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Week 203
Adolesence and Puberty
39
Medical
2nd Grade
09/26/2013

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Term
Define Adrenarche
Definition
The onset of androgen secretion by the adrenal cortex. Occurs at 6-8YO
Term
Define Thelarche
Definition
The onset of breast development in girls (budding). Occurs after 8YO. Usually first physical sign of puberty.
Term
Define Menarche
Definition
The onset of menstruation. Average onset is 13YO.
Term
The presence of what affects Stage 1 of gonadal development In Utero?
Definition
Presence of Y chromosome and SRY gene. If present, leads to testicular development, if absent leads to ovarian development.
Term
The presence of what affects the second stage of Gonadal differentiation In Utero?
Definition
The number of X chromosomes present
Term
Adolescence covers what age range?
Definition
10-24
Term
What factor secretion from the Hypothalamus initiates puberty?
Definition
Gonadotrophin Releasing Factors
Term
The release of Gonadotrophin Releasing Factors stimulates what?
Definition
The production and release of Luteinsing hormone and follicle stimulating hormone from the pituitary gland.
Term
What does the action of LH on the gonads result in?
Definition
Production of testosterone and oestrogen (male and female respectively)
Term
What does the action of FSH on the gonads result in?
Definition
Production of Ova or Sperm (female and male respectively)
Term
What is the first sign of puberty in boys?
Definition
Testicular growth >4mls in volume.
Term
What is the first sign of puberty in girls?
Definition
Breast development, this coincides with a growth spurt.
Term
Pubic and axillary hair growth in girls is in response to what?
Definition
Adrenal androgens, therefore this may be disconcordant to ovarian development.
Term
Describe the pattern of consonance in girls pubertal changes
Definition
Breast buds followed by pubic hair and growth spurt and later on
menstruation.
Term
Describe the pattern of consonance in boys pubertal changes
Definition
Testicular growth followed by penile growth with pubic hair, and then growth spurt
Term
Name 5 factors that can affect the initiation of puberty?
Definition
Genetic factors, nutritional status, exercise, chronic inflammatory conditions (Chrons) and environment.
Term
At what age would delayed puberty be considered in males and females?
Definition
Absence of breast development in a girl by age 13.5 or lack of menstruation 3 years post breast bud development.
Failure of testicular growth of >4mls in boys aged 14.
Term
What is the most common cuase of delayed puberty in boys?
Definition
Constitutional delay. Family history important.
Term
Name a disorder in which a lack of gonadotrophins are produced?
Definition
Kalmans
Term
Delayed onset of puberty accompanied by a loss of the sense of smell in 50% of people would lead you to what differential?
Definition
Kalmans Syndrome
Term
Name two conditions in which delayed onset of puberty is caused by a lack of gonadal response to gonadotrophins
Definition
Turners Syndrome (Single X)
Kleinfelters Syndrome (XXY)
Term
What environmental factors may delay onset of puberty?
Definition
Malnutrition and excess exercise (gymnasts).
Term
Describe the three degrees of severity in androgen insensitivity syndrome
Definition
Complete androgen insensitivity syndrome (CAIS): external genitalia are that of a normal female.
Mild androgen insensitivity syndrome (MAIS): external genitalia are that of a normal male.
Partial androgen insensitivity syndrome (PAIS): external genitalia are partially, but not fully, masculinized.
Term
In delayed onset of puberty with high LH/FSH, how would you treat?
Definition
High FSH/LH with no puberty commensal indicates gonadal failure therefore replace sex steroids which are not being produced.
Term
In delayed onset of puberty with low LH/FSH, how would you treat?
Definition
Low LH/FHS indicates Hypogonadism/pituitary disorder/constituent delay. Therefore sex steroids until puberty begins or as a replacement.
Term
At what age in puberty in a girl considered precocious?
Definition
Before the ages of 8
Term
At what age in puberty in a boy considered precocious?
Definition
Before the age of 9
Term
Considering consonance, if pubic hair growth begins as a sign of precocious puberty, what is likely to be the problem?
Definition
Gonadal or adrenal problem (or tumour)
Term
Considering consonance, if breast bud development begins as a sign of precocious puberty, what is likely to be the problem?
Definition
Hypothalmic/pituatary axis problem
Term
What is the most common cause of precocious puberty following a disconcordant pattern?
Definition
Congenital adrenal hyperplasia
Term
What is the most common enzyme defect in congenital adrenal hyperplasia?
Definition
21 hydroxylase deficiency causing a lack of cortisol production and a build up of 17 hydroxyprogesterone causing the formation of androstenedione and then testosterone
Term
How do you treat congenital adrenal hyperplasia?
Definition
Steroid replacement e.g hydrocortisone
Term
List Tanners 5 stages of Thelarche
Definition
Stage 1 – Pre-Adolescent
Stage 2 – Breast bud stage
Stage 3 – contd. dev of breast and areola but no
separation
Stage 4 – further enlargement with papilla and areola projecting above breast contour
Stage 5 – Mature breast, areola recesses, & papilla elevated
Term
What pubertal changes are due to adrenal and ovarian androgens?
Definition
Growth of axillary and pubic hair, acne and skin changes
Term
List the 5 stages of pubertal change of external genitalia in boys?
Definition
Stage 1 – pre-adolescent
Stage 2 – Enlargement of scrotum and testis
Stage 3 – Further growth with enlargement of penis.
Hair on upper lip and cheeks
Stage 4 – further enlargement with development of glans penis
Stage 5 – Adult stage - Facial hair on lower lip and chin
Term
What are the congenital causes of Hypogonadotrophic Hypogonadism (↓ FSH & ↓LH)?
Definition
Pituitary dysfunction, FSH /LH receptor defects, IHH,
-Syndromes – Kallmans, Prader Willi, etc
Term
What are the acquired causes of Hypogonadotrophic Hypogonadism (↓ FSH & ↓LH)?
Definition
Chronic disease, eating disorders, Thyroid disease, Hyper-prolactinaemia
- Pituitary tumours, craniopharyngioma
-Medication or substance abuse
-Head Trauma
Term
What are the congenital causes of Hypergonadotrophic Hypogonadism (↑ FSH & ↑ LH)?
Definition
-Gonadal Dysgenesis – Klinefelters, Turners syndromes
-Androgen Resistance (AIS)
- Steroid enzyme defects
Term
What are the acquired causes of Hypergonadotrophic Hypogonadism (↑ FSH & ↑ LH)?
Definition
Trauma / surgery
-Cryptorchidism
-Post- infective – mumps
- Chemotherapy or DXT
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