Week 203 Puberty Flashcards
Define puberty:
Normal puberty is the physiological sequence of events that starts in late childhood and is characterized by development of secondary sexual characteristics, acceleration of somatic growth, advancement of bone maturation as well as behavioural and psychological changes.
Define Adolescence
The transition from childhood dependence to adult autonomy
Adolescence is classed as 10-24 year old
Puberty reflects the physical changes that allow us to survive and reproduce in the adult world. Adolescence embodies the psychological and neurological changes that allow us to survive in the adult world as it is now
First sign of puberty in males?
Testicular enlargement - > 4ml.
How does LH act on the gonads?
LH acts on gonads (testes /ovary ) to increase the secretion of testosterone (male) or oestrogen (female) respectively which then promotes growth on target organs
First sig of puberty in females?
Breast development.
How does growth end?
Fusion of the epiphyses in response to Oestrogen.
Consonance suggests _____
Puberty follows a set pattern ie consonance
ie testicular growth followed by penile growth with pubic hair, and then growth spurt ; or breast buds followed by pubic hair and growth spurt and later on menstruation
So consonance suggests activation of the hypo pit gonadal axis ie central activation of gonadotropins (so >LH FSH and gonadal hs)
Summary of puberty
Hypothalamus –> Pituitary (+ Adrenals) –> Local gonadal effect -> Growth spurt
What initiates puberty?
1) Genetic factors eg females earlier than males. Also when did Mo and Dad start puberty ?
2) Nutritional status Malnutrition and eating disorders lead to low BMI and for puberty to occur need BMI of at least 17kg/m2 Need a fat mass of 17% of body weight and menarche needs 22% for regular cycles
Exercise has a role eg Gymnasts.
Chronic inflammatory conditions delay or arrest puberty Crohns
3) Environment eg caring ect
7 YO presents with body odour + Pubic hair. How do you approach problem?
Are there signs of puberty?
If so, do they follow a concordant pattern? (i.e. breast enlargement first)
Check growth/growth chart
Which investigations would you give fo 7yo f with pubic hair non conchordant growth?
FSH LH Adrenal hormones Growth Ultrasound ovaries
What is adrenarche?
Normal maturation of the adrenal gland, causing increased secretion of hormones. NORMAL PHYSIOLOGICAL PROGRESSION.
What defines delayed puberty?
Absence of breast development by 13.5 years or where menstruation has not commenced within 3 years of breast growth.
In males, 14 YO Testes not developed. 80% constitutional. Diff could be Meningitis, Congenital abnor, infection, tumour.
What is included in a hormonal profile?
FSH LH Androgens GtR Oestrogen/Testosterone
What is Kallman’s?
Genetic condition
Delayed puberty
Lack of smell
Treatment of delayed puberty in addition to hormonal profile?
Bone age
Pelvic USS
MRI
The ____ duct and ____ duct are v important for the development of the genital system in embryology.
Mesonephric (Wolffian)
Paramesonephric (Mullerian)
These start to appear in the 4th week of development.
What is the SRy gene?
The SRY protein (coded for by this gene) is a transcription factor, that initiates other processes.
Used to be used to try to ID as man, but no longer used.
In week 5, cells from the yolk sac to the ______
Gonadal ridge
Sertoli cells support the ______ _____
Developing somatocytes
Production of sertoli cells, and resulting hormone production, which structure fades out in males?
Paramesonephric duct
Mesonephric duct becomes?
Urethra, Epididymis, Bulbourethral glands, ejaculatory duct, vas deferens (in males)
(in females)
Which sex is produced if no Sry gene?
Female
In females, which duct fades out?
Mesonephric. PAramesonephric remains.
In females, the paramesonephric duct becomes what?
Uterovaginal primordium. This will form vagina (upper) and the uterus. The rest of the vagina, the urethra and part of the bladder is formed from the urogenital sinus.
AT 11 WEEKS, what can you see (in terms of reproductive embryology)
Distinct structures (albeit subtle) are beginning to form to differentiate between sexes. These are so minor though, that you CANNOT see it on US. Sex diagnosis is only really noticeable at 20 weeks.
Define Gubernaculum.
As the scrotum and labia majora form in males and females, respectively, the gubernaculum aids in the descent of the gonads (both testes and ovaries).
The testes descend to a greater degree than the ovaries and ultimately pass through the inguinal canal.
Kidney development begins in the ___ week.
Third
The /Pronephros/mesonephros/metanephros produces the ___ system.
Renal.
The ureteric bud forms the _______
Metanephros
Also causes formation of metanephric cap. Following this, major calyx form etc.
A testes that has not descended normally is called?
Cryptorchidism
Oncholisis is a clinical sign for what?
Refers to nails coming away from the nail bed. A sign of both hyper and hpo thyroidism.
What are you looking out for in the examination of the visual system (thyroid exMINATION)
Exopthalmus (protruding eyes) - common in Grave’s (HyPERthyroidism)
Occular movements - checking for diplopia.
Lid Lag test
Looking out for (thyroid exam - hands)
Oncholisis
Clubbing
A CYST THAT MOVES WHEN YOU STICK YOUR TONGUE OUT IS A _____
THYROGLOSSAL CYST
Thyroid examination - which nodes do you go through?
Infraclavicular supraclavicular Cervical chain Occipital Post-> pre auricular, sumandib, submental, thyroid - 3 fingers either sid, as for swallow at this point.
A Hand tremor in examination could be a sign of ___
Hyper thyroidism
What is Pretibial Myxoedema?
Pretibial myxoedema is a form of diffuse mucinosis in which there is an accumulation of excess glycosaminoglycans in the dermis and subcutis of the skin.
Glycosaminoglycans, also called mucopolysaccharides, are complex carbohydrates that are important for tissue hydration and lubrication. The main glycosaminoglycan in pretibial myxoedema is hyaluronic acid, which is made by cells called the fibroblasts.
How does FSH act?
FSH acts on gonads to produce ova or sperm
What is Adrenarche?
Adrenarche is a term used to describe normal maturation
of the adrenal gland leading to enhanced secretion of androgens eg androstenedione and dehydroepiandrosterone (DHEA).
14yr old boy with 8mls testes pubic hair and penile growth
Expected profile?
Raised FSH Raised LH Raised testosterone
Normal Puberty
Expected Profile?
Raised FSH Raised LH Raised oestrogen (female) raised testosterone (male)
14 yr old girl with bilateral breast formation pubic hair and onset of menstruation
Expected profile?
Raised FSH Raised LH Raised Oestrogen
Constitutional delay in Growth and Puberty
Expected profile?
Low FSH, LH and oestrogen or testosterone
16 year old boy with Klienfelters
Expected Profile?
Raised FSH Raised LH low testosterone
16 year old girl with newly diagnosed Turners
Expected profile?
Raised FSH Raised LH low or non detectable oestrogen
Puberty in consonance
Expected profile?
Raised FSH raised LH raised testosterone (male) or oestrogen (female)
Kalmans Syndrome in 16 yr old
Expected profile?
Low FSH, LH and oestrogen or testosterone
Premature Thelarche (breast development) ie 2 yr old
Expected profile?
LH normal oestrogen normal FSH Mildly raised
Virilisation with prepubescent testes and increased penile growth in a 4 year old
Expected Profile?
Normal FSH, LH, but raised 17 hydroxyprogesterone level
Premature Adrenarche ie 6 yrs old
Expected Profile?
FSH,LH, Oestrogen normal testosterone but mildly raised androstenedione normal 17 OH Progesterone
Virilisation with cliteromegaly and no breast formation in a 4 yr old
Expected Profile?
Normal FSH, LH, but raised 17 hydroxyprogesterone level
Virilisation with unilateral enlargement of one testis in a 4yr old
Expected Profile?
Normal FSH LH but raised testosterone and raised HCG
Cafe au lait patches with pubertal changes and dysostotic bone cysts in a 6year old
Expected Profile?
Low FSH,Low LH and Raised oestrogen (female) or testosterone (male)
What is Gonadarche?
Onset of puberty heralded by Increase in nocturnal secretion of Gonadotropin Releasing Hormone (GnRH) towards end of 1st decade leading to increased LH and FSH secretion from the pituitary and so increased Testosterone and Oestrogen from gonads in Male and Female respectively
What is Adrenarche?
results from increase in adrenal androgen secretion at 6-8yr old and gives pubic and axillary hair development. It is not under the influence of FSH or LH
Gonadarche and Adrenarche are 2 separate ___ ______
Maturational EVENTS
HUGE listof various reasons for delayed puberty - try your best!
Genetic component ie Constitutional Maturational delay eg one of parents has a history of delay ( suggesting Hypothalamic Site )
Disruption of hypthalamic or pituitary control due to any intracranial condition that affects the hypothalamic or pituitary function eg cns trauma or structural abnormalities of the brain eg septo optic dysplasia, or due to tumours, or as a result of infections
Specific Lack of Gonadotrophins Eg Kalmans (Anosmia)
Chronic illness Crohns Anorexia Nervosa
Environment ie Caring Excess exercise (gymnasts)
Lack of gonadal response to Gonadotrophins stimulus eg Turners or Kleinfelters or damage to gonads eg radiation, chemotherapy, trauma
Lack of organ response to testosterone eg Androgen Insensitivity Syndrome
Treatment of maturational delay?
Can in boys give testosterone which will give virilisation effects until gonadotropin secretion is initiated
Treatment of Gonadal problem
eg Turners Sex steroid replacement