Way Of Life. . . Mortality In Addition To Furin
Therefore, PAH was suspected based on the clinical findings. Lung biopsy was performed to verify the diagnosis. Microscopically, the small pulmonary arteries showed mild medial thickening but no intimal proliferation (HE classification: I). However, thrombi at varying stages were found in almost all peripheral small-sized pulmonary arteries (Figure 3(a)). Most thrombi were still in the process of formation (Figure 3(b)). In contrast, the pulmonary veins and respiratory system appeared normal. The detailed examination to investigate the cause of these thrombi was carried out based upon the above described lung biopsy findings, and partial congenital protein C deficiency was detected. PH was successfully controlled with anticoagulants and antithrombotic agents. Figure 3 Histopathological findings in small pulmonary MEK pathway arteries and pulmonary veins of case 3. (a) Almost all small pulmonary arteries have mildly thickened Furin media and are occluded by thrombi. (b) Peripheral small pulmonary artery showing almost completely occluded ... A 2-year-old boy (case 4) with rhabdomyosarcoma started receiving chemotherapy at the age of 17 months, and he developed hepatic venoocclusive disease during treatment. At the age of 24 months, he became dyspneic with hypoxemia. Mild PH was detected on the electrocardiogram and echocardiogram. After a few weeks of oxygen therapy, he experienced sudden chest-abdominal pain and died of sudden cardiopulmonary arrest at the hospital at the age of 26 months. Postmortem examination of the lungs was performed to determine the cause of death. The small pulmonary arteries (>200?��m in diameter) showed moderate medial thickening without intimal lesion (HE classification: I). Some small pulmonary arteries (Dacomitinib have reported PH due to PVOD after stem cell transplantation or chemotherapy [1�C3]. We also diagnosed in 1 patient (case 5) that the cause of unexplained PH developing after chemotherapy was PVOD [4]. The histopathology of PVOD typically shows extensive occlusion of pulmonary veins by intimal fibrosis [14] in the form of concentric layers [6, 7]. The present cases (cases 1�C4) exhibited a distinct pattern of thrombosis in pulmonary veins and small pulmonary arteries characterized by localized and eccentric intimal fibrosis.
