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

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.

Full Text:

sci-04-2017.07.04

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