Amyloidosis of the larynx.Amyloidosis AA amyloidosis secondary a. AL amyloidosis primary a. ATTR amyloidosis the most common form of familial amyloidosis (q.v.), associated with mutations of the gene encoding transthyretin. is characterized by the accumulation of insoluble fibrillar fi·bril·lar·y (-l r![]() )adj. protein (amyloid) in tissues and organs throughout the body. Some deposition processes are local and some are systemic. Of the several types of amyloidosis, nodular amyloidosis is the type found most frequently in the larynx and nasopharynx, as well as in the trachea and lungs. 1. Relating to a fibril. 2. A 48-year-old basketball coach presented with a 2.5-year history of progressive hoarseness, vocal fatigue, loss of high and low range, and pain when speaking. His problems had become severe enough to force him to retire from coaching. Prior to his visit, his otolaryngologist had diagnosed amyloidosis of the larynx. Histopathologic evaluation on H&E staining of a soft-tissue mass revealed normal surface squamous epithelium, chronic subepithelial inflammatory infiltrate, and diffuse eosinophilic 1. readily stainable with eosin. 2. pertaining to eosinophils. 3. pertaining to or characterized by eosinophilia. intercellular intercellular /in·ter·cel·lu·lar/ (-sel´u-lar) between or among cells.in·ter·cel·lu·lar ( n t deposits (some of which caused pressure necrosis of the surrounding stoma) (figure 1). Sections stained with Congo red Con·go red (k ng g )n. dye demonstrated the characteristic yellow and apple-green birefringence birefringence /bi·re·frin·gence/ (bi?-re-frin´jens) the quality of transmitting light unequally in different directions.birefrin´gentbi·re·frin·gence (b , and the lesion had the beta-pleated sheet structure and antiparallel antiparallel /an·ti·par·al·lel/ (-par´ah-lel) denoting molecules arranged side by side but in opposite directions. conformation con·for·ma·tion (k n f r-m by low-angle x-ray diffraction, typical of amyloidosis. (figure 2). The patient was referred to the senior author (R.T.S.) for further treatment. A thorough systemic workup revealed no evidence of amyloid outside the head and neck. Physical examination and videostroboscopy detected a large left supraglottic mass that obscured visualization of the anterior two-thirds of the left vocal fold (figure 3A). Endoscopy also revealed a partial supraglottic obstruction of the airway and a good subglottic airway, despite evidence of a tracheal mass (figure 3B). Videostroboscopy also detected both a pharyngeal (figure 3C) and a tongue base (figure 3D) mass. Surgical resection of the supraglottic mass resulted in an improved voice. From the Department of Otolaryngology-Head and Neck Surgery, Thomas Jefferson University, Philadelphia (Dr. Sataloff and Dr. M. Abaza); the Department of Otolaryngology-Head and Neck Surgery, Graduate Hospital, Philadelphia (Dr. Sataloff); the Department of Surgery, MCP-Hahnemann (Christian Friedrich) Samuel 1755-1843. German physician and founder of homeopathy. He postulated that medicine produces symptoms in healthy people that are similar to those that it relieves in sick people. |
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