Article

Steroid-Responsiveness in TBX4-Associated Pulmonary Hypertension and Interstitial Lung Disease

2026-04-20

Abstract excerpt

<h4>Background</h4> TBX4 variants are a recognised cause of paediatric pulmonary hypertension (PH), often associated with interstitial lung disease (ILD). Evidence for ILD-directed therapy in this group is lacking. <h4>Methods</h4> We conducted a retrospective study of children (≤18 years) with TBX4 -associated PH at a national centre (2001–2025). ILD was defined using ChILD-EU criteria. Patients treated with...

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Literature Corpus work: 7790c57f-ec09-5ea0-a5d5-63fade5a1e96
DOI: 10.64898/2026.04.19.26350630

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