Saturday, February 16, 2008

eponyms in medicine - part 13

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

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

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

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

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

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

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

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

129 - fetor hepaticus is “musty” or “sweet odor” from the formation of mercaptans by the action of GI bacteria on the sulfur-containing amino acid methionine and shunting of splanchnic blood from the portal into the system circulation (portosystemic shunting).

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

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