[Deficiency in placental sulfatase. Description of a recent case]. 1976

P Conquy, and M Bedin, and L Cedard, and E Papiernik-Berkhauer

Synthesis of oestrogens occurs, during the greater part of pregnancy, in the placenta starting with two fetal precursors: DHA-S and 16 OH DHA-S. In the case of a deficit in sulphatase, an enzyme which is mainly localised in the placenta, these precursors cannot be transformed so that the level of oestrogens stays very low, without the level of secretion of progesterone being altered. Faced with such hormonal abnormalities it is worth while remembering the diagnosis of placental enzyme deficiency. In fact, this abnormality has no repercussions on the progress of the pregnancy and carries no danger for the fetus. It is therefore unnecessary to carry out untimely treatments thinking: fetal distress or adrenal agenesis be it primary or secondary (due to anencephaly) will occur. The diagnosis depends on simple dynamic tests: the mother receives an injection of free DHA and DHA-S, and enzyme study of the placenta can be confirmed in vitro. There seems to be a link between this abnormality and the sex of the fetus. All cases were followed by the birth of a normal boy. Finally, placental suphatase deficiency is rare, but it would appear that increased usage of hormone levels will reveal a rising number of such cases.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297 Male Males
D010920 Placenta A highly vascularized mammalian fetal-maternal organ and major site of transport of oxygen, nutrients, and fetal waste products. It includes a fetal portion (CHORIONIC VILLI) derived from TROPHOBLASTS and a maternal portion (DECIDUA) derived from the uterine ENDOMETRIUM. The placenta produces an array of steroid, protein and peptide hormones (PLACENTAL HORMONES). Placentoma, Normal,Placentome,Placentas,Placentomes
D010922 Placenta Diseases Pathological processes or abnormal functions of the PLACENTA. Placenta Disorders,Placental Diseases,Disease, Placenta,Disease, Placental,Diseases, Placenta,Diseases, Placental,Disorder, Placenta,Disorders, Placenta,Placenta Disease,Placenta Disorder,Placental Disease
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D003687 Dehydroepiandrosterone A major C19 steroid produced by the ADRENAL CORTEX. It is also produced in small quantities in the TESTIS and the OVARY. Dehydroepiandrosterone (DHEA) can be converted to TESTOSTERONE; ANDROSTENEDIONE; ESTRADIOL; and ESTRONE. Most of DHEA is sulfated (DEHYDROEPIANDROSTERONE SULFATE) before secretion. Dehydroisoandrosterone,Prasterone,5-Androsten-3-beta-hydroxy-17-one,5-Androsten-3-ol-17-one,Androstenolone,DHEA,Prasterone, 3 alpha-Isomer,5 Androsten 3 beta hydroxy 17 one,5 Androsten 3 ol 17 one,Prasterone, 3 alpha Isomer
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D012737 Sex Factors Maleness or femaleness as a constituent element or influence contributing to the production of a result. It may be applicable to the cause or effect of a circumstance. It is used with human or animal concepts but should be differentiated from SEX CHARACTERISTICS, anatomical or physiological manifestations of sex, and from SEX DISTRIBUTION, the number of males and females in given circumstances. Factor, Sex,Factors, Sex,Sex Factor

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