Clinics of Pediatric Endocrinology, University of Health Sciences, Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital, Ankara, Turkey.
Clinics of Radiology, University of Health Sciences, Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital, Ankara, Turkey.
Turk J Pediatr. 2020;62(6):1088-1093. doi: 10.24953/turkjped.2020.06.022.
Preterm ovarian hyperstimulation syndrome (POHS) is an uncommon disorder characterized by prematurity, hypogastric and upper leg swelling, high serum estradiol and gonadotropin levels, and ovarian cysts. Immaturity of the gonadal axis is accepted as the cause. But still, other etiological factors are suspected.
A preterm baby who was born at 24 gestational weeks was referred to our clinic for ambiguous genitalia on day 118 of life. Labia majora and clitoris was edematous. Clitoris length was 1.5 cm. On laboratory evaluation: 17OH-Progesterone: 1.84 ng/ml, dehydroepiandrosterone sulphate (DHEA-S): 139 μg/dl, total testosterone (T.T): 88 ng/dl, luteinizing hormone (LH): 22.5 mIU/l, Follicle stimulating hormone (FSH): 15.7 mIU/l, estradiol (E2): 447 pg/ml. Karyotype analysis was 46, XX. There was a 25x14x12 mm ovarian cyst detected on ultrasound. On follow-up, E2 levels and cyst size increased, and there was 4 mm pericardial effusion on echocardiography at the time.
In this paper, we present a case with POHS and to discuss possible pathophysiological mechanisms and treatment. This is the first case of POHS developing pericardial effusion.
早产卵巢过度刺激综合征（POHS）是一种罕见的疾病，其特征为早产、下腹部和大腿肿胀、血清雌二醇和促性腺激素水平升高以及卵巢囊肿。性腺轴不成熟被认为是其病因。但仍怀疑存在其他病因。
一名 24 孕周的早产儿在出生后第 118 天因生殖器模糊被转至我科。大阴唇和阴蒂肿胀。阴蒂长度为 1.5 厘米。实验室检查：17-羟孕酮：1.84ng/ml，硫酸脱氢表雄酮（DHEA-S）：139μg/dl，总睾酮（T.T）：88ng/dl，促黄体生成素（LH）：22.5mIU/l，卵泡刺激素（FSH）：15.7mIU/l，雌二醇（E2）：447pg/ml。核型分析为 46，XX。超声检查发现一个 25x14x12mm 的卵巢囊肿。随访时，E2 水平和囊肿大小增加，超声心动图检查时发现 4mm 的心包积液。
本文报告了一例 POHS 病例，并讨论了可能的病理生理机制和治疗方法。这是首例发生心包积液的 POHS 病例。
Zotero 插件