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.31 - Cystic Fibrosis
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
Cystic fibrosis, also called fibrocystic disease of the pancreas, and mucoviscidosis, is a genetically determined disease of infants, children, and young adults. Most of its many manifestations result from the abnormally viscous mucus, which interferes with pulmonary function, and the insufficient production of pancreatic digestive enzymes, which causes nutritional deficiencies and developmental retardation.
Etiology
Among Caucasians, cystic fibrosis (CF) is the most common fatal disease having an autosomal recessive inheritance. Despite the primary involvement of several organs, the disease is caused by a single defective gene that is located on chromosome 7 and is carried by about 4 percent of the Caucasian population. Its expression is similar in both sexes.
Clinical Manifestations
CF manifests itself at birth in about 8 percent of cases through mechanical obstruction of the small intestine by the secretion of abnormally viscous mucus (meconium ileus). Symptoms of insufficient secretion of exocrine (noninsulin) digestive enzymes by the pancreas appear during the first year of life in 90 percent of cases. The development of such symptoms indicates that pancreatic function is less than 10 percent of normal; and the more severe the deficiency of pancreatic enzymes, the more severe the fecal excretion of undigested fat, usually as diarrhea. As much as 80 percent of dietary fat may be lost, thus partially explaining malnutrition. Loss of undigested nutrients can be corrected only partially by treatment with pancreatic enzyme tablets. Pulmonary disease is responsible for most of the debility and mortality. Onset occurs in the first 2 years of life in at least 75 percent of cases, and by the age of 6 years in most of the remaining cases.
- Type
- Chapter
- Information
- The Cambridge World History of Human Disease , pp. 657 - 658Publisher: Cambridge University PressPrint publication year: 1993
References
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