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INTRODUCTION

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Polycystic ovarian syndrome (PCOS) is a common endocrinopathy typified by oligoovulation or anovulation, signs of androgen excess, and multiple small ovarian cysts. These signs and symptoms vary widely between women and within individuals over time. Women with this endocrine disorder also have higher rates of dyslipidemia and insulin resistance, which increase long-term health risks. As a result, women with PCOS may first present to various medical specialists, including pediatricians, gynecologists, internists, endocrinologists, or dermatologists.

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DEFINITION

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Polycystic Ovarian Syndrome

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In Rotterdam, The Netherlands, PCOS was redefined in a consensus meeting between the European Society of Human Reproduction and Embryology and the American Society for Reproductive Medicine (ESHRE/ASRM)—The Rotterdam ESHRE/ASRM-Sponsored PCOS Consensus Workshop Group, 2004. As shown in Table 17-1, affected individuals must meet two out of three criteria. Importantly, because other etiologies, such as congenital adrenal hyperplasia, androgen-secreting tumors, and hyperprolactinemia, may also lead to oligoovulation and/or androgen excess, these must be excluded. Thus, PCOS currently is a diagnosis of exclusion.

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Table Graphic Jump Location
TABLE 17-1Definition of Polycystic Ovarian Syndrome
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The Rotterdam criteria constitute a broader spectrum than that formerly put forward by the National Institutes of Health (NIH) Conference in 1990 (Zawadzki, 1990). The prominent difference is that the NIH Conference defined PCOS without regard to ovarian sonographic appearance. Last, a third organization—The Androgen Excess and PCOS Society (AE-PCOS)—has also defined criteria for PCOS (Azziz, 2006). As is shown in Table 17-1, criteria are similar among these three groups, and controversy exists as to which is most appropriate.

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Ovarian Hyperthecosis and HAIRAN Syndrome

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Ovarian hyperthecosis, often considered a more severe form of PCOS, is a rare condition characterized by nests of luteinized theca cells distributed throughout the ovarian stroma. Affected women exhibit severe hyperandrogenism and may occasionally display frank virilization signs such as clitoromegaly, temporal balding, and voice deepening (Culiner, 1949). In addition, a much greater degree of insulin resistance and acanthosis nigricans typically is found (Nagamani, 1986).

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The hyperandrogenic-insulin resistant-acanthosis nigricans (HAIRAN) syndrome is also uncommon and consists of marked hyperandrogenism, severe insulin resistance, and acanthosis nigricans (Barbieri, 1983). The etiology of this disorder is unclear, and ...

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