Thursday, February 14, 2008

eponyms in medicine - part 5

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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