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

Inna Krynytska; Mariya Marushchak; Anna Mikolenko; Anzhela Bob; Iryna Smachylo; Ludmyla Radetska; Olga Sopel

doi:10.17305/bjbms.2017.2020

Authors

Inna Krynytska Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine
Mariya Marushchak Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine
Anna Mikolenko Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine
Anzhela Bob Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine
Iryna Smachylo Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine
Ludmyla Radetska Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine
Olga Sopel Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine

DOI:

https://doi.org/10.17305/bjbms.2017.2020

Keywords:

Hepatopulmonary syndrome, differential diagnosis, portopulmonary hypertension, hereditary hemorrhagic telangiectasia, PPH, HPS, HHT, orthotopic liver transplantation, OLT

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.

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Author Biographies

Inna Krynytska, Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine

Functional and Laboratory Diagnostics Department
Mariya Marushchak, Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine

Functional and Laboratory Diagnostics Department
Anna Mikolenko, Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine

Functional and Laboratory Diagnostics Department
Anzhela Bob, Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine

Functional and Laboratory Diagnostics Department
Iryna Smachylo, Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine

Functional and Laboratory Diagnostics Department
Ludmyla Radetska, Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine

Functional and Laboratory Diagnostics Department
Olga Sopel, Functional and Laboratory Diagnostics Department, I. Horbachevsky Ternopil State Medical University, Ternopil, Ukraine

Functional and Laboratory Diagnostics Department

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