[Clinical and biochemical studies in three pregnancies with placental sulphatase deficiency (author's transl)].
Three pregnancies (two I. parae and one II. para) with abnormally low urinary estrogen excretion of less than 5 mg estrogens per day have been investigated in this study. After intravenous injection of 100 mg DHA-S to the mothers no increase of estrogen production was observed. The level of human placental lactogen, was found within normal range. Ultrasonographic measurement of fetal size and monitoring of the fetal heart rate have demonstrated fetal benefit. At the end of pregnancy the cervix of both did not dilate after infusion of Syntocinon. Healthy male infants were delivered by Caesarean sections in both cases. The II. para, however, developed spontaneous labour and delivery at term. Clinical and laboratory tests have indicated the possibility of rare placental sulphatase deficiency. After delivery the placental steroid sulphatase activity was measured as the rate of hydrolysis of (6,7-3H) oestrone-sulphate or (4-14C) dehydroepiandrosterone-sulphate during in vitro incubation with the homogenate or the microsomal fraction from the pathological placentae. In all three cases the specific activity of 3-sulphatase was considerably (more than 80%) decreased when compared to three normal placentas, whereas the activity of the other enzymes (17beta-hydroxysteroidoxidoreductase, delta4/5-isomerase, aromatizing enzyme system) was found within the normal range.
D000328:Adult; D002585:Cesarean Section; D003687:Dehydroepiandrosterone; D004967:Estrogens / Q000652:urine; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D007744:Obstetric Labor Complications / Q000150:complications; D010920:Placenta / Q000201:enzymology*; D010922:Placenta Diseases / Q000175:diagnosis*; D010928:Placental Lactogen / Q000097:blood; D011247:Pregnancy; D011248:Pregnancy Complications / Q000097:blood / Q000175:diagnosis* / Q000652:urine; D013429:Sulfatases / Q000172:deficiency*
D004967:Estrogens; D003687:Dehydroepiandrosterone; D010928:Placental Lactogen; D013429:Sulfatases
Klinische und biochemische Untersuchungen bei drei graviden Patientinnen mit placentarem Sulfatasemangel.