Fatal pulmonary hypertension associated with an atypical case of Hunchinson-Gilford progeria.
We report the first autopsied case of an incomplete type of Hunchinson-Gilford progeria associated with fatal pulmonary hypertension. Histopathologic findings revealed abnormal deposition of collagen and elastic fibers, as well as cellular proliferation, at intima of the coronary arteries, pulmonary small arteries, and arterioles. These changes could be underlying conditions of the association of the two diseases.
Shiraishi
I
I
Division of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kawaramachi Hirokoji, Kamikyo-ku, Kyoto, Japan 602-8566. isao@koto.kpu-m.ac.jp
Hayashi
S
S
Hirai
E
E
Onouchi
Z
Z
Hamaoka
K
K
D002648:Child; D017809:Fatal Outcome; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000473:pathology; D008297:Male; D011371:Progeria / Q000150:complications* / Q000473:pathology