Journal
JOURNAL OF CLINICAL AND EXPERIMENTAL HEPATOLOGY
Volume 5, Issue 4, Pages 344-347Publisher
ELSEVIER - DIVISION REED ELSEVIER INDIA PVT LTD
DOI: 10.1016/j.jceh.2015.10.003
Keywords
chronic liver disease; dyskeratosis congenita; hepatopulmonary syndrome; liver transplantation; microaggregated albumin scan
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Dyskeratosis congenita is a multisystem genetic disorder. Although hepatic involvement is reported in about 7% of patients with dyskeratosis congenita, it is not well characterized and often attributed to hemochromatosis from frequent blood transfusions. A few case reports describe cirrhosis and hepatic cell necrosis in affected individuals in autosomal dominant pedigrees. Bone marrow failure and malignancies are the principal causes of death in dyskeratosis congenita. We describe the first case of living donor liver transplantation, in dyskeratosis congenita for decompensated cirrhosis with portal hypertension. The patient also had associated severe hepatopulmonary syndrome, interstitial lung disease, bilateral hip replacement for avascular necrosis of the head of femur, and a past history of bone marrow transplantation for bone marrow failure.
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