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Suggest Regarding Growth And Development In Cdgp Patient

Posted on Fri, 12 Oct 2012

Question: I have a question regarding growth and development in cdgp boys, specifically the hypothalamic-pituitary-gonadal axis.

I understand that a rhythm for this axis is present even before the clinical onset of puberty. Is this true also for CDGP boys?

i was 14.5 and startded 3 month oxandrolone . nothing happened and then at 15.0 i started another 3 month course. Right in the beginning of this secoind course i acheived rapid sexual maturation and rapid growth spurt (9cm by 15.3) . My testicles were hanging much klower during this time and i think i may been in XXXXXXX stage 3 . Another question: I understand onset of puberty starts 2 years or so before the changes i just described....does this apply to CDGP boys also?

Thanks and Regards,

XXXXXX

Answered by Dr. Anantharaman (2 hours later)

Hi,
Thanks for writing in.
Normal onset of puberty is very wide in boys after 9 years upto 14 years is considered normal. The first sign of puberty is increase in testicular volume.usually before this is detected the FSH and LH increase to above 0.3IU/l testosterone at this time is about 20ng/do.there are some other tests we can do inhibinB , hCG stimulated testosterone,GnRH stimulated LH etc.the tests will all show higher values in those with impending puberty but are expected to be negative in Hypogonadotropic hpogonadism. In reality there is a wide range of CDGP presentations and some times values can be so low with tests that they may mislead.There are some other clinical clues to CDGP some delay in bone age,low height centimes from early childhood,delayed adrenarche

Regarding the tempo of puberty that again is variable it is slower in those with earlier onset and faster in those with later onset.

Your development is highly suggestive of CDGP but I treat my patients older than 14 years with testosterone because producing some secondary sexual characteristics is one of the goals of treatment.CDGP tends to be familial.
In the longer run there is no difference in reproductive/sexual outcomes for persons with CDGP

I hope that answers your query feel free to write back for any more info
Regards
anantharaman

Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar

Follow up: Dr. Anantharaman (17 hours later)

Thank you for your answer Dr Anantharaman,

I want to followup on area of first signs of puberty and the background hormone levels. You mentioned that increase in testicular size is a sign of puberty but to what size roughly? Also isnt FSH involved in increasing testicular size and actual true therefore doesnt include LH and gonadal testosterone production OR does FSH presence mean LH is present also as the work synergistically?

Please allow me to explain my story:

I remember having some blood tests prior to my treatment when aged 14.6. The doctor said you are 'slow' maturing and maybe i have to wait long time for growth spurt 'we dont know' so she advised i take this oxandrolone and if i go into pubertal growth spurt then doesnt matter - i remember she mentioned my hormones are lazy. But it was such a LONG TIME AGO i dont have any blood test records or her notes. I can try to obtain them as im going back to london next week.

i remember having a followup a long time after treatment stopped, and remember asking her ' was this growth all down to me ?' She replied' It's difficult to tell...' then i asked again and she said yes alot of is down to you. I am really confused and concerned about this past experience. Can i reply you with my blood test records when i obtain them. Maybe they have LH and Test level tests there.

Sorry for the long followup , i hope you understand my anxieties.

Best Regards,
XXXXXXX

Answered by Dr. Anantharaman (6 hours later)

Hi,

I understand your concerns. Let me try to explain. It's true that FSH starts rising before LH, possibly a few years before LH. But it's the LH which predicts impending onset of puberty, so it is more useful clinically and gets measured preferentially for assessing pubertal development. We do measure FSH and it is important if it is Low as it may indicate hypogonadism.

Your doctor was right; CDGP is a normal variant. It's not possible to pinpoint a single or for that matter a few factors also responsible for it. But the good thing is that it is immaterial if your puberty is a few years later as later outcomes are normal.

It would indeed be interesting to know your old hormone reports

Regards
ANANTHARAMAN R

Above answer was peer-reviewed by : Dr. Prasad

Follow up: Dr. Anantharaman (17 hours later)

Good day Doctor Anantharaman,

so i want to ask.....Let us consider that at age 15.0 i acheived SMR 3.

Is it correct to assume that the onset of puberty occured around 2 years earlier to this landmark ? Would it still be within normal for the onset to have happened less than 2 years prior to this landmark?

My final important question: Would it be still be within 'normal' after acheving SMR 3 at age 15.0 for LH hormone value to be absolutely '0' 6 month previously at age 14.6 ?? Or is this physiologically or endocronologically inaccurate?

I am working on obtaining my records and have a feeling that i hade active LH levels but never know until i see them !

Warm Regards,
XXXXXX

Answered by Dr. Anantharaman (8 hours later)

Hi,

Thanks for the query.

Answer to first two questions is "yes".

Answer to second question - when you had the delayed puberty, the LH test assays did not have sufficient accuracy reports of 0.1 increments. It is likely that your LH would have been 0. The present day assays can give reports like 0.3, 0.2, etc., and you can have a finer interpretation. In short the answer will be "Yes".

If you did have a value of say 1 then you probably were diagnosed based on those tests.

Hope this answers your query.
Please close this discussion if you have no more queries.

Regards
ANANTHARAMAN

Above answer was peer-reviewed by : Dr. Prasad

Follow up: Dr. Anantharaman (15 hours later)

Good Day Dr,

Is LH secretory activity present in mid childhood and does this lead to testosterone secretory activity in minute quantitites? i understand just before puberty onset this activity increases but i'm interested in the years BEFORE this stage.

For example, is LH and gonadal Testostorone activity present around adrenarche stage?? i remember getting new small hair in pubic area and underarm at 12 1/2 years old and thinking wow im growing up! But laterr i now know this was likely adrenarche stage.

You mentioned before puberty onset some values for LH and TEstosterone, above 0.3IU/l and 20ng/do respectively.....how doesn one determine if this testosterone is truly gonadal and LH induced and not adrenal gland?

Thanks in advance
XXXXXX

Answered by Dr. Anantharaman (6 hours later)

Hi,
LH pulsatile activity is not detected during childhood- this is known as the juvenile pause. It only starts a few years before the pubertal signs appear.

Adrenarche is totally independent of puberty, it may occur before 8 years - so called premature adrenache- this actually a biochemical event where the DHEAS goes above a certain level. A hair growth and body odour may be detected because of these weak androgens.

Testosterone is not produced in sufficient quantities from the adrenal even the 20 ng/dl will signify a testicular source.
I hope this answers your questions. I think we should consider this consultation as closed.
Regards
Anantharaman

Above answer was peer-reviewed by : Dr. Shanthi.E

Follow up: Dr. Anantharaman (3 days later)

Dear Doctor XXXXXXX

I am very grateful for your feedback to my queries. I have paid to have these queries as its important to me. If you are busy and pass me to another endocrologist, i will understand.... if you can continue i will be further grateful.

You mentioned in the beginning onset of puberty is associated with FSH and LH increase to above 0.3IU/l. So by implication this means before this during childhood the framework for hypothalamic-pituitary-gonadal exists but below 0.3IU/l . Is this correct?

Also i want to know more about tempo of puberty. Is it possible for Peak height velocity to be acheived just 6 months after clinical onset of puberty (TEstis = 4ml)? Or is this impossible and PHV always requires around two year after the clinical onset of puberty??

Also at what bone age is XXXXXXX stage 3 attained?

Thanks and Regards,
XXXXXX

Thanks

Answered by Dr. Anantharaman (12 hours later)

Hi.

I will be pleased to answer your questions.

During juvenile phase - many years before childhood, LH is not detectable and so is testosterone. Both are present in small quantities but one cannot detect the levels using usual techniques. The first event to happen during puberty is pulsatile LH release. Again this happen only sometime in the night so when levels are measured in the morning can be low.

In a normal puberty what you are saying is true; peak height velocity happens few years into puberty in a normal puberty. In case of CDGP what you are saying may be possible.

Hope I answered your query.

Regards
Anantharaman

Above answer was peer-reviewed by : Dr. Prasad

Follow up: Dr. Anantharaman (14 hours later)

Hi Doctor ,

Thank you for your great feedback . i am ready to close but just before i do one final question.

In a 16 year 2 months old male subject, would a LH measurement of <0.1IU/l be indicative of HH ?

Thanks you DR XXXXXXX and my best regards ,
XXXXXX

Answered by Dr. Anantharaman (9 hours later)

Hi,

As I had mentioned previously it’s very hard to differentiate at times between CDGP and HH at times. a value of LH <0.1 at 16y 2m would make one think more in favour of HH. But then clinically we make a diagnosis of HH only after 18 years of age by which time most children with CDGP should attain puberty.

CDGP and HH are now considered to be a continuum; there is a possibility of spontaneous activation of HPG axis (so called reversal of HH) even in adults in up to 10% of HH patients.

Regards
Anantharaman

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Above answer was peer-reviewed by : Dr. Shanthi.E