Differential diagnosis of hepatopulmonary syndrome (HPS): Portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT)
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چکیده
منابع مشابه
Portopulmonary hypertension and hepatopulmonary syndrome.
The clinically and pathophysiologically distinct entities of portopulmonary hypertension and hepatopulmonary syndrome occur in a substantial proportion of patients who have advanced liver disease of different causes. These disorders are notoriously underdiagnosed, but they have a substantial impact on survival and require focused treatment. Abnormal intrapulmonary vascular dilatation, the hallm...
متن کاملHepatopulmonary syndrome following portopulmonary hypertension.
Portopulmonary hypertension (PPHTN) and hepatopulmonary syndrome (HPS) are distinct clinical entities that may accompany liver disease. While PPHTN and HPS have been infrequently described as occurring in the same patient, to the present authors' knowledge, the order of occurrence has always been the initial onset of HPS, with pulmonary hypertension developing either concurrently or subsequentl...
متن کاملA mouse model for hereditary hemorrhagic telangiectasia (HHT) type 2.
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal-dominant disorder characterized by the age-dependent development of focal arteriovenous malformations and telangiectases. HHT type 2 is caused by loss of function mutations in activin receptor-like kinase 1 (ACVRL1 or ALK1). However, the factors that initiate lesion formation and those that influence disease progression remain unknown....
متن کاملHereditary Hemorrhagic Telangiectasia (HHT, Osler–Weber–Rendu disease) — Symptoms and Diagnosis
Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu-Disease (OWRD) is a rare autosomal dominant disorder that affects blood vessels and consequently multiple systems resulting in a tendency to bleed. Also known as vascular dysplasia, the condition is more or less diagnosed clinically and has a variable prognosis depending on prompt recognition and severity. However, there is no cur...
متن کاملHereditary Hemorrhagic Telangiectasia (HHT, Osler–Weber–Rendu disease) — Symptoms and Diagnosis
Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu-Disease (OWRD) is a rare autosomal dominant disorder that affects blood vessels and consequently multiple systems resulting in a tendency to bleed. Also known as vascular dysplasia, the condition is more or less diagnosed clinically and has a variable prognosis depending on prompt recognition and severity. However, there is no cur...
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ژورنال
عنوان ژورنال: Bosnian Journal of Basic Medical Sciences
سال: 2017
ISSN: 1840-4812,1512-8601
DOI: 10.17305/bjbms.2017.2020