Review: Placental sulphatase deficiency

Taylor, Norman

doi:10.1007/bf01800173

Cited by 49 publications

(28 citation statements)

References 36 publications

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“…DHEAS levels are elevated in amniotic fluid (Braunstein et al, 1976;Osathanondh et al, 1976;H~ihnet et al, 1982) but, in most studies, not maternal blood or urine (Osathanondh et aI., 1976;Gips et aI., 1980). In contrast, 16~-hydroxy-DHEAS is normal in amniotic fluid but represents the major maternal urinary sulphated steroid in PSD (Taylor, 1982). In addition to the normal placental cholesterol sulphate levels reported here, preliminary studies suggest normal maternal blood and urine levels (Marinkovic-Ilsen, 1983;Muskiet et al, 1983).…”

Section: Discussionmentioning

confidence: 38%

Cholesterol sulphate in the microsomal sulphatase deficient placenta

Marinkovic-Ilsen

Williams²

1983

J of Inher Metab Disea

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Placental sulphatase deficiency (PSD) and recessive X-linked ichthyosis (RXLI) are known as a nosological entity, due to deficiency of the enzyme, steroid sulphatase. Prior studies have demonstrated high urinary excretion and some accumulation of sulphated steroids prenatally, and of accumulation of cholesterol sulphate postnatally. This study was undertaken to investigate the level of cholesterol sulphate in the sulphatase deficient placenta. Whereas cholesterol sulphate levels are elevated in blood, erythrocyte membrane and stratum corneum of patients with RXLI, cholesterol sulphate content of four investigated sulphatase deficient placentas was normal in comparison to six normal male controls and four male controls with low oestrogen excretion in the third trimester of pregnancy but with normal placental sulphatase activity. The explanation for normal cholesterol sulphate content of sulphatase deficient placentas is unknown but may be due to placental transport, rapid metabolism to other sulphated steroids or age-related differences in residual enzyme activities.

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Section: Discussionmentioning

confidence: 38%

Cholesterol sulphate in the microsomal sulphatase deficient placenta

Marinkovic-Ilsen

Williams²

1983

J of Inher Metab Disea

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“…An important point raised by several au thors is the possible pejorative incidence of the PSD on the mode of delivery. Indeed, the idea that these women should have difficul ties to deliver via the vaginal route, espe cially primiparas, was supported by some reports relating abnormalities in cervical ri pening and a high rate of cesarean section [2,5,13]. Also, the gestation length was consid ered to be increased in case of PSD [2,5,14].…”

Section: Introductionmentioning

confidence: 71%

Incidence of Placental Sulfatase Deficiency on the Mode of Termination of Pregnancy

Bedin

Fournier

Cabrol

et al. 1987

Gynecol Obstet Invest

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The mode of termination of pregnancy in case of placental sulfatase deficiency (PSD) has been considered in a 28-case series from our laboratory. After comparison with 75 other cases collected from the literature and also with reports concerned with normal pregnancies it appears that in the case of PSD: first, the gestation length is not increased; second, the parity of the patients has no influence on the mode of delivery; third, spontaneous vaginal delivery can occur (67.8% in our series, 43% in the literature series); fourth, the failure rate of labor, especially when induced, is higher than in a normal population; fifth, the high rate of C-section, largely due to the failure of labor, is enhanced by untimely interventions mostly in case of isolated PSD. If a pejorative incidence of PSD on the mode of delivery exists it must be at a moderate level, and intervention is unnecessary in absence of associated fetal and/or maternal complications.

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“…Preterm delivery is associated with increased maternal plasma and salivary oestriol, 2 whilst in steroid sulphatase deficiency, where oestriol levels are very low, gestation length is extended. 11 Increased oestrogen production in CAH may therefore counter, to some extent, prolongation of pregnancy due to glucocorticoid deficiency.…”

Section: Discussionmentioning

confidence: 99%