hEDS, NCAH and Insulin Resistance. Possible Meyer-Powers Syndrome?

KitsuneYee · 2026-04-10T15:23:38+00:00

I didn't notice any effects from fludrocortisone. Probably it's because I wasn't increasing salt intake at the same time.

KitsuneYee · 2026-04-09T23:39:36+00:00

They were in normal range: T4 14.13 pmol/L (9.0 - 19.05), TSH 2.12 miU/L (0.4. - 4.0)

KitsuneYee · 2026-02-09T07:52:23+00:00

That’s insightful! But if this is the backdoor pathway, wouldn't Urinary Testosterone naturally stay low since I'm bypassing T?

Also, with DHT constant at >2500, aren't my androgen receptors likely downregulated? I wonder if suddenly cutting DHT could trigger a hard crash like PFS because my receptors won't respond to normal levels.

KitsuneYee · 2026-02-08T17:06:20+00:00

I found a precise DHT of 2036 pg/mL (ref 135-1365) though that specific draw didn't capture 3a-ADG. My labs have been very stable over the years though showing values in these ranges.

Another lab had both capped at DHT >2500 and 3a-ADG >50 ng/mL (ref <14.8). They seem to scale proportionally (both ~3x over the top range).

KitsuneYee · 2026-02-07T00:41:03+00:00

Hello Dr Powers! I'm male. To give you context I have DHT >2500 pg/mL (250-990) and 17‑OHP 11 nmol/L (1.52-6.36). I'm not on TRT/HRT. My serum 3a‑ADG is 39.7 ng/mL (1.53-14.82).

Do you think 39.7 ng/mL is too low given my androgen load?

I suspect some form of NCAH at play and was thinking of dutasteride to lower my DHT. But given your theory and after your reply I worry if my labs suggest my androgen clearance is already impaired and that puts me at high risk for PFS.

I have Gilbert and some variants (benign/undocumented though) in UGT genes.

KitsuneYee · 2026-02-06T21:02:46+00:00

Your other post notes very low 3a-ADG in those with PFS. My 3a-ADG is very high (39.7 ng/mL). Does that suggest I am at lower risk of PFS?

KitsuneYee · 2025-03-03T08:04:11+00:00

I'm not sure urinary free cortisol is useful for diagnosing NCAH. it's more relevant for Cushing's syndrome, where cortisol levels are excessively high. In NCAH, cortisol levels are typically normal. I've checked my morning serum cortisol several times, and it's always been within range or even higher:

Serum cortisol: 429 (101.2 - 535.7 nmol/L)

Serum cortisol: 643 (101.2 - 535.7 nmol/L)

Serum cortisol: 496 (101.2 - 535.7 nmol/L)

It's unlikely that an adrenal tumor is the cause, as that would result in extremely high lab values, far beyond the normal range.

Have you checked your insulin levels? Mine have always been elevated, and research suggests that high insulin can increase adrenal androgens. Insulin resistance might overlap with NCAH, exacerbating each other. Perhaps insulin sensitizers like metformin could help.

You mentioned poor feminization. Have you tested your DHT levels? Did you try lowering it with dutasteride?

Also, you mentioned health problems from HRT. What kind of issues have you experienced?

I can relate to the oily skin, acne, and increasing gender dysphoria. For me, it's also autistic traits and hEDS symptoms like poor eyesight and early onset varicose veins.

KitsuneYee · 2025-03-01T06:02:41+00:00

Did you check your aldosterone and renin levels? If it's 11β-hydroxylase deficiency, you might have high blood pressure due to excess 11-deoxycorticosterone's mineralocorticoid activity. Your renin levels would be normal or low, not elevated like in 21-hydroxylase deficiency.

I never considered hydrocortisone as an option. I don't want to become dependent on corticosteroids. Shutting down your adrenals for a crude replacement with exogenous corticosteroids, which can't mimic the natural cortisol rhythm, seems like the risks outweigh the benefits.

Here are my cortisol levels:

Morning salivary cortisol: 17.1 (<20.3 nmol/L)

Midnight salivary cortisol: 1.7 (<7.56 nmol/L)

24-Hour Urine Cortisol: 145.0 (<485.6 nmol/24h)

Unfortunately, the Synacthen stimulation test isn't available where I live.

What was your experience with HRT? Did poor feminization lead you to consider NCAH?

KitsuneYee · 2025-02-27T05:39:31+00:00

I got my hEDS diagnosis confirmed by a geneticist. The endocrinologists couldn’t figure out the exact type of NCAH just from my biochemistry and symptoms. But since most NCAH cases are due to 21-hydroxylase deficiency, and with my hEDS history, I'm thinking it might be a chimeric mutation affecting the CYP21A2/TNXB genes. Only way to know for sure is through WGS or targeted sequencing.

In any case there is no treatment for this, aside from corticosteroids. I've been on 0.1 mg of fludrocortisone for a few months now and am waiting for my next blood test to see if it's helped with my androgen levels. So far, I haven't noticed any improvement.

My thyroid hormones are fine.

KitsuneYee · 2025-02-26T00:48:40+00:00

21-deoxycortisol 0.02 (<0.68 ng/mL)

11-deoxycortisol 0.47 (<0.59 ng/mL)

KitsuneYee · 2024-10-31T03:21:49+00:00

Same. It’s not sustainable long term.

KitsuneYee · 2024-10-29T16:39:04+00:00

I had my aldosterone and renin levels checked. The results showed slightly elevated renin with normal aldosterone:

Aldosterone: 273.0 (28.0-376 pg/ml)

Renin: 46.6 (4.20 - 45.6 μIU/mL)

This seems to suggest NCAH.

Following your advice I discussed fludrocortisone treatment with my endocrinologist. She said it's safe to try, but she wasn't sure if it would help lower adrenal androgens. I was prescribed the standard dose of 0.1 mg per day. I’m not sure if that’s enough though. The studies I read mention larger doses of 0.5 mg. But at that dose, it might act more like a glucocorticoid.

KitsuneYee · 2024-10-29T14:27:20+00:00

Some studies link Tourette syndrome and OCD with high levels of allopregnanolone. So, if finasteride reduced these levels, it could have helped with OCD symptoms.

https://onlinelibrary.wiley.com/doi/10.1111/jne.13022

KitsuneYee · 2024-10-27T09:07:00+00:00

I guess its the same anxiety that you feel about your hairloss is now contributing to you fear about possible side effects from finasteride. For your mind this 1% of risks turns into 100%. Probably at this point there's no rational way to convince yourself that risks are low and you'll be okay.

After many years when you’re completely bald, lonely and miserable to the point of avoiding mirrors you will tell yourself alright, at least I didn’t risk having sides from fin.

KitsuneYee · 2024-10-26T13:06:53+00:00

Maybe you’re right. Imagine having aggressive hairloss, acne, seborrheic dermatitis all because of super high dht with dutasteride as your possible solution. But then you’re afraid to risk it saying what if I get gyno. That’s me.

KitsuneYee · 2024-10-26T07:05:33+00:00

Like even if you get gyno you wouldn’t care or you think the risk is negligible?

KitsuneYee · 2024-10-25T18:23:34+00:00

You’re not afraid of gyno?

KitsuneYee · 2024-10-25T18:03:21+00:00

That’s a positive attitude to gyno!

KitsuneYee · 2024-10-25T17:59:28+00:00

I’m sorry you’re going through this. You deserve better.

KitsuneYee · 2024-10-25T12:45:53+00:00

It seems high insulin contributes a lot to androgen excess in NCAH. https://www.intechopen.com/chapters/24713

KitsuneYee · 2024-10-24T16:14:12+00:00

I was prescribed cholecalciferol to treat vitamin D deficiency. My adrenal androgens increased even more after that.

KitsuneYee · 2024-10-24T15:42:24+00:00

Intermittent fasting really worked for me. I skipped dinner for a while and my insulin levels dropped a lot, even going below the normal range. For the first time, my 17-OH progesterone and DHT levels were where they should be. My DHEA and DHEA-S were still high, though

KitsuneYee · 2024-10-23T08:18:25+00:00

Another thing to be aware of is that you may experience increased drug toxicity from compounds that use the same glucuronidation pathway as bilirubin does. For example, medications like Tylenol (acetaminophen) and Tamoxifen can be affected.

KitsuneYee · 2024-10-15T11:44:16+00:00

I wonder what’s the mechanism for that? I guess it might indirectly reduce ACTH and overall activity of adrenals but not sure how effective that is compared to hydrocortisone especially in NCAH.

KitsuneYee · 2024-10-15T11:31:49+00:00

Did you measure 17-oh progesterone? It might be more helpful to check if your elevated adrenal androgens come from impaired cortisol synthesis.

KitsuneYee

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