|NOTE,"Medical Eponyms. April 17, 2003, ver 1.681 © Andrew J. Yee, M.D. yee@post.harvard.edu http://eponyms.net.",,, "Adamkiewicz, artery of",artery responsible for anterior spinal syndrome; can be severed in AAA repair,,, Adamson’s fringe,"in tinea capitis, the location of the terminal tuft of hyphae; weakest point of hair located just about Adamson’s fringe",,, Addison’s disease,"primary adrenal insufficiency, bilateral adrenal destruction by TB used to be most common cause, now only accounts for 7-20% of cases; now autoimmune disease 70-90%, remainder caused by infectious disease, mets or lymphoma, adrenal hemorrhage, infarction, or drugs",,, Adie’s pupil,"tonic pupil, larger than contralateral unaffected pupil, reacts sluggishly to changes in illumination, see also Holmes-Adie syndrome; seen in young women; no neurologic significance",,, Adson’s sign,"in thoracic outlet syndrome, decrease in ipsilateral radial pulse and/or presence of subclavian bruit while patient extending neck maximally, rotating head towards side being tested, and holding breath",,, Ahumada-del Castillo syndrome,galactorrhea-amenorrhea not associated with pregnancy,,, Aicardi syndrome,"partial or complete agenesis of corpus callosum, infantile spasms, mental retardation, lacunae of the retina; occurs only in females; likely X-linked dominant with lethality in males; described in 1965",,, Alagille syndrome,"inherited cholestatic syndrome, associated with biliary hypoplasia (ductopenia), vertebral anomalies, prominent forehead, deep-set eyes, peripheral pulmonic stenosis",,, Albers-Schönberg disease,osteopetrosis or marble bone disease,,, Albright’s hereditary osteodystrophy,"polyostotic fibrous dysplasia, short stature, round face, skeletal anomalies (brachydactyly, also see Archibald’s sign), and heterotopic calcification, precocious puberty, café-au-lait spots on skin, low calcium, high phosphate, resistance to elevated PTH levels from mutation in Gsalpha coupling PTH receptor to adenylyl cyclase",,, Alder-Reilly anomaly,"large, dark, pink-purple granules in cytoplasm of neutrophils; autosomal recessive trait resulting in abnormal granule development in neutrophils resembling severe toxic granulation",,, Alexander’s disease,leukodystrophy-like neurodegenerative disease presenting in infancy or childhood; characterized by Rosenthal fibers,,, Alexander’s law,"peripheral vestibular nystagmus increases in intensity when the gaze is in the direction of the fast phase, and decreases in intensity when the gaze is away from the fast phase",,, Allen’s sign,"in pulmonary embolism, fever, tachycardia, and tachypnea, present in only 23% of cases",,, Allen’s test,"for demonstrating patent ulnar artery and an intact superficial palmar arch, patient’s hand is initially held high while fist is clenched and both radial and ulnar arteries are compressed, hand then lowered and fist is opened; after ulnar pressure released, color should return to the hand within 6 seconds",,, Alport’s syndrome,"hereditary nephritis accompanied by nerve deafness and various eye disorders, including lens dislocation, posterior cataracts, and corneal dystrophy; a few families have large platelets (20-25 fL), thrombocytopenia, and leukocyte inclusions which resemble May-Hegglin anomaly",,, Alström’s syndrome,"obesity, autosomal recessive, childhood blindness due to retinal degeneration, nerve deafness, vasopressin-resistant diabetes insipidus, and in males, hypogonadism with high plasma gonadotropin levels (end organ resistance to hormones), also baldness, hyperuricemia, hypertriglyceridemia, aminoaciduria",,, Alzheimer’s disease,"most frequent cause of dementia, pathologically characterized by neurofibrillary tangles, neuritic plaques, and granulovacuolar degeneration, degeneration of nucleus basalis of Meynert (principal origin of cholinergic innervation); described in 1906",,, Amadori product,products of early non enzymatic glycoslyation of proteins,,, Andersen disease,"type IV glycogen storage disease (or amylopectinosis), from branching enzyme deficiency, presents with cirrhosis with hepatosplenomegaly and failure to thrive in the first 18 months of life",,, Andersen’s syndrome,"periodic muscle paralysis, arrhythmias, short stature, macrocephaly, hypertelorism, brachydactyly, tapering fingers; from mutation in KCNJ2 which encodes a potassium channel Kir2.1; first described in 1971",,, Angel’s sign,performing an otoscopic exam while patient is blowing against a pinched nose; increases sensitivity of otoscopy for the detection of a perforated tympanic membrane by demonstrating fluid or pus in the external canal; described in 1994,,, Angelman syndrome,"“happy puppet” syndrome characterized by severe developmental delay, frequent laughing, easily excitable personality; from maternal deletion of 15q11-13; associated with mutation in maternally-imprinted ATP10C, a putative aminophospholipid translocase",,, Anitschkow myocytes,"in rheumatic fever, large mesenchymal cells in myocardial lesion",,, Anton’s syndrome,denial of cortical blindness; a form of anosognosia,,, Apert syndrome,"autosomal dominant disorder from mutation in FGFR2 characterized by craniosynostosis, mid-facial malformations, symmetric bony syndactyly of hands and feet, and varying degrees of mental retardation, from missense mutations in the fibroblast growth-factor-receptor 2 (FGFR2) genes",,, Apgar score,"developed in 1952, five parameters assessed: heart rate, respiratory effort, muscle tone, reflex irritability, and color; score 7 or greater said to be indicator of good health; 5 minute score most reliable; 5 min score of 0-3, mortality 244/1000 v. score of 7-10, mort 0.2/1000 (N Engl J Med 2001;344:467)",,, Apley grind test,"for meniscal tears, flex patient’s leg to 90 degrees, then grind the tibial condyles against the femoral condyles with rotation motion in the varus and then valgus positions",,, Apley scratch test,"asking patient to scratch the back from above or bottom, looking for furthest point reached (T4-T5 former, T7-T8 latter normal); if can’t reach, infraspinatus/teres minor tendonitis tear or subscapularis tendonitis/tear respectively",,, Apley’s law,"in pediatrics, the farther a chronically recurrent abdominal pain is from the umbilicus, the greater the likelihood of an organic cause for the pain",,, Apt test,"test which differentiates fetal from maternal hemoglobin in infant’s gastric contents, vomitus, or stool",,, Archibald’s sign,"in pseudohypoparathyroidism of Albert’s hereditary osteodystrophy, characteristic shortening of the fourth and fifth digits as dimpling over the knuckles of a clenched fist",,, Argyll Robertson pupils,"small irregular pupils, usually but not always caused by CNS syphilis, they accommodate, but don’t react; absence of miotic reaction to light, both direct and consensual, with preservation of a miotic reaction to near stimulus; lesion in tectum of midbrain; named after Douglas M. C. L. Argyll Robertson.",,, Arnold’s nerve,auricular branch of vagus nerve supplying posterior and inferior meatal skin of ear; stimulation can elicit cough reflex,,, Arnold’s reflux,ear cough reflux mediated by Arnold’s nerve,,, Arnold-Chiari malformation,"downward displacement of the cerebellar tonsils and medulla through the foramen magnum, results in pressure atrophy of displaced brain tissue, hydrocephalus from obstruction of the CSF outflow tract, is almost always characterized by the presence of a thoracolumbar meningomyelocele",,, Arthus reaction,localized area of tissue necrosis resulting from acute immune complex vasculitis; type III hypersensitivity,,, Aschoff body,"pathognomonic finding in rheumatic carditis, an area of local fibrinoid necrosis surrounded by inflammatory cells including lymphocytes, plasma cells, and macrophages that later resolve to fibrous scar tissue",,, Asherman’s syndrome,"intrauterine synechiae, typically occurs after curettage of the uterus, presenting as amenorrhea",,, Ashman’s phenomenon,"relationship of aberrancy to changes in the preceding cycle length; may persist for several cycles, usually exhibits RBBB morphology",,, ASPEN syndrome,"Association of Sickle cell disease, Priapism (usually major), Exchange transfusion, Neurological events including headache, lethargy, and seizures; may be due to abrupt cerebral hypoxemia from too-rapid exchange or release of vasoactive cytokines released from penile blood vessels; described in J Urol. 1993; 150:1480-1482.",,, Asperger’s disorder,"severe and sustained impairment in social interactions and the development of restricted, repetitive patterns of behavior, interests, and activities, but intellectually normal and no language delays, but with abnormalities of spoken language",,, Auenbrugger’s sign,epigastric bulge due to a massive pericardial effusion,,, Auer rods,"present in acute myelogenous leukemia or refractory anemia with excess blasts; granules form elongated needles, granules are all azurophilic, contain peroxidase; fused lysosomes",,, Auerbach’s plexus,"myenteric plexus, between the longitudinal and circular layers of muscle; provides motor innervation to the two muscle layers and secretomotor innervation to the mucosa",,, Auspitz’s sign,"for psoriasis; sign is positive when slight scratching or curetting of a scaly lesion reveals punctate bleeding points within the lesion; suggests psoriasis, but is not specific",,, Austin Flint murmur,"diastolic rumble in aortic regurgitation heard at cardiac apex, thought to be due to aortic jet impinging on the mitral valve, causing vibrations and also from simultaneous diastolic filling of the left ventricle from the left atrium and aorta closing the mitral valve in diastole, producing physiologic stenosis; named after Austin Flint (1812-1886)",,, Australia antigen,"HBsAg, found in the serum of an Australian aborigine",,, Austrian triad,"clinical triad of pneumococcal pneumonia, meningitis, and endocarditis (classically aortic valve endocarditis associated with aortic regurgitation); described by Robert Austrian",,, Babinski sign,"upper motor sign that indicates dysfunction of fibers within the pyramidal system; described in 1896 by Babinski, student of Charcot",,, Bachmann’s bundle,anterior internodal tract in atrial conduction system,,, Bainbridge reflex,compensatory increase in HR cause by a rise in right atrial pressure,,, Baker’s cyst,"popliteal cyst, a fluid-filled mass within the popliteal fossa",,, Balint’s syndrome,"optic ataxia (inability to visually guide limb movements), ocular ataxia (inability to direct eyes to a precise point in the visual field), inability to enumerate objects in a picture or extract meaning from a picture, and inability to avoid objects in one’s path, simultanagnosia, from infarction in unilateral or bilateral visual association due to watershed stroke between distal PCA and MCA",,, Balkan nephropathy,"degenerative interstitial nephropathy seen in Balkan areas (tributaries of Danube River), see tubular proteinuria, glycosuria, RTA, azotemia, associated with increased risk of upper tract transitional cell carcinoma",,, Ball’s disease,"intracerebral leukocytostasis, potentially fatal complication of acute leukemia (especially AML) when peripheral blast cell count >100,000/uL; leukemic cells capable of invading through endothelium and causing hemorrhage into brain; not generally seen with CLL or CML",,, Ballance’s sign,tender mass in the LUQ due to a spleen hematoma,,, Baló’s disease,"variant of multiple sclerosis, see concentric rings of demyelination separated by bands of preserved myelin",,, Baltic myoclonus,see Unverricht-Lundborg disease,,, bamboo spine,seen in ankylosing spondylitis,,, Bancroft’s sign,"in deep vein thrombosis, compression of calf forward against tibia causing more pain than horizontal compression when gastrocnemius muscle is lifted; also referred to as Moses’s sign",,, Bang’s disease,brucellosis,,, Bannayan-Ruvalcaba-Riley syndrome,see Bannayan-Zonana syndrome,,, Bannayan-Zonana syndrome,"hamartoma syndrome characterized by macrocephaly, multiple lipomas, and hemangiomas, speckled penis; associated with with germ line PTEN mutations; also known as Bannayan-Ruvalcaba-Riley syndrome ",,, Bannwarth’s syndrome,"in early disseminated Lyme disease, triad of lymphocytic meningitis, cranial nerve palsies (especially VII nerve which may be bilateral), and radiculoneuritis",,, Banti’s syndrome,"splenomegaly, hypersplenism, and portal hypertension, noncirrhotic, arises after subclinical occlusion of the portal vein, usually years after occlusive event",,, Bantu siderosis,unusual form of iron overloading resembling hereditary hemochromatosis in South African blacks ingesting large quantities of alcoholic beverages fermented in iron utensils,,, Bárány test,see Dix-Hallpike test,,, Bardet-Biedl syndrome,"mental retardation, pigmentary retinopathy, polydactyly, obesity, and hypogenitalism; genetically heterogeneous disorder with linkage to 7 loci; has been incorrectly called Laurence-Moon-Bardet-Biedl syndrome in the past",,, Barlow’s disease,mitral valve prolapse,,, Barlow’s maneuver,"for congenital hip dislocation, patient placed in supine position and attempt made to push femurs posteriorly with knees at 90 degrees/hip flexed and hip will dislocate",,, Barraquer-Simons syndrome,"acquired partial lipodystrophy; presents usually around 8-10, preceded generally by an acute viral infection; spares legs and hips; 1/3 of patients develop membranoproliferative glomerulonephritis",,, Barrett’s esophagus,esophageal strictures and epithelial metaplasia from squamous epithelium to a specialized columnar epithelium with intestinal metaplasia in 10% of severe GERD,,, Barth’s syndrome,"infantile X-linked dilated cardiomyopathy, short stature, myopathy, neutropenia, from mutation in G4.5 which encodes for tafazzins",,, Bartter’s syndrome,"hypokalemic, hypochloremic metabolic alkalosis with normal or low blood pressure despite increased renin and aldosterone levels and hyperplasia of juxtaglomerular apparatus due to mutations affecting diuretic-sensitive sodium-transport proteins",,, Bassen-Kornzweig syndrome,congenital abetalipoproteinemia,,, bat wing edema,pulmonary edema in perihilar distribution in approximately 5% of cases ,,, Bateman’s senile purpura,purpura following trauma to severely sun-damaged skin of the dorsal forearm of elderly persons; months may be required for resolution of pigmentation from hemorrhage,,, Batista procedure,"for treatment of heart failure, removal of portion of left ventricular free wall, i.e. partial left ventriculectomy",,, Batson's plexus,"portal vertebral venous communications, may be responsible for isolated bone metastases in sacrum or vertebral bodies from colorectal cancer",,, Batten’s disease,"later-onset ceroid lipofuscinosis, CLN3, also used to describe juvenile form specifically; in general a group of conditions characterized by mental impairment, worsening seizures, and progressive loss of sight and motor skills related to buildup of lipopigments",,, Battle’s sign,"ecchymoses over the mastoid process in basilar skull fractures, generally occurring approximately 48 hours after event",,, Bazex’s syndrome,"acrokeratosis paraneoplastica, consists of erythematous to violaceous psoriaform plaques occurring predominantly in acral areas; associated with Hodgkin’s disease and squamous cell carcinomas of digestive tract",,, Bazin’s disease,"nodular vasculitis or erythema induratum, a form of panniculitis classically associated with tuberculosis characterized histologically by caseation necrosis; described by Bazin in 1861",,, BCG,"Bacille bilié de Calmette-Guérin; Leon A. Calmette, French bacteriologist, 1863-1933; Camille Guérin, French bacteriologist, 1872-1961; attenuated strain of Mycobacterium bovis bacille Calmette-Guérin",,, Beau’s lines,"horizontal depressions across nail plate seen as nail grows out, caused by a transient arrest in nail growth, can occur during acute stress (e.g., high fever, circulatory shock, myocardial infarction, pulmonary embolism)",,, Bechterew’s disease,ankylosing spondylitis,,, Beck’s triad,"in pericardial tamponade, distended neck veins, distant heart sounds, hypotension, i.e. rising venous pressure, falling arterial pressure, and decreased heart sounds, Calude S. Beck thoracic surgeon 1935",,, Becker’s muscular dystrophy,"X-linked, normal levels of dystrophin but function altered, average onset 11 y.o., age at death 42 y.o., CK elevated",,, Becker’s sign,"in aortic regurgitation, visible pulsations of the retinal arterioles",,, Beckwith-Wiedemann syndrome,"exomphalos, macroglossia, gigantism; associated with neonatal hypoglycemia",,, Beevor’s sign,"lesions of T9-T10 paralyze lower but spare upper abdominal muscles, resulting in upward movement of umbilicus when abdominal wall contracts",,, Behçet’s disease ,"triad of aphthous ulcers, genital ulcerations, and ocular inflammation (posterior uveitis). Associated with erythema nodosum, cutaneous pustular vasculitis, also synovitis, CNS vasculitis involving brain stem, thrombophlebitis, and positive pathergy response. Described by Turkish dermatologist Behçet in 1937; also known as Silk Road disease due to clustering of cases along the Silk Road.",,, Bell’s palsy,"seventh nerve palsy; seen as a complication in diabetes, tumors, sarcoidosis, AIDS, and Lyme disease",,, Bell’s phenomenon,physiological upward rotation of the eyeball triggered by contraction of the ipsilateral orbicularis muscle with resulting closure of the eyelid,,, Bence Jones proteins,"free Ig light chains seen in plasma cell dyscrasias (e.g. multiple myeloma, AL-amyloidosis, light-chain deposition disease, Waldenström’s macroglobulinemia, MGUS, heavy-chain disease (mu) (rare), lymphoproliferative disease (rare), rifampin therapy (rare), filtered by glomerulus and then reabsorbed tubular cells; proteins are toxic to tubule cells",,, Benedikt syndrome,"clinical picture from paramedian midbrain infarction from occlusion of the paramedian penetrating branches of the basilar artery affecting the third nerve root fiber, red nucleus, cerebral peduncle resulting in ipsilateral medial rectus palsy with a fixed dilated pupil and contralateral tremor, chorea, and athetosis",,, Bennett’s fracture,fracture of the base of the first metacarpal with involvement of carpometacarpal joint,,, Bentall procedure,"for treating ascending aortic aneurysms, composite prosthetic graft consisting of prosthetic aortic valve sewn onto end of graft",,, Berardinelli-Seip syndrome,"congenital generalized lipodystrophy, apparent at birth, infants look very muscular due to absence of fat, associated with diabetes, hepatomegaly, acanthosis nigricans, enlarged external genitalia, and increased rate of skeletal growth.",,, Berger’s disease,"IgA nephropathy, ESRD develops in 15% of cases at 10 years and 20% at 20 years, treated with steroids in certain instances",,, Bergman minimal model,"determinants of glucose disposal: phi-1 (acute insulin secretion), phi-2 (sustained insulin secretion), Si (insulin sensitivity), Sg (glucose sensitivity)",,, Bergman’s triad,seen with fat emboli syndrome: 1. mental status changes; 2. petechiae (often in the axilla/thorax); 3. dyspnea,,, Bergmann gliosis,"in ethanol abuse, proliferation of astrocytes adjacent to lost Purkinje cells between depleted granular cell and molecular layer of cerebellum",,, Bernard-Soulier disease,"inherited disorder of platelet function where there is absence of Gp Ib/IX, the von Willebrand receptor, characterized by giant platelets and greater than expected bleeding for the degree of thrombocytopenia",,, Bernheim effect,"in aortic stenosis, right ventricular failure preceding left ventricular failure from hypertrophied ventricular septum bulging into and encroaching on right ventricular filling",,, "Bernheim effect, reverse","in pulmonary embolism, right ventricular failure causing septum to bulge into and compromise left ventricular filling",,, Bernstein test,"to test for GERD, acid perfusion test of esophagus with 0.1 N HCl and see if reproduces chest pain; limited sensitivity and specificity though",,, Berry’s sign,"in malignant thyromegaly, absence of carotid pulsation from tumor encasing carotid and muffling pulsations",,, "Bertin, renal columns of",the spaces between adjacent pyramids where cortical tissue extends into,,, Best disease,"vitelliform macular dystrophy type 2 (VMD2), characterized by gradual loss of visual acuity starting in their teenage years; accumulation of lipofuscin in retinal pigment epithelium; markedly abnormal electro-oculogram (EOG) in all stages of progression and in phenotypically normal carriers; autosomal dominant, from mutation in VMD2 gene; first described in 1905",,, Betz cells,"large pyramidal cells in layer 5 of primary motor cortex largest neurons in mammalian CNS; 30-40,000 Betz cells in precentral gyrus in one side of the brain",,, Bezold’s abscess,abscess of mastoid tip,,, Bezold-Jarisch reflex,"activation of receptors in the atria, great veins, and left ventricle causing increased parasympathetic tone and decreased sympathetic activity leading to a combination of hypotension and bradycardia with a sudden increase in coronary flow",,, Bickerstaff’s encephalitis,brain stem encephalitis,,, Bielschowsky’s tilt test,"in trochlear nerve palsy (which paralyzes the superior oblique muscle), elevation in the affected eye is greatest when the head is tilted toward the side of the involved eye and abolished by tilt in the opposite direction",,, Bier block,regional anesthesia of an extremity by placing a tourniquet and then infusing local anesthetic into a vein,,, Biette’s collarette,"in syphilis, a thin white ring of scales on the surface of a lesion",,, Billroth I,antrectomy with gastroduodenostomy,,, Billroth II,antrectomy with gastrojejunostomy,,, Billroth’s cords,"splenic cords found in the red pulp between the sinusoids, consisting mainly of fibrils and connective tissue cells",,, Bing’s sign,extensor plantar response by pricking the dorsal surface of the big toe with a pin suggesting upper motor neuron defect,,, Bing-Horton syndrome,"erythroprosopalgia, attacks of facial pain associated with marked reddening of the ipsilateral half of the face associated with tearing and watery discharge from the nose, occurs during sleep and is of brief duration, believed to be due to irritaiton in greater petrosal nerve",,, Binswanger’s disease,"subcortical arteriosclerotic encephalopathy, associated with hypertension; characterized by multiple lacunar infarcts and progressive demyelination limited to the subcortical area with characteristic sparing of cortex",,, Biot’s breathing,"succession of hyperpnea/hyperventilations and apneas (seen in increased ICP, drug-induced respiratory depression, brain damage, usually medullary level), but lacks typical crescendo-decrescendo pattern, abrupt beginning, and regularity of Cheyne-Stokes breathing (see also Cheyne-Stokes, cerebral level)",,, Birbeck’s granules,aka Langerhans’s granules; a small tennis racket-shaped membrane-bound granule with characteristic cross-striated internal ultrastructure seen in Langerhans cell histiocytosis,,, Bishop’s score,"scoring system for determining whether or not induction of labor will be successful, based on 5 criteria (scored from 0-3): position, cervical consistency, degree of effacement, dilatation, and station; score >9 suggests that induction will be successful",,, Bitot’s spots,"in vitamin A deficiency, small, circumscribed, lusterless, grayish white, foamy, greasy, triangular deposits on the bulbar conjunctiva adjacent to the cornea in the area of the palpebral fissure of both eyes",,, Bjork-Shiley valve,"single tilting disk prosthetic valve, production stopped in 1986; large valves removed from market in October 1985 because of strut fracture",,, Blalock-Taussig shunt,"for treating tetralogy of Fallot, direct end-to-side subclavian artery to pulmonary artery anastomosis on side opposite to arch of aorta (classic); alternatively graft placed between subclavian artery and pulmonary artery (modified)",,, "Blaschkow, lines of","patterning of a variety of linear nevi as well as linear patterning of commonly acquired diseases such as psoriasis, scleroderma, and lichen planus, including V shape over the upper spine, S shape of the abdomen, inverted U shape from the breast area, and perpendicular lines down lower extremities; cannot be explained by distribution of cutaneous nerve, lines of cleavage, nor blood vessels or lymphatics; first described in 1901",,, Blau’s syndrome,"granulomatous synovitis, non granulomatous uveitis, cranial neuropathies; resembles childhood sarcoidosis; no lung involvement; associated with mutations in NOD2/CARD15 gene; first described in 1985",,, Blomstrand dysplasia,rare lethal disorder characterized by an increase in bone density and advanced skeletal maturationfrom inactivating mutation in PTHR-1 gene (see also Jansen metaphyseal chondrodysplasia where there is an activating mutation),,, Bloom’s syndrome,"severe immunodeficiency, growth retardation, progeria, and predisposition to several types of cancers associated with hypersensitivity to a variety of DNA-damaging agents",,, Blount’s disease,idiopathic varus bowing of tibia,,, Blumberg sign,rebound tenderness,,, Blumer shelf,"carcinomatous metastasis from a primary site high up in the peritoneal cavity, may accumulate in the stomach, felt through the anterior rectal wall as a hard shelf in the rectovesical or rectouterine pouch (pouch of Douglas)",,, Boas’s sign,"right subscapular pain due to cholelithiasis, <7% sensitive",,, bobble-head syndrome,"in children with progressive hydrocephalus, rapid, rhythmic bobbing of the head",,, Bochdalek's hernia,"hernia through the posterior diaphragm, usually on the left, presents in infancy",,, Bockhart’s impetigo,follicular impetigo,,, Boerhaave’s syndrome,pressure rupture of the esophagus; can give rise to Hamman’s sign,,, Bohr effect,fall in pH leading to decrease in oxygen affinity of hemoglobin,,, Bohr equation,"Vd/Vt, for determining ratio of physiologic dead space",,, Bonnet’s sign,banking of veins distal to AV crossings (grade 3) in hypertensive retinopathy; compare with Salus’s sign and Gunn’s sign,,, Bonnevie-Ullrich syndrome,"skeletal and soft tissue abnormalities (e.g., lymphedema of hands and feet, nail dystrophy, skin laxity), short stature, webbed neck.",,, Bordet-Gengou medium,"for identifying Bordetella pertussis, medium contains high percentage of blood (20-30%) to inactivate inhibitors in blood; also has potato and glycerol",,, Bornholm disease,"coxsackie virus producing pleurodynia, fever, cough, sore throat, myalgias in shoulder, chest, and abdomen; Bornholm is a Danish island in Baltic sea",,, Boston sign,"in thyrotoxicosis, jerking of the lagging lid",,, Bouchard’s nodes,bony spurs at PIP in osteoarthritis,,, Bourneville’s disease,tuberous scelrosis,,, Bowditch staircase,increased heart rate increases the strength of contraction in a stepwise fashion as the intracellular calcium increases over several beats,,, Bowen’s disease,"squamous cacrinoma in situ, seen generally on sun-exposed areas",,, Boxer’s fracture,"fracture of the metacarpal neck, classically of small finger",,, Bradbury-Eggleston syndrome,pure autonomic dysfunction characterized by low circulating catecholamines,,, Brandt-Daroff maneuver,"home treatment maneuvers for benign positional vertigo, used when office treatment maneuvers; series of provocative maneuvers done three sets per day for two weeks (Arch Otolaryngol 1980; 106:484-5)",,, Branham’s sign,bradycardia after compression of AV fistula,,, Brechenmacher fibers,tracts which connect the atrium to the His bundle,,, Bricker procedure,"creation of a urinary diversion by removing a piece of ileum, connecting the ureters to it, and then creating a stoma through the abdominal wall",,, Brill-Symmer’s disease,nodular lymphoma,,, Brill-Zinser disease,"recrudescent form of epidemic typhus (Rickettsiae prowazekii); occurs 10-50 years after primary infection; presents abrupty with chills, devers, headache, malaise; rash after 4-6 days after onset of symptoms",,, Briquet’s syndrome,somatization disorder,,, Broca’s area,"left frontal speech area, important for articulating speech; in Broca’s aphasia, because Broca’s area near motor cortex and underlying internal capsule, a right hemiparesis and homonymous hemianopsia is almost always present in this type of aphasia",,, Brockenbrough sign,"in hypertrophic cardiomyopathy, increase in murmur after a premature ventricular contraction, related to increase in LV systolic pressure, increase in systolic gradient between LV and aorta and consequent decrease in systolic pressure and pulse pressure",,, Brodie’s abscess,"small, intraosseus abscess that frequently involves the cortex and is walled off by reactive bone",,, Brodie-Trendelenburg test,for testing great saphenous vein and branches communicating with deep femoral vein; limb is elevated until drained of venous blood and then tourniquet is applied; normally arterial flow from below fills veins in about 35 s; however faster filling indicates incompetence of communicating vein,,, Brodman area,"neuro anatomical classification of the cortex based on its cyto architecture into 52 areas; e.g. precentral gyrus Brodman 4, striate cortex Brodman 17",,, Brown’s syndrome,"in rheumatoid arthritis, vertical diplopia, clicking sensation when looking up and medially, and an apparent inferior oblique palsy, apparently from stenosing tenosynovitis of the superior oblique tendon and sheath",,, Brown-Séquard syndrome,"loss of tactile sense, vibration sense, and limb position sense on the ipsilateral side and loss of pain and temperature sense on the contralateral side",,, Brudzinski sign,"after flexing the neck, flexion of hips and knees in reaction suggests meningeal inflammation",,, Brueghel syndrome,"dystonia of the motor trigeminal nerve producing a widely opened mouth, named after painting by Flemish painter Brueghel (Neurol 1996;46:1768)",,, Brugada syndrome,"defect in an ion channel gene resulting in abnormal electrophysiologic activity in the right ventricle and characterized by (1) ST segment elevation in V1-V3, (2) right bundle branch block, (3) sudden cardiac death, (4) grossly normal heart",,, Brunner’s glands,"in duodenum, submucosal mucous glands that secrete bicarbonate, glycoproteins, and pepsinogen II, virtually indistinguishable from pyloric mucous glands",,, Brushfield’s spots,"in Down’s syndrome, small white spots on the periphery of the iris",,, Bruton’s tyrosine kinase,mutation causes X-linked agammaglobulinemia (XLA also associated with defect in intact membrane-bound m chain (it’s essential for B-cell development) Btk found only in B cells,,, Budd-Chiari syndrome,"occlusion of the hepatic vein, associated with polycythemia vera, pregnancy, postpartum state, oral contraceptives, paroxysmal nocturnal hemoglobinuria, and intra-abdominal cancers, particularly hepatocellular carcinoma",,, Buerger’s disease,"thromboangiitis obliterans, a nonatherosclerotic segmental inflammatory disease that most commonly affects the small and medium-sized arteries, veins, and nerves of the arms and legs. Acute phase reactants normal; strong association with tobacco use",,, Buerger’s sign,"in peripheral vascular disease, red foot becomes pale with elevation",,, Bunina bodies,intraneuronal inclusions felt to be pathognomonic for motor neuron disease,,, Burkitt lymphoma,"tumor manifesting at extranodal sites; associated with translocation of c-myc gene on chr 8 with IgH locus (chr 14), kappa (chr 2), or lambda light-chain (chr 22) locus; associated with EBV infection in African variety",,, Burnett’s syndrome,"far-advanced milk-alkali syndrome, due to long-standing calcium and alkali ingestion; severe hypercalcemia, irreversible renal failure, and phosphate retention, may be accompanied by ectopic calcification; see also Cope’s syndrome",,, Burow’s solution,"aluminum acetate in water, invented in mid-1800s by ophthalmologist Karl Burow (a.k.a. by its tradename Domeboro)",,, Buruli ulcer,"caused by Mycobacterium ulcerans; painless nodule to large, undermined ulcerative lesions that heal spontaneously but slowly; named after Buruli region of Nile River",,, "Buschke, scleredema of","uncommon dermatosis characterized by thickened, indurated skin associated with diabetes, sometimes with erythema; also known as scleredema diabeticorum",,, Buschke-Löwenstein tumor,"verrucous carcinoma involving penile glans and prepuce, associated with HPV",,, Byler’s disease,"progressive familial intrahepatic cholestasis from impaired biliary secretion of both bile acids and phosphatidylcholine, leads to death from liver failure before adolescence",,, Cabot ring,"in asplenia or malfunctional spleen, nuclear remnants on red blood cells as a thin, darkly-stained ring that follows the margin of the red cell",,, Cabrera’s sign,"in left bundle branch block complicated by MI, notching at 0.05 s in ascending limb of S wave in V3, V4; 27% sens for MI",,, CADASIL,"cerebral autosomal dominant arteropathy with subcortical infarcts and leukoencephalopathy, rare hereditary cause of stroke that may involve Notch3 gene characterized by recurrent strokes (usually infarcts) and dementia",,, Cagot ear,absence of ear lobe; assocated with region in Pyrennes,,, caisson disease,decompression sickness,,, Calabar swellings,"in loiasis, swellings 5-10 cm in diameter from localized areas of angioedema associated with migration of adult Loa loa worms through subcutaneous tissues",,, Call-Exner bodies,"in granulosa cell tumors, small follicles filled with eosinophilic secretion; an important diagnostic feature",,, Call-Fleming syndrome,"sudden-onset severe headache, focal neurological deficits, and seizures; associated with serotonin modulating drugs like SSRIs",,, Calot’s triangle,"the area bordered by the 1. cystic duct, 2. common hepatic duct, 3. lower edge of the liver; cystic artery, sometimes hepatic artery found here",,, Cameron lesions,"erosions within incarcerated hiatal hernias, seen in 5.2% of patients with hiatal hernias",,, Campbell de Morgan spots,cherry angioma,,, Campbell diagram,"used to determine the work of breathing, including the effects of chest wall compliance, lung compliance, and airway resistance",,, Campbell’s sign,"in chronic airway obstruction, downward motion of trachea during inspiration, perhaps due to downward pull of diaphragm",,, Canale-Smith syndrome,"childhood disorder, first described in 1967, characterized by lymphadenopathy and autoimmunity; associated with mutations in Fas; implicates gene in accumulation of lymphocytes and the autoimmunity characteristic of the syndrome",,, Canavan disease,"autosomal recessive infantile spongy degeneration of the brain with Alzheimer type II cells, increased prevalence among Ashkenazi from mutation in aspartoacylase leading to increased levels of N-acetylaspartic acid, described in 1931",,, Cantlie’s line,separates the right and left lobes of the liver--a line drawn from the IVC to just left of the gallbladder fossa,,, "Cantrell, pentalogy of","diaphragmatic defect (hernia), cardiac abnormality, omphalocele, pericardium malformation/absence, sternal cleft",,, Capgras syndrome,delusional belief that one or a few highly familiar people have been replaced by impostors who are physically very similar to the originals; seen rarely in schizophrenia.,,, Caplan’s syndrome,"coexistence of rheumatoid arthritis with a pneumoconiosis, leading to the development of distinctive pulmonary lesions that develop fairly rapidly; these nodular lesions have central necrosis surrounded by fibroblasts, macrophages, and collagen; can occur in asbestosis and silicosis",,, Carabello’s sign,"in severe aortic stenosis, during catheterization across aortic valve, an augmentation of the peripheral systolic pressure of more than 5 mm Hg during aortic pullback; from catheter further increasing the severity of the stenosis and removal resulting in a transient increase in stroke volume and systolic pressure (Am J Cardiol 1979;44:424–7)",,, carcinoid triad,"1. flushing, 2. diarrhea, 3. right-sided heart failure (also bronchospasm)",,, Carey Coombs murmur,"a blubbering apical mid diastolic murmur occurring in the acute stages of rheumatic mitral valvulitis and disappearing as the valvulitis subsides; described by Carey F. Coombs, English physician, 1879-1932",,, Carnett’s test,"head raise, tenderness persists in abdominal wall condition (rectus hematoma) whereas pain due to intraperitoneal disease lessens",,, Carney complex,"AD complex of cardiac myxomas with aggressive biologic behavior, spotty pigmentation, and association with endocrine tumors (causing e.g., Cushing syndrome); mutations in PRKAR1alpha gene (a protein kinase) account for half of the cases; unrelated to Carney syndrome",,, Carney syndrome (or triad),"nonfamilial disorder the includes combination of three rare tumors: gastric leiomyosarcoma, pulmonary chondroma, extraadrenal paraganglioma but no cardiac manifestations; unrelated to Carney complex",,, Caroli’s disease,larger ducts of the intrahepatic biliary tree are segmentally dilated and may contain inspissated bile; pure forms are rare; this disease is usually associated with portal tract fibrosis of the congenital hepatic fibrosis type,,, Carpentier-Edwards valve,"porcine valve, pressure-fixed, preserved in glutaraldehyde, mounted on a Teflon-covered Elgiloy strut",,, Carrión’s disease,see Oroya fever,,, Carvallo’s sign,"in tricuspid regurgitation, murmur increases with inspiration",,, Castellani’s paint,"topical fungicidal and bactericidal mixture of resorcinol (8 g), acetone (4 mL), magenta (0.4 g), phenol (4 g), boric acid (0.8 g), industrial methylated spirit 90% (8.5 mL), and water (to 100 mL); particularly effective against intertriginous tinea and acute Candida paronychia; named after Sir Aldo Castellani",,, Castle intrinsic factor,"intrinsic factor secreted by parietal cells, which binds luminal B12 and permits its absorption in the ileum",,, Castleman’s disease,"lymphoproliferative disorder, either localized or multicentric; presents with massive lymphadenopathy, fever, splenomegaly, hepatomegaly; characterized by hyperplastic lymphoid follicles with capillary proliferation; associated with HHV 8; HIV-infected individuals at increased risk for multicentric Castleman’s disease; multicentric can progressive to lymphoma",,, cat-scratch disease,"tender regional lymphadenopathy persisting for 3 weeks or longer, frequently preceded by primary skin lesion after contact with kits, appears to be caused by Bartonella (formerly Rochalimaea) henslae, a small, pleomorphic gram-negative bacillus",,, Chaddock’s sign,involuntary dorsiflexion of the toes when tapping from the lateral malleolus distally to the lateral dorsum of the foot in upper motor neuron defect,,, Chadwick’s sign,blue-red passive hyperemia of the cervix that may appear after 7th week of pregnancy; may be seen in association with tumor; results from congestion of mucosa and most visible in anterior vaginal wall,,, Chagas’s disease,"zoonosis caused by protozoan parasite Trypanosoma cruzi; causes destruction of the myenteric plexus of the esophagus, duodenum, colon, and ureter, with resultant dilatation of these structures as well as cardiac disease; transmitted by excreta of hematophagous organisms of the family Reduiviidae",,, Chapman’s sign,"in left bundle branch block complicated by MI, notching of ascending limb of R in I, aVL, or V6",,, Charcot’s disease,French eponym for ALS,,, Charcot’s joints,"neurogenic joint degeneration, can be secondary to syphilis, peripheral neuropathy",,, Charcot’s triad,"in multiple sclerosis, nystagmus, intention tremor, and staccato speech (or scanning speech)",,, Charcot’s triad,"in 70% of patients with bacterial cholangitis, right-upper-quadrant pain, jaundice, and fever; see also Reynold’s pentad",,, Charcot-Bouchard aneurysms,"minute brain aneurysms from chronic hypertension, occurring in vessels less than 300 microns in diameter (different from saccular aneurysms), most commonly found within basal ganglia",,, Charcot-Leyden’s crystals,"crystals in the shape of elongated double pyramids, formed from eosinophils (from lysophospholipase), found in the sputum in bronchial asthma and in other exudates or transudates containing eosinophils",,, Charcot-Marie-Tooth disease,"most common inherited peripheral neuropathy, 1/2500, autosomal dominant, clinically heterogeneous disorder characterized by slowly progressive atrophy of the distal muscles, mainly those innervated by peroneal nerve; progressive weakness of varying intensity and atrophy of the muscles of the feet, hands, and legs, leading to pes cavus, clawhand, and stork-leg appearance, usually beginning in the 2nd or 3rd decade. Enlarged greater auricular nerves may be visible and enlarged ulnar and peroneal nerves may be palpated in some patients. Cranial nerves rarely involved",,, CHARGE assocation,"coloboma, heart malformation, atresia choanae, retarded growth and development, and/or CNS anomalies, genital hypoplasia, ear anomalies and/or deafness",,, Charles Bonnet syndrome,"visual deprivation hallucinations, generally occurring in visually-impaired individuals; patients realize unreality of hallucinations; first described by Swiss philosopher Charles Bonnet in 1760",,, Charlin’s syndrome of neuralgia,"severe pain in the inner corner of the eye disproportionate to the degree of ocular inflammation, pain in the root of the nose, tearing, and ipsilateral nasal watery discharge believed to be due to irritation of the ciliary ganglion",,, Chédiak-Higashi syndrome,"autosomal recessive disorder from mutation in lysosomal-trafficking regulator, resulting in neutropenia, defective degranulation, and delayed microbial killing. Neutrophils and other leukocytes have giant granules. Delayed or decreased fusion of lysosomes with phagosomes in leukocytes impairs phagocytosis of bacteria. Increased infections with S. aureus, group A Strep, partial ocular and cutaneous albinism, silvery hair, peripheral neuropathy, easy bruising, mild mental retardation, severe periodontal disease",,, Cheyne-Stokes breathing,"regularly irregular pattern characterized by a progressive increase in the depth and at times frequency of respiration with a crescendo-decrescendo shape that eventually culminates in an apneic phase; seen in CHF but also in meningitis, CVA, pontine damage",,, Chiari-Frommel syndrome,persistent galactorrhea-amenhorrhea after pregnancy,,, Chilaiditi syndrome,when redundant loops of transverse colon slip between the liver and diaphragm and cause vovulus,,, Child-Pugh classification system,"classification for severity of liver disease according to degree of ascites, bilirubin, albumin, prothrombin time, and encephalopathy; initially used to predict mortality after surgery",,, Christmas disease,"hemophilia B, deficiency in factor IX",,, Churg-Strauss syndrome,"allergic angiitis and granulomatosis involvement in the lung, associated with eosinophilia; asthma is the cardinal feature (occurs in 95%) and precedes vasculitic phase by 8-10 years; 2/3 of patients have skin lesions which can appear as s.c. nodules on extensor surfaces; 70% have P-ANCA; 4/6 following criteria 85% sens and 99.7% spec: asthma; eosinophilia>10%; neuropathy; pulmonary opacities; paranasal sinus abnormality; biopsy of blood vessel showing eosinophils in extravascular area",,, Chuvash polycythemia,"autosomal recessive disorder with features of both primary and secondary polythemia, endemic to the mid-Volga River region of Russia (Chuvas); associated with homozygous mutation Arg200Trp in VHL gene (gene associated with von Hippel-Lindau syndrome); mutation impairs interaction of VHL with hypoxia-inducible factor 1 protein",,, Chvostek’s sign,"hypocalcemia, in latent tetany, tapping the facial nerve against the bone just anterior to the ear producing ipsilateral contraction of facial muscles",,, Civatte bodies,"aka colloid bodies; in lichen planus; anucleate, necrotic basal cells becoming incorporated into the inflamed papillary epidermis",,, Clagett’s procedure,"for treating empyema, open drainage followed by instillation of antibiotic solution",,, Clara cells,"cells found in the epithelium of terminal and respiratory bronchioles, devoid of cilia, present secretory granules in their apex and are known to secrete glycosoaminoglycans that probably protect the bronchiolar lining",,, Claude’s syndrome,third nerve palsy combined with contralateral ataxia with midbrain infarction and injury to both red nucleus and superior cerebellar peduncle (includes signs of both Nothnagel’s and Benedikt’s syndrome),,, clay shoveler’s fracture,fracture of spinous process of C7,,, Clerambault’s syndrome,"erotomania, delusional belief that someone (usually of higher social status) is in love with the person",,, Clichy criteria,"criteria for liver transplantation in fulminant hepatic failure: hepatic encephalopathy, and factor V level < 20% in patients younger than 30 y.o. and factor V level < 30% in patients > 30 y.o.",,, Clutton’s joints,"in congenital syphilis, symmetrical arthrosis, especially of the knee joints",,, Cockayne’s syndrome,"a form of progeria characterized by dwarfism, pigmentary degeneration of the retina, optic atrophy, deafness, sensitivity to sunlight, and mental retardation; autosomal recessive inheritance defect in DNA repair",,, Cockcroft-Gault formula,"for calculating clearance based on creatinine, age, weight: [(140-age) x lean body weight (in kg)]/[plasma creatinine (in mg/dL) x 72]; in women, multipled by 0.85 because of smaller muscle mass",,, Codman’s triangle,"in osteosarcoma, the triangular shadow between the cortex and raised ends of periosteum is known radiographically as Codman’s triangle, and is characteristic but not diagnostic of this tumor",,, Cogan syndrome,"multisystem inflammatory vascular disease particularly of the CNS characterized by interstitial keratitis, vestibuloauditory symptoms",,, Coley’s toxin,mixture of toxins from Strep and Serratia marcesens used by William Coley in the late 1800s to treat malignancy; active agent found to be lipopolysaccharide,,, Colles’s fracture,a fracture of the distal radius that occurs when persons fall with outstretched hands to try to catch themselves,,, Collier’s sign,lid retraction in lesion of posterior commissure,,, Conn’s syndrome,"primary hyperaldosteronism, caused by an aldosterone-secreting tumor, resulting in hypertension, hypokalemia, hypernatremia, metabolic alkalosis, and low plasma renin",,, Conradi-Hunermann syndrome,"chondrodysplasia punctata, characterized by stippled epiphyses from abnormal accumulation of calcium salts and skeletal changes",,, Conradi-Hunermann-Happle syndrome,"X-linked dominant variant of Conradi-Hunermann syndrome or chondrodysplasia punctata, associated with linear ichthyosis, cataract, and short stature; associated with mutation in delta8-delta7 sterol isomerase emopamil binding protein, a protein involved in cholesterol metabolism",,, Cooley’s anemia,homozygous beta thalassemia; Mediterranean anemia,,, Coombs test,"direct, ability of anti-IgG or anti-C3 antisera to agglutinate the patient’s red blood cells; cold reacting antibodies react with anti-C3 (mostly drug-related antibodies, IgM antibodies (generally to polysaccharide), IgG antibodies of low affinity); indirect Coombs, serum of the patient is incubated with normal red cells, though IgM antibodies may agglutinate directly",,, Cooper's hernia,hernia through the femoral canal and tracking into the scrotum or labia majus,,, Cope’s syndrome,subacute milk-alkali syndrome; see also Burnett’s syndrome,,, Cori’s disease,"glycogen storage disease type III, deficiency in debranching enzyme, amylo-1,6-glucosidase, leading to variable accumulation of glycogen in the liver, heart, or skeletal muscle, characterized by stunted growth, hepatomegaly, and hypoglycemia",,, Corrigan’s pulse,"in aortic regurgitation, pulses are of the water-hammer or collapsing type with abrupt distension and quick collapse, can be exaggerated by raising the patient’s arm",,, Costen’s syndrome,"ear pain, tinnitus, impaired hearing, and dizziness from temporomandibular joint dysfunction",,, Councilman bodies,"in apoptosis, hepatocytes that round up to form shrunken, pyknotic, and intensely eosinophilic bodies",,, Courvoisier’s law,"tumors that obstruct the common bile duct result in an enlarged bladder; obstructing stones do not, since the gallbladder is typically too scarred to allow enlargement; present in half of pancreatic CA",,, Cowden disease,"autosomal dominant condition associated with multiple hamartomatous lesions, especially of the skin, mucuous membranes, GI tract, breast and thyroid; associated with trichilemmomas (multiple skin-colored warty papules); associated with mutations in PTEN/MMAC1",,, Cowdry body,intranuclear inclusion seen in herpes virus infection,,, Cox maze procedure,surgical procedure developed in 1987 for atrial fibrillation where a tortuous path is created between the SA node to the AV node and incisions are placed so that atrial fibrillation cannot be sustained,,, Creutzfeld-Jakob disease,"fatal transmissible disorder of the CNS characterized by rapidly progressive dementia and variable focal involvement of the cerebral cortex, basal ganglia, cerebellum, brainstem, and spinal cord, attributable to prions, characterized by myoclonus often induced by a startle, extrapyramidal signs, cerebellar signs; slowing and periodic complexes on EEG; 1 per 167,000 in U.S.; fatal within 1 year after onset of symptoms",,, cri du chat,"5p-, severe mental retardation, microcephaly, catlike cry, low birth weight, hypertelorism, low-set ears, and epicanthal folds",,, Crigler-Najjar syndrome,"type I, no hepatic glucoronyltransferase activity, kernicterus, requires liver transplantation; type II, moderate deficiency of glucoronyltransferase, phenobarb induces activity",,, Crohn’s disease,"transmural mucosal inflammation that may involve the entire GI tract from mouth to the perinanal area that often leads to fibrosis and obstructive clinical presentations; 80% small bowel involvement, usually distal ileum; 1/3 exclusively ileitis; 50% ileocolitis; and 20% disease limited to colon",,, Cronkhite-Canada syndrome,"diffuse GI hamartoma polyps (i.e., no cancer potential) associated with malabsorption/weight loss, diarrhea and loss of electrolytes/protein; signs include alopecia, nail atrophy, and skin pigmentation",,, Crouzon syndrome,craniosynostosis correlated in mutations with the extracellular domain of FGFR2,,, Crowe’s sign,axillary or inguinal freckling seen in 20-50% of neurofibromatosis,,, Crow-Fukase syndrome,"POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes); known as Crow-Fukase syndrome in Japan",,, Cruveihiler-Baumgarten bruit,bruit heard over caput medusa in portal hypertension,,, Cullen’s sign,"a faintly blue coloration particularly of umbilicus as the result of retroperitoneal bleeding from any cause, but especially in ruptured ectopic pregnancy; also seen in acute pancreatitis (1-2%)",,, Curling’s ulcers,"stress erosions and ulcers occurring in the proximal duodenum and associated with severe burns or trauma, from ischemia of the gastric mucosa",,, Curschmann’s spirals,"spirally twisted masses of mucus plugs containing whirls of shed epithelium occurring in the sputum in bronchial asthma; Heinrich Curschmann, German physician, 1846-1910",,, Cushing reaction,"increase in intracranial pressure causes compression of the cerebral blood vessels and cerebral ischemia, reaction of elevation in pressure with simultaneous reduction in heart rate, respiratory slowing",,, Cushing’s disease,"hypercortisolism from pituitary corticotropin-(ACTH)-secreting corticotroph tumors, almost always benign and usually microadenomas",,, Cushing’s syndrome,hypercortisolism,,, Cushing’s triad,signs of increased ICP 1. hypertension 2. bradycardia 3. irregular respirations,,, Cushing’s ulcer,"acute ulcer of the stomach, proximal duodenum, or esophagus, frequently leads to hemorrhage or perforation, associated with intracranial injury or increases in intracranial pressure, associated with gastric acid hypersecretion",,, D’Espine’s sign,"breath sounds louder over C7 than adjacent lung, suggests lesion in posterior mediastinum, e.g. lymphoma, tuberculosis, etc. ",,, Da Costa syndrome,"neurocirculatory asthenia, pain localized typically to the cardiac apex and consists of dull, persistent ache that lasts for hours, etc.",,, Dacie’s syndrome,idiopathic hyperplastic enlargement of the spleen with anemia and neutropenia; progression to lymphoma in some cases,,, Dahl’s sign,"in COPD, protracted pressure applied by the elbows leads eventually to the formation of two patches of hyperpigmented calluses immediately above the knees",,, Dallas criteria,"for histologic diagnosis of myocarditis, active myocarditis if infiltrating lymphocytes and myocytolysis seen; borderline if only lymphocytic infiltration; and negative if both absent; introduced in 1986",,, Dalrymple sign,"retraction of the upper eyelid in Graves’s disease, causing abnormal wideness of the palpebral fissure",,, Daltonism,"color blindness, from John Dalton (who proposed atomic theory), born 1766; felt that color blindness was from vitreous humor being blue, disproved his assistant Joseph Ransome who examined his eyeballs post mortem",,, Damus-Kaye-Stansel procedure,repair of congenital transposition of the great arteries of the heart by dividing the pulmonary artery and attaching the proximal section to the ascending aorta and connecting distal section to right ventricle,,, Dance’s sign,empty right lower quadrant in children with ileocecal intussusception,,, Dandy-Walker syndrome,hydrocephalus resulting from failure of the foramina Luschka and Magendie to open; associated with an occipital meningocele and agenesis of the cerebellar vermis and splenium of the corpus callosum; associated with warfarin use during pregnancy,,, Dane particle,"mature HBV virion, 42 nm, double-layered, genome is double-stranded circular DNA, all regions of genome encode stuff",,, Darier’s sign,"in mastocytosis (urticaria pigmentosa), is positive when a brown macular or a slightly papular lesion becomes a palpable wheal after being vigorously rubbed with the blunt end of an instrument such as a pen; wheal may not appear for 5-10 minutes",,, "Darkschewitsch, nucleus of","an ovoid cell group in the ventral central gray substance rostral to the oculomotor nucleus, receiving fibers from the vestibular nuclei by way of the medial longitudinal fasiculus; projections are not known, although some cross in the posterior commissure",,, Darwin’s tubercle,"completely benign and congenital nodule, located near the superior aspect of the auricle (approximately 2/3 of the way from the bottom of the helix), described by Charles Darwin",,, Dawson’s fingers,"in multiple scelrosis, perivascular demyelination creating the appearance of finger projections oriented transversely on an axial scan",,, de Musset’s sign,"in aortic regurgitation, head bobbing, named after 19th century French poet who had luetic aortic insufficiency",,, de Quervain’s disease,a stenosing tenosynovitis of the thumb extensors and abductors; pain elicited with Finkelstein’s test,,, de Quervain’s thyroiditis,"subacute granulomatous thyroiditis, viral etiology suspected",,, Degos disease,"malignant atrophic papulosis; multisystem lymphocytic vasculitis characterized by thrombosis primarily of cutaneous small vessels as well as small vassels in GI tract, ocular, and CNS; has characteristic porcelain white atrophic lesion",,, Dejerine-Roussy syndrome,"thalamic lesions causing sensory loss, spontaneous pain, and perverted cutaneous sensation described in 1906",,, Dejerine-Sottas disease,"hereditary motor and sensory neuropathy III, recessive inheritance, slowly progressive demyelinating disorer, presents in infancy or childhood and progresses to cause severe disability by 30s; nerves typically enlarged",,, Delphian nodes,lymph nodes in midline of the thyrohyoid membrane; can be involved in thyroid cancer or subacute thyroiditis,,, Dennie’s lines,"in atopic dermatitis, an accentuated line or fold below the margin of the lower eyelid",,, Dent’s disease,"X-linked syndrome characterized by renal proximal tubular dysfunction, proteinuria, hypercalciuria, nephrocalcinosis, nephrolithiasis, and rickets due to mutation in voltage-gated chloride channel",,, Denver shunt,"for treating refractory ascites, peritoneovenous shunt connecting peritoneum and central venous system, compare with LeVeen shunt; Denver shunt uses valve that lies within a fluid-filled, compressible silicone chamber",,, Denys-Drash syndrome,gonadal dysgenesis (male pseudohermaphroditism) and nephropathy leading to renal failure with increased risk of Wilms’s tumor; dominant negative missense mutation of WT-1,,, Denys-Drash syndrome,"male pseudohermaphroditism, high risk of Wilms’s tumor development, and early renal failure; associated with mutation in WT1 gene",,, Dercum’s disease,"adipositas dolorsa, condition tending to affect obese women in middle age, mostly menopausal, consisting of multiple exquisitely tender lipomas",,, Desçemet’s membrane,membrane that forms the deepest layer of the cornea and functions as thin basement membrane for endothelium; location where copper is deposited in Wilson’s disease Kayser-Fleischer rings,,, Destot’s sign,scrotal hematoma suggesting pelvic fracture,,, Deutschlander’s fracture,"fracture from overuse, e.g. marching",,, Devic’s disease,neuromyelitis optica; relapsing-remitting demyelinating disorder characterizing by bilateral optic neuritis and transverse myelitis occurring in rapid succession; common in Asians; distinct from multiple sclerosis,,, Diamond-Blackfan anemia,"congenital pure red cell aplasia characterized by increased MCV, reticulocytopenia, bone marrow erythroblastopenia; can be diagnosed by increased erythrocyte adenosine deaminase; 40% associated with congenital abnormalities; associated with RPS19 ribosomal protein mutations in some; responds to steroids which increase Epo sensitivity",,, Dick test,"injection of erythogenic toxin of Strep. pyogenes, positive result in those lacking antitoxin",,, Dieulafoy lesion,"around 6 cm from the gastroesophageal junction, an uncommon cause of massive GI bleeding, a large submucosal artery which erodes the mucosa without any overlying ulceration or other obvious mucosal damage",,, DiGeorge syndrome,"failure of 3rd and 4th pharyngeal pouches to differentiate into the thymus and parathyroid glands, facial abnormalities result primarily from abnormal development of the first arch components during formation of face and ears, 22q11 deletion, CATCH-22 (cardiac abnormality/abnormal facies, T-cell deficit owing to thymic hypoplasia, cleft palate, and hypocalcemia)",,, DiGuglielmo’s disease,"AML M6, erythroleukemia",,, dimple sign,"in dermatofibroma, lateral compression with thumb and index finger produces a depression, or “dimple.”",,, "Disse, space of",subendothelial space in liver separating endothelial cells from underdlying hepatocytes which contains hepatocyte microvilli,,, Dix-Hallpike test,"for testing benign paroxysmal positional vertigo, examiner stands at the patient’s right side and rotates the patient’s head 45 degrees to the right to align the right posterior semicircular canal with the sagital plane of the body; the examiner moves the patient, whose eyes are open, from the seated to the supine right-ear-down position and then extends the patient’s neck slightly so that the chin is pointed slightly upward.",,, Dobrin’s syndrome,"TINU syndrome (tubulointerstitial nephritis with uveitis), associated with bone marrow granulomas, first described in 1975",,, Doderlein’s lactobacilli,"Lactobacillus acidophilus; metabolizes the glycogen in glycogen rich cells which are exfoliated in the vagina pre-menopause to lactic acid, resulting in a decrease in pH to 3.5-4",,, Döhle bodies,"irregularly shaped greenish inclusions in neutrophil cytoplasm, consisting of ribosomes and/or rough ER and are seen in severe bacterial infections",,, Doi’s sign,elicitation of diminished deep tendon reflexes after maximal voluntary contraction in Lambert-Eaton syndrome,,, Donath-Landsteiner antibody,"in paroxysmal cold hemoglobinuria, an antibody associated with syphilis and viral infections, directed against the P red cell antigen and can induce complement-mediated lysis; attacks precipitated by exposure to cold and are associated with hemoglobinemia and hemoglobinuria; chills and fever; back, leg, and abdominal pain; headache and malaise; recovery prompt; asymptomatic otherwise",,, Donovan bodies,"in Calymmatobacterium granulomatis or granuloma inguinale, bodies characterized by multiple organisms filling large histiocytes",,, Dor procedure,"endoventricular circular patch plasty, a surgical procedure for treating postinfarction aneurysm where a purse string stich is sewed around the aneursym",,, Dorello’s canal,"location where the sixth nerve penetrates the dura, an area where the nerve is liable to injury",,, "Douglas, pouch of",rectouterine pouch,,, Down syndrome,"trisomy 21 (in 95%), 1/700 births, 1% mosaics, 40% have congenital heart disease. trisomy 21, 10-20 fold increased risk of acute leukemia, virtually all older than 40 develop Alzheimer’s, abnormal immune system",,, Dressler’s beat,fusion beat seen in ventricular tachycardia,,, Dressler’s syndrome,"pericarditis, possible autoimmune etiology, found to develop 2 weeks to several months after acute MI",,, Druckrey relationship,"relationship between carcinogen dose and tumor induction time: dtn = k where d is the dose of carcinogen, t is latency period, n is the slope of the double log [plot of carcinogen dose versus induction time, and k is a constant",,, "Drummond, marginal artery of",vessel which lies along mesenteric border of colon and supplies vasa recta; arises from right and left branches of middle colic artery and left and right colic arteries; also supplies collateral connection between superior and inferior mesenteric arteries,,, Duane’s syndrome,"form of strabismus, congenital absence of cranial nerve 6, resulting in impaired abduction and/or adduction and eyeball retraction and narrowing of palpebral fissure during adduction of affected eye",,, Dubin-Johnson syndrome,mostly conjugated hyperbilirubinemia from defect in transport of bilirubin and other organic anions across the canaliculus; other liver function tests normal; accumulation of dark pigment in liver lysosomes,,, Duchenne’s muscular dystrophy,"X-linked, near or complete absence of dystrophin (normally stabilizes glycoprotein complex on cytoplasmic face of plasma membrane of muscle fibers and protects it from degradation), onset of weakness age 2-3, proximal weakness of limb muscles, Gower’s maneuver, pseudohypertrophy of calves caused by fatty infiltration, cardiomyopathy, frequently mental retardation, CK elevated, wheelchair bound by age of 12",,, Duffy blood group system,"named after Mr. Duffy, a patient with hemophilia in whom anti-Fya was discovered by Cutbush in 1950; abbreviation Fy used because “Du” had already been taken; Fy6 antigen is the receptor for Plasmodium vivax",,, Duncan’s disease,"X-linked lymphoproliferative syndrome, normal response to childhood infections but develop fatal lymphoproliferative disorders after infection with EBV; most patients with this syndrome die of infectious mononucleosis",,, Dunnigan syndrome,"partial lipodystrophy, loss of subcutaneous fat and increase in visceral fat, causing muscular appearance in arms and legs, due to mutation in laminin A/C",,, Dunphy sign,increased pain with coughing in appendicitis,,, Dupuytren’s contracture,palmar fibromatosis,,, Durant’s maneuver,"left lateral decubitus position, used in managing air embolism",,, Duret hemorrhage,hemorrhage from uncal herniation,,, Duroziez’s sign,"in aortic regurgitation, systolic murmur heard over the femoral artery when it is compressed proximally and a diastolic murmur when it is compressed distally",,, Dutcher bodies,"PAS-positive inclusions containing Ig in the nucleus of lymphocytes, plasma cells, and intermediate lymphocytes in Waldenström macroglobulinemia and multiple myeloma",,, Eagle effect,"failure of penicillin in streptococcal infection when bacteria aren’t growing, i.e., beta-lactams being more effective against rapidly growing bacteria in the early stages and less effective as bacterial growth slows (described by Eagle in 1952)",,, Eagle-Barrett syndrome,"prune-belly syndrome with triad of abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism",,, Eale’s disease,"isolated, peripheral retinal vasculitis",,, Ebstein’s anomaly,malformation characterized by the downward displacement of the tricuspid valve into the right ventricle due to anomalous attachment of the tricuspid leaflets; associated with maternal exposure to Li,,, economy class syndrome,"pulmonary embolism after travel, described by Symington and Stack in Br J Dis Chest 1977; 71:138-40",,, Edeiken pattern,normal variant ST segment elevation seen V2 or V3 where there is a shallow upward concavity; pattern disappears when leads are recorded one interspace lower than usual (Am Heart J 1954;48:331).,,, Edinger-Westphal nucleus,part of the cranial nerve III complex involved in direct and consensual light reflex involved in efferent limb of reflex arc,,, Edwards’s syndrome,"trisomy 18, 1/8,000 births, mental retardation, prominent occiput, micrognathia, low-set ears, rocker-bottom feet, flexion deformities of the fingers, and congenital heart disease",,, Ehlers-Danlos syndromes,"clinically and genetically heterogeneous group of disorders that result from defect in collagen synthesis or structure, at least 10 variants, characterized by hyperextensible skin and hypermobile joints",,, Ehrlich’s reaction,reaction where p-dimethylaminobenzaldehyde reacts with urobilinogen in a strong acid medium to produce a brown-orange color,,, Eisenmenger complex,"a ventricular septal defect with right ventricular hypertrophy, severe pulmonary hypertension, and frequent straddling of the defect by a misplaced aortic root",,, Eisenmenger syndrome,"cardiac failure with significant right to left shunt producing cyanosis due to higher pressure on the right side of the shunt; usually due to the Eisenmenger complex, any anomalous circulatory communication that leads to obliterative pulmonary vascular disease",,, Ekbom syndrome,restless leg syndrome,,, Ekiri syndrome,"extremely rare, fatal encephalopathy described in Japanese children with Shigella sonnei or Shigella flexneri infections",,, Elejalde syndrome,"neuroectodermal melanolysosomal disease characterized by silvery hair, CNS dysfunction, abnormal melanocytes and melanosomes, and abnormal inclusion bodies in fibroblasts and other cells; similar dermatologic features to Chédiak-Higashi syndrome and Griscelli syndrome but without defect in immune system",,, Ellis-van Creveld syndrome,"chondroectodermdal dysplasia, short-limbed dwarfism, polydactyly, single atrium or atrial septal defect, autosomal recessive, reported most often in Amish",,, Ellsworth-Howard test,for diagnosis of pseudohypoparathyroidism by assessing response to exogenous PTH,,, Elschnig spots,yellow (early) or hyperpigmented (late) patches of retinal pigment epithelium overlying infarcted choriocapillaris lobules in hypertensive retinopathy,,, Emery-Dreifuss muscular dystrophy,distinctive form of muscular dystrophy with humero-peroneal weakness and quite pronounced muscle contractions and by severe cardiac arrhythmias which may cause sudden death; due to mutations in lamin A/C gene on 1q21.2-q21.3,,, Epley maneuver,"in benign paroxysmal positional vertigo, a treatment maneuver for moving calcium carbonate debris to the common crus of the anterior and posterior canals and exit into the utricular canal; symptoms are improved in 90% of patients with no recurrence in 50-70% (also see Semont maneuver and Brandt-Daroff exercises)",,, eponym ,text,,, Epping jaundice,"outbreak of jaundice that occurred in 84 individuals after ingestion of bread made with flour contaminated with 4,4'-diaminodiphenylmethane in England in 1965",,, Epsom salts,"magnesium sulfate, laxative",,, Epstein’s pearls,"small, white cysts along the median raphe of the hard palate",,, Epstein-Barr virus,EBV binds to CD21 found on epithelial cells and B cells; however a large number of T suppressor cells and EBV specific cells are seen as atypical lymphocytes,,, Erb’s palsy,upper plexus palsy affecting C5 and C6 and +/- C7 nerve roots,,, Erlenmeyer’s flask deformity,"seen in osteopetrosis, where the ends of long bones are bulbous",,, Evan’s syndrome,ITP and immunohemolytic anemia,,, Ewart’s sign,"in large pericardial effusion, dullness to percussion of the left lung over the angle of the scapula may occur; due to compressive atelectasis by the large pericardial sac",,, Ewing’s sarcoma,"onion skinning; small round cell tumor of bone, 85% of cases there is a t(11;22)(q24:q12) translocation",,, Fabry’s disease,"X-linked recessive sphingolipidosis (sulfatidose) alpha-galactosidase deficiency resulting in increased globosides, reddish-purple skin rash, kidney and heart failure, peripheral neuropathy, pain in lower extremities (acroparesthesias); 1:117,000 live births; most common clinical features are telangiectases and corneal dystrophy; can be trated with biweekly infusions of recombinant human alpha-galactosidase A",,, factor V Leiden,"Arg(506)->Gln, results in resistance to cleavage by activated protein C (an anticoagulant), found in 20% of patients with venous thromboembolism, 6% of U.S. population",,, Fahr’s disease,"neurodegenerative syndrome associated with symmetric intracerebral calcifications in basal ganglia, associated with cognitive and movement disorders including spastic paralysis, athetosis",,, "Fallot, tetralogy of","1. ventricular septal defect; 2. infundibular, valvar, or supravalvar pulmonic stenosis; 3. an anteriorly displaced aorta that receives blood from both ventricles; 4. right ventricular hypertrophy",,, Fanconi’s anemia,"autosomal recessive, predisposal to aplastic anemia, progressive bone failure at age 5-7, congenital malformations (frequently defects in thumbs or forearms, kidneys, GI, skin); 20% develop cancer (primarily AML but also in skin, GI, GU); diagnosed by seeing increased sensitivity of FA cells to bifunctional alkylating agents (e.g. diepoxybutane or mitomycin C)",,, Fanconi’s syndrome,"generalized dysfunction of proximal renal tubule leading to glycosuria, hyperphosphaturia, hypophasphatemia, aminoaciduria, and systemic acidosis; may be associated with out-dated tetracyclines",,, Fanconi-Bickel syndrome,"type XI glycogen storage disease, hepatic glycogenosis with renal Fanconi syndrome; caused by defect in GLUT-2 transporter, characterized by proximal renal tubular dysfunction, impaired glucose and galactose utilization, liver and kidney glycogen accumulation; presents as failure to thrive in first year with hepatomegaly and kidney enlargement",,, Farber’s disease,"sphingolipidosis from ceraminidase deficiency leading to painful and progressively deformed joints, subcutaneous nodules, granulomas, fatal in early life",,, farmer’s lung,"hypersensitivity pneumonitis from exposure to antigens of microorganisms which colonize equipment used in farming, including from moldy hay, grain, silage ( e.g. thermophilic actinomycetes, fungus such as Aspergillus umbrosus)",,, farmer’s skin,cutis rhomboidalis nuchae,,, Fay sign,"in carotid arteritis, pressure along the carotid causing pain to spread in distal branches of external carotid to jaw, ear, and temple",,, Felty’s syndrome,"rheumatoid arthritis, splenomegaly, and neutropenia, and leg ulcers; associated with HLA-DR",,, Ferguson’s reflex,"anesthesia in ob, interruption of oxytocin release in response to cervical dilatation may cause uterine inhibition",,, fetor hepaticus,“musty” or “sweet odor” from the formation of mercaptans by the action of GI bacteria on the sulfur-containing amino acid methionine and shunting of splanchnic blood from the portal into the system circulation (portosystemic shunting),,, fifth disease,"erythema infectiosum, associated with parvovirus B19 infection, characterized by “slapped cheeks” and erythematous lacy eruption on the trunk and extremities",,, Finkelstein’s test,"in de Quervain’s tenosynovitis, dorsal thumb pain when the wrist is deviated in an ulnar fashion and the thumb is flexed across the palm",,, first disease,"measles, aka rubeola, caused by paramyxovirus, described in 1627",,, Fisher’s syndrome,see Miller-Fisher syndrome,,, Fitzgerald factor,"high molecular weight kininogen; in intrinsic phase of coagulation, part of complex between factor XII, prekallikrein, and subendothelial collagen; facilitates conversion of XII to XIIa",,, Fitz-Hugh-Curtis syndrome,associated with spread of gonococci or chlamydia: perihepatitis manifested by right upper quadrant or bilateral upper abdominal pain and tenderness and occasionally by a hepatic friction rib,,, Flatbush diabetes,GAD antibody negative diabetes in adult black subjects with diabetic ketoacidosis and increased frequency of human leukocyte antigen DR3 and DR4,,, Flatow-Dukes’s disease,"fourth disease, historically felt to be a separate form of rubella and then felt to be a non-entity but perhaps now is Staph scarlet fever Lancet 357:299 (2001)",,, Fletcher factor,"prekallikrein; in intrinsic phase of coagulation, converts XII to XIIa; deficiency results in elevated PTT but with no clinical bleeding ",,, Foix-Alajouanine disease,"angiodysgenetic necrotizing myelopathy, venous angiomatous malformation of the spinal cord and overlying meninges associated with ischemic damage and worsening neurologic symptoms in lumbosacral cord",,, Fontan procedure,"for treating tricuspid atresia, pulmonary atresia, or hypoplastic left heart syndrome, procedure developed in 1968 whereby IVC is connected to pulmonary artery; long term complications include arrhythmias and protein losing enteropathy because of edema in liver and small bowel",,, Forbes-Albright syndrome,galactorrhea-amenorrhea caused by a pituitary adenoma,,, Forchheimer spots,"in 20% of rubella, small, red macules on the soft palate that may later expand and coalesce; not pathognomonic since they may also appear in scarlet fever, roseola, mono",,, Fordyce’s spots,ectopic sebaceous glands seen in healthy mouth; may be confused with Koplik’s spots which has an erythematous halo by comparison,,, Forrester classification,"in myocardial infarction, I, PWP<18 and CI>2.2 L/min/m2, 2% mort; II, PWP>18 and CI>2.2, 10% mort; III, CI<2.2 and PWP<18, 12% mort; IV, PWP>18 and CI<2.2, 54% mort",,, Forscheimer spots,"in rubella, punctate soft palate macules",,, Foster Kennedy syndrome,"optic atrophy, contralateral papilledema, and anosmia; may be associated with olfactory groove meningioma",,, Fothergill’s sign,"in rectus sheath hematomas, tender mass that does not cross the midline and remains palpable when patient tenses the rectus muscle (bluish discoloration not usually seen until 3 or 4 days)",,, Fournier’s gangrene,necrotizing infection of the perineal and genital fascia,,, fourth disease,"Flatow-Dukes’s disease, aka Staph scarlet fever, caused by Staph aureus epidermolytic toxin, described in 1900",,, Fouville’s syndrome,"dorsal pontine injury giving rise to lateral gaze palsy, ipsilateral facial palsy, contralateral hemiparesis",,, Fox-Fordyce disease,"chronic, pruritic, papular eruption occurring in areas with apocrine glands, affecting mainly women",,, Fox's sign,"in hemorrhagic pancreatitis, ecchymosis of the inguinal ligament due to blood tracking from the retroperitoneum and collecting at the inguinal ligament",,, Frank’s sign,"earlobe crease, associated with CAD (N Engl J Med. 1973;289:327-8)",,, Franklin’s disease,"gamma heavy chain disease, characterized by LAD, fever, anemia, malaise, HSM, and weakness, most distinctive symptom palatal edema",,, Frasier syndrome,"female external genitalia in 46 XY patients, late renal failure, streak gonads, and high risk of gonadoblastoma; associated with mutation in WT1 gene",,, Freiberg disease,osteochondrosis of second metatarsal head; associated with avascular necrosis of metatarsal head; most patients are female,,, Frey syndrome,gustatory sweating or auriculotemporal syndrome seen in the context of parotid surgery; ipsilateral forehead becoming drenched with sweat apparently because of some salivery nerve fibers connecting with autonomic efferents,,, Friedreich’s ataxia,"a spinocerebellar degeneration, autosomal recessive form manifesting at 11 years, less common autosomal dominant form 20 years; initial symptoms: gait ataxia, hand clumsiness, dysarthria, DTRs absent (extensor plantar present), joint position and vibratory sense impaired, sometimes loss of pain and temperature, paralysis over course of 20 years, high incidence of diabetes and hypertrophic cardiomyopathy (dilated less common) and arrhythmias",,, Friedreich’s foot,"seen in Friedreich’s ataxia, pes cavus with hammer toe",,, Friedreich’s sign,"exaggerated y descent in patients with increased venous pressure, associated with an S3; also associated with constrictive pericarditis",,, Froin’s syndrome,"spinal block from tumor or inflammatory conditions, etc. causing increased protein in CSF, resulting in xanthochromia and increased coagulability in CSF",,, Froment’s sign,"diagnosis of ulnar nerve lesion; caused by flexor pollicus longus (median nerve) which comes into action when the patient attempts to grip a flat object between the thumb and the hand, and causes flexion at the interphalangeal joint",,, Gaisböck’s syndrome,"stress polycythemia, combination of modest elevation in hematocrit and normal red cell mass; unknown etiology, seen in hard-driving middle-aged males, usually smokers, who in addition tend to be overweight and hypertensive",,, Galeazzi fracture,fracture of the radius at the junction of the middle and distal thirds accompanied by disruption of the distal radioulnar joint,,, "Galen, great vein of",great cerebral vein,,, Gallavardin dissociation,"in aortic stenosis, high-velocity jet within the aortic root results in radiation of murmur upward to 2nd right intercostal space (in older patients, becomes harsh, noisy, and impure), whereas the murmur over apex is pure and often musical, mimicking mitral regurgitation.",,, gamekeeper’s thumb,injury to ulnar collateral ligament of the thumb,,, Gandy-Gamna nodules,"in congestive splenomegaly, organization of focal hemorrhages giving rise to foci of fibrosis containing deposits of iron and calcium salts encrusted on connective tissue and elastic fibers",,, Gardner’s syndrome,"familial adenomatous polyposis (now realized all patients with FAP have extraintestinal manifestations), exhibiting intestinal polyps identical to those in FAP combined with 1. sebaceous cysts, 2. osteomas (particularly of the mandible, skull, and long bones), 3. desmoid tumors; mutation in adenomatous polyposis coli (APC) gene in 5q",,, Gardos channel,"potassium-selective channel controlled by cytosolic calcium concentrations, one of two channels involved in red cell shrinkage playing a role in sickling; local tissue acidosis and deoxygenation-induced influx of calcium activate the Gardos channel resulting in shrinkage; clotrimazole potent blocker of this channel ",,, "Garré, sclerosing osteomyelitis of","typically develops in the jaw, associated with extensive new bone formation that obscures much of the underlying osseous structure",,, gastrinoma triangle,"a triangle where more than 90% of extrapancreatic gastrinomas are located bordered by 1. 3rd portion of duodenum, 2. Cystic duct, 3. Pancreatic neck",,, Gaucher’s disease,"autosomal recessive sphingolipidosis (sulfatidose), mutations in glucocerebrosidase gene on 1q21 (also known as acid beta-glucosidase), enzyme cleaves glucose residue from ceramide, frequently fatal; type I, chronic non-neuronopathic form, splenic and skeletal involvement; can be treated with infusions of macrophage-targeted human placental glucoerebrosidase, alglucerase (Ceredase), use described in 1991",,, "Geraldi, fossa of",name of the fossa between the testicle and epididymus,,, Gerhardt’s sign,"in aortic regurgitation, pulsation of the spleen in the presence of splenomegaly; see also Sailer’s sign",,, Gerota’s fascia,fascia surrounding the kidney,,, Gerstmann syndrome,"finger agnosia, agraphia, right-left disorientation, and dyscalculia",,, Gerstmann-Straussler-Scheinker syndrome,"slow central nervous system disease, hereditary as well as transmissible prion disease with same mutation, point mutation in codon 102 of prion protein as CJD; characterized by cerebellar dysfunction with multiple plaques",,, Ghon lesion,primary area of tuberculosis infection,,, Gianotti-Crosti syndrome,"reactive exanthem, first described in association with hepatitis B infection (papular acrodermatitis of childhood, papular eruption on face and limbs); also associated with EBV, CMV infection",,, Gibbs-Donnan equilibrium,"in RBCs, bicarbonate ions diffusing out and chloride diffusing in",,, Gilbert’s syndrome,"with the exception of hemolytic anemias, most common cause of mild unconjugated hyperbilirubinemia from mild decrease glucoronyltransferase activity, responds to phenobarbital, affects up to 7% of population",,, Gitelman’s syndrome,variant of Bartter’s syndrome where patients have hypomagnesemia and hypocalciuria due to mutations in thiazide-sensitive sodium-chloride transporter,,, Glanzmann’s thrombasthenia,inherited disorder of platelet function where Gp IIb/IIIa receptor for fibrinogen missing,,, Glauber’s salt,"sodium sulfate, laxative",,, Gleason score,prostate cancers stratified into 5 grades on basis of glandular patterns and degree of differentiation; grade 1 most differentiated and grade 5 no glandular differentiation; primary score and secondary score (for second most common histology) added together for score; therefore most differentiated tumor score of 2,,, Gleich syndrome,"episodic angioedema with eosinophilia characterzed by recurrent episodes of angioedema, urticaria, pruritus, fever, weight gain, elevated IgM, leukocytosis, and marked eosinophilia; blood eosinophilia parallels disease activity (N Engl J Med 1984;310:1621",,, Glenn shunt,"In treatment of hypoplastic left heart syndrome. Unidirectional: SVC to right pulmonary artery anastomosis with ligation of the proximal RPA and cardiac end of the SVC. Bidirectional: SVC to right pulmonary artery anastomosis with flow to both the RPA and LPA, part of Norwood procedure",,, Glisson's capsule,liver capsule,,, glycogen storage diseases,"type I, von Gierke’s disease; type II, Pompe’s disease; type III, Cori’s disease; type V, McAdle’s syndrome",,, Goetz sign,"in PDA, jet of unopacified blood from aorta into opacified blood of pulmonary artery",,, Goldblatt kidney,atrophic kidney from vasoconstriction of renal artery; results in hypertension,,, Goldenhar syndrome,syndrome of oculoauriculovertebral dysplasia characterized by unilateral deformity of the external ear and small ipsilateral half of the face with epibulbar lipodermoid and vertebral anomalies; autosomal dominant,,, Goldie-Coldman hypothesis,"malignant cells likely to acquire spontaneous resistance to cytotoxic drugs as they progressively grow and divide, even without any exposure to those drugs",,, Gonda’s maneuver,extensor plant response by flicking the little toe suggesting upper motor neuron defect,,, Good’s syndrome,"immunodeficiency, hypogammaglobulinemia associated with thymoma (mainly thymoma of spindle cell type); associated with recurrent pulmonary infections; described by Good in 1954",,, Goodell’s sign,softening of cervix associated with pregnancy that occurs at around 8th week,,, Goodpasture’s syndrome,"glomerulonephritis characterized by linear deposits of antibody along the glomerular basement membrane, antibodies interact with alveolar wall, leads to pulmonary hemorrhage and pulmonary fibrosis; Goodpasture antigen, resides in the noncollagenous portion of the alpha3 chain of collagen type IV; high prevalence of DRW15/DQW6, also see iron-deficiency anemia",,, Goodsall’s rule,anal fistulae course in a straight path anteriorly and take a curved path posteriorly,,, Gordon’s maneuver,extensor plant response by squeezing the calf muscle suggesting upper motor neuron defect,,, Gordon’s syndrome,"type 2 pseudohypoaldosteronism associated with volume expansion, hypertension, and otherwise normal renal function; felt be to be due to enhanced distal chloride reabsorption",,, Gorham-Stout disease,vanishing or disappearing bone disease; IL-6 has pathogenetic role,,, Gorlin-Goltz syndrome,"focal dermal hypoplasia characterzied by widespread lesions of dermal hypoplsia with herniation of adipose tissue, streaks of pigmentary disturbance following lines of Blaschko, and severe absence deformities of bone, mental retardation, defects of optic nerve",,, Gottron’s papules,"in dermatomyositis, scaling erythematous eruption or dark red patches over the knuckles, elbows, knees; may mimic psoriasis",,, Gower’s maneuver,"Duchenne’s muscular dystrophy, patient using hands to help himself get up",,, Gradenigo’s syndrome,"thrombosis of inferior petrosal sinus producing ipsilateral facial pain, lateral rectus muscle weakness",,, Grafenberg spot,erogenous area in anterior vaginal wall,,, Graham patch,piece of omentum used for repairing perforations,,, Graham Steell murmur,"early diastolic murmur of pulmonic regurgitation secondary to pulmonary hypertension, best heard in left 2nd interspace, may be indistinguishable from murmur of aortic regurgation; occurs in 12% of patients with tricuspid regurgiation; described by Graham Steell",,, Graham-Little syndrome,end-stage lichen planus of the scalp resulting in scarring alopecia of the scalp,,, Graves’s disease,"hyperthyroidism with diffuse goiter, ophthalmopathy, dermopathy from thyroid-stimulating Ig",,, Grawitz tumor,renal cell adenocarcinoma,,, Gregg effect,augmentation of left ventricular systolic performance with increase in coronary flow and perfusion pressure,,, Greig cephalopolydactyly syndrome,rare autosomal dominant developmental disorder characterized by craniofacial abnormalities and post-axial and pre-axial polydactyly as well as syndactyly of hands and feet from mutation in GLI3,,, Grey Turner’s sign,"local areas of discoloration about the umbilicus and particularly in the region of the loins, in acute hemorrhagic pancreatitis (1-2%) and other causes of retroperitoneal hemorrhage",,, Griffith’s sign,"in thyrotoxicosis, lag of the lower lids during elevation of the globes",,, Griscelli syndrome,"primary immunodeficiency syndrome associated with partial “albinism”; presents similarly to Chédiak-Higashi syndrome but without the giant intracytoplasmic inclusions; defect in exocytosis of cytolytic granules; due to mutation in RAB27A, a member of the RabGTPase family or mutation in myosin-Va",,, Grover’s disease,"transient acantholytic dermatosis; most common in males over 40, sudden onset of pruritus across trunk, neck, and proximal limbs; a polymorphic, pruritic, papulovesicular dermatosis characterized histologically by acantholysis",,, Guam disease,"Guam amyotrophic lateral sclerosis-parkinsonism-dementia linked to a plant excitant neurotoxin found in cycad plant eaten by Chamorro people, toxin believed to be excitotoxin beta-N-methylamino-L-alanine, a low-potency convulsant Science 1987;237:517-22",,, Guillain-Barré syndrome,"acute idiopathic polyneuropathy following minor infective illnesses, inoculations, or surgical procedures (suggested association with C. jejuni) resulting in immunologically-mediated demyelination and leading to progressive weakness",,, Gunn’s sign,tapering of veins on either side of AV crossing in hypertensive retinopathy (grade 3); see also Salus’s sign and Bonnet’s sign,,, Gunther’s disease,"AR congenital erythropoietic porphyria from decreased URO synthase activity, hemolytic anemia, cutaneous lesions",,, Guthrie test,"for diagnosis of PKU, a bacterial assay for phenylalanine",,, Guyon’s tunnel,ulnar tunnel,,, Hageman factor,factor XII,,, Hailey-Hailey disease,benign familial chronic pemphigus,,, Hakim-Adams syndrome,normal pressure hydrocephalus,,, Haldane effect,deoxygenated hemoglobin having a greater affinity for CO2 than oxygenated hemoglobin,,, Hallermann-Streiff syndrome,"autosomal recessive syndrome with bird-like facies with hypoplastic mandible and beaked nose, proportionate dwarfism, hypotrichosis, microphthalmia, and congenital cataract",,, Hallervorden-Spatz disease,"autosomal recessive juvenile-onset generalized neuroaxonal dystrophy characterized by progressive movement alterations such as dystonia, tardive dyskinesia, rigidity, choreoathetosis, pyramidal signs, mental retardation, and iron accumulation in the brain; associated with mutation in pantothenate kinase 2; Hallervorden, Nazi pathologist involved in Aktion T-4 adult euthanasia program",,, Ham’s test,"for diagnosing paroxysmal nocturnal hemoglobinuria, uses increased sensitivity of PNH-affected RBCs to lysis by complement; introduced in late 1930s",,, Hamman’s sign,"mediastinal crunch is a series of precordial crackles synchronous with the heart beat, not with respiration. Best heard in the left lateral position, it is due to mediastinal emphysema (pseudomediastinum), seen with Boerhaave’s syndrome",,, Hamman-Rich syndrome,"acute interstitial pneumonia, a rare fulminant form of lung injury that presents acutely (days to weeks from onset of symptoms), most commonly occurs in previously healthy individual, characterized by a temporally uniform lesion which reflects an episode of acute lung injury at a single point in time (different from usual interstitial pneumonia where lesions are different age); path shows diffuse alveolar damage (a nonspecific reaction pattern)",,, Hampton’s hump,"in pulmonary embolism/infarction, a wedge-shaped infiltrate that abuts the pleura; often associated with a small pleura effusion that is usually exudative and may be hemorrhagic; not specific",,, Hampton’s line,radiolucent collar of granulation tissue across the base of an ulcer,,, Hand-Schüller-Christian triad,"in multifocal Langerhans’ cell histiocytosis, triad of calvarial bone defects, diabetes insipidus, and exophthalmos",,, hangman’s fracture,"fracture of pars interarticularis of C2, hyperextension injury",,, Hannington-Kiff sign,ipsilateral loss of the adductor reflex with preservation of patellar reflex in strangulated obturator hernia,,, Hansel’s stain,special stain used to detect eosinophiluria on the urine sediment,,, Hansen’s disease,leprosy,,, Hardy-Weinberg equilibrium,p^2 + 2pq + q^2 for describing the frequency of these genotypes; sexual reproduction doesn’t cause a constant reduction in genetic variation in each generation but remains constant,,, harlequin syndrome,localized autonomic syndrome of heat or exercised-induced flushing and sweating limited to one side of the face with impairment of sweating and flushing on the contralateral side; possible link with Holmes-Adie syndrome,,, Harrison’s groove,"during active rickets, the protuberant rachitic abdomen pushes the plastic lower ribs outward on a fulcrum formed by the costal attachments of the diaphragm; the line of bending forms a groove or sulcus in the rib cage",,, Hartman’s procedure,proximal colostomy followed by stapled-off colon or rectum that is left in the peritoneal cavity,,, Hartnup disease,"an autosomal recessive disorder in which there is a reduction of small-intestinal and renal transport of certain neutral amino acids, including (Trp, a precursor of niacin), causing elevated amino acid excretion in urine and feces",,, Hashimoto’s encephalopathy,"neurologic complication of Hashimoto’s thyroiditis, mainly in people euthyroid, associated with a vasculitic condition with stroke-like episodes and cognitive impairment and diffuse progressive picture with dementia, seizures, movement disorders, psychosis, and sometimes delirium",,, Hashimoto’s thyroiditis,"first described in 1920, goitrous chronic autoimmune thyroiditis; in areas with sufficient iodine, elevated TSH is often viewed as evidence of chronic autoimmune thyroiditis as well as antithyroid antibodies; antithyroglobulin antibodies in 60% of patients and antithryoid microsomal antibodies in 95%",,, Hatchcock’s sign,upward pressure applied to the angle of the mandible (ramus) produces tenderness with mumps but no tenderness with adenitis,,, Hawkin’s sign,"in shoulder impingement, with the arm in a throwing position and flexed forward about 30 degress, forcibly internally rotate the humerus; pain suggests impingement of the supraspinatus tendon against the coracoacromial ligament",,, Hawthorne effect,"the effect (usually positive or beneficial) of being under study, upon the persons being studied; their knowledge of the study often influences behavior [city in Illinois; site of the Western Electric plant]",,, Heberden’s nodes,"characteristic in women, but not in men, represent prominent osteophytes at the DIP joints in osteoarthritis (enlargements of tubercles at the articular extremities of the distal phalanges",,, Heck’s disease,"oral focal epithelial hyperplasia associated with HPV 13, 32 infection",,, Heerfordt-Waldenström syndrome,"sarcoidosis associated with fever, parotid enlargement, anterior uveitis, and facial nerve palsy",,, Hegar’s sign,softening of the uterus at the junction between the cervix and the fundus during the first trimester of pregnancy,,, Heimlich maneuver,"abdominal thrust maneuver for clearing airway (blocked by foreign object, e.g. food) described in 1974 by Henry Heimlich ",,, Heineke-Mukulicz pyloroplasty,"longitudinal incision through all layers of the pylorus, sewed closed in a transverse direction to make the pylorus nonfunctional (used after truncal vagotomy)",,, Heiner syndrome,"cow milk allergy associated with pulmonary infiltrates, pulmonary hemosiderosis, anemia, recurrent pneumonia and failure to thrive.",,, Heinz bodies,seen in unstable hemoglobin and oxidant stress; precipitates of denatured hemoglobin on red blood cells; only visible when blood is supravital stained (crystal violet); not seen on routine blood smears,,, "Heister, spiral valves of","found in the neck of the gallbladder, where tiny folds of mucosal epithelium coalesce to form these valves, may assist in retaining bile between meals",,, HELLP syndrome,"hemolysis, elevated liver function tests, and low platelets; microangiopathic process seen in third trimester of pregnancy; seen in 20% of preeclampsia",,, hemoglobin Bart’s,"four gamma globin chains, seen in hydrops fetalis (alpha thalassemia), very high oxygen affinity",,, hemoglobin Constant Spring,"particularly common structural variant with alpha thalassemia in Asia, contains mutation which abolishes normal translation termination codon, so extra 31 residues read until another in-frame termination codon comees up",,, hemoglobin Gower,"embryonic, primitive hemoglobin. Gower 1, zeta2 episilon2; Gower 2, alpha2 epsilon2",,, hemoglobin Lepore,no beta chain; delta chain by delta-beta hybrid,,, hemoglobin Portland,"zeta2gamma2, primitive embryonic hemoglobin",,, HEMPAS,"Hereditary Erythrocyte Multinuclearity and Positive Acidified Serum test, also known as congenital dyserythropoietic anemia type II. A congenital anemia characterized by multinucleated red cell precursors and hemolysis of red blood cells of the patient with autologous serum during the Ham's test",,, "Henle, loop of",section of nephron between proximal and distal tubule located in the medulla and involved in reabsorbing 25-35% of filtered NaCl and reaborbing NaCl in excess of water,,, Henoch-Schönlein purpura,"systemic hypersensitivity disease of unknown cause characterized by purpuric rash, colicky abdominal pain (presumably due to focal hemorrhages into the GI tract), polyarthralgia, and acute glomerulonephritis; may result from deposition of circulating immune complexes within stuff; hypersensitivity purpura, etiology group A streptococci",,, "Hering, canals of","at the fringes of the portal tract, from the joining of bile canaliculi",,, Hering’s nerve,"carotid sinus nerve, CN IX, carries information to the vasomotor center in the brainstem",,, Hering-Breuer reflex,"stretch receptor (in smooth muscle of airways) reflex, responsible for apnea, i.e. decreased breathing frequency, as a result of lung inflation",,, Herlitz syndrome,"epidermolysis bullosa lethalis; mutation that prevents the folding of laminin 5 Gillis Herlitz, Swedish pediatrician, born 1902",,, Hermansky-Pudlak syndrome,"autosomal recessive syndrome from defect in AP-3 adaptor complex beta3A subunit or HPS1; characterized by idiopathic pulmonary fibrosis, oculocutaneous albinism, a platelet storage pool deficiency, and ceroid lipofuscinosis from defects of multiple cytoplasmic organelles: melanosomes, platelet dense granules and lysosomes.",,, Herring bodies,neurosecretory granules consisting of either vasopressin or oxytocin in neurohypophysis,,, Hers disease,"type VI glycogen storage disease, deficiency in liver phosphorylase; presents as hepatomegaly and growth retardation; hypoglycemic, hyperlipidemia, and hyperketosis usually mild if present; hepatomegaly and growth retardation improve with age and usually disppear at puberty",,, Hess test,see Rumpel-Leede sign. Noticed phenomenon while treating children with scurvy.,,, Hesselbach’s triangle,"where direct inguinal hernias occur, bound by the inguinal ligament, inferior epigastric a., and rectus abdominus muscle",,, Heyde’s syndrome,association between bleeding from gastrointestinal angiodysplasia and severe aortic stenosis which may resolve with aortic valve replacement; may be explained by acquired type IIA von Willebrand’s syndrome; first described by Hyde in N Engl J Med 1958;259:196.,,, Hickey-Hare test,"in evaluating hypernatremia, 5% NaCl infused at a rate of 0.05 mL/kg/min followed by measurements of urine volume and Uosm",,, Hill’s sign,"in aortic regurgitation, refers to popliteal cuff systolic pressure exceeding brachial cuff pressure by more than 60 mm Hg",,, Hippocratic fingers,clubbing,,, "Hippocratica, facies","the mask of death following peritonitis, i.e. as a result of vomiting and depressed circulation, the face becomes pinched and anxious, the cheeks hollow, and the eyes dim and beringed with dark circles",,, Hirano bodies,"in Alzheimer’s, intracytoplasmic proximal dendritic eosinophilic inclusions consisting of actin",,, Hirschprung’s disease,"megacolon; congenital disorder characterized by colonic dilatation proximal to an aganglionic, contracted distal colon and rectum; caused by gestational failure of neural crest cells to migrate to distal colon; an AD form has been reported with mutations of the RET gene, and an AR form with mutation of the endothelin-B-receptor gene",,, Hitzig zones,"in tabes dorsalis (syphilis), regions of reduced sensation found in central face, nipples, ulnar forearms, and peroneal legs, leading to painless ulcers and joint damage",,, Hodgkin’s disease,"lymphoma characterized by arising in a single node or chain of nodes, spreading in anatomically contiguous nodes, Reed-Sternberg cells, three subtypes (nodular sclerosis (most common, more common in women), mixed cellularity, lymphocyte predominance (lymphocyte depletion rarer))",,, Hoffman’s sign,"involuntary flexion of the digits when tapping or striking over the plamar digital aspects of the 2nd, 3rd, and 4th digits in upper motor neuron defect",,, Hoffman’s syndrome,"in hypothyroidism, diffuse muscle hypertrophy, accompanied by stiffness, weakness, painful muscle cramps, associated with elevated muscle enzymes",,, Hoigne reaction,"pseudoanaphylactic reaction, complication associated with intramuscular procaine penicillin, occurs in 1/1000 patients; characterized by tachycardia, elevated blood pressure, fear of imminent death, violent combativeness, unusual taste sensation, auditory or visual disturbances; rarely lasts longer than 30 min; felt to be due to inadvertent intracapillary infusion resulting in microembolization",,, Hollenhorst plaques,cholesterol emboli visible as small bright flecks lodged in arterial bifurcations in retina,,, Holme’s heart,single ventricle with normally-related great arteries,,, Holmes tremor,"symptomatic tremor caused by lesions in the brainstem, cerebellum, or thalamus; postural and/or action in nature and worsen during movement and markedly increase during goal-directed movements; affect predominantly proximal limbs, low frequency; previously labeled rubral tremor, midbrain tremor, thalamic tremor, myorhythmia, and Benedikt syndrome",,, Holmes-Adie syndrome,"frequently affects young women, large, often irregular pupils, unilateral at onset as well as segmental palsy and segmental spontaneous movement of the iris, delayed constriction in response to near vision, delayed redilation after near vision, impaired accommodation, absent light reflex, and absent deep tendon reflexes; may be caused by degeneration of ciliary ganglion, followed by aberrant reinnervation of the pupilloconstrictor muscles",,, Holter monitor,continuous ambulatory EKG monitoring; first described by Montana physician Normal Holter in 1949 (Science 1961;134:1214-1220),,, Holt-Oram syndrome,"AD syndrome, dysplasia of the upper limbs and atrial septal defect, reduction anomalies of the upper limbs, heart hand syndrome from mutation in TBX5",,, Homan’s sign,"deep thrombi in the larger outflow veins, causing edema of the foot and ankle and producing pain and tenderness on compression of the calf muscles (by either squeezing the calf muscles or forced dorsiflexion of the foot)",,, Homer-Wright pseudorosettes,"in neuroblastoma, tumor cells arranged about a central space filled with fibrillar extensions of the cells",,, honeymooner’s palsy,radial nerve palsy from compression of nerve between middle third of the humerus between the brachoradialis and forearm extensor muscles,,, Hoover’s sign,"a modification in the movement of the costal margins during respiration, caused by a flattening of the diaphragm; suggestive of empyema or other intrathoracic condition causing a change in the contour of the diaphragm",,, Hoover’s sign,"in hysterical weaknes, patient does not push down into your hand when you ask patient to lift weak extremity",,, Horner’s syndrome,"enophthalmos, ptosis, miosis, and anhidrosis, unilateral; small (miotic) pupil associated with mild ptosis (of the upper lid, not as pronounced as with oculomotor lesions) and sometimes loss of sweating (anhidrosis); if present since infancy, the ipsilateral iris is lighter and blue (heterochromia iridis); topical 4% cocaine will dilate normal pupil but not a desympathectomized pupil",,, Horton’s cephalalgia,cluster headache; also known as histaminic cephalalgia,,, Horton’s disease,temporal arteritis,,, Houndsfield unit,"scale named after inventor of CT, water 0 H, -1024 for air to 3000-4000 H for bone, relative scale",,, "Houston, valve of","rectal valves: superior, middle, and inferior",,, Howell-Evans syndrome,"inherited tylosis (keratoderma of palms and soles), strongly associated with esophageal squamous cell carcinoma",,, Howell-Jolly body,"in asplenia or malfunctional spleen, nuclear remnants on red blood cells as small, round, darkly-stained nuclear fragments; no special stains necessary; may be seen in 30-50% of adults but not in children with untreated celiac sprue",,, Howship’s lacunae,resorption pits on bone formed by osteoclasts,,, Howship-Romberg sign,pain along the inner aspect of the thigh; seen with an obturator hernia due to nerve compression,,, Hughes-Stovin syndrome,multiple pulmonary artery aneurysms with peripheral venous thrombosis,,, Hunner’s ulcer,"in 10% of interstitial cystitis, lesions that involve all layers of the bladder wall and appear as brownish-red patches on the bladder mucosa",,, Hunter’s syndrome,"mucopolysaccharidosis (II), iduronate sulfatase deficiency, X-linked, affecting degradation of dermatan sulfate and heparan sulfate, resulting in physical deformity and mental retardation, no corneal clouding",,, Huntington’s disease,"autosomal dominant from expanded CAG trinucleotide repeat 4p16.3 associated with chorea initially as restlessness, psychiatric symptoms, and dementia; Westphal variant, atypical variant where there is progressive rigidity and akinesia with little or no chorea",,, Hurler’s syndrome,"AR mucopolysaccharidosis (I H) caused by deficiency of alpha-L-iduronidase, with consequent accumulations of the mucopolysaccharides heparan sulfate and dermatan sulfate in the heart, brain, liver, and other organs; progressive deterioration, hepatosplenomegaly, dwarfism, gargoyle-like facies, stubby fingers, corneal clouding, progressive mental retardation, and death by age 10",,, Hurler-Scheie syndrome,"mucopolysaccharidosis I caused by deficiency of alpha-L-iduronidase (severe form is Hurler’s syndrome), have same problems as Hurler’s syndrome but progression is lower, little or no mental retardation, and they die in teens or 20s",,, Hurst’s disease,acute hemorrhagic leukoencephalitis,,, Hürthle cells,"in Hashimoto’s thyroiditis, deeply stained colloid or clusters of these oncocytes having an abundant, brightly eosinophilic granular cytoplasm, thought to represent a degenerated state of the follicular epithelium",,, Hutchinson freckle,"lentigo maligna, a nonfamilial precursor to lentigo maligna melanoma",,, Hutchinson pupil,blown third pupil in uncal herniation,,, Hutchinson’s sign,"in herpes zoster ophthalmicus, vesicular rash at nasal tip indicating involvement of the external nasal branch of CN V associated with increased incidence of ocular zoster",,, Hutchinson’s sign,pigment in the paronychial area suggesting melanoma,,, Hutchinson’s teeth,smaller and more widely spaced than normal and are notched on their biting surfaces; sign of congenital syphilis,,, Hutchinson’s triad,"congenital syphilis manifesting late at around 2 years old with triad of interstitial keratitis, notched incisors, and eighth nerve deafness",,, Hutchinson-Guilford syndrome,"aka progeria a condition in which the normal development of the first year is followed by gross retardation of growth, with a senile appearance characterized by dry wrinkled skin, total alopecia, and bird-like facies; genetics unclear",,, I-cell disease,"lysosomal disorder, from deficiency of ability to phosphorylate mannose, causing an incorrect targeting of potential lysosomal enzymes to extracellular sites instead of lysosomes, characterized by skeletal abnromalities, restricted joint movement, coarse facial features, severe pscyhomotor impairment, death by 8 y.o.",,, Imerslund-Grasbeck syndrome,"juvenile megaloblastic anemia, an autosomal recessive condition involving mutation in cubulin, receptor that binds IF-B12 complex; also associated with proteinuria",,, Irish’s node,"left axillary adenopathy associated with metastatic disease, e.g. gastric cancer",,, Isaacs’s syndrome,"neuromyotonia; continuous muscle stiffness, rippling muscle movements (myokymia), delayed relaxation following muscle contraction believed to be due to autoantibodies to presynaptic potassium channels",,, Ishihara plates,pseudoisochromatic plates (plates with color dots for numbers) for testing color vision,,, itai-itai disease,"bone disease from cadmium toxicity, characterized by multiple fractures, mixed pattern of osteoporosis and osteomalacia, and renal damage. Means “ouch-ouch” in Japanease, cadmium toxicity noted in exposed individuals living in Jinzu river basin in Japan who used river water contaminated with cadmium for irrigating rice fields",,, Ito cells,"vitamin A fat-storage cells, of mesenchymal origin found in the space of Disse; during development of cirrhosis, they become activated, transform into fibroblast-like cells",,, Ivor Lewis procedure,esophagectomy through right thoracotomy and intrathoracic esophagogastric anastomosis; described in 1946 by Ivor Lewis,,, Jaccoud’s arthritis,"in SLE, ulnar deviation of the fingers, swan neck deformities, and subluxations, initially reversible since not joint problem per se but can become fixed over time",,, jacksonian march,"in simple partial seizures, clonic movements of a single muscle group spreading to involve contiguous regions of the motor cortex",,, Jackson-Weiss syndrome,"craniosynostoses as well as limb defects, mutations if FGFR2, broad great toes",,, Jamaican vomiting sickness,poisoning from hypoglycin A in unripe ackee fruit; hypoglycin A is potent hypoglycemic agent that decreases rate of fatty-acid beta oxidation probably by inhibition of acyl dehydrogenase flavin-dependent oxidation; causes liver damage indistinguishable from Reye’s syndrome,,, James fibers,"tracts of atrial tissue running from the atria and inserting into the low AV node; functional significance not well established, controversial if associated with abnormal AV conduction",,, Janeway lesions,"in infective endocarditis; nonpainful, small, erythematous or hemorrhagic macules or nodules of palms or soles; more common in acute bacterial endocarditis but occur in subacute bacterial endocarditis",,, Jansen metaphyseal chondrodysplasia,"an autosomal dominant form of dwarfism resulting from an activating mutation in the PTHrP receptor, premature ossification from acceleration of the transition from proliferative to hypertrophic chondrocytes",,, Jansky-Bielschowsky disease,"late infantile neuronal ceroid lipofuscinosis, CLN2 disorder generally characterized by visual loss, epilepsy, and psychomotor deterioration",,, Jarisch-Herxheimer reaction,"sudden fevers, rigors, and persistent hypotension following antimicrobial treatment of louse-borne relapsing fever (spirochete Borrelia recurrentis infection) or syphilis; treatment with anti-TNF-alpha Fab before penicillin suppresses this reaction (NEJM 335:311)",,, Jatene’s arterial switch,"for treating transposition of great vessels, pulmonary artery and aorta are transected above valves and switched, and coronary arteries are moved from old aortic root to new aorta (former pulmonary root)",,, Jefferson fracture,fracture of C1 at more than one site; rarely associated with neurological deficits because of size of spinal canal at this level,,, Jendrassik maneuver,"for increasing sensitivity of deep tendon reflexes, have patient clasp hands together or grab wrists tightly while checking reflexes",,, Jerusalem syndrome,psychosis affecting visitors to Jerusalem whereby afflicted develop psychotic religious delusions; affects a handful of visitors each year,,, Jervell-Lange-Nielsen syndrome,long QT syndrome (autosomal recessive) associated with congenital deafness,,, Job’s syndrome,"immune deficiency where neutrophils fail to respond to chemotactic stimuli, associated with high levels of IgE; patients susceptible to cold staphylococcal abscesses",,, Jod-Basedow phenomenon,thyroid hyperfunction induced by excess iodine ingestion in patients with various thyroid disorders; Jod German for iodine; K. A. Von Basedow,,, Joffroy sign,"in thyrotoxicosis, absence of forehead wrinkling with upward gaze, the head being tilted down; disorder of the arithmetic faculty in the early stages of organic brain disease",,, John Thomas sign,"positive sign when penis inclined to side of the disorder radiographically (e.g., fractured hip); 70% sens 67% specific for JT sign in hip fracture, Med J. Aust. 1998;169:670",,, Jolly test,"in myasthenia gravis, a sequence of repetitive nerve stimulation (RNS) studies specifically designed to look for neuromuscular junction disease. A positive test is a >10% decremental response with 3 Hz repetitive stimulation, 50-80% sensitive. After Friedrich Jolly, German neurologist, 1844-1904",,, Jones criteria,"for diagnosing rheumatic fever, major criteria: carditis, polyarthritis, chorea, erythema marginatum, and subcutaneous nodules. minor criteria: arthralgia, fever, elevated ESR, CRP, prolonged PR. in setting of antecedent GAS infection, two major or one major and 2 minor suggests RF. throat cultures negative by the time rheumatic fever appears",,, Jones’s fracture,fracture at the base of the fifth metatarsal diaphysis,,, Jumping Frenchmen of Maine syndrome,"unusually extreme startle reaction that occurs in selected populations with reactions including echolalia, echopraxia; first described in French Canadian lumberjacks in the Moosehead Lake Region of Maine in the late 19th century; also described as latah in Malaysia",,, kabuki syndrome,"congenital mental retardation syndrome; postnatal dwarfism, a peculiar facies characterized by long palpebral fissures with eversion of the lateral third of the lower eyelids (reminiscent of the make-up of actors of Kabuki, a Japanese traditional theatrical form), a broad and depressed nasal tip, large prominent earlobes, a cleft or high-arched palate, scoliosis, short fifth finger, persistence of fingerpads, radiographic abnormalities of the vertebrae, hands, and hip joints, and recurrent otitis media in infancy; also known as Niikawa-Kuroki syndrome",,, Kahler’s disease,multiple myeloma,,, Kallman’s syndrome,"anosmia; hypogonadotropic hypogonadism stemming from failure of LHRH-expressing neurons to migrate, etc.",,, Kanavel's sign,four signs of tenosynovitis: 1. affected finger held in slight flexion; 2. pain over volar aspect of affected finger tendon upon palpation; 3. swelling of affected finger; 4. pain on passive extension of affected finger,,, Kandinsky-Clerambault's syndrome,"alienation from or loss of one’s own mental processes which are attributed to somebody else, combined with delusions of external influences, such as stealing or insertion of thoughts, described independently by Russian psychiatrist Victor Khrisanfovich Kandinsky (1849–1889) and French psychiatrist Gaëtan Gatian de Clerambault (1872–1934) ",,, Kaplan-Meier curve,curve that estimates the probability of survival of a defined group at a designated time interval (conditional probability) based on a non-parametric survival function,,, Kaposi’s sarcoma,"low-grade vascular tumor associated with HHV-8, four forms, classic (older men of Mediterranean or Jewish extraction), African, organ transplant-associated, and AIDS; differential for skin lesions includes bacillary angiomatosis from Bartonella",,, Kartagener’s syndrome,"a triad of sinusitis, bronchiectasis, and situs inversus; also associated with ciliary dysfunction; Manes Kartagener, Swiss physician, 1897-1975",,, Kasabach-Merritt syndrome,"capillary hemangioma associated with thrombocytopenic purpura and extensive and progressively enlarging vascular malformations which may involve large portions of their extremities; bleeding commonly develops in the first year of life, secondary to chronic DIC triggered by stagnant blood flow through the tortuous abnormal vessels; anemia caused by red cell damage as blood passes through deformed vessels of the tumor",,, Kashin-Beck disease,"deforming arthritis associated with selenium deficiency, identified in an area of China and Tibet where soil is extremely low in selenium; iodine deficiency risk factor",,, Katayama fever,"acute schistosomiasis, after Katayama valley in Japan where S. japonicum endemic; seen in travelers but not in native people because of exposure in utero; symptoms of prostration, fever, sweats, myalgia, and diarrhea",,, Kawasaki’s disease,"acute febrile illness of infants and children, characterized by cutaneous and mucosal erythema and edema with subsequent desquamation, cervical lymphadenitis, and complicated by coronary artery aneurysms (20%); associated with antiendothelial antibodies",,, Kayser-Fleischer rings,"a greenish yellow pigmented ring encircling the cornea just within the corneoscleral margin, seen in hepatolenticular degeneration, due to copper deposited in Desçemet’s membrane (posterior limiting layer of cornea); seen in Wilson’s disease (with neurologic involvement) and other cholestatic hepatic diseases",,, Kearns-Sayre-Daroff syndrome,"a mitochondrial disease with progressive ophthalmoplegia, retinal pigmentation degeneration, cerebellar ataxia, heart block, and elevated CSF protein",,, Kegel exercises,exercises for strengthening pelvic muscles in order to increase urethral closure mechanism in urinary incontinence,,, Kehr’s sign,pain in the left shoulder associated with splenic rupture,,, Kell blood group system,"red blood cell antigen, named after Mrs. Kelleher, pregnant woman in whom anti-K was discovered by Coombs in 1946; function of antigens in this system are unknown.",,, Kellgren’s arthritis,"severe form of primary osteoarthritis, erosive osteoarthritis, associated with severe osteoporosis of hands",,, Kelly’s sign,visible peristalsis of the ureter in response to squeezing or retraction; used to identify the ureter during surgery,,, Kennedy syndrome,"X-linked spinobulbar muscular atrophy; from CAG repeat expansion encoding androgen receptor; leads to distal limb amyotrophy, bulbar signs, fasiculations prominent, androgen insensitivity with gynecomastia, testicular atrophy, and oligospermia",,, "Kent, bundle of",AV bypass accessory tract in Wolf-Parkinson-White syndrome that directly connects atrial and ventricular myocardium,,, Kerandel’s hyperesthesia,"in African trypanosomiasis, excruciating pain after minor soft-tissue injury, e.g. in palms and ulnar region",,, Kerckring’s valves,plicae circulares (lining of small intestine),,, Kerley A lines,"in pulmonary edema, thin nonbranching lines several inches in length, radiating from hila, and not following the course of vessels or airways, attributed to thickening of connective tissue sheets which contain communicating perivenous and bronchoarterial lymphatics",,, Kerley B lines,"in pulmonary edema, transverse lines 1-3 cm in length and 1-2 mm in width, arranged in a horizontal stepladder pattern, 0.5-1 cm apart along the lower lateral lung margins, extending to the pleura; lines visible in this area because pulmonary lobules are well-developed in this area and lines reflect thickening of, or fluid in, lymphatic vessels in interlobular septa; also seen in lymphangitic spread of malignancies.",,, Kerley C lines,"in pulmonary edema, fine interlacing lines throughout the lung base producing a spider web appearance; controversial if unique lines v. crossing lines",,, Kernig sign,"first flex patient’s leg at both hip and knee, and then straighten knee; positive sign is pain and increased resistance to extending knee, suggesting meningeal irritation",,, Kernohan notch,"focal impression against the cerebral peduncle, pressure against notch in uncal herniation",,, Keshan disease,endemic cardiomyopathy in China associated with selenium deficiency,,, Keutel syndrome,"malformation syndrome characterized by multiple pulmonary stenoses, neural hearing loss, short terminal phalanges, and calcifications and/or ossification of the cartilage in ears, nose larynx, trachea, and ribs; due to mutation in gene encoding human matrix Gla protein",,, Kew Gardens spotted fever,"Rickettsialpox due to Rickettsia akari, from Kew Gardens, New York",,, Kidd blood group system,"named after Mrs. Kidd, pregnant woman in whom anti-Jka was discovered by Allen in 1951; antigens in this system are located on a red cell urea transporter",,, Kienböck disease,osteonecrosis of the carpal lunate that most commonly affects men between ages 20-40; sometimes history of trauma; may lead to end-stage arthritis of wrist with progression,,, Kiesselbach’s plexus,"vascular plexus on the anterior nasal septum, bleeding from this plexus associated with most common form of epistaxis",,, Kikuchi’s disease,"histiocytic necrotizing lymphadenitis, characterized by cervical lymphadenopathy with tenderness, fever, and night sweats; tends to occur in young women; histology can be confused with lymphoma",,, Killian-Jamieson area,region between oblique and transvere fibers of the cricopharyngeus muscle; potential area for muscular dehisence and Zencker’s diverticulum,,, Killip class,"described in 1967, in myocardial infarction, I, no heart failure, 0.5% mortality; II, S3 and/or basal lung crepitations, 2.2% mortality; III, acute pulmonary edema, 19.2% mortality; IV, cardiogenic shock, 61.3% mortality (mortality rates current)",,, Kimmelstiel-Wilson disease,intercapillary glomerulosclerosis from diabetes; lesion is PAS-positive material deposited at periphery of glomerular tufts,,, Kimura’s disease,large subcutaneous masses on head or neck of Asian males associated with eosinophilia,,, Klatskin tumors,"tumors arising from the part of the common bile duct between the cystic duct junction and the confluence of the right and left hepatic ducts at the liver hilus; notable for their slow growing behavior, marked sclerosing characteristics, and the infrequent occurrence of distal metastases",,, Kleihauer-Betke test,testing for the presence of fetal blood cells in maternal circ,,, Kleine-Levin syndrome,"a rare form of periodic hypersomnia, occurring in males aged 10 to 25 years, characterized by periods of ravenous binge eating alternating with prolonged sleep (as long as 18 hours), along with behavioral disturbances, impaired thought processes, and hallucinations; acute illness or fatigue may precede an episode, which may occur as often as several times a year; thought to be a disorder of hypothalamically mediated satiety",,, Klein-Waardenburg syndrome,see Waardenburg syndrome type III,,, Klinefelter syndrome,"XXY, first described in 1942 as constellation of enlarged breasts, sparse facial and body hair, small testes, long bone abnormality, and an inability to produce sperm; occurs in 1/500 to 1/1000 births",,, Klinger disease,Wegener’s disease; first described by Heinz Klinger in 1931 as a medical student and then described by Friederic Wegener in 1936,,, Klippel-Feil syndrome,"congenital fusion of two or more cervical vertebrae, producing a low posterior hairline, decreased movement, and a short neck that displaces the head anteriorly and inferiorly",,, Klippel-Trenaunay syndrome,"congenital condition characterized by port-wine stain (cutaneous capillary hemangiomas), soft tissue and bony hypertrophy, and venous malformations and lymphatic abnormalities, usually limited to one limb",,, Klippel-Trenaunay-Weber syndrome,"association of Klippel-Trenaunay syndrome with AV fistula; affects Casey Martin, golfer in disability case brought to U.S. Supreme Court 2001 against PGA",,, Klumpke’s palsy,lower plexus palsy affecting C8-T1 nerve roots,,, Klüver-Bucy syndrome,"1937 bilateral removal of temporal lobe (including amygdala and hippocampal formation) in monkeys, animals became tame, showed a flattening of emotions, exhibited remarkable oral tendencies (they put all manner of objects into their mouths), enormous increase in sexual behavior, including mounting of inappropriate objects and species, compulsive tendency to react to every object, failed to recognize familiar objects",,, Kniest dysplasia,autosomal dominant metatropic dwarfism associated with mutation in COL2A1,,, Koch’s bacillus,tubercule bacillus,,, Koch’s postulates,criteria for linking a specific microorganism to a disease 1. organism must be found in lesion of disease; 2. organism can be isolated in single colonies on a single medium; 3. inoculation of culture causes lesion in experimental animals; 4. organism can be recovered from lesions in these animals,,, Kocher’s incision,right subcostal incision for open cholecystectomy,,, Kocher’s maneuver,dissection of the duodenum from the right-sided peritoneal attachment to allow mobilization and visualization of the back of the duodenum/pancreas,,, Kocher’s test,"compression of lateral lobes of thyroid causing stridor; associated with thyroid carcinoma, goiter, or thyroiditis",,, Koebner’s phenomenon,"referring to physical trauma (rubbing or scratching) as a major factor in eliciting psoriasis lesions; also depigmented, sharply demarcated papules in vitiligo following minor trauma; may also be seen in lichen planus",,, Koeppe’s nodules,"projections into the pupil seen in sarcoidosis, tuberculosis, and other uveitides",,, Köhler’s disease,aseptic necrosis or osteochondrosis of the navicular bone,,, "Kohn, pores of",connections between alveoli,,, Koplik’s spots,"small, white spots (often on an reddened background) that occur on the inside of the cheeks early in the course of measles; considered to be pathognomonic exanthem in measles",,, Korotkoff sounds,"sounds heard in between systolic and diastolic pressure; origin related to the spurt of blood passing under the cuff and meeting a static column of blood; I, first appearance of low-frequency tapping sounds; II, softer and longer sounds; III, crisper and louder sounds; IV, muffled and softer sounds; V, complete disappearance of sounds",,, Korsakoff’s syndrome,profound memory loss; mammillary bodies as well as portions of medial thalamus believed to be destroyed from thiamine deficiency accompanying alcoholism,,, Kostmann syndrome,"inherited neutropenia, responds to G-CSF, may be due to environmental insults in bone marrow, characterized typically by a granulopoeisis impairment at the promyelocyte stage",,, Krabbe’s disease,"AR sphingolipidosis (sulfatidose), from beta-galactosidase deficiency, resulting in increased galactocerebrosides, mental retardation, blindness, deafness, paralysis, convulsions, total absence of myelin, globoid bodies in white matter of brain, fatal in early life",,, Krause’s end-bulbs,sensory receptors for cold,,, Krukenberg’s tumor,"metastatic GI neoplasia to the ovaries, produces bilateral metastases of mucin-producing, signet-ring cancer cells, most often of gastric origin",,, Kübler-Ross dying stages,"1. denial and isolation, 2. anger, 3. bargaining, 4. depression, 5. acceptance; described in 1969 by Elisabeth Kübler-Ross",,, Kufs disease,adult neuronal ceroid lipofuscinosis,,, Kugelberg-Welander disease,see under Wohlfart-Kugelberg-Welander disease,,, Kulchitsky’s cells,"neuroendocrine argentaffin cells present along the bronchial epithelium, particularly in the fetus and neonate; small cell carcinoma has granules similar to Kulchitsky’s cells; origin of carcinoid tumors",,, Kupffer cells,"phagocytic cells of the mononuclear phagocyte series found on the luminal surface of endothelial cells in hepatic sinusoids; they metabolize old RBCs, digest hemoglobin",,, Kussmaul’s respiration,"hyperpnea, associated with acidosis, especially DKA but also in uremia",,, Kussmaul’s sign,"jugular vein distension during inspiration (reversal of normal pattern of decreasing jugular venous pressure during inspiration); classically seen in constrictive pericarditis because of normal increase in venous return with increased abdominal pressure from diaphragmatic contraction leading to increase in right atrial pressure from non-complaint right ventricle, though most common contemporary cause is severe right-sided heart failure (JAMA 1996: 275:632); generally negative in cardiac tamponade",,, Kussmaul-Maier disease,old name for polyarteritis nodosa,,, Kveim antigen,saline suspension of human sarcoid tissue prepared from spleen of an individual with active sarcoidosis; used in Kveim-Sitzbach test,,, Kveim-Siltzbach test,"an intradermal test for the detection of sarcoidosis, done by injecting Kveim antigen (see elsewhere) and examining skin biopsies after 3 and 6 weeks; positive test, a noncaseating granulomatous reaction; sensitivity 35-88%, specificity 75-99% Morton A. Kveim, Norwegian dermatologist, born 1892",,, Kyrle disease,"chronic generalized dermatosis, formation of large papules with central keratin plugs; associated with diabetes mellitus and renal failure",,, La Crosse encephalitis,"transmitted by tree hole mosquito, Aedes trisereiatus and causes most common arboviral infection in children in N. America; hyponatremia and fever associated with worse outcome",,, Lachman maneuver,"for diagnosing ACL tear, pull on tibia in anterior direction with knee flexed at 20-25 degrees (if tibia slides anteriorly >2 mm, positive)",,, Ladin’s sign,an area of elasticity on the anterior face of the uterus just above the cervix; felt on palpation through the vagina as early as the 5th or 6th week,,, Lady Windermere syndrome,"mycobacterium avium complex pulmonary disease described in fastidious elderly women in the dependent portion of the lingula or the right middle lobe due to proposed habitual voluntary suppression of cough leading to the development of nonspecific inflammatory processes in these poorly draining lung regions, upon which MAC-PD engrafted. Chest 1992;101:1605-9",,, Laennec’s cirrhosis,"in alcoholic cirrhosis, residual parenchymal nodules that protrude like “hobnails” from the surface of the liver",,, Lafora’s disease,"progressive myoclonus epilepsy, autosomal recessive, onset teenage years, characterized by seizures and cumulative neurological deterioration, death usually within ten years of first symptoms; associated with intracellular inclusions (Lafora bodies); associated with mutation in gene EPM2A ",,, Laimer triangle,area defined by cricopharyngeus muscle and esophageal muscles,,, "Lambert, canals of",direct accessory bronchioalveolar connections,,, Lambert