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w207 puberty > puberty > Flashcards

puberty Flashcards

1
Q

Where is GnRH secreted?

A

The hypothalamus

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2
Q

Where is LH and FSH secreted?

A

The anterior pituitary

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3
Q

What do FSH and LH act upon?

A

The Testes and the ovaries

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4
Q

What does FSH do to the testes?

A
  • It acts on sertoli cells in the testes which produces sperm.
  • It also creates a hormone called inbitin that acts as the feed back to stop GnRH and FSH release
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5
Q

What does LH do to the testes?

A
  • It acts on leydig cells to produce testosterone.

* Testosterone act on the hypothalamus and the pituitary as a -ve feedback loop

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6
Q

What does FSH do to the ovaries?

A
  • lt stimulates the ovary to produce an egg

* It also produces (with LH) oestradiol

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7
Q

What does LH do to the ovaries?

A
  • It stimulates the Egg release from ovary

* Creates precursor hormones to Oestradiol

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8
Q

What is delayed CENTRAL puberty also called?

A

Hypogonadotrophic Hyogonadism

  • The Pituitary/Hypothalamus axis doesn’t work properly so there is low LH/FSH/GnRH secretion
  • or it doesnt act on -ve feedback
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9
Q

What is delayed PERIPHERAL puberty also called?

A
  • Gonadal failure

* Despite LH/FSH secretion, Testosterone, oestrogen, progesterone are not secreted by the testes/ovaries.

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10
Q

At what age is puberty delayed in a female?

A

> 13 yrs (.15 for menarche).

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11
Q

At what age is puberty delayed in a boy?

A

> 14 yrs

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12
Q

What are the 2 main causes of delayed CENTRAL puberty?

A
  1. Constitutional
  2. Chronic disease
Other (ie rare):
•	Kallman = isolated central; “smell test”
•	Prior pituitary problems
•	Hypothyroidism
•	Pituitary tumour
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13
Q

What is the main cause of delayed PERIPHERAL puberty in boys?

A

Male:

  1. Chromosomal abnormality
    - > Kleinfelters (47XXY)
    - > Mixed gonadal dysgenesis

Other:
• Bliateral testicular damage
• Noonan’s
• Steroid hormone disorder (LH resistance)

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14
Q

What is the main cause of delayed PERIPHERAL puberty in Girls?

A

Female:

  1. Chromosomal abnormality
    - > Turners (45XO)
    - > Mixed gonadal dysgenesis
  2. Autoimmune ovarian failure

Other:
• Ovarian damage
• Androgen insensitivity syndrome
• Steroid hormone disorder (LH resistance)

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15
Q

At what age is puberty considered precocious in girls?

A

<8 yrs

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16
Q

At what age is puberty considered precocious in boys?

17
Q

What is the commonest cause of precocious puberty in girls?

A

Idiopathic

18
Q

What is the commonest cause of precocious puberty in boys?

A

tumours/masses

19
Q

What are the causes of CENTRAL precocious puberty?

A
  1. Idiopathic = commonest
  2. Secondary
    - > CP/Hydrocephalus
    - > Tumours/masses (MALE)
    - > Trauma
    - > Chronic inflamma’n
    - > RadioTx
    - > Sexual abuse
    - > Adoption
20
Q

What are the causes of PERIPHERAL precocious puberty?

A
  1. Genetic

•McCune-Albright, Testoxicosis

  1. Precocious breast development

•premature thelarche, thelarche variant, hypothyroidism, ovarian cyst

  1. Virilisation
  • adrenarche
  • CAH
  • Cushings
  • Adrenal tumour
  1. Other
    • Tumour producing hormones (Exogenous)
21
Q

What is Kallmanns syndrome? what is its presentation? What is its treatment?

A
  • Pathology: No GnRH secretion, congenital, otherwise axis is normal
  • Presenation: No smell, fail to start/complete puberty, hypogonadism
  • Treatment: Gonadal steroid replacement Tx
22
Q

What is Kleinfelters syndrome? what is its presentation? What is its treatment?

A
  • Pathology: 47XXY
  • Presentation: Gynaecomastia, tall woth long limbs, delayed/incomplete puberty, ED, osteoporosis, learning/speech, behavior
  • treatment: Androgen replacement
23
Q

What is Turners syndrome? what is its presentation? What is its treatment?

A
  • Pathology: 45XO
  • Presentation: Short, web neck, delayed puberty, ↓fertility, absent breasts, ovarian failure, high arched palate
  • Treatment: ?GH, ?Oestrogen replacement
24
Q

What is Noonans syndrome? what is its presentation? What is its treatment?

A
  • Pathology: Males & females; mutation in autosomal dominant gene
  • Presentation: Broad forehead, drooping eyelids, wide set eyes, +-mild learning difficulties, web neck, pectus excavatum, short, congenital heart disease (esp. pulmonary stenosis, atrial septal defect)
  • Treatment: Nothing +-symptomatic (eg for heart defects)
25
Q

What is constitutional delay? what is its presentation? What is its treatment?

A
  • Pathology: Genetic
  • Presentation: Late start to puberty & slow growth before puberty; parents were “late bloomers”
  • Treatment: Nothing; reassurance
26
Q

What is CAH (congenital adrenal hyperplasia)? what is its presentation? What is its treatment?

A
  • “Virilising adrenal hyperplasia”
  • Autosomal recessive; cortisol/aldosterone deficiency (this is the one where mineralcorticoid & glucocorticoid doesn’t work); is due to 21-hydroxylase deficiency
27
Q

what is the presentation for CAH?

A
  • Early hair, large genetalia/females look like male genetalia, males have salt loss/tall stature/precocious puberty
  • BEWARE salt wasting in very young neonates; Low sodium, high potassium, metabolic acidosis, hypoglycaemia
28
Q

what is the treatment for CAH?

A
  • Observation, long-term glucocorticoids / aldosterone
  • Salt crisis => urgent IV hydrocortisone,
  • Saline & glucose
  • Surgery for females

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