Recognising placental steroid sulphatase deficiency

Recognising placental steroid sulphatase deficiency

bad, with variable results in between. Amputation of a digit is sometimes thought to produce a quicker return to work for the patient than replantation, but this is not borne out by published results."' The outlook in the leg is less clear. Functional results are often poor if a leg is replanted. Nevertheless, filleting may produce useful soft tissue which may be used as a vascularised free tra...

Placental sulphatase deficiency.

INTRODUCTION Placental sulphatase deficiency/congenital ichthyosis is an X-linked inborn error of metabolism which was first described in 1969 by France and Liggins.1 It is an enzymatic defect affecting steroid metabolism, clinically manifested by diminished oestrogen production during fetal life and by congenital ichthyosis post-natally. This disorder has a reported incidence of between 1: 6,0...

The steroid sulphatase of Patella vulgata.

Antenatal diagnosis of steroid sulphatase deficiency: case report and literature survey.

Steroid sulphatase deficiency is a recently recognised genetically determined inborn error of metabolism. Originally identified as an enzyme disorder of the placenta (commonly termed placental sulphatase deficiency), it is now known that the progeny of affected pregnancies have a generalised steroid sulphatase deficiency and that the enzyme defect persists throughout life. The disorder is chara...

Steroid Sulphatase, Arylsulphatase and ,b-glucuronidase in Marine Invertebrates

The widespread occurrence of sulphatases in the Mollusca was first demonstrated by Soda & Hattori (1933a, b) who showed that these enzymes are mainly concentrated in the digestive glands. That extracts from marine molluscs also possess ,B-glucuronidase activity was first noted by Dodgson, Lewis & Spencer (1952) who studied the optimum conditions for the activity ofthis enzyme and arylsulphatase...