Liver disease in mitochondrial disorders.

Liver disease in mitochondrial disorders. Research Abstract Details 

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Liver disease in mitochondrial disorders. Abstract Text:

Way S Lee,Ronald J Sokol,

Liver involvement, a common feature in childhood mitochondrial hepatopathies, particularly in the neonatal period, may manifest as neonatal acute liver failure, hepatic steatohepatitis, cholestasis, or cirrhosis with chronic liver failure of insidious onset. There are usually significant neuromuscular symptoms, multisystem involvement, and lactic acidemia. The liver disease is usually progressive and eventually fatal. Current medical therapy of mitochondrial hepatopathies is largely ineffective, and the prognosis is usually poor. The role of liver transplantation in patients with liver failure remains poorly defined because of the systemic nature of the disease that does not respond to transplantation. Several specific molecular defects (mutations in nuclear genes such as SCO1, BCS1L, POLG, DGUOK, and MPV17 and deletion or rearrangement of mitochondrial DNA) have been identified in recent years. Prospective, longitudinal multicenter studies will be needed to address the gaps in our knowledge in these rare liver diseases.

Liver disease in mitochondrial disorders. Publishing Authors By Initials

WS Lee,RJ Sokol,

For similar nutritional and metabolic diseases: metabolic diseases: mitochondrial diseases research abstracts see: nutritional and metabolic diseases: metabolic diseases: mitochondrial diseases research

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Liver disease in mitochondrial disorders. Journal Published:

PUBLICATION TYPE: Review

Journal: Seminars in liver disease

VOLUME: 27

Page Numbers: 259-73

Journal Abbreviation: Semin. Liver Dis.

ISSN: 0272-8087

DAY: 3

MONTH: Aug

YEAR: 2007

Liver disease in mitochondrial disorders. Information

Number of References: 83

LANGUAGE: eng

NlmUniqueID: 8110297

Liver disease in mitochondrial disorders. Keywords Mesh Terms:

KEYWORDS: Mitochondrial Diseases

MESH TERMS: therapy

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Grant and Affiliation Information for Liver disease in mitochondrial disorders.

AFFILIATION: Department of Paediatrics, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.

Country: United States

AGENCY: United States NIDDK

GRANT: U54DK078377

ACRONYM: DK

MEDLINETA: Semin Liver Dis

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