title: Reversal of severe hepato-pulmonary syndrome in congenital hepatic fibrosis after living-related liver transplantation.
abstract: 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.
authors.affiliation: Department of Gastroenterology, Jaslok Hospital and Research Center, Mumbai, India. nagral@vsnl.com
authors.forename: Aabha
authors.initials: A
authors.lastname: Nagral
authors.forename: Fazal
authors.initials: F
authors.lastname: Nabi
authors.forename: Abhi
authors.initials: A
authors.lastname: Humar
authors.forename: Sanjay
authors.initials: S
authors.lastname: Nagral
authors.forename: Nilesh
authors.initials: N
authors.lastname: Doctor
authors.forename: S R
authors.initials: SR
authors.lastname: Khubchandani
authors.forename: Y K
authors.initials: YK
authors.lastname: Amdekar
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D020065:Hepatopulmonary Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D008103:Liver Cirrhosis / Q000151:congenital* / Q000000981:diagnostic imaging / Q000601:surgery; D016031:Liver Transplantation; D019520:Living Donors; D008168:Lung / Q000000981:diagnostic imaging; D011877:Radionuclide Imaging; D013668:Technetium Tc 99m Aggregated Albumin; D016896:Treatment Outcome
chemical_list: D013668:Technetium Tc 99m Aggregated Albumin