Wednesday, March 5, 2008

eponyms in medicine - part 41

401 - PANDAS is Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections. Consists of tics - movement disorders - emotional-lability - and obsessive-compulsive behavior associated with streptococcal infection. Caused by IgG antibodies which recognize group A streptococcal cell wall proteins as well as neuronal cytoplasmic antigens of the basal ganglia. Related to Sydenham's chorea.

402 - Haldane effect is deoxygenated hemoglobin having a greater affinity for CO2 than oxygenated hemoglobin.

403 - Brugada syndrome is defect in an ion channel gene resulting in abnormal electrophysiologic activity in the right ventricle and characterized by (1) ST segment elevation in V1-V3 - (2) right bundle branch block - (3) sudden cardiac death - (4) grossly normal heart.

404 - ASPEN syndrome is Association of Sickle cell disease - Priapism (usually major) - Exchange transfusion - Neurological events including headache - lethargy - and seizures; may be due to abrupt cerebral hypoxemia from too-rapid exchange or release of vasoactive cytokines released from penile blood vessels; described in J Urol. 1993; 150:1480-1482.

405 - Wright’s stain is stain used for blood and bone marrow films.

406 - Mibelli - porokeratosis of is classic porokeratosis - localized - chronically progressive - hyperkeratotic - irregular plaques with central atrophy and prominent peripheral keratotic ridge; characterized pathologically by presence of cornoid lamella.

407 - Lambert-Eaton myasthenic syndrome is disease of neuromuscular junction associated with paraneoplastic process with proximal muscle weakness that improves with repetitive stimulation; associated with P/Q type presynaptic voltage-gated calcium channel antibodies; 70% have malignancy - usually small cell cancer.

408 - Heberden’s nodes is characteristic in women - but not in men - represent prominent osteophytes at the DIP joints in osteoarthritis (enlargements of tubercles at the articular extremities of the distal phalanges.

409 - Pick bodies is in neurons - cytoplasmic - round to oval - filamentous inclusions that strongly stain with silver - weakly eosinophilic; composed of neurofilaments - vesiculated endoplasmic reticulum - and paired helical filaments that are immunocytochemically similar to those found in Alzheimer’s; don’t survive death of host neuron like they do in Alzheimer’s.

410 - Monteggia fracture is fracture of the proximal third of the ulna with a dislocation of radial head.

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