Hypertension in a neonate with 11 beta-hydroxylase deficiency.

Abstract

A female newborn infant with ambiguous genitalia was found to have hypertension (121/82 mm Hg) immediately after birth. The plasma testosterone (T) (0.73 nmol/l), delta 4-androstenedione (delta 4-A) (5.9 nmol/l), dehydroepiandrosterone (DHEA) (8.9 nmol/l), as well as 17 OH-hydroxyprogesterone (17 OHP) (152 nmol/l) were elevated. The diagnosis of 11 beta-hydroxylase deficiency was finally established on the basis of elevated plasma eleven-deoxycortisol (compound S) (greater than 0.6 mumol/l) and confirmed by the normalisation of the blood pressure during hydrocortisone therapy. Our case is probably the youngest patient with 11 beta-hydroxylase deficiency in whom the hypertension was found at birth.

• Authors: M Mimouni, H Kaufman, A Roitman, C Morag, N Sadan
• Journal: European journal of pediatrics (Eur J Pediatr) Vol. 143 Issue 3 Pg. 231-3 (Jan 1985) ISSN: 0340-6199 [Print] Germany
• PMID: 3872797 (Publication Type: Case Reports, Journal Article)

Chemical References

• Steroid Hydroxylases
• Hydrocortisone

Topics

• Adrenal Hyperplasia, Congenital (drug therapy, etiology)
• Female
• Humans
• Hydrocortisone (therapeutic use)
• Hypertension (drug therapy, etiology)
• Infant, Newborn
• Steroid Hydroxylases (deficiency)