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The pathophysiology of pulmonary hypertension (PH) and heart failure (HF) includes fibrogenic remodeling associated with the loss of pulmonary arterial (PA) and cardiac compliance. We and others have previously identified transglutaminase 2 (TG2) as a participant in adverse fibrogenic remodeling. However, little is known about the biologic. Most participants were predominantly in the age range of 21–30 years (14), belonging to the middle class (17), with a bachelor’s (11) or master’s (7) degree, and currently employed in helping (9), creative (7) and corporate (4) professions. Five out of the six cases in our series were women and in the middle age group (range, 15 to 56 years; mean, 38 years). Case 1 A year-old girl presented with a history of painless, progressive swelling on the left side of the face of 2 months duration.
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