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Progeria: Autopsy Report of One Case, with a Review of Pathologic Findings Reported in the Literature *
Author(s) -
ISHII TOSHIHARU
Publication year - 1976
Publication title -
journal of the american geriatrics society
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.992
H-Index - 232
eISSN - 1532-5415
pISSN - 0002-8614
DOI - 10.1111/j.1532-5415.1976.tb06779.x
Subject(s) - progeria , medicine , autopsy , atrophy , scleroderma (fungus) , pathology , connective tissue , degeneration (medical) , dermatology , dystrophic calcification , endocrine system , scalp , calcification , hormone , biochemistry , chemistry , inoculation , gene
An autopsy case of progeria associated with elastotic degeneration of the skin and preservation of the scalp hair, in a 20‐year‐old woman, is presented. The pathologic findings were scleroderma‐like skin atrophy, elastotic degeneration of the skin, arteriosclerosis, atrophy of the endocrine glands, and acute peritonitis after appendectomy. In the reported cases of progeria, as in Werner's syndrome, no responsible parenchymatous organ has been identified. Rather, connective tissue, widely distributed throughout the entire body, may play an important role. The possible abnormal metabolism of connective tissue in progeria deserves further study.