Nagral, AabhaNabi, FazalHumar, AbhiNagral, SanjayDoctor, NileshKhubchandani, S RAmdekar, Y K2007-03-152009-05-292007-03-152009-05-292007-03-15Nagral 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): 88-9http://imsear.searo.who.int/handle/123456789/65122We 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.engBrain --radionuclide imagingChild, PreschoolFemaleFollow-Up StudiesHepatopulmonary Syndrome --complicationsHumansLiver Cirrhosis --congenitalLiver TransplantationLiving DonorsLung --radionuclide imagingTechnetium Tc 99m Aggregated Albumin --diagnostic useTreatment OutcomeCase Reports