Relation of Macrophage Migration Inhibitory Factor to Pulmonary Hemodynamics and Vascular Structure and Carbamyl-Phosphate Synthetase I Genetic Variations in Pediatric Patients with Congenital Cardiac Shunts

Figure 4

Photomicrographs of arteries accompanying terminal bronchioles showing severe hypertrophy of the medial layer and absence of intimal lesions. Miller’s elastic stain, objective magnification 20x for both panels. (a) 9-month-old patient with Down syndrome, with atrioventricular septal defect. Peripheral oxygen saturation was 88%, and pulmonary vascular resistance was 5.3 Wood decreasing to 1.8 during nitric oxide inhalation. Z-score transformed pulmonary artery wall thickness was 8.1, and serum MIF level was 12305 upi by chemiluminescence. (b) 4-month-old patient, with ventricular septal defect. Peripheral oxygen saturation was 95%, and pulmonary vascular resistance was 4.0 Wood decreasing to 1.3 while on nitric oxide. Pulmonary artery wall thickness was 21.2 (Z-score), and serum MIF level corresponded to 11014 upi. Both patients had AC CPSI T1405N genotype.

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