Loading...
Histiocytosis, Langerhans-Cell: 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.Histiocytosis: 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.Histiocytosis, Sinus: Benign, non-Langerhans-cell, histiocytic proliferative disorder that primarily affects the lymph nodes. It is often referred to as sinus histiocytosis with massive lymphadenopathy.Histiocytic Sarcoma: Malignant neoplasms composed of MACROPHAGES or DENDRITIC CELLS. Most histiocytic sarcomas present as localized tumor masses without a leukemic phase. Though the biological behavior of these neoplasms resemble lymphomas, their cell lineage is histiocytic not lymphoid.Histiocytes: Macrophages found in the TISSUES, as opposed to those found in the blood (MONOCYTES) or serous cavities (SEROUS MEMBRANE).Lymphatic Diseases: Diseases of LYMPH; LYMPH NODES; or LYMPHATIC VESSELS.Histiocytosis, Non-Langerhans-Cell: 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).Erdheim-Chester Disease: A rare form of non-Langerhans-cell histiocytosis (HISTIOCYTOSIS, NON-LANGERHANS-CELL) with onset in middle age. The systemic disease is characterized by infiltration of lipid-laden macrophages, multinucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones.Eosinophilic Granuloma: The most benign and common form of Langerhans-cell histiocytosis which involves localized nodular lesions predominantly of the bones but also of the gastric mucosa, small intestine, lungs, or skin, with infiltration by EOSINOPHILS.Sea-Blue Histiocyte Syndrome: A congenital disease caused by an inborn error involving APOLIPOPROTEINS E leading to abnormal LIPID METABOLISM and the accumulation of GLYCOSPHINGOLIPIDS, particularly SPHINGOMYELINS in the HISTIOCYTES. This disorder is characterized by SPLENOMEGALY and the sea-blue histiocytes in the spleen and bone marrow after May Grunwald staining.Xanthogranuloma, Juvenile: Benign disorder of infants and children caused by proliferation of HISTIOCYTES, macrophages found in tissues. These histiocytes, usually lipid-laden non-Langerhans cells, form multiple yellow-red nodules most often in the skin, the eye, and sometimes in the viscera. Patients appear to have normal lipid metabolism and are classified as a normolipemic non-Langerhans cell histiocytosis.Cranial Sinuses: Large endothelium-lined venous channels situated between the two layers of DURA MATER, the endosteal and the meningeal layers. They are devoid of valves and are parts of the venous system of dura mater. Major cranial sinuses include a postero-superior group (such as superior sagittal, inferior sagittal, straight, transverse, and occipital) and an antero-inferior group (such as cavernous, petrosal, and basilar plexus).Maxillary Sinus: The air space located in the body of the MAXILLARY BONE near each cheek. Each maxillary sinus communicates with the middle passage (meatus) of the NASAL CAVITY on the same side.Paranasal Sinuses: Air-filled spaces located within the bones around the NASAL CAVITY. They are extensions of the nasal cavity and lined by the ciliated NASAL MUCOSA. Each sinus is named for the cranial bone in which it is located, such as the ETHMOID SINUS; the FRONTAL SINUS; the MAXILLARY SINUS; and the SPHENOID SINUS.Sphenoid Sinus: One of the paired air spaces located in the body of the SPHENOID BONE behind the ETHMOID BONE in the middle of the skull. Sphenoid sinus communicates with the posterosuperior part of NASAL CAVITY on the same side.Cavernous Sinus: An irregularly shaped venous space in the dura mater at either side of the sphenoid bone.Sinus of Valsalva: The dilatation of the aortic wall behind each of the cusps of the aortic valve.Orbital Diseases: Diseases of the bony orbit and contents except the eyeball.Carotid Sinus: The dilated portion of the common carotid artery at its bifurcation into external and internal carotids. It contains baroreceptors which, when stimulated, cause slowing of the heart, vasodilatation, and a fall in blood pressure.Bone Diseases: Diseases of BONES.Langerhans Cells: Recirculating, dendritic, antigen-presenting cells containing characteristic racket-shaped granules (Birbeck granules). They are found principally in the stratum spinosum of the EPIDERMIS and are rich in Class II MAJOR HISTOCOMPATIBILITY COMPLEX molecules. Langerhans cells were the first dendritic cell to be described and have been a model of study for other dendritic cells (DCs), especially other migrating DCs such as dermal DCs and INTERSTITIAL DENDRITIC CELLS.Frontal Sinus: One of the paired, but seldom symmetrical, air spaces located between the inner and outer compact layers of the FRONTAL BONE in the forehead.Paranasal Sinus Diseases: Diseases affecting or involving the PARANASAL SINUSES and generally manifesting as inflammation, abscesses, cysts, or tumors.Coronary Sinus: A short vein that collects about two thirds of the venous blood from the MYOCARDIUM and drains into the RIGHT ATRIUM. Coronary sinus, normally located between the LEFT ATRIUM and LEFT VENTRICLE on the posterior surface of the heart, can serve as an anatomical reference for cardiac procedures.Diabetes Insipidus: A disease that is characterized by frequent urination, excretion of large amounts of dilute URINE, and excessive THIRST. Etiologies of diabetes insipidus include deficiency of antidiuretic hormone (also known as ADH or VASOPRESSIN) secreted by the NEUROHYPOPHYSIS, impaired KIDNEY response to ADH, and impaired hypothalamic regulation of thirst.Vulvar Diseases: Pathological processes of the VULVA.Histiocytic Disorders, Malignant: Distinctive neoplastic disorders of histiocytes. Included are malignant neoplasms of MACROPHAGES and DENDRITIC CELLS.Sinus Thrombosis, Intracranial: Formation or presence of a blood clot (THROMBUS) in the CRANIAL SINUSES, large endothelium-lined venous channels situated within the SKULL. Intracranial sinuses, also called cranial venous sinuses, include the superior sagittal, cavernous, lateral, petrous sinuses, and many others. Cranial sinus thrombosis can lead to severe HEADACHE; SEIZURE; and other neurological defects.Antigens, CD1: Glycoproteins expressed on cortical thymocytes and on some dendritic cells and B-cells. Their structure is similar to that of MHC Class I and their function has been postulated as similar also. CD1 antigens are highly specific markers for human LANGERHANS CELLS.Sick Sinus Syndrome: A condition caused by dysfunctions related to the SINOATRIAL NODE including impulse generation (CARDIAC SINUS ARREST) and impulse conduction (SINOATRIAL EXIT BLOCK). It is characterized by persistent BRADYCARDIA, chronic ATRIAL FIBRILLATION, and failure to resume sinus rhythm following CARDIOVERSION. This syndrome can be congenital or acquired, particularly after surgical correction for heart defects.Ethmoid Sinus: The numerous (6-12) small thin-walled spaces or air cells in the ETHMOID BONE located between the eyes. These air cells form an ethmoidal labyrinth.Mucinoses: Mucoid states characterized by the elevated deposition and accumulation of mucin (mucopolysaccharides) in dermal tissue. The fibroblasts are responsible for the production of acid mucopolysaccharides (GLYCOSAMINOGLYCANS) in the ground substance of the connective tissue system. When fibroblasts produce abnormally large quantities of mucopolysaccharides as hyaluronic acid, chondroitin sulfate, or heparin, they accumulate in large amounts in the dermis.Hypothalamic Diseases: Neoplastic, inflammatory, infectious, and other diseases of the hypothalamus. Clinical manifestations include appetite disorders; AUTONOMIC NERVOUS SYSTEM DISEASES; SLEEP DISORDERS; behavioral symptoms related to dysfunction of the LIMBIC SYSTEM; and neuroendocrine disorders.Granuloma, Lethal Midline: A condition that is characterized by inflammation, ulceration, and perforation of the nose and the PALATE with progressive destruction of midline facial structures. This syndrome can be manifested in several diseases including the nasal type of EXTRANODAL NK-T-CELL LYMPHOMA and GRANULOMATOSIS WITH POLYANGIITIS.Exophthalmos: Abnormal protrusion of both eyes; may be caused by endocrine gland malfunction, malignancy, injury, or paralysis of the extrinsic muscles of the eye.Paranasal Sinus Neoplasms: Tumors or cancer of the PARANASAL SINUSES.Tomography, X-Ray Computed: Tomography using x-ray transmission and a computer algorithm to reconstruct the image.Fatal Outcome: Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept.Skin DiseasesRemission, Spontaneous: A spontaneous diminution or abatement of a disease over time, without formal treatment.Tachycardia, Sinus: Simple rapid heartbeats caused by rapid discharge of impulses from the SINOATRIAL NODE, usually between 100 and 180 beats/min in adults. It is characterized by a gradual onset and termination. Sinus tachycardia is common in infants, young children, and adults during strenuous physical activities.Biopsy: Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.Nucleoside Transport Proteins: Proteins involved in the transport of NUCLEOSIDES across cellular membranes.Polydipsia: Excessive thirst manifested by excessive fluid intake. It is characteristic of many diseases such as DIABETES MELLITUS; DIABETES INSIPIDUS; and NEPHROGENIC DIABETES INSIPIDUS. The condition may be psychogenic in origin.Maxillary Sinus Neoplasms: Tumors or cancer of the MAXILLARY SINUS. They represent the majority of paranasal neoplasms.Lymph Nodes: They are oval or bean shaped bodies (1 - 30 mm in diameter) located along the lymphatic system.Mastoiditis: Inflammation of the honeycomb-like MASTOID BONE in the skull just behind the ear. It is usually a complication of OTITIS MEDIA.Lung Diseases: Pathological processes involving any part of the LUNG.Scleral Diseases: General disorders of the sclera or white of the eye. They may include anatomic, embryologic, degenerative, or pigmentation defects.S100 Proteins: A family of highly acidic calcium-binding proteins found in large concentration in the brain and believed to be glial in origin. They are also found in other organs in the body. They have in common the EF-hand motif (EF HAND MOTIFS) found on a number of calcium binding proteins. The name of this family derives from the property of being soluble in a 100% saturated ammonium sulfate solution.Parietal Bone: One of a pair of irregularly shaped quadrilateral bones situated between the FRONTAL BONE and OCCIPITAL BONE, which together form the sides of the CRANIUM.Pilonidal Sinus: A hair-containing cyst or sinus, occurring chiefly in the coccygeal region.Penile Diseases: Pathological processes involving the PENIS or its component tissues.Vinblastine: Antitumor alkaloid isolated from Vinca rosea. (Merck, 11th ed.)Alcohol Amnestic Disorder: A mental disorder associated with chronic ethanol abuse (ALCOHOLISM) and nutritional deficiencies characterized by short term memory loss, confabulations, and disturbances of attention. (Adams et al., Principles of Neurology, 6th ed, p1139)Forehead: The part of the face above the eyes.Dog Diseases: Diseases of the domestic dog (Canis familiaris). This term does not include diseases of wild dogs, WOLVES; FOXES; and other Canidae for which the heading CARNIVORA is used.Transverse Sinuses: The two large endothelium-lined venous channels that begin at the internal occipital protuberance at the back and lower part of the CRANIUM and travels laterally and forward ending in the internal jugular vein (JUGULAR VEINS). One of the transverse sinuses, usually the right one, is the continuation of the SUPERIOR SAGITTAL SINUS. The other transverse sinus is the continuation of the straight sinus.Intestinal Neoplasms: Tumors or cancer of the INTESTINES.Hypoaldosteronism: A congenital or acquired condition of insufficient production of ALDOSTERONE by the ADRENAL CORTEX leading to diminished aldosterone-mediated synthesis of Na(+)-K(+)-EXCHANGING ATPASE in renal tubular cells. Clinical symptoms include HYPERKALEMIA, sodium-wasting, HYPOTENSION, and sometimes metabolic ACIDOSIS.Lymphadenitis: Inflammation of the lymph nodes.Orbital Neoplasms: Neoplasms of the bony orbit and contents except the eyeball.Cladribine: An antineoplastic agent used in the treatment of lymphoproliferative diseases including hairy-cell leukemia.Pneumothorax: An accumulation of air or gas in the PLEURAL CAVITY, which may occur spontaneously or as a result of trauma or a pathological process. The gas may also be introduced deliberately during PNEUMOTHORAX, ARTIFICIAL.Spinal DiseasesHistiocytoma, Benign Fibrous: A benign tumor composed, wholly or in part, of cells with the morphologic characteristics of HISTIOCYTES and with various fibroblastic components. Fibrous histiocytomas can occur anywhere in the body. When they occur in the skin, they are called dermatofibromas or sclerosing hemangiomas. (From DeVita Jr et al., Cancer: Principles & Practice of Oncology, 5th ed, p1747)Prednisone: A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver.Superior Sagittal Sinus: The long large endothelium-lined venous channel on the top outer surface of the brain. It receives blood from a vein in the nasal cavity, runs backwards, and gradually increases in size as blood drains from veins of the brain and the DURA MATER. Near the lower back of the CRANIUM, the superior sagittal sinus deviates to one side (usually the right) and continues on as one of the TRANSVERSE SINUSES.Brain Diseases: Pathologic conditions affecting the BRAIN, which is composed of the intracranial components of the CENTRAL NERVOUS SYSTEM. This includes (but is not limited to) the CEREBRAL CORTEX; intracranial white matter; BASAL GANGLIA; THALAMUS; HYPOTHALAMUS; BRAIN STEM; and CEREBELLUM.Diabetes Insipidus, Neurogenic: A genetic or acquired polyuric disorder caused by a deficiency of VASOPRESSINS secreted by the NEUROHYPOPHYSIS. Clinical signs include the excretion of large volumes of dilute URINE; HYPERNATREMIA; THIRST; and polydipsia. Etiologies include HEAD TRAUMA; surgeries and diseases involving the HYPOTHALAMUS and the PITUITARY GLAND. This disorder may also be caused by mutations of genes such as ARVP encoding vasopressin and its corresponding neurophysin (NEUROPHYSINS).