Women Can Fight an Almost Secret Syndrome
By JANE E. BRODY

It may seem hard to believe that a disorder first identified nearly 70 years ago that affects as many as 10 percent of women would still be unrecognized by many doctors and in most of those who have it.

But that is the case with a condition called polycystic ovary syndrome, or PCOS, a bodywide metabolic disorder characterized by abnormal hormone levels that can result in distressing and sometimes life-threatening problems, including infertility, obesity, acne, excessive facial and body hair, diabetes, heart disease and uterine cancer.

The syndrome was first described in 1935 by two American gynecologists, Dr. Irving Stein and Dr. Michael Leventhal, and until recently it was known as the Stein-Leventhal syndrome. It was renamed after tests revealed that in many women with the syndrome, the ovaries are covered with beadlike cysts, representing eggs that failed to mature fully and to be released, as would happen in normal ovulation.

Typically, it can take years and visits to many specialists before a woman with PCOS (pronounced PEE-kose) receives a correct diagnosis, followed by treatment that can relieve symptoms and reduce the risk of serious complications.

In fact, many women first learn the cause of their diverse symptoms when they fail to become pregnant and consult infertility specialists.

To be sure, PCOS is hard to diagnose, with symptoms varying widely. And, out of embarrassment, some women fail to tell their doctors what is bothering them. Even when the symptoms are accurately conveyed, women are often told that the symptoms will go away, that their symptoms are normal and that the problems are caused by stress. PCOS symptoms can start in adolescence but may be noticed any time in a woman's reproductive life.

Hormones in Disarray

The body's network of hormones is a finely tuned system, and when one or more is out of balance with the others, bodywide havoc can result.

With PCOS, there is an imbalance of two pituitary hormones, FSH and LH, which normally stimulate the ovaries to mature and release eggs and form a progesterone-producing mass, the corpus luteum. It in turn sustains an early pregnancy or, if no egg is fertilized, results in menstruation.

But when FSH and LH are out of whack, a woman may not ovulate or menstruate regularly, if at all. This makes PCOS a leading cause of infertility, possibly accounting for as many as half of all cases of female infertility.

In addition, if a woman fails to menstruate regularly, the uterine lining becomes overstimulated by estrogen, which can lead to endometrial cancer.

But an imbalance of reproductive hormones is only part of the problem. Many women with PCOS also have excessive amounts of insulin in their blood because of the resistance of their cells to this hormone.

Insulin's primary task is to maintain a normal blood level of glucose by moving this sugar, produced by the digestion of carbohydrates, into cells that use it for energy. But insulin also fosters the storage of fats, and people with high blood levels of this hormone often gain weight on a normal diet and have a great deal of difficulty losing weight.

Half or more of women with PCOS become obese. When insulin resistance is untreated, the continual pressure on the pancreas to overproduce insulin can lead to Type 2 diabetes.

Even this is not the end of the story. In women with PCOS, blood levels of testosterone are also likely to be elevated, resulting in distressing cosmetic symptoms, like acne that will not respond well to ordinary treatments; dark, coarse facial hair; hair on the abdomen and chest; and male-pattern balding.

Other symptoms include dark overgrown skin at the nape of the neck and around the armpits, which are marks of insulin resistance, and high blood levels of heart-damaging triglycerides and low levels of protective high-density lipoprotein cholesterol, greatly increasing the risk of heart disease and stroke.

PCOS tends to run in families, and there is some evidence that men may also be affected. So when a woman learns she has this condition, it makes sense to test others in the family for hormonal irregularities.

Diagnosis and Treatment
Because of the varied nature of PCOS and because its symptoms may also apply to other serious disorders, like tumors, several procedures may be needed to gain a correct diagnosis. The work-up is best done by an endocrinologist familiar with hormonal disturbances.

A woman should be tested for blood levels of prolactin, thyroid-stimulating hormone, LH and FSH, progesterone, testosterone and another male hormone Dheas (dehydroepiandrosterone sulfate), blood lipids (the two forms of cholesterol and triglycerides), insulin and her ability to process blood glucose (via a glucose tolerance test).

The doctor may also recommend a transvaginal ultrasound examination to reveal ovarian enlargement or cysts or overgrowth of the uterine lining. An endometrial biopsy may be performed to check for cancer.

With a syndrome so diverse, the treatments are also varied. Some trial and error may be involved since different approaches work better in some women than in others.

Many women are helped by birth control pills, particularly the combination oral contraceptives that contain low-androgenic progestins, like Ortho-Cyclen and Ovulen. This regulates the menstrual cycle, suppresses FSH and LH release, lowers testosterone levels (relieving symptoms like acne and excess hair growth), raises the level of the protective cholesterol and protects the uterine lining.

If a woman with PCOS then wishes to become pregnant, she is advised to stop the pill and immediately begin trying to conceive before her hormone levels become abnormal again. The ovulation-stimulating drug Clomid is also often prescribed in such cases.

Those found to be insulin resistant are helped by the insulin-sensitizing medications used to treat Type 2 diabetes, most often metformin (Glucophage) or the newer drugs, pioglitazone (Actos) and rosiglitazone (Avandia).

Treatment of insulin resistance can help a woman who is trying to shed excess weight.

Many women with PCOS have found that in addition to regular exercise, a diet relatively low in carbohydrates helps control weight by reducing the level of insulin the body must produce to process glucose.

But since women with PCOS are already at risk of developing heart disease, overconsuming saturated fats and cholesterol is unwise. Focus instead on[ b]lean meats, fish and poultry[/b] and low-fat dairy products with lots of nonstarchy vegetables and low-calorie (low sugar) fruits, like berries and cantaloupe.

Eat grain products in modest amounts and preferably unrefined — whole wheat breads and cereals, oats and brown rice.

The Emotional Connection

Depression, embarrassment, discouragement, stress, anxiety and feelings of hopelessness are not uncommon among women with PCOS, especially before they receive proper diagnostic work-ups and effective treatments. Many find help in support groups, sometimes through chapters of the Polycystic Ovarian Syndrome Association (on the Web at pcosupport.org). The group can also be reached at P.O. Box 80517, Portland, Ore. 97280 or by phone at 877-775-PCOS (877-755-7267).

A new book, "Living With PCOS" (Addicus Books, $14.95), by Angela Best-Boss and Evelina Weidman Sterling with Dr. Richard S. Legro, contains inspiring stories from affected women and information about diagnosis and treatment.

http://www.nytimes.com/2002/10/08/h....html?tntemail0