Abnormal endothelial factor VIII associated with pulmonary hypertension and congenital heart defects.

摘要: In patients with pulmonary hypertension associated congenital heart defects, ultrastructural abnormalities are observed in endothelial cells, which suggest heightened metabolic function. If production of the von Willebrand factor (vWF) is increased, this may be abnormal interactions platelets leading to worsening hypertension. We therefore evaluated vWF 30 (25 defects) and individuals normal arterial pressure (12 defects). measured antigenic (vWF: Ag) biologic (VWF: rist) activity plasma assessed vWF: Ag directly by an immunoperoxidase stain applied lung biopsy tissue. Because considerable variance small size, group five without defects were excluded from statistical analyses. Patients had significant higher levels than (p less .05), whereas values those intermediate. tissue available 29 study 11 others we previously reported, immunostain endothelium for was intense (suggesting increased production) 32 only one eight .01). Only three Ag, however, rist. Compatible discrepancy a loss high-molecular weight forms as determined both crossed immunoelectrophoresis multimeric analysis. Our results that most biologically deficient molecule lacking forms.