Exploiting Phenotypic Plasticity for the Treatment of Hepatopulmonary Shunting in Abernethy Malformation
Autor: | Michael Finch, Michael R. Peterson, Joel E. Lavine, Franklin J. Miller, Michael D. Kuo |
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Rok vydání: | 2010 |
Předmět: |
Male
medicine.medical_specialty Portal Vein business.industry medicine.medical_treatment Portal venous system Signs and symptoms Exercise intolerance medicine.disease Shunt (medical) Surgery Shunting Treatment Outcome Pulmonary Veins Chronic hypoxemia medicine Humans Radiology Nuclear Medicine and imaging medicine.symptom Child Cardiology and Cardiovascular Medicine business Hepatopulmonary syndrome Transjugular intrahepatic portosystemic shunt Hepatopulmonary Syndrome |
Zdroj: | Journal of Vascular and Interventional Radiology. 21:917-922 |
ISSN: | 1051-0443 |
Popis: | An 11-year-old boy presented with exercise intolerance due to chronic hypoxemia. Work-up revealed a diagnosis of hepatopulmonary syndrome (HPS) secondary to a congenital extrahepatic portal-venous shunt (Abernethy malformation). Plasticity in the developing liver was exploited as a strategy for the treatment of HPS. With use of a staged endovascular approach, the portosystemic vascular circuitry was modified in a manner that facilitated progressive growth and development of the severely hypoplastic and underdeveloped intrahepatic portal venous system. After completion of the final procedure, the patient's intrahepatic portal veins were normal in appearance; 2 months later, signs and symptoms of HPS completely resolved. The patient remains free of HPS stigmata after 2 years. |
Databáze: | OpenAIRE |
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