A case report of congenital glycogen storage liver cirrhosis treated with bone marrow derived stem cells.
Autor: | Wehbe TW; Department of Hematology, The Notre Dame University Hospital, Jounieh, Lebanon., Abi Chahine NH; Department of Neurosurgery, The Lebanese-Canadian Hospital, Beirut, Lebanon., Annous AA; Hanan Lebanese-French Laboratory for Pathology, University of Balamand and Open Arab University, Balamand Al Kurah, Lebanon., Ferri MA; Al-Salam Hospital, Department of Gastroenterology, Tripoli, Lebanon., Boulous RC; Department of Pathology, The Notre Dame University Hospital, Beirut, Lebanon., El-Mestrah MF; The Lebanese University, Beirut, Lebanon. |
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Jazyk: | angličtina |
Zdroj: | Stem cell investigation [Stem Cell Investig] 2017 Sep 07; Vol. 4, pp. 73. Date of Electronic Publication: 2017 Sep 07 (Print Publication: 2017). |
DOI: | 10.21037/sci.2017.07.04 |
Abstrakt: | 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. Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare. |
Databáze: | MEDLINE |
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