2. Define
Transitional stage from childhood to adulthood manifested by physiological changes & development of SSC .
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3. Timing Usually occurs between the ages of 10 & 16 years .
Major determination is genetic. Other factor .
1.Geographic location
2. Exposure to light.
3.General health & nutrition:
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4. A.Critical body weight of 47.8 Kg (Frisch hypothesis).
B.A greater percentage of body fat (16% to 23.5%) may serve as initiating signal. Moderately obese girls have earlier menarche. Anorectics have delayed menarche. Puberty is delayed in morbid obesity, other factors are involved.
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5. What
When
How
1. Thelarche
Development of breast, 5Taner stages
±10 yrs, first sign of puberty
E2
2. Adrenarche
Development of pubic hair, 5 Taner stages & axillary hair, 3Taner stages
PH: 1yr after Thelarche
AH is the final SCC
Adrenal androgen
3. Spurt of growth
Accelerated growth, 6-11cm/yr
With adrenarche
GH & E2
4. Menarche
The first menstruation
±12yr
(9-17.7)
Midpubertal E2
Stages (Physiological changes)
The pubertal sequence requires 4.5 yrs. (range, 1.5-6 yrs)
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10. Uterus: enlarge, U/C:2/1 Ovaries: .increase in size, almond shape .300 thousands primary follicle at menarche (2 million at birth)
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11. Summary of pubertal events 1.FSH & LH rise moderately before the age of 10 yrs, followed by a rise in E2. LH pulse frequency increases are first seen in sleep but then are extended throughout the day. The final adult pattern is 1.5 to 2 hrs intervals between pulse. 2.Increased levels of E2 (gonadarche) results in Maturation of SSC. Increased skeletal growth at low levels of E2. Increased GH & IGF-1
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13. 3. Adrenal androgen cause adrenarche (pelvic & axillary hair). No major role in growth. It is an independent event.
4. Midpuberty levels of E2 are sufficient to induce menstruation.
5. Postmenarchal periods are irregular for 12-18 mo {LH surge is late pubertal event}
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15. Leptin & puberty
Leptin is a peptide secreted by adipose tissues; it acts on CNS neurons, regulating eating behavior & energy balance.
Higher levels of leptin correspond to earlier menarche. Girls with idiopathic precocious puberty have higher leptin levels. Leptin levels decrease with increasing Tanner stage. They have increased sensitivity to leptin. The decrease may allow greater food intake.
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16. Growth hormone:
At puberty its secretion is critically dependent on sex steroid.
It stimulates IGF-1 in cartilage & IGF-1 production in liver.
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18. Etiology, Classification
A. True, GnRH dependent, complete, central, isosexual: Activation of the HPO axis, development of the gonads, SSC & ovulation
1.Constitutional, idiopathic: (85%)diagnosed by exclusion.
2.CNS: Meningitis, encephalitis, hydrocephalus
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19. B. Pseudo, GnRH independent, incomplete, peripheral, iso or hetrosexual :
No activation of the HPO axis, but extrapituitary HCG or sex steroid exposure.
No developments of the gonads, No ovulation but development of SSC.
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20. 1. Isosexual:
Feminizing T (Granulosa-Theca cell, malignant teratoma).
Estrogen intake.
Albright S (precocious puberty, café-au-lait skin patches, cystic bony changes).
Hypothyroidism (Short stature & retarded bone age).
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21. The small cyst like space is similar to the Call-Exner bodies normally seen in granulosa cells
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23. 2. Hetrosexual: Virilizing T: virilization but no uterine bleeding. Androgen intake. CAH
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24. C. Partial: premature thelarche or adrenarche. It is due to end-organ increased sensitivity to normal circulating low E or A. Follow-up
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25. Diagnosis
History
Examination
i. Growth: Tanner stage, height & weight percentile
ii. External genitalia changes
iii. Abdominal, pelvic & neurological examination.
IV. Signs of androgenization
v. Other findings: signs of Albright S, hypothyroidism
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26. d. X ray of the lower ends of radius & ulna:bone age
a. Retarded: hypothyroidism b. Normal: Partial
c. Advanced:
FSH: Low (<2 IU/ml) ---- pseudo-----follow up
Normal (> 2 mIU/ml) ----- true:
CT or MRI --------Normal (idiopathic)
Abnormal (CNS lesion)
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27. Treatment Objectives:
•Arrest maturation until normal pubertal age.
•Attenuate & diminish established precocious characteristics.
•Maximize adult height.
•Avoid abuse, reduce emotional & social problems
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28. Treatment of the cause:
•Albright S ( MPA or Testolactone, aromatase inhibitor).
•Ovarian or CNS tumor (excision).
•Hypothyriodism, CAH
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29. Constitutional: GnRh analogue Drug of choice because it achieves all objectives. It acts by binding to the anterior pituitary receptors causing down-regulation & desensitization of the pituitary.
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30. Regression of symptoms occurs in the first year {Regression of pubertal characteristics, amenorrhea & decreased growth velocity}. Delayed epiphyseal fusion; treatment more effective if begun before bone age >12 yrs.
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31. Maintain E2 at <10 pg/mL.
Children require higher doses than adults for suppression.
Adrenarche will continue.
Treatment is continued until the epiphyses are fused or the appropriate pubertal & chronological ages are matched.
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32. SSC do not develop by the age of 14 y or no menstruation till age of 16y
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33. It is either :
* Delayed onset: Breast bud does not appear till 13 years or menarche does not occur till 16 years . or
* Delayed progreession : Menarche does not occur within 5 years after breast bud .
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34. Causes Early cycles are anovulatory E unopposed by P endometrial hyperplasia Treatment for 3 cycles: Norethistrone acetate 5mg twice daily for 21 d or OCP
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