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Article

Decreased smooth muscle cells and fibrous thickening of the tunica media in peripheral pulmonary artery stenosis in Alagille syndrome.

Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology - 1 Jan 2000

Ogawa Yosuke, Yamamoto Amane, Yamazawa Sho, Ikemura Masako, Hirata Yasutaka, Inuzuka Ryo

Abstract excerpt

Alagille syndrome is caused by mutations in genes involved in NOTCH signaling, specifically JAG1 and NOTCH2, and is associated with a high rate of peripheral pulmonary artery stenosis. In this study, we report the case of an infant with Alagille syndrome caused by a JAG1 mutation, who succumbed to acute exacerbation of right heart failure due to severe peripheral pulmonary artery stenosis. The autopsy revealed...

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Source

Identifiers

PMID: 39069193
DOI: 10.1016/j.carpath.2024.107677

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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