Wednesday, March 5, 2008

eponyms in medicine - part 39

381 - Parinaud’s oculoglandular syndrome is preauricular node enlargement associated with chronic granulomatous conjunctivitis; atypical form of cat scratch disease from inoculation near eye.

382 - Reinke’s edema is vocal cord polyposis in female smokers - 50s-70s.

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

384 - Fontan procedure is for treating tricuspid atresia - pulmonary atresia - or hypoplastic left heart syndrome - procedure developed in 1968 whereby IVC is connected to pulmonary artery; long term complications include arrhythmias and protein losing enteropathy because of edema in liver and small bowel.

385 - Gleason score is prostate cancers stratified into 5 grades on basis of glandular patterns and degree of differentiation; grade 1 most differentiated and grade 5 no glandular differentiation; primary score and secondary score (for second most common histology) added together for score; therefore most differentiated tumor score of 2.

386 - Gianotti-Crosti syndrome is reactive exanthem - first described in association with hepatitis B infection (papular acrodermatitis of childhood - papular eruption on face and limbs); also associated with EBV - CMV infection.

387 - Naxos disease is initially described on Greek island of Naxos - synrome of arrhythmogenic right ventricular cardiomyopathy - non-epidermolytic palmoplantar keratoderma - and woolly hair - due to deletion in plakoglobin - protein in adherens and desmosomal junctions.

388 - Carney complex is AD complex of cardiac myxomas with aggressive biologic behavior - spotty pigmentation - and association with endocrine tumors (causing e.g. - Cushing syndrome); mutations in PRKAR1alpha gene (a protein kinase) account for half of the cases; unrelated to Carney syndrome.

389 - Klinger disease is Wegener’s disease; first described by Heinz Klinger in 1931 as a medical student and then described by Friederic Wegener in 1936.

390 - Paget’s disease of breast is breast cancer involving nipple and areola - appears as eczematous rash over breast with crusting - scaling - or erosion.

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