Open AccessProgeria with cardiac hypertrophy and review of 12 autopsy cases in the literature
Author(s) -
Shozawa Takeshi,
Sageshima Masato,
Okada Eikichi
Publication year - 1984
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1984.tb07608.x
Subject(s) - progeria , autopsy , pathology , medicine , cardiac hypertrophy , muscle hypertrophy , cardiology , biology , genetics , gene
An autopsy of an 11 years 5 months old boy with progeria is presented. Hyaluronuria, low growth rate of skin fibroblasts, decreased number of T‐cells in lymphocyte subpopulation, increase of anti DNA antibody, and antibody for microsome of thyroid gland were detected clinically. He had suffered an attack of cerebral infarct and died of congestive cardiac failure. Pathologic findings were scleroderma‐like skin atrophy, moderate arteriosclerosis of the aorta and great arteries, severely narrowed coronary sclerosis with an extensive subendo‐cardial fibrosis, a large left cerebral infarct with pin‐holed stenosis of both internal carotid arteries, cortical atrophy of thymus, and atrophic lymph nodes. The cardiac muscle fibers were slightly hypertrophied and measured 15.5 ± 2.8 m̈ in diameter. Histologic findings suggest that increasing of collagen in the connective tissue may play an important role in progeria. Further study of metabolic disturbance in the connective tissue of progeria is necessary. Acta pathol. jpn. 34: 797∼811, 1984.