Prepubertal Hypertrichosis
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Prepubertal Hypertrichosis
Prepubertal hypertrichosis, also known as childhood hypertrichosis, is a cutaneous condition characterized by increased hair growth, found in otherwise healthy infants and children. Prepubertal hypertrichosis is a cosmetic condition and does not affect any other health aspect. Individuals with this condition may suffer with low self esteem and mental health issues due to societal perceptions of what a "normal" appearance should be. The mechanism of prepubertal hypertrichosis is unclear, but causes may include genetics, systemic illnesses, or medications. While hypertrichosis affects men and women equally, hypertrichosis of the ears, hypertrichosis of the nose, and hereditary hypertrichosis primarily affect males. Prepubertal hypertrichosis can be present at birth or develop later on during childhood. Management strategies for prepubertal hypertrichosis include pharmacological therapy, drug discontinuation if caused by a drug, and chemical or physical hair removal or alteration meth ...
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Vellus Hair
Vellus hair is short, thin, light-colored, and barely noticeable hair that develops on most of a human’s body during childhood. Exceptions include the lips, the back of the ear, the palm of the hand, the sole of the foot, some external genital areas, the navel, and scar tissue. The density of hair – the number of hair follicles per area of skin – varies from person to person. Each strand of vellus hair is usually less than 2 mm (1/13 inch) long and the follicle is not connected to a sebaceous gland. Vellus hair is most easily observed on children and adult women, who generally have less terminal hair to obscure it. Vellus hair is not lanugo hair. Lanugo hair is a much thicker type of hair that normally grows only on fetuses. Vellus hair is differentiated from the more visible terminal or androgenic hair, which develops only during and after puberty, usually to a greater extent on men than it does on women. The Latin language uses the word ''vellus'' to designate "a fl ...
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Chemical Depilatory
A chemical depilatory is a cosmetic preparation used to remove hair from the skin. Common active ingredients are salts of thioglycolic acid and thiolactic acids. These compounds break the disulfide bonds in keratin and also hydrolyze the hair so that it is easily removed. Formerly, sulfides such as strontium sulfide were used, but due to their unpleasant odor, they have been replaced by thiols. The main chemical reaction effected by the thioglycolate is: : Chemical depilatories contain 5–6% calcium thioglycolate in a cream base (to avoid runoff). Calcium hydroxide or strontium hydroxide maintain a pH of about 12. Hair destruction requires about 10 minutes. Depilation is followed by careful rinsing with water, and various conditioners are applied to restore the skin's pH to normal. Depilation does not destroy the dermal papilla, and the hair grows back. Chemical depilatories are available in gel, cream, lotion, aerosol, roll-on, and powder forms. Common brands include N ...
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Bleach
Bleach is the generic name for any chemical product that is used industrially or domestically to remove color (whitening) from a fabric or fiber or to clean or to remove stains in a process called bleaching. It often refers specifically, to a dilute solution of sodium hypochlorite, also called "liquid bleach". Many bleaches have broad spectrum bactericidal properties, making them useful for disinfecting and sterilizing. They are used in swimming pool sanitation to control bacteria, viruses, and algae, and in many places where sterile conditions are required. They are also used in many industrial processes, notably in the bleaching of wood pulp. Bleaches also have other minor uses like removing mildew, killing weeds, and increasing the longevity of cut flowers. Bleaches work by reacting with many colored organic compounds, such as natural pigments, and turning them into colorless ones. While most bleaches are oxidizing agents (chemicals that can remove electrons from othe ...
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Psychosocial
The psychosocial approach looks at individuals in the context of the combined influence that psychological factors and the surrounding social environment have on their physical and mental wellness and their ability to function. This approach is used in a broad range of helping professions in health and social care settings as well as by medical and social science researchers. Background Adolf Meyer in the late 19th century stated that; "We cannot understand the individual presentation of mental illness, nd perpetuating factorswithout knowing how that person functions in the environment." Psychosocial assessment stems from this idea. The relationship between mental and emotional wellbeing and the environment was first commonly applied by Erik Erikson in his description of the stages of psychosocial development. Mary Richmond considered there to be a strict relationship between cause and effect, in a diagnostic process. In 1941 Gordon Hamilton renamed the existing (1917) con ...
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Mucopolysaccharidoses
Mucopolysaccharidoses are a group of metabolic disorders caused by the absence or malfunctioning of lysosomal enzymes needed to break down molecules called glycosaminoglycans (GAGs). These long chains of sugar carbohydrates occur within the cells that help build bone, cartilage, tendons, corneas, skin and connective tissue. GAGs (formerly called mucopolysaccharides) are also found in the fluids that lubricate joints. Individuals with mucopolysaccharidosis either do not produce enough of one of the eleven enzymes required to break down these sugar chains into simpler molecules, or they produce enzymes that do not work properly. Over time, these GAGs collect in the cells, blood and connective tissues. The result is permanent, progressive cellular damage which affects appearance, physical abilities, organ and system functioning. The mucopolysaccharidoses are part of the lysosomal storage disease family, a group of more than 40 genetic disorders that result when the lysosome or ...
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Cornelia De Lange Syndrome
Cornelia de Lange syndrome (CdLS) is a genetic disorder. People with this syndrome experience a range of physical, cognitive, and medical challenges ranging from mild to severe. The syndrome has a widely varied phenotype, meaning people with the syndrome have varied features and challenges. The typical features of CdLS include thick or long eyebrows, a small nose, small stature, developmental delay, long or smooth philtrum, thin upper lip and downturned mouth. The syndrome is named after Dutch pediatrician Cornelia Catharina de Lange, who described it in 1933. It is often termed Brachmann de Lange syndrome or Bushy syndrome and is also known as Amsterdam dwarfism. Its exact incidence is unknown, but it is estimated at 1 in 10,000 to 30,000. Signs and symptoms The phenotype of CdLS is highly varied and is described as a spectrum; from Classic CdLS (with a greater number of key features) to mild variations with only a few features. Some people will have a small number of feature ...
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Gunther Disease
Gunther disease is a congenital form of erythropoietic porphyria. The word porphyria originated from the Greek word porphura. Porphura actually means "purple pigment", which, in suggestion, the color that the body fluid changes when a person has Gunther's disease. It is a rare, autosomal recessive metabolic disorder affecting heme, caused by deficiency of the enzyme uroporphyrinogen cosynthetase. It is extremely rare, with a prevalence estimated at 1 in 1,000,000 or less. There have been times that prior to birth of a fetus, Gunther's disease has been shown to lead to anemia. In milder cases patients have not presented any symptoms until they have reached adulthood. In Gunther's disease, porphyrins are accumulated in the teeth and bones and an increased amount are seen in the plasma, bone marrow, feces, red blood cells, and urine. Symptoms and signs Though expressivity is varied depending on the mutation responsible for decrease in enzyme function, severe cutaneous sensitivity is ...
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Leprechaunism
Donohue syndrome (also known as leprechaunism) is an extremely rare and severe genetic disorder. ''Leprechaunism'' derives its name from the hallmark elvish features (small stature, bulging eyes, thick lips, and upturned nostrils) exhibited by the affected individuals. The disease is caused by a mutation in the INSR gene, which contains the genetic information for the formation of insulin receptors. As a result, affected individuals have either a decreased number of insulin receptors, or insulin receptor with greatly impaired functionality. The lack and impairment of insulin receptor functionality leads to an inability to regulate blood glucose levels through severe insulin resistance. This will ultimately lead to affected development of tissues and organs throughout the body. In addition to the physical abnormalities, leprechaunism is also characterized by endocrine system abnormalities that can lead to conditions such as hyperglycemia (high blood glucose levels), hypoglycemia (low ...
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Cantú Syndrome
Cantú syndrome is a rare condition characterized by hypertrichosis, osteochondrodysplasia, and cardiomegaly.Initial posting 2014 Less than 50 cases have been described in the literature; they are associated with a mutation in the ''ABCC9''-gene that codes for the ABCC9-protein. Signs and symptoms The main features of this condition are hypertrichosis, osteochondrodysplasia, and cardiomegaly. There is also a characteristic facies. Other features include patent ductus arteriosus, congenital hypertrophy of the left ventricle, and pericardial effusions. Neurodevelopmental outcome appears normal, but obsessive traits and anxiety have been reported. It may also be associated with recurrent infections with low immunoglobulin levels and gastric bleeding, and additional possible associations include lymphoedema and heterochromia iridis. Cause Cantú syndrome apparently is inherited in an autosomal dominant fashion and appears to be affected by the ''ABCC9'' gene. ABCC9 gene provid ...
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