Placental steroid sulphatase deficiency: an approach to antenatal care and delivery.

Abstract:

:Placental steroid sulphatase deficiency (SSD) is an X-linked inborn error of metabolism. Congenital X-linked ichthyosis (XLI) is a genetic disorder of keratinisation caused by steroid sulphatase (STS) deficiency, which results in a scaling skin condition in male infants shortly after birth. It may be associated with failed induction of labor and prolonged labor leading to cesarean delivery due to 'cervical dystocia'. We present two cases of congenital ichthyosis. Thorough counselling of women with a previously affected pregnancy during the antenatal period should include discussion about mode of delivery and a critical review of the complexities of prenatal diagnosis in this condition. We propose a clinical management pathway to offer women with a previous pregnancy affected by this rare condition. SIMILAR CASES PUBLISHED: Less than 50 cases reported.

journal_name

Ann Saudi Med

journal_title

Annals of Saudi medicine

authors

Dreyer FE,Abdulrahman GO,Waring G,Hinshaw K

doi

10.5144/0256-4947.2018.445

subject

Has Abstract

pub_date

2018-11-01 00:00:00

pages

445-449

issue

6

eissn

0256-4947

issn

0975-4466

journal_volume

38

pub_type

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