Wednesday, January 23, 2013

Causes of AFLP

The general cause of AFLP is unknown, but it is believed to be caused by an autosomal recessive Fatty Acid Oxidation Defect (FAOD) known as LCHAD (Long Chain 3-Hydroxyacyl-CoA Dehydrogenase) deficiency in the fetus. LCHAD, an enzyme in the mitochondria, is responsible for the beta-oxidation of  long-chain fatty acids to give energy.

A deficiency of LCHAD would result in the liver's inability to oxidise these long-chain fatty acids, which blocks the body's energy supply. These unoxidised fatty acids may accumulate in the liver, heart and kidneys and disrupt their functions. It also causes the liver to produce acid to break down the fatty acids, which attacks the mother's and fetus' organs.

AFLP may result in a mother who is a heterozygous carrier of the LCHAD enzyme deficiency and whose unborn child has the same condition.
Source: http://www.socialstyrelsen.se/rarediseases/lchaddeficiency

 If left untreated, AFLP can cause liver failure, which may result in a need for a liver transplant or death. Babies whose mothers have AFLP may also die from cardiac arrest and Hypoxic Ischemic Encephalopathy, which cuts of oxygen supply from the brain, leading to extensive brain damage.





References:
The Swedish Information Centre for Rare Diseases.2011. LCHAD deficiency. [ONLINE] Available at:http://www.socialstyrelsen.se/rarediseases/lchaddeficiency. [Accessed 15 January 2013]
Rachel Slaugh. 1995. Discription of Disorders. [ONLINE] Available at: http://www.fodsupport.org/lchad.htm. [Accessed 15 January 2013].
N Engl J Med .1997. Liver Diseases in Pregnancy. [ONLINE} Available at:http://www.nejm.org/doi/full/10.1056/NEJM199701303360514#t=article. [Accessed 15 January 2013]
Kansas- Department of Health and Environment. 2013. Long Chain 3-hydroxyacyl-CoA Dehydrogenase (LCHAD) Deficiency Information for Healthcare Professionals. [ONLINE] Available at:http://www.kdheks.gov/newborn_screening/download/ACT/LCHADD_Info_for_Health_Professionals.pdf. [Accessed 15 January 2013].
Marjorie Nimis. 2013. About AFLP. [ONLINE] Available at:http://www.acutefattyliverofpregnancy.co.uk/pageID_7446872.html. [Accessed 15 January 2013].

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