Skull Bossing
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Skull Bossing
Skull bossing is a descriptive term in medical physical examination indicating a protuberance of the skull, most often in the frontal bones of the forehead ("frontal bossing"). Although prominence of the skull bones may be normal, skull bossing may be associated with certain medical conditions, including nutritional, metabolic, hormonal, and hematologic disorders. Frontal bossing Frontal bossing is the development of an unusually pronounced forehead which may also be associated with a heavier than normal brow ridge. It is caused by enlargement of the frontal bone, often in conjunction with abnormal enlargement of other facial bones, skull, mandible, and bones of the hands and feet. Frontal bossing may be seen in a few rare medical syndromes such as acromegaly – a chronic medical disorder in which the anterior pituitary gland produces excess growth hormone (GH). Frontal bossing may also occur in diseases resulting in chronic anemia, where there is increased hematopoiesis and enl ...
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Frontal Bone
In the human skull, the frontal bone or sincipital bone is an unpaired bone which consists of two portions.'' Gray's Anatomy'' (1918) These are the vertically oriented squamous part, and the horizontally oriented orbital part, making up the bony part of the forehead, part of the bony orbital cavity holding the eye, and part of the bony part of the nose respectively. The name comes from the Latin word ''frons'' (meaning "forehead"). Structure The frontal bone is made up of two main parts. These are the squamous part, and the orbital part. The squamous part marks the vertical, flat, and also the biggest part, and the main region of the forehead. The orbital part is the horizontal and second biggest region of the frontal bone. It enters into the formation of the roofs of the orbital and nasal cavities. Sometimes a third part is included as the nasal part of the frontal bone, and sometimes this is included with the squamous part. The nasal part is between the brow ridges, ...
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Crouzon Syndrome
Crouzon syndrome is an autosomal dominant genetic disorder known as a branchial arch syndrome. Specifically, this syndrome affects the first branchial (or pharyngeal) arch, which is the precursor of the maxilla and mandible. Because the branchial arches are important developmental features in a growing embryo, disturbances in their development create lasting and widespread effects. The syndrome is caused by a mutation in a gene on chromosome 10 that controls the body's production of fibroblast growth factor receptor 2 (''FGFR2''). Crouzon syndrome is named for Octave Crouzon, a French physician who first described this disorder. First called "craniofacial dysostosis" ("craniofacial" refers to the skull and face, and " dysostosis" refers to malformation of bone), the disorder was characterized by a number of clinical features which can be described by the rudimentary meanings of its former name. The developing fetus's skull and facial bones fuse early or are unable to expand. Thu ...
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Beta-thalassemia
Beta-thalassemia (β-thalassemia) is an inherited blood disorder, a form of thalassemia resulting in variable outcomes ranging from clinically asymptomatic to severe anemia individuals. It is caused by reduced or absent synthesis of the beta chains of hemoglobin, the molecule that carries oxygen in the blood. Symptoms depend on the extent to which hemoglobin is deficient, and include anemia, pallor, tiredness, enlargement of the spleen, jaundice, and gallstones. In severe cases death ensues. Beta thalassemia occurs due to a mutation of the HBB gene leading to deficient production of the hemoglobin subunit beta-globin; the severity of the disease depends on the nature of the mutation, and whether or not the mutation is homozygous. The body's inability to construct beta-globin leads to reduced or zero production of adult hemoglobin thus causing anemia. The other component of hemoglobin, alpha-globin, accumulates in excess leading to ineffective production of red blood cells, ...
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Trimethadione
Trimethadione (Tridione) is an oxazolidinedione anticonvulsant. It is most commonly used to treat epileptic conditions that are resistant to other treatments. It is primarily effective in treating absence seizures, but can also be used in refractory temporal lobe epilepsy. It is usually administered 3 or 4 times daily, with the total daily dose ranging from 900 mg to 2.4 g. Treatment is most effective when the concentration of its active metabolite, dimethadione, is above 700 μg/mL. Severe adverse reactions are possible, including Steven Johnson syndrome, nephrotoxicity, hepatitis, aplastic anemia, neutropenia, or agranulocytosis. More common adverse effects include drowsiness, hemeralopia, and hiccups. Fetal trimethadione syndrome If administered during pregnancy, fetal trimethadione syndrome may result causing facial dysmorphism (short upturned nose, slanted eyebrows), cardiac defects, intrauterine growth restriction Intrauterine growth restriction (IUGR), or fetal grow ...
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