Monday, March 3, 2008

eponyms in medicine - part 28

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

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

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

274 - Behçet’s disease is triad of aphthous ulcers - genital ulcerations - and ocular inflammation (posterior uveitis). Associated with erythema nodosum - cutaneous pustular vasculitis - also synovitis - CNS vasculitis involving brain stem - thrombophlebitis - and positive pathergy response. Described by Turkish dermatologist Behçet in 1937; also known as Silk Road disease due to clustering of cases along the Silk Road.

275 - Kaposi’s sarcoma is low-grade vascular tumor associated with HHV-8 - four forms - classic (older men of Mediterranean or Jewish extraction) - African - organ transplant-associated - and AIDS; differential for skin lesions includes bacillary angiomatosis from Bartonella.

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

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

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

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

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

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

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

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