Multiple pulmonary arteriovenous fistulas in juvenile cirrhosis

Pulmonary–Hepatic vascular Disorders (PHD)

“…However, the few detailed pathological studies were carried out before the standardisation of a universal definition of HPS and prior to the availability of imaging modalities used to detect IPVD. Nonetheless, dilatation of capillary vessels in alveolar regions [1,45] is a central prerequisite and the principal pathophysiological hallmark of arterial deoxygenation in both human and experimental HPS [46,47]. In addition, in animal models, intravascular accumulation of macrophages in the pulmonary microcirculation and increased numbers of pulmonary capillaries have been shown, suggesting a vasculogenic response [47,48].…”

Failure of hypoxic pulmonary vasoconstriction in patients with liver cirrhosis

“…(Circu-mixture as the cause of the arterial hypoxemia. Necropsy studies, however, in patients with liver cirrhosis have revealed intrapulmonary arterio-venous anastomoses or portopulmonary venous communications in only a few patients (9,10).…”

Respiration and Circulation in Patients with Portal Cirrhosis of the Liver

“…It is conceivable that hypoxemia, if present, may stimulate red cell production and induce a relative or absolute polycythemia. Two such patients with cirrhosis of the liver and secondary polycythemia have been reported in the literature.8' 18 The mechanism causing the rise in plasma volume is unknown. But a similar hypervolemia is observed in other conditions associated with …”