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von Hippelova-Lindauova nemoc

A rare inherited disorder in which blood vessels grow abnormally in the eyes, brain, spinal cord, adrenal glands, or other parts of the body. People with von Hippel-Lindau syndrome have a higher risk of developing some types of cancer.
NCI

An autosomal dominant disorder caused by mutations in a tumor suppressor gene. This syndrome is characterized by abnormal growth of small blood vessels leading to a host of neoplasms. They include HEMANGIOBLASTOMA in the RETINA; CEREBELLUM; and SPINAL CORD; PHEOCHROMOCYTOMA; pancreatic tumors; and renal cell carcinoma (see CARCINOMA, RENAL CELL). Common clinical signs include HYPERTENSION and neurological dysfunctions.
MSH

autosomal dominant disorder associated with cerebellar and retinal neoplasms; the most common manifestations are neurologic deficits associated with intracranial hemangioblastomas which may hemorrhage, causing ataxia, intracranial hypertension, and other signs of neurologic dysfunction.
CSP

Von Hippel-Lindau disease (VHL) is a rare, genetic disease that causes tumors and cysts to grow in your body. The tumors can be either cancerous or benign. They can grow in your brain and spinal cord, kidneys, pancreas and, in men, their genital tract. Symptoms of VHL vary and depend on the size and location of the tumors. They may include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems and high blood pressure.

Detecting and treating VHL early is important. Treatment usually involves surgery or sometimes radiation therapy. The goal is to treat growths while they are small and before they do permanent damage.

NIH: National Institute of Neurological Disorders and Stroke


MEDLINEPLUS

An inherited familial cancer syndrome which is characterized by development of capillary hemangioblastomas of the central nervous system and retina; clear cell renal carcinoma; pheochromocytoma; pancreatic tumors; and inner ear tumors. The syndrome is associated with germline mutations of the VHL tumor suppressor gene, located on chromosome 3p25-26. Symptoms of VHL syndrome may not be apparent until the third decade of life. CNS hemangioblastoma is the most common cause of death, followed by clear cell renal cell carcinoma. –2004
NCI

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hipokampus

A curved elevation of gray matter extending the entire length of the floor of the temporal horn of the lateral ventricle. The hippocampus, subiculum, and DENTATE GYRUS constitute the hippocampal formation. Sometimes authors include the ENTORHINAL CORTEX in the hippocampal formation.
MSH

a layer of gray matter lying along the floor of the lateral ventricle of the brain, comprised of cholinergic and possibly glutamatergic fibers, believed to be the critical brain structure underlying learning and memory.
CSP

a kind of brain structure
CHV

A curved gray matter structure of the temporal lobe lying on the floor of the lateral ventricle of the brain.
NCI

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Hippokratova přísaha

An oath, attributed to Hippocrates, that serves as an ethical guide for the medical profession.
MSH

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hipuráty

Salts and esters of hippuric acid.
MSH

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Hirschsprungova nemoc

Congenital MEGACOLON resulting from the absence of ganglion cells (aganglionosis) in a distal segment of the LARGE INTESTINE. The aganglionic segment is permanently contracted thus causing dilatation proximal to it. In most cases, the aganglionic segment is within the RECTUM and SIGMOID COLON.
MSH

abnormally large or dilated colon due to congenital absence of myenteric ganglion cells in a distal segment of the large bowel; resultant loss of motor function in this segment causes massive hypertrophic dilatation of the normal proximal colon; condition appears soon after birth; called also Hirschsprung`s disease, aganglionic megacolon and pelvirectal achalasia.
CSP

A congenital disorder characterized by the absence of myenteric ganglion cells in the distal colon. It results in a functional stenosis of the distal colon and a massive distention of the proximal colon.
NCI

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hirzutismus

A condition observed in WOMEN and CHILDREN when there is excess coarse body hair of an adult male distribution pattern, such as facial and chest areas. It is the result of elevated ANDROGENS from the OVARIES, the ADRENAL GLANDS, or exogenous sources. The concept does not include HYPERTRICHOSIS, which is an androgen-independent excessive hair growth.
MSH

excess hair in females and children with an adult male pattern of distribution.
CSP

A disorder characterized by the presence of excessive hair growth in women in anatomic sites where growth is considered to be a secondary male characteristic (beard, moustache, chest, abdomen), where hair growth is under androgen control.
NCI

A disorder characterized by the presence of excess hair growth in women in anatomic sites where growth is considered to be a secondary male characteristic and under androgen control (beard, moustache, chest, abdomen).
NCI

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Hirudin

A 65-residue polypeptide from LEECHES.
MSH

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Američané španělského původu

a person of Mexican, Puerto Rican, Cuban, Central or South American or other western hemisphere Hispanic culture or origin, regardless of race; index NTs instead where possible; for inhabitants of countries outside the United States of America index geographical region as well.
CSP

Persons living in the United States of Mexican (MEXICAN AMERICANS), Puerto Rican, Cuban, Central or South American, or other Spanish culture or origin. The concept does not include Brazilian Americans or Portuguese Americans.
MSH

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histaminasa

A group of enzymes including those oxidizing primary monoamines, diamines, and histamine. They are copper proteins, and, as their action depends on a carbonyl group, they are sensitive to inhibition by semicarbazide. EC 1.4.3.6.
MSH

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histamin

An amine derived by enzymatic decarboxylation of HISTIDINE. It is a powerful stimulant of gastric secretion, a constrictor of bronchial smooth muscle, a vasodilator, and also a centrally acting neurotransmitter.
MSH

depressor amine derived by enzymatic decarboxylation of histidine, present in the secretory granules of mast cells; a powerful stimulant of gastric secretion, a constrictor of bronchial smooth muscle, a vasodilator, and also a centrally acting neurotransmitter.
CSP

a chemical substance in the body that produces allergic reactions
CHV

A substance that has many effects in the body. It is released from some types of white blood cells during allergic reactions. It causes small blood vessels to dilate (widen) and become leaky, which can cause tissues to swell. It also causes smooth muscles to contract, gastric acid to be made, and the heart rate to increase. Histamine is used in tests for allergies, asthma, and gastric acid secretion. It is a type of neurotransmitter.
NCI

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histamin – antagonisté

Drugs that bind to but do not activate histamine receptors, thereby blocking the actions of histamine or histamine agonists. Classical antihistaminics block the histamine H1 receptors only.
MSH

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histamin H1 – antagonisté

Drugs that selectively bind to but do not activate histamine H1 receptors, thereby blocking the actions of endogenous histamine. Included here are the classical antihistaminics that antagonize or prevent the action of histamine mainly in immediate hypersensitivity. They act in the bronchi, capillaries, and some other smooth muscles, and are used to prevent or allay motion sickness, seasonal rhinitis, and allergic dermatitis and to induce somnolence. The effects of blocking central nervous system H1 receptors are not as well understood.
MSH

A type of drug that blocks the action of histamines, which can cause fever, itching, sneezing, a runny nose, and watery eyes. Antihistamines are used to prevent fevers in patients receiving blood transfusions and to treat allergies, coughs, and colds.
NCI

Any agent that binds to the histamine-1 (H-1) receptor, thereby antagonizing histamine mediated allergic reactions, such as bronchoconstriction, vasodilation and up-regulated capillary permeability.
NCI

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histamin H2 – antagonisté

Drugs that selectively bind to but do not activate histamine H2 receptors, thereby blocking the actions of histamine. Their clinically most important action is the inhibition of acid secretion in the treatment of gastrointestinal ulcers. Smooth muscle may also be affected. Some drugs in this class have strong effects in the central nervous system, but these actions are not well understood.
MSH

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histamin – uvolňování

secretion of histamine from mast cell and basophil granules by exocytosis; this can be initiated by a number of factors, all of which involve binding of IgE, cross-linked by antigen, to the mast cell or basophil`s Fc receptors; once released, histamine binds to a number of different target cell receptors and exerts a wide variety of effects.
CSP

The secretion of histamine from mast cell and basophil granules by exocytosis. This can be initiated by a number of factors, all of which involve binding of IgE, cross-linked by antigen, to the mast cell or basophil`s Fc receptors. Once released, histamine binds to a number of different target cell receptors and exerts a wide variety of effects.
MSH

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histamin-N-methyltransferasa

An enzyme that catalyzes the transfer of a methyl group from S-adenosylmethionine to histamine, forming N-methylhistamine, the major metabolite of histamine in man. EC 2.1.1.8.
MSH

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histidinamoniaklyasa

An enzyme that catalyzes the first step of histidine catabolism, forming UROCANIC ACID and AMMONIA from HISTIDINE. Deficiency of this enzyme is associated with elevated levels of serum histidine and is called histidinemia (AMINO ACID METABOLISM, INBORN ERRORS).
MSH

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histidin

An essential amino acid that is required for the production of HISTAMINE.
MSH

essential amino acid that contains the imidazole ring system.
CSP

Amino acid with side chain -CH2-C3H3N2.
NCI

An essential amino acid that is required for the production of HISTAMINE
CHV

A semi-essential amino acid (children should obtain it from food) needed in humans for growth and tissue repair, Histidine is important for maintenance of myelin sheaths that protect nerve cells and is metabolized to the neurotransmitter histamine. Histamines play many roles in immunity, gastric secretion, and sexual functions. Histidine is also required for blood cell manufacture and protects tissues against damage caused by radiation and heavy metals. (NCI04)
NCI

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histidindekarboxylasa

An enzyme that catalyzes the decarboxylation of histidine to histamine and carbon dioxide. It requires pyridoxal phosphate in animal tissues, but not in microorganisms. EC 4.1.1.22.
MSH

Histidine decarboxylase (662 aa, ~74 kDa) is encoded by the human HDC gene. This protein plays a role in the biosynthesis of histamine from histidine.
NCI

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histidinol

The penultimate step in the pathway of histidine biosynthesis. Oxidation of the alcohol group on the side chain gives the acid group forming histidine. Histidinol has also been used as an inhibitor of protein synthesis.
MSH

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histidinolfosfatasa

An enzyme that catalyzes the hydrolysis of histidinol-phosphate to histidinol. One of the regulatory enzymes in histidine biosynthesis. EC 3.1.3.15.
MSH

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histidin-tRNA-ligasa

An enzyme that activates histidine with its specific transfer RNA. EC 6.1.1.21.
MSH

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histiocyty

Macrophages found in the TISSUES, as opposed to those found in the blood (MONOCYTES) or serous cavities (SEROUS MEMBRANE).
MSH

A macrophage present in connective tissue.
NCI

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histiocytární poruchy maligní

Distinctive neoplastic disorders of histiocytes. Included are malignant neoplasms of MACROPHAGES and DENDRITIC CELLS.
MSH

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histiocytóza

General term for the abnormal appearance of histiocytes in the blood. Based on the pathological features of the cells involved rather than on clinical findings, the histiocytic diseases are subdivided into three groups: HISTIOCYTOSIS, LANGERHANS CELL; HISTIOCYTOSIS, NON-LANGERHANS-CELL; and HISTIOCYTIC DISORDERS, MALIGNANT.
MSH

A morphologic finding indicating tissue infiltration by non-neoplastic or neoplastic histiocytes.
NCI

a rare blood disease
CHV

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histiocytóza z Langerhansových buněk

A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder.
MSH

group of disorders of histiocyte proliferation which includes Letterer-Siwe disease; Hand-Schueller-Christian syndrome; and eosinophilic granuloma; Langerhans cells are components of the lesions.
CSP

A group of rare disorders in which too many Langerhans cells (a type of white blood cell) grow in certain tissues and organs including the bones, skin, and lungs, and damage them. Langerhans cell histiocytosis may also affect the pituitary gland (which makes hormones that control other glands and many body functions, especially growth). Langerhans cell histiocytosis is most common in children and young adults.
NCI

A multifocal, unisystem form of Langerhans-cell histiocytosis. There is involvement of multiple sites in one organ system, most frequently the bone. Patients are usually young children presenting with multiple destructive bone lesions.
NCI

A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001)
NCI

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histiocytóza maligní

A rare, usually progressive disorder comprising an invasive proliferation of neoplastic HISTIOCYTES. Many previous cases are now thought to represent ANAPLASTIC LARGE-CELL LYMPHOMA.
MSH

An antiquated term referring to cases of systemic non-Hodgkin lymphomas which are composed of large, atypical neoplastic lymphoid cells and cases of hemophagocytic syndromes. In the past, cases of anaplastic large cells lymphoma were called malignant histiocytosis.
NCI

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histiocytóza z non-Langerhansových buněk

Group of disorders which feature accumulations of active HISTIOCYTES and LYMPHOCYTES, but where the histiocytes are not LANGERHANS CELLS. The group includes HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; SINUS HISTIOCYTOSIS; xanthogranuloma; reticulohistiocytoma; JUVENILE XANTHOGRANULOMA; xanthoma disseminatum; as well as the lipid storage diseases (SEA-BLUE HISTIOCYTE SYNDROME; and NIEMANN-PICK DISEASES).
MSH

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histiocytóza sinusová

Benign, non-Langerhans-cell, histiocytic proliferative disorder that primarily affects the lymph nodes. It is often referred to as sinus histiocytosis with massive lymphadenopathy.
MSH

A morphologic finding indicating the presence of histiocytic infiltrates within distended lymph node sinuses.
NCI

A rare non-neoplastic disorder of unknown etiology characterized by distention of the lymph node sinuses and sinusoidal histiocytic infiltration. The histiocytes characteristically contain ingested lymphocytes. It is usually manifested with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia. . It can affect extranodal sites, including the skin, bones, and the respiratory tract. It usually regresses spontaneously.
NCI

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histokompatibilita

The degree of antigenic similarity between the tissues of different individuals, which determines the acceptance or rejection of allografts.
MSH

ability to accept grafts between individuals.
CSP

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histokompatibilita – antigeny

A group of antigens that includes both the major and minor histocompatibility antigens. The former are genetically determined by the major histocompatibility complex. They determine tissue type for transplantation and cause allograft rejections. The latter are systems of allelic alloantigens that can cause weak transplant rejection.
MSH

group of antigens that includes major histocompatibility antigens, genetically determined by the major histocompatibility complex, which determine tissue type for transplantation and cause allograft rejection; and minor histocompatibility antigens, systems of allelic alloantigens that can cause weak transplant rejection.
CSP

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