Puberty Flashcards

1
Q

What is puberty?

A
  • The period of age at which a person is first capable of sexual reproduction of offspring
  • Begins in late childhood
  • 8-13 years for females
  • 9-14 years for males
  • Characterised by maturation of the hypothalamic pituitary gondal axis
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2
Q

What occurs during puberty?

A

- Appearance of secondary sex characteristics:
- Physical growth
- Sexual development
- Psychological development
- Acceleration of growth
- Mass and fat distribution
- Bone maturation
- Adult height
- Capacity for fertilisation
- Spermatogenesis
- Ovulation

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3
Q

Whar are the hormonal changes in puberty?

A
  • Adrenarche - adrenal gland component of puberty = the activation of production of androgens by the adrenal cortex, begins before age 8. responsible for the appearance of puvbic and auxillary and acne (Pubarche)
  • Gonadarche - the ovary or testes component of puberty = the activation of the gonads by the pituitary hormones FSH and LH. Responsible for the production oestrogens and testosterone. Sexual maturation and development of reproductive maturity
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4
Q

What is the physiology of puberty?

A
  • GNRH is a hypothalamic tropic hormone that stimulates the secretion of gonadotropins (LH-FSH)
  • Hypothalamus releases GnRH in bursts occuring at approx 2-hr intervals
  • This causes rates of FSH and LH secretion to rise during bursts and fall between bursts
  • At the beginning of puberty , pulsatile GnRH secretion rises dramatically in response to a change in brain activity that alters neural input to the hypothalamus
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5
Q

What is the hypothalamus GnRH release pathway?

A
  • Pre-puberty : hormornal feedback/ central neural suppression of GnRH release suppress onset of puberty
  • Hypothalamus-piuitary-gondal axis starts working in foetus. After bitrth, sex hormones and FSH/LH found in adult levels
  • Levels reduce in months after birth; pulsatile GnRH reduces in childhood and increases in frequency and amplitude before puberty
  • In males - negative feedback mechanism driven by circulating gonadal steroids and inhibin which results in reciprocal changes in the secretion of pituitary gonadotropins
  • Testosterone and estradiol in the male have independent effects on LH secretion
  • Inhibition of LH by testosterone requires aromatization for its piuitary effects, and estradiol-induced negative feedback on LH occurs at the level of the hypothalamus
  • In the female - cyclic secretion involves a positive feedback mechanism driven by oestrogens, of sufficient duration
  • This initiates the synchronous release of LH and FSH that is characteristic of the normal adult woman before menopause
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6
Q

What is the timing and onset of puberty modulation?

A
  • Genetic factors : 50-80% of variation in pubertal timing
  • Balance in the inhibitory and excitatory factors through coordinated changes in transsynaptic anf glial-neuronal communication
  • Glial cells affect GnRH secretion through growth factor- dependent cell-cell signalong coordinated by numerous unrelated genes
  • Environmental factors
  • e.g nutritional status ( obese are more likely to be earlier), excessive exercise
  • Alteration of body metabolism linked to energy metabolism may affect the CNS restraints on pubertal onset
  • Pyscohological factors
  • Things like stress or anxiety can also affect the CNS control of the onset of puberty
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7
Q

What is Tanner stage?

A
  • a way to classify the time, course and progress of changes that occur during puberty
  • Based upon attainment of secondary sex characteristics:
  • Genital development in males
  • Breast development in females
  • Pubic hair development in both genders
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8
Q

Describe female development during puberty.

A

1.) Thelarche :
- development of the breast
- Under the control of oestrogens
- Breast bud appears at an average age of 9 years
- Starts and completed in ~ 3 years
- Ductal proliferation
- Site specific adipose deposition
- Enlargement of the areola and nipple
- Prolactin, glucocoticoids and insulin
2.) Pubarche - growth of pubic and axillary hair
- under the control of androgens
- occurs at age 12.5 years
3.) Growth spurt
- Under the control of oestrogens
- Global process involving skeletal growth rate
- Muscle mass
- Growth of all internal organs
4.) Menarche
- Daily rise of oestradiol
- First menstrual bleeding
- It is observed 2-3 years adter thelarche

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9
Q

Describe the stages of male development.

A
  • Enlargement of testes
  • Followed by scrotum pigmentation and thinning of scrotal skin
  • Penis enlarges
  • Pubic hair develops
  • Growth spurt
  • Breaking of the voice at the time of puberty is indicative of androgen-induced enlargement of the larynx
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10
Q

What is an orchidometer?

A
  • Helps to determine testicular volume
  • Prepubertal - 1-3ml
  • Pubertal - 4-12 ml
  • Adult - 15-25 ml
  • Can be first indicators of some disorders
  • Hypogonadism (small testes)
  • Fragile X syndrome (large testes)
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11
Q

What are some disorders of puberty?

A
  • Precocious Puberty
  • Delayed Puberty
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12
Q

What is precocious puberty?

A
  • Premature sexual development before:
  • 8 years in females
  • 9 years in males
  • Dominant in girls, usually idiopathic
  • Rarer in boys, underlying lesion
  • Divided into :
  • Gonadotropin-dependent precocious puberty (GDPP or true):
  • involves the premature activation of the hypothalamic- pituitary- gonadal (HPG) axis

Gonadotropin- independent precocious puberty ( GIPP or pseudo):
The presence of sex steroids is independent of pituitary gonadotropun release.

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13
Q

What is the treatment of precocious puberty?

A

GDPP :
- dependent on the cause of puberty : Identifiable CNS lesion, therapy is directed toward the underlying pathology
- Primary treatment option = GNRH antagonist which slows accelerated puberty and improves final height
- Dependent on child’s age - the rate of pubertal progression - height velocity - rate of bone advancement

GIPP:
- Does not repsond to GnRH antagonist therapy. Treatment is directed at the underlying pathology
- Children with tumours of the testis, adrenal gland and ovary treated by surgery.
- HCG- secreting tumours combination of surgery, radiation therapy and chemotherapy depending upon the site and histologic type

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14
Q

What is inomplete puberty?

A
  • isolated manifestation of precocity without development of other signs of puberty
  • Premature thelarche : Transient condition of isolated breast development in the first 2 yrs of lifem often persists for 3-5 years , and is rarely progressive. Mostly idiopathic
  • Premature pubarche : Appearance of sexual hair before the age of 8 yrs in girls, or 9 years in boys without other evidence of maturation
  • Premature menarche : isolated vaginal bleeding in the absence of other secondary sexual characteristics. Very rare.
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15
Q

What is delayed puberty?

A
  • indicated if no signs of puberty are observed in a girl by 14 and in a boy by 15 years
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16
Q

What is hyperogonadotropic hypogonadism?

A
  • Circulating levels of FSH and LH are high
  • Congenital
  • Turner Syndrome
  • Klinefelter’s Syndrome
  • Complete androgen insensitivity
    Acquired
  • Chemotherapy/Radiation/Surgery
  • Post infectious (i.e. mumps orchitis, coxsackievirus infection, dengue, shigella, malaria,
    varicella)
  • Testicular torsion
  • Autoimmune/metabolic (autoimmune polyglandular syndromes)
  • “Vanishing Testes syndrome”
  • “Resistant Ovaries syndrome” (gonadotropin receptor problems)
    varicella )
17
Q
A
18
Q

What is Hypogonadotropic Hypogonadism?

A
  • Hypogonadotropic Hypogonadism
  • Constitutional Delay of Puberty
    Malnutrition
  • Excessive Exercise
    Growth Hormone Deficiency
  • Isolated Gonadotropin Deficiency
  • Endocrine Causes
    Miscellaneous syndrome complexes
    Brain tumors
  • Craniopharyngioma, astrocytoma,
    gliomas, histiocytosis X, germinomas,
    prolactinomas
  • Iron overload (pituitary damage)
    GnRH receptor abnormalities
19
Q

What is Eugonadotropic pubertal delay?

A

Congenital Anatomic Anomalies
Imperforate hymen
Vaginal atresia
Vaginal aplasia
PCOS (Polycystic ovary syndrome)
Hypothyroidism
— Interferes with gonadotropin secretion
Hyperprolactinemia
— Interfere with gonadotropin production
PCOS ( Polycystic ovary syndrome )

20
Q

What is the treatment of delayed puberty?

A

Male:
Testosterone is usually continued until there is clear evidence of spontaneous puberty
(testicular growth). The duration and dosage of therapy should be monitored by a
pediatric endocrinologist as over dosage or excessively long courses can reduce the
period of pubertal growth.
hCG (human chorionic gonadotropin): to stimulate development of secondary sexual
characteristics . Increases testicular size.
Female:
Oestrogen replacement:
Attainment of secondary sexual characteristics
Attainment of menses
— Stimulation of pubertal growth spurt
Acquisition of bone mineral mass
Uterine development

21
Q

We know the changes that occur in puberty and have ways of staging this development:
Thinking about the physiological onset of puberty, what drives this?
How could this process/regulation go wrong causing an early onset or a delayed puberty? What could cause this dysfunction?

A

An early onset (precocious) due to either HPG activation (Gonadotropin- dependent precocious), or presence of sex steroid independently from the HPG axis (Gonadotropin-independent precocious).
A delayed onset, where levels of FSH and LH are either low
(Hypergonadotropic Hypogonadism), high (Hypogonadotropic Hypogonadism, or normal (Eugonadotropic delay). The most common is
Hypergonadotropic Hypogonadism from either a congenital or acquired source but there is a lot of variety in the underlying causes. The three
subsets based on the FSH/LH levels can be used to narrow down the potential underlying cause (look back at slides 31, 32 and 33).