The signs and symptoms of thalassemia major appear within the first 2 years of life. Children develop life-threatening anemia. They do not gain weight and grow at the expected rate (failure to thrive) and may develop yellowing of the skin and whites of the eyes (jaundice). Affected individuals may have an enlarged spleen, liver, and heart, and their bones may be misshapen. Puberty is delayed in some adolescents with thalassemia major.
Thalassemia intermedia is milder than thalassemia major. The signs and symptoms of thalassemia intermedia appear in early childhood or later in life. Affected individuals have mild to moderate anemia and may also have slow growth, bone abnormalities, and an increased risk of developing abnormal blood clots.
The symptoms and severity of beta thalassemia varies greatly from one person to another. Individuals with beta thalassemia minor do not develop symptoms of the disorder but may have a mild anemia. Many individuals with beta thalassemia minor go through life never knowing they carry an altered gene for the disorder.
A beta thalassemia major diagnosis is usually made during the first two years of life and individuals require regular blood transfusions and lifelong medical care to survive. When the disorder develops later during life, a diagnosis of beta thalassemia intermedia is given; individuals may only require blood transfusions on rare, specific instances.
Even when treated, complications may develop, specifically the buildup of iron in the body (iron overload). Iron overload results from the blood transfusions required to treat individuals with beta thalassemia major. In addition, affected individuals experience greater iron absorption from the gastrointestinal tract, which contributes to iron overload (although this primarily occurs in untreated individuals). Iron overload can cause tissue damage and impaired function of affected organs such as the heart, liver and endocrine glands. Iron overload can damage the heart causing abnormal heart rhythms, inflammation of the membrane (pericardium) that lines the heart (pericarditis), enlargement of the heart and disease of the heart muscle (dilated cardiomyopathy). Heart involvement can progress to life-threatening complications such as heart failure. Liver involvement can cause scarring and inflammation of the liver (cirrhosis) and high pressure of the main liver vein (portal hypertension). Endocrine gland involvement can cause insufficiency of certain glands such as the thyroid (hypothyroidism) and, in rare cases, diabetes mellitus. Iron overload can also be associated with growth retardation and the failure or delay of sexual maturation.
Normal hemoglobin is made up of specialized proteins called globins, specifically two alpha chains and two beta chain proteins attached to a central heme ring. The HBB gene creates (encodes) beta globin protein chains. A mutation in one HBB gene results in either reduced or no production of beta chains from that gene. Regardless, the second (unaffected) copy of the HBB gene functions normally and produces enough beta chain protein to avoid symptoms, although red blood cells are still abnormally small and mild anemia can still develop. A mutation in two HBB genes results in either significantly reduced levels of beta chains (beta thalassemia intermedia) or an almost complete lack of beta chains (beta thalassemia major). The reduction or lack of beta globin protein chains leads to an imbalance with the normally-produced alpha globin protein chains and, ultimately, the defective formation of red blood cells, a lack of functional hemoglobin, and the failure to deliver sufficient amounts of oxygen to the body.
A diagnosis of beta thalassemia is based upon identification of characteristic symptoms, a clinical evaluation and a variety of specialized tests. With beta thalassemia major, initial symptoms often become apparent during the first two years of life and include failure to thrive, a swollen abdomen, and symptoms of anemia. Beta thalassemia intermedia may be suspected in individuals who present with similar (yet milder) symptoms, but at a later age.
Treatment of additional complications of beta thalassemia or iron overload is symptomatic and supportive. Special attention is recommended for the early diagnosis and prompt treatment of heart disease potentially associated with iron overload. Hearat disease is the main life-threatening complication in individuals with beta thalassemia.
Safety. Beta blockers are generally safe to take. Side effects tend to be annoying, not life-threatening. Doctors long withheld beta blockers from people with chronic obstructive pulmonary disease over worries that these drugs would worsen symptoms, but a report in the Archives of Internal Medicine showed that judicious use of beta blockers may decrease flare-ups of this common breathing problem and improve survival.
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Conclusion: The association between LBW and insulin sensitivity markers may occur in healthy middle-aged adults before overt glucose metabolism disturbances. Our data are coherent with the detection of early life events consequent with insulin resistance markers that could contribute to the risk of glucose metabolism disturbances.
People with moderate and severe forms of thalassemia usually find out about their condition in childhood, since they have symptoms of severe anemia early in life. People with less severe forms of thalassemia may only find out because they are having symptoms of anemia, or maybe because a doctor finds anemia on a routine blood test or a test done for another reason.
Strontium-90 is a commonly used beta emitter used in industrial sources. It decays to yttrium-90, which is itself a beta emitter. It is also used as a thermal power source in radioisotope thermoelectric generator (RTG) power packs. These use heat produced by radioactive decay of strontium-90 to generate heat, which can be converted to electricity using a thermocouple. Strontium-90 has a shorter half-life, produces less power, and requires more shielding than plutonium-238, but is cheaper as it is a fission product and is present in a high concentration in nuclear waste and can be relatively easily chemically extracted. Strontium-90 based RTGs have been used to power remote lighthouses. As strontium is water-soluble, the perovskite form strontium titanate is usually employed as it is not water-soluble and has a high melting point.
Tritium is a low-energy beta emitter commonly used as a radiotracer in research and in traser[check spelling] self-powered lightings. The half-life of tritium is 12.3 years. The electrons from beta emission from tritium are so low in energy (average decay energy 5.7 keV) that a Geiger counter cannot be used to detect them. An advantage of the low energy of the decay is that it is easy to shield, since the low energy electrons penetrate only to shallow depths, reducing the safety issues in deal with the isotope.
Phosphorus-32 is a short-lived high energy beta emitter, which is used in research in radiotracers. It has a half-life of 14 days. It can be used in DNA research. Phosphorus-32 can be made by the neutron irradiation (np reaction) of sulfur-32 or from phosphorus-31 by neutron capture.
Nickel-63 is a radioisotope of nickel that can be used as an energy source in Radioisotope Piezoelectric Generators. It has a half-life of 100.1 years. It can be created by irradiating nickel-62 with neutrons in a nuclear reactor.
People with this condition will need frequent blood transfusions and may not live a normal lifespan. Iron builds up in the heart and other organs from blood transfusions. This can cause heart failure as early as the teens or early 20s.
Children with beta thalassemia intermedia or major may not show any symptoms at birth, but usually develop anemia in the first 2 years of life. Signs of anemia in beta thalassemia intermedia or major may include:
Children with beta thalassemia intermedia or major need lifelong medical care. The best way for your child to live their healthiest life is to get regular medical care, which includes transfusions and chelation.
Premature infantsPreterm infants have low liver stores of vitamin A at birth, and their plasma concentrations of retinol often remain low throughout the first year of life [26,27]. Preterm infants with vitamin A deficiency have a higher risk of eye and chronic lung diseases [28,29]. However, in high-income countries, clinical vitamin A deficiency is rare in infants and occurs only in those with malabsorption disorders .
People with cystic fibrosisUp to 90% of people with cystic fibrosis have pancreatic insufficiency, which increases their risk of vitamin A deficiency due to difficulty absorbing fat [1,37]. Studies in Australia and the Netherlands indicate that 2% to 13% of children and adolescents with cystic fibrosis have vitamin A deficiency [38,39]. As a result, standard care for cystic fibrosis includes lifelong treatment with vitamin A (daily amounts of 750 mcg RAE to 3,000 mcg RAE, depending on age, are recommended in the United States and Australia), other fat-soluble vitamins, and pancreatic enzymes [37,39].
The radionuclides decay at a characteristic rate that remains constant regardless of external influences, such as temperature or pressure. The time that it takes for half the radionuclides to disintegrate or decay is called half-life. This differs for each radioelement, ranging from fractions of a second to billions of years. For example, the half-life of Iodine 131 is eight days, but for Uranium 238, which is present in varying amounts all over the world, it is 4.5 billion years. Potassium 40, the main source of radioactivity in our bodies, has a half-life of 1.42 billion years. 59ce067264