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PUBERTY,�PUBERTAL ABNORMALITIES & DELAYED PUBERTY

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Dr. James Bitrus, FWACS.

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OUTLINE

  • Introduction
  • Age at puberty
  • Events during puberty
  • Milestones of puberty
  • Mechanism of puberty
  • Pubertal abnormalities
  • Delayed puberty

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INTRODUCTION

  • A period of transition from childhood to adulthood characterised by the development of secondary sexual characteristics leading to full sexual maturity and fertility potential

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  • It is endocrine driven and has biological, morphological and psychological changes

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AGE AT PUBERTY

  • Age at puberty is variable

8-13yrs in females

9-14 yrs in males

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  • It is affected by environment, nutrition and genetic factors

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EVENTS DURING PUBERTY

  • Thelarche
  • Pubarche
  • Menarche: 1st menstrual period.
  • Adrenarche: ↑adrenal androgen secretion responsible for axillary & pubic hair growth

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MILESTONES OF PUBERTY

  • Thelarche 8-10
  • Adrenarche 9-11
  • Growth Spurt 10-12
  • Pubarche 10-12
  • Menarche 11-15
  • Ovulation 12-17

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MECHANISM OF PUBERTY…

  • Both Adrenals and gonads are concerned with puberty.

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  • In childhood the output of gonadal steroids and adrenal androgens is low.

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  • Before puberty, tiny amounts of steroids produced by the prepubertal gonad is enough to inhibit gonadotrophin output.

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…MECHANISM OF PUBERTY

  • At puberty the sensitivity of the hypothalamus to the steroids diminishes.

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  • Amount of steroids are no longer enough to inhibit gonadotrophin output and so the gonadotropins (FSH,LH) rise.

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  • This begins in about the 8th year of life; and usually culminating in the onset of puberty and menstruation between the ages of 11 and 16 years in girls (Average 13 years).

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…MECHANISM OF PUBERTY

  • Estrogen (E), though low, give negative feedback to the very sensitive hypothalamic-pituitary system (Gonadostat) so that very little or no gonadal steroids and gonadotropins are secreted in early childhood years.

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  • Sensitivity is lost as puberty approaches, so that these hormones are secreted and so cause endocrine changes

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…MECHANISM OF PUBERTY

  • Pulsatile secretion of gonadotropins leading to gonadal stimulation (Increased GnRH, FSH, LH, Ovarian follicular development, Estrogen).
  • This gonadarche leads to the following changes:

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  • Uterus: uterus:cervix ratio of 1:2 becomes 1:1and later 2:1;

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  • Endometrium: proliferates and when E level drops, it sheds and bleeding is visible (menarche)

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…MECHANISM OF PUBERTY

  • Vagina: thin epithelium becomes stratified; cells rich in glycogen; Doderlein’s bacili convert glycogen to lactic acid leading to acid PH of 4-5),

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  • Vulva: mons pubis and labia minora increase in size

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  • Breasts: duct system proliferate, fat is deposited and breasts become rounded, progesterone causes increase in acini development.

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TANNER STAGING

STAGE

BREAST

PUBIC HAIR

STAGE I

Prepubertal stage; elevation of papilla only

No pubic hair present

STAGE II

Breast buds and papilla slightly elevated, and side of labia areola begin to enlarge

Sparse, long hair on either majora

STAGE III

Further enlargement of the entire breast tissue

Darker, coarser and curly hair over the mons pubis

STAGE IV

Secondary mound of areola and papilla projecting above the breast tissue

Adult type hair covering the mons only

STAGE V

Areola recessed to general contour of breast (14.5 yrs)

Adult hair with an inverse triangle distribution (female escutcheon) covering the medial thighs (13.7 yrs)

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Growth Spurt in Puberty

  • Before puberty there is relatively little difference between the average heights and weights of girls and boys.

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  • Between the ages of 8 and 13, girls tend to be heavier than boys of similar age because of the earlier pubertal growth spurt before menarche. Little growth occurs afterwards.

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  • Boys are finally taller than girls

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…Growth spurt in puberty

  • Appearance of pubic hair and growth spurt are associated with adrenal androgens.

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  • In female they depend almost entirely on the adrenals.

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  • In the male, testicular androgens modify the distribution of pubic hair and forehead hair line and increase the intensity of the spurt.

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PUBERTAL ABNORMALITIES

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TYPES OF PUBERTAL ABNORMALITIES

PRECOCIOUS PUBERTY

  • Any secondary sexual characteristics before age 8 or menstruation before age of 10.
  • Isosexual if due to excess estrogen
  • Heterosexual if due to excess androgens

GnRH Dependant (complete, central, isosexual or true)

  • Complete – Constitutional, juvenile primary hypothyroidism, intracranial lesions
  • Incomplete – premature thelarche, premature pubarche, premature menarche

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TYPES OF PUBERTAL ABNORMALITIES

GnRH Independent (precocious pseudopuberty or peripheral)

  • Ovary – granulosa cell tumour, theca cell tumour, Leydig cell tumour, chorionic epithelioma, androblastoma, McCune-Albright syndrome
  • Adrenal - hyperplasia, tumour
  • Liver – hepatoblastoma
  • Iatrogenic – estrogen or androgen intake

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DIAGNOSIS OF PUBERTAL ABNORMALITIES

  • History – family, time of development of sexual characteristics, others
  • Examination – Tanner stage, others
  • Investigations – x-ray for bone age, Ultrasound scan, skull x-ray, CT Scan, MRI, serum hormones – HCG,FSH, LH,TSH, T4/T3,E, testosterone, DHEA, 17OH P, EEG, vaginal smear

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MANAGEMENT OF PUBERTAL ABNORMALITIES

  • Treat cause
  • Reduce gonadotropins
  • Supress steroidogenesis
  • Protect from sex abuse

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DELAYED PUBERTY

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DEFINITION

Absence of breast tissue or pubic hair appearance by 13-14 years of age OR menarche appears as late as 16 years

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CAUSES OF DELAYED PUBERTY

  • Hypergonadotropic hypogonadism (gonadal dysgenesis 45X0, pure gonadal dysgenesis 46XY,46XX, ovarian failure );
  • Hypogonadotropic hypogonadism (constitutional delay, chronic illness, primary hyperthyroidism, Kallman’s syndrome, intracranial lesions, )
  • Eugonadism (Mullerian agenesis, imperforate hymen, transvaginal septum, androgen insensitivity syndrome)

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TREATMENT OF DELAYED PUBERTY

  • Treat according to cause

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CONCLUSION