1 - Lorenzo’s oil is after Lorenzo Odone - diagnosed with adrenoleukodystrophy in 1984 - the triglycerides of monounsaturated oleic acid and erucic acid 4:1 - felt to reduce very long chain fatty acids to normal levels in ALD.
2 - Mayo - veins of is vein overlying pylorus
3 - Hutchinson freckle is lentigo maligna - a nonfamilial precursor to lentigo maligna melanoma
4 - Chadwick’s sign is blue-red passive hyperemia of the cervix that may appear after 7th week of pregnancy; may be seen in association with tumor; results from congestion of mucosa and most visible in anterior vaginal wall.
5 - Cabot ring is 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
6 - Epstein-Barr virus is EBV binds to CD21 found on epithelial cells and B cells; however a large number of T suppressor cells and EBV specific cells are seen as atypical lymphocytes
7 - Sugiura procedure is for treating esophageal varices - esophageal and gastric devascularization - esophageal transection
8 - Sneddon-Wilkinson disease is subcorneal pustular dermatosis; rare - chronic - recurrent pustular eruption characterized by subcorneal pustules that contain abundant neutrophils
9 - von Braun-Fernwald’s sign is Piskacek’s sign.
10 - Leydig cell tumors is testicular tumor derived from the stroma - may find Reinke crystals (q.v.)
11 - Guthrie test is for diagnosis of PKU - a bacterial assay for phenylalanine.
12 - Ghon lesion is primary area of tuberculosis infection.
13 - Paneth cells is in small intestine crypts - cells with apically oriented bright eosinophilic granules and which appear to play a role in the mucosal immune system.
14 - Stellwag’s sign is incomplete and infrequent blinking in Graves’s disease.
15 - Virchow’s node is supraclavicular adenopathy associated with a malignancy - often on left side - associated with stomach cancer among other neoplasms but also GI and pelvic malignancies in general. First described by Virchow in 1848 - more cases added by Troisier in 1886; referred to as Troisier’s node in France.
16 - Lynch's syndrome I and II
Associated persons:
Henry T. Lynch
Description:
Lynch syndrome I:
Familial predisposition to colorectal cancer with right-sided predominance. Predominantly early-onset proximal colon carcinomas.
Lynch syndrome II:
Familial predisposition for other primary cancers in addition to the predisposition for colon cancer; site is often female reproductive organs. Predominantly early onset proximal colon carcinoma associated with other extracolonic adenocarcinomas, particularly endometrial carcinoma.
Both disorders are inherited as autosomal dominant traits.
First described 1913 by Alder Scott Warthin, 1867-1931.
Lynch syndrome II is cancer family syndrome - all features of Lynch I with early onset of carcinoma at other sites including endometrium - ovaries - and stomach - also upper tract TCC.
17 - Barraquer-Simons syndrome is acquired partial lipodystrophy; presents usually around 8-10 - preceded generally by an acute viral infection; spares legs and hips; 1/3 of patients develop membranoproliferative glomerulonephritis.
18 - McLeod phenotype is occurs from the absence of the Kx red blood cell antigen; characterized by acanthocytes; compensated hemolytic anemia; decreased Kell system antigens; increased CKMM isoenzymes; and progressive neurologic changes including areflexia - choreiform movements - dysarthria - wasting of muscles - and cardiomyopathy; may be associated with X-linked chronic granulomatous disease.
19 - Monod’s sign is in aspergillomas - radiolucent crescent seen around solitary 3-5 cm lesion on chest X-ray.
20 - TRALI syndrome is Transfusion Related Acute Lung Injury; transfusion reaction occurring within 6 hours after transfusion of blood product - characterized by pulmonary edema; due to anti-granulocyte antibodies in donor’s plasma causing pulmonary sequestration of recipient leukocytes in susceptible patient.
21 - Hering-Breuer reflex is stretch receptor (in smooth muscle of airways) reflex - responsible for apnea - i.e. decreased breathing frequency - as a result of lung inflation.
22 - Ransons’ criteria is for evaluating acute pancreatitis at presentation - age>55; WBC>16 -000; glucose>200; AST>250; LDH>350. during initial 48 hrs - base deficit>4; BUN increase>5; fluid sequestration>6L; Ca <8;>10; pO2<60.>
23 - Weibel-Palade bodies is found only in endothelial cells of vessels larger than capillaries; granules contain von Willebrand’s factor (VIII) and P-selectin.
24 - Dennie’s lines is in atopic dermatitis - an accentuated line or fold below the margin of the lower eyelid.
25 - Guam disease is Guam amyotrophic lateral sclerosis-parkinsonism-dementia linked to a plant excitant neurotoxin found in cycad plant eaten by Chamorro people - toxin believed to be excitotoxin beta-N-methylamino-L-alanine - a low-potency convulsant Science 1987;237:517-22.
26 - Kent - bundle of is AV bypass accessory tract in Wolf-Parkinson-White syndrome that directly connects atrial and ventricular myocardium.
27 - Graham-Little syndrome is end-stage lichen planus of the scalp resulting in scarring alopecia of the scalp.
28 - Van der Woude syndrome is an AD condition in which lip pits are seen in all gene carriers but only some individuals have cleft lips with or without cleft palate owing to variable expressivity.
29 - MacConkey medium is medium for growing nonfastidious Gram-negative rods.
30 - Hegar’s sign is softening of the uterus at the junction between the cervix and the fundus during the first trimester of pregnancy.
31 - Lachman maneuver is for diagnosing ACL tear - pull on tibia in anterior direction with knee flexed at 20-25 degrees (if tibia slides anteriorly >2 mm - positive).
32 - Allen’s sign is in pulmonary embolism - fever - tachycardia - and tachypnea - present in only 23% of cases.
33 - Isaacs’s syndrome is neuromyotonia; continuous muscle stiffness - rippling muscle movements (myokymia) - delayed relaxation following muscle contraction believed to be due to autoantibodies to presynaptic potassium channels.
34 - Stevens-Johnson syndrome is extensive and symptomatic febrile form of erythema multiforme - more common in children; 1-6 cases/million person-years.
35 - Betz cells is large pyramidal cells in layer 5 of primary motor cortex largest neurons in mammalian CNS; 30-40 -000 Betz cells in precentral gyrus in one side of the brain.
36 - Thomsen’s disease is myotonia congenita - autosomal dominant.
37 - Trietz - ligament of is the suspensory muscle of the duodenum which supports the duodenojejunal flexure.
38 - Bier block is regional anesthesia of an extremity by placing a tourniquet and then infusing local anesthetic into a vein.
39 - Trendelenburg position is position where angle of the head of bed or table is inclined at 45 degrees down; used in surgery to push the abdominal organs towards the chest.
40 - Crohn’s disease is transmural mucosal inflammation that may involve the entire GI tract from mouth to the perinanal area that often leads to fibrosis and obstructive clinical presentations; 80% small bowel involvement - usually distal ileum; 1/3 exclusively ileitis; 50% ileocolitis; and 20% disease limited to colon.
41 - Blaschkow - lines of is patterning of a variety of linear nevi as well as linear patterning of commonly acquired diseases such as psoriasis - scleroderma - and lichen planus - including V shape over the upper spine - S shape of the abdomen - inverted U shape from the breast area - and perpendicular lines down lower extremities; cannot be explained by distribution of cutaneous nerve - lines of cleavage - nor blood vessels or lymphatics; first described in 1901.
42 - Tamm-Horsfall protein is uromodulin - major component of renal casts; 30-50 mg secreted per day by cells in thick ascending limb; homologous to GP2 - a protein secreted from the acinar cell and a major component of plugs in noncalcific chronic pancreatitis.
43 - Pringle maneuver is clamping of porta hepatis to control hemorrhage.
44 - Treacher Collins syndrome is first arch syndrome - mandibulofacial dysplasia - caused by autosomal dominant gene - resulting in malar hypoplasia with down-slanting palpebral fissures - defects in the lower eyelids - deformed external ears - and sometimes abnormalities of the middle and internal ears.
45 - Reiter’s syndrome is triad of arthritis - urethritis - and conjunctivitis described by Reiter in 1916 (Nazi sympathizer); 80% possess HLA-B27; associated with Shigella flexneri; ReA develops in 20% of exposed B27+ individuals; subset of reactive arthritis; triad present in 1/3 of patients; incidence estimated as 3.5/100K in males under age of 50; balanitis circinata (penis) and keratoderma blenorrhagica.
46 - Gunther’s disease is AR congenital erythropoietic porphyria from decreased URO synthase activity - hemolytic anemia - cutaneous lesions.
47 - Von Myenburg complexes is close to or within portal tracts - small clusters of modestly dilated bile ducts embedded in a fibrous - sometimes hyalinized stroma; contain inspissated bile concrements and may communicate with the biliary tree; common and usually without clinical significance.
48 - Aicardi syndrome is partial or complete agenesis of corpus callosum - infantile spasms - mental retardation - lacunae of the retina; occurs only in females; likely X-linked dominant with lethality in males; described in 1965.
49 - Lyme disease is inflammatory disease caused by spirochete Borrelia burgdorferi - spread by Ixodes tick - characterized by early local disease with erythema migrans - myalgias - followed by early disseminated disease characterized by carditis - neurologic findings (lymphocytic meningitis - cranial nerve palsies - also Bannwarth’s syndrome) - and late disease characterized by arthralgias and arthritis.
50 - Froment’s sign is diagnosis of ulnar nerve lesion; caused by flexor pollicus longus (median nerve) which comes into action when the patient attempts to grip a flat object between the thumb and the hand - and causes flexion at the interphalangeal joint.
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