Fatal pulmonary hypertension associated with an atypical case of Hunchinson-Gilford progeria

Authors
Shiraishi, I Hayashi, S Hirai, E Onouchi, Z Hamaoka, K
Citation
I. Shiraishi et al., Fatal pulmonary hypertension associated with an atypical case of Hunchinson-Gilford progeria, PEDIAT CARD, 22(6), 2001, pp. 530-533
Citations number
12
Categorie Soggetti
Pediatrics
Journal title
PEDIATRIC CARDIOLOGY
ISSN journal
01720643 → ACNP
Volume
22
Issue
6
Year of publication
2001
Pages
530 - 533
Database
ISI
SICI code
0172-0643(200111/12)22:6<530:FPHAWA>2.0.ZU;2-R
Abstract
We report the first autopsied case of an incomplete type of Hunchinson-Gilf ord progeria associated with fatal pulmonary hypertension. Histopathologic findings revealed abnormal deposition of collagen and elastic fibers. as we ll as cellular proliferation, at intima of the coronary arteries, pulmonary small arteries, and arterioles. These changes could be underlying conditio ns of the association of the two diseases.