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1047 Cards in this Set

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adult onset Still’s disease
polyarthritis associated with sudden onset of high spiking fever, sore throat, and an evanescent erythematous salmon-colored rash
Alagille syndrome
inherited cholestatic syndrome, associated with biliary hypoplasia (ductopenia), vertebral anomalies, prominent forehead, deep-set eyes, peripheral pulmonic stenosis
Albers-Schönberg disease
osteopetrosis or marble bone disease
Albright’s hereditary osteodystrophy
polyostotic fibrous dysplasia, short stature, round face, skeletal anomalies (brachydactyly), and heterotopic calcification, precocious puberty, café-au-lait spots on skin, low calcium, high phosphate, resistance to elevated PTH levels from mutation in Gs coupling PTH receptor to adenylyl cyclase; also see Archibald’s sign
Alder-Reilly anomaly
large, dark, pink-purple granules in cytoplasm of neutrophils; AR trait resulting in abnormal granule development in neutrophils resembling severe toxic granulation
Alport’s syndrome
hereditary nephritis accompanied by nerve deafness and various eye disorders, including lens dislocation, posterior cataracts, and corneal dystrophy
Alström’s syndrome
obesity, autosomal recessive, childhood blindness due to retinal degeneration, nerve deafness, vasopressin-resistant diabetes insipidus, and in males hypogonadism with high plasma gonadotropin levels (end organ resistance to hormones), also baldness, hyperuricemia, hypertriglyceridemia, aminoaciduria
angle of Louis
sternal angle
Anitschkow myocytes
in rheumatic fever, large mesenchymal cells in myocardial lesion
Archibald’s sign
in pseudohypoparathyroidism of Albert’s hereditary osteodystrophy, characteristic shortening of the fourth and fifth digits as dimpling over the knuckles of a clenched fist
Argyll Robertson pupils
small irregular pupils; accommodate, but don’t react; absence of miotic reaction to light, both direct and consensual, with preservation of a miotic reaction to near stimulus); usually but not always caused by *CNS syphillis*
Arnold-Chiari malformation
downward displacement of the cerebellar tonsils and medulla through the foramen magnum, results in pressure atrophy of displaced brain tissue, hydrocephalus from obstruction of the CSF outflow tract, is almost always characterized by the presence of a thoracolumbar meningomyelocele
artery of Adamkiewicz
artery responsible for anterior spinal syndrome; could be bagged in AAA repair
Arthus reaction
localized area of tissue necrosis resulting from acute immune complex vasculitis; type III hypersensitivity
Aschoff body
pathognomonic finding in rheumatic carditis, an area of local fibrinoid necrosis surrounded by inflammatory cells including lymphocytes, plasma cells, and macrophages that later resolve to fibrous scar tissue
Asherman’s syndrome
intrauterine synechiae, typically occurs after curettage of the uterus, presenting as amenorrhea
Asperger’s disorder
severe and sustained impairment in social interactions and the development of restrictred, repetitive patterns of behavior, intersts, and activities, but intellectually normal and no language delays, but with abnormalities of spoken language
atrial conduction system
anterior internodal tract, Bachmann’s bundle middle i.t., Wenckebach’s bundle posterior i.t., Thorel’s pathway
Auer rods
present in acute myelogenous leukemia or refractory anemia with excess blasts; granules form elongated needles, granules are all azurophilic, contain peroxidase; fused lysosomes
Auerbach’s plexus
myenteric plexus, between the longitudinal and circular layers of muscle; provides motor innervation to the two muscle layers and secretomotor innervation to the mucosa
Auspitz’s sign
for psoriasis; sign is positive when slight scratching or curetting of a scaly lesion reveals punctate bleeding points within the lesion; suggests psoriasis, but is not specific
Austin Flint murmur
diastoic rumble in aortic insufficiency heard in cardiac apex, thought to be due to aortic jet impinging on the mitral valve, causing it to vibrate and also from simultaneous diastolic filling of the left ventricle from the left atrium and aorta tends to close the mitral valve in diastole, producing physiologic stenosis
Australia antigen
HBsAg, found in the serum of an Australian aborigine
Ballance’s sign
tender mass in the LUQ due to a spleen hematoma
bamboo spine
seen in ankylosing spondylitis
Banti’s syndrome
splenomegaly, hypersplenism, and portal hypertension, noncirrhotic, arises after subclinical occlusion of the portal vein, usually years after occlusive event
Bantu siderosis
unusual form of iron overloading resembling hereditary hemochromatosis in South African blacks ingesting large quantities of alcoholic beverages fermented in iron utensils
Barlow’s maneuver
for congenital hip dislocation, patient placed in supine position and attempt made to push femurs posteriorly with knees at 90 degrees/hip flexed and hip will dislocate
Barrett’s esophagus
esophageal strictures and epithelial metaplasia from squamous epithelium to a specialized columnar epithelium with intestinal metaplasia in 10% of severe GERD
Bartter’s syndrome
primary elevation in renin due to idiopathic hyperfunction of the juxtaglomerular apparatus but with normal blood pressure
basal nucleus of Meynert
degenerates in Alzheimer’s; uses ACh
Batson's plexus
portal vertebral venous communications, may be responsible for isolated bone mets in sacrum or vertebral bodies from colorectal cancer
Battle’s sign
ecchymoses over the mastoid process in basilar skull fractures
BCG
Bacille bilié de Calmette-Guérin; Leon A. Calmette, French bacteriologist, 1863-1933; Camille Guérin, French bacteriologist, 1872-1961; attenuated strain of Mycobacterium bovis bacille Calmette-Guérin
Beau’s lines
horizontal depressions across nail plate, caused by a transient arrest in nail growth, can occur during acute stress (e.g., high fever, circulatory shock, myocardial infarction, pulmonary embolism); will manifest as Beau’s lines as nail grows out
Bechterew’s disease
ankylosing spondylitis
Beck’s triad
in pericardial tamponade, distended neck veins, distant heart sounds, hypotension, i.e. rising venous pressure, falling arterial pressure, and decreased heart sounds
Beckwith-Wiedemann syndrome
exomphalos, macroglossia, gigantism
Behcet’s diease
aphthous ulcers, genital ulcerations, ocular inflammation (posterior uveitis), erythema nodosum, cutaneous pustular vasculitis, also synoviits, neurologic issues, and thrombophlebitis
Bell’s palsy
seventh nerve palsy; seen as a complication in diabetes, tumors, sarcoidosis, AIDS, and Lyme disease
Bell’s phenomenon
when an attempt is made to close the eyelid, the eyeball on the affected side may turn upward
Bence Jones proteins
free L chains of immunoglobulin seen ins plasma cell dyscrasias (e.g. multiple myelomas), filtered by glomerulus and then reabsorbed tubular cells; proteins are toxic to tubule cells
Benedikt syndrome
paramedian midbrain syndrome
Bennett’s fracture
fracture of the base of the first metacarpal with involvement of carpometacarpal joint
Bergman’s triad
seen with fat emboli syndrome: 1) mental status changes 2) petechiae (often in the axilla/thorax) 3) dyspnea
Bernard-Soulier disease
absence of Gp Ib/IX, the von Willebrand receptor
Bernheim effect
in aortic stenosis, right ventricular failure preceding left ventricular failure from hypertrophied ventricular septum bulging into and encroaching on right ventricular filling
Bernstein test
to test for GERD, acid perfusion test of esophagus with 0.1 N HCl and see if reproduces chest pain; limited sensitivity and specificity though
Betz cells
large pyramidal cells in layer 5 of primary motor cortex largest neurons in mammalian CNS; 30-40,000 Betz cells in precentral gyrus in one side of the brain
Bier block
regional anesthesia of an extremity by placing a tourniquet and then infusing local anesthetic into a vein
Billroth I
antrectomy with gastroduodenostomy
Billroth II
antrectomy with gastrojejunostomy
Billroth’s cords
the splenic cords found in the red pulp between the sinusoids
Binswanger’s disease
subcortical leukoencephaly, is associated with hypertension; it is characterized by the presence of multiple lacunar infarcts and progressive demyelination limited to the subcortical area, with characteristic sparing of cortex
Birbeck’s granules
aka Langerhans’s granules; a small tennis racket-shaped membrane-bound granule with characteristic cross-striated internal ultrastructure; first reported in Langerhans’s cells of the epidermis
Bitot’s spots
in vitamin A deficiency, small, circumscribed, lusterless, grayish white, foamy, greasy, triangular deposits on the bulbar conjunctiva adjacent to the cornea in the area of the palpebral fissure of both eyes
Bloom’s syndrome
severe immunodeficiency, growth retardation, and predisposition to several types of cancers associated with hypersensitivity to a variety of DNA-damaging agents
Blount’s disease
idiopathic varus bowing of tibia
Blumberg sign
rebound tenderness
Blumer shelf
carcinomatous metastasis from a primary site high up in the peritoneal cavity, may accumulate in the stomach, felt through the anterior rectal wall as a hard shelf in the rectovesical or rectouterine pouch (pouch of Douglas)
Boas’s sign
right subscapular pain due to cholelithiasis
Bochdalek's hernia
hernia through the posterior diaphram, usually on the left, presents in infancy
Boerhaave’s syndrome
pressure rupture of the esophagus; can give rise to Hamman’s sign
Bohr effect
fall in pH leading to decrease in oxygen affinity of hemoglobin
Bohr equation
Vd/Vt, for determining ratio of physiologic dead space
Bonnet’s sign
banking of veins distal to AV crossings (grade 3) in hypertensive retinopathy; c.f. with Salus’s sign and Gunn’s sign
Bordet-Gengou medium
for identifying Bordetella pertussis, medium contains high percentage of blood (20-30%) to inactivate inhibitors in blood; also has potato and glycerol
Bornholm disease
coxsackie group B virus producing pleurodynia, fever
Boston sign
in thyrotoxicosis, jerking of the lagging lid
Bouchard’s nodes
bony spurs at PIP in OA
Bowditch staircase
increased heart rate increases the strength of contraction in a stepwise fashion as the intracellular calcium increases over several beats
Bowen’s disease
carcinoma in situ of penis or scrotum, usually presents as a single erythematous plaque, most often on the shaft of the penis or on the scrotum, peak incidence after 50s; or more generally, squamous carcinoma in situ
Boxer’s fracture
fracture of the metacarpal neck, classically of msall finger
Brill-Zinser disease
recurrent form of epidemic typhus (Rickettsiae prowazekii); persistently infected as a source
Briquet’s syndrome
somatization disorder
Broca’s area
left frontal speech area, important for articulating speech; in Broca’s aphasia, because Broca’s area near motor cortex and underlying internal capsule, a right hemiparesis and homonymous hemianopsia is almost always present in this type of aphasia
Brodie’s abscess
small, intraosseus abscess that frequently involves the cortex and is walled off by reactive bone
Brown-Séquard syndrome
Loss of tactile sense, vibration sense, and limb position sense on the ipsilateral side and loss of pain and temperature sense on the contralateral side
Brudzinski sign
flex the neck, watch the hips and knees in reaction to maneuver positive sign, flexion of hips and knees, suggests meningeal inflammation
Brunner’s glands
in duodenum, submucosal mucous glands that secrete bicarbonate, glycoproteins, and pepsinogen II, virtually indistinguishable from pyloric mucous glands
Brushfield’s spots
in Down’s syndrome, small white spots on the periphery of the iris
Bruton’s tyrosine kinase
mutation causes X-linked agammaglobulinemia (XLA also associated with defect in intact membrane-bound  chain (it’s essential for B-cell development) Btk found only in B cells
Budd-Chiari syndrome
occlusion of the hepatic vein, associated with polycythemia vera, pregnancy, postpartum state, oral contraceptives, paroxysmal nocturnal hemoglobinuria, and intra-abdominal cancers, particularly hepatocellular carcinoma
Burnett’s syndrome
far-advanced milk-alkali syndrome, due to long-standing calcium and alkali ingestion; severe hypercalcemia, irreversible renal failure, and phosphate retention, may be accompanied by ectopic calcification
Buschke-Löwenstein tumor
giant anal condyloma
Cabot ring
in asplenia or malfunctional spleen, nuclear remnants on red blood cells as a thin, darkly-stained ring that follows the margin of the red cell
Caisson disease
decompression sickness
Call-Exner bodies
in granulosa cell tumors, small follicles filled with eosinophilic secretion; an important diagnostic feature
Calot’s triangle
the area bordered by the 1. cystic duct, 2. common hepatic duct, 3. lower edge of the liver; cystic artery, sometimes hepatic artery found here
Campbell de Morgan spots
cherry angioma
Canale-Smith syndrome
childhood disorder, first described in 1967, characterized by lymphadenopathy and autoimmunity; associated with mutations in Fas; implicates gene in accumulation of lymphocytes and the autoimmunity characteristic of the syndrome
canals of Hering
at the fringes of the portal tract, from the joining of bile canaliculi
canals of Lambert
direct accessory bronchioalveolar connections
Cantlie’s line
separates the right and left lobes of the liver—a line drawn from the IVC to just left of the gallbladder fossa
Caplan’s syndrome
coexistence of rheumatoid arthritis with a pneumoconiosis, leading to the development of distinctive pulmonary lesions that develop fairly rapidly; these nodular lesions have central necrosis surrounded by fibroblasts, macrophages, and collagen; can occur in asbestosis and silicosis
carcinoid triad
1) flushing 2) diarrhea 3) right-sided heart failure (also bronchospasm)
Carey Coombs murmur
a blubbering apical mid diastolic murmur occurring in the acute stages of rheumatic mitral valvulitis and disappearing as the valvulitis subsides; Carey Coombs, English physician, 1879-1932
Carnett’s test
head raise, tenderness persists in abdominal wall condition (rectus hematoma) whereas pain due to intraperitoneal disease lessens
Carney syndrome
AD complex cardiac myxomas, aggressive biologic behavior, spotty pigmentation, issue with endocrine tumors
Caroli’s disease
larger ducts of the intrahepatic biliary tree are segmentally dilated and may contain inspissated bile; pure forms are rare; this disease is usually associated with portal tract fibrosis of the congenital hepatic fibrosis type
Carrión’s disease
see Oroya fever
Carvallo’s sign
in tricuspid regurgitation, murmur increases with inspiration
Castleman’s disease
benign lymphoproliferative disorder, characterized by hyperplastic lymphoid follicles with capillary proliferation
cat-scratch disease
tender regional lymphadenopathy persisting for 3 weeks or longer, frequently preceded by primary skin lesion after contact with kits, appears to be caused by Bartonella (formerly Rochalimaea) henslae, a small, pleomorphic gram-negative bacillus
Chadwick’s sign
blue-red passive hyperemia of the cervix, characteristic of pregnancy
Chagas’s disease
zoonosis caused by protozoan parasite Trypanosoma cruzi; causes destruction of the myenteric plexus of the esophagus, duodenum, colon, and ureter, with resultant dilatation of these structures
Charcot’s joints
neurogenic joint degeneration, can be secondary to syphillis, peripheral neuropathy
Charcot’s triad
in multiple sclerosis, nystagmus, intention tremor, and staccato speech (or scanning speech)
Charcot’s triad
in 70% of patients with bacterial cholangitis, right-upper-quadrant pain, jaundice, and fever; c.f. Reynold’s pentad
Charcot-Leyden’s crystals
crystals in the shape of elongated double pyramids, formed from eosinophils, found in the sputum in bronchial asthma and in other exudates or transudates containing eosinophils
Chédiak-Higashi syndrome
autosomal recessive disorder, neutropenia, defective degranulation, and delayed microbial killing. Neutrophils and other leukocytes have giant granules (can be seen on blood smears). Effect of microtubule polymerization causes delayed or decreased fusion of lysosomes with phagosomes in leukocytes and thus impairs phagocytosis of bacteria (Robbins 24)
Chilaiditi syndrome
when redundant loops of transverse colon slip between the liver and diaphragm and cause vovulus
Christmas disease
hemophilia B, deficiency in factor IX
Churg-Strauss syndrome
allergic angiitis and granulomatosis involvement in the lung
Chvostek’s sign
hypocalcemia, in latent tetany, tapping the facial nerve against the bone just anterior to the ear producing ipsilateral contraction of facial muscles
Civatte bodies
aka colloid bodies; in lichen planus; anucleate, necrotic basal cells becoming incorporated into the inflamed papillary epidermis
Clara cells
cells found in the epithelium of terminal and respiratory bronchioles, devoid of cilia, present secretory granules in their apex and are known to secrete glycosoaminoglycans that probably protect the bronchiolar lining
clay shoveler’s fracture
fracture of spinous process of C7
Clutton’s joints
in congenital syphillis, symmetrical arthrosis, especially of the knee joints
Cockayne’s syndrome
dwarfism, precociously senile appearance, pigmentary degeneration of the retina, optic atrophy, deafness, sensitivity to sunlight, and mental retardation; autosomal recessive inheritance defect in DNA repair?
Codman’s triangle
in osteosarcoma, the triangular shadow between the cortex and raised ends of periosteum is known radiographically as Codman’s triangle, and is characteristic but not diagnostic of this tumor
Colles’s fracture
a fracture of the distal radius that occurs when persons fall with outstretched hands to try to catch themselves
conjoint tendon
aponeurotic attachments of the transversus abdominis to the pubic tubercle (the classic conjoining of the aponeurosis of the intenal oblique and transversus aponeurosis <4%)
Conn’s syndrome
primary hyperaldosteronism, caused by an aldosterone-secreting tumor, resulting in hypertension, hypokalemia, hypernatremia, metabolic alkalosis, and low plasma renin
Cooley’s anemia
homozygous  thalassemia; Mediterranean anemia
Coombs test
direct, ability of anti-IgG or anti-C3 antisera to agglutinate the patient’s red blood cells; cold reacting antibodies react with anti-C3 (mostly drug-related antibodies, IgM antibodies (generally to polysaccharide), IgG antibodies of low affinity); indirect Coombs, serum of the patient is incubated with normal red cells, though IgM antibodies may agglutinate directly
Cooper's hernia
hernia through the femoral canal and tracking into the scrotum or labia majus
Cori’s disease
glycogen storage disease type III, deficiency in debranching enzyme, amylo-1,6-glucosidase, leading to variable accumulation of glycogen in the liver, heart, or skeletal muscle, characterized by stunted growth, hepatomegaly, and hypoglycemia
Corrigan’s pulse
in aortic regurgitation, pulses are of the water-hammer or collapsing type with abrupt distension and quick collapse, can be exaggerated by raising the patient’s arm
Councilman bodies
in apoptosis, hepatocytes that round up to form shrunken, pyknotic, and intensely eosinophilic bodies
Courvoisier’s law
tumors that obstruct the common bile duct result in an enlarged bladder; obstructing stones do not, since the gallbladder is typically too scarred to allow enlargement; present in half of pancreatic CA
Cowden disease
some rare hereditary cancer on chr 10; associated with mutations in PTEN/MMAC1
cri du chat
5p-, severe mental retardation, microcephaly, catlike cry, low birth weight, hypertelorism, low-set ears, and epicanthal folds
Crigler-Najjar syndrome
type I, no hepatic glucoronyltransferase activity, kernicterus, requires liver transplantation; type II, moderate deficiency of glucoronyltransferase, phenobarb induces activity
Cronkhite-Canada syndrome
diffuse GI hamartoma polyps (i.e., no cancer potential) associated with malabsorption/weight loss, diarhea and loss of electrolytes/protein; signs include alopecia, nail atrophy, and skin pigmentation
Crouzon syndrome
craniosynostosis correlated in mutations with the extracellular domain of FGFR2
Cruveihiler-Baumgarten bruit
bruit heard over caput medusa in portal hypertension
Cullen’s sign
a faintly blue coloration particularly of umbilicus as the result of retroperitoneal bleeding from any cause, but especially in ruptured ectopic pregnancy; also seen in acute pancreatitis (1-2%)
Curling’s ulcers
stress erosions and ulcers occurring in the proximal duodenum and associated with severe burns or trauma, from ischemia of the gastric mucosa
Curschmann’s spirals
spirally twisted masses of mucus plugs containing whirls of shed epithelium occurring in the sputum in bronchial asthma; Heinrich Curschmann, German physician, 1846-1910
Cushing reaction
increase in intracranial pressure cause compression of the cerebral blood vessels and cerebral ischemia, reaction of elevation in pressure with simultaneous reduction in heart rate, respiratory slowing
Cushing’s triad
signs of increased ICP 1. hypertension 2. bradycardia 3. irregular respirations
Cushing’s ulcer
acute ulcer of the stomach, proximal duodenum, or esophagus, frequently leads to hemorrhage or perforation, associated with intracranial injury or increases in intracranial pressure, associated with gastric acid hypersecretion
Da Costa syndrome
neurocirculatory asthenia, pain localized typically to the cardiac apex and consists of dull, persistent ache that lasts for hours, etc.
Dalrymple sign
retraction of the upper eyelid in Graves’s disease, causing abnormal wideness of the palpebral fissure
Dance’s sign
empty right lower quadrant in children with ileocecal intussusception
Dandy-Walker syndrome
hydrocephalus resulting from failure of the foramina Luschka and Magendie to open; associated with an occipital meningocele and agenesis of the cerebellar vermis and splenium of the corpus callosum; associated with warfarin use during pregnancy
Dane particle
mature HBV virion, 42 nm, double-layered, genome is double-stranded circular DNA, all regions of genome encode stuff
Darier’s sign
in mastocytosis (urticaria pigmentosa), is positive when a brown macular or a slightly papular lesion becomes a palpable wheal after being vigorously rubbed with the blunt end of an instrument such as a pen; wheal may not appear for 5-10 minutes
de Musset’s sign
in aortic regurgitation, head bobbing
De Quervain’s disease
a stenosing tenosynovitis of the thumb extensors and abductors
de Quervain’s thyroiditis
subacute granulomatous thyroiditis, viral etiology suspected
Dejerine-Roussy syndrome
thalamic lesions causing sensory loss, spontaneous pain, and perverted cutaneous sensation described in 1906
DENNIE’S LINES
in atopic dermatitis, an accentuated line or fold below the margin of the lower eyelid
Di Guglielmo syndrome
a non-nutritive megaloblastic anemia in which malignant red cell precursors are particularly evident
Diamond-Blackfan anemia
congenital pure red cell aplasia
Dick test
injection of erythogenic toxin of Strep. pyogenes, positive result in those lacking antitoxin
Dieulafoy’s aneurysm
AV malformation of the stomach (around 6 cm from the gastroesophageal junction), an uncommon cause of massive GI bleeding, a large submucosal artery erodes the mucosa without any overlying ulceration or other obvious mucosal damage
DiGeorge syndrome
failure of 3rd and 4th pharyngeal pouches to differentiate into the thymus and parathyroid glands, facial abnormalities result primarily from abnormal development of the first arch components during formation of face and ears
dimple sign
in dermatofibroma, lateral compression with thumb and index finger produces a depression, or “dimple.”
Döhle bodies
irregularly shaped greenish inclusions in neutrophil cytoplasm, consisting of ribosomes and/or rough ER and are seen in severe bacterial infections
Donath-Landsteiner antibody
in paroxysmal cold hemoglobinuria, an antibody associated with syphillis and viral infections, directed against the P antibody complex and can induce complement-mediated lysis; attacks precipitated by exposure to cold and are associated with hemoglobinemia and hemoglobinuria; chills and fever; back, leg, and abdominal pain; headache and malaise; recovery prompt; asymptomatic otherwise
Donovan bodies
in Calymmatobacterium granulomatis or granuloma inguinale, bodies characterized by multiple organisms filling large histiocytes
Dressler’s syndrome
pericarditis, possible autoimmune etiology, found to develop 2 weeks to several months after acute MI
Druckrey relationship
relationship between carcinogen dose and tumor induction time: dtn = k where d is the dose of carcinogen, t is latency period, n is the slope of the double log [plot of carcinogen dose versus induction time, and k is a constant
Dubin-Johnson syndrome
mostly conjugated hyperbilirubinemia from defect in transport of bilirubin and other organic anions across the canaliculus; other liver function tests normal; accumulation of dark pigment in liver lysosomes
duct of Santorini
dorsal duct of pancreas, embryologically, the biggest duct, clinically the smaller pancreatic duct
duct of Wirsung
embryologically confined to the ventral pancreas, becomes functionally the main pancreatic duct after duct fusion occurs; drains the bulk of pancreatic secretion through the major papilla
ducts of Luschka
small tubular channels found buried within the gallbladder wall adjacent to the liver, communicates with the biliary tree, rarely patent accessory bile secretory ducts
dumping syndrome
delivery of a large amount of hyperosmolar chyme into the small bowel, usually after vagotomy and a gastric drainage procedure, results in autonomic instability, abdominal pain, and diarrhea
Dunphy sign
increased pain with coughing in appendicitis
Dupuytren’s contracture
palmar fibromatosis
Duroziez’s sign
in aortic regurgitation, systolic murmur heard over the femoral artery when it is compressed proximally and a diastolic murmur when it is compressed distally
Ebstein’s anomaly
malformation characterized by the downward displacement of the tricuspid valve into the right ventricle due to anomalous attachment of the tricuspid leaflets; associated with maternal exposure to Li
Edwards’s syndrome
trisomy 18, mental retardation, prominent occiput, micrognathia, low-set ears, rocker-bottom feet, flexion deformities of the fingers, and congenital heart disease
Eisenmenger complex
a ventricular septal defect with right ventricular hypertrophy, severe pulmonary hypertension, and frequent straddling of the defect by a misplaced aortic root
Eisenmenger syndrome
cardiac failure with significant right to left shunt producing cyanosis due to higher pressure on the right side of the shunt; usually due to the Eisenmenger complex any anomalous circulatory communication that leads to obliterative pulmonary vascular disease; or maybe
Ellsworth-Howard test
for diagnosis of pseudohypoparathyroidism, can administer PTH
Elschnig spots
yellow (early) or hyperpigmented (late) patches of retinal pigment epithelium overlying infarcted choriocapillaris lobules in hypertensive retinopathy
Emery Dreifus muscular dystrophy
proximal weakness with quite pronounced muscle contractions and by severe cardiac arrhythmias which may cause sudden death
Epsom salts
magnesium sulfate, laxative
Epstein’s pearls
small, white cysts along the median raphe of the hard palate
Epstein-Barr virus
EBV binds to CD21 found on epithelial cells and B cells; however a large number of T suppressor cells and EBV specific cells are seen as atypical lymphocytes
Erb palsy
C5 and C6 nerve roots
Erlenmeyer’s flask deformity
seen in osteopetrosis, where the ends of long bones are bulbous
erythroplasia of Queyrat
carcinoma in situ of the glans penis Auguste Queyrat, French dermatologist, born 1872
Evan’s syndrome
ITP and immunohemolytic anemia
EWART’S SIGN
in large pericardial effusion, dullness to percussion of the left lung over the angle of the scapula may occur; due to compressive atelectasis by the large pericardial sac
Ewing’s sarcoma
onion skinning
facies Hippocratica
the mask of death following peritonitis, i.e. as a result of vomiting and depressed circulation, the face becomes pinched and anxious, the cheeks hollow, and the eyes dim and beringed with dark circles
factor V Leiden
Arg(506)  Gln, results in resistance to cleavage by activated protein C (an anticoagulant), found in 20% of patients with venous thromboembolism, 6% of U.S. population
Fanconi’s anemia
autosomal recessive, predisposal to aplastic anemia, progressive bone failure at age 5-7, congenital malformations
Fanconi’s syndrome
generalized dysfunction of proximal renal tubule leading to glycosuria, hyperphosphaturia, hypophasphatemia, aminoaciduria, and systemic acidosis; may be associated with out-dated tetracyclines
farmer’s skin
cutis rhomboidalis nuchae
Felty’s syndrome
the combined features of rheumatoid arthritis, splenomegaly, and neutropenia, and leg ulcers; associated with HLA-DR;
Ferguson’s reflex
anesthesia in ob, interruption of oxytocin release in response to cervical dilatation may cause uterine inhibition
fifth disease
erythema infectiosum, associated with parvovirus B19 infection, characterized by “slapped cheeks” and erythematous lacy eruption on the trunk and extremities; other 4 childhood rash diseases: measles, rubella, scarlet fever, and roseola (herpesvirus VI)
Fisher’s syndrome
ataxia with ophthalmoplegia and areflexia; a form of polyneuroradiculitis
Fitz-Hugh-Curtis syndrome
associated with spread of gonococci or chlamydiae: perihepatitis manifested by right upper quadrant or bilateral upper abdominal pain and tenderness and occasionally by a hepatic friction rib
foramen of Magendie
midline foramen exiting out of fourth ventricle
foramen of Winslow
anteior portal triad; posterior IVC and right crus of diaphragm; superior caduate lobe; inferior superior part of duodenum, portal triad
foramina of Luschka
two laterally placed foramens exiting out of fourth ventricle
foramina of Monro
connects each of the lateral ventricles with the third ventricle
Foster Kennedy syndrome
optic atrophy, contralateral papilledema, and anosmia; may be associated with olfactory groove meningioma
Fothergill’s sign
in rectus sheath hematomas, tender mass that does not cross the midline and remains palpable when patient tenses the rectus muscle (bluish discoloration not usualy seen until 3 or 4 days)
Fox's sign
in hemorrhagic pancreatitis, ecchymosis of the inguinal ligament due to blood tracking from the retroperitoneum and collecting at the inguinal ligament
Franklin’s disease
gamma heavy chain disease, characterized by LAD, fever, anemia, malaise, HSM, and weakness, most distinctive symptom palatal edema
Freidreich’s foot
seen in Freidreich6s ataxia, pes cavus with hammer toe
Friedreich’s ataxia
a spinocerebellar degeneration, AR manifesting at 11 years, a less common AD 20 years; initial symptoms, gait ataxia, hand clumsiness, dysarthria, DTRs absent (extensor plantar present), joint position and vibratory sense impaired, sometimes loss of pain and temperature, paralyzed over course of 20 years, high incidence of diabetes and hypertrophic cardiomyopathy (dilated less common) and arrhythmias
Froment’s sign
dx of ulnar nerve lesion; caused by flexor pollicus longus (median nerve) which comes into action when the patient attempts to grip a flat object between the thumb and the hand, and causes flexion at the interphalangeal joint
Gaisböck’s syndrome
stress polycythemia, combination of modest elevation in hematocrit and normal red cell mass; unknown etiology, seen in hard-driving middle-aged males, usually smokers, who in addition tend to be overweight and hypertensive
Galeazzi fracture
fracture of the radius at the junction of the middle and distal thirds accompanied by disruption of the distal radioulnar joint
Gallavardin effect
midsystolic murmur of AS may be well transmitted to the apex, especially in older patients where it becomes harsh and slightly higher pitched, the so-called Gallavardin effect
Gallivardin’s phenomenon
systolic ejection murmor in aortic stenosis best heard in aortic area, often disappears over sternum, reappears in apical area, mimicking mitral regurgitation
Gandy-Gamna nodules
in congestive splenomegaly, organization of focal hemorrhages giving rise to foci of fibrosis containing deposits of iron and calcium salts encrusted on connective tissue and elastic fibers
Gardner’s syndrome
familial adenomatous polyposis (now realized all patients with FAP have extraintestinal manifestations), exhibiting intestinal polyps identical to those in FAP combined with 1. sebaceous cysts, 2. osteomas (particularly of the mandible, skull, and long bones), 3. desmoid tumors; mutation in adenomatous polyposis coli (APC) gene in 5q
gastrinoma triangle
a triangle where more than 90% of extrapancreatic gastrinomas are located bordered by 1. Third portion of duodenum, 2. Cystic duct, 3. Pancreatic neck
Gaucher’s disease
AR, mutations in glucocerebrosidase gene on 1q21, enzyme cleaves glucose residue from ceramide; type I, chronic non-neuronopathic form, splenic and skeletal involvement
Gerota’s fascia
fascia surrounding the kidney
Gerstmann-Straussler-Scheinker syndrome
slow central nervous system disease with same mutation, point mutation in codon 102 of prion protein as CJD
Ghon lesion
primary area of tuberculosis infection
Gibbs-Donnan equilibrium
in RBCs, biocarbonate ions diffusing out and chloride diffusing in
Gilbert’s syndrome
with the exception of hemolytic anemias, most common cause of mild unconjugated hyperbilirubinemia from mild decrease glucoronyltransferase activity, responds to phenobarbital, affects up to 7% of population
glands of Montgomery
sebaceous glands of areola
Glanzmann’s thrombasthenia
Gp IIb/IIIa receptor for fibrinogen missing
Glauber’s salt
sodium sulfate, laxative
Glisson's capsule
liver capsule
glycogen storage diseases
type I, von Gierke’s disease type II, Pompe’s disease type III, Cori’s disease type V, McAdle’s syndrome
Goetz sign
in PDA, jet of unopacified blood from aorta into opacified blood of pulmonary artery
Goldie-Coldman hypothesis
malignant cells likely to acquire spontaneous resistance to cytotoxic drugs as they progressively grow and divide, even without any exposure to those drugs
Goodpasture’s syndrome
glomerulonephritis characterized by linear deposits of antibody along the glomerular basement membrane, antibodies interact with alveolar wall, leads to pulmonary hemorrhage and pulmonary fibrosis; Goodpasture antigen, resides in the noncollagenous portion of the 3 chain of collagen type IV; high prevalence of DRW15/DQW6
Goodsall’s rule
anal fistulae course in a straight path anteriorly and take a curved path posteriorly
Gorham-Stout disease
vanishing or disappearing bone disease; IL-6 has pathogenetic role
Gottron’s papules
violaceous papules over knuckle prominences found in dermatomyositis
Gower’s maneuver
Duchenne’s muscular dystrophy, patient using hands to help himself get up
Grey Turner’s sign
local areas of discoloration about the umbilicus and particularly in the region of the *loins*, in acute hemorrhagic pancreatitis (1-2%) and other causes of retroperitoneal hemorrhage
Griffith’s sign
in thyrotoxicosis, lag of the lower lids during elevation of the globes
Grotton’s lesions
in dermatomyositis, scaling erythematous eruption or dark red patches over the knuckles, elbows, knees
Gunn’s sign
tapering of veins on either side of AV crossing in hypertensive retinopathy (grade 3); c.f. Salus’s sign and Bonnet’s sign
Gunther’s disease
AR congenital erythropoietic porphyria from decreased URO synthase activity, hemolytic anemia, cutaneous lesions
Guthrie test
for dx of PKU, a bacterial assay for phenylalanine
Hailey-Hailey disease
benign familial chronic pemphigus
Haldane effect
deoxygenated hemoglobin having a greater affinity for CO2 than oxygenated hemoglobin
Ham’s test
for diagnosing paroxysmal nocturnal hemoglobinuria, uses increased sensitivity of PNH-affected RBCs to lysis by complement; introduced in late 1930’s
Hamman’s sign
mediastinal crunch is a series of precordial crackles synchronous with the heart beat, not with respiration. Best heard in the left lateral position, it is due to mediastinal emphysema (pseudomediastinum), seen with Boerhaave’s syndrome
Hamman-Rich syndrome
idiopathic pulmonary fibrosis; immune complex disease with progressive fibrosis of the alveolar wall
Hampton’s hump
pulmonary infarction is classically described as wedge-shaped infiltrate that abuts the pleura; often associated with a small pleura effusion that is usually exudative and may be hemorrhagic; not specific
Hampton’s line
radiolucent collar of granulation tissue across the base of an ulcer
Hand-Schüller-Christian triad
in multifocal Langerhans’ cell histiocytosis, triad of calvarial bone defects, diabetes insipidus, and exophthalmos
hangman’s fracture
fracture of pars interarticularis of C2, hyperextension injury
Hansen’s stain
special stain used to detect eosinophiluria on the urine sediment
Harrison’s groove
during active rickets, the protuberant rachitic abdomen pushes the plastic lower ribs outward on a fulcrum formed by the costal attachments of the diaphragm; the line of bending forms a groove or sulcus in the rib cage
Hartnup disease
an autosomal recessive disorder in which there is a reduction of small-intestinal and renal transport of certain neutral amino acids, including (trp, a precursor of niacin), causing elevated amino acid excretion in urine and feces
Hashimoto’s thyroiditis
first described in 1920, goitrous chronic autoimmune thyroiditis; in areas with sufficient iodine, elevated TSH is often viewed as evidence of chronic autoimmune thyroiditis as well as antithyroid antibodies; antithyroglobulin antibodies in 60% of patients and antithryoid microsomal antibodies in 95%
Hatchcock’s sign
upward pressure applied to the angle of the mandible (ramus) produces tenderness with mumps but no tenderness with adenitis
Hawthorne effect
the effect (usually positive or beneficial) of being under study, upon the persons being studied; their knowledge of the study often influences behavior [city in Illinois; site of the Western Electric plant]
Heberden’s nodes
characteristic in women, but not in men, represent prominent osteophytes at the distal interphalangeal joints in OA (enlargments of tubercles at the articular extremities of the distal phalanges
Heerfordt-Waldenström syndrome
sarcoidosis associated with fever, parotid enlargement, anterior uveitis, and facial nerve palsy
Heineke-Mukulicz pyloroplasty
longitudinal incision through all layers of the pylorus, sewed closed in a transverse direction to make the pylorus nonfunctional (used after truncal vagotom)
Heinz bodies
seen in unstable hemoglobin and oxidant stress; precipitates of denatured hemoglobin on red blood cells; *only visible* when blood is supravital stained (crystal violet); not seen on routine blood smears
hemoglobin Bart’s
four  globin chains, seen in hydrops fetalis ( thalassemia), very high oxygen affinity
hemoglobin Lepore
no  chain;  chain by - hybrid
hemoglobin Portland
22
Henoch-Schönlein purpura
systemic hypersensitivity disease of unknown cause characterized by purpuric rash, colicky abdominal pain (presumably due to focal hemorrhages into the GI tract), polyarthralgia, and acute glomerulonephritis; may result from deposition of circulating immune complexes within stuff; hypersensitivity purpura, etiology group A streptococci
Hering’s nerve
carotid sinus nerve, CN IX, carries information to the vasomotor center in the brainstem
Hering-Breuer reflex
stretch receptor (in smooth muscle of airways) reflex, responsible for apnea, i.e. decreased breathing frequency, as a result of lung inflation
Herlitz syndrome
epidermolysis bullosa lethalis; mutation that prevents the folding of laminin 5 Gillis Herlitz, Swedish pediatrician, born 1902
Hesselbach’s triangle
where direct inguinal hernias occur, bound by the inguinal ligament, inferior epigastric a., and rectus abdominus muscle
Hill’s sign
in aortic regurgitation, refers to popliteal cuff systolic pressure exceeding brachial cuff pressure by more than 60 mm Hg
Hippocratic fingers
clubbing
Hirano bodies
in Alzheimer’s, intracytoplasmic proximal dendritic eosinophilic inclusions consisting of actin
Hirschprung’s disease
megacolon; congenital disorder characterized by colonic dilatation proximal to an aganglionic, contracted distal colon and rectum; caused by gestational failure of neural crest cells to migrate to distal colon; an AD form has been reported with mutations of the RET gene, and an AR form with mutation of the endothelin-B-receptor gene
Hoffman sign
thumb adduction in response to flexion of the distal phalanx of the third digit, an example of abnormal upper limb reflex caused by *damage to the descending cortical fibers*
Hollenhorst plaques
cholesterol emboli visible as small bright flecks lodged in arterial bifurcations in retina
Holmes-Adie syndrome
Adie’s pupil, frequently affects young women, benign familial disorder that may be associated with depressed DTRs (especially in legs), segmental anhidrosis, orthostatic hypotension, or cardiovascular autonomic instability, may be caused by degeneration of ciliary ganglion, followed by aberrant reinnervation of the pupilloconstrictor muscles
Homan’s sign
deep thrombi in the larger outflow veins, causing edema of the foot and ankle and producing pain and tenderness on compression of the calf muscles (by either squeezing the calf muscles or forced dorsiflexion of the foot)
Hoover’s sign
a modification in the movement of the costal margins during respiration, caused by a flattening of the diaphragm; suggestive of empyema or other intrathoracic condition causing a change in the contour of the diaphragm
Horner’s syndrome
enophthalmos, ptosis, miosis, and anhidrosis, unilateral; small (miotic) pupil associated with mild ptosis (of the upper lid, not as pronounced as with oculomotor lesions) and sometimes loss of sweating (anhidrosis); if present since infancy, the ipsilateral iris is lighter and blue (heterochromia iridis); topical 4% cocaine will dilate normal pupil but not a desympathectomized pupil
Howell-Jolly body
in asplenia or malfunctional spleen, *nuclear remnants* on red blood cells as small, round, darkly-stained nuclear fragments; no special stains necessary; may be seen in 30-50% of adults but not in children with untreated celiac sprue
Howship’s lacunae
resorption pits on bone formed by osteoclasts
Howship-Romberg sign
pain along the inner aspect of the thigh; seen with an obturator hernia due to nerve compression
Hurler’s syndrome
AR mucopolysaccharidosis that is caused by deficiency of -L-iduronidase, with consequent accumulations of the mucopolysaccharides heparan sulfate and dermatan sulfate in the heart, brain, liver, and other organs; progressive deterioration, hepatosplenomegaly, dwarfism, gargoyle-like facies, stubby fingers, corneal clouding, progressive mental retardation, and death by age 10
Hürthle cells
in Hashimoto’s thyroiditis, deeply stained colloid or clusters of these oncocytes having an abundant, brightly eosinophilic granular cytoplasm, thought to represent a degenerated state of the follicular epithelium
Hutchinson freckle
lentigo maligna, a nonfamilial precursor to lentigo maligna melanoma
Hutchinson’s teeth
smaller and more widely spaced than normal and are notched on their biting surfaces; sign of congenital syphillis
Hutchinson-Guilford syndrome
aka progeria a condition in which the normal development of the first year is followed by gross retardation of growth, with a senile appearance characterized by dry wrinkled skin, total alopecia, and bird-like facies; genetics unclear
Imerslund-Grösbeck syndrome
an autosomal recessive condition where transport inward of cobalamin from ileal receptors is faulty; associated with megaloblastic anemia, proteinuria, renal tubular defects, and various congenital abnormalities of the renal pelvis and ureter
infarct of Zahn
in occlusion of an intrahepatic branch of portal vein, sharply demarcated area of red-blue discoloration, not infarct, not necrosis, only marked stasis in distended sinusoids, with secondary hepatocellular atrophy
Irish’s node
left axillary adenopathy associated with metastatic disease, e.g. gastric CA
Isaacs’s syndrome
continuous muscle stiffness, rippling muscle movements (myokymia), delayed relaxation following muscle contraction
Ito cells
vitamin A fat-storage cells, of mesenchymal origin found in the space of Disse; during development of cirrhosis, they become activated, transform into fibroblast-like cells
Jaccoud’s arthritis
in SLE, ulnar deviation of the fingers, swan neck deformities, and subluxations, initially reversible but can become fixed
Jackson-Weiss syndrome
craniosynostoses as well as limb defects, mutations if FGFR2, broad great toes
Janeway lesions
in infective endocarditis; nonpainful, small, erythematous or hemorrhagic macules or nodules of palms or soles; more common in acute bacterial endocarditis but occur in subacute bacterial endocarditis
Jansen metaphyseal chondrodysplasia
an autosomal dominant form of dwarfism resulting from an activating mutation in the PTHrP receptor, premature ossification from acceleration of the transition from proliferative to hypertrophic chondrocytes
Jarisch-Herxheimer reaction
sudden fevers, rigors, and persistent hypotension following antimicrobial treatment of louse-borne relapsing fever (Borrelia recurrentis (spirochete) infection) or syphilis; treatment with anti-TNF- Fab before penicillin suppresses this reaction (NEJM 335:311)
Jod-Basedow phenomenon
thyroid hyperfunction induced by excess iodine ingestion in patients with various thyroid disorders; Jod German for iodine; K. A. Von Basedow
Joffroy sign
in thyrotoxicosis, absence of forehead wrinkling with upward gaze, the head being tilted down; disorder of the arithmetic faculty in the early stages of organic brain disease
Jolly test
good test for distinguishing between Lambert-Eton syndrome and myasthenia gravis. Friedrich Jolly, German neurologist, 1844-1904
Jones’s fracture
fracture at the base of the fifth metatarsal diaphysis
Kallman’s syndrome
anosmia; hypogonadotropic hypogonadism stemming from failure of LHRH-expressing neurons to migrate, etc.
Kanavel's sign
four signs of tenosynovitis: 1. affected finger held in slight flexion; 2. pain over volar aspect of affected finger tendon upon palpation; 3. swelling of affected finger; 4. pain on passive extension of affected figner
Kartagener’s syndrome
a triad of sinusitis, bronchiectasis, and situs inversus; also associated with ciliary dysfunction; Manes Kartagener, Swiss physician, 1897-1975
Kasabach-Merritt syndrome
capillary hemangioma associated with thrombocytopenic purpura and extensive and progressively enlarging vascular malformations which may involve large portions of their extremities; bleeding commonly develops in the first year of life, secondary to chronic DIC triggered by stagnant blood flow through the tortuous abnormal vessels; anemia caused by red cell damage as blood passes through deformed vessels of the tumor
Kawasaki’s disease
acute febrile illness of infants and children, characterized by cutaneous and mucosal erythema and edema with subsequent desquamation, cervical lymphadenitis, and complicated by coronary artery aneurysms (20%)
Kayser-Fleischer rings
a greenish yellow pigmented ring encircling the cornea just within the corneoscleral margin, seen in hepatolenticular degeneration, due to copper deposited in Desçemet’s membrane (posterior limiting layer of cornea); seen in *Wilson’s disease* (with neurologic involvement) and other cholestatic hepatic diseases
Kearns Sayre syndrome
a mitochondrial disease, salient features are progressive opthalmoplegia with retinal pigmentation degeneration and heart block
Kehr’s sign
pain in the left shoulder associated with splenic rupture
Kelly’s sign
visible peristalsis of the ureter in response to squeezing or retraction; used to identify the ureter during surgery
Kerckring’s valves
plicae circulares (lining of small intestine)
Kerley B lines
a radiographic evidence of pulmonary venous hypertension, horizontal linear opacities on chest radiograph often found in the periphery due to separation of the interlobular space, as seen in pulmonary edema or fibrosis, reflecting thickening of, or fluid in, lymphatic vessels in interlobular septae, a consequence of interstitial edema; also seen in lymphangitic spread of malignancies
Kernig sign
flex patient’s leg at both hip and knee, and then straighten knee; positive sign—pain and increased resistance to extending knee  suggests meningeal irritation
Kernohan notch
tentorial edge, pressure against seen in uncal herniation
Kiesselbach’s plexus
vascular plexus on the anterior nasal septum, bleeding from, leads to most common form of epistaxis
Kikuchi’s disease
histiocytic necrotizing lymphadenitis, characterized by cervical lymphadenopathy with tenderness, fever, and night sweats
Kimmelstiel-Wilson disease
intercapillary glomerulosclerosis from diabetes; lesion is PAS-positive material deposited at periphery of glomerular tufts
Klatskin tumors
tumors arising from the part of the common bile duct between the cystic duct junction and the confluence of the right and left hepatic ducts at the liver hilus; notable for their slow growing behavior, marked sclerosing characteristics, and the infrequent occurrence of distal metastases
Kleihauer-Betke test
testing for the presence of fetal blood cells in maternal circ
Kleine-Levin syndrome
a rare form of periodic hypersomnia associated with bulimia, occurring in males aged 10 to 25 years, characterized by periods of ravenous appetite alternating with prolonged sleep (as long as 18 hours), along with behavioral disturbances, impaired thought processes, and hallucinations; acute illness or fatigue may precede an episode, which may occur as often as several times a year
Klumpke palsy
C8-T2 nerve roots
Klüver-Bucy syndrome
1937 bilateral removal of temporal lobe (including amygdala and hippocampal formation) in monkeys, animals became tame, showed a flattening of emotions, exhibited remarkable oral tendencies (they put all manner of objects into their mouths), enormous increase in sexual behavior, including mounting of inappropriate objects and species, compulsive tendency to react to every object, failed to recognize familiar objects
Koebner’s phenomenon
referring to physical trauma (rubbing or scratching) as a major factor in eliciting *psoriasis lesions*; also depigmented, sharply demarcated papules in vitiligo following minor trauma; may also be seen in *lichen planus*
Köhler’s bone disease
aseptic necrosis of the navicular bone
koilocyte
characteristic cytoplasmic vacuole, hallmark of infection by papillomavirus
Koplik’s spots
pathognomonic exanthem in measles
Korotkoff sounds
sounds heard in between systolic and diastolic pressure; origin related to the spurt of blood passing under the cuff and meeting a static column of blood
Korsakoff’s syndrome
profound memory loss; mammillary bodies as well as portions of medial thalamus believed to be destroyed from thiamine deficiency accompanying alcoholism
Kostmann syndrome
inherited neutropenia, responds to G-CSF, may be due to environmental insults in bone marrow, characterized typically by a granulopoeisis impairment at the promyelocyte stage
Krukenberg’s tumor
metastatic GI neoplasia to the ovaries, produces bilateral metastases of mucin-producing, signet-ring cancer cells, most often of gastric origin
Kübler-Ross dying stages
denial, anger, bargaining, grieving, acceptance
Kulchitsky’s cells
neuroendocrine argentaffin cells present along the bronchial epithelium, particularly in the fetus and neonate; small cell carcinoma has granules similar to Kulchitsky’s cells
Kussmaul’s respiration
paroxysmal air hunger, associated with acidosis, especially DKA
Kussmaul’s sign
in constrictive pericarditis, jugular vein becomes more distended during inspiration (normally, jugular venous pressure decreases with inspiration since blood drains into heart with inspiration); though most common contemporary cause is severe right-sided heart failure (JAMA 1996: 275:632); generally negative in cardiac tamponade
Kveim test
an intradermal test for the detection of *sarcoidosis*, done by injecting Kveim antigen (a saline suspension of human sarcoid tissue prepared from the spleen of an individual with active sarcoidosis) and examining skin biopsies after 3 and 6 weeks; positive test, a noncaseating granulomnatous reaction; sensitivity 35-88%, specificity 75-99% Morton A. Kveim, Norweigian physician, born 1892
Laennec’s cirrhosis
in alcoholic cirrhosis, residual parenchymal nodules that protrude like “hobnails” from the surface of the liver
Laplace’s law
wall tension = pressure x radius (thus colon perforates preferentially at the cecum because of the increased radius and resultant increased wall tension)
Laron dwarfism
autosomal recessive, growth hormone receptor defects, low IGF-1 levels
Lasègue’s sign
when patient is supine with hip flexed, dorsiflexion of ankle causing pain or muscle spasm in the posterior thigh indicates lumbar root or sciatic nerve irritation
Laurence-Moon-Biedl syndrome
obesity, retinitis pigmentosa, metal retardation, skull deformities, polydactyly, and syndactyly
Legg-Calvé-Perthes disease
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
Leiner’s disease
seborrheic erythroderma associated with diarrhea and failure to thrive and to generate C5a chemotactic factor
leprechaunism
1 in 4 million births, associated with elfin-like facies, decreased subcu adipose tissue, acanthosis nigricans, and growth retardation, failure to thrive, and early death, insulin resistance
Leriche’s syndrome
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)
Lesch-Nyan syndrome
a complete lack of hypoxanthine guanine phosphoribosyl transferase (HGPRT, involved in salvage pathway in purine synthesis), X-linked, hyperuricemia, severe neurologic deficits with mental retardation, self-mutilation, and in some cases gouty arthritis
Leser-Trélat sign
the sudden appearance and rapid increase in the number and size of seborrheic keratoses with pruritus; associated with internal malignancy
Letterer-Siwe disease
acute disseminated Langerhans’ cell histocytosis; associated with development of cutaneous lesions that resemble a seborrheic eruption secondary to infiltrations of Langerhans’ histocytes over the front and back of the trunk and on the scalp; concurrent hepatosplenomegaly, lymphadenopathy, pulmonary lesions, and eventually destructive osteolytic bone lesions
Levine’s sign
when describing angina, defining the constricting discomfort with a clenched fist over sternum; q.v. angina for sens and spec; Samuel A. Levine, U.S. cardiologist, 1891-1966; designated hand movements 80% sens 49% spec; Levine sign itself 14% sens for cardiac pain (BMJ 1995;311:1660)
Lewy bodies
eosinophilic intracytoplasmic bodies in neurons of substantia nigra and locus ceruleus present in Parkinson’s disease
Lhermitte’s sign
sudden electric-like shocks extending down the spine on flexing the head; may result from toxic effects of radiation; (may be found in vitamin B6 toxicity); (may be found in vitamin B12 deficiency)
Libman-Sacks disease
in systemic lupus erythematosus, intense mitral and tricuspid valvulitis with development of small, sterile vegetations
Li-Fraumeni syndrome
mutant p53 allele inherited, predisposition toward breast carcinomas, sarcomas, and brain tumors
ligament of Trietz
the suspensory muscle of the duodenum which supports the duodenojejunal flexure
lines of Zahn
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
Lisch nodules
in type I neurofibromatosis, pigmented iris hamartomas
Lissauer’s tract
small diameter primary sensory axons (presumably mediating pain and temperature senses) on their way into the dorsal horn
Littre's hernia
hernia involving a Meckel's diverticulum
Löffler’s syndrome
simple pulmonary eosinophilia, characterized by transient pulmonary lesions, eosinophilia in the blood, and a benign clinical course; lungs show alveoli whose septa are thickened by an infiltrate composed of eosinophils and occasional interspersed giant cells, but there is no vasculitis, fibrosis, or necrosis; associated with ascaris, strongyloides
Löfgren’s syndrome
sarcoidosis associated with erythema nodosum and fever and transient arthritis, bilateral hilar adenopathy; 10-15% of sarcoidosis present this way
Looser’s zones
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
Lovibond’s angle
the angle made by the proximal nail fold and the nail plate
Löwenstein-Jensen’s medium
for growing out M. tuberculosis; contains malachite green, a triphenlyamine die like crystal violent, inhibits growth of unwanted organisms during 6 week incubation period as well as complex nutrients
Lown-Ganong-Levine syndrome
enhanced AV node pathways
Ludwig’s angina
aggressive infectious process of the submandibular, sublingual, and submental fascial spaces frequently occuring as a result of infection from 2nd and 3rd lower molar; 54% mortality in preantibiotic era, now 4% described in 1836 by Wilhelm Frederick von Ludwig
Lugol’s solution
iodine and KI
Lund’s node
lymph node found in Calot's triangle, aka Calot's node
Lutembacher’s syndrome
atrial septal defect with mitral stenosis; though mitral stenosis is often of rheumatic origin
Lynch syndrome I
AD produces multiple colon cnacers 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
Lynch syndrome II
cancer family syndrome, all features of Lynch I with early onset of carcinoma at other sites including endometrium, ovaries, and stomach
MacCallum’s plaques
irregular thickenings, usually in the left atrium, from subendocardial lesions, usually exacerbated by regurgitant jets
Maffucci’s syndrome
endochondromatosis associated with soft tissue hemangiomas; associated with ovarian carcinomas and brain gliomas; essentially Ollier’s disease with hemangiomas
Mallory body
“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
Mallory-Weiss tears
small defects in gastroesophageal junction, from violent retching and beef with alcoholic gastritis, can bleed like crazy, 87% occur below gastroesophageal junction; bleeding stops 90% without intervention
Malta fever
brucellosis
Maltese cross
fat droplets in urine
Marcus Gunn pupil
afferent pupillary defect, pupil dilates instead of constricts because of optic nerve defect
Marie-Strümpell disease
refers to ankylosing spondylitis in Europe
Marjolin's ulcer
squamous cell carcinoma ulceration overlying chronic osteomyelitis or burn scar
Markle sign
jar tenderness in abdomen from heel drop as a localizing sign of peritoneal irritation; described in 1973
Mayer-Rokitansky-Küster-Hauser syndrome
absence of vagina from abnormality of müllerian development
May-Hegglin anomaly
neutrophils with large pale blue inclusions resembling Döhle bodies, giant platelets; rare AD asymptomatic trait
Mazzotti reaction
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
McArdle’s syndrome
glycogen storage disease V, deficiency in muscle phosphorylase, with consequent glycogen accumulation in skeletal muscle, produces painful muscle cramps and muscle weakness following exercise
McBurney’s point
one-third the distance from the ASIS to the umbilicus
McBurney’s sign
tenderness at McBurney’s point in appendicitis
McCune-Albright syndrome
triad of irregular café au lait spots, fibrous dysplasia of long bones with cysts, and precocious puberty
Means-Lerman scratch
in hyperdynamic heart (as in hyperthyroidism), a systolic scratch occasionally heard in the second left intercostal space during expiration, presumed to be secondary to rubbing together of normal pleural and pericardial surfaces
Meckel scan
99Tc pertechnetate scan that selectively tags acid secreting cells (gastric mucosa); it is used most often for unexplained bleeding in infants and young adults
Meckel’s cartilage
branchial arch 1
Meckel’s diverticulum
persistence of vitelline duct, contains all 3 layers of bowel (mucosa, submucosa, muscularis propria), antimesenteric, present in 2% of population, usually within 30 cm of ileocecal valve; the five 2s: 2” long, 2 feet from ileocecal valve, 2% of population, commonly persists in first 2 years of life, may have 2 types of epithelia
Mees’s lines
horizontal white bands of the nails seen in chronic arsenical poisoning, and occasionally in leprosy; R.A. Mees, 20th century Dutch physician
Meigs’s syndrome
unusual combination of hydrothorax, ascites, and ovarian fibroma
Meissner’s plexus
submucous plexus; innervates glandular epithelium, muscularis mucosa, intestinal endocrine cells, and submucosal blood vessels
Mendelson’s syndrome
chemical pneumonitis after aspiration of gastric contents
Ménétrier’s disease
giant cerebriform enlargement of the rugal folds of the gastric mucosa, results from profound hyperplasia of the surface mucous cells with accompanying glandular atrophy, most often encountered in men (3:1), 40s-60s, sometimes in children, may produce epigastric discomfort, weight loss, and sometimes bleeding related to superficial rugal erosions, gastric secretions mostly mucous, little HCl, may be sufficient protein loss to produce hypoalbuminemia
Méniere’s disease
1. fluctuating sensorineural loss, classically involving the low frequencies; 2. Vertiginous episodes; 3. Aural pressure; 4. Tinnitus that is most frequently described roaring; pathologic changes are said to consist of a dilation of the endolymphatic system that leads to a degeneration of the delicate vestibular and cochlear hair cells
Menke’s disease
X-linked, problem in the distribution of copper in the body, amount of copper and ceruloplasmin in serum reduced, excess of copper in intestinal mucosa, muscle, spleen, and kidney; usually fatal by age 3
Metzer index
MCV/RBC ratio; >13 iron def, < 13 thalassemia
Meyer’s loop
a portion of the optic radiations subserving vision from the superior field coursing rostrally within the temporal lobe before heading caudally to the primary visual cortex; lesions produce contralatteral upper quarantanopia (pie in the sky)
Mikulicz’s syndrome
bilateral inflammatory enlargement of the parotid, submaxillary, and sublingual, and lacrimal glands and xerostomia, secondary to sarcoid, leukemia, lymphoma, etc.
Milkman’s fractures
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
Mirrizi’s syndrome
extrinsic obstruction of the common bile duct from a cystic duct gallstone
mitral facies
malar flush with pinched and blue facies in mitral stenosis
Möbius syndrome
congenital facial paralysis with or without limb defects associated with misoprostol use
Modigliani syndrome
thyroid in normal position but people with long curving necks enhance prominence and palpation of thyroid
Mönckeberg’s arteriosclerosis
ring-like calcifications within the media of medium-sized to small muscular arteries (femoral, tibial, radial, and ulnar arteries, genital arteries), occurs almost exclusively in individuals over 50 years old; doesn’t narrow lumen, distinct from atherosclerosis
Mondor’s disease
thrombophlebitis of superficial breast veins
Monge’s disease
chronic mountain sickness, loss of high altitude tolerance after prolonged exposure, characterized by extreme polycythemia, exaggerated hypoxemia, and reduced mental and physical capacity; relieved by descent
Monsel’s solution
ferric subsulfate
Monteggia fracture
fracture of the proximal third of the ulna with a dislocation of radial head
Mooren corneal ulcers
chronic, painful ulcers, involves circumference of peripheral cornea and may progress to vision loss; associated with hep C
Morgagni's hernia
anterior parasternal diaphragmatic hernia, right more common than left
Morrison’s pouch
hepatorenal recess; the most posterior cavity in the peritoneal cavity
Mucha-Habermann disease
pityriasis lichenoides et varioliformis acuta, scattered necrotic papules and vesicles that can resemble insect bites but usually are more generalized and symmetric
Muehrcke’s nails
paired narrow horizontal white bands that are immobile as nail grows; seen in hypoalbuminemia, nephrotic syndrome
Müller’s maneuver
reverse Valsalva
Müller’s sign
in aortic regurgitation, systolic pulsations of the uvula
Müller-Lyer illusion
the two horizontal lines stuff with arrows
Munro’s microabscesses
in psoriasis, when neutrophils form small aggregates within the parakeratotic stratum corneum
Murphy’s sign
a sharp increase in tenderness with a sudden stop in inspiratory effort, sign of acute cholecystitis
Myerson’s sign
persistent blinking with glabellar stuff
Nägele’s rule
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
Nardi test
narcotic-indcued stimulation or spasm reproducing the abdominal pain and amylase elevation of relapsing pancreatitis (for inferring sphincteric disease in any pancreatic or biliary ductal system without a gallbaldder), presumably accurate in the diagnosis of perisphincteric disease
Negri bodies
in rabies virus-infected brain neurons, eosinophlic cytoplasmic inclusions
Nelson’s syndrome
enlargement of a pituitary adenoma in a patient with Cushing’s disease whose adrenals have been removed from loss of feedback inhibition of cortisol
nerve endings
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
nevus of Ota
pigmentation mostly involves the skin and mucous membranes innervated by the first and second branches of the trigeminal nerve
Niemann-Pick disease
unifying feature, lysosomal accumulation of sphingomyelin and cholesterol, type A&B (*deficiency of sphingomyelin-cleaving enzyme sphingomyelinase*) and type C&D (enzyme normal or nearly normal, defect in esterification and transport); type A, 75-80% of all cases, extensive neurologic involvement, marked visceral accumulations of sphingomyelin, and progressive wasting and early death within the first 3 years of life); half have cherry red spot in macula as in Tay-Sachs
Nikolsky’s sign
a peculiar vulnerability of the skin in pemphigus vulgaris; the apparently normal epidermis may be separated at the basal layer and rubbed off when pressed with a sliding motion
Nissl bodies
in neurons, rough ER
Nissl stain
stains cell bodies dye binds to acid groups, in particular the RNAs of the ribosomes located within the cell body; Franz Nissl, German medical student at time
Norwalk virus
outbreak in a school in Norwalk, Ohio in 1969
nucleus of Darkschewitsch
an ovoid cell group in the ventral central gray substance rostral to the oculomotor nucleus, receiving fibers from the vestibular nuclei by way of the medial longitudinal fasiculus; projections are not known, although some cross in the posterior commissure
obturator sign
pain upon internal rotation of the legg with the hip and knee flexed; seen in appendicitis, pelvic abscess
Ogilvie’s syndrome
massive idiopathic non-obstructive dilatation of the colon
Ollier’s disease
syndrome of multiple enchondromas
Ondine’s curse
after a mythological tale in which the suitor of Neptune’s daughter was cursed to lose automatic control over all bodily functions
Oroya fever
from Bartonella bacilliformis, sandfly vector Phlebotomus found in valleys of the Andes mountains, 600-2500 m, profound *intravascular hemolytic* anemia of a few weeks’ duration, associated with lesions called verruga peruana resembling Kaposi’s sarcoma; aka Carrión’s disease
Ortner’s syndrome
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
Ortolani’s sign
in congenital hip dislocatin, rotate hip with patient in supine position and hip abducted; a “clunk” or “click” represents congenitally dislocated hip
Osborn wave
EKG with distinctive convex “hump” at J point associated with hypothermia
Osglood-Schlatter’s disease
epiphysitis of tibial tubercle resulting from repeated powerful contractions of the quadriceps seen in adolescents with open physis
Osler’s nodes
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
Osler’s sign
palpable brachial or radial artery when cuff > systolic pressure
Osler-Weber-Rendu disease
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; bust out aminocaproic acid (an antifibrinolytic agent)
Ouchterlony reaction
double diffusion with antigen and antibody stuff
Paget’s disease of bone
osteitis deformans, characterized by an initial osteolytic stage followed by a mixed osteoclastic-osteoblastic stage, which ends with a predominance of osteoblastic activity and evolves ultimately into a burnt-out quiescent osteosclerotic stage; increased alkaline phosphatase and increased urine hydroxyproline
Pancoast’s tumor
apical lung cancers in the superior pulmonary sulcus tend to invade the neural structures around the trachea, including the cervical sympathetic plexus, and produce a group of clinical findings that include severe pain in the distribution of the ulnar nerve and Horner’s syndrome on the same side of the lesion
Paneth cells
in small intestine crypts, cells with apically oriented bright eosinophilic granules and which appear to play a role in the mucosal immune system
Pappenheimer bodies
small dark blue irregularly shaped granules often in clusters, composed of iron, seen in sideroblastic anemia following splenectomy
Parinaud’s oculoglandular syndrome
preauricular node enlargement associated with chronic granulomatous conjunctivitis
Parinaud’s syndrome
lid retraction caused by tumors in the pineal region; associated with lesions in s. colliculus and pretectal area causing paralysis of upward and downward gaze, pupillary disturbances, and absence of convergence; compression of cerebral aqueduct resulting in noncommunicable hydrocephalus
Parkland formula
total body surface area % burned x kg x 4; _ in first 8 hours, second _ given next 16 hours
Pastia’s sign
associated with scarlet fever (GAS or S. aureus rarely); finely punctate erythema has become confluent (scarlatiniform) on the lower trunk and thighs with petechiae having a linear configuration in the inguinal regions
Patau’s syndrome
trisomy 13, mental retardation, microcephaly, microphthalmia, brain abnormalities, cleft lip and palate, polydactyly, rocker-bottom feet, and congenital heart disease
Paul-Bunnell-Davidsohn test
extension of classic Paul-Bunnell test for heterophil antibody; antibodies not absorbed by guinea pig kidney cells but cause sheep erythrocytes to agglutinate
Pautrier’s microabcesses
q.v. Sézary-Lutzner cells
Pel-Ebstein fever
in Hodgkin’s disease, unusual systemic manifestation of a periodic fever that is present for some days, remits, and then returns
Pelger-Hüet anomaly
seen in blood of AML (e.g., M2) or myelodysplastic syndromes or inherited as autosomal recessive trait and maybe sideroblastic
Pelizeus-Merzbacher disease
mutation in proteolipid protein on X chromosome; results in hypomyelination confined to the CNS, seizures, mental retardation, and death in childhood
pentalogy of Cantrell
Diaphragmatic defect (hernia), Cardiac abnormality, Omphalocele, Pericardium malformation/absence, Sternal cleft
Peutz-Jeghers syndrome
rare AD syndrome characterized by multiple hamartomatous polyps scattered throughout the entire GI tract and melanotic mucosal and cutaneous pigmentation around the lips, oral mucosa, face, genitalia, and palmar surfaces; patients have increased risk of carinomas of pancreas, breast, lung, ovary, and uterus
Peyronie’s disease
penile fibromatosis, a palpable induration or mass appears on the dorsolateral aspect of the penis. It may cause eventually abnormal curvature of the shaft or constriction of the urethra, or both
Pfeiffer syndrome
craniosynostoses as well as limb defects, mutations in FGFR1, broad thumbs, broad great toes
Phalen’s maneuver
median nerve compression, palmar flexion of the wrist for 1 minute exacerbates or reproduces symptoms; 75% sens 47% spec
pheochromactyoma rule of 10s
10% bilateral, 10% malignant, 10% in children, 10% extraadrenal, 10% have multiple tumors
pheochromacytoma triad
1. palpitations 2. headache 3. episodic diaphoresis
Philadelphia chromosome
seen in 95% of chronic myelogenous leukemia (210 kD tyrosine kinase), 2-5% childhood ALL (180 kD TK), higher percent in adult; reciprocal and balanced translocation between chr22 (bcr, breakpoint cluster region) and chr9 (c-abl); c-abl-bcr encodes a chimeric protein with tyrosine kinase activity; genomic imprinting, chr9 paternal and chr22 maternal
Pick’s disease
more frequent in women, characterized by marked cortical atrophy, especially of the temporal and frontal lobes, by swollen neurons, and by Pick bodies, round intracytoplasmic inclusions consisting of neurofilaments
Pierre Robin syndrome
micrognathia and abnormal smallness of the tongue, often with cleft palate, severe myopia, congenital glaucoma, and retinal detachment; French pediatrician, 1867-1950
Pittsburgh pneumonia agent
Legionella micdadei
Plummer’s disease
toxic multinodular goiter
Plummer’s nail
onycholysis as a sign of hyperthyroidism, especially when it affects the ring finger
Plummer-Vinson syndrome
from iron deficiency, a microcytic hypochromic anemia, atrophic glossitis, and esophageal webs (upper esophagus); 10% develop squamous cell carcinoma
Poiseuille’s law
flow proportional to fourth power of radius, inversely proportional to length
Poland syndrome
amastic asociated with hypoplasia of ipsilateral musculature and chest wall
Polle syndrome
children who are abused by being given laxatives
Pompe’s disease
type II glycogen storage disease, deficiency in -1,4-glucosidase (lysosomal enzyme) with consequent accumulation of glycogen, especially in the liver, heart, and skeletal muscle, characterized by cardiomegaly, muscle hypotonia, and splenomegaly, death from cardiorespiratory failure before age 3
pores of Kohn
connections between alveoli
Pott’s disease
tuberculous involvement of the spine
Pott’s fracture
fracture of distal fibula
Potter’s sequence
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
Poupart’s ligament
inguinal ligament
Prader-Willi syndrome
deletion of 15(q11-q13), paternally derived
Prehn's sign
elevation of painful testicle decreases pain of epididymitis
Prinzmetal’s angina
variant angina occurs at rest, manifests on EKG as episodic ST segment elevations, caused by coronary artery spasms with or without superimposed coronary artery disease. patients more likely to develop ventricular arrhythmias
psammoma bodies
papillary thyroid cancer
psoas sign
pain elicited by extending the hip with the knee in full extension, seen with appendicitis and psoas inflammation
Puestow procedure
in chronic pancreatitis, surgical decompression of a dilated main pancreatic duct providing pain relief
Puestow procedure
for chronic pancreatitis, side-to-side anastomosis of the pancreas and jejunum, thereby decompressing dilated main pancreatic duct and providing pain relief
Purtscher’s angiopathic retinopathy
in acute pancreatitis, sudden and severe loss of vision due to posterior retinal artery occlusion with aggregated granulocytes; discrete flame-shaped hemorrhages with cotton-wool spots; also seen in fat embolization; first described in 1919 by Othmar Purtscher
Quellung reaction
swelling of bacterial capsule when exposed to antibody; used for diagnosis of S. pneumoniae, H. influ type B, N. meningitidis groups A and C
Quinicke’s sign
in aortic regurgitation, capillary pulsations detected by pressing a glass slide on the patient’s lip or by transmitting a light through the patient’s fingertips
Rabson-Mendenhall syndrome
congenital syndrome characterized by insulin resistance, acanthosis nigricans, and growth retardation; associated with developmental abnormalities of bones and teeth, PCOD, genitomegaly, and pineal gland hyperplasia; associated with mutation in insulin receptor
raccoon eyes
bilateral black eyes in basilar skull fracture
Ramsay Hunt syndrome
herpes zoster infection of the geniculate ganglion; facial nerve involvement (ear, palate, pharynx, or neck); pain and vesicles appear in external auditory canal, and patients lose their sense of taste in anterior 2/3 of tongue while developing ipsilateral facial palsy
Ranke complex
combination of Ghon lesion and involved lymph nodes in tuberculosis
Rapoport-Luebering shunt
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
Rathke’s pouch
a divertic involved in develoment of pituitary gland, vestigial remnants lead to craniopharyngioma
Rebuck skin window
dermal abrasion technique for testing tissue penetration of neutrophils, scraping forearm, then putting coverslip over it, checking glass for neutrophils
Reed-Sternberg cells
in Hodgkin’s lymphoma, giant macrophage-like cells with two nuclei
refeeding syndrome
hypokalemia, hypomagnesemia, and hypophosphatemia after refeeding a starved patient
Refsum’s disease
AR; phytanic acid accumulates as a result of an absence of the enzyme -phytanic acid -hydroxylase involved in its catabolism; give patient’s large amounts of nicotinic acid or triparanol, chemicals that inhibit lipid synthesis; dryness and scaling similar to the appearance of icthyosis develop
Reichert’s cartilage
branchial arch 2
Reid index
comparing the relative thickness of the mucous glands with the total thickness of the airway wall; increased in patients with chronic bronchitis (normally < 0.4)
Reinke crystals
in 25% of Leydig cell tumors, intracytoplasmic rod-shaped crystalloids
Reinke’s edema
vocal cord polyposis in female smokers, 50s-70s
Reiter’s syndrome
In 1916, Reiter described a triad of arthritis, urethritis, and conjunctivitis; 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
renal columns of Bertin
the spaces between adjacent pyramids where cortical tissue extends into
Renshaw cells
inhibitory cells in the ventral horn of the spinal cord
Reye’s syndrome
rare disease characterized by fatty change in liver and encephalopathy that in its most severe forms may be fatal; associated with VZV and influenza virus B in children given aspirin
Reynold’s pentad
Charcot’s triad plus altered mental status and shock in cholangitis
Richter syndrome
the evolution of chronic lymphocytic leukemia to a large cell lymphoma with high fever, weight loss, enlarging lymph nodes, and hepatosplenomegaly
Riedel’s lobe
in some persons, especially those with a lanky build, the liver tends to be somewhat elongated so that its right lobe is easily palpable as it projects downward toward the iliac crest; such elongation, called Riedel’s lobe
Riedel’s thyroiditis
unknown etiology, marked by glandular atrophy, hypothyroidism, and replacement of the thyroid by fibrous tissue with adhesion to surrounding structures
Riggler’s sign
see wall of bowel in perforation
Riley-Day syndrome
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, impaired pain and temperature sensation, and areflexia
Rinne test
sensorineural loss, AC>BC conduct loss, BC>AC
Ritter’s disease
Staph scaled skin syndrome
Roger’s disease
small congenital VSD defect <0.5 cm in diameter (most are muscular); Henri L. Roger, French physician, 1809-1891
Rokitansky-Aschoff sinuses
small outpouchings of the gallbladder mucosa that may penetrate into and through the muscle wall; prominence in the settings of inflammation and gallstone formation (e.g. chronic cholecystitis) suggests that they are acquired herniations
Romaña’s sign
in Chagas disease, unilateral periorbital edema and swelling of the eyelid associated with reduviid bug of eye
Romberg test
patient stands feet together, eyes open and then closes both eyes for 20 to 30 sec without support; checking for cerebral ataxia; positive test indicative of grossly impaired joint sensation in the legs
Rosenbach’s sign
in thyrotoxicosis, tremor of the closed eyelids
Roth’s spots
in bacterial endocarditis and other retinal hemorrhagic conditions, a round white spot surrounded by hemorrhage (secondary to microemboli in endocarditis)
Rotor’s syndrome
poorly defined defects in hepatic uptake and storage of bilirubin; resembles Dubin-Johnson syndrome, but liver pigment missing
Rotter’s lymph nodes
lymph nodes between the pectoralis minor and pectoralis major
Rovsing’s sign
pain in the right lower quadrant during left-sided pressure suggests appendicitis; so does right lower quadrant pain on quick withdrawal (referred rebound tenderness)
Russel bodies
endoplasmic reticulum of plasma cells engaged in active synthesis of immunoglobulins may become hugely distended, producing large, homogenous eosinophilic inclusions called Russell bodies
RUSSELL’S SIGN
lanugo, dry skin, hand calluses, associated with purging and bulimia
Sabouraud’s agar
for growing fungi, low pH of medium and chloramphenicol and cycloheximide
Saint’s triad
1. cholelithiasis, 2. hiatal hernia, 3. diverticular disease
Salus’s sign
deflection of veins at AV crossings in hypertensive retinopathy (grade 2); c.f. Bonnet’s and Gunn’s sign
Samter’s triad
nasal polyps, bronchial asthma, aspirin sensitivity
Santos’s syndrome
Hirschsprung’s disease with renal agenesis, polydactyly, hypertelorsim, and deafness
Scahmberg’s disease
idiopathic capillaritis in which inflammation weakens capillaries, causes petechial lesions like cayenne pepper
SCHAMROTH’S WINDOW TEST
for testing clubbing
Schatzki’s ring
esophageal rings and webs in lower esophagus, located at or just above the squamocolumnar junction; most common cause of intermittent solid food obstruction
Schaumann’s bodies
laminated concretions composed of calcium and proteins, seen in granulomatous diseases (e.g. sarcoidosis)
Schick’s test
for testing immune status to Cornyebacterium diphtheriae, intradermal injection of 0.1 mL of purified standardized toxin; if no inflammation, antitoxin present
Schilling test
measuring cobalamin absorption by determining the fraction of an orally administered dose of radioactive cobalamin excreted in urine over 24 to 48 hours
Schirmer’s test
measures quantity of tears secreted in 5 minutes in response to irritation from a filter strip placed under each lower eyelid; normal young person moistens 15 mm; 33% of elderly wet only 10 mm in 5 minutes; in Sjögren’s syndrome, <5 mm in 5 min, 85% sensitivity, 85% specificity
Schlesinger’s solution
morphine and scopolamine
Schmidt metaphyseal chondrodysplasia
mutation in collagen X, mechanical pressure reduces growth
Schmidt’s syndrome
type II polyglandular syndrome, coexistent adrenal and thyroid disease, sometimes accompanied by IDDM
Schober test
measures distraction between 2 marks on the skin during forward flexion in ankylosing spondylitis
Schwartz’s dictum
no acid, no ulcer
sclerosing osteomyelitis of Garr
typically develops in the jaw, associated with extensive new bone formation that obscures much of the underlying osseous structure
Scott syndrome
defect in primary homeostasis, prolonged PT, deficient in platelet coagulant activity which provides the phospholipid surface and landing pad for prothrombinase in the presence of calcium
Seligmann’s disease
 heavy chain disease, characterized by infiltration of the lamina propria of the small intestine with lymphoplasmacytoid cells that secrete truncated  chains
Senear-Usher syndrome
pemphigus erythematosus; a localized variety of pemphigus foliaceus confined to seborrheic sites
Sengstaken-Blakemore tube
tube a double-balloon system, one for stomach, one for esophagus, for tamponade of bleeding varices
Sever’s disease
apophysitis of the calcaneus, common (but frequently source of heel pain), condition occurs before or during the peak growth spurt, often resolves two weeks or two months after initiation of conservative treatment
Sézary’s syndrome
rare special variant of cutaneous T-cell lymphoma characterized by generalized or universal erythroderma, peripheral lymphadenopathy, and cellular infiltrates of atypical lymphocytes (Sézary cells) in the skin and blood
Sézary-Lutzner cells
found in cutaneous T-cell lymphoma, T-helper cells that characteristically form band-like aggregates within the superficial dermis and invade the epidermis as single cells and small clusters (Pautrier’s microabcesses)
Sheehan’s syndrome
postpartum pituitary necrosis, syndrome results from sudden infarction of the anterior lobe precipitated by obstetric hemorrhage or shock (pregnancy, pituitary enlarges to almost twice its normal size, compressing blood supply)
Shiraz dwarfism
zinc deficiency, in Iran, short stature and aspermia
Shohl’s solution
alkalinizing solution in hyporeninemic hypoaldosteronism associated with renal tubular acidosis associated with diabetic nephropathy
Shulman’s syndrome
eosinophilic fascitis
Shwartzman reaction
two iv injections of sublethal lipopolysaccharide, 24 hrs apart, causing DIC in rabbits; TNF obligatory mediator
Shy-Drager syndrome
degenerative disorder characterized by parkinsonian features (leading to postural hypotension, anhidrosis, disturbance of sphincter control, impotence, etc.) And signs of more widespread neurologic involvement (pyramidal or lower motor neuron signs and often a cerebellar deficit)
Siegrist streaks
linear hyperpigmented areas over choroidal vessels in hypertensive retinopathy
sign of Cabrera
nochin at 0.05s in ascending limb of S wave in V3, V4; 27% sens for MI
signe de Dance
in intussusception, in the common entercolic variety, almost from the beginning of the illness the right iliac fossa will appear empty on palpation due to the taking up of the cecum into the advancing invagination
silk glove sign
indirect hernia sac in the pediatric patient; the sac feels like a finger of a silk glove when rolled under the examining finger
Simmond’s disease
pituitary cachexia (e.g. from Sheehan’s syndrome)
Sims-Huhner test
post coital test, done 2-4 hrs after intercourse to assess number and motility of sperm that have entered the cervical canal
singers’s nodule
is a small, benign laryngeal polyp, usually induced by chronic irritation, such as excessive use of the voice, and is associated most commonly with heavy cigarette smoking; is usually localized to the true vocal cords
Sipple’s syndrome
MEN type IIa; pheochromacytoma, medullary carcinoma of the thyroid, and hyperparathyroidism due to hyperplasia or tumor
Sister Mary Joseph nodule
abdominal carcinoma, especially gastric, may metastasize to the navel; Sister Joseph, in the early days of the Mayo Clinic, noted periumbilical nodules in patients with intraabdominal cancer
Sjögren’s syndrome
lymphocytic infiltration affects salivary and lacrimal glands and is associated with dry mouth and dry eyes (keratoconjunctivitis sicca); may have either interstitial pulmonary fibrosis or a lymphocytic infiltration of the alveolar walls (may have a malignant transformation with the development of a lymphoma)
Skirrow’s medium
contains vancomycin, trimethoprim, cephalothin, polymixin, and amphotericin B; for growing e.g. Campylobacter
Smith’s fracture
opposite of Colle’s fracture; fracture of the distal radius, but from falling on the dorsum of the hand
Sneddon’s syndrome
livedo reticularis associated with stroke-like episodes
Somogyi phenomenon
rebound hyperglycemia following an episode of hypoglycemia due to counterregulatory hormone release
space of Retzius
the preperitoneal space anterior to the bladder
Spigelian hernia
hernia through the linea semilunaris, aka spontaneous lateral ventral hernia
spiral valves of Heister
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
St. Anthony’s fire
ergotism; disease caused by excess ergot alkaloid; classically an epidemic caused by consumption of grain that’s contaminated by the ergot fungus; any of several inflammations or gangrenous conditions of the skin (erysipelas)
St. Vitus’s dance
q.v. Sydenham’s chorea
Stauffer’s syndrome
elevation of LFTs due to cholestasis in renal cell carcinoma
Steele-Richardson-Olszewski syndrome
aka progressive supranuclear palsy
Stein-Leventhal syndrome
polycystic ovarian disease
Stellwag’s sign
incomplete and infrequent blinking in Graves’s disease
Stevens-Johnson syndrome
extensive and symptomatic febrile form of erythema multiforme, more common in children; 1-6 cases/million person-years
Stewart-Treve syndrome
lymphedema following mastectomy leading to lymphangiosarcoma
Stickler syndrome
mild spondyloepiphyseal dysplasia, osteoarthritis, and sensorineural hearing loss, some forms associated with a dominant negative mutation in the human COL11A2 gene encoding the 2(XI) chain
Still’s murmur
described by George Still in 1909; normal vibratory midsystolic murmur; innocent murmur
Stokes-Adams attacks
fainting spells associated with complete heart block (or other types of bradycardia)
Sturge-Weber syndrome
association of port-wine stain with vascular malformations in the eye (glaucoma) and leptomeninges and superficial calcifications of the brain; attributed to faulty development of certain mesodermal and ectodermal elements, and associated with mental retardation, seizures, hemiplegia, and radiopacities in the skull
Sweet’s syndrome
acute febrile neutrophilic dermatosis or Sweet syndrome, initially described in 1964 by Robert Sweet. It is characterized fever, neutrophilic leucocytosis, abrupt appearance of erythematous, painful, cutaneous plaques and dense dermal infiltrate consisting of mature neutrophils without vasculitis signs.
Sydenham’s chorea
aka St. Vitus’s dance A postinfectious chorea appearing several months after a streptococcal infection with subsequent rheumatic fever. The chorea typically involves the distal limbs and is assoc. with hypotonia and emotional lability. Improvement occurs over weeks or months and exacerbations occur without assoc. infection occurrence.
Sylvian aqueduct
cerebral aqueduct of the midbrain that connects the third and fourth ventricle
Takayasu’s disease
aortic arch syndrome, pulseless disease; panartertis of the great vessels that’s most common in Asian women
Tamm Horsfall protein
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
Tay-Sachs disease
autosomal recessive; GM2 gangliosidosis, results from mutations that affect chr15 and cause a severe deficiency in hexosaminidase A; blindness and cherry-red spot
Terry’s nails
mostly whitish with a distal band of reddish brown; may be seen with aging and in people with chronic diseases such as cirrhosis of the liver, congestive heart failure, and non-insulin-dependent diabetes; seen in 10% of uremics
tetralogy of Fallot
1. ventricular septal defect; 2. infundibular, valvar, or supravalvar pulmonic stenosis; 3. an anteriorly displaced aorta that receives blood from both ventricles; 4. right ventricular hypertrophy
Thompson's test
verifies if gastroc-soleus complex intact; squeeze calf belly, foot should plantar flex
Thomsen’s disease
myotonia congenita
Tietze syndrome
discomfort localized in swelling of the costochondral and costosternal joints, which are painful on palpation; may be perceived as breast pain
Tinel’s sign
a sensation of tingling or pins and needles felt in distal extremity when percussion is made over the site of an injured nerve; it indicates a partial lesion or early regeneration of the nerve; 60% sens, 67% spec
Todd's paralysis
transient hemiparesis in postictal period, suggests focal brain lesion as cause, warrants further investigation
Towne’s view
AP view with the X ray tube angled caudad to show the occipital bone
Traube’s sign
in aortic regurgitation “pistol shot sounds” referring to booming systolic and diastolic sounds over the femoral artery
Traube’s space
a crescentic space about 12 cm wide, bounded medially by the left border of the sternum, above by an oblique line from the 6th costal cartilage to the lower border of the 8th or 9th rib in the mid-axillary line and below by the costal margin; the percussion tone here is normally tympanitic, because of the underlying stomach, but is modified by pulmonary emphysema, a pleural effusion, or an enlarged spleen
Treacher Collins syndrome
first arch syndrome, mandibulofacial dysplasia, caused by AD 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
Trousseau’s sign
in hypocalcemia and latent tetany, carpal spasm induced by occluding the brachial artery for 3 min with an inflated BP cuff
Trousseau’s syndrome
migratory thrombophlebitis, may be encountered with deep-seated cancers, most often with carcinomas of the pancreas or lung
Turcot’s syndrome
rare variant of familial adenomatous polyposis, with combination of adenomatous colonic polyposis and tumors of the CNS, mostly gliblastoma multiforme
Tzanck smear
cytologic technique most often used in the diagnosis of herpesvirus infections (simplex or varicella-zoster); multinucleated giant cells suggest the presence of herpes; named after Arnault Tzanck
unhappy triad
lateral knee injury resulting in ACL tear, MCL tear, and medial meniscal injury
van den Bergh reaction
used to distinguish between unconjugated and conjugated bilirubin; bilirubin pigments are exposed to sulfanilic acid to generate diazo conjugates, forming chromogenic products
van der Woude syndrome
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
van Wyk-Grumbach syndrome
primary hypothyroidism associated with precocious puberty and galactorrhea
van’t Hoff’s law
for calculating osmotic pressure
vein of Mayo
vein overlying pylorus
veins of Retzius
numerous small veins in the retroperitoneum that connect the retroperitoneal viscera to the posterior abdominal wall; dilated in portal hypertension
Vincent’s infection
acute necrotizing ulcerative gingivitis, occurring in patients with decreased resistance to infection, from concurrent infection with the symbiotic bacteria Fusobacterium fusiforme and Borrelia vincentii
Virchow’s node
supraclavicular adenopathy associated with a malignancy, often on left side, associated with stomach cancer among other neoplasms
Virchow’s triad
predisposing factors in thrombus formation 1. endothelial injury, 2. hypercoagulability 3. stasis or turbulence of blood flow
Virchow-Robin spaces
perivascular spaces in brain; become unusually widened in edema of the brain
Volkmann contracture
contraction of forearm flexores because of compartment syndrome sequelae
von Gierke’s disease
glycogen storage disease type I, deficiency in glucose-6-phosphatase, results in hepatomegaly and hypoglycemia
von Graefe sign
in Graves’s disease, lag of the upper eyelid as it follows the rotation of the eyeball downward
von Hippel-Lindau disease
hemangioblastoma or cavernous hemangioma of the cerebellum, brain stem, or retina, adenomas, and cysts of the liver, kidney, pancreas, and other organs, 35% develop renal cell carcinoma, 3p
von Myenburg complexes
close to or within portal tracts, these are small clusters of modestly dilated bile ducts embedded in a fibrous, sometimes hyalinized stroma; these bile duct “microhamartomas” contain inspissated bile concrements and may communicate with the biliary tree; rather common and usually without clinical significance
von Recklinghausen’s disease
neurofibromatosis
von Recklinghausen’s disease of bone
generalized osteitis fibrosa cystica; hallmark of severe hyperparathyroidism, including increased bone cell activity, peritrabecular fibrosis, and cystic brown tumors
von Willebrand’s disease
deficiency in von Willebrand factor
von Zumbusch psoriasis
aka generalized acute pustular psoriasis
Waardenburg’s syndrome
Hirschprung’s disease characterized by deafness, white forelock, abnormal pigmentation (maybe heterochromia) due to developmental defect caused by defective neural crest migration; mutation in PAX3 gene or endothelin-B-receptor gene
Waldenström’s macroglobulinemia
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; hyper viscosity
Waldeyer’s throat ring
the broken ring of lymphoid tissue, formed of the lingual, facial, and pharyngeal tonsils, commonly involved in non-Hodgkin’s lymphoma and rarely in Hodgkin’s disease
Wallenberg syndrome
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 inpsilateral 4. inferior cerebellar peduncle, ipsilateral limb ataxia 5. anterolateral system, reduced pain and temperature on contraleral 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
Warburg effect
in malignant transformation, increased anaerobic glycolysis leads to increased lactic acid production
Warthin’s tumor
papillary cystadenoma lymphomatosum, parotid gland involved, benign, more in males than females, 50s-70s
Warthin-Finkeldey cells
in measles pneumonia, cells with multiple nuclei and eosinophilic intranuclear inclusions
Warthin-Starry stain
a silver stain, will stain H. pylori, Bartonella henselae
Waterhouse-Friderichsen syndrome
with N. meningitidis or gonococci, pneumococci, or Staph.: a form of septicemia characterized by hypotension leading to shock; DIC with widespread purpura, adrenocortical insufficiency associated with massive bilateral adrenal hemorrhage
Weber syndrome
medial midbrain syndrome
Weber test
sensorineural loss, sound from normal ear conduct loss, sound from affected ear
Weber-Christian disease
relapsing febrile nodular nonsuppurative nonvasculitic panniculitis (an inflammatory reaction in the subcutaneous fat)
Wegener’s granulomatosis
systemic vasculitis, presumed autoimmune, defined by a clinical triad of manifestations that includes involvement of the upper airways, lungs, and kidneys and by a pathological triad consisting of necrotizing granuloma in the upper respiratory tract and lungs, vasculitis involving both arteries and veins, and focal glomerulonephritis; untreated, mean survival 5 months, 1 year mortality 82%; treat with Bactrim; 1) pulm hemorrhages, 2) iron def anemia, 3) glomerulonephritis
Weibel-Palade bodies
found only in endothelial cells of vessels larger than capillaries; granules contain von Willebrand’s factor (VIII) and P-selectin
Weigert stain
iron hematoxylin, preceded by a dichromate mordant, stains myelin
Weil-Felix reaction
Proteus cell wall O antigens, such as OX-2, OX-19, and OX-K, cross-reacting with antigens of several species of rickettsiae
Well’s syndrome
eosinophilic cellulitis, characterized by recurrent cutaneous swellings which resemble acute bacterial cellulitis, and by distinctive histopathological changes. Skin lesions show dermal eosinophilic infiltration and the characteristic “flame figures” are composed of eosinophil major protein deposited on collagen bundles
Werdnig-Hoffman syndrome
infantile progressive spinal muscular atrophy autosomal recessive lower motor neuron disease that manifests clinically in infancy
Wermer’s syndrome
MEN type I, hyperplasias or tumors of the thyroid, parathyroid, adrenal cortex, pancreatic islets, or pituitary
Werner’s syndrome
A disorder consisting of scleroderma-like skin changes, bilateral juvenile cataracts, progeria, hypogonadism, and diabetes mellitus; autosomal recessive inheritance
Wernicke’s area
important cortical center for recognizing speech, found in the superior temporal gyrus
Wernicke’s encephalopathy
chronic alcoholics, thiamine deficiency (alcohol impairs thiamine absorption) ataxia, global confusion, ophthalmoplegia, and often nystagmus; sometimes, thiamine deficiency, can lead to a particular focal necrotizing encephalopathy affecting the hypothalamus, medial thalamus, and oculomotor nuclear groups in the periventricular brainstem; can be *precipitated by the administration of glucose* to patient depleted of thiamine; after treatment with thiamine, a minority of patients have profound memory deficit  Korsakoff’s syndrome
Westermark’s sign
in chest film, an abrupt tapering of a vessel caused by pulmonary embolism, focal oligemia
Wharton’s duct
submandibular duct
Whipple procedure
pancreaticoduodenectomy with cholecystectomy, truncal vagotomy, choledochojejunostomy, pancreaticojejunostomy, gastrojejunostomy
Whipple’s disease
small intestinal mucosa laden with distended macrophages in the lamina propria, PAS positive granules, with no inflammation; gram+ actinomycete Tropheryma whippelii
Whipple’s triad
in insulinoma, 1) attacks precipitated by fasting or exertion 2) fasting blood glucose <50 mg/dL 3) sx relieved by glucose administration
white lines of Toldt
the peritoneal reflections of the ascending and descending colon
Wickham’s striae
in lichen planus, papules are highlighted by a shiny surface with a lacy white pattern
Widal test
in salmonellosis, rise in antibody titer in patient’s serum
Williams’s syndrome
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
Wilms’s tumor
WT-1, cancer suppressor gene on 11p13
Wilson’s disease
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
Wintrobe indices
mean cell volume; mean cell hemoglobin; mean cell hemoglobin concentration
Wiskott-Aldrich syndrome
X-linked characterized by triad of eczema, thrombocytopenia (from autoantibodies), and repeated infections; failure to express sialic acid-rich glycoprotein, sialophorin or CD15, ending in early death
Wolff-Chaikoff effect
when increasing doses of iodide inhibit organification and hormonogenesis of thyroid hormone
Wolff-Parkinson-White triad
1) wide QRS complex, 2) relatively short PR interval, 3) slurring of initial part of QRS delta wave
Wright's maneuver
looking for thoracic outlet obstruction: evaluating the radial pulse at the wrist with the shoulder in external rotation and abduction, positive sign if it reproduces shoulder and arm symptoms and obliterates radial pulse
Zenker’s diverticulum
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
Zollinger-Ellison syndrome
hallmark, circulating hypergastrinemia; gastric acid hypersecretion and severe peptic ulcer diathesis secondary to unbridled release of gastrin from a gastrinoma; associated with peptic ulcers and diarrhea; 60% malignant, only 20% resectable; 25% of gastrinoma patients have MEN I; >80% of gastrinomas found in gastrinoma triangle
Antianginal therapy
p.319
Name four determinants of the level of myocardial oxygen consumption
There are five: end diastolic volume, blood pressure, heart rate, contractility, ejection time
Do nitrates affect preload or afterload?
preload
Do Beta-blockers affect preload or afterload?
afterload
What is the effect of nitrates on: diastolic volume, blood pressure, contractility, heart rate, ejection time?
decrease EDV, decrease BP, increase contractility (reflex response), increase HR (reflex response), decrease ejection time
What is the effect of Beta-blockers on: diastolic volume, blood pressure, contractility, heart rate, ejection time?
increase EDV, decrease BP, decrease contractility, decreased HR, increase ejection time
The effects of using nitrates and Beta-blockers together will: a) decrease myocardial oxygen demands by the same amount as using either alone, b) decrease myocardial oxygen demands by an amount greater than if each were used alone, or c) have no effect on myocardial oxygen demand
b) Decrease myocardial oxygen demands by an amount greater that if each were used alone
Nifedipine blocks -- channels
calcium
In its effects on myocardial oxygen consumption, is Nifedipine similar to Nitrates or B-blockers?
Nitrates (Nifedipine is similar to Nitrates)
In its effects on myocardial oxygen consumption, is Verapamil similar to Nitrates or B-blockers?
B-blockers
Nitroglycerine, isosorbide dinitrate
p.319
Dose nitroglycerin dilate arteries or veins more?
Veins&gt;&gt;arteries
Does nitroglycerin increase or decrease cGMP in smooth muscle?
Increase
In industrial exposure to nitroglycerine, weekend withdrawal is characterized by which three symptoms?
Tachycardia, dizziness , and headache (&quot;Monday disease&quot;)
Toxic dosage of nitroglycerine causes which three symptoms?
Tachycardia, hypotension, headache
Cardiac drugs: sites of action
p.320
Digitalis has its action on which cell membrane transporter?
Na/K ATPase
Ryanodine has its action on which channel?
Calcium release channel in the sarcoplasmic receptor
Calcium enters cardiac cells through which channel?
Voltage-gated calcium channel
Cytoplasmic calcium concentrations in cardiac cells can be decreased by sequestering calcium in the sarcoplasmic reticulum. Calcium enters the SR through which transporter?
Calcium pump in the wall of the SR
Calcium channel blockers have their effect on which calcium transporters?
Voltage-gated calcium channel
Parkinson's dz
Nigrostriatal dopamine depletion
Perosteal elevation on x-ray
pyogenic osteomylitis
Putz-Jeghers syndrome
benign polyposis
Peyronie's dz
Penile fibrosis
Myosite disarray
Hypertrophic
Cardiomyopathy
Wdfaf
Afdaf
In the ventricular action potential, what causes the Phase 0 rapid upswing?
opening of voltage gated Na channels
In the phase 2 plateau, Ca++ influx triggers Ca++ release from the SR causing ….
myocyte contraction
What effect does Ach have on HR?
decrease
What effect do catecholamines have on HR
increase
Name 4 things that increase contractility:
catecholamines, digitalis, an increase in intracellular Ca++, or a decrease in extracellular Na+
What effect does increased afterload have on myocardial oxygen demand?
increased
how do catecholamines increase contractility?
increase the activity of Ca++ pump in the SR
What effect will and MI have on contractile state of the heart?
decrease
what does S1 correspond to?
mitral and tricuspid valve closure
what does S2 correspond to?
aortic and pulmonary valve closure
what does S3 correspond to?
the end of rapid ventricular filling
what is S3 associated with?
dilated CHF
what is S4 and what does it indicate?
an atrial kick associated with a hyptertrophic ventricle (high atrial pressure/stiff ventricle)
what is the a wave?
atrial contraction
what is the c wave?
RV contraction when the tricuspid valve bulges into atrium
what is the v wave?
increase in atrial pressure due to filling against a closed tricuspid valve
what is physiologic S2 splitting
aortic valve closes just before the pulmonic - the difference is increased on inspiration
what is paradoxical splitting
when the split is heard on expiration instead of inspiration … associated with aortic stenosis
what is meant by "HIZ" shrinkage
the H, I, and Z band decrease in length on muscle contraction, the A band (myosin) stays the same length
describe the pathway of smooth muscle contraction
ap - > sm muslce depolarization - > open Ca++ channels -> increase in cytosol Ca++ -> Ca++ bind Calmodulin -> activates MLCK -> relaxation ->MLCP forms a cross bridge and contraction by binding myosin to actin - > MLCK relaxes … cycle continues while Ca is present.
in an EKG, what does the P wave represent?
atrial depolarization
in an EKG, what does the PR segment represent?
conduction delay through the AV (200 msec)
in an EKG, what does the QRS complex represent?
ventricular depolarization(120 msec)
in an EKG, what does the QT interval represent?
mechanical contraction of the ventricle
in and EKG, what does the T wave represent?
ventricular repolarization
do you see atrial repolarization on an EKG?
no, it is masked by the QRS complex
in an EKG, what does the ST segment represent?
isoelectric, ventricles are depolarized
on an EKG, what is a U wave?
caused by hypokalemia
what is Wolff-Parkinson-White syndrome
when an accessory conduction pathway exists from atria to ventricle (bundle of Kent) - bypasses the AV node and ventricles partially depoliarize earlier giving rise to a delta wave. WPW syndrome may lead to recurrent entry and SVTs.
What do you see on and EKG with atrial fibrillation?
chaotic erratic baseline with no disrete P waves in between iregularly spaced QRS complexes
What do you see on an EKG with atrial flutter?
sawtooth, identical back to back P waves
How many kinds of AV block are there?
4: 1st degree, mobitz I, mobitz II, complete
What is 1st degree AV block?
asymptomatic - PR interval longer than 200 msec
What is mobitz II AV block?
driooed beats not proceeded by a progressively lengthening PR interval. Pathological … can progress to complete block
What is mobitz I AV block?
progressive PR lengthening until a beat is dropped … usually asymptomatic
what is a 3rd degree AV block
complete AV block, atria and ventricles beat independently … rx with pacemaker
what is v-fib
completely erradic rhythm with no identifiable waves, fatal without immediate defibrillation
what is the normal right atrial pressure?
5
what is the normal left atrial pressure
12 (approx with pcwp - swan-ganz)
what is the normal right ventricular blood pressure?
25/5
what is the normal pulmonary artery pressure?
25/10
what is the normal left ventricular pressure?
130/10
what is a normal aortic blood pressure?
130/90
of the total body weight, what proportion is blood?
8% … the rest is body fluids and tissues
of the total body blood, what proportion is plasma?
55% plasma … the rest is hematocrit.
what is serum?
plasma without clotting factors
what is the makeup of plasma?
91.5% water, 7% protein, salts, lipids, enzymes, and vitamins
what is the makeup of plasma proteins?
55% albumin, 38% globulin, 7% fibrinogen
what are the three kinds of cells in hematocrit?
erythrocytes, leukocytes, and platelets
what is the typical makeup of WBCs in the blood?
40-70% PMNS, 20-40% lymphocytes, 2-10% monocytes, 1-6% eosinophils, <1% basophils
what is the net filtration pressure
Pnet=[(Pc-Pi)-(πc-πi)]
what are 4 common causes edema?
increased capillary pressure, decreased plasma proteins, increased capillary permeability, or increased interstitial colloid pressure
what is a common cause of increased capillary pressure
heart failure
what is a common cause of decreased plasma proteins
nephrotic syndrome or liver failure
what may cause increased capillary permeability?
toxins, infections, burns
what could cause increased colloid presure?
lymphatic blockage
what are the three structures in the carotid sheath?
internal jugular v. (lateral), common carotid a. (medial), vagus n. (posterior)
in most cases, what supplies the SA and AV nodes?
RCA
80% of the time, what supplies the inferior portion of the LV via the PD artery?
RCA (right dominant)
when do the coronary arteries fill?
during diastole
what supplies the anterior interventricular septum?
LAD
what is the most posterior part of the heart? enlargment can cause what?
left atrium; dysphagia
what artery gives off large obtuse marginal branches that supply the lateral and posterior walls of the LV?
circumflex
what supplies the anterior 2/3 of IV septum, apical portion of anterior papillary muscle, and the anterior surface of the LV?
LAD
where does the RCA travel?
in the right AV groove
what supplies the inferior and posterior wals of the ventricles and the posterior 1/3 of the IV septum?
posterior descending (typically a branch of RCA)
Cardiac output = ?
stroke volume x heart rate
what is the Fick principle?
CO = rate of O2 consumption/ (arterial O2 content - venous O2 content)
how do you calculate mean arterial pressure?
CO x TPR = 1/3SBP + 2/3 DBP
pulse pressure = ?
systolic - diastolic (approx SV)
stroke volume = ?
CO/HR = EDV-ESV
what variables affect stroke volume?
contractility, afterload, and preload - SV CAP
what effect does increased intracellular calcium have on contractility and SV?
increases contractility and SV
how does decreased extracellular sodium affect contractility and SV?
increases contractility and SV
5 things that decrease contractility and SV?
B1 block, heart failure, acidosis, hypoxea/hypercapnea, Ca++ channel blockers
what effect does digitalis have on contractility and SV?
increases them
preload is equal to what volume?
end diastolic volume
afterload is equal to what presure?
systolic arterial pressure (proportional to peripheral resistance)
what effect do venous dilators (e.g. nitroglycerin) have?
decrease preload
what effect do vasodilators (e.g. hydralazine) have?
decrease afterload
this increases slightly with exercise, increased blood volume (overtransfusion), and excitement (sympathetics)
preload
ejection fraction = ?
SV/EDV = (EDV-ESV)/EDV = index of contractility
ejection fraction is normally greater than or equal to?
55%
resistance is directly proportional to what?
viscosity
resistance is inversely proportional to what?
radius to the 4th power
delta P = ?
Q x R
resistance = ?
[delta P/Q]/[8xviscosityxlengthxr to the 4th]
what does viscosity mostly depend on? when is it increased?
hematocrit: incrased in polycythemia, hyperproteinemic states (e.g. multiple myeloma), hereditary spherocytosis
when in the cardiac cycle is O2 consumption highest?
isovolumetric contraction
this is the period between mitral valve closing and aortic valve opening
isovolumetric contraction
this is the period between aortic valve opening and closing
systolic ejection
this is the period between aortic valve closing and mitral vale opening
isovolumetric relaxation
when does rapid filling occur?
just after mitral valve opens
when does slow filling occur?
just before mitral valve closes
when is JVD seen?
right heart failure
what is cardiac muscle contraction dependent on?
extracellular calcium, which enters the cells during plateau of action potential and stimulates calcium release from the sarcoplasmic reticulum (calcium-induced calcium release)
what is the plateau in the cardiac muscle action potential due to?
calcium influx
where does the pacemaker action potential occur?
SA and AV nodes
what accounts for the automaticity of the SA and AV nodes?
slow diastolic depolarization - membrane potential spontaneously depolarizes as Na+ conductance increases (funny channel)
what determines heart rate?
slope of phase 4 in the SA node
what are the peripheral chemoreceptors? what do they respond to?
carotid and aortic bodies - they respond to decreased (less than 60 mmHg)/increased PCO2, decreased pH of blood
what do central chemoreceptors respond to?
changes in pH and PCO2 of brain interstitial fluid, which in turn are influenced by arterial CO2
what does the aortic arch respond to? how does it transmit and to where?
responds to increased BP; transmits via vagus to medulla
how and to where does the carotid sinus transmit information?
via glossopharyngeal to the medulla
how do the central chemoreceptors respond to increased intracranial pressure?
hypertension (sympathetic response) and bradycardia (parasympathetic response)
what organ receives the larges share of systemic cardiac output?
liver
what organ receivest eh highest blood flow per gramof tissue?
kidney
how does the heart meet increased O2 demand?
incrased coronary blood flow, not by increased extraction of O2
hypoxia in the pulmonary vasculature causes what?
vasoconstriction (unique because in other organs hypoxia causes vasodilation)
what are the local factors in the heart that determine autoregulaton?
O2, adenosine, NO
what are the local factors in the brain that determine autoregulation?
CO2 (pH)
how do the kidneys determine autoregulation?
myogenic and tuboglomerular feedback
what local factors determine autoregulation in skeletal muscle?
lactate, adenosine, K+
what is the most important mechanism for determining autoregulation in the skin?
sympathetic stimulation - temperature control
what is the effect of carotid massage?
incrases pressure on carotid artery - increasing stretch and leading to decreased HR
what is the ultimate consequence of baroreceptors sensing hypotension?
vasoconstriction, incrased HR, contractility, and BP - important in the response to severe hemorrhage
sound of mitral and tricuspid valve closure
S1
In the ventricular action potential, what causes the Phase 0 rapid upswing?
opening of voltage gated Na channels
In the phase 2 plateau, Ca++ influx triggers Ca++ release from the SR causing ….
myocyte contraction
What effect does Ach have on HR?
decrease
What effect do catecholamines have on HR
increase
Name 4 things that increase contractility:
catecholamines, digitalis, an increase in intracellular Ca++, or a decrease in extracellular Na+
What effect does increased afterload have on myocardial oxygen demand?
increased
how do catecholamines increase contractility?
increase the activity of Ca++ pump in the SR
What effect will and MI have on contractile state of the heart?
decrease
what does S1 correspond to?
mitral and tricuspid valve closure
what does S2 correspond to?
aortic and pulmonary valve closure
what does S3 correspond to?
the end of rapid ventricular filling
what is S3 associated with?
dilated CHF
what is S4 and what does it indicate?
an atrial kick associated with a hyptertrophic ventricle (high atrial pressure/stiff ventricle)
what is the a wave?
atrial contraction
what is the c wave?
RV contraction when the tricuspid valve bulges into atrium
what is the v wave?
increase in atrial pressure due to filling against a closed tricuspid valve
what is physiologic S2 splitting
aortic valve closes just before the pulmonic - the difference is increased on inspiration
what is paradoxical splitting
when the split is heard on expiration instead of inspiration … associated with aortic stenosis
what is meant by "HIZ" shrinkage
the H, I, and Z band decrease in length on muscle contraction, the A band (myosin) stays the same length
describe the pathway of smooth muscle contraction
ap - > sm muslce depolarization - > open Ca++ channels -> increase in cytosol Ca++ -> Ca++ bind Calmodulin -> activates MLCK -> relaxation ->MLCP forms a cross bridge and contraction by binding myosin to actin - > MLCK relaxes … cycle continues while Ca is present.
in an EKG, what does the P wave represent?
atrial depolarization
in an EKG, what does the PR segment represent?
conduction delay through the AV (200 msec)
in an EKG, what does the QRS complex represent?
ventricular depolarization(120 msec)
in an EKG, what does the QT interval represent?
mechanical contraction of the ventricle
in and EKG, what does the T wave represent?
ventricular repolarization
do you see atrial repolarization on an EKG?
no, it is masked by the QRS complex
in an EKG, what does the ST segment represent?
isoelectric, ventricles are depolarized
on an EKG, what is a U wave?
caused by hypokalemia
what is Wolff-Parkinson-White syndrome
when an accessory conduction pathway exists from atria to ventricle (bundle of Kent) - bypasses the AV node and ventricles partially depoliarize earlier giving rise to a delta wave. WPW syndrome may lead to recurrent entry and SVTs.
What do you see on and EKG with atrial fibrillation?
chaotic erratic baseline with no disrete P waves in between iregularly spaced QRS complexes
What do you see on an EKG with atrial flutter?
sawtooth, identical back to back P waves
How many kinds of AV block are there?
4: 1st degree, mobitz I, mobitz II, complete
What is 1st degree AV block?
asymptomatic - PR interval longer than 200 msec
What is mobitz II AV block?
driooed beats not proceeded by a progressively lengthening PR interval. Pathological … can progress to complete block
What is mobitz I AV block?
progressive PR lengthening until a beat is dropped … usually asymptomatic
what is a 3rd degree AV block
complete AV block, atria and ventricles beat independently … rx with pacemaker
what is v-fib
completely erradic rhythm with no identifiable waves, fatal without immediate defibrillation
what is the normal right atrial pressure?
5
what is the normal left atrial pressure
12 (approx with pcwp - swan-ganz)
what is the normal right ventricular blood pressure?
25/5
what is the normal pulmonary artery pressure?
25/10
what is the normal left ventricular pressure?
130/10
what is a normal aortic blood pressure?
130/90
of the total body weight, what proportion is blood?
8% … the rest is body fluids and tissues
of the total body blood, what proportion is plasma?
55% plasma … the rest is hematocrit.
what is serum?
plasma without clotting factors
what is the makeup of plasma?
91.5% water, 7% protein, salts, lipids, enzymes, and vitamins
what is the makeup of plasma proteins?
55% albumin, 38% globulin, 7% fibrinogen
what are the three kinds of cells in hematocrit?
erythrocytes, leukocytes, and platelets
what is the typical makeup of WBCs in the blood?
40-70% PMNS, 20-40% lymphocytes, 2-10% monocytes, 1-6% eosinophils, <1% basophils
what is the net filtration pressure
Pnet=[(Pc-Pi)-(πc-πi)]
what are 4 common causes edema?
increased capillary pressure, decreased plasma proteins, increased capillary permeability, or increased interstitial colloid pressure
what is a common cause of increased capillary pressure
heart failure
what is a common cause of decreased plasma proteins
nephrotic syndrome or liver failure
what may cause increased capillary permeability?
toxins, infections, burns
what could cause increased colloid presure?
lymphatic blockage
what are the three structures in the carotid sheath?
internal jugular v. (lateral), common carotid a. (medial), vagus n. (posterior)
in most cases, what supplies the SA and AV nodes?
RCA
80% of the time, what supplies the inferior portion of the LV via the PD artery?
RCA (right dominant)
when do the coronary arteries fill?
during diastole
what supplies the anterior interventricular septum?
LAD
what is the most posterior part of the heart? enlargment can cause what?
left atrium; dysphagia
what artery gives off large obtuse marginal branches that supply the lateral and posterior walls of the LV?
circumflex
what supplies the anterior 2/3 of IV septum, apical portion of anterior papillary muscle, and the anterior surface of the LV?
LAD
where does the RCA travel?
in the right AV groove
what supplies the inferior and posterior wals of the ventricles and the posterior 1/3 of the IV septum?
posterior descending (typically a branch of RCA)
Cardiac output = ?
stroke volume x heart rate
what is the Fick principle?
CO = rate of O2 consumption/ (arterial O2 content - venous O2 content)
how do you calculate mean arterial pressure?
CO x TPR = 1/3SBP + 2/3 DBP
pulse pressure = ?
systolic - diastolic (approx SV)
stroke volume = ?
CO/HR = EDV-ESV
what variables affect stroke volume?
contractility, afterload, and preload - SV CAP
what effect does increased intracellular calcium have on contractility and SV?
increases contractility and SV
how does decreased extracellular sodium affect contractility and SV?
increases contractility and SV
5 things that decrease contractility and SV?
B1 block, heart failure, acidosis, hypoxea/hypercapnea, Ca++ channel blockers
what effect does digitalis have on contractility and SV?
increases them
preload is equal to what volume?
end diastolic volume
afterload is equal to what presure?
systolic arterial pressure (proportional to peripheral resistance)
what effect do venous dilators (e.g. nitroglycerin) have?
decrease preload
what effect do vasodilators (e.g. hydralazine) have?
decrease afterload
this increases slightly with exercise, increased blood volume (overtransfusion), and excitement (sympathetics)
preload
ejection fraction = ?
SV/EDV = (EDV-ESV)/EDV = index of contractility
ejection fraction is normally greater than or equal to?
55%
resistance is directly proportional to what?
viscosity
resistance is inversely proportional to what?
radius to the 4th power
delta P = ?
Q x R
resistance = ?
[delta P/Q]/[8xviscosityxlengthxr to the 4th]
what does viscosity mostly depend on? when is it increased?
hematocrit: incrased in polycythemia, hyperproteinemic states (e.g. multiple myeloma), hereditary spherocytosis
when in the cardiac cycle is O2 consumption highest?
isovolumetric contraction
this is the period between mitral valve closing and aortic valve opening
isovolumetric contraction
this is the period between aortic valve opening and closing
systolic ejection
this is the period between aortic valve closing and mitral vale opening
isovolumetric relaxation
when does rapid filling occur?
just after mitral valve opens
when does slow filling occur?
just before mitral valve closes
when is JVD seen?
right heart failure
what is cardiac muscle contraction dependent on?
extracellular calcium, which enters the cells during plateau of action potential and stimulates calcium release from the sarcoplasmic reticulum (calcium-induced calcium release)
what is the plateau in the cardiac muscle action potential due to?
calcium influx
where does the pacemaker action potential occur?
SA and AV nodes
what accounts for the automaticity of the SA and AV nodes?
slow diastolic depolarization - membrane potential spontaneously depolarizes as Na+ conductance increases (funny channel)
what determines heart rate?
slope of phase 4 in the SA node
what are the peripheral chemoreceptors? what do they respond to?
carotid and aortic bodies - they respond to decreased (less than 60 mmHg)/increased PCO2, decreased pH of blood
what do central chemoreceptors respond to?
changes in pH and PCO2 of brain interstitial fluid, which in turn are influenced by arterial CO2
what does the aortic arch respond to? how does it transmit and to where?
responds to increased BP; transmits via vagus to medulla
how and to where does the carotid sinus transmit information?
via glossopharyngeal to the medulla
how do the central chemoreceptors respond to increased intracranial pressure?
hypertension (sympathetic response) and bradycardia (parasympathetic response)
what organ receives the larges share of systemic cardiac output?
liver
what organ receivest eh highest blood flow per gramof tissue?
kidney
how does the heart meet increased O2 demand?
incrased coronary blood flow, not by increased extraction of O2
hypoxia in the pulmonary vasculature causes what?
vasoconstriction (unique because in other organs hypoxia causes vasodilation)
what are the local factors in the heart that determine autoregulaton?
O2, adenosine, NO
what are the local factors in the brain that determine autoregulation?
CO2 (pH)
how do the kidneys determine autoregulation?
myogenic and tuboglomerular feedback
what local factors determine autoregulation in skeletal muscle?
lactate, adenosine, K+
what is the most important mechanism for determining autoregulation in the skin?
sympathetic stimulation - temperature control
what is the effect of carotid massage?
incrases pressure on carotid artery - increasing stretch and leading to decreased HR
what is the ultimate consequence of baroreceptors sensing hypotension?
vasoconstriction, incrased HR, contractility, and BP - important in the response to severe hemorrhage
sound of mitral and tricuspid valve closure
S1
2. Which hypothalamic nucleus is involved in ovulation?
Arcuate Nucleus: generates GnRH → FSH, LH
6. What cells of the corpus luteum secrete progesterone and estrogen?
theca lutein cells
(formerly called theca interna cells prior to ovulation)
granulosa lutein cells
(formerly called granulosa cells prior to ovulation)
(granulosa lutein cells and theca lutein cells are collectively called luteal (lutein) cells)
7. What is the 2 cell theory of estridiol production?
Theca cell stimulated by LH
Cholesterol → androstendione (desmolase)
Androstendione → granulose cell
Granulosa cell stimulated by FSH
Androstendione → estrogen (aromatase)
What uterine pathology matches the following description: • excess unopposed estrogen is the main risk factor
Endometrial hyperplasia and carcinoma
• What uterine pathology matches the following description: What uterine pathology matches the following description: menorrhagia with an enlarged uterus and no pelvic pain
Leiomyoma
• What uterine pathology matches the following description: pelvic pain that is present only during menstruation
Endometriosis
• What uterine pathology matches the following description: diagnosed by endometrial biopsy in clinic
Endometrial hyperplasian and carcinoma
• What uterine pathology matches the following description: definitive diagnosis and treatment is by laproscopy
Endometriosis
• What uterine pathology matches the following description: menstruating tissue within the myometrium
Adenomyosis
What uterine pathology matches the following description: • malignant tumor of the uterine smooth muscle
Leiomyomasarcoma
• What uterine pathology matches the following description: most common gynecologic malignancy
Endometrial carcinoma
13. What are the 4 main categories of ovarian tumors? (FA p448 - FA p449)
epithelial (65% of ovarian tumors, 90% of ovarian cancers)
germ cell
stromal
metastatic (GI, breast, endometrium)
What are the main types of epithelial cell ovarian tumors (which account for 65% of ovarian tumors and 90% of ovarian cancers)?
serous,
mucinous,
endometroid,
clear cell,
Brenner,
mixed
(hint: MY Med Students Consistently Beat Exams
What are the main types of germ cell ovarian tumors?
teratoma,
dysgerminoma,
endodermal sinus,
choriocarcinoma
What are the main types of stromal/sex cord ovarian tumors?
granulosa-theca cell,
Sertoli-Leydig cell,
fibroma
• What ovarian tumor matches the following statement: produces AFP
Yolk sac
• What ovarian tumor matches the following statement: estrogen secreting → precocious puberty
Granulosa theca cell tumor
• What ovarian tumor matches the following statement: intraperitoneal accumulation of mucinous material
Mucinous cyst andenocarcinoma
• testosterone secreting → virilization
Leydig cell tumor
• What ovarian tumor matches the following statement: psammoma bodies
Serouscyst adenocarcinoma
• multiple different tissue types
Teratoma
• What ovarian tumor matches the following statement: lined with fallopian tube-like epithelium
Serous cyst adenocarcinoma
• What ovarian tumor matches the following statement:ovarian tumor + ascites + pleural effusions
Fibromas
• What ovarian tumor matches the following statement: Call-Exner bodies
Granulos cell tumor
• What ovarian tumor matches the following statement:resembles bladder epithelium
Brenner tumors looks like bladder
• What ovarian tumor matches the following statement:elevated hCG
Choriocarcinoma or disgerminoma
15. HYQ (FA p438): obese woman presents with hirsutism and increased levels of serum testosterone what is the diagnosis
PCOS
16. HYQ: pt with polycystic ovarian disease is most at risk for developing which type of CA? ~
Endometrial carcinoma
18. HYQ: under what circumstances would you expect to see an elevated LH? (3)
Right before ovulation and LH surge
PCOS
Menopause
What breast pathology fits the following description: most common breast tumor in women under 25
Fibroadenoma
What breast pathology fits the following description: most common breast mass in postmenopausal women
Invasive ductal carcinoma
What breast pathology fits the following description: most common breast mass in premenopausal women
Fibrocystic disease
What breast pathology fits the following description: most common form of breast cancer
Infiltrating ductal carcinoma
What breast pathology fits the following description: small, mobile, firm mass with sharp edges in 24 yr old woman
Fibroadenoma
What breast pathology fits the following description: histological "Ieaflike projections"
Phylloides tumor
What breast pathology fits the following description: signet ring cells
LCIS
What breast pathology fits the following description: loss of e-cadherin cell adhesion gene on chrom 16
Invasive lobular carcinoma
What breast pathology fits the following description: always ER(+) and PR(+)
LCIS
What breast pathology fits the following description: commonly presents with nipple discharge
Intraductal papilloma
What breast pathology fits the following description: eczematous patches on nipple
Paget’s
What breast pathology fits the following description: multiple bilateral fluid-filled lesions with diffuse breast pain
Fibrocystic change
What breast pathology fits the following description: firm, fibrous mass in a 55 yr old woman
Invasive ductal carcinoma
3. What are the pros and cons of oral contraceptive pill use? (FA p453)
Cons: increase triglycerides, increase hypercoagulable state
• State whether the following statement described estrogen or progesterone: production of thick mucus that inhibits entry of sperm into the uterus
Progesterone
• State whether the following statement described estrogen or progesterone: uterine smooth muscle relaxation
Progesterone
• State whether the following statement described estrogen or progesterone: follicle growth
Estrogen
• State whether the following statement described estrogen or progesterone: maintenance of pregnancy
Progesterone
• State whether the following statement described estrogen or progesterone: hepatic synthesis of transport proteins
Estrogen
• State whether the following statement described estrogen or progesterone: withdrawal leads to menstruation
Progesterone
6. When does beta-hCG appear in the urine during pregnancy? (FA p443)
4 week after menstruation
2 weeks after conception
7. What hormonal changes are seen during menopause? (FA p444)
↑ FSH and LH
↑ GnRH
↓ estrogen
8. What is the underlying cause of PCOS? (FA p448) What are the clinical manifestations? What is the treatment
Mech: ↑ LH production leads to annovulation, hyperandrogenism due to deranged steroid syntheisis
CM: ↑ LH, ↓ FSH, ↑ testosterone
Amenorrhea, infertility, obesity, hirsutism
↑ risk endometrial cancer
TX:
OCPs
Clomiphene
Metformin
Spironolactone
• What testicular tumor is described by the following statement: composed of cytotrophoblasts and syncytiotrophoblasts
Choricarcinoma
• What testicular tumor is described by the following statement: may present initially with gynecomastia
Leydigcell tumor
• What testicular tumor is described by the following statement: elevated AFP
Embryonic carincoma
Yolk sac
• What testicular tumor is described by the following statement: elevated hCG
Embryonic carcinoma
Choriocarcinoma
• What testicular tumor is described by the following statement: most common testicular tumor
Seminoma
• What testicular tumor is described by the following statement: most common testicular tumor in infants and children up to 3yrs of age
Leydid cell tumor
• What testicular tumor is described by the following statement: most common testicular tumor in men over age 60
Spermatic seminoma
• What testicular tumor is described by the following statement: histologic appearance similar to kiolocytes (cytoplasmic clearing)
Seminoma
• What testicular tumor is described by the following statement: histologically may have alveolar or tubular appearancesometimes with papillary convulutions
Embryonal carcinoma
• What testicular tumor is described by the following statement: composed of multiple tissue types
Teratoma
• What testicular tumor is described by the following statement: histologic endodermal sinus structures (Schiller-Duval bodies)
Yolk sac
• What testicular tumor is described by the following statement: What testicular tumor is described by the following statement: 25% have cytoplasmic rod-shaped crystalloids of Reinke
Leydig cell
• androgen producing and associated with precocious puberty
Leydig cell or sertoli
\
33. HYQ: 55 y/oman undergoing treatment for BPH has increased testosterone and decreased DHT as well as gynecomastia and edema what is the drug?
5 α reducatse inhibitor
6. What is the difference between androgen insensitivity and 5-alpha-reductase deficiency? (FA p445)
AI: will not become male
5ARD: will become male at puberty

AI: normal appearing female with rudimentary vagina, and testes in labia majora. Levels of testosterone, estrogen and LH are all high
5ARD: unable to convert testosterone to DHT. Ambiguous genitalia until puberty
8. How many carbon molecules are found on testosterone and on andostenedione? (FA p277)
19
9. What structures develop from the mesonephric duct system? (FA p131)
SEVEN UP
• What is the male homologue to the following female structure? vestibular bulbs
Corpus spongiosum
(arises from urogenital sinus)

DHT for male; estrogen for female
• What is the male homologue to the following female structure? labia minora
Ventral shaft of penis
(arises urogenital folds)

DHT for male; estrogen for female
• What is the male homologue to the following female structure? Bartholin glands
Bulbouretral glands (of Cowper)
(arises from urogenital sinus)

DHT for male; estrogen for female
• What is the male homologue to the following female structure? urethral and paraurethral glands (of Skene)
Prostate gland
(arises from urogenital sinus

DHT for male; estrogen for female
What is the male homologue to the following female structure? Glans clitoris
Glans penis
(arises from genital tubercle)
What is the male homologue to the following female structure? Labia majora
Scrotum
(arises from labioscrotal swelling)
Which two drugs inhibit cGMP phosphodiesterase, leading to smooth muscle relaxation in the corpus cavernosum and penile erection?
Sildenafil and Verdenafil --they fill the penis
What class of drugs are used tto treat erectile dysfunction
cGMP Inhibitors
CGMP inibitors taken with ____have a high risk of liofe-threeatening hypotension
nitrates
Which drugs is a partial agonist of estrogen recpetors in the pituitary gland, stimulating increase in LH and FSH, which stimulates ovulation to treat infertility
clomiphene
Clomiphene's side effects include:
Hotflashes, ovarian enlargment, multiple simultaneous pregnancies, visual disturbances
What abortifacient is a competitite inhibitor of preogestins at progesterone recpetor and may lead to heavy menstrual-like bleeding?
Mifepristone (RU486)
The advantage of this drug is that it is reliable, decreases incidence of ectopic pregnancy, decreases risk of pelvic infections, and regulates menses; however it also puts you in a hypercoagulable stat and may increase your trigylcerides, weight, and blood pressure
Oral Contracpetices - syntheitc progestins/estrogen
MOA finasteride
5-alpha reductase blocker
Uses :bph and baldness
MOA flutamide
competitive inhibitor of androgen at testosterone receptor
Uses: prostate carcinoma
MOA ketoconazole in reproductive system
blocks 17,20 lyase and 3-beta-hydroxylase, inhibiting steroid synthesis
uses of finasteride
BPH male pattern baldness
uses of flutamide
prostate cancer
uses of ketoconazole
pcos, to prevent hirsutism
MOA leuprolide
GnRH analog with agonist properties when used in pulsatile manner antagonist properties when used in continuous fashion
Leuploride can be used in Lieu of GnRH
uses for leuprolide
infertility (pulsatile) prostate cancer (continuous, used with flutamide) uterine fibroids
toxicity of leurpolide
antiandrogen n/v
MOA sildenafil
inhibits cGMP phosphodiesterase --> increased cGMP --> maintains smooth muscle relaxation in corpus cavernosum --> increased blood flow and erection
clinical use of sildenafil
erectile dysfunction
toxicity of sildenafil
blue-green color vision h/a flushing dyspepsia LIFE THREATENING HYPOTENSION IN PTS TAKING NITRATES!!!!!
MOA clomiphene
partial estrogen agonist at pituitary gland this stimulates LH and FSH release → ovulation
use of clomiphene
fertility treatment
toxicity of clomiphene
hot flashes ovarian enlargement multiple pregnancies visual disturbances
MOA mifepristone
competitive inhibitor of progestin at progesterone receptors
use of mifepristone
prevents implantation of fetus
toxicity of mifepristone
heavy bleeding GI effects abdominal pain
risks associated with HRT
endometrial cancer, if regimen is just with estrogen
Spironolactone MOA and Use
MOA: inhibits steroid binding
use PCOS
Side effects of Ketoconalzole and Spironolactone
Gynecomastia
amenorrhea
Dinoprostone
PGE2 analog causing cervical dilation and uterine contraction, inducing labor
Ritodrine/terbutaline
Β 2 agonists that relax the uterus
Anastrozole
Armoatase inhibitor used in postmenopausal women with brease cancer
Testosterone uses
Treat ER + brease cancer
Stimulation of anabolism to promote recovery after brun or injury
Lipid probile changes with testosterone
↑ LDL
↓ HDL
(unfavorable, estrogen is favorable)
Estrogens (ethinyl estradiol, DES, mestranol) and cancer
Used in men with androgen dependent prostate cancer. Contraindicated in women with ER + breast cancer
Tamoxifene
MOA
Antagonist on breast tissue
Tamoxifene use
Treat ER+ breast cancer
Raloxifen USE and MOA
Agonist on bone, reduces resporption of bone

Usead to treat osteoporosis
What are the Estrogen partial agonists (selective estrogen receptor modulators SERMS?)
Clomiphene
Tamoxifene
raloxifene