CTICasesHepatopulmonary Syndrome in Dyskeratosis Congenita
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Imaging findings
Numerous abnormal, dilated vessels in the mediastinum extending into the hila and out to the pleural surfaces, indicative of hepatopulmonary syndrome. A nuclear medicine shunt study showed a 68% shunt into kidneys and brain.
Key takeaways
Hepatopulmonary syndrome can occur in patients with dyskeratosis congenita, even without obvious liver dysfunction, presenting with profound hypoxemia and orthodoxia. The mechanism of pulmonary shunting in liver disease remains mysterious, possibly involving 'evil humors' or vasoactive compounds.
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