Reversal of severe hepato-pulmonary syndrome in congenital hepatic fibrosis after living-related liver transplantation.
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2007-03-15
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We report a 5-year-old girl with congenital hepatic fibrosis who presented with clubbing and cyanosis. Partial pressure of oxygen was 40 mmHg with oxy-gen saturation of 70% on room air, which improved to 128 mmHg and 92% on inhalation of 100% oxygen. Macroaggregated albumin scan showed 58% shunting to the brain, suggestive of severe hepatopulmonary syndrome. Echocardiogram and pulmonary angiogram ruled out pulmonary hypertension. Four weeks after living-related liver transplantation, she had normal blood gases and reduction in shunting to 7% on macroaggregated albumin scan.
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Nagral A, Nabi F, Humar A, Nagral S, Doctor N, Khubchandani SR, Amdekar YK. Reversal of severe hepato-pulmonary syndrome in congenital hepatic fibrosis after living-related liver transplantation. Indian Journal of Gastroenterology. 2007 Mar-Apr; 26(2): 88-9