ISSN: 1308-5727 | E-ISSN: 1308-5735
Volume: 16 Issue: 1 Year: 2024
Forms

Abstracting & Indexing
Turkish Society for Pediatric Endocrinology and Diabetes
A Giant Ovarian Cyst in a Neonate with Classical 21-Hydroxylase Deficiency with Very High Testosterone Levels Demonstrating a High-Dose Hook Effect [J Clin Res Pediatr Endocrinol]
J Clin Res Pediatr Endocrinol. 2012; 4(3): 151-153 | DOI: 10.4274/Jcrpe.685

A Giant Ovarian Cyst in a Neonate with Classical 21-Hydroxylase Deficiency with Very High Testosterone Levels Demonstrating a High-Dose Hook Effect

Tülay Güran1, Gözde Yeşil2, Ömer Güran3, Suna Cesur4, Oktav Bosnalı5, Ayşenur Celayir5, Sevilay Topçuoğlu6, Abdullah Bereket1
1Marmara University, Pediatric Endocrinology And Diabetes, Istanbul, Turkey
2Zeynep Kamil Maternity And Childrens Diseases Research And Training State Hospital, Genetics, İstanbul, Turkey
3Şisli Etfal Research And Training State Hospital, Neonatal Intensive Care Unit, İstanbul, Turkey
4Zeynep Kamil Maternity And Childrens Diseases Research And Training State Hospital, Pathology, İstanbul, Turkey
5Zeynep Kamil Maternity And Childrens Diseases Research And Training State Hospital, Pediatric Surgery, İstanbul, Turkey
6Zeynep Kamil Maternity And Childrens Diseases Research And Training State Hospital, Neonatal Intensive Care Unit, İstanbul, Turkey

Congenital adrenal hyperplasia (CAH) is a group of disorders affecting the adrenal steroid synthesis. The most common form, 21-hydroxylase deficiency (21-OHD), leads to decreased production of cortisol and aldosterone with increased androgen secretion. In classic CAH, glucocorticoid treatment can be life-saving and serves to bring the symptoms under control. However, the treatment challenge is to effectively control the excess androgen effect by using the lowest possible glucocorticoid dose. Previous studies suggested a relationship between ovarian cyst formation and adrenal androgen excess, but neonatal large ovarian cysts have been very rarely reported in newborns with CAH. Here, we present the unique case of a neonate with classical 21-OHD who underwent surgery for a giant (10x8x7 cm) unilateral solitary ovarian follicular cyst on the 2nd postnatal day. Hormonal evaluation of the patient revealed high-dose hook effect for serum testosterone levels for the first time by a two-site immunoradiometric assay. Possible mechanisms by which androgen excess may cause ovarian cyst formation are discussed.

Keywords: 21-hydroxylase deficiency,Ovarian cyst,testosterone,ambiguous genitalia,hook effect,Neonate

Tülay Güran, Gözde Yeşil, Ömer Güran, Suna Cesur, Oktav Bosnalı, Ayşenur Celayir, Sevilay Topçuoğlu, Abdullah Bereket. A Giant Ovarian Cyst in a Neonate with Classical 21-Hydroxylase Deficiency with Very High Testosterone Levels Demonstrating a High-Dose Hook Effect. J Clin Res Pediatr Endocrinol. 2012; 4(3): 151-153
Manuscript Language: English
LookUs & Online Makale