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.127 - Sickle-Cell Anemia
from Part VIII - Major Human Diseases Past and Present
Published online by Cambridge University Press: 28 March 2008
Edited by
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
Summary
Sickle-cell disease is an inherited disorder resulting from an abnormality in the structure of a protein in the red blood cell called hemoglobin. It represents a spectrum of disorders ranging from the full-blown form, sickle-cell anemia, to the carrier state called sickle-cell trait. Also included in this spectrum are several other variant hemoglobin disorders, which all have the sickle hemoglobin. Sickle-cell anemia is the prototype for most molecular diseases and was the first disease to have its cause isolated to a single molecular change in the human genetic structure. This single change is responsible for all of the dramatic physiological changes and clinical events that occur in this disease.
Sickle-cell trait occurs when the individual is heterozygous for the sickle-cell gene and results in red blood cell concentrations of the abnormal hemoglobin (hemoglobin S) of less than 50 percent. It generally does not result in serious illness although this generalization has recently been disputed. In addition to sickle-cell trait, several sickle-cell syndromes occur when hemoglobin S is present in a heterozygous state with other hemoglobin variants – some with similar properties. Common examples of these include hemoglobin C and hemoglobin E.
Distribution and Incidence
Sickle-cell anemia is found in as many as 4 percent of Africans and in 1 percent of black Americans (1 per 500). Upward of 40 percent of Africans carry the sickle-cell trait as compared to 9 percent of black Americans. In some Mediterranean cultures the trait is also present. It is now generally believed that the sickle-cell gene mutation occurred independently in several areas of Africa.
- Type
- Chapter
- Information
- The Cambridge World History of Human Disease , pp. 1006 - 1008Publisher: Cambridge University PressPrint publication year: 1993
References
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