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PUBERTAL DEVELOPMENT

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

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  1. PUBERTAL DEVELOPMENT Dr Assunta Albanese St George’s Hospital London

  2. PUBERTY Gonadal maturation with acquisition of secondary sexual characteristics and associated growth spurt FERTILITY AND FINAL HEIGHT

  3. 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

  4. TANNER’S STAGING OF PUBERTY IN GIRLS

  5. OVARIAN REGULATION Hypothalamus GnRH release Pituitary gland FSH LH Ovary Inhibin Oestrogen

  6. Oestrogens produced by ovaries induce/ maintain secondary sexual characteristics and sustain germ cell production • Breast development • Libido • Body composition • Bone mineralization

  7. ASSESSMENT OF OVARIAN FUNCTION • Pubertal staging • Hormone levels (LH, FSH, Oestradiol, Inhibin B, progesterone) • Pelvic USS

  8. LH, FSH and E2 and PUBERTAL STAGE in GIRLS

  9. Patterns of LH secretion during pubertal development

  10. 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

  11. 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

  12. 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

  13. TANNER’S STAGING OF PUBERTY IN BOYS

  14. TESTICULAR REGULATION Hypothalamus GnRH release Pituitary gland FSH LH Testis Inhibin Testosterone (From the Sertoli cells) (From the Leyding cells)

  15. 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

  16. ASSESSMENT OF TESTICULAR FUNCTION • Pubertal staging • Hormone levels (LH, FSH, testosterone, Inhibin B) • Sperm count and analysis

  17. LH, FSH and E2 and PUBERTAL STAGE in BOYS

  18. "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)

  19. DELAYED PUBERTY • Onset of puberty after: • 13.4 yrs in girls • 13.8 yrs in boys

  20. 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

  21. CLASSIFICATION OF DELAYED SEXUAL MATURATION • CDGP • Secondary delay: • Chronic systemic illness • Steroid treatment • Psychosocial growth disturbance • Anorexia

  22. 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

  23. 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

  24. 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

  25. 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

  26. CLASSIFICATION OF PRECOCIOUS SEXUAL MATURATION • Gonadotrophin-Dependent (True precocious puberty) • Gonadotrophin-Independent (Pseudo precocious puberty) • Variants of Precocious Sexual Maturation

  27. GONADOTROPHIN-DEPENDENT • Central precocious puberty • Idiopathic • Secondary to CNS abnormalities • Congenital anomalies (hydrocephalus) • Tumours • Acquired (infections, surgery, irradiation) • Primary hypothyroidism

  28. CENTRAL PRECOCIOUS PUBERTY SEXUAL DIMORPHISM • Usually idiopathic in girls (90% or more) • Almost always secondary to lesions in CNS in boys

  29. 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

  30. 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

  31. 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

  32. 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

  33. 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)

  34. 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

  35. 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

  36. VARIANTS OF PRECOCIOUS SEXUAL MATURATION • Isolated premature thelarche • Isolated menarche • Premature adrenarche • Unclassified forms

  37. 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

  38. 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

  39. 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

  40. DELAYED PUBERTY • Onset of puberty after: • 13.4 yrs in girls • 13.8 yrs in boys

  41. 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

  42. CLASSIFICATION OF DELAYED SEXUAL MATURATION • CDGP • Secondary delay: • Chronic systemic illness • Steroid treatment • Psychosocial growth disturbance • Anorexia

  43. 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

  44. 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

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