VIII.88 Measles
- VIII.89 Meningitis
- VIII.90 Milk Sickness (Tremetol Poisoning)
- VIII.91 Multiple Sclerosis
- VIII.92 Mumps
- VIII.93 Muscular Dystrophy
- VIII.94 Myasthenia Gravis
- VIII.95 Nematode Infections
- VIII.96 Onchocerciasis
- VIII.97 Ophthalmia (Conjunctivitis and Trachoma)
- VIII.98 Osteoarthritis
- VIII.99 Osteoporosis
- VIII.100 Paget’s Disease of Bone
- VIII.101 Paragonimiasis
- VIII.102 Parkinson’s Disease
- VIII.103 Pellagra
- VIII.104 Periodontal Disease (Pyorrhea)
- VIII.105 Pica
- VIII.106 Pinta
- VIII.107 Plague of Athens
- VIII.108 Pneumocystis Pneumonia (Interstitial Plasma Cell Pneumonia, Pneumocystosis)
- VIII.109 Pneumonia
- VIII.110 Poliomyelitis
- VIII.111 Protein-Energy Malnutrition
- VIII.112 Protozoan Infections
- VIII.113 Puerperal Fever
- VIII.114 Q Fever
- VIII.115 Rabies
- VIII.116 Relapsing Fever
- VIII.117 Rheumatic Fever and Rheumatic Heart Disease
- VIII.118 Rickets and Osteomalacia
- VIII.119 Rickettsial Diseases
- VIII.120 Rocky Mountain Spotted Fever and the Spotted Fever Group Diseases
- VIII.121 Rubella
- VIII.122 St. Anthony’s Fire
- VIII.123 Scarlet Fever
- VIII.124 Schistosomiasis
- VIII.125 Scrofula (Scrophula)
- VIII.126 Scurvy
- VIII.127 Sickle-Cell Anemia
- VIII.128 Smallpox
- VIII.129 Streptococcal Diseases
- VIII.130 Strongyloidiasis
- VIII.131 Sudden Infant Death Syndrome
- VIII.132 Sudden Unexplained Death Syndrome (Asian)
- VIII.133 Sweating Sickness
- VIII.134 Syphilis
- VIII.135 Syphilis, Nonvenereal
- VIII.136 Tapeworm
- VIII.137 Tay-Sachs Disease
- VIII.138 Tetanus
- VIII.139 Tetanus, Neonatal
- VIII.140 Tetany
- VIII.141 Toxoplasmosis
- VIII.142 Trematode Infections
- VIII.143 Trench Fever
- VIII.144 The Treponematoses
- VIII.145 Trichinosis
- VIII.146 Trichuriasis
- VIII.147 Tuberculosis
- VIII.148 Tularemia
- VIII.149 Typhoid Fever
- VIII.150 Typhomalarial Fever
- VIII.151 Typhus, Epidemic
- VIII.152 Typhus, Murine
- VIII.153 Typhus, Scrub (Tsutsugamushi)
- VIII.154 Urolithiasis (Renal and Urinary Bladder Stone Disease)
- VIII.155 Varicella Zoster
- VIII.156 Whooping Cough
- VIII.157 Yaws
- VIII.158 Yellow Fever
- Indexes
- References
Summary
The existence of a hereditary tendency to excessive bleeding was recognized in the second century A.D. by Rabbi Judah, who exempted from circumcision the son of a woman whose earlier sons had bled to death after this rite. But only in this century has expanding knowledge of the physiology of hemostasis – the arrest of bleeding – made evident the diverse nature of inherited bleeding disorders. In addition, only recently has it been recognized that a tendency to thrombosis might likewise be due to an inherited hemostatic defect.
Physiology of Hemostasis
The mechanisms by which blood loss in mammals is stopped after vascular disruption are complex. Small vascular injuries are sealed by platelets that adhere to the site of damage, where they attract other circulating platelets, so as to form an occlusive aggregate or plug that can close small gaps. Larger defects in vessel walls are occluded by coagulation of blood – that is, by its transformation from a fluid to a gel-like state. Uncontrolled bleeding and its antithesis, thrombosis (the formation of a clot within a blood vessel), are important pathogenetic factors for human disease, including a large variety of hereditary disorders.
The basic structure of both the occlusive clots that halt blood loss and pathological intravascular clots (or thrombi). is a meshwork of fibrous protein (fibrin) that entraps blood cells. Plato and Aristotle both described the fibers found in shed blood. When the blood vessel wall is disrupted, whether by trauma or disease, a soluble plasma protein, fibrinogen (factor I), is transformed into the insoluble strand of fibrin.
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- The Cambridge World History of Human Disease , pp. 618 - 623Publisher: Cambridge University PressPrint publication year: 1993