reproductive Flashcards
what is kallmann syndrome
genetic condition
hypogonadotropic hypogonadism which results in failure to start puberty
condition where male testes/ female ovaries produce little to no sex hormones due to problems with the pituitary gland or hypothalamus
clinical presentation of kallman syndrome
anosomia
undescended testicles
small penile size
facial defects e.g. cleft lip or palate
undeveloped secondary sex characteristics e.g. facial hair, voice, periods, breast development, growth spurts
hormonal investigations for kallmann syndrome
serum fsh and lh - low
growth hormone testing
serum prolactin
imaging for kallmann syndrome
xray- assess bone age
pelvic ultrasound- ovaries and other pelvic organs
MRI of brain -pituitary pathology
management for kallmann syndrome
treat underlying condition
replacement sex hormones
what is premature sexual development
development of secondary sexual characteristics
before 8 years old in females and 9 years old in males
what is precocious puberty
early pubertal development
before 8 yrs in girls and before 9yrs in boys
more worrying if its presents in boys
classification of precocious puberty
central precocious puberty
precocious pseudopuberty
what is central precocious puberty
gonadotrophin- dependent type. premature activation of the hpg axis
what is precocious pseudopuberty
independent of gonadotrophin release
gonads mature without maturation of hpg axis and so GnRH stimulation
causes of central precocious puberty
idiopathic
hypothyroidism
cns abnormalities e.g. hydrocephalus, tumours
causes of precocious pseudopuberty
adrenal disorders
ovarian/ testicular tumour
exogenous sex steroids
what does bilateral enlargement of teste suggest in precocious puberty
intracranial lesion
what do small testes testes suggest in precocious puberty
adrenal cuase
what does unilateral enlargement of teste suggest in precocious puberty
gonadal tumour
