Diabetes of bearded women – PCOS 2

Close-up of female suffering menstrual pain in lower abdomen

Polycystic ovarian syndrome (PCOS) has only been considered a disease in the last century, but it is actually an ancient disorder. Originally described as a gynecological curiosity, it has evolved into the most common endocrine disorder of young women, involving multiple organ systems.

In ancient Greece, the father of modern medicine, Hippocrates (460 BC–377 BC), described “women whose menstruation is less than three days or is meagre, are robust, with a healthy complexion and a masculine appearance; yet they are not concerned about bearing children nor do they become pregnant”. This description of PCOS existed not only in ancient Greece, but is found in ancient medical texts throughout the world.

Soranus of Ephesus (c.98–138 AD), near modern day Turkey, observed “that the majority of those (women) not menstruating are rather robust, like mannish and sterile women”. The renaissance French barber surgeon and obstetrician Ambroise Paré (1510–1590 AD) noted that many infertile women with irregular menses are “stout, or manly women; therefore their voice is loud and bigge, like unto a mans, and they become bearded”. It’s quite an accurate description from a doctor who can apparently cut your hair, cut your leg off, or deliver children.

The Italian scientist Antonio Vallisneri connected these masculinizing features with the abnormal shape of the ovaries into a single disease. He described several young, married infertile peasant women whose ovaries were shiny with a white surface and the size of pigeon eggs

In 1921, Achard and Thiers described a syndrome whose main features included masculinizing features (acne, balding or receding hairline, excessive facial hair) and type 2 diabetes. Further cases in 1928 cemented the link between what is now called PCOS with type 2 diabetes and described in the classic article ‘Diabetes of Bearded Women’.

Careful observation had already revealed to these astute clinicians a syndrome whose main features included menstrual irregularities (now known to be anovulatory cycles), infertility, masculine features (hair growth), and stoutness (obesity) with its related type 2 diabetes. The only essential feature they missed from the modern definition of PCOS was the multiple cysts on the ovary, because of the lack of simple non-invasive imaging.

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The modern era

Drs. Stein and Leventhal ushered in the modern era of PCOS in 1935 with their description of seven women with all the current diagnostic features – masculinizing features, irregular menses and polycystic ovaries. The breakthrough occurred by making the connection between the lack of menstruation with the presence of enlarged ovaries and merging them into a single syndrome – PCOS. At that time, the detection of enlarged cystic ovaries was difficult and Stein and Leventhal achieved this either by direct surgical observation (laparotomy) or using a now-defunct x-ray technique called pneumoroentgenography. This procedure involved making an abdominal incision to introduce air and then taking x-rays. The shadow of the enlarged ovary could now be seen. However, in an era prior to effective antibiotics, this was a risky procedure.

Dr. Stein hypothesized that some as yet undetermined hormonal imbalance caused the ovaries to become cystic and he suggested that surgically removing a wedge of the ovary may help reverse the syndrome. And indeed, this crude surgery worked. All seven women began to menstruate again and two even got pregnant. With its main features defined, interest in PCOS surged as reflected by the large increase in PCOS articles in the medical literature.

Subsequently, Drs. Stein and Leventhal performed ovarian wedge resection on another 75 women with restoration of menstrual cycles in 90% of cases and restored fertility in 65%. Defining the syndrome and delineating a reasonable treatment was such an accomplishment that this disease became known as the Stein-Leventhal Syndrome. With the advent of modern medical solutions, particularly the medication clomiphene citrate, the ovarian wedge resection today is rarely done.

Through the 1960s and 1970s, improved radioimmunoassay techniques allowed for easier detection of the typical hormonal abnormalities of PCOS. The masculine appearance was caused largely by excessive male sex hormones called androgens, of which testosterone is the best known. The biochemical diagnosis of PCOS is problematic because androgen levels are only modestly elevated and unreliable due to their variation throughout the day and throughout the menstrual cycle. However, the effect of excessive androgens is obvious in the masculinizing features of these women (acne, male pattern baldness, facial hair growth), but measuring these androgens is not as useful for the diagnosis of PCOS as you may have thought.

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By the 1980s, the increasing availability of real-time ultrasound revolutionized the diagnosis of PCOS. Laparotomy was no longer necessary to confirm the enlargement of the ovaries. In 1981, Swanson standardized the definition of polycystic ovaries on ultrasound allowing researchers to easily compare cases. Further refinements included the introduction of trans-vaginal ultrasound which is far superior for detection of ovarian cysts. This technology soon made clear that many otherwise normal women also had multiple cysts on their ovaries. Almost ¼ of the population had polycystic ovaries without any other symptoms. Thus, it is important to distinguish between the mere presence of polycystic ovaries, and polycystic ovary syndrome (PCOS).

The 1980s also saw a revolution in our understanding of the underlying cause of PCOS. The disease had originally been thought to be caused by excessive exposure of female fetuses to androgens, but this hypothesis was ultimately refuted. Instead, studies increasingly linked PCOS with insulin resistance and hyperinsulinemia. The prefix ‘hyper’ means ‘too much’, and the suffix ‘-emia’ means ‘in the blood’, so the word ‘hyperinsulinemia’ literally means ‘too much insulin in the blood’.

The syndrome was still known by a multitude of different names – polycystic ovaries disorder, a syndrome of polycystic ovaries, functional ovary androgenism, hyperandrogenic, chronic anovulation, polycystic ovarian syndrome, ovarian dysmetabolic syndrome, sclerotic polycystic ovary syndrome and so forth. This significantly hampered scientific progress as researchers did not always know whether they were talking about the same disease.

Standardization of the terms was necessary to move forward in proper identification and diagnosis. The first step was taken at the 1990 National Institutes of Child Health and Human Development (NICHD) Conference on PCOS. At that conference, the consensus criteria included specifically:

  1. Evidence of excess androgens (symptomatic or biochemical) and
  2. Persistent rare or absent ovulatory cycles.

Because these symptoms are not specific to PCOS, other diseases would need to be ruled out. This so-called NIH criteria was a giant leap forward. Proper classification allowed international collaboration between universities and researchers. Interestingly, the NIH criteria does not require evidence of polycystic ovaries, obviously a problem for a disease known as polycystic ovary syndrome.

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In 2003, the second international conference on PCOS was held in Rotterdam, the Netherlands. Two innovative features were added to the consensus criteria becoming now known as the Rotterdam criteria. First, it corrected the seemingly obvious oversight of mentioning that Polycystic Ovary Syndrome patients actually might have polycystic ovaries. It took a mere 14 years to correct that little oversight.

Secondly, PCOS was recognized to represent a spectrum of disease and that not all symptoms may appear in all patients. Thus, only two of three criteria were needed to classify patients as PCOS. This includes:

Hyperandrogenism – from the prefix ‘Hyper’ meaning ‘too much’ and suffix ‘-ism’ meaning ‘a state of’. Hyperandrogenism is literally, a state of too much androgens
Oligo-anovulation – the prefix ‘oligo’ meaning ‘few’ and ‘a’ meaning ‘absence of’. This term means that there are few or no ovulatory menstrual cycles

Polycystic ovaries

In 2006, a further refinement to the criteria was made by the Androgen Excess Society (AES) who recommended that hyperandrogenism be considered the clinical and biochemical hallmark of PCOS. This would be the sine qua nonof PCOS. Without evidence of hyperandrogenism, you simply cannot make the diagnosis. This refinement focused researchers and doctors on the underlying causeof disease, rather than merely the presence or absence of polycystic ovaries. The Rotterdam criteria considered all three of the main elements equal.

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The NIH criteria, being somewhat older, are rarely used today. In 2012, an NIH Expert Panel recommended that the Rotterdam criteria be used for diagnosis. The AES 2006 recommendations are commonly used as well, being fairly similar to the Rotterdam criteria.

It is important to note here that although obesity, insulin resistance and type 2 diabetes are commonly found in association with PCOS, they are not part of the diagnostic criteria.


Dr. Jason Fung

 

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22 comments

Top comments

  1. Robin
    I don't have PCOS, but my sister does. This is the most insulting title for an article. Way to offend and insult women about a sensitive topic.
    Reply: #4
    Read more →
  2. Tricia
    Great article! I have suffered from PCOS since menarche. It was interesting to read the history of studies and thoughts over the years on this disease.

    For those complaining about the title, if you actually read the article you will understand why the title was chosen ;). I often joke around that I am a bearded lady. It sucks A LOT...but the title of this article is not offensive. It actually drew me in to read the article.

    Read more →

All comments

  1. Rosemary
    Does hyperinsulinemia cause PCOS or does PCOS cause hyperinsulinemia? Also, in Hippocrates time weren’t diets relatively lower carb and higher fat? If keto/lchf can help treat PCOS wouldn’t it have been practically unheard of at that time?
    Reply: #21
  2. Robin
    I don't have PCOS, but my sister does. This is the most insulting title for an article. Way to offend and insult women about a sensitive topic.
    Reply: #4
  3. Joanne
    I have a good friend who has PCOS. I was taken aback by the title of the article. These women suffer already with the processes their bodies go thru. More decorum could and should be taken. Clearly, this has been a medical issue for centuries, but has just lately been taken seriously. I don't want to sound like "one of those people" BUT.... if this were a male problem and tables were turned ....I'm sure there would have been hospitals built, for "those poor men, having "female-like" syndrome's". It's a reminder to all, that's it's not for us to judge another's outward presentation. This is a real disease and I have personally watched my friend, struggle for years, to try and keep her weight down, spend thousands of dollars on laser hair removal ( so she won't look like a bearded lady!) I hope more research dollars can and will be put towards this issue. If a 1/4 of women have this, and a smaller percentage present with the outward physical appearance's PCOS. I was going to share this article, but the title just yanked my chain too much!
  4. Lynda
    I agree, completely insensitive
  5. Ify
    I am having high androgen level which has made me have full facial hair and because of it i have lost self confidence.
    Reply: #12
  6. Sue
    I am also a sufferer of PCOS, am I the only one who love the first part of the article? I am surprised that it has been cited so far back?

    Keto restarted my periods for about two months and they didn’t return (not that I am moaning!) but my hair has absolutely reduced and especially on my face.

    Veet will go bankrupt if they’re not careful! Lol

    Thank you for this article, very interesting read :)

    Rebecca

  7. ross
    I deal with a number of young woman who suffer from PCOS and all are addicted to junk food and convenient food. It might be a sign of the times but not one of the young people I am dealing with knows or wants to cook proper food. It is all too easy to go out and buy a ready meal, pop into a fast food joint or eat a processed sandwich that has sat on a shelf for 4 days. All is also consider themselve to be fussy eaters....they don't trust proper food, would never eat chicken on a bone or fish that has skin, but happy to eat processed food as long as it has no resemblance to where it orientated from. In an image conscience world maybe for these people a few home truths is not such a bad thing.
    For me the main problem is still that the guidelines given out by doctors and health workers is still a very boring low fat diet. From my own 56 years experience these diets do not work long term and the cravings for carb always get you in the end. A little bit of everything is very difficult to sustain as the bad stuff always creeps in and as we all know naughty is so nice! LCHF diets work as they not only cut the carb cravings, everything tastes sweeter naturally, you eat far less naturally and the health benefits are amazing.....Simple really but not reinforced by our health practitioners.
  8. Tricia
    Great article! I have suffered from PCOS since menarche. It was interesting to read the history of studies and thoughts over the years on this disease.

    For those complaining about the title, if you actually read the article you will understand why the title was chosen ;). I often joke around that I am a bearded lady. It sucks A LOT...but the title of this article is not offensive. It actually drew me in to read the article.

  9. Patricia Rodriguez
    My 17-year-old daughter has PCOS. Her symptoms appear when she was about 12, We had no idea what was going on and watched as her weight increased, she grew hair all over her body, and her skin broke out.

    Luckily a nurse commented that it looked like my daughter has PCOS

    We took her to a very caring endocrinologist who treated all of my daughter's symptoms.

    My daughter dropped more than 20 pounds in three months, her skin cleared and she stopped growing hair on her face, back and bottom.

    She takes a series of medications, but it is so worth it. Her last blood test revealed that her testosterone levels are normal for the first time.

    Perhaps the key to living with this is good medical help, diet and exercise. I'm just happy my daughter feels "normal" now.

    Reply: #17
  10. Shannon
    Can you elaborate on how Keto/Intermittent fasting could improve this problem?

    I've had PCOS for 25+ years, resulting in a complete hysterectomy at 43. My facial hair growth hasn't diminished at all after the hysterectomy.

    Will Keto/Intermittent fasting improve this, or do you recommend anything else that can improve this? It really sucks!

  11. Delaney
    My gosh, people, don't be so sensitive! I have PCOS--I'm 36 and I was diagnosed with it at 16 due to increased hairiness, no periods whatsoever at that point, and struggle to lose weight no matter what. Not only did the title of the article NOT OFFEND ME (because I had a hunch that the circus bearded ladies probably did suffer from PCOS but it wasn't known at the time), but I found the article interesting.

    Like Sue (I think her name was) and Tricia, I think the history of things is pretty fascinating...it was being documented long before doctors even had the ability to do intrusive procedures.

    I was told, at 16, I'd never have kids. I have two, and my younger one came after I had a LEEP procedure to remove a part of my cervix because it had pre-cancerous cells. My doctor told me my fertility was plummeting faster than normal and if I was going to have more kids, I needed to do it soon.

    I still struggle with weight, but low-carb eating enables me to lose anything I do gain pretty quickly. I am also able to exercise like I never have before--even when I was in high school playing sports. I still shave--a lot--but this WOE has helped reduced some of it, I think. And my skin has never been better!!

    Good article. Thank you for publishing it.

    Reply: #22
  12. Delaney
    Something that has helped me a lot--you can buy little electric facial razors at a drug store. I bought one and use it on my entire face and I love it. Helps my foundation go on smoother and you can't see all my whiskers in the light.
  13. Anne Mullens
    Shannon - there are a number of articles about PCOS on the Diet Doctor site, including these two:
    https://www.dietdoctor.com/low-carb/benefits/pcos
    and https://www.dietdoctor.com/low-carb/benefits/pcos/8-reasons.
    The short answer to your question is that PCOS is highly linked to insulin resistance and both intermittent fasting and the LCHF or ketogenic diet lowers insulin.
    Even women, who are past reproductive age but have PCOS in their medical history -- like me -- find great improvement in general health and symptoms on the LCHF diet.
    I even wrote about how life-long achy muscles with exercise disappeared on the diet - a complete surprise to me.
    https://www.dietdoctor.com/keto-diet-happy-muscles-running-fat.
    Good luck exploring the diet. I hope it gives you similar improvements that I and others with PCOS have found.
  14. Marisol
    I was diagnosed with PCOS in my late 20’s while my husband and I struggled with infertility. My fertility Dr. Suggested I follow a low carb higher protein diet to help me lose weight and increase my chances of pregnancy during our IVF trials. I did get pregnant and a few years later when we went back to try for another pregnancy our previous Dr. Had left the practice. The next Dr did not suggest losing weight and when I asked her about it she waved me off saying “Well it could help but most patients won’t do it”. I was shocked, instead of being encouraging, she was rudely dismissive and UN encouraging. Luckily I took her on as a challenge. Lost some weight and did get pregnant. But of course gained it all back and then some with pregnancy. Now, I know I’m losing the weight for good through Keto and IF. Thanks for this interesting article, and I don’t mind the title at all. Thanks Dr Fung!
  15. Betty-Anne
    Grandma, sister, daughter and myself have all had PCOS at various levels - so it must be genetic? I have had a hysterectomy and I have a low level Diabetes 2 - do I not take my diabetic meds when I am fasting? Tried full Keto and gained weight, sugar count went from 6 to 9 -- I also have hypothyroidism and need some carbs to keep my thyroid working. Interesting article - did get pregnant without problems, menzes were unpredictable and extremely painful and often flooded. I have a nono and tweezers for facial hair.
  16. Rose
    I had a hysterectomy with a very large benign tumor on utritus and one ovary at the age of 28. I am overweight and diabetic but not yet taking insulin. Also have a lot of facial hair, which I hate. Trying the keto diet.It has made my a1c go up a lot but I want to stick with it to try to loose weight. I am 63 now.
  17. Kira
    I am so glad to hear that she found a great doctor! I am 36 and was diagnosed with PCOS at 13...and the doctors didn't know anything really about it. I remember my OBGYN coming in and saying to me that with my hormone levels he was surprised I didn't have a deep voice and beard. I struggled...and I mean struggled my whole life trying to keep the weight off, dealing with severe cystic acne, severe insulin resistance, facial hair, and the list goes on to no avail. I've only been on the keto diet for 10 days, but it has changed my life. I've lost 9lbs, feel amazing for the first time in my life...and my symptoms are improving more every day. It took me 20+ years to get to this point so it makes me THRILLED that young girls are getting the real help and advice they need at an early age and diagnosis!!
  18. Kama
    You all quit complaining about the title. This is not all about you and your oversensitivity. This is an excellent article about a sensitive subject, all women should read this.
  19. Sammy
    I'm fascinated by hormones and neurotransmitters and the way they impact on our diets. The answer to most of our female issues lay in our food and lifestyle (except maybe endometriosis). If you are one of the bearded ladies then ensure you supplement with B6, magnesium and taurine as well as fast.
  20. Sam
    I enjoyed the title and the article! It's telling it as it is. I do appreciate that.
  21. Mc
    From what I’ve learned over the years they make the other worse, but pcos is the underlying factor as it causes the hormone imbalance in the first place. Also at that stage in history diets were high in carbs, especially for peasants. They were past the hunter/ gatherer stage.
  22. Stacie
    Thank you, Delaney! I completely agree!! My daughter has PCOS, and I wasn’t offended in the least. People are too sensitive and easily offended these days. This article is quite informative and meant to be helpful - their whole website is here to inform and help us, not to mock people with conditions like PCOS.

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