Is Trisomy 21 a Risk Factor for Rapid Progression of Pulmonary Arteriopathy?　― Revisiting Histopathological Characteristics Using 282 Lung Biopsy Specimens

Is Trisomy 21 a Risk Factor for Rapid Progression of Pulmonary Arteriopathy?　― Revisiting Histopathological Characteristics Using 282 Lung Biopsy Specimens

Background:Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically.Methods and Results:A retrospective review of a lung biopsy registry identified 282 patients: 188 patien...

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Veröffentlicht in:Circulation Journal 2018/05/25, Vol.82(6), pp.1682-1687
Hauptverfasser: Masaki, Naoki, Saiki, Yuriko, Endo, Masato, Maeda, Kay, Adachi, Osamu, Akiyama, Masatoshi, Kawamoto, Shunsuke, Saiki, Yoshikatsu
Format: Artikel
Sprache:eng
Schlagworte:
Atrioventricular septal defect
Pulmonary arteriopathy
Trisomy 21, Ventricular septal defect
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Zusammenfassung:Background:Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically.Methods and Results:A retrospective review of a lung biopsy registry identified 282 patients: 188 patients with trisomy 21 (Group D) and 94 without (Group N). The mean age at lung biopsy was 3 and 7 months (P
ISSN:1346-9843
1347-4820
DOI:10.1253/circj.CJ-17-0754