query: How is thalassemia defined?

title: Thalassemia

text: Thalassemia Thalassemias are inherited blood disorders characterized by abnormal hemoglobin production. Symptoms depend on the type and can vary from none to severe. Often there is mild to severe anemia (low red blood cells). Anemia can result in feeling tired and pale skin. There may also be bone problems, an enlarged spleen, yellowish skin, and dark urine. Slow growth may occur in children. Thalassemias are genetic disorders inherited from a person's parents. There are two main types, alpha thalassemia and beta thalassemia. The severity of alpha and beta thalassemia depends on how many of the four genes for alpha globin

title: Thalassemia

text: in favor of voluntary premarital screening to detect carriers of thalassemia and marriage between both carriers are strongly discouraged. Mild thalassemia: people with thalassemia traits do not require medical or follow-up care after the initial diagnosis is made. People with β-thalassemia trait should be warned that their condition can be misdiagnosed as the more common iron deficiency anemia. They should avoid routine use of iron supplements; iron deficiency can develop, though, during pregnancy or from chronic bleeding. Counseling is indicated in all persons with genetic disorders, especially when the family is at risk of a severe form of disease that

title: Thalassemia

text: transcription. Efforts involve trying to disrupt the fetal globin gene promoter. Thalassemia Thalassemias are inherited blood disorders characterized by abnormal hemoglobin production. Symptoms depend on the type and can vary from none to severe. Often there is mild to severe anemia (low red blood cells). Anemia can result in feeling tired and pale skin. There may also be bone problems, an enlarged spleen, yellowish skin, and dark urine. Slow growth may occur in children. Thalassemias are genetic disorders inherited from a person's parents. There are two main types, alpha thalassemia and beta thalassemia. The severity of alpha and beta thalassemia

title: Sickle cell-beta thalassemia

text: Sickle cell-beta thalassemia "Sickle cell-beta thalassemia" (Sickle cell-β thalassemia) is an inherited blood disorder. The disease may range in severity from being relatively benign and like sickle cell trait to being similar to sickle cell disease. Patients with sickle cell-beta thalassemia may present with painful crises similar to patients with sickle cell disease Sickle cell-beta thalassemia is caused by inheritance of a sickle cell allele from one parent and a beta thalassemia allele from the other. A sickle allele is always the same mutation of the beta-globin gene (glutamic acid to valine at amino acid six). In contrast, beta-thalassemia alleles

title: Sickle cell-beta thalassemia

text: and splenectomy is frequently performed. Vaccination against encapsulated bacteria including Streptococcus pneumoniae is recommended. Sickle cell-beta thalassemia "Sickle cell-beta thalassemia" (Sickle cell-β thalassemia) is an inherited blood disorder. The disease may range in severity from being relatively benign and like sickle cell trait to being similar to sickle cell disease. Patients with sickle cell-beta thalassemia may present with painful crises similar to patients with sickle cell disease Sickle cell-beta thalassemia is caused by inheritance of a sickle cell allele from one parent and a beta thalassemia allele from the other. A sickle allele is always the same mutation of the

title: Alpha thalassemia abnormal morphogenesis

text: normal hemoglobin, these variants tend to cause few or no health problems. A loss of two of the four alpha-globin alleles results in alpha thalassemia trait. People with alpha thalassemia trait may have unusually small, pale red blood cells and mild anemia. A loss of one alpha-globin allele is found in alpha thalassemia silent carriers. These individuals typically live a life with no thalassemia related signs or symptoms. Alpha thalassemia is a faintly common blood disorder worldwide. Thousands of infants with Hb Bart syndrome and HbH disease are born each year, particularly in Southeast Asia. Alpha thalassemia also occurs frequently

title: Beta thalassemia

text: patient age is 23 with only 1% of consultants the patient is older than 75 and 69% were 15-59 year olds. The Children’s Hospital Oakland formed an international network to combat thalassemia. “It is the world’s most common genetic blood disorder and is rapidly increasing”. 7% of the world’s population are carriers and 400,000 babies are born with the trait annually. It is usually fatal in infancy if blood transfusions are not initiated immediately. Beta thalassemia Beta thalassemias (β thalassemias) are a group of inherited blood disorders. They are forms of thalassemia caused by reduced or absent synthesis of the

text: Alpha thalassemia abnormal morphogenesis Alpha Thalassemia-Abnormal Morphogensis is a group of blood disorders that affect the way the body makes hemoglobin. Hemoglobin is a protein found in red blood cells that carries oxygen throughout the body. It's made up of alpha globin and beta globin. Normally each person has four genes for alpha globin, Alpha thalassemia happens when one or more of the genes that control the making of alpha globin is absent or defective. It can cause anemia ranging from mild to severe. Also causing Genital abnormalities and terminal transverse limb defectiveness which is why it is called abnormal

text: blood pressure with swelling (preeclampsia), premature delivery, and abnormal bleeding. Alpha thalassemia abnormal morphogenesis Alpha Thalassemia-Abnormal Morphogensis is a group of blood disorders that affect the way the body makes hemoglobin. Hemoglobin is a protein found in red blood cells that carries oxygen throughout the body. It's made up of alpha globin and beta globin. Normally each person has four genes for alpha globin, Alpha thalassemia happens when one or more of the genes that control the making of alpha globin is absent or defective. It can cause anemia ranging from mild to severe. Also causing Genital abnormalities and terminal

title: Beta thalassemia

text: survival advantage on carriers (known as heterozygous advantage), thus perpetuating the mutation. In that respect, the various thalassemias resemble another genetic disorder affecting hemoglobin, sickle-cell disease. The disorder affects all genders but is more prevalent in certain ethnicities and age groups. 20 people die per year causing thalassemia to be listed as a “rare disease”. In the United States, thalassemia’s prevalence is approximately 1 in 272,000 or 1,000 people. There have been 4,000 hospitalized cases in England in 2002 and 9,233 consultant episodes for thalassemia. Men accounted for 53% of hospital consultant episodes and women accounted for 47%. The mean

title: Management of thalassemia

text: very young people. Management of thalassemia Treatment of the inherited blood disorder thalassemia depends upon the level of severity. For mild forms of the condition, advice and counseling are often all that are necessary. For more severe forms, treatment may consist in blood transfusion; chelation therapy to reverse iron overload, using drugs such as deferoxamine, deferiprone, or deferasirox; medication with the antioxidant indicaxanthin to prevent the breakdown of hemoglobin; or a bone marrow transplant using material from a compatible donor, or from the patient's mother. Population screening has had some success as a preventive measure. Patients with thalassemia gradually accumulate

title: Management of thalassemia

text: Management of thalassemia Treatment of the inherited blood disorder thalassemia depends upon the level of severity. For mild forms of the condition, advice and counseling are often all that are necessary. For more severe forms, treatment may consist in blood transfusion; chelation therapy to reverse iron overload, using drugs such as deferoxamine, deferiprone, or deferasirox; medication with the antioxidant indicaxanthin to prevent the breakdown of hemoglobin; or a bone marrow transplant using material from a compatible donor, or from the patient's mother. Population screening has had some success as a preventive measure. Patients with thalassemia gradually accumulate high levels of

title: Beta thalassemia

text: Beta thalassemia Beta thalassemias (β thalassemias) are a group of inherited blood disorders. They are forms of thalassemia caused by reduced or absent synthesis of the beta chains of hemoglobin that result in variable outcomes ranging from severe anemia to clinically asymptomatic individuals. Global annual incidence is estimated at one in 100,000. Beta thalassemias are caused by mutations in the "HBB" gene on chromosome 11, inherited in an autosomal recessive fashion. The severity of the disease depends on the nature of the mutation. HBB blockage over time leads to decreased beta-chain synthesis. The body's inability to construct new beta-chains leads

title: Alpha-thalassemia

text: Alpha-thalassemia Alpha-thalassemia (α-thalassemia, α-thalassaemia) is a form of thalassemia involving the genes "HBA1" and "HBA2". Alpha-thalassemia is due to impaired production of alpha chains from 1, 2, 3, or all 4 of the alpha globin genes, leading to a relative excess of beta globin chains. The degree of impairment is based on which clinical phenotype is present (how many genes are affected). The presentation of individuals with alpha-thalassemia consists of: Alpha-thalassemias are most commonly inherited in a Mendelian recessive manner. They are also associated with deletions of chromosome 16p. Alpha thalassemia can also be acquired under rare circumstances. The mechanism

title: Alpha-thalassemia

text: are carriers of beta-thalassaemia genes, while 10% of the population carry alpha-thalassaemia genes. Alpha-thalassemia Alpha-thalassemia (α-thalassemia, α-thalassaemia) is a form of thalassemia involving the genes "HBA1" and "HBA2". Alpha-thalassemia is due to impaired production of alpha chains from 1, 2, 3, or all 4 of the alpha globin genes, leading to a relative excess of beta globin chains. The degree of impairment is based on which clinical phenotype is present (how many genes are affected). The presentation of individuals with alpha-thalassemia consists of: Alpha-thalassemias are most commonly inherited in a Mendelian recessive manner. They are also associated with deletions of

title: Beta thalassemia

text: and thalassemia minor. All people with thalassemia are susceptible to health complications that involve the spleen (which is often enlarged and frequently removed) and gallstones. These complications are mostly found in thalassemia major and intermedia patients. Individuals with beta thalassemia major usually present within the first two years of life with severe anemia, poor growth, and skeletal abnormalities during infancy. Untreated thalassemia major eventually leads to death, usually by heart failure; therefore, birth screening is very important. Excess iron causes serious complications within the liver, heart, and endocrine glands. Severe symptoms include liver cirrhosis, liver fibrosis, and in extreme cases,

title: Delta-beta thalassemia

text: diagnosis of beta thalassemia trait. In beta thalassemia, an increase in hemoglobin A2 results. However, the co-existence of a delta-beta thalassemia mutation will decrease the value of the hemoglobin A2 into the normal range, thereby obscuring the diagnosis of beta thalassemia trait The diagnosis of delta-beta thalassemia is done via hypochromic microcytic red cell indices. This test is a part of a CBC, and could be employed to diagnose the reason the individual might have anemia, in this case due to thalassemia. In terms of treatment for delta-beta thalassemia one possible concern would be anemia, where, therefore, blood transfusions would

title: Delta-beta thalassemia

text: Delta-beta thalassemia Delta-beta thalassemia is a form of thalassemia, and is autosomal recessive in terms of heredity. It is associated with "hemoglobin subunit delta." An individual with delta-beta thalassemia is usually asymptomatic, however microcytosis can occur where the red blood cells are abnormally small. In regards to genetics, delta-beta thalassemia is autosomal recessive, which means both parents (two copies of the gene) must be present. A carrier gets a normal gene to produce hemoglobin A, from one parent and the other parent supplies a gene which makes no hemoglobin A. Delta-beta thalassemia is considered rare. In terms of the pathophysiology

title: Beta thalassemia

text: or grandparents suffered from beta thalassemia there is a high probability of the mutated gene being inherited by an offspring. Even if a child does not have beta thalassemia major, they can still be a carrier resulting in future offspring having beta thalassemia. Another risk factor is because of certain ancestry. Beta thalassemia occurs most often in people of Italian, Greek, Middle Eastern, Southern Asian, and African ancestry. Abdominal pain due to hypersplenism and splenic infarction and right-upper quadrant pain caused by gallstones are major clinical manifestations. However, diagnosing thalassemiæ from symptoms alone is inadequate. Physicians note these signs as

title: Thalassemia

text: β-thalassemia have some protection against coronary heart disease. Normally, the majority of adult hemoglobin (HbA) is composed of four protein chains, two α and two β globin chains arranged into a heterotetramer. In thalassemia, patients have defects in either the α or β globin chain, causing production of abnormal red blood cells (In sickle-cell disease, the mutation is specific to β globin). The thalassemias are classified according to which chain of the hemoglobin molecule is affected. In α-thalassemias, production of the α globin chain is affected, while in β-thalassemia, production of the β globin chain is affected. The β globin

title: Thalassemia

text: poorer populations only have access to limited diagnostic facilities together with blood transfusions. In some developing countries, there are virtually no facilities for diagnosis or management of thalassemia. The word "thalassemia" () derives from the Greek " thalassa" (θάλασσα), "sea", and New Latin "-emia" (from Greek "haema" (αἷμα), "blood"). It was coined because the condition called "Mediterranean anemia" was first described in people of Mediterranean ethnicities. "Mediterranean anemia" was renamed thalassemia major once the genetics were better understood. The word "thalassemia" was first used in 1932. In 2008, in Spain, a baby was selectively implanted to be a cure for

title: Thalassemia

text: other hemoglobinopathies. The most common of these are: Thalassemia can be diagnosed via a complete blood count, hemoglobin electrophoresis, and DNA testing. The American College of Obstetricians and Gynecologists recommends all people thinking of becoming pregnant be tested to see if they have thalassemia. Genetic counseling and genetic testing are recommended for families who carry a thalassemia trait. A screening policy exists in Cyprus to reduce the rate of thalassemia, which, since the program's implementation in the 1970s (which also includes prenatal screening and abortion), has reduced the number of children born with the disease from one of every 158

title: Delta-beta thalassemia

text: be given to the affected individual (though blood transfusions might introduce complications, as well). Stem cell transplant is another option, but the donor and the individual who will receive the bone marrow transplant must be compatible, the risks involved should be evaluated, as well Delta-beta thalassemia Delta-beta thalassemia is a form of thalassemia, and is autosomal recessive in terms of heredity. It is associated with "hemoglobin subunit delta." An individual with delta-beta thalassemia is usually asymptomatic, however microcytosis can occur where the red blood cells are abnormally small. In regards to genetics, delta-beta thalassemia is autosomal recessive, which means both

title: Thalassemia

text: his brother's thalassemia. The child was born from an embryo screened to be free of the disease before implantation with "in vitro" fertilization. The baby's supply of immunologically compatible cord blood was saved for transplantation to his brother. The transplantation was considered successful. In 2009, a group of doctors and specialists in Chennai and Coimbatore registered the successful treatment of thalassemia in a child using an unaffected sibling's umbilical cord blood. Gene therapy is being studied for thalassemia. The procedure involves collecting hematopoietic stem cells (HSCs) from the affected person's blood. The HSCs then have a beta-globin gene added using

title: Thalassemia

text: may be prevented. People with severe thalassemia require medical treatment. A blood transfusion regimen was the first measure effective in prolonging life. Multiple blood transfusions can result in iron overload. The iron overload related to thalassemia may be treated by chelation therapy with the medications deferoxamine, deferiprone, or deferasirox. These treatments have resulted in improving life expectancy in those with thalassemia major. Deferoxamine is only effective via daily injections which makes its long-term use more difficult. It has the benefit of being inexpensive and decent long-term safety. Adverse effects are primary skin reactions around the injection site and hearing loss.

title: Thalassemia

text: thalassemia. Bone marrow transplantation may offer the possibility of a cure in young people who have an HLA-matched donor. Success rates have been in the 80–90% range. Mortality from the procedure is about 3%. There are no randomized controlled trials which have tested the safety and efficacy of non-identical donor bone marrow transplantation in persons with β- thalassemia who are dependent on blood transfusion. If the person does not have an HLA-matched compatible donor, another method called bone marrow transplantation (BMT) from haploidentical mother to child (mismatched donor) may be used. In a study of 31 people, the thalassemia-free survival

text: morphogenesis. This is most commonly found in people of African, Middle Eastern, Chinese, Southeast Asian and occasionally, Mediterranean descent. Thalassemia is an inherited condition. It's carried in the genes and passed on from parents to children. People who are carriers of a thalassemia gene show no thalassemia symptoms and might not even know they are carriers. If both parents are carriers, they can pass the disease to their kids. Thalassemia is not contagious. While there are many different types of thalassemia, the two main are alpha and beta. Beta thalassemia happens when the body has a problem producing beta globin,

title: Beta thalassemia

text: liver cancer. Heart failure, growth impairment, diabetes and osteoporosis are life-threatening contributors brought upon by TM. The main cardiac abnormalities seen to have resulted from thalassemia and iron overload include left ventricular systolic and diastolic dysfunction, pulmonary hypertension, valveulopathies, arrhythmias, and pericarditis. Increased gastrointestinal iron absorption is seen in all grades of beta thalassemia and increased red blood cell destruction by the spleen due to ineffective erythropoiesis further releases additional iron into the bloodstream. Two major groups of mutations can be distinguished: Beta thalassemia is a hereditary disease affecting hemoglobin. As with about half of all hereditary diseases, an inherited

title: Alpha-thalassemia

text: cases found that in utero transfusion can lead to favorable outcomes. Successful hematopoietic cell transplantation was eventually carried out in four patients. Worldwide distribution of inherited alpha-thalassemia corresponds to areas of malaria exposure, suggesting a protective role. Thus, alpha-thalassemia is common in sub-Saharan Africa, the Mediterranean Basin, and generally tropical (and subtropical) regions. The epidemiology of alpha-thalassemia in the US reflects this global distribution pattern. More specifically, HbH disease is seen in Southeast Asia and the Middle East, while Hb Bart hydrops fetalis is acknowledged in Southeast Asia only. The data indicate that 15% of the Greek and Turkish Cypriots

title: Beta thalassemia

text: Further genetic analysis may include HPLC should routine electrophoresis prove difficult. Beta thalassemia is a hereditary disease allowing for a preventative treatment by carrier screening and prenatal diagnosis. It can be prevented if one parent has normal genes, giving rise to screenings that empower carriers to select partners with normal hemoglobin. A study aimed at detecting the genes that could give rise to offspring with sickle cell disease. Patients diagnosed with beta thalassemia have MCH ≤ 26 pg and an RDW < 19. Of 10,148 patients, 1,739 patients had a hemoglobin phenotype and RDW consistent with beta thalassemia. After the

title: Thalassemia

text: and on the presence of mutations in one or both alleles. Mutated alleles are called β when partial function is conserved (either the protein has a reduced function, or it functions normally but is produced in reduced quantity) or β, when no functioning protein is produced. The situation of both alleles determines the clinical picture: As well as alpha and beta chains present in hemoglobin, about 3% of adult hemoglobin is made of alpha and delta chains. Just as with beta thalassemia, mutations that affect the ability of this gene to produce delta chains can occur. Thalassemia can coexist with

title: Thalassemia

text: rate 70%, rejection 23%, and mortality 7%. The best results are with very young people. The beta form of thalassemia is particularly prevalent among Mediterranean peoples, and this geographical association is responsible for its original name. Thalassemia resulted in 25,000 deaths in 2013 down from 36,000 deaths in 1990. In Europe, the highest concentrations of the disease are found in Greece, coastal regions in Turkey (particularly the Aegean Region such as Izmir, Balikesir, Aydin, Mugla, and Mediterranean Region such as Antalya, Adana, Mersin), in parts of Italy, particularly southern Italy and the lower Po valley. The major Mediterranean islands (except

title: Delta-beta thalassemia

text: of delta-beta thalassemia, one finds delta and beta chains have deletion, therefore gamma genes are what is transcribed (made an RNA copy of) on the impaired chromosome. When one inherits two delta mutations, no hemoglobin A2 (alpha2, delta2) can be formed. Hematologically, however, this is innocuous because only 2-3% of normal adult hemoglobin is hemoglobin A2. The individual will have normal hematological parameters (erythrocyte count, total hemoglobin, mean corpuscular volume). The delta-beta thalassemia demonstrates one mutation is at the +69 position. The importance of recognizing the existence of delta-beta thalassemia is seen best in cases where it may mask the

title: Thalassemia

text: may be required. As of 2013, thalassemia occurs in about 280 million people, with about 439,000 having severe disease. It is most common among people of Italian, Greek, Middle Eastern, South Asian, and African descent. Males and females have similar rates of disease. It resulted in 16,800 deaths in 2015, down from 36,000 deaths in 1990. Those who have minor degrees of thalassemia, similar to those with sickle-cell trait, have some protection against malaria, explaining why they are more common in regions of the world where malaria exists. Both α- and β-thalassemias are often inherited in an autosomal recessive manner.

title: Beta thalassemia

text: narrowing of patients, the HbA2 levels were tested presenting 77 patients with beta thalassemia. This screening procedure proved insensitive in populations of West African ancestry because of the indicators has high prevalence of alpha thalassemia. Countries have programs distributing information about the reproductive risks associated with carriers of haemoglobinopathies. Thalassemia carrier screening programs have educational programs in schools, armed forces, and through mass media as well as providing counseling to carriers and carrier couples. Screening has showed reduced incidence; by 1995 the prevalence in Italy reduced from 1:250 to 1:4000, and a 95% decrease in that region. The decrease in

title: Alpha-thalassemia mental retardation syndrome

text: Alpha-thalassemia mental retardation syndrome Alpha-thalassemia mental retardation syndrome (ATRX), also called alpha-thalassemia X-linked mental retardation, nondeletion type or ATR-X syndrome, is a condition caused by a mutated gene. Females with this mutated gene have no specific signs or features, but may demonstrate skewed X chromosome inactivation. Hemizygous males tend to be moderately intellectually disabled and have physical characteristics including coarse facial features, microcephaly (small head size), hypertelorism (widely spaced eyes), a depressed nasal bridge, a tented upper lip, and an everted lower lip. Mild or moderate anemia, associated with alpha-thalassemia, is part of the condition. It is associated with "ATRX".

title: Thalassemia

text: Latin America. Estimates suggest that approximately 1.5% of the global population (80 - 90 million people) are β-thalassemia carriers. However, exact data on carrier rates in many populations are lacking, particularly in developing areas of the world known or expected to be heavily affected. Because of the prevalence of the disease in countries with little knowledge of thalassemia, access to proper treatment and diagnosis can be difficult. While there are some diagnostic and treatment facilities in developing countries, in most cases these are not provided by government services, and are available only to patients that can afford them. In general,

title: Beta thalassemia

text: incidence has benefitted those affected with thalassemia, as the demand for blood has decreased, therefore improving the supply of treatment. Affected children require regular lifelong blood transfusion and can have complications, which may involve the spleen. Bone marrow transplants can be curative for some children. Patients receive frequent blood transfusions that lead to or potentiate iron overload. Iron chelation treatment is necessary to prevent damage to internal organs. Advances in iron chelation treatments allow patients with thalassemia major to live long lives with access to proper treatment. Popular chelators include deferoxamine and deferiprone. The most common patient deferoxamine complaint is

title: Alpha-thalassemia

text: alpha-thalassemia is primarily by laboratory evaluation and molecular diagnosis. Alpha-thalassemia can be mistaken for iron-deficiency anaemia on a full blood count or blood film, as both conditions have a microcytic anaemia. Serum iron and serum ferritin can be used to exclude iron-deficiency anaemia. Two genetic loci exist for α globin, thus four alleles are in diploid cells. Two alleles are maternal and two alleles are paternal in origin. The severity of the α-thalassemias is correlated with the number of affected α-globin; alleles: the greater, the more severe will be the manifestations of the disease. When noting the genotype, an "α"

title: Beta thalassemia

text: feasibly treat both beta-thalassemia and sickle cell disease. The technology is based on delivery of a lentiviral vector carrying both the human β-globin gene and an ankyrin insulator to improve gene transcription and translation, and boost levels of β-globin production. Patients with thalassemia major are more inclined to have a splenectomy. The medical cases of splenectomies have been declining in recent years due to decreased prevalence of hypersplenism in adequately transfused patients. Patients with hypersplenism are inclined to have a lower amount of healthy blood cells in their body than normal and reveal symptoms of anemia. Iron rich patients need

title: Alpha-thalassemia

text: indicates a functional alpha chain, and '-' a pathological one. Treatment for alpha-thalassemia may consist of blood transfusions, and possible splenectomy; additionally, gallstones may be a problem that would require surgery. Secondary complications from febrile episode should be monitored, and most individuals live without any need for treatment Additionally, stem cell transplantation should be considered as a treatment (and cure), which is best done in early age. Other options, such as gene therapy, are still being developed. A study by Kreger et al combining a retrospective review of three cases of alpha thalassemia major and a literature review of 17

text: Xq13.3 and XH2 belongs to the subgroup SNF2 . This group is important for regulating the transcription of the alpha genes . Alpha-thalassemia mental retardation syndrome Alpha-thalassemia mental retardation syndrome (ATRX), also called alpha-thalassemia X-linked mental retardation, nondeletion type or ATR-X syndrome, is a condition caused by a mutated gene. Females with this mutated gene have no specific signs or features, but may demonstrate skewed X chromosome inactivation. Hemizygous males tend to be moderately intellectually disabled and have physical characteristics including coarse facial features, microcephaly (small head size), hypertelorism (widely spaced eyes), a depressed nasal bridge, a tented upper lip,

title: Management of thalassemia

text: in a child using a sibling's umbilical cord blood. It is possible to be cured, by a bone marrow transplantation (BMT) from compatible donor. In low-risk young people, the thalassemia-free survival rate is 87%; the mortality risk is 3%. The drawback is that this method requires an HLA-matched compatible donor. If the person does not have an HLA-matched compatible donor, there is another method called bone marrow transplantation from haploidentical mother to child (mismatched donor), in which the donor is the mother. The results are these: thalassemia-free survival rate 70%, rejection 23%, and mortality 7%. The best results are with

text: in people from Mediterranean countries, North Africa, the Middle East, India, and Central Asia. People with alpha thalassemia abnormal morphogenesis encounter many different types of symptoms and characteristics of carrying and living with this trait. People with the characteristic features of alpha thalassemia experience a reduction in the amount of hemoglobin, that prevents enough oxygen to reach the body’s tissues. Affected individuals also have a shortage of red blood cells (Anemia) causing pale skin, weakness, fatigue, and more serious complications. There are two types of Alpha Thalassemia Abnormal Morphogenesis that can cause more major health problems. The more severe one

title: Beta thalassemia

text: to the underproduction of HbA. Reductions in HbA available overall to fill the red blood cells in turn leads to microcytic anemia. Microcytic anemia ultimately develops in respect to inadequate HBB protein for sufficient red blood cell functioning. Due to this factor, the patient may require blood transfusions to make up for the blockage in the beta-chains. Repeated blood transfusions can lead to build-up of iron overload, ultimately resulting in iron toxicity. This iron toxicity can cause various problems, including myocardial siderosis and heart failure leading to the patient’s death. Three main forms have been described: thalassemia major, thalassemia intermedia,

title: Management of thalassemia

text: to radicals. The mechanism of its function, however, is still unknown. Indicaxanthin has high bioavailability and minimal side effects, like vomiting or diarrhea. In 2008, in Spain, a baby was selectively implanted in order to be a cure for his brother's thalassemia. The child was born from an embryo screened to be free of the disease before implantation with In vitro fertilization. The baby's supply of immunologically compatible cord blood was saved for transplantation to his brother. The transplantation was considered successful. In 2009, a group of doctors and specialists in Chennai and Coimbatore registered the successful treatment of thalassemia

title: Management of thalassemia

text: by patients receiving blood transfusions for thalassemia. An additional benefit of the use of deferasirox instead of desferoxamine is that, unlike desferoxamine, early studies have indicated that deferasirox does not have a significant impact on the growth and development of pediatric thalassemia patients. In a study by Cappellini et al. it was shown that children receiving the treatment displayed continual near-normal growth and development over a 5-year study period. Deferasirox can, however, have a wide variety of side effects. These may include headaches, nausea, vomiting, and joint pains. Some evidence has been shown of a link to gastrointestinal disorders experienced

title: Management of thalassemia

text: the plasma, and transfer the iron to deferoxamine which goes on to expel it from the body. DFP was also found to be significantly more effective than deferoxamine in treating myocardial siderosis in patients with thalassemia major: DFP is thought to improve the function of mitochondria in the heart by accessing and redistributing labile iron in cardiac cells. Thalassemia patients may also be faced with potential oxidative damage to brain cells as the brain has high oxygen demands, but contains relatively low levels of antioxidant agents for protection against oxidation. The presence of excess iron in the brain may lead

text: making proteins called Alpha goblin which is a component of hemoglobin. People have two copies of the HBA2 gene in each cell. Each copy is called an allele. For each gene, one allele is inherited from a person’s father, and the other is inherited from a person’s mother. As a result, there are four alleles that produce alpha globin. The different types of alpha thalassemia result from the loss of some sort or all of these alleles. Hb Bart syndrome, the most severe form of alpha thalassemia, results from the loss of all four alpha globin alleles. HbH disease is

text: has 46 of them. Each package is arranged into 23 pairs with one of each pair coming from the mother and from the father. When a child has alpha thalassemia, there is a change in chromosome 16. Alpha globin is made on chromosome 16, so if any gene that tells chromosome 16 to produce alpha globin is missing or mutated, less hemoglobin is made. This affects hemoglobin and decreases the ability of red blood cells to transport oxygen to the body. Alpha Thalassemia typically results from deletion involving the HBA1 and HBA2 genes. Both of these genes provide instructions for

text: a component of hemoglobin. It is caused by mutations in the "HBB" gene which make instructions for making the protein beta globin. Alpha Thalassemia happens when the body has a problem producing alpha globin. This prevents oxygen from reaching the body's tissues and can cause a loss of red blood cells. Some children with alpha thalassemia have no symptoms and require no treatment. Others with more severe cases need regular blood transfusions to treat anemia and other symptoms. They also may require surgery in early years to repair the abnormalities they were born with or else physical and occupational therapy

text: histone H3 lysine-4 trimethyltransferase, which is a known target for ATRX and 2-BHMT2 encodes for betaine-homocysteine methyltransferase, which catalyzes the methylation of homocysteine . ATR association can be separated into two groups. ATR-16 syndrome patients have a 1-2Mb deletion on the top of the chromosome 16 p-arm and are associated with a Mendelian inheritance of a-thalassemia . ATR-X syndrome patients have no deletion in chromosome 16, a-thalassemia is rare, and this syndrome is consistent with X-linked recessive inheritance . However, both groups have similar phenotypes . The phenotypes resulting from ATR-X are due to skewed x-inactivation . When X- inactivation

title: Thalassemia

text: a lentiviral vector. After destroying the affected person's bone marrow with a dose of chemotherapy (a myeloablative conditioning regimen), the altered HSCs are infused back into the affected person where they become engrafted in the bone marrow where they proliferate. This potentially results in a progressive increase in hemoglobin A2 synthesis in all subsequent developing red blood cells, with resultant resolution of the anemia. While one person with beta thalassemia has no longer required blood transfusions following treatment within a research trial, it is not an approved treatment as of 2018. HbF induction is an attempt to reactivatefetal globin gene

title: Thalassemia

text: the Balearics) such as Sicily, Sardinia, Malta, Corsica, Cyprus, and Crete are heavily affected in particular. Other Mediterranean people, as well as those in the vicinity of the Mediterranean, also have high rates of thalassemia, including people from West Asia and North Africa. Far from the Mediterranean, South Asians are also affected, with the world's highest concentration of carriers (30% of the population) being in the Maldives. Nowadays, it is found in populations living in Africa, the Americas, and in Tharu people in the Terai region of Nepal and India. It is believed to account for much lower malaria sicknesses

text: caused by loss of three of the four alpha globin alleles. In these two conditions, a shortage of alpha globin prevents cells from making normal hemoglobin. Instead, cells produce abnormal forms of hemoglobin called hemoglobin Bart (Hb Bart) or hemoglobin H (HbH). These abnormal hemoglobin molecules cannot effectively carry oxygen to the body’s tissues. The substitution of Hb Bart or HbH for normal hemoglobin causes anemia and the other serious health problems associated with Alpha Thalassemia abnormal morphogenesis. Two additional variants of alpha thalassemia abnormal morphogenesis are rated to a reduced amount of alpha globin. Because cells still produce some

title: Thalassemia

text: and deaths, accounting for the historic ability of Tharus to survive in areas with heavy malaria infestation, where others could not. Thalassemias are particularly associated with people of Mediterranean origin, Arabs (especially Palestinians and people of Palestinian descent), and Asians. The Maldives has the highest incidence of thalassemia in the world with a carrier rate of 18% of the population. The estimated prevalence is 16% in people from Cyprus, 1% in Thailand, and 3–8% in populations from Bangladesh, China, India, Malaysia and Pakistan. Thalassemias also occur in descendants of people from Mediterranean countries (e.g. Greece, Italy, Spain, and others), in

title: Thalassemia

text: births to almost zero. Greece also has a screening program to identify people who are carriers. In Iran as a premarital screening, the man's red cell indices are checked first, if he has microcytosis (mean cell hemoglobin < 27 pg or mean red cell volume < 80 fl), the woman is tested. When both are microcytic, their hemoglobin A2 concentrations are measured. If both have a concentration above 3.5% (diagnostic of thalassemia trait) they are referred to the local designated health post for genetic counseling. Large scale awareness campaigns are being organized in India both by government and non-government organizations

title: Thalassemia

text: Cases of dominantly inherited α- and β-thalassemias have been reported, the first of which was in an Irish family with two deletions of 4 and 11 bp in exon 3 interrupted by an insertion of 5 bp in the β-globin gene. For the autosomal recessive forms of the disease, both parents must be carriers for a child to be affected. If both parents carry a hemoglobinopathy trait, the risk is 25% for each pregnancy for an affected child. Having a single genetic variant for thalassemia may protect against malaria and thus can be an advantage. People diagnosed with heterozygous (carrier)

title: Beta thalassemia

text: Although not life-threatening on its own, it can affect quality of life due to the anemia. Minor often coexists with other conditions such as asthma and can cause iron overload of the liver and in those with non-alcoholic fatty liver disease, lead to more severe outcomes. The beta form of thalassemia is particularly prevalent among the Mediterranean peoples and this geographical association is responsible for its naming: "thalassa" ("θάλασσα") is the Greek word for sea and "haema" ("αἷμα") is the Greek word for blood. In Europe, the highest concentrations of the disease are found in Greece and the Turkish coastal

title: Beta thalassemia

text: in Europe in 2018. Iron overload is an unavoidable consequence of chronic transfusion therapy, necessary for patients with beta thalassemia. Iron chelation is a medical therapy that avoids the complications of iron overload. The iron overload can be removed by Deferasirox, an oral iron chelator, which has a dose- dependent effect on iron burden. Every unit of transfused blood contains 200–250 mg of iron and the body has no natural mechanism to remove excess iron. Deferasirox is a vital part in the patients health after blood transfusions.During normal iron homeostasis the circulating iron is bound to transferrin, but with an

title: Beta thalassemia

text: hemoglobin A2 and slightly increased hemoglobin F. Skeletal changes associated with expansion of the bone marrow: All beta thalassemias may exhibit abnormal red blood cells, a family history is followed by DNA analysis. This test is used to investigate deletions and mutations in the alpha- and beta-globin-producing genes. Family studies can be done to evaluate carrier status and the types of mutations present in other family members. DNA testing is not routine, but can help diagnose thalassemia and determine carrier status. In most cases the treating physician uses a clinical prediagnosis assessing anemia symptoms: fatigue, breathlessness and poor exercise tolerance.

title: Thalassemia

text: chains are encoded by a single gene on chromosome 11; α globin chains are encoded by two closely linked genes on chromosome 16. Thus, in a normal person with two copies of each chromosome, two loci encode the β chain, and four loci encode the α chain. Deletion of one of the α loci has a high prevalence in people of African or Asian descent, making them more likely to develop α-thalassemia. β-Thalassemias are not only common in Africans, but also in Greeks and Italians. The α-thalassemias involve the genes "HBA1" and "HBA2", inherited in a Mendelian recessive fashion. Two

title: Beta thalassemia

text: regions. The major Mediterranean islands (except the Balearics) such as Sicily, Sardinia, Corsica, Cyprus, Malta and Crete are heavily affected in particular. Other Mediterranean peoples, as well as those in the vicinity of the Mediterranean, also have high incidence rates, including people from West Asia and North Africa. The data indicate that 15% of the Greek and Turkish Cypriots are carriers of beta-thalassaemia genes, while 10% of the population carry alpha-thalassaemia genes. The thalassemia trait may confer a degree of protection against malaria, which is or was prevalent in the regions where the trait is common, thus conferring a selective

title: Beta thalassemia

text: that they are painful and inconvenient. The oral chelator deferasirox was approved for use in 2005 in some countries, it offers some hope with compliance at a higher cost. Bone marrow transplantation is the only cure and is indicated for patients with severe thalassemia major. Transplantation can eliminate a patient's dependence on transfusions. Absent a matching donor, a savior sibling can be conceived by preimplantation genetic diagnosis (PGD) to be free of the disease as well as to match the recipient's human leukocyte antigen (HLA) type. Scientists at Weill Cornell Medical College have developed a gene therapy strategy that could

title: Beta thalassemia

text: left arm elevated above the head to properly make the incision. The camera is inserted along with four other trocars: one placed in the left subcostal area, one inserted at the midpoint between the first and third, one 4 cm right of the midline, and the fourth positioned on the midline to retract the spleen. Long-term transfusion therapy to maintain the patient’s hemoglobin level above 9-10 g/dL (normal levels are 13.8 for males, and 12.1 for females). Patients are transfused by meeting strict criteria ensuring their safety. They must have: confirmed laboratory diagnosis of thalassemia major, and hemoglobin levels less

title: Beta thalassemia

text: associative due to this disease's complexity. The following associative signs can attest to the severity of the phenotype: pallor, poor growth, inadequate food intake, splenomegaly, jaundice, maxillary hyperplasia, dental malocclusion, cholelithiasis, systolic ejection murmur in the presence of severe anemia and pathologic fractures. Based on symptoms, tests are ordered for a differential diagnosis. These tests include complete blood count; hemoglobin electrophoresis; serum transferrin, ferritin, total iron-binding capacity; urine urobilin and urobilogen; peripheral blood smear, which may show codocytes, or target cells; hematocrit; and serum bilirubin. The expected pattern on hemoglobin electrophoresis in people with beta-thalassemia is an increased level of

title: Beta thalassemia

text: mutation damages the assembly of the messenger-type RNA (mRNA) that is transcribed from a chromosome. DNA contains both the instructions (genes) for stringing amino acids together into proteins, as well as stretches of DNA that play important roles in regulating produced protein levels. In thalassemia, an additional, contiguous length or a discontinuous fragment of non-coding instructions are included in the mRNA. This happens because the mutation obliterates the boundary between the intronic and exonic portions. Because all the coding sections may still be present, normal hemoglobin may be produced and the added material, if it produces pathology, instead disrupts regulatory

title: Microcytosis

text: Microcytosis Microcytosis is a condition in which red blood cells are unusually small as measured by their mean corpuscular volume. It is also known as "microcythemia". When associated with anemia, it is known as microcytic anemia. Microcytic anemia is not caused by reduced DNA synthesis. Thalassemia can cause microcytosis. Depending upon how the terms are being defined, thalassemia can be considered a cause of microcytic anemia, or it can be considered a cause of microcytosis but not a cause of microcytic anemia. There are many causes of microcytosis, which is essentially only a descriptor. Cells can be small because of

title: Management of thalassemia

text: by some people who have received the treatment. Indicaxanthin is a pigment derived from the cactus pear fruit and can be used as an antioxidant. Dietary indicaxanthin has been shown to have protective effects on RBCs in people with beta thalassemia. It has a structure similar to that of amino acids, and is amphiphilic: it is able to bind to cell membranes through charge-related interactions with polar head groups of membrane constituents, as well through adsorption to the lipid aggregates. Upon ex vivo introduction to thalassemic blood, indicaxanthin was shown to accumulate within RBCs. Hb undergoes the following oxidation reaction

title: Management of thalassemia

text: plasma; transferrin acts as a transporter, carrying iron through blood and providing cells with the metal through endocytosis. Transferrin is highly specific to iron (III), and binds to it with an equilibrium constant of 10 M at a pH of 7.4. Thalassemia results in nontransferrin-bound iron being available in blood as all the transferrin becomes fully saturated. This free iron is toxic to the body since it catalyzes reactions that generate free hydroxyl radicals. These radicals may induce lipid peroxidation of organelles like lysosomes, mitochondria, and sarcoplasmic membranes. The resulting lipid peroxides may interact with other molecules to form cross

title: Beta thalassemia

text: functions enough to produce anemia. Hemoblogin's normal alpha and beta subunits each have an iron-containing central portion (heme) that allows the protein chain of a subunit to fold around it. Normal adult hemoglobin contains 2 alpha and 2 beta subunits. Thalassemias typically affect only the mRNAs for production of the beta chains (hence the name). Since the mutation may be a change in only a single base (single-nucleotide polymorphism), on-going efforts seek gene therapies to make that single correction. Family history and ancestry are factors that increase the risk of beta thalassemia. Depending on your family history, if your parents

title: Homer Defined

text: not afraid to tell the truth: that everything is just fine." In the book, Gray says this scene is used by the show's producers to criticize "how often the news is wholly toothless, sacrificing journalism for sales, and leaving us not with important public information, but with America's Favorite Pencil." Homer Defined "Homer Defined" is the fifth episode of "The Simpsons"<nowiki>'</nowiki> third season. It originally aired on the Fox network in the United States on October 17, 1991. In the episode, Homer accidentally saves the Springfield Nuclear Power Plant from meltdown by arbitrarily choosing the emergency override button via a

text: to help live with terminal transverse limbs. The only way to get alpha thalassemia abnormal morphogenesis is by inheriting it from his or her parents from their genes. Humans are made up of trillions of cells that form the structure of our bodies and carry out specialized jobs like taking nutrients from food and turning them into energy. Red blood cells, which contain hemoglobin deliver oxygen to all parts of the body. All cells have a nucleus at their center which is kind of like a brain or command post of the cell. The nucleus directs the cell telling it

title: Defined contribution plan

text: of a defined contribution plan is "a plan providing for an individual account for each participant, and for benefits based solely on the amount contributed to the account, plus or minus income, gains, expenses and losses allocated to the account" (see ). Defined contribution plans are subject to IRS limits on how much can be contributed, known as the section 415 limit. In 2013, the total deferral amount, including employee contribution plus employer contribution, is limited to $51,000 or 100% of compensation, whichever is less. The employee-only limit in 2018 is $18,500 with a $6,000 catch-up. These numbers continue to

title: How It Is

text: the world as mud ("E fango è il mondo") and therefore, a kind of purgatory, as well as Dante's image of souls gulping mud in the Stygian marsh of the "Inferno" (Canto VII, 109–126, in Palma's translation): Dante's Belacqua and his foetal position also are referenced in "How It Is" and the following quotation is an example of the work's unpunctuated, dense, and poetic style: The novel served as inspiration for Miroslaw Balka's 2009 work, "How It Is", in Tate Modern's Turbine Hall. How It Is How It Is is a novel by Samuel Beckett first published in French as

text: Epigenetics “The role of ATRX as a regulator of heterochromatin dynamics raises the possibility that mutations in ATRX may lead to downstream transcriptional effects across the complex of genes or repetitive regions involved in the global context of the disorder, in addition to explaining phenotypical differences in these patients. For example, ATRX mutations affect the expression of alpha-globin gene cluster, causing alpha-thalassemia” . ATRX interacts with the transcription co-factor DAXX and the alpha-globin gene cluster . Together they are all responsible for depositing the histone H3.3 at telomeric and pericentromeric regions. They are also responsible for regulating gene expression at

title: How Long Is Forever?

text: novella has also been translated to other languages, such as Thai (as ตลอดกาลน่ะนานแค่ไหน, by Sirindhorn) and Turkish (as "Sonsuz Ne Kadar Uzun?", by Nuri Razi). "How Long Is Forever?" has been adapted into a 2001 Chinese TV drama series directed by Chen Weiming, starring Xu Fan as Bai Daxing. How Long Is Forever? How Long Is Forever?, also translated as How Far Is Forever? and How Long Will Eternity Last?, is a 1999 Chinese novella by Tie Ning. Set in Beijing, the novella follows a sincere and altruistic-to-a-fault girl named Bai Daxing who never changes: forever like a meek child,

title: Well-defined

text: for assignment is "right-to-left-associative" which means that codice_5 is defined as codice_6. In the programming language APL there is only one rule: from right to left − but parentheses first. A solution to a partial differential equation is said to be well-defined if it is determined by the boundary conditions in a continuous way as the boundary conditions are changed. Well-defined In mathematics, an expression is called well-defined or "unambiguous" if its definition assigns it a unique interpretation or value. Otherwise, the expression is said to be "not well-defined" or "ambiguous". A function is well-defined if it gives the same

title: Well-defined

text: Well-defined In mathematics, an expression is called well-defined or "unambiguous" if its definition assigns it a unique interpretation or value. Otherwise, the expression is said to be "not well-defined" or "ambiguous". A function is well-defined if it gives the same result when the representation of the input is changed without changing the value of the input. For instance if "f" takes real numbers as input, and if "f"(0.5) does not equal "f"(1/2) then "f" is not well-defined (and thus: not a function). The term "well-defined" is also used to indicate whether a logical statement is unambiguous. A function that is

title: This Is How (Burroughs)

text: This Is How (Burroughs) This Is How: Proven Aid in Overcoming Shyness, Molestation, Fatness, Spinsterhood, Grief, Disease, Lushery, Decrepitude & More for Young and Old Alike is the eighth book by Augusten Burroughs. It was released on May 8, 2012. According to the publisher's synopsis, the book "explores how to survive the 'un-survivable'" in a different way from most self-help books. Burroughs has said that the question he is asked more often than any other is how he survived certain experiences in his life. Burroughs wrote the book as a response to those questions. Burroughs says the book is about

title: How Soon Is Now?

text: How Soon Is Now? "How Soon Is Now?" is a song by the English rock band the Smiths, written by singer Morrissey and guitarist Johnny Marr. Originally a B-side of the 1984 single "William, It Was Really Nothing", "How Soon Is Now?" was subsequently featured on the compilation album "Hatful of Hollow" and on US, Canadian, Australian, and Warner UK editions of "Meat Is Murder". Belatedly released as a single in the UK in 1985, it reached No. 24 on the UK Singles Chart. When re-released in 1992, it reached No. 16. In 2007, Marr said "How Soon Is Now?"

title: How Long Is Forever?

text: How Long Is Forever? How Long Is Forever?, also translated as How Far Is Forever? and How Long Will Eternity Last?, is a 1999 Chinese novella by Tie Ning. Set in Beijing, the novella follows a sincere and altruistic-to-a-fault girl named Bai Daxing who never changes: forever like a meek child, she always gives in to others' demands even as she gets used and dumped over and over again. Through the static Bai Daxing, Tie Ning also explores the changing values in Beijing from the 1970s Cultural Revolution to the 1990s as China experiences rapid economic growth and urbanization. The

title: How It Is

text: How It Is How It Is is a novel by Samuel Beckett first published in French as "Comment c'est" by Les Editions de Minuit in 1961. The Grove Press (New York) published Beckett's English translation in 1964. An advance text of his English translation of the third part appeared in the 1962 issue of the Australian literary journal, "Arna". "L'Image", an early variant version of "Comment c'est", was published in the British arts review, "X: A Quarterly Review" (1959), and is the first appearance of the novel in any form. The novel is a monologue by the narrator as he

title: On How Life Is

text: Life Is" was met with highly positive reviews from music critics upon its release, with many praising Gray's songwriting and vocal performance. "Q" rated the album four out of five stars, calling it a "confident, bluesy soul debut [...] with a lived-in sound – as if Rod Stewart were a girl." Sample credits Credits adapted from the liner notes of "On How Life Is". Musicians Technical Artwork On How Life Is On How Life Is is the debut studio album by American singer and songwriter Macy Gray. It was released on July 1, 1999, by Epic Records. Produced by Andrew

title: How Soon Is Now?

text: vocals. From the original creators, Marr found t.A.T.u.'s version "just silly", but Morrissey viewed it much more favourably: "How Soon Is Now?" debuted at number 37 in Australia, however it fell out at number 43 the next week. It managed to score the top ten in both the Finnish Singles Chart and Swedish Singles Chart, peaking at eight and ten. Elsewhere, the song was generally moderate in other European singles chart. How Soon Is Now? "How Soon Is Now?" is a song by the English rock band the Smiths, written by singer Morrissey and guitarist Johnny Marr. Originally a B-side

title: Well-defined

text: not well-defined is not the same as a function that is undefined. For example, if "f"("x") = 1/"x", then "f"(0) is undefined, but this has nothing to do with the question of whether "f"("x") = 1/"x" is well-defined. It is; 0 is simply not in the domain of the function. Let formula_1 be sets, let formula_2 and "define" formula_3 as formula_4 if formula_5 and formula_6 if formula_7. Then formula_8 is well-defined if formula_9. This is e. g. the case when formula_10 (then "f"("a") happens to be formula_11). If however formula_12 then formula_8 is not well-defined because formula_14 is "ambiguous" for

title: Densely defined operator

text: Densely defined operator In mathematics – specifically, in operator theory – a densely defined operator or partially defined operator is a type of partially defined function. In a topological sense, it is a linear operator that is defined "almost everywhere". Densely defined operators often arise in functional analysis as operations that one would like to apply to a larger class of objects than those for which they "a priori" "make sense". A densely defined linear operator "T" from one topological vector space, "X", to another one, "Y", is a linear operator that is defined on a dense linear subspace dom("T")

title: On How Life Is

text: On How Life Is On How Life Is is the debut studio album by American singer and songwriter Macy Gray. It was released on July 1, 1999, by Epic Records. Produced by Andrew Slater, it became Gray's best-selling album to date, selling 3.2 million copies in the United States and seven million copies worldwide. The album's second single, "I Try", became an international hit, topping the charts in Australia, Ireland, and New Zealand, while reaching number five on the US "Billboard" Hot 100. The song also won a Grammy Award for Best Female Pop Vocal Performance in 2001. "On How

title: Well-defined

text: formula_31 and formula_32 are the integers modulo "m" and formula_33 denotes the congruence class of "n" mod "m". N.B.: formula_34 is a reference to the element formula_35, and formula_36 is the argument of "f". The function "f" is well-defined, because In particular, the term well-defined is used with respect to (binary) operations on cosets. In this case one can view the operation as a function of two variables and the property of being well-defined is the same as that for a function. For example, addition on the integers modulo some "n" can be defined naturally in terms of integer addition.

title: How Deep Is the Ocean?

text: How Deep Is the Ocean? "How Deep Is the Ocean " is a popular song written by Irving Berlin in 1932. The song was developed from an earlier Berlin song "To My Mammy" which was sung by Al Jolson in his film "Mammy" (1930). In the earlier song, the lyrics include the questions "How deep is the ocean? / How high is the sky?" and this was the genesis of "How Deep Is the Ocean?". The song was written at a low point in Berlin's professional and personal life, and is among the select few of his numbers that were

title: Defined contribution plan

text: as a large expense avoidable by disbanding the defined benefit plan and instead offering a defined contribution plan. Money contributed can either be from employee salary deferral or from employer contributions. The portability of defined contribution plans is legally no different from the portability of defined benefit plans. However, because of the cost of administration and ease of determining the plan sponsor's liability for defined contribution plans (no actuary is needed to calculate the lump sum equivalent unlike for defined benefit plans), in practice, defined contribution plans have become generally portable. In a defined contribution plan, investment risk and investment

title: Titia de Lange

text: she completed her master's thesis. Her thesis focused on DNA translocation in γβ-thalassemia, a very rare form of thalassemia. Her research identified a patient with γβ-thalassemia with a DNA translocation that caused the inactivation of the β-Globin gene. de Lange spoke highly of the lab saying "That was where I first saw how science is really done. … It was a very vibrant, competitive, international lab. It was a lot of fun, so that made me stay in science." De Lange started to gain interest in telomeres while earning her Ph.D. at the Netherlands Cancer Institute. Telomeres gradually became the

title: Densely defined operator

text: of "X" and takes values in "Y", written "T" : dom("T") ⊆ "X" → "Y". Sometimes this is abbreviated as "T" : "X" → "Y" when the context makes it clear that "X" might not be the set-theoretic domain of "T". Densely defined operator In mathematics – specifically, in operator theory – a densely defined operator or partially defined operator is a type of partially defined function. In a topological sense, it is a linear operator that is defined "almost everywhere". Densely defined operators often arise in functional analysis as operations that one would like to apply to a larger

title: How Is Your Fish Today?

text: How Is Your Fish Today? How Is Your Fish Today?, also known as Jin Tian De Yu Zen Me Yang?, is a 2007 Chinese film written by Xiaolu Guo and Hui Rao. It was directed by Xiaolu Guo. The film is a drama set in modern China, focusing on the intertwined stories of two main characters; a frustrated writer (Hui Rao) and the subject of his latest work, Lin Hao (Zijiang Yang). "How Is Your Fish Today" won 4 international awards and was well received by critics, but was not commercially successful. "How Is Your Fish Today?" was consistently given

title: This Is How You Die

text: idea can be stretched without breaking". There are 31 stories contained within "This Is How You Die": This Is How You Die This Is How You Die: Stories of the Inscrutable, Infallible, Inescapable Machine of Death is a 2013 collection of science fiction short stories, which is a follow up anthology to the "Machine of Death", edited by Ryan North, Matthew Bennardo, and David Malki. "Kirkus Reviews" called it "(f)unny, frightening, (and) clever", while "Publishers Weekly" found it to be "fun, thoughtful, and sometimes dark". "The A.V. Club" noted the many "strikingly different perspectives" of how society would be affected

title: This Is How You Die

text: This Is How You Die This Is How You Die: Stories of the Inscrutable, Infallible, Inescapable Machine of Death is a 2013 collection of science fiction short stories, which is a follow up anthology to the "Machine of Death", edited by Ryan North, Matthew Bennardo, and David Malki. "Kirkus Reviews" called it "(f)unny, frightening, (and) clever", while "Publishers Weekly" found it to be "fun, thoughtful, and sometimes dark". "The A.V. Club" noted the many "strikingly different perspectives" of how society would be affected by the Machine, and lauded the book as "a celebration of creativity, exploring how impressively far one

title: Well-defined

text: If and only if formula_9, we get a function formula_8, and the formula_8 of "definition" is well-defined (as a function). On the other hand: if formula_12 then for an formula_15 there is both, formula_25 "and" formula_26, and the binary relation formula_8 is not "functional" as defined in Binary relation#Special types of binary relations and thus not well-defined (as a function). Colloquially, the "function" formula_8 is called ambiguous at point formula_29 (although there is "per definitionem" never an "ambiguous function"), and the original "definition" is pointless. Despite these subtle logical problems, it is quite common to anticipatorily use the term definition

title: User-defined function

text: User-defined function A user-defined function (UDF) is a function provided by the user of a program or environment, in a context where the usual assumption is that functions are built into the program or environment. In some old implementations of the BASIC programming language, user-defined functions are defined using the "DEF FN" syntax. More modern dialects of BASIC are influenced by the structured programming paradigm, where most or all of the code is written as user-defined functions or procedures, and the concept becomes practically redundant. In relational database management systems, a user-defined function provides a mechanism for extending the functionality

title: Know-how

text: of industrial property (now generally viewed as intellectual property or IP), know-how is a component in the transfer of technology in national and international environments, co-existing with or separate from other IP rights such as patents, trademarks and copyright and is an economic asset. When it is transferred by itself, know-how should be converted into a trade secret before transfer in a legal agreement. Know-how can be defined as "confidentially held", or better, "closely held" information in the form of unpatented inventions, formulae, designs, drawings, procedures and methods, together with accumulated skills and experience in the hands of a licensor

title: Well-defined

text: that the formula_46 operation is understood as addition of the opposite, thus formula_48 is the same as formula_49, and is called "well-defined". Division is also non-associative. However, in the case of formula_50 the convention formula_51 is not so well established, so this expression is considered ill-defined. Unlike with functions, the notational ambiguities can be overcome more or less easily by means of additional definitions, i. e. rules of precedence, and/or associativity of the operators. In the programming language C e. g. the operator codice_1 for subtraction is "left-to-right-associative" which means that codice_2 is defined as codice_3 and the operator codice_4