51 - Marshall - vein of is oblique vein of left atrium.
52 - Cushing’s disease is hypercortisolism from pituitary corticotropin-(ACTH)-secreting corticotroph tumors - almost always benign and usually microadenomas.
53 - Bezold-Jarisch reflex is activation of receptors in the atria - great veins - and left ventricle causing increased parasympathetic tone and decreased sympathetic activity leading to a combination of hypotension and bradycardia with a sudden increase in coronary flow.
54 - Simon focus is in tuberculosis - nodule in subapical region which develops from hematogenous spread from initial infection in lower half of lung.
55 - Bell’s palsy is seventh nerve palsy; seen as a complication in diabetes - tumors - sarcoidosis - AIDS - and Lyme disease.
56 - Syme’s amputation is amputation at the ankle with removal of the malleoli and placement of the heel pad over the end of the remaining tibia; devised in 1842.
57 - Billroth’s cords is splenic cords found in the red pulp between the sinusoids - consisting mainly of fibrils and connective tissue cells.
58 - Louvel’s sign is in deep vein thrombosis - venous pain induced by coughing - prevented by pressing over proximal end of vein.
59 - Von Willebrand’s disease is deficiency in von Willebrand factor; frequency of 1% - most common inherited disorder of bleeding; type 1 and 3 reduced quantity of vWF; type 2 qualitative defects in vWF.
60 - Kussmaul’s respiration is hyperpnea - associated with acidosis - especially DKA but also in uremia.
61 - Berardinelli-Seip syndrome is congenital generalized lipodystrophy - apparent at birth - infants look very muscular due to absence of fat - associated with diabetes - hepatomegaly - acanthosis nigricans - enlarged external genitalia - and increased rate of skeletal growth.
62 - Kussmaul’s sign is jugular vein distension during inspiration (reversal of normal pattern of decreasing jugular venous pressure during inspiration); classically seen in constrictive pericarditis because of normal increase in venous return with increased abdominal pressure from diaphragmatic contraction leading to increase in right atrial pressure from non-complaint right ventricle - though most common contemporary cause is severe right-sided heart failure (JAMA 1996: 275:632); generally negative in cardiac tamponade.
63 - Nijmegen breakage syndrome is primary immunodeficiency autosomal recessive disorder that presents as microcephaly - a distinctive “bird-like” facies - growth retardation - recurrent pulmonary infections - chromosomal instability - radiation hypersensitivity - and increased incidence of malignancies - especially lymphomas from mutation in nibrin protein - a protein involved in DNA repair; cellular defect similar to ataxia-telangiectasia.
64 - Dobrin’s syndrome is TINU syndrome (tubulointerstitial nephritis with uveitis) - associated with bone marrow granulomas - first described in 1975.
65 - Westermark’s sign is in chest film - an abrupt tapering of a vessel caused by pulmonary embolism - focal oligemia.
66 - Jod-Basedow phenomenon is thyroid hyperfunction induced by excess iodine ingestion in patients with various thyroid disorders; Jod German for iodine; K. A. Von Basedow.
67 - Toldt - white lines of is the peritoneal reflections of the ascending and descending colon.
68 - Möbius syndrome is congenital facial paralysis with or without limb defects associated with maternal misoprostol use.
69 - Landolfi’s sign is in aortic regurgitation - systolic contraction and diastolic dilation of the pupil.
70 - Rasmussen’s encephalitis is progressive childhood disease characterized by severe epilepsy - hemiplegia - dementia - and inflammation of the brain potentially from autoantibodies to GluR3 antigen.
71 - La Crosse encephalitis is transmitted by tree hole mosquito - Aedes trisereiatus and causes most common arboviral infection in children in N. America; hyponatremia and fever associated with worse outcome.
72 - Carney syndrome (or triad) is nonfamilial disorder the includes combination of three rare tumors: gastric leiomyosarcoma - pulmonary chondroma - extraadrenal paraganglioma but no cardiac manifestations; unrelated to Carney complex.
73 - Keutel syndrome is malformation syndrome characterized by multiple pulmonary stenoses - neural hearing loss - short terminal phalanges - and calcifications and/or ossification of the cartilage in ears - nose larynx - trachea - and ribs; due to mutation in gene encoding human matrix Gla protein.
74 - Watson-Schwartz reaction is screening test for porphobilinogen (elevated for example in acute intermittent porphyria); mixing 4-dimethylaminobenzaldehyde with urine and then extract with chloroform; porphobilinogen extracts into upper aqueous layer giving it a pink color and urobilinogen extracts into chloroform layer; 50% positive at urinary concentration 5x upper limit of normal and consistently positive 10-20x upper limit of normal.
75 - Stuart factor is factor X.
76 - Pulfrich phenomenon is misperception of trajectory of moving objects (an optical illusion that can be elicited in normal subjects) - can be associated with optic neuritis.
77 - May-Thurner syndromne is iliofemoral deep vein thrombosis from impaired venous return because of compression of the left common iliac vein by overlying right common iliac artery.
78 - Bergmann gliosis is in ethanol abuse - proliferation of astrocytes adjacent to lost Purkinje cells between depleted granular cell and molecular layer of cerebellum.
79 - Preiser disease is spontaneous - non-traumatic osteonecrosis of the scaphoid bone.
80 - Winter’s formula is gives expected pCO2 (respiratory compensation) in uncomplicated metabolic acidosis; expected CO2=[HCO3]*1.54 + 8.36.
81 - Ruffini’s corpuscles is sensory receptors for heat.
82 - Corrigan’s pulse is in aortic regurgitation - pulses are of the water-hammer or collapsing type with abrupt distension and quick collapse - can be exaggerated by raising the patient’s arm.
83 - Jolly test is in myasthenia gravis - a sequence of repetitive nerve stimulation (RNS) studies specifically designed to look for neuromuscular junction disease. A positive test is a >10% decremental response with 3 Hz repetitive stimulation - 50-80% sensitive. After Friedrich Jolly - German neurologist - 1844-1904.
84 - Kocher’s test is compression of lateral lobes of thyroid causing stridor; associated with thyroid carcinoma - goiter - or thyroiditis.
85 - Lieberkühn - crypts of is intestinal glands found between villi.
86 - Werdnig-Hoffman syndrome is spinal muscular atrophy type I - autosomal recessive lower motor neuron disease - survival motor neuron protein affected (linked to 5q11.2-13.3) - presents between birth and 6 months of age - death before 2 y.o.
87 - Blau’s syndrome is granulomatous synovitis - non granulomatous uveitis - cranial neuropathies; resembles childhood sarcoidosis; no lung involvement; associated with mutations in NOD2/CARD15 gene; first described in 1985.
88 - Conradi-Hunermann syndrome is chondrodysplasia punctata - characterized by stippled epiphyses from abnormal accumulation of calcium salts and skeletal changes.
89 - Biette’s collarette is in syphilis - a thin white ring of scales on the surface of a lesion.
90 - Marjolin's ulcer is squamous cell carcinoma ulceration overlying chronic osteomyelitis or burn scar.
91 - Wilson’s disease is hepatolenticular degeneration due to mutation in gene involved in incorporation of copper into ceruloplasmin and excretion of copper into bile; Kayser-Fleischer ring; gene on chr 13 - cation transporting P-type ATPase; incidence 1:200 -000 - diagnosis based on decrease in serum ceruloplasmin - increased urinary excretion of copper - increase in hepatic copper content; 40% have neurologic findings (Parkinson’s - psychosis) and subclinical liver.
92 - Wolman disease is lysosomal acid lipase deficiency hydrolysis of cholesteryl esters and triglycerides in the lysosome leading to mild mental retardation - hepatomegaly - adrenal calcification - fatal in infancy with inanition - malabsorption - and intractable diarrhea.
93 - Turner’s syndrome is females with XO - short - low-set ears - shield chest - congenital heart defect (usually coarctation) - café-au-lait spots - freckles - webbed neck - lymphedema.
94 - Wada test is test for hemispheric dominance for language by injecting amobarbital into carotid artery.
95 - Wallerian degeneration is pattern of degeneration of distal portion of nerve following axonal injury with break down of axon and formation of myelin ovoids from catabolized axon fragments.
96 - Hitzig zones is in tabes dorsalis (syphilis) - regions of reduced sensation found in central face - nipples - ulnar forearms - and peroneal legs - leading to painless ulcers and joint damage.
97 - Blumberg sign is rebound tenderness.
98 - Frank’s sign is earlobe crease - associated with CAD (N Engl J Med. 1973;289:327-8).
99 - Gorham-Stout disease is vanishing or disappearing bone disease; IL-6 has pathogenetic role.
100 - Holme’s heart is single ventricle with normally-related great arteries.
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