Short-chain acyl-coenzyme A (CoA) dehydrogenase (SCAD; MIM 606885) deficiency (SCADD, MIM 201470) is an autosomal recessive inborn error of mitochondrial fatty acid β-oxidation, presenting with a variety of clinical signs and symptoms. Developmental delay, hyper- and hypotonia, ketotic hypoglycemia, and epilepsy are most frequently reported.^1-9 SCAD is the first enzyme of the short-chain fatty acid β-oxidation spiral, which catalyzes the dehydrogenation of butyryl-CoA (C4-CoA).

Plasma hemopexin activity, associated with increased vascular permeability, was evaluated in healthy pregnant and non-pregnant women and in pre-eclamptic women. Hemopexin activity and the hemopexin inhibitor, extracellular ATP, were assayed in plasma from pregnant (n = 10), preeclamptic (n = 9), and non-pregnant women (n = 10) using standard methods. Abdominal fascia tissue fragments from preeclamptic and pregnant women were immunohistochemically stained for vascular ecto-apyrase or ecto-5'nucleotidase. The data show significantly enhanced Hx activity exclusively in plasma from pregnant women and significantly enhanced plasma ATP in pre-eclamptic women compared with the other groups. Dephosphorylation of preeclamptic plasma resulted in reactivation of Hx activity. Fascia tissue-samples from preeclamptic women showed reduced ecto-apyrase activity and enhanced ecto-5'nucleotidase activity compared to pregnant women. Enhanced hemopexin activity may be associated with normal pregnancy, but not with preeclampsia. Decreased hemopexin in pre-eclamptic patients may be due to enhanced plasma ATP, which is possibly promoted by diminished activity of vascular ecto-apyrase.