deck 3 Flashcards
(76 cards)
hyperpituitarism
- primary - rare to have hyper secretion of pituitary hormones in peds
- secondary - from target hormone deficiencies resulting in decreased hormonal feedback - i.e. in hypogonadism, hypoadrenalism or hypothyroidism
Definition of precocious puberty
secondary sex characteristics
before 8 years in girls
before 9 years in boys
Classification of precocious puberty
central - gonadotropin dependent - alwasys isosexual
peripheral - independent
precocius puberty
leads to advanced linear growth, advanced bone age, shorter height
Differential of tall stature and overgrowth
most common - is familial/constitutional
fetal overgrowth - maternal DM, cerebral gigatism, Weaver, syndrome, Beckwith-Wiedemann Post Natal overgrowth (childhood tall stature) : familial tall stature, cerebral gigantism, exogenous obesity excess GH secretion (pituitary) McCune Albright syndrome Precocious puberty marfan Klinefelter (XXY) Weaver syndrome Fragile X syndrome Homocystinuria XYY hyperthyroidism Post Natal overgowth to adult tall stature - familial, androgen or estrogen deficiency o estrogen resistence testicular feminization ACTH or cortisol deficiecny or resistnace excess GH secretion Marfan Klinefelter XYY
Feature sod Klinefelter
tall stature learning disabilities gynecomastia decreased upper o lower body segment hypotonia, clinodactyly, hypertelorism small balls - bus androgen can be low nomal infertiliy small phallus hypospadias, cryptorchism
hyperthyroidism in teens - what is usual adult height?
normal
but will have rapid growth in adolescence
most commonly caused by Graves disease -moer in girls
exogenous obesity and growth?
rapid linear growth and early onset of puberty, usually normal adult height
Appropach to Tall stature:
Ddx of normal vs rare pathology
if familial tall stature and normal PE then no investigations needed
Can do a bone age to predict adult height and help guide management
If we think pathology then do some labs:
1. IGF1 and IGF binding protein 3 - screening tests
2. if these suggest high HG then do a MRI of the pituitary
can consider chromosome in boys - especially if ratio of upper to lower is decreased (aka long legs) or if developmental disability
3. if suspect marfan or homocystinuria then refer to cardio/optho
4. TSH can be considered to rule out hyperthyroidism
5. consider the status of puberty - i.e. precocious or delayed - precocious leads to accelerated growth but shorter adult height
- delayed puberty can sometimes lead to prolonged growth and increased or normal adult height
Who to treat with tall stature?
> 3-4SD above the mean and evidence of significant psychosocial impairment
treatment choices:
- can consider sex steroids - need to give early in puberty to help with epiphyseal fusion
boys - before bone age 14 (can do testosterone ) - although used rarely
girls: oral estrogens - before bone age of 12
if needed add progesterone after 1 year of estrogen
Side effects of estrogen therapy in girls
benign breast disease, cholelithiasis, hypertension, irregular periods, weight gain, nausea, limb pain, galactorrhea and thrombosis
may have reduced fertility later in life
not that much experience so should look at risks and benefits
how it works: affects growth hormone axis as well as direct on epiphyseal fusion
treatment of klinefelter
can give testosterone in puberty to help with secondary sex characteristics, can diminish adult height
Which is the most common pituitary tumour in adolescents?
prolactinoma
clinical manifestations:
headache, amenorrhea, galactorrhea
more girls than boys - usually after initial puberty
labs: elevated prolactin levels
30% get hypopituitarism, especially GH deficiency
DDx for prolactinoma
primary hypothyroidism can lead to hyperprolactinemia and pituitary hyperplasia
can also get elevated prolactin with mediations - i.e. antipsychotics
pituitary dysfunction from craniopharyngioma
other benign conditions
Treatment: dopamine agonist bromocriptine or long acting cabergoline
if medical treatment is unsuccessful then do transsphenoidal surgery
What is the most sensitive indicator of excess glucocorticoid secretion in children?
growth failure - usually precedes other manifestations
Cushing’s disease - when you have a ACTH producing pituitary adenoma **more common than primary adrenal causes of Cushing syndrome except in younger kids (more likely to have adrenal carcinomas)
McCune Albright syndrome
cafe au lait (coast of main)
fibrous dysplasia
precocious puberty
How to diagnose GH excess?
failure to suppress GH levels with glucose challenge
single measurement is not adequate
elevated IGF1 will increase suspicion of GH excess
if suggest GH excess then should do a MRI brain
Causes of excess GH secretion
pituitary - rare most common is pituitary adenoma
Other tumours: i.e. MEN syndromes from pancreas tumours
get gigantism if open growth places, acromegaly if after
syndromes: McCune Albright can cause increased GH
increased GH with tumours associated with neurofibromatosis, Tuberous Slerosis and Carney
Treatment of excess GH
pituitary adenoma - resection of tumour
pituitary irradiation and medical therapy
medical therapy: somatostatin analogues - ie octreotide
if also have prolactin over secretion, also do a dopamine agoniest (i.e. bromocriptine)
Pegvisomant - GH receptor antagonist
A child is born >90th percentile for length and weight at birth, as well as increased HC
rapidly growth and is 99th percentile by age 1 .
kid has large dolichocephalic head (dolicocephaly is a long head) , hypertelorism, down slanted palpebral tissues (nelson says antimongoloid, so racist), high arched palate,
what syndrome should you think of?
SOTOS syndrome:
large hands and feet with thickened SC tissue, clumsy and awkward gain, and trouble with sports
most have some developmental disability
usually bone age consistent with chronological age
common to have abnormal EEG, can have dilated ventricles on imaging
can have increased risk of cancer: especially hepatic carcinoma, Wilms, ovarian and parotid tumours
Large Neonate - Differential
most common cause - maternal diabetes (even without clinical symptoms or FHx)
Syndromes:
- Beckwith-Wiedemann is most common - clinical features at birth: microsomia with macroglossia, hepatosplenomegaly, nephromegaly and omphalocele
hypoglycaemia from hyperinsulinemia from pancreatic beta cell hyperplasia
increased chance of cancers: Wilms timor and adrenocortical carcinoma
imprinting disorder
Definition of precocious puberty
secondary sex characteristics
before 8 years in girls
before 9 years in boys
How does puberty start
gonadarche - pulsatile GnRH -
leads to LH and FSH secretion
adrenocortical androgens also have a role in puberty - increased DHEA and DHEAS happens at 6-8 yeas of age, before any change in LH or sex hormones - aka adrenarche
adrenarche usually before gonadarche by a few years, but are NOT causally related
which hormone more related to ultimate epiphyseal fusion and osseous maturation, estrogen or androgen?
estrogen more likely