NDLI: Liver Transplantation After Bone Marrow Transplantation for End Stage Liver Disease with Severe Hepatopulmonary Syndrome in Dyskeratosis Congenita: A Literature First.

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Liver Transplantation After Bone Marrow Transplantation for End Stage Liver Disease with Severe Hepatopulmonary Syndrome in Dyskeratosis Congenita: A Literature First.

Content Provider	Europe PMC
Author	Mahansaria, Shyam Sunder Kumar, Senthil Bharathy, Kishore G.S. Kumar, Sachin Pamecha, Viniyendra
Copyright Year	2015
Abstract	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.
Related Links	https://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC4723656&blobtype=pdf
ISSN	09736883
Volume Number	5
DOI	10.1016/j.jceh.2015.10.003
PubMed Central reference number	PMC4723656
Issue Number	4
PubMed reference number	26900277
Journal	Journal of Clinical and Experimental Hepatology [J Clin Exp Hepatol]
e-ISSN	22133453
Language	English
Publisher	Elsevier
Publisher Date	2015-11-12
Access Restriction	Open
Rights License	© 2015, INASL.
Subject Keyword	BMF, bone marrow failure DKC, dyskeratosis congenita HPS, hepatopulmonary syndrome LDLT, living donor liver transplantation MAA, microaggregated albumin scan PHT, portal hypertension SCE, saline contrast echocardiography chronic liver disease dyskeratosis congenita hepatopulmonary syndrome liver transplantation microaggregated albumin scan
Content Type	Text
Resource Type	Article
Subject	Hepatology

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