Differential diagnosis of hepatopulmonary syndrome (HPS): Portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT)

Abstract

Hepatopulmonary syndrome (HPS) is a severe complication of advanced liver disease associated with an extremely poor prognosis. HPS is diagnosed in 4-47% of patients with cirrhosis and in 15-20% of candidates for liver transplantation. In addition, severe hypoxia is associated with a high risk of complications of liver transplantation (a 30% chance during the first 90 days) and increases the gap between transplantation and improving arterial oxygenation. The pathogenesis of HPS is not fully understood, and no effective pharmacological treatment has been developed yet. Currently, the treatment of choice for HPS is orthotopic liver transplantation. Non-specific clinical criteria and the lack of standardized diagnostic criteria for determining HPS can lead to diagnostic errors. Portopulmonary hypertension and hereditary hemorrhagic telangiectasia, also known as Osler-Weber-Rendu syndrome, are pulmonary complications of liver disease which should be differentially diagnosed from HPS.

The indices of endogenous intoxication in rats with different models of hepatopulmonary syndrome

“…"The authors declare no conflict of interest regarding this article" "The authors declare that all the procedures and experiments of this study respect the ethical standards in the Helsinki Declaration of 1975, as revised in 2008 (5), as well as the national law. Informed consent was obtained from all the patients included in the study" "All institutional and national guidelines for the care and use of laboratory animals were followed"…”

“…True HPS is estimated to occur in at least 10% of all patients with cirrhosis and portal hypertension 4 . The most frequent clinical symptoms of HPS are progressive dyspnea and platypnea 5 , but neither is specific for HPS. Digital clubbing, with a positive predictive value of 75%, was the best clinical sign associated with the presence of HPS, while a correlation between the presence of spider angiomas and HPS was also demonstrated recently, suggesting that they could be used as skin markers of HPS [6][7] .…”

The indices of nitrogen (II) oxide system in experimental hepatopulmonary syndrome

“…Although it commonly oc curs, shortness of breath is a nonspecific symptom of HPS. The most common manifestations of HPS are platypnea (increased shortness of breath when the body is in a vertical position) and orthodeoxia (310 mmHg reduction in PaO 2 in capillary blood during transition from horizontal to vertical posi tion) [6].…”

The effect of immunoregulatory bacteria on the transcriptional activity of Foxp3 and RORyt genes in the gut-associated lymphoid tissue with Salmonella-induced inflammation in the presence of vancomycin and Ваcteroides fragilis

“…RAR-related orphan receptor gamma (RORγt) promotes thymocyte differentiation into Th17 cells. By contrast, Treg cells produce anti-inflammatory cytokines IL-10 and TGF-β, suppress activity of a variety of immune cells, inhibiting immune responses ( 8 , 9 ). Recent studies have identified intestinal RORγt+ Treg cells and found that these microbiota-induced Treg cells were required for the regulation of specific T helper cells in the gut.…”