Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease.

@article{Yang2002ProspectiveSF,
  title={Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease.},
  author={Zi Yang and Jennifer Yamada and Yiwen Zhao and Arnold Strauss and Jamal A Ibdah},
  journal={JAMA},
  year={2002},
  volume={288 17},
  pages={
          2163-6
        }
}
CONTEXT Acute fatty liver of pregnancy (AFLP) and hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome are serious complications of pregnancy. Studies in families with recessively inherited mitochondrial trifunctional protein defects documented an association between these maternal illnesses and fetal deficiency of long-chain 3-hydroxyacyl coenzyme A dehydrogenase; this enzyme resides in the alpha subunit of the trifunctional protein and catalyzes the third step in long-chain… CONTINUE READING

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