LCHAD and HELLP – pro
There are no guidelines that recommend testing women with eclampsia or HELLPP for LCHAD. There is some evidence of an association. Recent studies document that infants born of affected pregnancies can be deficient in one of the enzymes of mitochondrial beta oxidation of fatty acids, long chain 3-hydroxyl-acyl CoA dehydrogenase (LCHAD). Some women affected with AFLP have been shown to be heterozygous deficient for LCHAD, and it appears that any cases of HELLPP are due to an inherited partial deficiency. Some studies show that up to 16% of women with HELLP of Fatty Liver of Pregnancy(FAOD) have babies with LCHAD deficiency.
in beta oxidation of fatty acids, brought out in susceptible women by a fetus that is fully deficient, or by the stress of preeclampsia but not all investigators had been able to confirm this association.
There is no understanding of how the information about LCHAD deficiency affects medical plan for a woman with HELLPP.
Roe, C.R. and Ding, J. Mitochondrial Fatty Acid Oxidation Disorders. In, The Metabolic and Molecular Basis of Inherited Disease. 8th Edition, 2001. Scriver, Beaudet, et al. McGraw-Hill. Chapter 101, pg. 2297-2326.
Maternal-fetal medicine: principles and practice By Robert K. Creasy, Robert Resnik, Jay D. Iams (2003), p. 1130.
Roe, C.R. and Ding, J. Mitochondrial Fatty Acid Oxidation Disorders. In, The Metabolic and Molecular Basis of Inherited Disease. 8th Edition, 2001. Scriver, Beaudet, et al. McGraw-Hill. Chapter 101, pg. 2297-2326.