Article Abstract

A case report of congenital glycogen storage liver cirrhosis treated with bone marrow derived stem cells

Authors: Terek W. Wehbe, Nassim H. Abi Chahine, Abdul-Rahman A. Annous, Mohammad A. Ferri, Robert C. Boulous, Majid F. El-Mestrah

Abstract

Liver cirrhosis represents a state of end-stage failure that is usually fatal. The condition results in liver dysfunction, recurrent ascites, encephalopathy, renal failure, splenomegaly, bleeding, and a poor quality of life in general. With the current severe shortage of donated organs, the only available treatment in the developing countries remains palliative care. We report a case of congenital metabolic liver cirrhosis due to glycogen storage disease diagnosed at age eight. The patient, a male, received bone marrow derived mononuclear cells (BMMC) at age 16 and again at age 17 with significant improvement of his biochemical liver function tests, ascites build-up, asthenia, splenomegaly and quality of life. Furthermore, liver biopsies showed clear reduction of the inflammation and fibrosis from Ishak score dropped from 3 to 1 paralleling the symptomatic improvement of the patient.

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