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Encyclopedia > Sickle cell anemia
Sickle-shaped red blood cells
Sickle-shaped red blood cells

Sickle cell anemia (American English), sickle cell anaemia (British English) or sickle cell disease is a genetic disease in which red blood cells may change shape under certain circumstances. This causes the cells to become stuck in capillaries which deprives the downstream tissues of oxygen and causes ischemia and infarction. The disease usually occurs in periodic painful attacks, eventually leading to damage of internal organs, stroke, or anemia, and usually resulting in decreased lifespan. It is common in people from countries with a high incidence of malaria, and especially in West Africa. Micrograph of sickled red blood cells, taken from the NIH (US government agency) site at http://www. ... United States English, U.S. English, or American English is the form of the English language used mostly in the United States of America. ... British English is a term primarily used by people outside of the UK to refer to the form of the English language spoken in the British Isles. ... A genetic disorder, or genetic disease is a disease caused, at least in part, by the genes of the person with the disease. ... Human red blood cells Red blood cells are the most common type of blood cell and are the vertebrate bodys principal means of delivering oxygen to body tissues via the blood. ... Capillaries are the smallest of a bodys blood vessels, measuring 5-10 μm. ... General Name, Symbol, Number oxygen, O, 8 Chemical series nonmetals Group, Period, Block 16, 2, p Appearance colorless Atomic mass 15. ... In medicine, ischemia (Greek ισχαιμία, isch- is restriction, hema or haema is blood) is a restriction in blood supply, generally due to factors in the blood vessels, with resultant damage or dysfunction of tissue. ... In medicine, infarction is necrosis of tissue due to upstream obstruction of its arterial blood supply. ... A stroke or cerebrovascular accident (CVA) occurs when the blood supply to a part of the brain is suddenly interrupted by occlusion (an ischemic stroke- approximately 90%of strokes) or by hemorrhage (a hemorrhagic stroke - approximately 10% of strokes). ... Anemia (American English) or anaemia (Commonwealth English), which literally means without blood, is a lack of red blood cells and/or hemoglobin. ... Red blood cell infected with Malaria (Italian: bad air; formerly called ague or marsh fever in English) is an infectious disease which in humans causes about 350-500 million infections and over 1 million deaths annually, mainly in the tropics and sub-Saharan Africa. ... West Africa is the region of western Africa generally considered to include these countries: Benin Burkina Faso Cameroon Côte dIvoire (Ivory Coast) Equatorial Guinea Gabon The Gambia Ghana Guinea Guinea-Bissau Liberia Mali Niger Nigeria Republic of the Congo (Congo-Brazzaville) Senegal Sierra Leone Togo Chad, Mauritania, and...

Contents


Signs and symptoms

Patients have baseline anemia that varies in severity, with hemoglobin levels of 7-9 mg/dl typical. Often white blood cell counts are elevated simply due to marrow hyperactivity. Reticulocyte counts are elevated, reflecting new red blood cells replacing the rapidly cleared older cells - red blood cell life span is markedly reduced in this disease.


Vasoocclusive crises

Vasoocclusive crises are caused by sickled red blood cells that obstruct capillaries and restrict bloodflow to an organ, resulting in ischemia, pain, and organ damage. In medicine, ischemia (Greek ισχαιμία, isch- is restriction, hema or haema is blood) is a restriction in blood supply, generally due to factors in the blood vessels, with resultant damage or dysfunction of tissue. ... According to the International Society for the Study of Pain, there are two different terms: pain and nociception. ...


Because of its narrow vessels and function in clearing defective red blood cells, the spleen is frequently affected. It is usually infarcted before the end of childhood in individuals suffering from sickle cell anaemia. This autosplenectomy increases the risk of infection from encapsulated organisms; preventive antibiotics & vaccinations are recommended for those with such asplenia. Liver failure may also occur with time. The spleen is a ductless, vertebrate gland that is not necessary for life but is closely associated with the circulatory system, where it functions in the destruction of old red blood cells and removal of other debris from the bloodstream, and also in holding a reservoir of blood. ... In medicine, infarction is necrosis of tissue due to upstream obstruction of its arterial blood supply. ... An autosplenectomy occurs when a disease damages the spleen to such an extect that it is non-functioning and so equivalent to the spleen having been surgically removed (splenectomy). ... Asplenia refers to the absence (a-) of normal spleen function and is associated with some risks. ... The liver is an organ in vertebrates, including humans. ...


Bone is also a common target of vasoocclusive damage, especially when the bone is particularly weight-bearing. Such damage may result in avascular necrosis (especially of the femur) and bone deterioration. The pain experienced by sickle-cell patients is also due to the bone ischemia. to fuck/have sex eg: i boned your mum last night ... Avascular necrosis is a disease resulting from the temporary or permanent loss of the blood supply to the bones. ... Anterior view of the femur The femur or thigh bone is the longest (length), largest (volume) and strongest (mechanical ability to resist deformity) bone of the human body. ...


A recognised type of sickle crisis is the acute chest crisis, a condition characterized by fever, chest pain, and pulmonary infiltrate on chest x-ray. Given that pneumonia and intra-pulmonary sickling can both produce these symptoms, the patient is treated for both conditions. Treatment consists of admission, oxygen, close monitoring, and intravenous antibiotics.


Other sickle cell crises

Aplastic crisis - acute worsening of the patient's baseline anemia producing pallor, tachycardia, and fatigue. This crisis is triggered by parvovirus B19, which directly affects erythropoiesis (production of red blood cells). Reticulocyte counts drop dramatically during the illness and the rapid turnover of red cells leads to the drop in hemoglobin. Most patients can be managed supportively, some need blood transfusion. Parvovirus B19 (B19 virus) was the first human parvovirus to be discovered, by chance in 1975 by the Australian virologist Yvonne Cossart. ... Erythropoiesis is the process by which red blood cells (erythrocytes) are produced. ... Reticulocytes are immature red blood cells, that normally comprise about 1% of the red cells in the human body. ...


Splenic sequestration crisis - acute enlargement of the spleen causing pain. Management is supportive, sometimes with blood transfusion.


Complications

Sickle cell anaemia can lead to various complications, including:

Penicillin is a β-lactam antibiotic used in the treatment of bacterial infections caused by susceptible, usually Gram-positive, organisms. ... Binomial name Haemophilus influenzae Haemophilus influenzae, formerly called Pfeiffers bacillus, is a non-motile Gram-negative coccobacillus first described in 1892 by Dr. Robert Pfeiffer during the influenza pandemic. ... Binomial name Streptococcus pneumoniae Streptococcus pneumoniae is a species of Streptococcus that is a major human pathogen. ... Neisseria is a genus of bacteria, included among the proteobacteria, a large group of gram-negative forms. ... A stroke or cerebrovascular accident (CVA) occurs when the blood supply to a part of the brain is suddenly interrupted by occlusion (an ischemic stroke- approximately 90% of strokes), by hemorrhage (a hemorrhagic stroke - less than 10% of strokes) or other causes. ... In the anatomy of animals, the brain, or encephalon, is the supervisory center of the nervous system. ... In medicine, gallstones are crystalline bodies formed within the body by accretion or concretion of normal or abnormal bile components. ... Cholecystitis is inflammation of the gallbladder. ... Hemolysis (alternative spelling haemolysis) is the excessive breakdown of red blood cells. ... Bilirubin is a yellow breakdown product of haem (heme in American English). ... In medicine, gallstones are crystalline bodies formed within the body by accretion or concretion of normal or abnormal bile components. ... Aseptic bone necrosis (ABN), also called avascular necrosis or osteonecrosis, is where bone and marrow die in the absence of an infective agent. ... The immune system is the organ system that protects an organism from outside biological influences. ... Asplenia refers to the absence (a-) of normal spleen function and is associated with some risks. ... Priapism is a painful and potentially harmful medical condition in which the erect penis (erection) does not return to its flaccid state (despite the absence of both physical and psychological stimulation). ... In medicine, infarction is necrosis of tissue due to upstream obstruction of its arterial blood supply. ... Species Salmonella bongori Salmonella choleraesuis Salmonella enterica Salmonella enteritidis Salmonella nyanza Salmonella paratyphi Salmonella typhi Salmonella typhimurium Salmonella virginia Salmonella is a genus of rod-shaped Gram-negative enterobacteria that causes typhoid fever, paratyphoid and foodborne illness. ... An opioid is any agent that binds to opioid receptors found principally in the central nervous system and gastrointestinal tract. ...

Diagnosis

Attacks are diagnosed clinically, i.e. there is no gold standard diagnostic test. Hemolysis (anemia and jaundice) is often present, although for painful crises the diagnosis depends essentially on how the patient describes the pain. A gold standard is the best test to determine whether something exists or not. ... Hemolysis (alternative spelling haemolysis) is the excessive breakdown of red blood cells. ... Anemia (American English) or anaemia (Commonwealth English), which literally means without blood, is a lack of red blood cells and/or hemoglobin. ... Jaundice, technically known as icterus, is yellowing of the skin, sclera (the white of the eyes) and mucous membranes caused by increased levels of bilirubin in the system. ...


Abnormal hemoglobin forms are detected on hemoglobin electrophoresis, a form of gel electrophoresis on which the various types of hemoglobin move at varying speed. Sickle cell hemoglobin (HbSS) and Hemoglobin C with sickling (HbSC)--the two most common forms--can be identified from there. Genetic testing is rarely performed. 3-dimensional structure of hemoglobin Hemoglobin or haemoglobin is the iron-containing oxygen-transport metalloprotein in the red cells of the blood in mammals and other animals. ... SDS-PAGE autoradiography DNA agarose gel Gel electrophoresis is a group of techniques used by scientists to separate molecules based on physical characteristics such as size, shape, or isoelectric point. ... Genetic testing allows the genetic diagnosis of vulnerabilities to inherited diseases, and can also be used to determine a persons ancestry. ...


Pathophysiology

Sickle cell anemia is caused by a mutation in the β-globin chain of hemoglobin, replacing glutamic acid with less polar valine at the sixth position of the β chain . The association of two wild type α-globin subunits with two mutant β-globin subunits forms hemoglobin S, which polymerises under low oxygen conditions causing distortion of red blood cells and a tendency for them to lose their elasticity. Mutations are permanent, sometimes transmissible (if the change is to a germ cell) changes to the genetic material (usually DNA or RNA) of a cell. ... 3-dimensional structure of hemoglobin Hemoglobin or haemoglobin is the iron-containing oxygen-transport metalloprotein in the red cells of the blood in mammals and other animals. ... Glutamic acid or glutamate is one of the 20 most common natural amino acids. ... Valine is one of the 20 most common natural amino acids on Earth, and is coded for in DNA. Nutritionally, valine is also an essential amino acid. ... In biology, a wild type is one of the major genotypes of a species that occur in nature, in contrast to induced mutations or artificial cross-breeding. ... General Name, Symbol, Number oxygen, O, 8 Chemical series nonmetals Group, Period, Block 16, 2, p Appearance colorless Atomic mass 15. ...


New erythrocytes are quite elastic, which allows the cells to deform to pass through capillaries. Often a cycle occurs because as the cells sickle, they cause a region of low oxygen concentration which causes more red blood cells to sickle. Repeated episodes of sickling causes loss of this elasticity and the cells fail to return to normal shape when oxygen concentration increases. These rigid red blood cells are unable to flow through narrow capillaries, causing vessel occlusion and ischemia. Human red blood cells Red blood cells are the most common type of blood cell and are the vertebrate bodys principal means of delivering oxygen to body tissues via the blood. ... In medicine, ischemia (Greek ισχαιμία, isch- is restriction, hema or haema is blood) is a restriction in blood supply, generally due to factors in the blood vessels, with resultant damage or dysfunction of tissue. ...


Genetics

The allele responsible for sickle cell anaemia is autosomal recessive. A person who receives the defective gene from both father and mother develops the disease; a person who receives one defective and one healthy allele remains healthy, but can pass on the disease and is known as a carrier. If two parents who are carriers have a child, there is a 1-in-4 chance of their child developing the illness and a 1-in-2 chance of their child just being a carrier. An allele is any one of a number of alternative forms of the same gene occupying a given locus (position) on a chromosome. ... In genetics, the term recessive gene refers to an allele that causes a phenotype (visible or detectable characteristic) that is only seen in a homozygous genotype (an organism that has two copies of the same allele). ... An asymptomatic carrier (or carrier), is a person who is infected with the agent of an infectious disease, or carries the abnormal gene of a recessive genetic disorder. ...

The gene defect is a known mutation of a single nucleotide (U to A) of the β-globin gene, which results in glutamic acid to be substituted for valine at position 6. Hemoglobins with this mutation are referred to as HbS, as opposed to the more normal adult HbA. The genetic disorder is due to the mutation of a single nucleotide, to a GAG to GTG codon mutation. This is normally a benign mutation, causing no apparent effects on the secondary, tertiary, or quaternary structure of hemoglobin. What it does allow for, under conditions of low oxygen concentration, is the polymerization of the HbS itself. In people heterozygous for HbS (carriers of sickling hemoglobin), the polymerization problems are minor. In people homozygous for HbS, the presence of long chain polymers of HbS distort the shape of the red blood cell, from a smooth doughnut-like shape to ragged and full of spikes, making it fragile and susceptible to breaking within capillaries. Carriers only have symptoms if they are deprived of oxygen (for example, while climbing a mountain) or while severely dehydrated. For those afflicted with the disease, however, vasocclusive events can be a painful part of life (normally they occur 0.8 times per year per patient). The occurs when HbS becomes deoxygenated it undergoes an abnormal change in shape and consistency which can lead to a change in the shape and plasticity in the cell well of the RBC, an occurrence called sickling. Diagram showing how recessive genes are transmitted and expressed. ... Mutations are permanent, sometimes transmissible (if the change is to a germ cell) changes to the genetic material (usually DNA or RNA) of a cell. ... A nucleotide is a monomer or the structural unit of nucleotide chains forming nucleic acids as RNA and DNA. A nucleotide consists of a heterocyclic nucleobase, a pentose sugar, and a phosphate or polyphosphate group. ... Glutamic acid or glutamate is one of the 20 most common natural amino acids. ... Valine is one of the 20 most common natural amino acids on Earth, and is coded for in DNA. Nutritionally, valine is also an essential amino acid. ... 3-dimensional structure of hemoglobin Hemoglobin or haemoglobin is the iron-containing oxygen-transport metalloprotein in the red cells of the blood in mammals and other animals. ... Mutations are permanent, sometimes transmissible (if the change is to a germ cell) changes to the genetic material (usually DNA or RNA) of a cell. ... A nucleotide is a monomer or the structural unit of nucleotide chains forming nucleic acids as RNA and DNA. A nucleotide consists of a heterocyclic nucleobase, a pentose sugar, and a phosphate or polyphosphate group. ... RNA codons. ... Mutations are permanent, sometimes transmissible (if the change is to a germ cell) changes to the genetic material (usually DNA or RNA) of a cell. ... A representation of the 3D structure of the Myoglobin protein. ... In biochemistry, the tertiary structure of a protein is its overall shape. ... In biochemistry, many proteins are actually assemblies of more than one protein molecule, which in the context of the larger assemblage are known as protein subunits. ... General Name, Symbol, Number oxygen, O, 8 Chemical series nonmetals Group, Period, Block 16, 2, p Appearance colorless Atomic mass 15. ... Polymerization is the formation of long, repeating organic polymer chains. ... Heterozygote cells are diploid or polyploid and have different alleles at a locus (position) on homologous chromosomes. ... Homozygote cells are diploid or polyploid and have the same alleles at a locus (position) on homologous chromosomes. ... Glazed doughnuts A doughnut, or donut, is a deep-fried piece of dough or batter. ... Capillaries are the smallest of a bodys blood vessels, measuring 5-10 μm. ... Dehydration is the removal of water (hydor in ancient Greek) from an object. ...


The sufferers of the illness have a reduced life span. It is believed that carriers (sickle cell trait) are relatively resistant to malaria. Since the gene is incompletely recessive, carriers have a few sickle red blood cells at all times, not enough to cause symptoms, but enough to give resistance to malaria. Because of this, heterozygotes have a higher fitness than either of the homozyogotes. This is known as heterozygote advantage. Red blood cell infected with Malaria (Italian: bad air; formerly called ague or marsh fever in English) is an infectious disease which in humans causes about 350-500 million infections and over 1 million deaths annually, mainly in the tropics and sub-Saharan Africa. ... Fitness in biology refers to individuals ability to propagate its genes. ... A heterozygote advantage (heterozygous advantage or overdominance) describes the case in which in which the heterozygote genotype has a higher relative fitness than either the homozygote dominant or homozygote recessive genotype. ...


The malaria parasite has a complex life cycle and spends part of it in red blood cells. In a carrier, the presence of the malaria parasite causes the red blood cell to rupture, making the plasmodium unable to reproduce. Further, the polymerization of Hb affects the ability of the parasite to digest Hb in the first place. Therefore, in areas where malaria is a problem, people's chances of survival actually increase if they carry sickle cell anaemia.


Due to the above phenomenon, the illness is still prevalent, especially among people with recent ancestry in malaria-striken areas, such as Africa, the Mediterranean, India and the Middle East. In fact, sickle-cell anaemia is the most common genetic disorder among African Americans; about 1 in every 12 is a carrier. Africa is the worlds second-largest continent in both area and population, after Asia. ... The Mediterranean Sea is an intercontinental sea positioned between Europe to the north, Africa to the south and Asia to the east, covering an approximate area of 2. ... A map showing countries commonly considered to be part of the Middle East The Middle East is a region comprising the lands around the southern and eastern parts of the Mediterranean Sea, a territory that extends from the eastern Mediterranean Sea to the Persian Gulf. ...


The evolution of sickle-cell anaemia is probably an example of Baldwinian evolution, whereby humans modify their environment and thus change the selective pressures. As humans in tropical areas in Africa and elsewhere developed agriculture and animal husbandry, they expanded the niche for Anopheles mosquitoes that could transmit the malaria parasite. James Mark Baldwin (Columbia, South Carolina, 1861—1934) was an American philosopher, educated at Princeton and several German universities. ... Some Species Anopheles beklemishevi Anopheles coustani Anopheles crypticus Anopheles farauti Anopheles forattinii Anopheles funestus Anopheles gambiae Anopheles grabhamii Anopheles hailarensis Anopheles halophylus Anopheles hyrcanus Anopheles kosiensis Anopheles maculipennis Anopheles minimus Anopheles moucheti Anopheles nili Anopheles ovengensis Anopheles pampanae Anopheles peytoni Anopheles quadrimaculatus Anopheles rennellensis Anopheles rivulorum Anopheles triannulatus Anopheles is...


It is interesting that in the USA, where there is no endemic malaria, the incident of sickle cell anaemia amongst people of African descent is much lower than in West Africa and falling. Without endemic malaria from Africa, the condition is purely disadvantageous, and will tend to be bred out of the affected population. See the Price equation article for a simplified mathematical model of the genetic evolution of sickle cell anemia. African Americans, also known as Afro-Americans or black Americans, are an ethnic group in the United States of America whose ancestors, usually in predominant part, were indigenous to Sub-Saharan and West Africa. ... West Africa is the region of western Africa generally considered to include these countries: Benin Burkina Faso Cameroon Côte dIvoire (Ivory Coast) Equatorial Guinea Gabon The Gambia Ghana Guinea Guinea-Bissau Liberia Mali Niger Nigeria Republic of the Congo (Congo-Brazzaville) Senegal Sierra Leone Togo Chad, Mauritania, and... The Price equation (also known as Prices equation) is a covariance equation which is a mathematical description of evolution and natural selection. ...


Treatment

Febrile illness

Children with fever are screened for bacteremia - complete blood count, reticulocyte count and blood culture. Younger children (varies from center to center) are admitted for intravenous antibiotics while older children with reassuring white cell counts are managed at home with oral antibiotics. Children with previous bacteremic episodes should be admitted. Bacteremia is the presence of bacteria in the blood, and is the means by which local infections spread hematogenously to distant organs. ...


Painful (vaso-occlusive) crises

While a small subset of patients have frequent pain crises, most require less than one acute care visit per year. Painful crises are treated symptomatically with analgesics; a subgroup of patients manages on NSAIDs (such as diclofenac or naproxen), but many require opioid administration at regular intervals until the crisis has settled. Morphine, fentanyl, diamorphine (heroin - not available in the US) are used commonly; this has unfortunately led to a high rate of opioid addiction amongst sickle-cell patients, although this issues is much less common in children and adolescents. Some patients require inpatient management for intravenous opioids; patient-controlled analgesia (PCA) devices are commonly used in this setting. Diphenhydramine is effective for the itching associated with the opioid use. An analgesic (colloquially known as painkiller) is any member of the diverse group of drugs used to relieve pain. ... Non-steroidal anti-inflammatory drugs, usually abbreviated to NSAIDs, are drugs with analgesic, antipyretic and anti-inflammatory effects - they reduce pain, fever and inflammation. ... Diclofenac (marketed as Voltaren®, Voltarol® and Cataflam®) is a non-steroidal anti-inflammatory drug (NSAID) taken to reduce inflammation, such as in arthritis or acute injury. ... Naproxen (trade names: Aleve, Anaprox, Naprosyn, Naprelan) is a non-steroidal anti-inflammatory drug (NSAID) commonly used for the reduction of mild to moderate pain, fever, inflammation and stiffness caused by conditions such as osteoarthritis, rheumatoid arthritis, psoriatic arthritis, gout, ankylosing spondylitis, injury, menstrual cramps, tendinitis, bursitis, and the treatment... An opioid is any agent that binds to opioid receptors found principally in the central nervous system and gastrointestinal tract. ... Morphine (INN), the principal active agent in opium, is a powerful opioid analgesic drug. ... Fentanyl is an opioid analgesic, first synthesized in Belgium in the late 1950s, with an analgesic potency of about 80 times that of morphine. ... Heroin or diamorphine (INN) (colloquially referred to as junk, babania, horse, golden brown, smack, black tar, H, big H, lady H, dope, skag, juice, diesel, etc. ... Patient-controlled analgesia (PCA) is any method of allowing a person in pain to adminster their own pain relief. ... Diphenhydramine hydrochloride (trade name Benadryl®, or Dimedrol outside the US) is an over-the-counter (OTC) antihistamine and sedative. ...


Acute chest crisis

Management is similar to general painful crises with the addition of antibiotics (usually a third generation cephalosporin), oxygen supplementation for hypoxia, and close observation. Should the pulmonary infiltrate worsens or the oxygen requirements increase, simple blood transfusion or exchange transfusion is indicated. The latter involve the exchange of a significant portion of the patients red cell mass for normal red cells, which decreases the percent hemoglobin S in the patient's blood. The cephalosporins, are a class of ß-lactam antibiotics. ... Hypoxia has several meanings: Hypoxia is the lack of oxygen in tissues, see Hypoxia (medical) Hypoxia is the lack of oxygen in a water body leading to the death of organisms, see Hypoxia (water) This is a disambiguation page — a navigational aid which lists other pages that might otherwise share...


Hydroxyurea

The first approved drug for the causative treatment of sickle cell anaemia, hydroxyurea, was shown to decrease the number and severeness of attacks in a study in 1995 (Charache et al) and shown to increase survival time in a study in 2003. This is achieved by reactivating fetal hemoglobin production in place of the hemoglobin S that causes sickle cell anaemia. Hydroxyurea had previously been used as a chemotherapy agent, and there is some concern that long-term use may be harmful, but it is likely that the benefits outweigh the risks. Hydroxyurea or hydroxycarbamide (brand names include Hydrea®) is an antineoplastic drug used in hematological malignancies. ... 1995 was a common year starting on Sunday of the Gregorian calendar. ... 2003 is a common year starting on Wednesday of the Gregorian calendar. ... Fetal hemoglobin protein structure Fetal hemoglobin (also hemoglobin F or HbF) is the main oxygen transport protein in the fetus during the last seven months of development in the womb. ... Chemotherapy is the use of chemical substances to treat disease. ...


Situation of carriers

People who are known carriers of the disease often undergo genetic counseling before they have a child. A test to see if an unborn child has the disease takes either a blood sample from the unborn or a sample of amniotic fluid. Since taking a blood sample from a fetus has risks, the latter test is usually used. Genetic counseling generally refers to prenatal counseling done when a genetic condition is suspected in a pregnancy. ... Red blood cells (erythrocytes) are present in the blood and help carry oxygen to the rest of the cells in the body Blood is a circulating tissue composed of fluid plasma and cells (red blood cells, white blood cells, platelets). ... The amniotic sac is a tough but thin transparent pair of membranes which holds a developing embryo (and later fetus) until shortly before birth. ...


After the mutation responsible for this disease was discovered in 1979, the U.S. Air Force required African American applicants to test for the mutation. It dismissed 143 applicants because they were carriers, even though none of them had the condition. It eventually withdrew the requirement, but only after a trainee filed a lawsuit. Now, some insurance companies are doing the same thing to eliminate, in their terminology, "unwise investments". 1979 is a common year starting on Monday. ... Seal of the Air Force. ...


History

The cause of this collection of clinical findings was unknown until the description of the sickle cells in 1910 by the Chicago cardiologist and professor of medicine James B. Herrick (1861-1954) whose intern Ernest Edward Irons (1877-1959) found "peculiar elongated and sickle shaped" cells in the blood of Walter Clement Noel, a 20 year old first year dental student from Grenada after Noel was admitted to the Presbyterian Hospital in December 1904 suffering from anemia. Noel was readmitted several times over the next three years for "muscular rheumatism" and "bilious attacks" while an undergraduate. Noel completed his studies and returned to capital of Grenada (St. George's) to practice dentistry. He died of pneumonia in 1916 and is buried in the Catholic cemetery at Sauteurs in the north of Grenada. 1910 was a common year starting on Saturday (see link for calendar). ... Chicago (officially named the City of Chicago) is the third largest city in the United States (after New York City and Los Angeles), with an official population of 2,896,016, as of the 2000 census. ... Dr James Bryan Herrick (August 11, 1861 - March 7, 1954) was as American doctor who is credited with the description of several important phenomena in 20th century medicine. ... 1861 is a common year starting on Tuesday. ... 1954 was a common year starting on Friday of the Gregorian calendar. ... 1877 was a common year starting on Monday (see link for calendar). ... 1959 was a common year starting on Thursday of the Gregorian calendar. ... Red blood cells (erythrocytes) are present in the blood and help carry oxygen to the rest of the cells in the body Blood is a circulating tissue composed of fluid plasma and cells (red blood cells, white blood cells, platelets). ... New York-Presbyterian Hospital is a prominent university hospital in New York City, composed of two medical centers, Columbia University Medical Center and New York Weill Cornell Medical Center, each affiliated with an Ivy League University. ... 1904 is a leap year starting on a Friday (link will take you to calendar). ... Anemia (American English) or anaemia (Commonwealth English), which literally means without blood, is a lack of red blood cells and/or hemoglobin. ... Pneumonia (the ancient Greek word for lungs) is defined as an inflamation, usually caused by infection, involving the alveoli of the lungs. ... 1916 is a leap year starting on Saturday (link will take you to calendar) // Events January-February January 1 -The first successful blood transfusion using blood that had been stored and cooled. ...


The disease is very occasionally called "Herrick's syndrome" for this reason.


The disease was named "sickle cell anemia" by Vernon Mason in 1922. In retrospect some elements of the disease had been recognized earlier: a paper in the Southern Journal of Medical Pharmacology in 1846 described the absence of a spleen in the autopsy of a runaway slave. The African medical literature reported this condition in the 1870's where it was known locally as ogbanjes ('children who come and go') because of the very high infant mortality in this condition. Also, the practice of using tar soap to cover blemishes caused by sickle cell sores was prevalent in the African American community. 1922 was a common year starting on Sunday (see link for calendar). ... An autopsy (also known as a post-mortem examination or necropsy) is a medical investigation of a corpse to determine the cause of death. ...


Related topics

Thalassemia (American English) (or Thalassaemia in British English) is an inherited disease of the red blood cells, classified as a hemoglobinopathy. ... For other non-genetic uses of the term dominance, see Dominance. ... Genetic counseling generally refers to prenatal counseling done when a genetic condition is suspected in a pregnancy. ...

References

  • Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, Orringer E, Bellevue R, Olivieri N, Eckman J, Varma M, Ramirez G, Adler B, Smith W, Carlos T, Ataga K, DeCastro L, Bigelow C, Saunthararajah Y, Telfer M, Vichinsky E, Claster S, Shurin S, Bridges K, Waclawiw M, Bonds D, Terrin M. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995;332:1317-22. PMID 12672732.
  • Savitt TL, Goldberg MF. Herrick's 1910 case report of sickle cell anemia. The rest of the story. JAMA. 1989;261:266-271. PMID 2642320.

The New England Journal of Medicine (NEJM) is a peer-reviewed medical journal published by the Massachusetts Medical Society. ...

External links


Health science - Medicine - Hematology
Hematological malignancy and White blood cells
Leukemia (ALL, AML, CLL, CML) - Lymphoma (Hodgkin's disease, NHL) - Multiple myeloma - MDS - Myelofibrosis - Myeloproliferative disease (Essential thrombocytosis, Polycythemia) - Neutropenia
Red blood cells
Anemia - Hemochromatosis - Sickle-cell anemia - Thalassemia - G6PD - other hemoglobinopathies
Coagulation and Platelets
Thrombosis - Deep venous thrombosis - Pulmonary embolism - Hemophilia - ITP - TTP

  Results from FactBites:
 
What Is Sickle Cell Anemia? (415 words)
Sickle cell anemia is a serious disease in which the body makes abnormally shaped red blood cells.
Sickle cell anemia is an inherited (genetic) disorder.
Anemia is the term for having a shortage of red blood cells in your blood.
Sickle Cell Anemia (1687 words)
Sickle cell disease is an inherited blood disorder characterized by defective hemoglobin.
Sickle cell hemoglobin molecules are stiff and form into the shape of a sickle or a scythe.
Sickle cell disease is an inherited disease caused by a genetic mutation.
  More results at FactBites »

 
 

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