Case reportMultiple pulmonary arteriovenous fistulas in juvenile cirrhosis☆
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Non-invasive assessment of intrapulmonary shunt and ventilation to perfusion ratio in children with hepatopulmonary syndrome before and after liver transplantation
2021, Respiratory MedicineCitation Excerpt :The principal abnormality in HPS is the vasodilatation of the alveolar capillaries which results in an increased amount of blood flowing in normally ventilated alveoli and causes a mismatch in the ventilation to perfusion ratio (VA/Q) and hence arterial hypoxaemia [1]. Hypoxaemia in HPS is also caused by intrapulmonary vascular dilatation and shunting via arteriovenous communications [2–4]. HPS is associated with significant morbidity such as refractory hypoxaemia and need for liver transplantation (LT) [5].
50 Years Ago in THE JOURNAL OF PEDIATRICS: Hepatopulmonary Syndrome: Transplantation Is the Big Difference
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2021, Encyclopedia of Respiratory Medicine, Second EditionAn unusual solitary endobronchial pulmonary arteriovenous malformation in a 10 year-old child
2011, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :The condition is primarily congenital with hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant disorder also known as Osler–Weber–Rendu Syndrome, accounting for 50–70% of all PAVMs [6,7]. Acquired causes include trauma [8], mitral stenosis [8], actinomycosis [8], Fanconi syndrome [9], hepatic cirrhosis [10–14], systemic amyloidosis, schistosomiasis [15], and metastatic thyroid carcinoma [16]. Genetic linkage has been established and PAVMs are attributed to mutations in endoglin (ENG), a TGF-B binding protein, and activin receptor-like kinase (ALK-1), a serine-threonine kinase that binds TGF-B [17–24].
Hepatopulmonary syndrome: a liver-induced oxygenation defect
2023, ERS MonographTHE PULMONARY CIRCULATION IN LIVER DISEASE
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This investigation was supported in part by a contract from the office of the Surgeon General, United States Army, Washington, D. C.
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From the Departments of Pathology and Medicine, University of Minnesota, School of Medicine, Minneapolis, Minnesota.