Reversal of severe hepato-pulmonary syndrome in congenital hepatic fibrosis after living-related liver transplantation.
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.
Nagral
Aabha
A
Department of Gastroenterology, Jaslok Hospital and Research Center, Mumbai, India. nagral@vsnl.com
Nabi
Fazal
F
Humar
Abhi
A
Nagral
Sanjay
S
Doctor
Nilesh
N
Khubchandani
S R
SR
Amdekar
Y K
YK
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
D013668:Technetium Tc 99m Aggregated Albumin