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Year 2 Medicine > Physiology of Puberty > Flashcards

Physiology of Puberty Flashcards

1
Q

Define Puberty

A
  • The stage of physical maturation in which an individual becomes physiologically capable of pro-creation (sexual reproduction)
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2
Q

Physical Changes in Puberty

  • … spurt
  • … … characteristics
  • M…/s…
A
  • Growth spurt
  • Secondary sex characteristics
  • Menarche/spermatogenesis
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3
Q

Physical Changes in Girls

  1. … …
    • 8 - 14 yrs​
    • 6 - 10 cm / yr peak
    • 2.5 yrs duration
  2. … … (…)
    • 8 - 13 yrs ( 11 yrs )
    • Usually the first sign
  3. … … (…)
    • 9 - 13 yrs
    • 1st Pubertal Sign in …%
  4. … … (…)
    • 9.5 - 15 yrs
    • May follow Menarche
  5. … (…)
    • 10 - 16 yrs ( 13 yrs )
A
  1. GROWTH SPURT
    • 8 - 14 yrs​
    • 6 - 10 cm / yr peak
    • 2.5 yrs duration
  2. BREAST GROWTH (Thelarche)
    • 8 - 13 yrs ( 11 yrs )
    • Usually the first sign
  3. PUBIC HAIR (Pubarche)
    • 9 - 13 yrs
    • 1st Pubertal Sign in 25%
  4. AXILLARY HAIR (Adrenarche)
    • 9.5 - 15 yrs
    • May fllow Menarche
  5. MENSTRUATION (Menarche)
    • 10 - 16 yrs ( 13 yrs )
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4
Q

Endocrine Changes in Puberty

  • …-…-… synchrony – established in … life
  • Until … – neural mechanisms suppress … release
  • At …-… yrs – pulsatile nocturnal … release
A
  • H-P-G synchrony – established in fetal life
  • Until puberty – neural mechanisms suppress GnRH release
  • At 6-9 yrs – pulsatile nocturnal GnRH release
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5
Q

Endocrine Changes in Puberty

  • Pulsatile … release (nocturnal) every … - … min - 6 to 9 years
  • increased … leads to increased … and increased …
  • …/… become sensitized to the effects of these
  • Final phase: development of …/… feedback mechanism
  • … stimulates the adrenals – … & … hair
A
  • Pulsatile GnRH release (nocturnal) every 90-120 min - 6 to 9 y
  • increased GnRH leads to increased FSH and increased LH
  • Ovaries/testes become sensitized to the effects of FSH and LH
  • Final phase: development of positive/negative feedback mechanism
  • ACTH stimulates the adrenals – Pubic & Axillary hair
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6
Q

Testes

  • Production of … (…)
    • … cells under FSH control
  • Production of … (Testosterone)
    • … cells under LH control
    • …% from testes, …% from adrenals
  • Testosterone in blood – converted to … in the target organs
A
  • Production of gametes (sperms)
  • Sertoli cells under FSH control
  • Production of androgens (Testosterone)
  • Leydig cells under LH control
  • 95% from testes, 5% from adrenals
  • Testosterone in blood – converted to DHT in the target organs
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7
Q

The Ovarian Cycle

  • … phase
    • Initially E rises (…) with LH surge in …
    • … occurs
  • … phase
  • … feedback after ovulation
  • No further … in the same cycle
A
  • Follicular phase
  • Initially E rises (FSH) with LH surge in mid cycle
  • Ovulation occurs
  • Luteal phase
  • Negative feedback after ovulation
  • No further ovulation in the same cycle
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8
Q

What is this showing?

A

Endometrial cycle

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9
Q

Age of Menarche

  • Related to general health, genetic and nutritional factors
  • Mean age is … at a rate of … months per decade
    • Mean age in 1840 = … y; 1990 = … y
  • One in … girls now reaches menarche while at primary school
  • Body … and % … is also important
    • Mean weight at menarche is …kg
    • …-…% fat
    • …, patients with … – late onset
A
  • Related to general health, genetic and nutritional factors
  • Mean age is falling at a rate of 4 months per decade
    • Mean age in 1840 = 16.5 y; 1990 = 12.8 y
  • One in 8 girls now reaches menarche while at primary school
  • Body weight and % fat is also important
    • Mean weight at menarche is 47.8kg
    • 16-24% fat
    • Athletes, patients with anorexia – late onset
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10
Q

Adrenarche

  • … … – responsible for axillary and pubic hair
  • … stimulates zona … of adrenal cortex
  • DHEAS & Androstenedione
    • Girls – starts by …, adequate levels by …
    • Boys – starts by …, adequate levels by …
A
  • Adrenal androgens – responsible for axillary and pubic hair
  • ACTH stimulates zona reticularis of adrenal cortex
  • DHEAS & Androstenedione
    • Girls – starts by 6, adequate levels by 8
    • Boys – starts by 8, adequate levels by 10
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11
Q

Define Adrenarche

  • Adrenarche refers to the time during puberty when the adrenal glands increase their production and secretion of adrenal …
  • Plasma concentrations of dehydroepiandrosterone (…) and DHEA-sulfate(s), the most important adrenal androgens, begin to increase in children by approximately … to …years.
  • However, the signs of adrenarche, such as … and … … development, a…, and body …, do not typically occur until … to ….
A
  • Adrenarche refers to the time during puberty when the adrenal glands increase their production and secretion of adrenal androgens. Plasma concentrations of dehydroepiandrosterone (DHEA) and DHEA-sulfate(s), the most important adrenal androgens, begin to increase in children by approximately 6 to 8 years. However, the signs of adrenarche, such as pubic and axillary hair development, acne, and body odor, do not typically occur until early to midpuberty.
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12
Q

Chronological Order of Puberty - Girls

  • … ….
  • … development
  • … hair
  • … hair
  • …
A
  • Growth spurt
  • Breast development
  • Pubic hair
  • Axillary hair
  • Menarche
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13
Q

Chronological Order of Puberty - Boys

  • … volume
  • … length
  • … hair
  • … …
  • … / … hair
  • … voice
A
  • Testicular volume
  • Penile length
  • Pubic hair
  • Growth spurt
  • Axillary / Facial hair
  • Deep voice
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14
Q

For each row, what is the subtitle?

A
  • Beginning of puberty
  • Growth Spurt
  • Years of Puberty
  • Menarche/Voice Deepening
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15
Q

Disorders of Puberty - Early or Precocious

  • Girls – under … yrs
  • Boys – under … yrs
A
  • Girls – under 8 yrs
  • Boys – under 9 yrs
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16
Q

Disorders of Puberty - Delayed

  • Girls – over … yrs
  • Boys – over … yrs
A
  • Girls – over 14 yrs
  • Boys – over 14 yrs
17
Q

Precocious Puberty

  • … or … puberty
  • Presence of true pubertal features at an … & … age
    • Central or True precocious puberty
      • … dependent
    • Peripheral or Pseudo-precocious puberty
      • … independent
  • Normal variants
    • … T…
    • … A…
A
  • Early or Premature puberty
  • Presence of true pubertal features at an young & inappropriate age
    • Central or True precocious puberty
      • Gonadotrophin dependent
    • Peripheral or Pseudo-precocious puberty
      • Gonadotrophin independent
  • Normal variants
    • Premature Thelarche
    • Premature Adrenarche
18
Q

Concerns raised by early onset puberty

  • Possible underlying sinister cause
    • … – up to …%
  • … & …-… upheaval at an inappropriately young age
  • Early cessation of … leading to decreased final … …
A
  • Possible underlying sinister cause
    • Boys – upto 80%
  • Emotional & pyscho-social upheaval at an inappropriately young age
  • Early cessation of growth leading to decreased final adult height
19
Q

What is the Diagnosis?

  • Emileigh, 14 months
    • Blood clots in nappy
    • Bilat breast buds
    • Ht & Wt >97th centile
    • LH 2.2, FSH 3.2,
    • 17β estradiol 432
    • Uterus – enlarged
    • Bone age – 2.8 yrs
    • MRI scan shown
A
  • Hypothalamic Hamartoma - premature true puberty - LH and FSH should be undetectable, uterus enlarged, blood clots in nappy, bones advanced (oestrogen), Breast buds
20
Q

Precocious Puberty - Central (testing / treatment)

  • Long acting … analog therapy
    • Sustained supra-physiological … levels
    • Paradoxical cessation of … release
    • Stops further … …
  • Pubertal progression resumes when treatment stopped (at …-… yrs)
A
  • Long acting LHRH analog therapy
    • Sustained supra-physiological LHRH levels
    • Paradoxical cessation of gonadotrophin release
    • Stops further pubertal progression
  • Pubertal progression resumes when treatment stopped (at 10-12 yrs)
21
Q

Normal Variants - Precocious Puberty

  • Premature …
    • Isolated … development
    • Usually seen in children
  • Premature …
    • Isolated … … development
    • Caution: first sign of … in some

​

A
  • Premature thelarche
    • Isolated breast development
    • Usually seen in children <2-3 yrs of age
  • Premature adrenarche
    • Isolated pubic hair development
    • Caution: first sign of puberty in some
22
Q

Pseudo-precocious puberty - Females / Males

  • Females:
    • Iso-sexual or feminising
      • Causes - MAS - stands for… , O…/A…
    • Hetero-sexual or masculinising
      • C.., O../A…
  • Males:
    • Iso-sexual or masculinising
      • Causes - C…, A…l/L… cell …
    • Hetero-sexual or feminising
      • A…
A
  • Females:
    • Iso-sexual or feminising
      • Causes - MAS - stands for McCune-Albright syndrome​ , Ovarian/Adrenal
    • Hetero-sexual or masculinising
      • CAH, Ovarian/Adrenal
    • Hetero-sexual or masculinising
      • CAH, Ovarian/Adrenal
  • Males:
    • Iso-sexual or masculinising
      • Causes - CAH, Adrenal/Leydig cell tumour
    • Hetero-sexual or feminising
      • Adrenal
23
Q

What is the diagnosis?

  • Mairna, 6 yrs
  • Obese
  • Pubic hair stage 2
  • No breast bud
  • LH <1, FSH 1.4,
  • 17β estradiol 32
  • Steroid profile
A

Congenital Adrenal Hyperplasia

24
Q
  • Congenital adrenal hyperplasia (CAH) is a group of autosomal … disorders characterized by impaired … synthesis.
  • It results from the deficiency of one of the … enzymes required for the synthesis of … in the adrenal ….
A
  • Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders characterized by impaired cortisol synthesis.
  • It results from the deficiency of one of the five enzymes required for the synthesis of cortisol in the adrenal cortex.
25
This is showing ... biosynthesis
**Steroid biosynthesis**
26
27
_Delayed Puberty_ * Absence of ... pubertal onset at an ... age * Not necessarily lack of ... in a ... * ...-files – very important * Normal ... * Constitutional ... & ... delay
* Absence of **true** pubertal onset at an **appropriate** age * Not necessarily lack of **periods** in a **girl** * **X**-files – very important * Normal **variant** * Constitutional **growth & pubertal** delay
28
_Concerns raised by delay in Puberty_ * Possible sinister underlying cause * ... that puberty will never occur * Emotional and psychosocial upset of ..., specially when associated with ... stature * Long term sequelae: Reduced ... ...
* Possible sinister underlying cause * **Fear** that puberty will never occur * Emotional and psychosocial upset of **immaturity**, specially when associated with **short** stature * Long term sequelae: Reduced **bone mineralization**
29
_What is the diagnosis?_ * Steven, 16 yrs * No pubertal progress * Learning difficulties * Tall, 98th centile * PH-2, G-2, TV-6ml * LH 12, FSH 16, T 40 * Bone age – 14.5 yrs
**Klinefelter Syndrome**
30
_Klinefelter Syndrome_ * 1 in ... ... infants * 47 ... / Multiple ... * ... problems * ... deficiency * ...spermia / ... * (Micro ... -\> Sex change) * Lifelong ... replacement therapy
* 1 in **1000 male** infants * 47 **XXY** / Multiple **X** * **Behavioural** problems * **Androgen** deficiency * **Azoospermia / Infertility** * (Micro **genitalia** -\> Sex change) * Lifelong **testosterone** replacement therapy
31
_What is the diagnosis?_ * Rosie, 12 yrs * Short Stature * No pubertal onset * Recurrent ear infections * Increased carrying angle * Widely spaced nipples * LH 56, FSH 95, * 17β estradiol 45 * Karyotype
**Turner Syndrome**
32
_Turner Syndrome_ * 1 in ... live ... births * Triad - ... stature, ... gonads, primary ... * ... features – Webbing of neck, cubitus valgus * ... of aorta, horse shoe ... * Early clue - L... * Surprisingly ... !!! * Turner ...
* 1 in **2000** live **female** births * Triad - **Short** stature, **streak** gonads, primary **amenorrhoea** * **Dysmorphic** features – Webbing of neck, cubitus valgus * **Coarctation** of aorta, horse shoe **kidneys** * Early clue - **Lymphedema** * Surprisingly **normal**!!! * Turner **Mosaic**
33
_Turner Syndrome_ * Exclude co-existing ... anomalies * ... Hormone therapy * Pubertal induction + ongoing ... * Active monitoring to detect ... * Assisted ...
* Exclude co-existing **congenital** anomalies * **Growth** Hormone therapy * Pubertal induction + ongoing **HRT** * Active monitoring to detect **co-morbidities** * Assisted **conception**
34
_What is the diagnosis?_ * James, 15 yrs * Absent smell sensation * PH-1, G-1, AxH-1, TV-2ml each * LH \<1, FSH 1.2, T 0.8 * LHRH stimulation test – peak LH remained \<1 * HCG stimulation test – min. increase in T * MRI scan – Normal * Kallman gene analysis – Negative * Lifelong Testosterone replacement therapy
**Gonadotrophin deficiency**
35
_Normal Variant - Delayed puberty_ * Constitutional delayed growth & puberty * More common in ... * ... & ... in school days * ... onset of puberty * ... age delayed slightly * ... history – often present * Normal ... ... * Pubertal ... – sometimes necessary
* Constitutional delayed growth & puberty * More common in **boys** * **Small & Short** in school days * Late onset of **puberty** * **Bone age** delayed slightly * **Family** history – often present * **Normal adult height** * Pubertal **induction** – sometimes necessary

Year 2 Medicine (162 decks)

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