title: Primary hereditary systemic amyloidosis (Meretoja's syndrome): clinical features and treatment by plastic surgery.
abstract: The characteristic, bloodhound-like appearance, which degenerates gradually, of patients with primary hereditary systemic amyloidosis, also called Meretoja's syndrome (MS), is attributable to amyloid degeneration of the craniofacial skin and peripheral facial nerves, but apparently also to amyloid deposits in the muscles; a finding not previously described. A material of five patients treated with plastic surgery is presented, and the peculiarities and differences of this rare disease in comparison with other peripheral neuropathies is discussed from a reconstructive viewpoint.
authors.affiliation: I Department of Surgery, Helsinki University Central Hospital, Finland.
authors.forename: A E
authors.initials: AE
authors.lastname: Rintala
authors.forename: A
authors.initials: A
authors.lastname: Alanko
authors.forename: J
authors.initials: J
authors.lastname: Mäkinen
authors.forename: R
authors.initials: R
authors.lastname: Nordström
authors.forename: H
authors.initials: H
authors.lastname: Salo
mesh_terms: D000328:Adult; D000368:Aged; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000235:genetics* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D003317:Corneal Dystrophies, Hereditary / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D003483:Cutis Laxa / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D005152:Facial Muscles / Q000378:metabolism / Q000473:pathology; D005158:Facial Paralysis / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D005260:Female; D005387:Finland; D006801:Humans; D008297:Male; D008875:Middle Aged; D013518:Surgery, Plastic; D013577:Syndrome
chemical_list: D000682:Amyloid