Liver Transplantation After Bone Marrow Transplantation for End Stage Liver Disease with Severe Hepatopulmonary Syndrome in Dyskeratosis Congenita: A Literature First
| Autor: | Shyam Sunder Mahansaria, Viniyendra Pamecha, Kishore G. S. Bharathy, Sachin Kumar, Senthil Kumar |
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| Jazyk: | angličtina |
| Rok vydání: | 2015 |
| Předmět: |
musculoskeletal diseases
Pathology medicine.medical_specialty Cirrhosis Hepatology business.industry medicine.medical_treatment Bone marrow failure Avascular necrosis Case Report Liver transplantation medicine.disease Chronic liver disease hemic and lymphatic diseases Medicine business Hepatopulmonary syndrome Dyskeratosis congenita Hemochromatosis |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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