21. Hypopituitarism - etiology, clinical manifestation, treatment Flashcards
what is hypopiturism ?
classification of hypopitutrism ?
diminished or absent secretion of one or more pituitary hormones
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primary
caused by destruction of the anterior pituitary gland
seoncdary
deficiency of hypothalamic stimulatory factors acting on the pituitary
what is the etiology of hypopituatrism ?
developmental and structural
pituatory dysplasia and apslpasa
SEPTO OPTIC DYSPLASIA -
encephalocele
primary empty sella - pituitary gland not seen on the MRI can - sella is filled with csf which compresses the pituatory gland
kallman syndrome
prader willi -
traumatic
surgeical resection
radiation
infection
histoplasmosis- fungal
toxoplasmosisi - parasitic
neoplastic
pitatory adenoma
rathke cysts
infiltrative :
lymphocytic hypophysitis
hemochrombtosis
sarcoidosis
vascular
pituitary apoplexy
infraction with diabetes
sickle cell disease
what are the clinical ?manifestations of hypopiturism
gonadoptrpin hormne deficicny = growth disrder
menstrual disroder = amenorrhea , or metrorrhagia
loss of secondary sexual chareteristics in men
TSH = hypothyroidism
and growth defects
ACTH
addison disease
PRL =
filure of lactation
posterioir pituroy
= diabeets insipidus
diagnosis ?
low tsh
and t4
low acth
and low cortisol
what is pituitary apoplexy ? and what causes it ?
it is an endocrine emergency
bleeding into o impaired blood supply to pitutory gland
acute intrapituatory hemorrhagic vascular event
adenoma
postpartum - sheehan syndrome associated with diabetes
haemorrhage of rathke cyst
hypertension ,
acute shock
what are the clinical manifestation of pituitary apoplexy ?
CNS haemorrhage
and death
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acute symptoms - SUDDEN headache - located behind the eye or temples
meningeal irritation signs - neck rigidity , intolerance to bright light , decreased levels of consciousness
pressure on optic nerve = bilatemporal visual loss
visual acuity reduced , double vision , mydriasis
opthamoplegia
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sudden lack in cortisol =addison
low blood pressure
hypoglycemia
and in severe cases cardiovascular collapse
diagnosis of apoplexy the pitutory gland ?
MRI
ct
infarction of pitutory gland
lumbar puncture to rule out meningitis
treatment of pituitary apoplexy ?
Treatment for acute adrenal insufficiency requires the administration of intravenous saline or dextrose solution
followed by the administration of hydrocortisone
transphenoidal surgery The decision on whether to surgically decompress the pituitary gland and remove any adenoma
hormone replacement therapy
what is sheehan syndrome ?
Ischemic damage to the pituitary following postpartum hemorrhage and vascular collapse
hypertrophy of prolactin-producing regions increases the size of the pituitary gland, making it very sensitive to ischemia.
Hypotensionalongwithvasospasmof the hypophysial arteries.
Usually the posterior pituitary is not damaged because of non portal circulation
what are the signs and symptoms of sheehan syndrome ?
most common initial symptoms of Sheehan’s syndrome are agalactorrhea
gonadotropin deficiency
amenorrhea or oligomenorrhea after delivery
secondary hypothyroidism (hypothyroidism due to pituitary dysfunction) with tiredness, intolerance to cold, constipation, weight gain, hair loss and slowed thinking, as well as a slowed heart rate and low blood pressure
secondary adrenal insufficiency
Growth-hormone deficiency
what is prader willi ?
hypogonadotropic hypogonadism
Deletion/mutation of paternal gene copy + maternal gene methylation
signs and symptoms of prader willi
Muscular hypotonia
Increased appetite (hyperphagia) and obesity
Short stature =
hypogonadism , genital hypoplasia
Facial dysmorphia
mental retardation and adult onset diabetes mellitus
diagnosis of PW ?
genetic tests
Fluorescence in situ hybridization (FISH)
Methylation testing
treatment of prader willi ?
Calorie restriction
Substitution of growth hormone and sex hormones
what is Kallmann syndrome ?
hypogonadotropic hypogonadism, associated with hyposmia/anosmia
most common in male individuals due to genetic mutations
clinical features of Kallmann syndrome ?
Anosmia or hyposmia
Infertility
In male individuals: cryptorchidism and low sperm count
In female individuals: primary amenorrhea
Absent or attenuated pubertal changes
Associated disorders
Renal agenesis
Cleft lip/palate
diagnosis of Kallmann syndrome ?
Clinical presentation
Hormone levels: low levels of GnRH, FSH, LH, estrogen/testosterone (
treatment of Kallmann syndrome ?
Hormone replacement therapy is given in puberty to stimulate the development of secondary sexual characteristics.
For men: testosterone
For women: estrogen and progesterone combination therapy
Gonadotropins or pulsatile GnRH therapy is used to increase fertility.
In men: stimulates testicular growth and sperm production
In women: stimulates ovulation
what is septo - optic - dysplasia
congenital malformation syndrome that feautures a combination of the underdevelopment of the optic nerve, pituitary gland dysfunction, and absence of the septum pellucidum
signs and symptoms of septa-optic-dysplasia ?
Optic nerve underdevelopment :
visual impairment in one or both eyes,
There may also be nystagmus (involuntary eye movements, often side-to-side) or other eye abnormalities
Pituitary hormone abnormalities
Underdevelopment of the pituitary gland in SOD leads to hypopituitarism, most commonly in the form of growth hormone deficiency.
Mid-line brain abnormalities
In SOD mid-line brain structures, such as the corpus callosum and the septum pellucidum may fail to develop normally, leading to neurological problems such as seizures or development delay.
leads to diabetes insidious , GH deficiency =short stature and TSH deficicny ,
what is the diagnosis of sod ?
when at least two of the following triad are present: optic nerve underdevelopment; pituitary hormone abnormalities; or mid-line brain abnormalities.
MRI scans
blood hormone levels
what is the treatemnet for SOD?
no cure
hormone replacement
what are the hormone replacement therapy regimes ?
ACTH hormone replacement
- hydrocortisone
10-20mg in morning - HIGHEST IN THE MORNING
5-10mg in evening
or
cortisone acetate
or prednisone
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FSH AND LH
in males
testosterone enthanate
or testosterone skin patches
in females
conjugated estrogen and progesterone
estradiol skin patches
for fertility - gonadotropins and human chorionic gonadotropins
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GH
adults - somatotropoinin
vasopressin
intranasal desmopressin
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TSH
L thyroxine
