Critical re-examination of the premature aging concept in progeria: A light and electron microscopic study

While premature aging is traditionally considered the underlying process in progeria, previous studies of post-mortem material have failed to provide compelling support for this concept. Lipofuscin accumulation, a marker of aging in certain tissues, was studied by light and electron microscopy in a 19-year-old male with progeria. Although lipofuscin was demonstrated in several viscera and brain, it was neither excessive nor morphologically abnormal. As in previously reported cases, the post-mortem findings demonstrated atherosclerosis of the coronary arteries, aorta and heart valves, as well as moderate nephrosclerosis. There was hypoplasia of the mandible and clavicles, and coxa valga deformities of the hips. The morphological abnormalities in progeria occur mainly in tissues of mesodermal derivation. It is suggested that progeria represents either a developmental disturbance or a degenerative process in mesodermal tissues rather than a generalized accelerated aging process.