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PUBERTAL DEVELOPMENT. Dr Assunta Albanese St George’s Hospital London. PUBERTY. Gonadal maturation with acquisition of secondary sexual characteristics and associated growth spurt FERTILITY AND FINAL HEIGHT. PUBERTY. Average age of onset: 11.4 years in girls 12.0 years in boys

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pubertal development
PUBERTAL DEVELOPMENT

Dr Assunta Albanese

St George’s Hospital

London

puberty
PUBERTY

Gonadal maturation with acquisition of secondary sexual characteristics and associated growth spurt

FERTILITY AND FINAL HEIGHT

puberty3
PUBERTY

Average age of onset:

  • 11.4 years in girls
  • 12.0 years in boys

First signs of pubertal maturation:

  • breast budding in girls
  • increase in testicular volume in boys
slide6

OVARIAN REGULATION

Hypothalamus

GnRH release

Pituitary gland

FSH LH

Ovary

Inhibin

Oestrogen

slide7

Oestrogens produced by ovaries induce/ maintain secondary sexual characteristics and sustain germ cell production

      • Breast development
      • Libido
      • Body composition
      • Bone mineralization
assessment of ovarian function
ASSESSMENT OF OVARIAN FUNCTION
  • Pubertal staging
  • Hormone levels (LH, FSH, Oestradiol, Inhibin B, progesterone)
  • Pelvic USS
diagnostic value of pelvic uss
DIAGNOSTIC VALUE OF PELVIC USS
  • Depend on experience of examiner!
  • Size and shape of uterus and ovarian volume and appearance are a indicator of the degree of pubertal development
evaluation of ovaries at uss
EVALUATION OF OVARIES AT USS
  • Shape is oval. If smaller than 1 ml  prepubertal. In young adult  ~6.5 ml
  • Ovarian follicles can be detected from any age of early infancy onward
  • Follicles increase progressively in size and number after 8.5 yrs.
  • 3-4 small cysts (~ diameter 5 mm) normal at any age
evaluation of uterus at uss
EVALUATION OF UTERUS AT USS
  • Shape depends on the age of child:
    • During neonatal period and infancy  drop shaped
    • By 8 yrs  tubular form
    • During puberty  pear shape
  • Cervix to corpus ratio:
    • 2:1 pre-puberty
    • 1:2 post-puberty
  • Angle between corpus and cervix only seen after puberty
  • Endometrium thickness not seen in prepuberty
slide16

TESTICULAR REGULATION

Hypothalamus

GnRH release

Pituitary gland

FSH LH

Testis

Inhibin

Testosterone

(From the Sertoli cells)

(From the Leyding cells)

slide17

Testosterone produced by Leyding cells induces/ maintains secondary sexual characteristics and sustain germ cell production

    • Virilization of external genitalia
    • Phallus growth
    • Pubic, axillary, facial hair
    • Libido
    • Erections/ejaculate
    • Voice change
    • Body composition
    • Bone mineralization
assessment of testicular function
ASSESSMENT OF TESTICULAR FUNCTION
  • Pubertal staging
  • Hormone levels (LH, FSH, testosterone, Inhibin B)
  • Sperm count and analysis
consonance of puberty
"CONSONANCE" OF PUBERTY
  • Close relationship between secondary sexual characteristics and pubertal growth spurt
  • In girls the pubertal growth spurt occurs early in puberty, (B2-3)
  • In boys the pubertal growth spurt occurs late in puberty, (G3-4, 10 ml testicular volume)
slide24

DELAYED PUBERTY

  • Onset of puberty after:
  • 13.4 yrs in girls
  • 13.8 yrs in boys
concerns raised by delayed puberty
CONCERNS RAISED BY DELAYED PUBERTY
  • Possibly sinister underlying cause
  • Fear that puberty will never occur
  • Emotional and psychosocial upset of immaturity, specially when associated with short stature
  • Long term sequelae: ? Reduced bone mineralization
classification of delayed sexual maturation
CLASSIFICATION OF DELAYED SEXUAL MATURATION
  • CDGP
  • Secondary delay:
    • Chronic systemic illness
    • Steroid treatment
    • Psychosocial growth disturbance
    • Anorexia
slide27
Hypogonadotrophic hypogonadism
    • Isolated gonadotrophin deficiency
    • Multiple pituitary hormone deficiency
    • Secondary to CNS tumours or cranial irradiation
  • Hypergonadotrophic hypogonadism
    • Klinefelter’s and Turner’s Syndromes
    • Primary or secondary gonadal failure
  • Dysmorphic syndromes
    • Noonan’s syndrome, Prader-Willi, etc
delayed puberty
DELAYED PUBERTY
  • Absence of a clear pattern of pulsatile gonadotrophin secretion
  • Pre-pubertal LH and FSH levels
  • Development of secondary sexual characteristics
  • Normal "Consonance"
  • Bone age delay
  • Final height is not impaired except if severe degree of delay
conclusion
CONCLUSION
  • A good understanding of normal puberty is necessary to fully assess disorders of growth and puberty
  • The commonest disorders of precocious/delayed puberty are idiopathic
  • Psychological disturbances is the commonest indication for intervention
slide30

Precocious Puberty

  • Onset of puberty before:
  • 8 yrs in girls
  • 9 yrs in boys
  • Early Puberty
  • Onset of puberty between:
  • 8 - 9 yrs in girls
  • 9 - 10 yrs in boys
classification of precocious sexual maturation
CLASSIFICATION OF PRECOCIOUS SEXUAL MATURATION
  • Gonadotrophin-Dependent (True precocious puberty)
  • Gonadotrophin-Independent (Pseudo precocious puberty)
  • Variants of Precocious Sexual Maturation
gonadotrophin dependent
GONADOTROPHIN-DEPENDENT
  • Central precocious puberty
    • Idiopathic
    • Secondary to CNS abnormalities
      • Congenital anomalies (hydrocephalus)
      • Tumours
      • Acquired (infections, surgery, irradiation)
  • Primary hypothyroidism
central precocious puberty
CENTRAL PRECOCIOUS PUBERTY

SEXUAL DIMORPHISM

  • Usually idiopathic in girls (90% or more)
  • Almost always secondary to lesions in CNS in boys
gonadotrophin dependent34
GONADOTROPHIN-DEPENDENT
  • Pulsatile gonadotrophin secretion, especially overnight
  • LH : FSH ratio > 1
  • Gonadal activation with sex steroid production
  • Development of secondary sexual characteristics
  • Normal "Consonance"
  • Bone age acceleration
  • Final height impairment
g onadotrophin independent
GONADOTROPHIN-INDEPENDENT
  • Adrenal disorders
      • Tumours secreting sex steroids
      • Congenital adrenal hyperplasia
  • Gonadal disorders
      • Ovarian cyst/tumours secreting sex steroids
      • Leydig cell tumour
  • Exogenous sex steroids
  • McCune-Albright Syndrome
  • Testotoxicosis
gonadotrophin independent
GONADOTROPHIN-INDEPENDENT
  • Sex steroid production from gonads or adrenal gland or exogenous source
  • Suppressed LH and FSH levels
  • Secondary sexual characteristics or virilization
  • Growth acceleration
  • Bone age acceleration with final height impairment
mccune albright syndrome
McCune - Albright Syndrome
  • Fibrous dysplasia of skull and long bone
  • "Cafe-au lait" patches with serrated edges
  • Autonomous endocrine overactivity :
      • Precocious puberty
      • Hyperthyroidism
      • Hypercortisolism
      • Pituitary adenomas secreting GH/ PRL
      • Hyperparathyroidism
mccune albright syndrome38
McCune - Albright Syndrome
  • Precious puberty mainly described in girls
    • First phase: intermittent periods of breast development and vaginal bleeding (gonadotrophin independent)
    • Second phase: Central precocious puberty (gonadotrophin dependent)
mccune albright syndrome39
McCune - Albright Syndrome
  • Gene mutation for the a-subunit of the G protein, which stimulate cAMP formation
  • Activation of receptors that operate with a cAMP-dependent mechanism
  • The somatic mutationoccurs early in embriogenesis
testotoxicosis
TESTOTOXICOSIS
  • Occurs in boys, familiar, Autosomic Dominant
  • Normal "Consonance"
  • Extreme degree of virilization compared to the testicular enlargement
  • Prepubertal values of FSH and LH
  • Failure to respond to GnRH analogue treatment
  • Due to a mutation of LH receptor with constant activation of the G protein even without ligand
variants of precocious sexual maturation
VARIANTS OF PRECOCIOUS SEXUAL MATURATION
  • Isolated premature thelarche
  • Isolated menarche
  • Premature adrenarche
  • Unclassified forms
isolated premature thelarche
ISOLATED PREMATURE THELARCHE
  • Isolated cyclic breast enlargement, usually < 2 yrs old
  • Absence of other signs of puberty
  • Absence of behavioural problems
  • Normal growth and bone maturation
  • Predominant FSH pulsatility
  • Development of follicular ovarian cysts
premature pubarche
PREMATURE PUBARCHE
  • Usually begins at around 6-8 years of age
  • Early appearance of pubic hair, with or without axillary hair
  • Puberty usually occurs at a normal time
  • Slight growth spurt and advance in bone maturation
  • Final height prognosis is not compromised
premature pubarche45
PREMATURE PUBARCHE
  • Increased adrenal production of sex hormones
  • Gonadotrophin secretion is prepubertal

Clitoral virilization in girls and phallic enlargement in boys together with excessive bone age maturation should suggest excessive production of sex hormones due to CAH or an adrenal tumour

slide46

DELAYED PUBERTY

  • Onset of puberty after:
  • 13.4 yrs in girls
  • 13.8 yrs in boys
concerns raised by delayed puberty47
CONCERNS RAISED BY DELAYED PUBERTY
  • Possibly sinister underlying cause
  • Fear that puberty will never occur
  • Emotional and psychosocial upset of immaturity, specially when associated with short stature
  • Long term sequelae: ? Reduced bone mineralization
classification of delayed sexual maturation48
CLASSIFICATION OF DELAYED SEXUAL MATURATION
  • CDGP
  • Secondary delay:
    • Chronic systemic illness
    • Steroid treatment
    • Psychosocial growth disturbance
    • Anorexia
slide49
Hypogonadotrophic hypogonadism
    • Isolated gonadotrophin deficiency
    • Multiple pituitary hormone deficiency
    • Secondary to CNS tumours or cranial irradiation
  • Hypergonadotrophic hypogonadism
    • Klinefelter’s and Turner’s Syndromes
    • Primary or secondary gonadal failure
  • Dysmorphic syndromes
    • Noonan’s syndrome, Prader-Willi, etc
delayed puberty50
DELAYED PUBERTY
  • Absence of a clear pattern of pulsatile gonadotrophin secretion
  • Pre-pubertal LH and FSH levels
  • Development of secondary sexual characteristics
  • Normal "Consonance"
  • Bone age delay
  • Final height is not impaired except if severe degree of delay
conclusion51
CONCLUSION
  • A good understanding of normal puberty is necessary to fully assess disorders of growth and puberty
  • The commonest disorders of precocious/delayed puberty are idiopathic
  • Psychological disturbances is the commonest indication for intervention