[Successful treatment and pregnancy in a women with the non-classic form of congenital adrenal hyperplasia].
In an 18-year-old woman non-classic 21-hydroxylase deficiency was diagnosed and dexamethasone treatment was instituted. Ten years later, she became pregnant for the first time; at 37 weeks unexpected intrauterine foetal death was found to have occurred. A second pregnancy ended with a spontaneous abortion following a 12-week period of amenorrhoea. At the third pregnancy, the medication was replaced with hydrocortisone as it was suspected that the use of dexamethasone may have played a role in the intrauterine foetal death and the spontaneous abortion. The patient gave birth to a healthy, but dysmature, daughter. Female patients with non-classic congenital adrenal hyperplasia present with signs of androgen excess. Treatment with glucocorticoids reduces the symptoms and restores the menstrual cycle and fertility. Preconceptional advice by a clinical geneticist is recommended, because of the risk of an affected child. If there is no risk of having a child with congenital adrenal hyperplasia, hydrocortisone or prednisone is the treatment of choice during pregnancy as neither cross the placenta.
van den Akker
E S A
ES
Atrium Medisch Centrum, afd. Verloskunde en Gynaecologie, Postbus 4446, 6401 CX Heerlen. evdakker@scarlet.nl
Stikkelbroeck
M M L
MM
Menheere
P P C A
PP
Roumen
F J M E
FJ
Otten
B J
BJ
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000188:drug therapy*; D000328:Adult; D003907:Dexamethasone / Q000009:adverse effects* / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use*; D011247:Pregnancy; D011248:Pregnancy Complications / Q000188:drug therapy / Q000209:etiology*; D011256:Pregnancy Outcome; D016896:Treatment Outcome
D003907:Dexamethasone; D006854:Hydrocortisone