82 - eponyms in medicine (1to500)

1 - Lorenzo’s oil is after Lorenzo Odone - diagnosed with adrenoleukodystrophy in 1984 - the triglycerides of monounsaturated oleic acid and erucic acid 4:1 - felt to reduce very long chain fatty acids to normal levels in ALD.

2 - veins of Mayo are veins overlying pylorus.

3 - Hutchinson freckle is lentigo maligna - a nonfamilial precursor to lentigo maligna melanoma.

4 - Chadwick’s sign is 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. This is also called as the jacquiemer’s sign.

5 - Cabot ring is seen 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 . Cabot's rings may be it the form of partial loops, loops, or figure eights. Their origin is not known and they may be artifacts. They are also rare. Cabot's ring; may be present in lead poisoning, pernicious anemia, thalassemia and other severe anemias. Cabot rings are red-purple staining threadlike filaments in the shape of a ring or figure 8 in the red blood cell, often in association with basophilic stippling. They may also appear as granules in a linear array rather than as complete rings. Cabot rings are thought to be microtubules from a mitotic spindle or remnants of the nuclear membrane. Cabot rings are seen in severe anemia including megaloblastic anemia, leukemia and lead poisoning. They are distinguished from the ring forms of Plasmodium by their larger size and by the absence of a red chromatin mass (Miale, J B: Laboratory Medicine Hematology 6th ed.,CV Mosby Co.,1982, p.488).

6 - Epstein-Barr virus, frequently referred to as EBV, is a member of the herpesvirus family and one of the most common human viruses. The virus occurs worldwide, and most people become infected with EBV sometime during their lives. In the United States, as many as 95% of adults between 35 and 40 years of age have been infected. Infants become susceptible to EBV as soon as maternal antibody protection (present at birth) disappears. Many children become infected with EBV, and these infections usually cause no symptoms or are indistinguishable from the other mild, brief illnesses of childhood. In the United States and in other developed countries, many persons are not infected with EBV in their childhood years. When infection with EBV occurs during adolescence or young adulthood, it causes infectious mononucleosis 35% to 50% of the time.Epstein-Barr virus is 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.

7 - Sugiura procedure is for treating esophageal varices - esophageal and gastric devascularization - esophageal transection.

8 - Sneddon-Wilkinson disease is subcorneal pustular dermatosis; rare - chronic - recurrent pustular eruption characterized by subcorneal pustules that contain abundant neutrophils.

9 - von Braun-Fernwald’s sign is Piskacek’s sign. asymmetry of the uterus with a well-defined soft prominence of the cornu, due to implantation near one of the cornua."

10 - Leydig cell tumors is testicular tumor derived from the stroma - may find Reinke crystals (q.v.)

11 - Guthrie test is used for the diagnosis of PKU - a bacterial assay for phenylalanine. The Guthrie test, also known as the Guthrie bacterial inhibition assay, is a medical test performed on newborn infants to detect phenylketonuria, an inborn error of amino acid metabolism. The Guthrie test is named after Robert Guthrie, an American bacteriologist and physician, who devised it in 1962. The Guthrie test is a semiquantitative assay designed to detect elevated blood levels of the amino acid phenylalanine, using the ability of phenylalanine to facilitate bacterial growth in a culture medium with an inhibitor.A drop of blood is usually obtained by pricking the heel of a newborn infant in a hospital nursery on the second or third day of life. The blood is collected on a piece of filter paper and mailed to a central laboratory. A small disk of the filter paper is punched out and placed on an agar gel plate containing Bacillus subtilis and B-2-thienylalanine. Each gel holds 60-80 disks. The agar gel is able to support bacterial growth but the B-2-thienylalanine inhibits bacterial growth. However, in the presence of extra phenylalanine leached from the impregnated filter paper disk, the inhibition is overcome and the bacteria grow. Within a day the bacterial growth surrounding the paper disk is visible to the eye. The amount of growth, measured as the diameter of the colony, is roughly proportional to the amount of phenylalanine in the serum. The result is read by comparing the diameter of each sample disk's colony to the colonies of a series of reference disks with standard phenylalanine content included on each large plate. The Guthrie assay is sensitive enough to detect serum phenylalanine levels of 180-240 μmol/L (3-4 mg/dL). In healthy normal people, phenylalanine levels are usually under 120 μmol/L. In recent years it is gradually being replaced in many areas by newer techniques such as tandem mass spectrometry that can detect a wider variety of congenital diseases.

12 - Ghon lesion is primary area of tuberculosis infection.

13 - Paneth cells are located in small intestine crypts - cells with apically oriented bright eosinophilic granules and which appear to play a role in the mucosal immune system.

14 - Stellwag’s sign is incomplete and infrequent blinking in Graves’s disease. ( sehwag plays infrequently , incompletely and inconsistently ) .

15 - Virchow’s node is supraclavicular adenopathy associated with a malignancy - often on left side - associated with stomach cancer among other neoplasms but also GI and pelvic malignancies in general. First described by Virchow in 1848 - more cases added by Troisier in 1886; referred to as Troisier’s node in France. The presence of this node enlargement is also called the troisier’s sign and seen in many malignancies .

16 - Lynch's syndrome I and II

Associated persons: Henry T. Lynch

Description: Lynch syndrome I: Familial predisposition to colorectal cancer with right-sided predominance. Predominantly early-onset proximal colon carcinomas.

Lynch syndrome II: Familial predisposition for other primary cancers in addition to the predisposition for colon cancer; site is often female reproductive organs. Predominantly early onset proximal colon carcinoma associated with other extracolonic adenocarcinomas, particularly endometrial carcinoma.

Both disorders are inherited as autosomal dominant traits.

First described 1913 by Alder Scott Warthin, 1867-1931.

Lynch syndrome II is cancer family syndrome - all features of Lynch I with early onset of carcinoma at other sites including endometrium - ovaries - and stomach - also upper tract TCC.

17 - Barraquer-Simons syndrome is acquired partial lipodystrophy; presents usually around 8-10 years of age - preceded generally by an acute viral infection; spares legs and hips; 1/3 of patients develop membranoproliferative glomerulonephritis.

18 - McLeod phenotype occurs from the absence of the Kx red blood cell antigen; characterized by acanthocytes; compensated hemolytic anemia; decreased Kell system antigens; increased CKMM isoenzymes; and progressive neurologic changes including areflexia - choreiform movements - dysarthria - wasting of muscles - and cardiomyopathy; may be associated with X-linked chronic granulomatous disease.

19 - Monod’s sign is seen in aspergillomas - radioluscent crescent seen around solitary 3-5 cm lesion on chest X-ray.

20 - TRALI syndrome is Transfusion Related Acute Lung Injury; transfusion reaction occurring within 6 hours after transfusion of blood product - characterized by pulmonary edema; due to anti-granulocyte antibodies in donor’s plasma causing pulmonary sequestration of recipient leukocytes in susceptible patient.

21 - Hering-Breuer reflex is stretch receptor (in smooth muscle of airways) reflex - responsible for apnea - i.e. decreased breathing frequency - as a result of lung inflation.

22 - Ransons’ criteria is for evaluating acute pancreatitis at presentation - age>55; WBC>16 -000; glucose>200; AST>250; LDH>350. during initial 48 hrs - base deficit>4; BUN increase>5; fluid sequestration>6L; Ca <8;>10; pO2<60.>

23 - Weibel-Palade bodies is found only in endothelial cells of vessels larger than capillaries; granules contain von Willebrand’s factor (VIII) and P-selectin.

24 - Dennie’s lines is in atopic dermatitis - an accentuated line or fold below the margin of the lower eyelid.

25 - Guam disease is 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.

26 - bundle of Kent is a AV bypass accessory tract in Wolf-Parkinson-White syndrome that directly connects atrial and ventricular myocardium.

27 - Graham-Little syndrome is end-stage lichen planus of the scalp resulting in scarring alopecia of the scalp.

28 - Van der Woude syndrome is an AD condition in which lip pits are seen in all gene carriers but only some individuals have cleft lips with or without cleft palate owing to variable expressivity.

29 - MacConkey medium is medium for growing nonfastidious Gram-negative rods.

30 - Hegar’s sign is softening of the uterus at the junction between the cervix and the fundus during the first trimester of pregnancy. So this is softening of the isthmus .

31 - Lachman maneuver is for diagnosing ACL tear - pull on tibia in anterior direction with knee flexed at 20-25 degrees (if tibia slides anteriorly >2 mm - positive).

32 - Allen’s sign is in pulmonary embolism - fever - tachycardia - and tachypnea - present in only 23% of cases.

33 - Isaacs’s syndrome is neuromyotonia; continuous muscle stiffness - rippling muscle movements (myokymia) - delayed relaxation following muscle contraction believed to be due to autoantibodies to presynaptic potassium channels.

34 - Stevens-Johnson syndrome is extensive and symptomatic febrile form of erythema multiforme - more common in children; 1-6 cases/million person-years.

35 - Betz cells is 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.

36 - Thomsen’s disease is myotonia congenita - autosomal dominant.

37 - ligament of Trietz - is the suspensory muscle of the duodenum which supports the duodenojejunal flexure.

38 - Bier block is regional anesthesia of an extremity by placing a tourniquet and then infusing local anesthetic into a vein.

39 - Trendelenburg position is position where angle of the head of bed or table is inclined at 45 degrees down; used in surgery to push the abdominal organs towards the chest.

40 - Crohn’s disease is 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.

41 - lines of Blaschkow - is 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.

42 - Tamm-Horsfall protein is uromodulin - major component of renal casts; 30-50 mg secreted per day by cells in thick ascending limb; homologous to GP2 - a protein secreted from the acinar cell and a major component of plugs in noncalcific chronic pancreatitis.

43 - Pringle maneuver is clamping of porta hepatis to control hemorrhage. placing an atraumatic clamp across the foramen of winslow .

44 - Treacher Collins syndrome is first arch syndrome - mandibulofacial dysplasia - caused by autosomal dominant gene - resulting in malar hypoplasia with down-slanting palpebral fissures - defects in the lower eyelids - deformed external ears - and sometimes abnormalities of the middle and internal ears.

45 - Reiter’s syndrome is triad of arthritis - urethritis - and conjunctivitis described by Reiter in 1916 (Nazi sympathizer); 80% possess HLA-B27; associated with Shigella flexneri; ReA develops in 20% of exposed B27+ individuals; subset of reactive arthritis; triad present in 1/3 of patients; incidence estimated as 3.5/100K in males under age of 50; balanitis circinata (penis) and keratoderma blenorrhagica.

46 - Gunther’s disease is AR congenital erythropoietic porphyria from decreased URO synthase activity - hemolytic anemia - cutaneous lesions.

47 - Von Myenburg complexes is close to or within portal tracts - small clusters of modestly dilated bile ducts embedded in a fibrous - sometimes hyalinized stroma; contain inspissated bile concrements and may communicate with the biliary tree; common and usually without clinical significance.

48 - Aicardi syndrome is 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.

49 - Lyme disease is inflammatory disease caused by spirochete Borrelia burgdorferi - spread by Ixodes tick - characterized by early local disease with erythema migrans - myalgias - followed by early disseminated disease characterized by carditis - neurologic findings (lymphocytic meningitis - cranial nerve palsies - also Bannwarth’s syndrome) - and late disease characterized by arthralgias and arthritis.

50 - Froment’s sign is 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.

51 - Marshall - vein of is oblique vein of left atrium.

52 - Cushing’s disease is hypercortisolism from pituitary corticotropin-(ACTH)-secreting corticotroph tumors - almost always benign and usually microadenomas.

53 - Bezold-Jarisch reflex is 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.

54 - Simon focus is in tuberculosis - nodule in subapical region which develops from hematogenous spread from initial infection in lower half of lung.

55 - Bell’s palsy is seventh nerve palsy; seen as a complication in diabetes - tumors - sarcoidosis - AIDS - and Lyme disease.

56 - Syme’s amputation is amputation at the ankle with removal of the malleoli and placement of the heel pad over the end of the remaining tibia; devised in 1842.

57 - Billroth’s cords is splenic cords found in the red pulp between the sinusoids - consisting mainly of fibrils and connective tissue cells.

58 - Louvel’s sign is in deep vein thrombosis - venous pain induced by coughing - prevented by pressing over proximal end of vein.

59 - Von Willebrand’s disease is deficiency in von Willebrand factor; frequency of 1% - most common inherited disorder of bleeding; type 1 and 3 reduced quantity of vWF; type 2 qualitative defects in vWF.

60 - Kussmaul’s respiration is hyperpnea - associated with acidosis - especially DKA but also in uremia.

61 - Berardinelli-Seip syndrome is 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.

62 - Kussmaul’s sign is 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.

63 - Nijmegen breakage syndrome is primary immunodeficiency autosomal recessive disorder that presents as microcephaly - a distinctive “bird-like” facies - growth retardation - recurrent pulmonary infections - chromosomal instability - radiation hypersensitivity - and increased incidence of malignancies - especially lymphomas from mutation in nibrin protein - a protein involved in DNA repair; cellular defect similar to ataxia-telangiectasia.

64 - Dobrin’s syndrome is TINU syndrome (tubulointerstitial nephritis with uveitis) - associated with bone marrow granulomas - first described in 1975.

65 - Westermark’s sign is in chest film - an abrupt tapering of a vessel caused by pulmonary embolism - focal oligemia.

66 - Jod-Basedow phenomenon is thyroid hyperfunction induced by excess iodine ingestion in patients with various thyroid disorders; Jod German for iodine; K. A. Von Basedow.

67 - Toldt - white lines of is the peritoneal reflections of the ascending and descending colon.

68 - Möbius syndrome is congenital facial paralysis with or without limb defects associated with maternal misoprostol use.

69 - Landolfi’s sign is in aortic regurgitation - systolic contraction and diastolic dilation of the pupil.

70 - Rasmussen’s encephalitis is progressive childhood disease characterized by severe epilepsy - hemiplegia - dementia - and inflammation of the brain potentially from autoantibodies to GluR3 antigen.

71 - La Crosse encephalitis is 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.

72 - Carney syndrome (or triad) is nonfamilial disorder the includes combination of three rare tumors: gastric leiomyosarcoma - pulmonary chondroma - extraadrenal paraganglioma but no cardiac manifestations; unrelated to Carney complex.

73 - Keutel syndrome is 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.

74 - Watson-Schwartz reaction is screening test for porphobilinogen (elevated for example in acute intermittent porphyria); mixing 4-dimethylaminobenzaldehyde with urine and then extract with chloroform; porphobilinogen extracts into upper aqueous layer giving it a pink color and urobilinogen extracts into chloroform layer; 50% positive at urinary concentration 5x upper limit of normal and consistently positive 10-20x upper limit of normal.

75 - Stuart factor is factor X.

76 - Pulfrich phenomenon is misperception of trajectory of moving objects (an optical illusion that can be elicited in normal subjects) - can be associated with optic neuritis.

77 - May-Thurner syndrome is iliofemoral deep vein thrombosis from impaired venous return because of compression of the left common iliac vein by overlying right common iliac artery.

78 - Bergmann gliosis is in ethanol abuse - proliferation of astrocytes adjacent to lost Purkinje cells between depleted granular cell and molecular layer of cerebellum.

79 - Preiser disease is spontaneous - non-traumatic osteonecrosis of the scaphoid bone.

80 - Winter’s formula is gives expected pCO2 (respiratory compensation) in uncomplicated metabolic acidosis; expected CO2=[HCO3]*1.54 + 8.36.

81 - Ruffini’s corpuscles is sensory receptors for heat.

82 - Corrigan’s pulse is 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.

83 - Jolly test is 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.

84 - Kocher’s test is compression of lateral lobes of thyroid causing stridor; associated with thyroid carcinoma - goiter - or thyroiditis.

85 - Lieberkühn - crypts of is intestinal glands found between villi.

86 - Werdnig-Hoffman syndrome is spinal muscular atrophy type I - autosomal recessive lower motor neuron disease - survival motor neuron protein affected (linked to 5q11.2-13.3) - presents between birth and 6 months of age - death before 2 y.o.

87 - Blau’s syndrome is granulomatous synovitis - non granulomatous uveitis - cranial neuropathies; resembles childhood sarcoidosis; no lung involvement; associated with mutations in NOD2/CARD15 gene; first described in 1985.

88 - Conradi-Hunermann syndrome is chondrodysplasia punctata - characterized by stippled epiphyses from abnormal accumulation of calcium salts and skeletal changes.

89 - Biette’s collarette is in syphilis - a thin white ring of scales on the surface of a lesion.

90 - Marjolin's ulcer is squamous cell carcinoma ulceration overlying chronic osteomyelitis or burn scar.

91 - Wilson’s disease is hepatolenticular degeneration due to mutation in gene involved in incorporation of copper into ceruloplasmin and excretion of copper into bile; Kayser-Fleischer ring; gene on chr 13 - cation transporting P-type ATPase; incidence 1:200 -000 - diagnosis based on decrease in serum ceruloplasmin - increased urinary excretion of copper - increase in hepatic copper content; 40% have neurologic findings (Parkinson’s - psychosis) and subclinical liver.

92 - Wolman disease is lysosomal acid lipase deficiency hydrolysis of cholesteryl esters and triglycerides in the lysosome leading to mild mental retardation - hepatomegaly - adrenal calcification - fatal in infancy with inanition - malabsorption - and intractable diarrhea.

93 - Turner’s syndrome is females with XO - short - low-set ears - shield chest - congenital heart defect (usually coarctation) - café-au-lait spots - freckles - webbed neck - lymphedema.

94 - Wada test is test for hemispheric dominance for language by injecting amobarbital into carotid artery.

95 - Wallerian degeneration is pattern of degeneration of distal portion of nerve following axonal injury with break down of axon and formation of myelin ovoids from catabolized axon fragments.

96 - Hitzig zones is 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.

97 - Blumberg sign is rebound tenderness.

98 - Frank’s sign is earlobe crease - associated with CAD (N Engl J Med. 1973;289:327-8).

99 - Gorham-Stout disease is vanishing or disappearing bone disease; IL-6 has pathogenetic role.

100 - Holme’s heart is single ventricle with normally-related great arteries.

101 - Kleihauer-Betke test is testing for the presence of fetal blood cells in maternal circulation .

102 - Van Wyk-Grumbach syndrome is primary hypothyroidism associated with precocious puberty and galactorrhea.

103 - Riley-Day syndrome is hereditary sensory and autonomic neuropathy type III (familial dysautonomia) - recessive disorder that commences in infancy and is characterized by conspicuous autonomic dysfunction (absent tearing - labile temperature - and blood pressure) - and accompanied by absent taste sensation - absent fungiform papillae on tongue - impaired pain and temperature sensation - and areflexia; occurs among Ashkenazi; associated with mutation in IKBKAP gene.

104 - pink disease is acrodynia - occurs from exposure to high concentrations of mercury vapor - characterized by a body rash - swelling and irritation of palms and feet followed by skin desquamation - irritability - photophobia - fever - insomnia and profuse sweating - which may also follow oral exposure to mercury compounds.

105 - Amadori product is products of early non enzymatic glycoslyation of proteins.

106 - Marshall-Smith syndrome is accelerated skeletal maturation - failure to thrive - and dysmorphic facial features with death in early infancy or childhood from pulmonary infections.

107 - Lowe’s syndrome is X-linked - oculocerebrorenal syndrome characterized by congenital cataracts - mental retardation - renal tubular acidosis type 2; from defect in lipid phosphatase - phosphatidylinositol 4 -5 bisphosphate [PtdIns(4 -5)P2]5-phosphatase - which localizes to the Golgi apparatus and is suspected to play a role in Golgi vesicular transport.

108 - hemoglobin Lepore is no beta chain; delta chain by delta-beta hybrid.

109 - gamekeeper’s thumb is injury to ulnar collateral ligament of the thumb.

110 - Batten’s disease is 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.

111 - Bishop’s score is 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.

112 - Gerstmann syndrome is finger agnosia - agraphia - right-left disorientation - and dyscalculia.

113 - Milkman’s fractures is in osteomalacia - radiolucent narrow lines that lie either at right angles or obliquely to the cortical outlines of bones and often transect them; bilateral and symmetric - found at the axillary margins of the scapula - lower ribs - neck of the proximal femurs - and posterior regions of the proximal ulnas; related either to stress fractures or to mechanical erosion by penetrating nutrient arteries; aka Looser’s zones.

114 - hemoglobin Constant Spring is 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.

115 - Stockholm syndrome is victims sympathizing with victimizer - e.g. - kidnapper; described after incident in summer of 1973 in Stockholm where hostages were taken in a failed bank robbery and after end of their captivity in six days - the hostages resisted rescue.

116 - Kegel exercises is exercises for strengthening pelvic muscles in order to increase urethral closure mechanism in urinary incontinence.

117 - Charcot-Marie-Tooth disease is 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.

118 - Spigelian hernia is hernia through the linea semilunaris - aka spontaneous lateral ventral hernia.

119 - Vater - ampulla is location where common bile duct enters the duodenum; described by German anatomist Vater (1684-1751).

120 - Von Graefe sign is in Graves’s disease - lag of the upper eyelid as it follows the rotation of the eyeball downward.

121 - Waldenström’s macroglobulinemia is marked by diffuse - leukemia-like infiltration of the bone marrow by lymphocytes - plasma cells - and hybrid forms that synthesize a monoclonal IgM - leading to macroglobulinemia; disease of old age - macroglubilinemia giving rise to visual impairment - neurologic problems - bleeding - cryoglobulinemia; hyperviscosity.

122 - St. Vitus’s dance is Sydenham’s chorea.

123 - Bing’s sign is extensor plantar response by pricking the dorsal surface of the big toe with a pin suggesting upper motor neuron defect.

124 - Thorel’s pathway is posterior internodal tract in atrial conduction system.

125 - Parkland formula is total body surface area % burned x kg x 4; 1/2 in first 8 hours - second 1/2 given next 16 hours.

126 - Millard-Gubler syndrome is ventral pontine injury causing symptoms similar to Fouville’s syndrome except lateral rectus weakness only - instead of gaze palsy.

127 - Mazzotti reaction is reaction to proteins released by dying onchocerca - including fevers - rashes - ocular damage - joint and muscle pain - and lymphangitis as well as hypotension - pyrexia - respiratory distress - and prostration.

128 - Werner’s syndrome is a form of progeria characterized by scleroderma-like skin changes (especially in extremities) - bilateral juvenile cataracts - subcutaneous calcifications - wizened and prematurely-aged facies - hypogonadism - and diabetes mellitus; autosomal recessive inheritance; from mutation in RecQ helicase.

129 - fetor hepaticus is “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).

130 - Lynch syndrome I is cancer family syndrome with multiple colon cancers 2 to 3 decades earlier - predilection for proximal colon; Lynch syndromes most common forms of familial colon cancer - 5-10% of all cases of colon cancer; better prognosis than sporadic colon cancer; deficiency in mismatch repair genes in 85% of Lynch syndromes; autosomal dominant.

131 - Prehn's sign is elevation of painful testicle decreases pain of epididymitis.

132 - Ramirez sign is in deep vein thrombosis - sphygmomanometer cuff placed above knee inflated to 40 mm Hg causing pain at site of thrombosis.

133 - second disease is scarlet fever - aka scarlatina - caused by Strep pyogenes exotoxin - first described in 1626.

134 - Burkitt lymphoma is 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.

135 - Gordon’s maneuver is extensor plant response by squeezing the calf muscle suggesting upper motor neuron defect.

136 - Jones’s fracture is fracture at the base of the fifth metatarsal diaphysis.

137 - Hughes-Stovin syndrome is multiple pulmonary artery aneurysms with peripheral venous thrombosis.

138 - Siegrist streaks is linear hyperpigmented areas over choroidal vessels in hypertensive retinopathy.

139 - Blomstrand dysplasia is rare lethal disorder characterized by an increase in bone density and advanced skeletal maturationfrom inactivating mutation in PTHR-1 gene (also Jansen metaphyseal chondrodysplasia where there is an activating mutation).

140 - MELAS is mitochondrial encephalopathy with acidosis and stroke - onset in childhood - stroke-like episodes with hemiparesis - hemianopia - or cortical blindness; full expression of the disease leads to death often before age 20; 80-90% have point mutation in leucine tRNA in mtDNA.

141 - Hampton’s line is radiolucent collar of granulation tissue across the base of an ulcer.

142 - Clerambault’s syndrome is erotomania - delusional belief that someone (usually of higher social status) is in love with the person.

143 - Tolosa-Hunt syndrome is idiopathic inflammation of the cavernous sinus producing painful palsy of third - fourth - or sixth cranial nerve.

144 - Lundberg A wave is in ICP monitoring - waves that have a duration of 5-20 minutes and an amplitude of 50 mm Hg over the baseline ICP - after which ICP is reset to a higher baseline level; sign of severely compromised intracranial compliance and can result in significant decrease in cerebral perfusion pressure and herniation.

145 - Denver shunt is 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.

146 - Dorello’s canal is location where the sixth nerve penetrates the dura - an area where the nerve is liable to injury.

147 - Hageman factor is factor XII.

148 - Reynold’s pentad is Charcot’s triad plus altered mental status and shock in cholangitis.

149 - Morton’s neuroma is interdigitial palmar neuroma.

150 - Castle intrinsic factor is intrinsic factor secreted by parietal cells - which binds luminal B12 and permits its absorption in the ileum.

151 - Kernig sign is 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.

152 - Lisker’s sign is in thrombophlebitis or deep vein thrombosis - tenderness to percussion of the medial anterior tibial surface.

153 - Flatbush diabetes is GAD antibody negative diabetes in adult black subjects with diabetic ketoacidosis and increased frequency of human leukocyte antigen DR3 and DR4.

154 - Canavan disease is 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.

155 - Marie’s sign is fine tremor in hyperthyroidism.

156 - Wright’s stain is stain used for blood and bone marrow films.

157 - Morgagni - hydatids of is fallopian tube cysts found near the fimbriated end or in the broad ligaments.

158 - Zieve’s syndrome is jaundice - hyperlipemia - and hemolytic anemia in alcoholic liver disease; described in 1958.

159 - Cushing’s triad is signs of increased ICP 1. hypertension 2. bradycardia 3. irregular respirations.

160 - Meissner’s plexus is submucous plexus; innervates glandular epithelium - muscularis mucosa - intestinal endocrine cells - and submucosal blood vessels.

161 - Auerbach’s plexus is myenteric plexus - between the longitudinal and circular layers of muscle; provides motor innervation to the two muscle layers and secretomotor innervation to the mucosa.

162 - Magendie - foramen of is midline foramen exiting out of fourth ventricle.

163 - Bertin - renal columns of is the spaces between adjacent pyramids where cortical tissue extends into .

164 - Hutchinson-Guilford syndrome is 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.

165 - Stein-Leventhal syndrome is polycystic ovarian disease.

166 - Maroni sign is erythema of the skin overlying thyroid in hyperthyroidism.

167 - Lhermitte-Duclos disease is dysplastic cerebellar gangliocytoma; associated with germ line PTEN mutations; felt to be a part of multiple hamartoma syndrome or Cowden’s syndrome.

168 - Ball’s disease is 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.

169 - Costen’s syndrome is ear pain - tinnitus - impaired hearing - and dizziness from temporomandibular joint dysfunction.

170 - Bohr equation is Vd/Vt - for determining ratio of physiologic dead space.

171 - Gorlin-Goltz syndrome is 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.

172 - Doi’s sign is elicitation of diminished deep tendon reflexes after maximal voluntary contraction in Lambert-Eaton syndrome.

173 - Kussmaul-Maier disease is old name for polyarteritis nodosa.

174 - Lafora’s disease is 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.

175 - Kveim antigen is saline suspension of human sarcoid tissue prepared from spleen of an individual with active sarcoidosis; used in Kveim-Sitzbach test.

176 - Schick’s test is for testing immune status to Cornyebacterium diphtheriae - intradermal injection of 0.1 mL of purified standardized toxin; if no inflammation - antitoxin present.

177 - Swyer-James syndrome is in pediatrics - acquired hypoplastic lung that develops after severe obliterative bronchiolitis associated with bronchiolar obstruction - bronchiectasis - and distal air-space destruction.

178 - Kernohan notch is focal impression against the cerebral peduncle - pressure against notch in uncal herniation.

179 - stiff-man syndrome is chronic disorder of involuntary stiffness - painful muscle spasms - and rigidity - predominantly in the axial muscles; often occurs in conjunction with autoimmune diseases - particularly type I diabetes - associated with circulating antibodies to glutamic acid decarboxylase.

180 - Steele-Richardson-Olszewski syndrome is aka progressive supranuclear palsy.

181 - Hollenhorst plaques is cholesterol emboli visible as small bright flecks lodged in arterial bifurcations in retina.

182 - Reinke crystals is in 25% of Leydig cell tumors - intracytoplasmic rod-shaped crystalloids.

183 - Heyde’s syndrome is 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.

184 - Bannayan-Ruvalcaba-Riley syndrome is Bannayan-Zonana syndrome

185 - Brodman area is neuro anatomical classification of the cortex based on its cyto architecture into 52 areas; e.g. precentral gyrus Brodman 4 - striate cortex Brodman 17

186 - Dressler’s beat is fusion beat seen in ventricular tachycardia.

187 - Leydig cell tumors is testicular tumor derived from the stroma - may find Reinke crystals (q.v.).

188 - Jatene’s arterial switch is 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).

189 - Kartagener’s syndrome is a triad of sinusitis - bronchiectasis - and situs inversus; also associated with ciliary dysfunction; Manes Kartagener - Swiss physician - 1897-1975.

190 - Hippocratic fingers is clubbing.

191 - Oliver’s sign is in aortic aneurysm - pulsation of aorta felt through cricoid cartilage when chin is elevated.

192 - Brodie’s abscess is small - intraosseus abscess that frequently involves the cortex and is walled off by reactive bone.

193 - Spurling’s test is for diagnosing cervical radiculopathy - exerting downward pressure on the head while rotating the head towards the symptomatic side creating pain radiating into affected extremity.

194 - Parkland formula is total body surface area % burned x kg x 4; 1/2 in first 8 hours - second 1/2 given next 16 hours.

195 - farmer’s lung is 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).

196 - Drummond - marginal artery of is 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.

197 - Heimlich maneuver is abdominal thrust maneuver for clearing airway (blocked by foreign object - e.g. food) described in 1974 by Henry Heimlich.

198 - Möbius’s sign is in Graves’s ophthalmopathy - failure of ocular convergence following close accommodation at a distance of 5 inches.

199 - Cheyne-Stokes breathing is 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.

200 - Sly’s syndrome is mucopolysaccharidosis (VII) - from beta-glucoronidase deficiency - resulting in hepatosplenomegaly - physical deformity from defect in degradation of dermatan sulfate and heparan sulfate.

201 - The Mentzer index is used to differentiate iron deficiency anemia from beta thalassemia. It is calculated from the results of a complete blood count. If the quotient of the mean corpuscular volume divided by the red blood cell count is less than 13, thalassemia is more likely. If the result is greater than 14, then iron-deficiency anemia is more likely.

202 - Myerson's sign is a medical condition where a patient is unable to resist blinking when tapped on the glabella, the area above the nose and between the eyebrows. It is often an early symptom of Parkinson's disease.

203 - Swedish porphyria is acute intermittent porphyria.

204 - Bornholm disease is coxsackie virus producing pleurodynia - fever - cough - sore throat - myalgias in shoulder - chest - and abdomen; Bornholm is a Danish island in Baltic sea.

205 - Hamman-Rich syndrome is 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).

206 - Reichert’s cartilage is branchial arch 2.

207 - Potter’s sequence is from oligohydramnios (from e.g. renal agenesis - amniotic leak) leading to amnion nodosum - fetal compression which leads to pulmonary hypoplasia - altered facies - positioning defects of feet - hands - and breech presentation.

208 - CADASIL is 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.

209 - Bechterew’s disease is ankylosing spondylitis.

210 - Osler’s nodes is tender to painful - purplish - split pea-sized - subcutaneous nodules in the pulp of the fingers and/or toes and thenar and hypothenar eminences; transient - disappearing within several days (5% of patients); in acute bacterial endocarditis - associated with minute infective emboli; aspiration may reveal the causative organism; in subacute bacterial endocarditis - associated with immune complexes and small-vessel arteritis of skin.

211 - Eagle effect is 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).

212 - Ortner’s syndrome is hoarseness from compression of left recurrent laryngeal nerve by a greatly dilated left atrium (e.g. - in mitral stenosis) - enlarged tracheobronchial lymph nodes - and dilated pulmonary artery.

213 - Weber test is 512 Hz tuning fork placed on patients’s forehead; sound localizes towards side with conductive hearing loss; localizes away from the side with soensorineural loss.

214 - Hering - canals of is at the fringes of the portal tract - from the joining of bile canaliculi.

215 - Pott’s disease is tuberculous involvement of the spine; occurs in about 2% of TB cases.

216 - Hunter’s syndrome is 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.Hunter syndrome is A syndrome with variable manifestations exhibiting mainly microcephaly characteristic facies mental retardation short stature acral skeletal anomalies with occasional craniosynostosis and congenital heart defects.

217 - Imerslund-Grasbeck syndrome is juvenile megaloblastic anemia - an autosomal recessive condition involving mutation in cubulin - receptor that binds IF-B12 complex; also associated with proteinuria.

218 - Jerusalem syndrome is psychosis affecting visitors to Jerusalem whereby afflicted develop psychotic religious delusions; affects a handful of visitors each year.

219 - Archibald’s sign is in pseudohypoparathyroidism of Albert’s hereditary osteodystrophy - characteristic shortening of the fourth and fifth digits as dimpling over the knuckles of a clenched fist.

220 - Susac syndrome is microangiopathy of the inner ear - retina - and brain leading to deafness - retinal artery occlusion - and encephalopathy.

221 - lockwood's operation - a type of LOW operation done for femoral hernia .

222 - raspberry tumor - other name for - umbilical adenoma - congenital .

223 - gibbon's hernia - hernia with hydrocele .

224 - moore's classification - classification of omphalocele ( exampholos ) .

225 - berger's hernia - hernia into the pouch of the douglas is called berger's hernia .

226 - guaiac test - used to test for occult blood in stool .

227 - spider leg appearance on IVP is suggestive of - RENAL CYST .

228 - flower vase pattern of the pelvis in an intravenous urogram is seen in - horse shoe kidney .

229 - golf hole ureter - seen in tuberculosis of the ureter .

230 - Fanconi’s syndrome is generalized dysfunction of proximal renal tubule leading to glycosuria - hyperphosphaturia - hypophasphatemia - aminoaciduria - and systemic acidosis; may be associated with out-dated tetracyclines.

231 - hangman’s fracture is fracture of pars interarticularis of C2 - hyperextension injury.

232 - Castellani’s paint is 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.

233 - McDonald’s sign is uterine corpus and cervix can be easily flexed on each other due to Hegar’s sign.

234 - Paget’s disease of vulva is intraepithelial adenocarcinoma - less than 1% of all vulva malignancies.

235 - Finkelstein’s test is 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.

236 - Rotor’s syndrome is poorly defined defects in hepatic uptake and storage of bilirubin; resembles Dubin-Johnson syndrome - but liver pigment missing.

237 - Duffy blood group system is 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.

238 - Dubin-Johnson syndrome is 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.

239 - Vincent’s infection is acute necrotizing ulcerative gingivitis - occurring in patients with decreased resistance to infection - from concurrent infection with the symbiotic bacteria Fusobacterium fusiforme and Borrelia vincentii.

240 - Dance’s sign is empty right lower quadrant in children with ileocecal intussusception.

241 - Pickwickian syndrome is obesity hypoventilation syndrome defined by extreme obesity and alveolar hypoventilation during wakefulness - characterized by hypersomnolence - dyspnea - hypoxemia (cyanosis - polycythemia - and plethora) - and pulmonary hypertension leading to RV failure and edema; based upon Charles Dickens’ book “The Posthumous Papers of the Pickwick Club” and the character Joe who was a “wonderfully fat boy - standing upright with his eyes closed”.

242 - Voight - lines of is boundaries which delimit distribution area of main cutaneous nerves; seen in black and Asian skin and rarely in white subjects.

243 - Aaron's sign is a referred pain felt in the epigastrium upon continuous firm pressure over McBurney's point. It is indicative of appendicitis.Aaron's sign is named for Charles Dettie Aaron, an American gastroenterologist.

244 - Gianotti-Crosti syndrome is 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.

245 - Claude’s syndrome is 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).

246 - Parinaud’s syndrome is dorsal midbrain syndrome with supranuclear vertical gaze disorder from damage to posterior commissure with loss of upgaze - convergence-retraction nystagmus - downward ocular deviation - lid retraction - due to hydrocephalus from aqueductal stenosis - pineal region tumors.

247 - Fay sign is in carotid arteritis - pressure along the carotid causing pain to spread in distal branches of external carotid to jaw - ear - and temple.

248 - fox sign is seen in acute pancreatitis .Fox's sign is in hemorrhagic pancreatitis - ecchymosis of the inguinal ligament due to blood tracking from the retroperitoneum and collecting at the inguinal ligament.

249 - floating water lily sign is seen in x-ray of hydatid cyst of liver .

250 - HACEK group of organisms - HEMOPHILUS , ACTINOBACILLUS , CARDIOBACTERIUM , EIKINELLA and KINGINELLA .

251 - Duncan’s disease is 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.

252 - Gerstmann syndrome is finger agnosia - agraphia - right-left disorientation - and dyscalculia.

253 - Jamaican vomiting sickness is 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.

254 - Adie syndrome, sometimes known as Holmes-Adie's syndrome or Adie's Tonic Pupil, is a neurological disorder which affects the pupil of the eye and the autonomic nervous system.[1] It is caused by damage to the postganglionic fibers of the parasympathetic innervation of the eye, usually by a viral or bacterial infection which causes inflammation, and characterized by a tonically dilated pupil.Adie syndrome presents with three hallmark symptoms, namely abnormal pupil size, loss of deep tendon reflexes and diaphoresis.[1] Other signs may include hyperopia due to accommodative paresis, photophobia and difficulty reading.

255 - clay shoveler’s fracture is fracture of spinous process of C7.

256 - jacksonian march is in simple partial seizures - clonic movements of a single muscle group spreading to involve contiguous regions of the motor cortex.

257 - Goldenhar syndrome is 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.

258 - Schwartz’s dictum is no acid - no ulcer.

259 - Young’s syndrome is clinical features similar to cystic fibrosis - including bronchiectasis - sinusitis - and obstructive azoospermia - but don’t have increased sweat chloride values - nor pancreatic insufficiency - nor abnormal nasal potential differences - nor the CF delta F508 mutation; affected individuals are often middle-aged males identified during evaluation for infertility.

260 - Mayer-Rokitansky-Küster-Hauser syndrome is absence of vagina from abnormality of müllerian development.

261 - Lambert - canals of is direct accessory bronchioalveolar connections.

262 - Carnett’s test is head raise - tenderness persists in abdominal wall condition (rectus hematoma) whereas pain due to intraperitoneal disease lessens.

263 - Hardy-Weinberg equilibrium is 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.

264 - Pendred’s syndrome is autosomal recessive goiter and congenital sensorineural deafness from mutation in pendrin - a transport protein that affects organification of thyroglobulin; also malformations of cochlea and hypothyroidism (40%); pendrin involved in transport of chloride and iodide.

265 - Cullen’s sign is 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%).

266 - Jefferson fracture is fracture of C1 at more than one site; rarely associated with neurological deficits because of size of spinal canal at this level.

267 - Takayasu’s disease is aortic arch syndrome - pulseless disease; panartertis of the great vessels that’s most common in Asian women.

268 - baghdad boil - Leishmaniasis - This disease is also known as Leichmaniosis, Leishmaniose, leishmaniose, and formerly, Orient Boils, Baghdad Boil, kala azar, black fever, sandfly disease, Dum-Dum fever or espundia.Leishmaniasis is a disease caused by protozoan parasites that belong to the genus Leishmania and is transmitted by the bite of certain species of sand fly, including flies in the genus Lutzomyia in the New World and Phlebotomus in the Old World. The disease was named in 1901 for the Scottish pathologist William Boog Leishman.

269 - Van Wyk-Grumbach syndrome is primary hypothyroidism associated with precocious puberty and galactorrhea.

270 - Destot’s sign is scrotal hematoma suggesting pelvic fracture.

271 - Rosenthal fibers is inclusions that develop in astrocytes in chronic reactive and neoplastic proliferations; abundant in Alexander’s disease.

272 - Klein-Waardenburg syndrome is Waardenburg syndrome type III.

273 - Boas’s sign is right subscapular pain due to cholelithiasis - <7%>

274 - Behçet’s disease is 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.

275 - Kaposi’s sarcoma is 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.

276 - Charcot’s joints is neurogenic joint degeneration - can be secondary to syphilis - peripheral neuropathy.

277 - There are two sets of Charcot's triads, both of which are sets of clinical signs relating to quite separate diseases. One pertains to multiple sclerosis while the other refers to ascending cholangitis. Charcot's triads are named for Jean-Martin Charcot (1825-1893), the French neurologist who first described these combinations of signs in relation to these diseases.

Charcot's triad 1: The combination of nystagmus, intention tremor, and scanning or staccato speech. This triad is sometimes associated with multiple sclerosis but is not, however, as previously considered by some authors, pathognomonic for multiple sclerosis.

Charcot's triad 2: The combination of jaundice; fever, usually with rigors; and right upper quadrant abdominal pain. Occurs as a result of ascending cholangitis. When the presentation also includes hypotension and mental status changes, it is known as Reynolds' pentad.

278 - Rebuck skin window is dermal abrasion technique for testing tissue penetration of neutrophils - scraping forearm - then putting coverslip over it - checking glass for neutrophils.

279 - Mustard procedure is for treating transposition of great vessels - now rarely used; an atrial inversion procedure which connects RA to LV - which pumps out to pulmonary arteries - and connects LA to RV - which becomes systemic pump to aorta; variant uses pericardial or prosthetic intraatrial baffles.

280 - Stransky’s sign is involuntary dorsiflexion of the toes after firmly abducting the 5th digit for 2 seconds - and then acutely letting it go in upper motor neuron defects.

281 - Civatte bodies is aka colloid bodies; in lichen planus; anucleate - necrotic basal cells becoming incorporated into the inflamed papillary epidermis.

282 - Miller Fisher test is in normal pressure hydrocephalus - objective gait assessment before and after 30 cc CSF removed reflecting prognosis for shunting.

283 - Osler-Weber-Rendu disease is hereditary hemorrhagic telangiectasia - larger lesions can be a source of chronic blood loss - systemic emboli - hypoxemia - hepatic dysfunction - and a high-output cardiac failure; important risk factor for brain abscess - especially in affected patients with clubbing - cyanosis - and/or polycythemia; use aminocaproic acid (an antifibrinolytic agent).

284 - Rapoport-Luebering shunt is in red blood cells - pathway converting 1 -3-diphosphoglyceric acid to 2 -3-DPG and then to 3-phosphoglyceric acid; enzyme is diphosphoglycerate synthetase; 2 -3-DPG reduces affinity of hemoglobin for oxygen; 2 -3-DPG rises with alkalosis and decreases with acidosis - result of effect of pH on enzyme.

285 - Alexander’s disease is leukodystrophy-like neurodegenerative disease presenting in infancy or childhood; characterized by Rosenthal fibers.

286 - Hirschprung’s disease is 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.

287 - Looser’s zones is radiolucent narrow lines that lie either at right angles or obliquely to the cortical outlines of bones and often transect them; bilateral and symmetric - found at the axillary margins of the scapula - lower ribs - neck of the proximal femurs - and posterior regions of the proximal ulnas; related either to stress fractures or to mechanical erosion by penetrating nutrient arteries; aka Milkman’s fractures.

288 - Williams’s syndrome is supravalvular aortic stenosis - mental retardation - elfin facies - association with hypercalcemia due to abnormal sensitivity to vitamin D - idiopathic hypercalcemia of pregnancy - loquacious personality - abnormally sensitive hearing; from deletion in elastin gene and probably several adjacent genes.

289 - Burnett’s syndrome is 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; also Cope’s syndrome.

290 - Prader-Willi syndrome is deletion of 15q11-q13 - paternally derived; uncontrollable hyperphagia after 12 months.

291 - Uhl’s anomaly is paper thin parietal myocardium - usually but not always limited to right ventricle - presents as heart failure in infancy or early childhood.

292 - Muir-Torre syndrome is patients with hereditary nonpolyposis colon cancer (HNPCC) who also develop benign or malignant sebaceous skin tumors (often basal cell or squamous cell).

293 - Gerhardt’s sign is in aortic regurgitation - pulsation of the spleen in the presence of splenomegaly; also Sailer’s sign.

294 - Volkmann contracture is sequelae of compartment syndrome where there is contraction of forearm flexors.

295 - Homer-Wright pseudorosettes is in neuroblastoma - tumor cells arranged about a central space filled with fibrillar extensions of the cells.

296 - Gilbert’s syndrome is 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.

297 - Austrian triad is clinical triad of pneumococcal pneumonia - meningitis - and endocarditis (classically aortic valve endocarditis associated with aortic regurgitation); described by Robert Austrian.

298 - Chvostek’s sign is hypocalcemia - in latent tetany - tapping the facial nerve against the bone just anterior to the ear producing ipsilateral contraction of facial muscles.

299 - Eagle-Barrett syndrome is prune-belly syndrome with triad of abdominal muscle deficiency - urinary tract abnormalities - and cryptorchidism.

300 - Desçemet’s membrane is 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.

301 - Degos disease is 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.

302 - Clara cells is 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.

303 - Kikuchi’s disease is 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.

304 - Retzius - veins of is numerous small veins in the retroperitoneum that connect the retroperitoneal viscera to the posterior abdominal wall; dilated in portal hypertension.

305 - Mounier-Kuhn syndrome is tracheomegaly - tracheobronchomegaly associated with recurrent pneumonia - copious purulent sputum production - hoarseness - load cough; presents in 30s-40s - more common in males; tracheal lumen increases with Valsalva; autosomal recessive.

306 - Marie-Strümpell disease is refers to ankylosing spondylitis in Europe.

307 - McMurray test is for diagnosing meniscal tear - passively flex hip and knee until heel touches buttock - steady the knee with one hand - externally rotate foot - then extend the knee to 90 degrees - return ot the beginning and then internally rotate the foot - then passively extend the knee to 90 degrees - feeling for clicks - PPV 83% for tears.

308 - Bateman’s senile purpura is 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.

309 - RSH syndrome is Smith-Lemli-Opitz syndrome.

310 - Burow’s solution is aluminum acetate in water - invented in mid-1800s by ophthalmologist Karl Burow (a.k.a. by its tradename Domeboro).

311 - Kupffer cells is phagocytic cells of the mononuclear phagocyte series found on the luminal surface of endothelial cells in hepatic sinusoids; they metabolize old RBCs - digest hemoglobin.

312 - Boston sign is in thyrotoxicosis - jerking of the lagging lid.

313 - Oppenheim’s sign is involuntary dorsiflexion of the toes when stroking the medial/anterior tibial surface superiorly to inferiorly - indicating upper motor neuron defect.

314 - Ross’s syndrome is tonic pupils (generally bilateral) - anhydrosis - and areflexia which may appear in a different pattern distribution; possible link with Holmes-Adie syndrome.

315 - Buerger’s sign is in peripheral vascular disease - red foot becomes pale with elevation.

316 - Hutchinson’s teeth is smaller and more widely spaced than normal and are notched on their biting surfaces; sign of congenital syphilis.the diagnostic criteria of the congenital syphilis are called the hutchinsons triad and they are

a- HUTCHINSONS TEETH

b- INTERSTITIAL KERATITIS

c- EIGTH NERVE DEAFNESS ( VESTIBULO COCHLEAR NERVE ).

317 - job's syndrome - RECURRENT STAPHYLOCOCCAL CUTANEOUS INFECTIONS ARE MORE COMMON AMONG INDIVIDUALS WHO HAVE EOSINOPHILIA AND ELEVATED SERUM LEVELS OF IMMUNOGLOBULIN E. THIS IS CALLED JOB'S SYNDROME.

318 - Holmes-Adie syndrome is 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.

319 - Felty’s syndrome is rheumatoid arthritis - splenomegaly - and neutropenia - and leg ulcers; associated with HLA-DR.

320 - Romaña’s sign is in the first week of Chagas disease - unilateral periorbital edema and swelling of the eyelid associated with reduviid bug of eye.

321 - Modigliani syndrome is thyroid in normal position but people with long curving necks enhance prominence and palpation of thyroid.

322 - Kew Gardens spotted fever is Rickettsialpox due to Rickettsia akari - from Kew Gardens - New York.

323 - Kennedy syndrome is 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.

324 - Berger’s disease is IgA nephropathy - ESRD develops in 15% of cases at 10 years and 20% at 20 years - treated with steroids in certain instances.

325 - Schwachman-Diamond syndrome is combination of neutropenia - metaphyseal dysplasia - and pancreatic insufficiency - associated with recurrent infections beginning in the first year of life in the sinuses - bones - lungs - skin and urinary tract; associated with increased risk of aplasia - myelodysplasia - and leukemia; life expectancy is 35 years.

326 - Thompson's test is verifies if gastroc-soleus complex intact; squeeze calf belly - foot should plantar flex.

327 - Steele-Richardson-Olszewski syndrome is aka progressive supranuclear palsy.

328 - Bancroft’s sign is 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.

329 - Bang’s disease is brucellosis.

330 - Williams’s syndrome is supravalvular aortic stenosis - mental retardation - elfin facies - association with hypercalcemia due to abnormal sensitivity to vitamin D - idiopathic hypercalcemia of pregnancy - loquacious personality - abnormally sensitive hearing; from deletion in elastin gene and probably several adjacent genes.

331 - Wiskott-Aldrich syndrome is X-linked characterized by triad of eczema - thrombocytopenia (from autoantibodies) - and repeated infections; small platelets (3-5 fL); failure to express sialic acid-rich glycoprotein - sialophorin or CD15 - ending in early death.

332 - Bowen’s disease is squamous cacrinoma in situ - seen generally on sun-exposed areas.

333 - Lennox-Gastaut syndrome is childhood seizure disorder associated with multiple seizure types (including drop attacks - atypical absences general tonic clonic seizures - and myoclonus) - slow generalized spike-and-slow-wave EEG - mental retardation - and resistant to standard anti-epileptic medications.

334 - de Musset’s sign is in aortic regurgitation - head bobbing - named after 19th century French poet who had luetic aortic insufficiency.

335 - Lassa fever is hemorrhagic fever caused by Lassa virus - first isolated in 1969 in Nigeria - an arenavirus - spread through contact with secretions (e.g. - urine) from infected rats; fatality 15-20% of hospitalized patients; can be treated with ribavirin.

336 - Froin’s syndrome is spinal block from tumor or inflammatory conditions - etc. causing increased protein in CSF - resulting in xanthochromia and increased coagulability in CSF.

337 - Pautrier’s microabcesses is q.v. Sézary-Lutzner cells.

338 - Markesbery distal myopathy is distal myopathy associated with rimmed vacuoles.

339 - Alexander’s law is 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.

340 - Kocher’s maneuver is dissection of the duodenum from the right-sided peritoneal attachment to allow mobilization and visualization of the back of the duodenum/pancreas.(2nd part).

341 - Capgras syndrome is 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.

342 - Mallory body is “alcoholic hyalin -” an eosinophilic intracytoplasmic inclusion in liver cells that is characteristic of alcoholic liver disease but seen in many other conditions as well (e.g. - primary biliary cirrhosis - Wilson’s disease - chronic cholestatic syndromes - focal nodular hyperplasia - and hepatocellular carcinoma); inclusions composed largely of intermediate filaments of prekeratin.

343 - Forrester classification is in myocardial infarction - I - PWP<18>2.2 L/min/m2 - 2% mort; II - PWP>18 and CI>2.2 - 10% mort; III - CI<2.2>18 and CI<2.2>

344 - Paget’s disease of bone is osteitis deformans - characterized by initial osteolytic stage followed by a mixed osteoclastic-osteoblastic stage - which ends with osteoblastic activity and evolves into a burnt-out osteosclerotic stage; increased alkaline phosphatase and increased urine hydroxyproline.

345 - Hoover’s sign is 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.

346 - Maddrey discriminant function is for predicting mortality in patients with alcoholic hepatitis - Maddrey DF = 4.6 x (PT-control PT) + serum bilirubin; DF > 32 associated with high short-term mortality - 1 month mort 35% in absence of encephalopathy and 45% if encephalopathy present.

347 - Vater - ampulla is location where common bile duct enters the duodenum; described by German anatomist Vater (1684-1751).

348 - Starr-Edwards valve is ball and cage valve - oldest prosthetic valve in continuous use - first used in 1965.

349 - Nothnagel’s syndrome is injury to superior cerebellar peduncle causing ipsilateral oculomotor palsy and contralateral cerebellar ataxia.

350 - Martin-Lewis agar is variant of chocolate agar medium for growing Neisseria.

351 - Maffucci’s syndrome is enchondromatosis associated with soft tissue hemangiomas; associated with ovarian carcinomas and brain gliomas; essentially Ollier’s disease with hemangiomas.

352 - Heyde’s syndrome is 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.

353 - Hansel’s stain is special stain used to detect eosinophiluria on the urine sediment.

354 - Conn’s syndrome is primary hyperaldosteronism - caused by an aldosterone-secreting tumor - resulting in hypertension - hypokalemia - hypernatremia - metabolic alkalosis - and low plasma renin.

355 - Kienböck disease is 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.

356 - Pancoast’s tumor is apical lung cancers in the superior pulmonary sulcus which invade neural structures around the trachea - including the cervical sympathetic plexus - leading to severe pain in distribution of the ulnar nerve and Horner’s syndrome on same side of the lesion.

357 - Ewing’s sarcoma is onion skinning; small round cell tumor of bone - 85% of cases there is a t(11;22)(q24:q12) translocation.

358 - Schafer’s maneuver is extensor plantar response by squeezing Achilles tendon suggesting upper motor neuron defect.

359 - Bernheim effect - reverse is in pulmonary embolism - right ventricular failure causing septum to bulge into and compromise left ventricular filling.

360 - Poiseuille’s law is flow proportional to fourth power of radius - inversely proportional to length.

361 - Lewis blood group system is named after Mrs. Lewis - pregnant woman in whom anti-Lea was discovered by Mourant in 1946; function of antigens in this system are unknown.

362 - Louis - angle of is sternal angle.

363 - Yuzpe regimen is ethinyl estradiol 100 mcg and levonorgestrel 0.5 mg taken twice -12 hours apart - reduces number of anticipated pregnancies by 75-80%.

364 - Kallman’s syndrome is anosmia; hypogonadotropic hypogonadism stemming from failure of LHRH-expressing neurons to migrate - etc.

365 - Tarlov cyst is perineural cyst found in the lower spinal cord.

366 - San Joaquin valley fever is coccidiomycosis (from infection with Coccidiomycosis imites).

367 - nerve endings is free includes Merkel cell associated: found in the epidermis as small aggregates called tactile corpuscles (Merkel cell has neural crest and squamous properties); encapsulated includes Meissner’s: asymmetrical - lamellated; Pacinian: symmetrical - lamellated; Ruffini: no lamellation.

368 - Bickerstaff’s encephalitis is brain stem encephalitis

369 - Legg-Calvé-Perthes disease is self-limiting hip disorder of children - 4-8 y.o. (M:F 8:1) involving vascular compromise of the capital femoral epiphysis; perhaps some aseptic necrosis action too.

370 - Raynaud’s phenomenon is exaggerated vascular response to cold temperatures or emotional stress - manifested by symmetrical - sharply demarcated color changes of the skin of the digits due to abnormal vasoconstriction of digital arteries and cutaneous arterioles.

371 - Trendelenburg position is position where angle of the head of bed or table is inclined at 45 degrees down; used in surgery to push the abdominal organs towards the chest.

372 - Scheie’s syndrome is mucopolysaccharidosis (I S) - from alpha-L-iduronidase deficiency (same as Hurler’s syndrome) - milder than Hurler’s syndrome - resulting in corneal clouding - stiff joints - aortic valve disease - normal intelligence and potentially normal life span.

373 - Councilman bodies is in apoptosis - hepatocytes that round up to form shrunken - pyknotic - and intensely eosinophilic bodies.A Councilman body, also known as a Councilman hyaline body, is an eosinophilic globule often found in the liver of individuals suffering from yellow fever. Each Councilman body results from the apoptotic death of a single liver cell.Until recently the presence of Councilman bodies in a liver biopsy was thought to be sufficient evidence for a diagnosis of yellow fever. However, they have since been found also to be present in other viral hemorrhagic fevers along with acute viral hepatitis, and thus may no longer be considered as diagnostic for yellow fever.

Councilman bodies are named after American pathologist William T. Councilman (1854-1933), who discovered them.

374 - Cockcroft-Gault formula is 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.

375 - Riedel’s thyroiditis is unknown etiology - marked by glandular atrophy - hypothyroidism - and replacement of the thyroid by fibrous tissue with adhesion to surrounding structures.

376 - Nägele’s rule is means of estimating date of delivery by counting back 3 months from the first day of the last menstrual period and adding seven days; full term 38 weeks after fertilization - 40 weeks after LNMP.

377 - St. Jude valve is prosthetic valve - bileaflet - with two semicircular discs that pivot between open and closed positions without supporting struts - first used in 1977.

378 - Dercum’s disease is adipositas dolorsa - condition tending to affect obese women in middle age - mostly menopausal - consisting of multiple exquisitely tender lipomas.

379 - Gower’s maneuver is Duchenne’s muscular dystrophy - patient using hands to help himself get up.

380 - Weil’s disease is severe form of leptospirosis with hepatic dysfunction - renal dysfunction - and hemorrhage.

381 - Parinaud’s oculoglandular syndrome is preauricular node enlargement associated with chronic granulomatous conjunctivitis; atypical form of cat scratch disease from inoculation near eye.

382 - Reinke’s edema is vocal cord polyposis in female smokers - 50s-70s.

383 - Rosenthal fibers is inclusions that develop in astrocytes in chronic reactive and neoplastic proliferations; abundant in Alexander’s disease.

384 - Fontan procedure is 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.

385 - Gleason score is 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.

386 - Gianotti-Crosti syndrome is 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.

387 - Naxos disease is initially described on Greek island of Naxos - synrome of arrhythmogenic right ventricular cardiomyopathy - non-epidermolytic palmoplantar keratoderma - and woolly hair - due to deletion in plakoglobin - protein in adherens and desmosomal junctions.

388 - Carney complex is 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.

389 - Klinger disease is Wegener’s disease; first described by Heinz Klinger in 1931 as a medical student and then described by Friederic Wegener in 1936.

390 - Paget’s disease of breast is breast cancer involving nipple and areola - appears as eczematous rash over breast with crusting - scaling - or erosion.

391 - Von Recklinghausen’s disease is neurofibromatosis.

392 - Calot’s triangle is 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.

393 - Von Willebrand factor is vWF bridges collagen and platelets and favors platelet aggregation - ensuring platelet and vessel wall interactions; glycoprotein Ib-IX major receptor for vWF; vWF also serves as carrier for factor VIII; made in endothelial cells and megakaryocytes.

394 - Zenker’s diverticulum is a pharyngeal diverticulum from premature contraction of the cricopharyngeus muscle on swallowing - leads to progressive UES narrowing - leading to a posteriorly directed hypopharynx; causes progressive food stasis and dysphagia.

395 - D’Espine’s sign is breath sounds louder over C7 than adjacent lung - suggests lesion in posterior mediastinum - e.g. lymphoma - tuberculosis - etc.

396 - Warthin-Finkeldey cells is in measles pneumonia - cells with multiple nuclei and eosinophilic intranuclear inclusions.

397 - Cabrera’s sign is 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.

398 - Curling’s ulcers is stress erosions and ulcers occurring in the proximal duodenum and associated with severe burns or trauma - from ischemia of the gastric mucosa.

399 - Roger’s disease is small congenital VSD defect <0.5>

400 - Best disease is 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.

401 - PANDAS is Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections. Consists of tics - movement disorders - emotional-lability - and obsessive-compulsive behavior associated with streptococcal infection. Caused by IgG antibodies which recognize group A streptococcal cell wall proteins as well as neuronal cytoplasmic antigens of the basal ganglia. Related to Sydenham's chorea.

402 - Haldane effect is deoxygenated hemoglobin having a greater affinity for CO2 than oxygenated hemoglobin.

403 - Brugada syndrome is 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.

404 - ASPEN syndrome is 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.

405 - Wright’s stain is stain used for blood and bone marrow films.

406 - Mibelli - porokeratosis of is classic porokeratosis - localized - chronically progressive - hyperkeratotic - irregular plaques with central atrophy and prominent peripheral keratotic ridge; characterized pathologically by presence of cornoid lamella.

407 - Lambert-Eaton myasthenic syndrome is disease of neuromuscular junction associated with paraneoplastic process with proximal muscle weakness that improves with repetitive stimulation; associated with P/Q type presynaptic voltage-gated calcium channel antibodies; 70% have malignancy - usually small cell cancer.

408 - Heberden’s nodes is 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.

409 - Pick bodies is in neurons - cytoplasmic - round to oval - filamentous inclusions that strongly stain with silver - weakly eosinophilic; composed of neurofilaments - vesiculated endoplasmic reticulum - and paired helical filaments that are immunocytochemically similar to those found in Alzheimer’s; don’t survive death of host neuron like they do in Alzheimer’s.

410 - Monteggia fracture is fracture of the proximal third of the ulna with a dislocation of radial head.

411 - Krabbe’s disease is 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.

412 - Tourette’s syndrome is chronic multiple motor and verbal tics - symptoms typically begin before 21 y.o. - first signs motor tics in 80% and vocal tics in 20% but eventually both motor and vocal; higher than expected number of left-handedness and ambidexterity; 40-50% involve self-mutilation such as nail-biting - hair-pulling - etc.

413 - Fletcher factor is prekallikrein; in intrinsic phase of coagulation - converts XII to XIIa; deficiency results in elevated PTT but with no clinical bleeding.

414 - Ellsworth-Howard test is for diagnosis of pseudohypoparathyroidism by assessing response to exogenous PTH.

415 - moyamoya disease is occlusive disease involving large intracranial arteries - especially the distal ICA and stem of the MCA and ACA; “puff of smoke” (in Japanese - moya moya) appearance on angiography from lenticulostriate arteries developing rich collateral circulation around the middle cerebral occlusion that; anticoagulation risky because of occurrence of SAH from rupture of the transdural anastomotic channels.

416 - Senear-Usher syndrome is pemphigus erythematosus; a localized variety of pemphigus foliaceus confined to seborrheic sites.

417 - Tangier disease is rare recessive disease characterized by enlarged orange tonsils - peripheral neuropathy affecting small fibers involved in pain and temperature - and a near-complete absence of HDL-cholesterol; ABC1 - the ATP binding-cassette transporter 1 gene - mutated in Tagier disease; gene mediates efflux of cholesterol from cells; named after small island in Chesapeake Bay where disease first identified in five-year-old boy.

418 - factor V Leiden is 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.

419 - Miller Fisher syndrome is variant of Guillain-Barré where cranial nerves are affected - leading to triad of ataxia - areflexia and ophthalmoplegia; cross-reacting antibodies to GQ1b ganglioside have been found; triggered by certain strains of C. jejuni.

420 - Hutchinson’s sign is pigment in the paronychial area suggesting melanoma.

421 - Spanish flu is 1918 influenza with 20-100 million deaths worldwide with 2.5% mortality (influenza normally 0.1% mortality).

422 - Wernicke’s area is important cortical center for recognizing speech - found in the superior temporal gyrus; communicates with Broca’s area with arcuate fasciulus.

423 - Cruveihiler-Baumgarten bruit is bruit heard over caput medusa in portal hypertension.

424 - Dacie’s syndrome is idiopathic hyperplastic enlargement of the spleen with anemia and neutropenia; progression to lymphoma in some cases.

425 - Forscheimer spots is in rubella - punctate soft palate macules.

426 - Sudeck’s syndrome is reflex sympathetic dystrophy syndrome occurring in older people characterized by cystic changes and subchondral erosion in bone - diffuse osteoporosis - and muscle atrophy - but not necessarily associated with trauma.

427 - Denver shunt is 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.

428 - Schilder’s disease is adrenoleukodystrophy - X-linked disorder - presents in childhood with weakness - spasticity - leading to dementia - blindness - and quadriparesis - associated with adrenal insufficiency; from defect in beta-oxidation of fatty acids in peroxisomes leading to accumulation of very long-chain saturated fatty acids.

429 - Kulchitsky’s cells is 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.

430 - Boxer’s fracture is fracture of the metacarpal neck - classically of small finger.

431 - Erb’s palsy is upper plexus palsy affecting C5 and C6 and +/- C7 nerve roots. lower brachial plexus palsy is called klumpke's palsy .

432 - Denys-Drash syndrome is gonadal dysgenesis (male pseudohermaphroditism) and nephropathy leading to renal failure with increased risk of Wilms’s tumor; dominant negative missense mutation of WT-1.

433 - Hers disease is 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.

434 - Trendelenburg’s gait is waddling gait in people with weakness or paresis of gluteal muscles; seen in progressive muscular dystrophy.

435 - Cori’s disease is 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.

436 - Lafora’s disease is 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.

437 - Bruton’s tyrosine kinase is 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

438 - Call-Fleming syndrome is sudden-onset severe headache - focal neurological deficits - and seizures; associated with serotonin modulating drugs like SSRIs.

439 - dimple sign is in dermatofibroma - lateral compression with thumb and index finger produces a depression - or “dimple.”

440 - Nissen fundoplication is for treating gastroesophageal reflux disease - surrounding lower end of esophagus with cuff of gastric fundal muscle to increase lower esophageal sphincter competence.

441 - "Dawson's Fingers" is a condition affecting the brain of Multiple Sclerosis patients. The condition is the result of inflamation around long axis of medular veins. This results in a finger-like appearance of the lesions extending mainly off the ventricles within the brain. The term is named for histologist James Walker Dawson, who first defined the condition .

442 - Ebstein’s anomaly is 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.

443 - Döhle bodies are light blue-gray, basophilic, leukocyte inclusions located in the peripheral cytoplasm of neutrophils. They measure 1-3 µm in diameter. Not much is known about their formation. They are named after German pathologist, Karl Gottfried Paul Döhle (1855-1928)

They are seen in:

* Burns

* Infections

* Physical trauma

* Neoplastic diseases

* Wissler’s disease

* May-Hegglin’s anomaly

* Chédiak-Steinbrinck-Higashi’s syndrome.

444 - Sipple’s syndrome is MEN type IIa; pheochromocytoma - medullary carcinoma of the thyroid - and hyperparathyroidism due to hyperplasia or tumor.

445 - Stroop test is stress test - also used for checking frontal function - where patients are tested for the ability to read off the text color of text spelling potentially different colors.

446 - Montgomery - glands of is sebaceous glands of areola.

447 - Goodell's sign is an indication of pregnancy. It is a significant softening of the vaginal portion of the cervix.

448 - Brunner’s glands is in duodenum - submucosal mucous glands that secrete bicarbonate - glycoproteins - and pepsinogen II - virtually indistinguishable from pyloric mucous glands.

449 - Grafenberg spot is erogenous area in anterior vaginal wall ( also called G SPOT ) .

450 - Goodsall's rule is applied to the examination of anal fistula. It states that if the fistula lies in the anterior half of the anal area then it opens directly into the anal canal, while if a fistula lies in the posterior half of the canal then it tracks around the anus laterally and opens into the midline posteriorly.

451 - Rothmann-Makai syndrome is a variant of idiopathic lobular panniculitis seen in infants - tends to be localized and spontaneously resolves; associated with autoimmune diseases such as juvenile rheumatoid arthritis - diabetes - and Hashimoto’s thyroiditis.

452 - Lugol’s solution is 5% iodine and 10% KI - a dose of 6.3 mg of I per drop - for giving iodine.

453 - Stoppa repair is hernia repair using tension-free intraparietal prosthetic mesh.

454 - Auenbrugger’s sign is epigastric bulge due to a massive pericardial effusion.

455 - Zahn - lines of is thrombi formed within a cardiac chamber or the aorta - may have apparent laminations - produced by alternating layers of paler platelets admixed with some fibrin - separated by darker layers containing more red cells.

456 - Cagot ear is absence of ear lobe; assocated with region in Pyrennes.

457 - Ham’s test is for diagnosing paroxysmal nocturnal hemoglobinuria - uses increased sensitivity of PNH-affected RBCs to lysis by complement; introduced in late 1930s.

458 - Forscheimer spots is in rubella - punctate soft palate macules.

459 - Apley grind test is 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.

460 - clay shoveler’s fracture is fracture of spinous process of C7.

461 - Christmas disease is hemophilia B - deficiency in factor IX.

462 - de Quervain’s disease is a stenosing tenosynovitis of the thumb extensors and abductors; pain elicited with Finkelstein’s test.

463 - Munchausen’s syndrome is factitious disorder - eponym given by Asher in 1951 after Baron Karl Munchausen (1720-1797) - retired German cavalry officer with a reputation for preposterous stories about his activities as a soldier - hunter.

464 - MacCallum’s plaques is irregular thickenings - usually in the left atrium - from subendocardial lesions - usually exacerbated by regurgitant jets.

465 -POEMS syndrome is polyneuropathy - organomegaly - endocrinopahty - M protein - and skin changes which may be seen in plasma cell dyscrasias; also known as Crow-Fukase syndrome in Japan.

466 - Santorini’s fissures is fissures in anterior aspect of cartilaginous portion of ear canal through which neurovascular tissues pass; may allow spread of ear disease to parotid joint - TMJ - soft tissues of upper neck.

467 - Glauber’s salt is sodium sulfate - laxative.

468 - Hampton's hump, also Hampton hump, is a radiologic sign seen on chest X-rays indicating pulmonary infarction and classically due to pulmonary embolism induced atelectasis.

It consists of a pleura based shallow wedge-shaped consolidation in the lung periphery with the base against the pleural surface. It is named after Aubrey Otis Hampton.

469 - In chest radiography, the Westermark Sign, is a sign that represents a focus of oligemia (vasoconstriction) seen distal to a pulmonary embolus (PE).[1] While the chest x-ray is abnormal in the majority of PE cases, the Westermark sign is seen in only 2% of patients.[2]

The sign results from a combination of:

1. the dilation of the pulmonary arteries proximal to the embolus and

2. the collapse of the distal vasculature creating the appearance of a sharp cut off on chest radiography.

470 - Wickham’s striae is in lichen planus - papules are highlighted by a shiny surface with a lacy white pattern.

471 - Döhle bodies is irregularly shaped greenish inclusions in neutrophil cytoplasm - consisting of ribosomes and/or rough ER and are seen in severe bacterial infections.

472 - Silk Road disease is Behçet’s disease.

473 - Herring bodies is neurosecretory granules consisting of either vasopressin or oxytocin in neurohypophysis.

474 - Unna boot is compression dressing impregnated with medication for treating venous stasis ulcers; named after Paul Gerson Unna - German dermatologist 1850-1929.

475 - Rigler's sign, also known as the double wall sign, is seen on an x-ray of the abdomen when air is present on both sides of the intestine; a Rigler's sign is present when air is present on the inside (lumenal side) and the outside (peritoneal side). Air in the peritoneum is considered abnormal, unless the patient had a recent abdominal surgery.

A false double wall sign can result from two loops of bowel being in contact with one another.

476 - Muckle-Wells syndrome is syndrome of urticaria - progressive perceptive deafness - and amyloidosis; from mutation in cryopyrin; autosomal dominant; of note familial cold autoinflammatory syndrome - commonly known as familial cold urticaria - characterized by intermittent episodes of rash - arthralgia - fever and conjunctivitis after generalized exposure to cold is also associated with mutations in cryopyrin.

477 - Birbeck’s granules is aka Langerhans’s granules; a small tennis racket-shaped membrane-bound granule with characteristic cross-striated internal ultrastructure seen in Langerhans cell histiocytosis.

478 - Sellick’s maneuver is cricoid pressure during intubation.

479 - The football sign refers to a large oval radiolucency in the shape of an American football, which is seen on supine abdominal X-ray films. The football sign is most frequently seen in infants with spontaneous or iatrogenic gastric perforation causing pneumoperitoneum.

480 - Wenckebach block is second-degree AV blcok - Mobitz type I.

481 - Willis - circle of is cerebral arterial circle - an anastomosis between the two vertebral and two internal carotid arteries.

482 - Leriche’s syndrome is aortoiliac occlusive disease producing distal ischemic symptoms and signs - e.g. pulseless femoral artery: 1. claudication of buttocks - 2. impotence - 3. atrophy of buttocks (seen with iliac occlusive disease).

483 - Riddoch’s sign is in cerebellar disease - with outstretched hands - the hand on the affected side begins to hyperpronate - so that the palm faces outward - and rises above the level of the other hand.

484 - Jumping Frenchmen of Maine syndrome is 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.

485 - Quincke's sign - pulsation of the capillary bed in the nail - seen in aortic regurgitation .

486 - Reynolds' pentad is a collection of signs and symptoms suggesting the diagnosis of septic (ascending) cholangitis, a serious infection of the biliary system. It is a combination of Charcot's triad (jaundice, fever, abdominal pain) with hypotension (shock - low blood pressure) and an altered mental state. It was named after the surgeon, B.M. Reynolds, who described it.

487 - Pfeiffer syndrome is craniosynostoses as well as limb defects - mutations in FGFR1 - broad thumbs - broad great toes.

488 - Monro-Kellie doctrine is cranial cavity is a closed rigid box - change in the amount of intracranial blood can occur only through changes in CSF quanitity.

489 - Edinger-Westphal nucleus is part of the cranial nerve III complex involved in direct and consensual light reflex involved in efferent limb of reflex arc.

490 - Rovsing's sign is a sign of appendicitis. If palpation of the lower left quadrant of a person's abdomen results in more pain in the right lower quadrant, the patient is said to have a positive Rovsing's sign and may have appendicitis.

This anomaly occurs because the pain nerves deep in the intestines do not localize well to an exact spot on the abdominal wall, unlike pain nerves in muscles. Pain from a stomach ulcer or gallstone can be interpreted by the brain as pain from the stomach, liver, gall bladder, duodenum, or first part of the small intestine. It will "refer" pain often to the mid upper abdomen.

Because the appendix is a piece of intestine, it follows a similar referral pattern.

491 - Sappey - veins of is retroperitoneal veins located around liver and diaphragm connecting epigastric and internal mammary veins with azygos and which become dilated in portal hypertension.

492 - Tinel's sign is a way to detect irritated nerves. It is performed by lightly tapping (percussing) over the nerve to elicit a sensation of tingling or "pins and needles" in the distribution of the nerve.

For example, in carpal tunnel syndrome where median nerve is compressed at the wrist, Tinel's sign is often "positive" causing tingling in the thumb, index, and middle finger. Tinel's sign is sometimes referred to as "distal tingling on percussion" or DTP.

493 - Wolff-Parkinson-White syndrome is paroxysmal supraventricular tachycardia caused by conduction through abnormal accessory bypass tract (bundle of Kent) between atria and ventricles characterized by triad of 1. wide QRS complex - 2. relatively short PR interval - 3. slurring of initial part of QRS delta wave; in familial WPW - associated with mutation in gamma2 regulatory subunit of AMP-activated protein kinase (PRKAG2).

494 - Lowenberg’s sign is in deep vein thrombosis - two calves are wrapped with cuffs to if there is assymetry in tolerance to pressure of 180 mm Hg.

495 - Hoffmann's sign, named after the German neurologist, Johann Hoffmann (born 1857, Rheinhesse; died 1919, Heidelberg), is a finding elicited by a reflex test which verifies the presence or absence of problems in the corticospinal tract. It is also known as the finger flexor reflex.

The test involves tapping the nail or flicking the terminal phalanx of the third or fourth finger. A positive response is seen with flexion of the terminal phalanx of the thumb.

496 - Sipple’s syndrome is MEN type IIa; pheochromacytoma - medullary carcinoma of the thyroid - and hyperparathyroidism due to hyperplasia or tumor.

497 - Heister - spiral valves of is 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.

498 - McConnell’s sign is in acute pulmonary embolism - distinct regional pattern of RV dysfunction - with akinesia of the mid-free wall but normal motion at the apex - 77% sensitive - 94% specific for PE.

499 - Occam’s razor is described in 14th century - “plurality must not be posited without necessity -” in other words - parsimony in diagnosis.

500 - Wallenberg syndrome is infarction in posterior inferior cerebellar artery (PICA) - lateral medulla 1. lesion in nucleus ambiguus - difficulty in swallowing and hoarseness - loss of gag reflex 2. vestibular nucleus - dizziness and nystagmus 3. trigeminal - loss of pain and temperature on ipsilateral 4. inferior cerebellar peduncle - ipsilateral limb ataxia 5. anterolateral system - reduced pain and temperature on contralateral limb 6. ipsilateral Horner’s syndrome hiccup - for reasons not known solitary nucleus may also be destroyed - leading to loss of taste on ipsilateral half of tongue.