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What is hirsutism
- Increase in terminal hair due to increased
androgen sensitivity
- Exclude endocrine disorders if menstrual
disturbances and acne
o Polycystic ovaries
o Adrenal gland disorders
These are generally benign but may need
endocrine assessment as they may indicate
abnormal early puberty
Premature adrenarche
- Present earlier than expected with
o Secondary sexual hair
o Acne and seborrhea
o Increased body odour
- Low birth weigth children has increased
frequency
- Some advancement of bone age
- Mild increase in plasma adrenal androgens
Important to exclude adrenal or gonadal
pathology
Treatment
- Females may present later with polycycstic
ovaries
Premature thelarche
- Breast development BEFORE 8 with NO
other pubertal signs
- Diagnosis = must have prepubertal
oestradiol levels
Important to exclude
- Ecogenous oestrogen source
- True precocious puberty with advanced
bone age and elevated oestradiol
Treatment = none, may regress
OTHER pathological variations in puberty
that require evaluation
- Vaginal bleeding but absent or early breast
development
- Small testicles, young age and penile
enlargement
What conditions can cause absent puberty
and what can cause early puberty
Causes
- Genetic pituitary deficiency
- Tumour replacing normal pituitary tissue
- Trauma = pituitary stalk especially
- Surgery often to remove tumour
Hypothalamus problem
Low FSH, LH and low oestradiol in females and low
testosterone in males (HENCE it is
indistinguishable on blood test from secondary
gonadal failure. GnRH is NOT measurable in
peripheral blood
Causes
- Isolated GnRH deficiency or Kallman
syndrome (has anosmia)
- Head trauma
- Iron deposition Iron overload in
thalassemia major
- Tumour
- Raddiation or sugery therapy for tumour
- CHARGE syndrome, Prader Williw syndrome
Again, OFTEN associated with other hormone
deficiencies
GnRH is available as injection and is used in
female infertility
What is primary amenorrhoea?
Absent menarche from 16 years onwards
- Imperforated hymen
- Hypothalamic, pituitary or ovary
disorder
- Mullerian agensis 46XX = normal breast
development with absent uterus, fallopian
tubes and upper 2/3 vagina
- Complete androgen insensitivity
syndrome
o Receptor abnormality, XY
karyotype, femininsed with
endogenous oestradiol from
testosterone
o 46 XY
o Tall stature, female phenotype with
little body hair, inguinal gonads,
lower 1/3 vagina only (upper
structures regress with mullerian
inhibitory factor)
What is precocious puberty?
Puberty occurring TOO early
CCP = central precocious puberty
Therapy in adolescence
Complaince = health care professional driven
Adherence therapeutic alliance
Capacitance ability of the patient
Concordance implies both patient and helath
professional negotiate, understood and agreed
upon a medical course of action
Measurement of therapy adherence
- Patient spontaneously owns up
- Rate their adherence out of 10
- Loss of disease control or failure to respond
to empirically supported interventions
- Poor clinic attendance record
- Questionnaire
Rate of prescription refill
Pill count
Plasma or urine for drug substance levels