Gonadotropin-resistant ovary syndrome

Gonadotropin-resistant Ovary Syndrome

Gonadotropin-resistant ovary syndrome (GROS), also known as Savage syndrome, is a rare condition characterized by the failure of the ovaries to respond to gonadotropin stimulation. This condition leads to primary ovarian insufficiency, resulting in amenorrhea and infertility in affected women.

Pathophysiology

GROS is primarily caused by a defect in the ovarian receptors for gonadotropins, specifically the follicle-stimulating hormone (FSH) and luteinizing hormone (LH). These hormones are crucial for the normal development and function of the ovaries. In GROS, despite normal or elevated levels of circulating gonadotropins, the ovaries fail to respond, leading to anovulation and estrogen deficiency.

Clinical Presentation

Women with gonadotropin-resistant ovary syndrome typically present with:

• Primary or secondary amenorrhea
• Infertility
• Symptoms of estrogen deficiency, such as hot flashes and vaginal dryness

Diagnosis

The diagnosis of GROS is made based on clinical presentation, laboratory findings, and exclusion of other causes of ovarian insufficiency. Key diagnostic criteria include:

• Elevated serum gonadotropin levels (FSH and LH)
• Low serum estradiol levels
• Normal karyotype (46,XX)
• Exclusion of autoimmune and genetic causes of ovarian failure

Treatment

Management of GROS focuses on addressing the symptoms of estrogen deficiency and infertility. Treatment options include:

• Hormone replacement therapy (HRT) to alleviate symptoms of estrogen deficiency
• Assisted reproductive technologies (ART) such as in vitro fertilization (IVF) with donor oocytes for women desiring pregnancy

Prognosis

The prognosis for women with GROS in terms of fertility is generally poor without the use of donor oocytes. However, hormone replacement therapy can effectively manage symptoms of estrogen deficiency and improve quality of life.

Also see

• Primary ovarian insufficiency
• Follicle-stimulating hormone
• Luteinizing hormone
• In vitro fertilization