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Pure Autonomic Failure
Pure autonomic failure (PAF) is an uncommon, sporadic neurodegenerative condition marked by a steadily declining autonomic regulation. Bradbury and Eggleston originally described pure autonomic failure in 1925. Patients usually present with orthostatic hypotension or syncope in midlife or later. In addition, genitourinary, thermoregulatory, and bowel dysfunction can be signs of autonomic failure. Pure autonomic failure originates from peripheral autonomic nervous system lesions. The diagnosis of pure autonomic failure relies on the absence of other neurologic abnormalities, specifically Parkinsonism, cognitive impairment, cerebellar ataxia, or tremors, and on compatible clinical features of subtle, progressive pan autonomic failure, most notably orthostatic hypotension. Signs and symptoms The majority of symptoms that patients with PAF exhibit are associated with neurogenic orthostatic hypotension, or orthostatic hypotension brought on by severe sympathetic failure. Within th ...
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Autonomic Nervous System
The autonomic nervous system (ANS), sometimes called the visceral nervous system and formerly the vegetative nervous system, is a division of the nervous system that operates viscera, internal organs, smooth muscle and glands. The autonomic nervous system is a control system that acts largely unconsciously and regulates bodily functions, such as the heart rate, its Myocardial contractility, force of contraction, digestion, respiratory rate, pupillary dilation, pupillary response, Micturition, urination, and Animal sexual behaviour, sexual arousal. The fight-or-flight response, also known as the acute stress response, is set into action by the autonomic nervous system. The autonomic nervous system is regulated by integrated reflexes through the brainstem to the spinal cord and organ (anatomy), organs. Autonomic functions include control of respiration, heart rate, cardiac regulation (the cardiac control center), vasomotor activity (the vasomotor center), and certain reflex, reflex ...
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Pathology
Pathology is the study of disease. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in the context of modern medical treatment, the term is often used in a narrower fashion to refer to processes and tests that fall within the contemporary medical field of "general pathology", an area that includes a number of distinct but inter-related medical specialties that diagnose disease, mostly through analysis of tissue (biology), tissue and human cell samples. Idiomatically, "a pathology" may also refer to the predicted or actual progression of particular diseases (as in the statement "the many different forms of cancer have diverse pathologies", in which case a more proper choice of word would be "Pathophysiology, pathophysiologies"). The suffix ''pathy'' is sometimes used to indicate a state of disease in cases of both physical ailment (as in cardiomyopathy) and psych ...
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Samuel Bradbury
Samuel is a figure who, in the narratives of the Hebrew Bible, plays a key role in the transition from the biblical judges to the United Kingdom of Israel under Saul, and again in the monarchy's transition from Saul to David. He is venerated as a prophet in Judaism, Christianity, and Islam. In addition to his role in the Bible, Samuel is mentioned in Jewish rabbinical literature, in the Christian New Testament, and in the second chapter of the Quran (although the text does not mention him by name). He is also treated in the fifth through seventh books of ''Antiquities of the Jews'', written by the Jewish scholar Josephus in the first century. He is first called "the Seer" in 1 Samuel 9:9. Biblical account Family Samuel's mother was Hannah and his father was Elkanah. Elkanah lived at Ramathaim in the district of Zuph. His genealogy is also found in a pedigree of the Kohathites (1 Chronicles 6:3–15) and in that of Heman the Ezrahite, apparently his grandson (1 Chronicles 6: ...
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Primary Autonomic Failure
Primary autonomic failure (also called primary dysautonomia) refers to a category of dysautonomias — conditions in which the autonomic nervous system does not function properly. In primary dysautonomias, the autonomic dysfunction occurs as a primary condition (as opposed to resulting from another disease). Autonomic failure is categorized as "primary" when believed to result from a chronic condition characterized by degeneration of the autonomic nervous system, or where autonomic failure is the predominant symptom and its cause is unknown. Such "primary" dysautonomias are distinguished from secondary dysautonomias, where the dysfunction of the autonomic nervous system is believed to be caused by another disease (e.g. diabetes). Diseases categorized as primary autonomic failure usually include pure autonomic failure and multiple system atrophy. Many scientists also categorize Parkinson disease Parkinson may refer to: *Parkinson (surname) * ''Parkinson'' (TV series), British ch ...
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Compression Stockings
Compression stockings (Flight Socks, Support Bandage) are a specialized hosiery designed to help prevent the occurrence of, and guard against further progression of, venous disorders such as edema, phlebitis and thrombosis. Compression stockings are elastic compression garments worn around the leg, compressing the limb. This reduces the diameter of distended veins and increases venous blood flow velocity and valve effectiveness. Compression therapy helps decrease venous pressure, prevents venous stasis and impairments of venous walls, and relieves heavy and aching legs. Knee-high compression stockings are used not only to help increase circulation, but also to help prevent the formation of blood clots in the lower legs. They also aid in the treatment of ulcers of the lower legs. Unlike traditional dress or athletic stockings and socks, compression stockings use stronger elastics to create significant pressure on the legs, ankles and feet. Compression stockings are tightest at the ...
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Vasopressor
An antihypotensive, also known as a vasopressor, is an agent that raises blood pressure by constricting blood vessels, thereby increasing systemic vascular resistance. This is different from inotropes which increase the force of cardiac contraction. Some substances do both (e.g. dopamine, dobutamine). If low blood pressure is due to blood loss, then preparations increasing volume of blood circulation—plasma-substituting solutions such as colloid and crystalloid solutions (salt solutions)—will raise the blood pressure without any direct vasopressor activity. Packed red blood cells, plasma or whole blood should not be used solely for volume expansion or to increase oncotic pressure of circulating blood. Blood products should only be used if reduced oxygen carrying capacity or coagulopathy is present. Other causes of either absolute (dehydration, loss of plasma via wound/burns) or relative ( third space losses) vascular volume depletion also respond, although blood products ar ...
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Erythropoietin
Erythropoietin (; EPO), also known as erythropoetin, haematopoietin, or haemopoietin, is a glycoprotein cytokine secreted mainly by the kidneys in response to cellular hypoxia; it stimulates red blood cell production ( erythropoiesis) in the bone marrow. Low levels of EPO (around 10  mU/mL) are constantly secreted in sufficient quantities to compensate for normal red blood cell turnover. Common causes of cellular hypoxia resulting in elevated levels of EPO (up to 10 000 mU/mL) include any anemia, and hypoxemia due to chronic lung disease. Erythropoietin is largely synthesized by fibroblast-like type-1 interstitial cells, located primarily in the deep renal cortex in close association with the peritubular capillaries and proximal convoluted tubule; it is also produced in perisinusoidal cells in the liver. Liver production predominates in the fetal and perinatal period; renal production predominates in adulthood. It is homologous with thrombopoietin. ...
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Somatostatin
Somatostatin, also known as growth hormone-inhibiting hormone (GHIH) or by #Nomenclature, several other names, is a peptide hormone that regulates the endocrine system and affects neurotransmission and cell proliferation via interaction with G protein-coupled somatostatin receptors and inhibition of the release of numerous secondary hormones. Somatostatin inhibits insulin and glucagon secretion. Somatostatin has two active forms produced by the alternative cleavage of a single preproprotein: one consisting of 14 amino acids (shown in infobox to right), the other consisting of 28 amino acids. Among the vertebrates, there exist six different somatostatin genes that have been named: ''SS1'', ''SS2'', ''SS3'', ''SS4'', ''SS5'' and ''SS6''. Zebrafish have all six. The six different genes, along with the five different somatostatin receptors, allow somatostatin to possess a large range of functions. Humans have only one somatostatin gene, ''SST''. Nomenclature Synonyms of "somatost ...
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Midodrine
Midodrine, sold under the brand names ProAmatine and Orvaten among others, is an antihypotensive medication used to treat orthostatic hypotension (low blood pressure when standing) and urinary incontinence. It is taken oral administration, by mouth. Side effects of midodrine include hypertension (high blood pressure), paresthesia, pruritus, itching, goosebumps, chills, urinary urgency, urinary retention, and urinary frequency. Midodrine is a prodrug of its active metabolite desglymidodrine. This metabolite acts as a binding selectivity, selective agonist of the alpha-1 adrenergic receptor, α1-adrenergic receptor. This in turn results in vasoconstriction and increased blood pressure. Midodrine was discovered by 1971 and was introduced for medical use in the United States in 1996. Medical uses Midodrine is indicated for the treatment of symptomatic orthostatic hypotension. It can reduce dizziness and faints by about a third, but can be limited by troublesome goose bumps, prur ...
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Fludrocortisone
Fludrocortisone, sold under the brand name Florinef among others, is a corticosteroid used to treat congenital adrenal hyperplasia, postural hypotension, and adrenal insufficiency. In adrenal insufficiency, it is generally taken together with hydrocortisone. Fludrocortisone is taken by mouth and is most commonly used in its acetate form. Common side effects of fludrocortisone include high blood pressure, swelling, heart failure, and low blood potassium. Other serious side effects can include low immune-system function, cataracts, muscle weakness, and mood changes. Whether use of fludrocortisone during pregnancy is safe for the fetus is unknown. Fludrocortisone is mostly a mineralocorticoid, but it also has glucocorticoid effects. Fludrocortisone was patented in 1953. It is on the World Health Organization's List of Essential Medicines. Medical uses Fludrocortisone has been used in the treatment of cerebral salt-wasting syndrome. It is used primarily to replace the mis ...
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Neurons
A neuron (American English), neurone (British English), or nerve cell, is an membrane potential#Cell excitability, excitable cell (biology), cell that fires electric signals called action potentials across a neural network (biology), neural network in the nervous system. They are located in the nervous system and help to receive and conduct impulses. Neurons communicate with other cells via synapses, which are specialized connections that commonly use minute amounts of chemical neurotransmitters to pass the electric signal from the presynaptic neuron to the target cell through the synaptic gap. Neurons are the main components of nervous tissue in all Animalia, animals except sponges and placozoans. Plants and fungi do not have nerve cells. Molecular evidence suggests that the ability to generate electric signals first appeared in evolution some 700 to 800 million years ago, during the Tonian period. Predecessors of neurons were the peptidergic secretory cells. They eventually ga ...
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Catecholamine
A catecholamine (; abbreviated CA), most typically a 3,4-dihydroxyphenethylamine, is a monoamine neurotransmitter, an organic compound that has a catechol (benzene with two hydroxyl side groups next to each other) and a side-chain amine. Catechol can be either a free molecule or a substituent of a larger molecule, where it represents a 1,2-dihydroxybenzene group. Catecholamines are derived from the amino acid tyrosine, which is derived from dietary sources as well as synthesis from phenylalanine. Catecholamines are water-soluble and are 50% bound to plasma proteins in circulation. Included among catecholamines are epinephrine (adrenaline), norepinephrine (noradrenaline), and dopamine. Release of the hormones epinephrine and norepinephrine from the adrenal medulla of the adrenal glands is part of the fight-or-flight response. Tyrosine is created from phenylalanine by hydroxylation by the enzyme phenylalanine hydroxylase. Tyrosine is also ingested directly from dietary prote ...
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