• enter puberty average age 11-12

• Tanner assessment

  • Breast:

    • 1) Prepubertal

      • appearance of breast mound and papilla

    • 2) Breast bud (10yo {8-13})

      • elevation of breast and papilla as small mound

    • 3) Further enlargmeent

      • further enlargement

        • no separation of contours between breast and areola

    • 4) secondary mound

      • areola and papilla project to form secondary mound above level of breast

    • 5) adult breast

      • projection of areola recedes to megre with general controus of breast

  • Genital

    • 1) prepubertal

    • 2) scrotum and tests enlarge

    • 3) penis enlarges

    • 4) scrotum enlarges further

      • increase penis lenght nad bredth with development of glans

    • 5) adult genitalia

• Pubarche

  • adrenal maturation or adnrenarche

    • doens’t include breast development or testicular enlargement

  • adrenarche

    • several years earlier gonadarche

    -

• Gonadarche

  • increase secretion fo gonadal sex steroids

Delayed pubery

• absence of pubertal development

  • girls >13o (13)

    • no breast budding

  • boys >14yo

    • no enlargement of testes greater than 3mL in boys (14yo)

• also delyaed if starts but fails to progress

  • boys;

    • 4mL -> 12mL over 2 years

    • testicular volume \<12mL @ 15yrs

    • change in texture and size of scrotum likely to represent onset of puberty

  • girls

    • menarche

      • 2yrs after breast budding

      • absent @ 15yo

• bone mineralisation begins in puberty

  • delayed or absent puberty can disrupt this process

causes:

constitutional delay

• familial

• commonest cause

• delayed growth and bone age

  • bone age 2SD below mean

  • spont. progression to puberty when bone age 11-12yo

• normal adult height usually obtained

• low dose testosterone may be required

hypogonadotropic hypogonadism

• may be difficult to distinguish from constitutional delay

• precludes entry to gonadarche

• adrenarche may occur to some extent

• Isolated gonadotropin deficiency

  • inability to release gonadotropins but no other abnormality

    • absent GnRH

  • congenital hypopit

    • midline defects

    • tumours

    • infiltrative disease

    • syndromes

      • Kallman syndrome

        • absence of smell

        • one hand copying other

        • shorened fourth metacarpal bone

        • absent kidney

• abnormalities of CNS

  • panhypopituitarism

    • plyuria and polydipsia

      • low ADH

      • lethargy

        • low ACTH, TSH, GH

      • delayed puberty

        • low luteinising hormone and follicle stimulating hormone

      • braintumour = craniopharyngioma

        • glioma

  • pituitary adenoma

  • germinoma

  • glioma

  • prolactinoma

  • craniopharyngioma

    • peak incidenc teenage years

    • any type of ant. or posterio hormone deficiecny

    • may impinge on optic chiasm

• idiopathic hypopituitarism

  • congenital absence of various combinations

• Syndromes

  • athletic amenorrhoea

  • chronic illness

  • poor nutition

    • anorexia nervosa

  • hypothyroidism

Hypergonadotropic hypogonadism

• elevated gonadotropin and low sex steroids

  • primarygonadal failure

  • Ovarian failure

    • Turner’s syndrome

      • 45XO

      • lack germ cells within ovary

      • primary amenorrhoea and infertility

      • short stature and delayed puberty = common presenting features

      • 1:2000-1:3000 live births

      • consider in any female who is short without contributory history

  • Testicular failure

    • Klinefleter (seminiferous tubular dysgensis)

      • 47XXY

      • 1;500 - 1;1000 males

      • testosterone levels may be close to normla

        • until mid puberty

        • Leydig cell function may be spared

      • LH normal elevated

      • FSH elevated

  • Primary amenorrheoa

    • when determined no secondary sexual developement present

      • serum gonadotropin levels should be obtained

      • determine hypo//hypergonadotropic

    • considerations

      • hypo/hypergonadotropic hypogonadism

      • anatomical

        • normal sexual characteristics

          • imperforate hymen

          • vaginal septum

      • androgen insensitivity

        • noraml feminization

        • absence of pubic/axillary heair

        • primary amenorrhoea

        • allmullerian structures:

          • ovaries

          • fallopian tubes

          • upper 1/3 vagina lacking

          • 46XY

          • intra-abdominal tests

      • Turners

Precocious puberty

• secondary sexual development before

  • 9yo M

  • 8yo F

• central - constitutional/familal

  • premature activation of hypothalamic pituitary-gonadal axis

  • normal but early development

    • tall stature

    • advanced bone age

    • inc. sex steroid and pulsatile gonadotropin secretion

    • inc. response of LF to GbRH

  • clinical course may wax and wane

  • obesity - earlier adrenarche/menarche

  • boys inc. incidence of cns disorders

  • CNS tumour must be considered

    • Hamartomas

      • nonmalignant tumours of tuber cinereum

    • Optic / hypothalmac gliomas (+/- neurofibromatosis)

    • astrocytomas

    • ependymomas

• peripheral

  • no H-P-G axis dependant

  • McCune - ALbright syndrome

    • F>M

    • cafe-au-lait spots

  • adrenal carcinoma

Evaulation

• examination

  • androgen production

• laboratory

  • sex steroid

    • testosterone

    • oestriadiol

    • DHEA-S

    • baseline gonadotropin

      • if elevated into normal pubertal range

        • central = likely

      • if low:

        • no conclusion

        • need GnRH stimulation

          • prepubertal = FSH predominant

          • pubertal = LH predominant

    • thyroid determination

      • severe primary hypothyroidism

        • incomplete prevocious puberty

Isolated premature thlarche (premature breast development)

• isolated appearance of unilateral or bilateral breast tissue in girls

  • 6mo - 3yrs

  • no other signs of puberty

  • no evidence of excessive oestrogen effect

    • vaginal bleeding

    • thickening of vaginal secretions

    • inc. height velocity

    • bone age acceleartion

• exclude endogenous oestrogen

  • cream

  • ingestion

• laboratory investigations not usually necessary

• pelvic uss to exclude ovarian disease

• re-evulated at 6-12mo

  • ensure apparent permature thelarche not beginning of precocious piuberty

  • prognosis = excellent

    • if no progression occurs

Gynaecomastia

• some degree in 45-75% of noraml pubertal boys

• N) androgens -> oestrogen

  • early puberty; only modest androgens rpoduced

  • oestrogen effect can overwhelm androgen effects

• can also suggest possibility of Klinefelter

• prepubertal = unusual source of oestrogen