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PedAM

Pediatric Disease Annotations & Medicines




Disease n syndrome
Phenotype C0020541|portal hypertension
Sentences 2
PubMedID- 24581591 Primary budd-chiari syndrome, characterized by obstruction of hepatic venous outflow, must be distinguished from sinusoidal obstruction syndrome, a cause of portal hypertension associated with exposure to toxic plants or therapeutic agents.
PubMedID- 24790454 The clinical course of the disease is characterized by a “compensated” or asymptomatic phase followed by a rapidly progressive phase “decompensated”, resulting in development of complications (jaundice, variceal hemorrhage, ascites, encephalopathy) secondary to the two main syndromes associated with cirrhosis, ie, portal hypertension and hepatic insufficiency.2,3 therefore, cirrhosis is classified into four stages, ie, two compensated stages (absence of ascites or presence of nonhemorrhagic esophageal varices) and two decompensated stages (presence of ascites and variceal bleeding), which are related to hemodynamic changes in terms of portal pressure gradient and histological stages of fibrosis.2,4 recently, a fifth stage has been proposed, that consists of infection (sepsis) in critically ill patients, and its presence has been associated with increased mortality5 (figure 1).

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