21. Hypopituitarism - etiology, clinical manifestation, treatment Flashcards

1
Q

what is hypopiturism ?

classification of hypopitutrism ?

A

diminished or absent secretion of one or more pituitary hormones

========

primary
caused by destruction of the anterior pituitary gland

seoncdary
deficiency of hypothalamic stimulatory factors acting on the pituitary

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2
Q

what is the etiology of hypopituatrism ?

A

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

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3
Q

what are the clinical ?manifestations of hypopiturism

A

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

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4
Q

diagnosis ?

A

low tsh
and t4

low acth
and low cortisol

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5
Q

what is pituitary apoplexy ? and what causes it ?

A

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

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6
Q

what are the clinical manifestation of pituitary apoplexy ?

A

CNS haemorrhage
and death

=====

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

======
sudden lack in cortisol =addison
low blood pressure
hypoglycemia

and in severe cases cardiovascular collapse

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7
Q

diagnosis of apoplexy the pitutory gland ?

A

MRI
ct
infarction of pitutory gland

lumbar puncture to rule out meningitis

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8
Q

treatment of pituitary apoplexy ?

A

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

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9
Q

what is sheehan syndrome ?

A

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

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10
Q

what are the signs and symptoms of sheehan syndrome ?

A

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

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11
Q

what is prader willi ?

A

hypogonadotropic hypogonadism

Deletion/mutation of paternal gene copy + maternal gene methylation

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12
Q

signs and symptoms of prader willi

A

Muscular hypotonia

Increased appetite (hyperphagia) and obesity

Short stature =

hypogonadism , genital hypoplasia

Facial dysmorphia

mental retardation and adult onset diabetes mellitus

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13
Q

diagnosis of PW ?

A

genetic tests
Fluorescence in situ hybridization (FISH)
Methylation testing

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14
Q

treatment of prader willi ?

A

Calorie restriction

Substitution of growth hormone and sex hormones

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15
Q

what is Kallmann syndrome ?

A

hypogonadotropic hypogonadism, associated with hyposmia/anosmia
most common in male individuals due to genetic mutations

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16
Q

clinical features of Kallmann syndrome ?

A

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

17
Q

diagnosis of Kallmann syndrome ?

A

Clinical presentation

Hormone levels: low levels of GnRH, FSH, LH, estrogen/testosterone (

18
Q

treatment of Kallmann syndrome ?

A

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

19
Q

what is septo - optic - dysplasia

A

congenital malformation syndrome that feautures a combination of the underdevelopment of the optic nerve, pituitary gland dysfunction, and absence of the septum pellucidum

20
Q

signs and symptoms of septa-optic-dysplasia ?

A

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 ,

21
Q

what is the diagnosis of sod ?

A

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

22
Q

what is the treatemnet for SOD?

A

no cure

hormone replacement

23
Q

what are the hormone replacement therapy regimes ?

A

ACTH hormone replacement
- hydrocortisone
10-20mg in morning - HIGHEST IN THE MORNING
5-10mg in evening

or

cortisone acetate

or prednisone

========
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

=======
GH

adults - somatotropoinin
vasopressin
intranasal desmopressin

======
TSH
L thyroxine