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panhypopituitarism
Pattawan Wongwijitsook
Maharat Nakhon Ratchasima hospital
17 Nov 2013
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PITUITARY GLAND
(HYPOPHYSIS CEREBRI)
The master of endocrine glandsmaster of endocrine glands
It is a small oval structure 1 cm in diameter.
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PITUITARY GLAND
X-RAY SKULL: LATERAL VIEW SAGITTAL SECTION OF HEAD & NECK
Hypophyseal fossa
Sphenoidal air sinus
Pituitarygland
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A fold of dura mater (Diaphragma sellae) covers the pituitary
gland & has an opening for passage of infundibulum
(pituitary stalk) connecting the gland to hypothalamus.
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BLOOD SUPPLY OF PITUITARY GLAND
ARTERIES: ARTERIES: Superior & inferior hypophyseal arteries
(branches of internal carotid artery)
VEINS:VEINS: Hypophyseal veins drain into Cavernous Sinuses.
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PITUITARY GLAND
• Anterior pituitary
• Posterior pituitary
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ANTERIOR PITUITARY CELLS
Anterior pituitary cell hormone effect
somatotrophs GH -Stimulate general body growth
-Regulate metabolism
thyrothrophs TSH -control secretion and others
activity of thyroid gland
gonadotrophs FSH
LH-Stimulate secretion of estrogen
and progesterone
- secretion of testosterone
- maturation of oocyte in ovary
and sperm in testes
Pituitary society
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Anterior pituitary cells hormone effect
Lactothrophs prolactin - Initiated milk
production in
mammary gland
corticothrophs ACTH
MSH
-Stimulate adrenal
cortex secrete
glucocorticoid
-Affect skin
pigmentation
Posterior pituitary
(storage from hypothalamus)
Oxytocin
ADH
-Contract uterine
(delivery)
- milk inject (breast
feeding)
- control body water
balancePituitary society
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•
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Harrison’s 18th edition
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hypopituitary
• Definition
Partial or complete deficiency of one or
more pituitary hormone.
It may arises as a congenital defect during the
development of the pituitary gland or as a result of acquired
disease of the pituitary gland . The parasellar structures , or
the hypothalamus.
Williams Textbook of endocrinology 10 edition
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Epidermiology
• Prevalence 45 cases per 100,000
• Incidence of about 4 cases per 100,000 per
year in the normal population.
• Pituitary adenomas or incidentalomas found
27% of postmortem studies
10% of MRI studies
Williams Textbook of endocrinology 10 edition
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Epidermiology
• No specific gender , ethnic , geographic or age
group
• Associated 1.8 fold higher mortality compared
with an age- and sex- match population.
• Cardiovascular and cerebrovascular death rate
are higher
Williams Textbook of endocrinology 10 edition , pituitary society
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hypopituitary
• The clinical presentation of anterior pituitary
Acquired pituitary insufficiency
- degree of hormone deficiency
- the number of hormone impaired
- the rapidity of onset
Williams Textbook of endocrinology 10 edition
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hypopituitary
• The clinical presentation of anterior pituitary
Congenital pituitary insufficiency
- the eariler the age of onset
- the greater thr severity of thyroid ,
gonadal , adrenal , growth , or water
disturbance
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hypopituitary
• The resilience of the individual pituitary cell
lineages in the presence of each causes also
differs.
• Prolactin deficiency is rare except for
complete pituitary destruction or genetic
syndrome
Williams Textbook of endocrinology 10 edition
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hypopituitary
• The lactotrophs cell is often hyperfunctional
as a result of decrased tonic inhibitory signals.
• GH was diminished function prior than others
trophic hormones.
• The corticotrophs and thyrotrophs cell are
usaully the last to lost function.
Williams Textbook of endocrinology 10 edition
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Hypopituitary causes
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Symptom of hypopituitarism
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Symptom of hypopituitarism
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Gonadotropin Deficiency
Women
• Oligomenorrhea or
amenorrhea
• Loss of libido
• Vaginal dryness or
dyspareunia
• Loss of secondary sex
characteristics (estrogen
deficiency)
Men
• Loss of libido
• Erectile dysfunction
• Infertility
• Loss of secondary sex characteristics (testosterone deficiency)
• Atrophy of the testes
• Gynecomastia (testosterone deficiency)
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ACTH Deficiency
• Results in hypocortisolism
– Malaise
– Anorexia
– Weight-loss
– Gastrointestinal disturbances
– Hyponatremia
• Pale complexion
– Unable to tan or maintain a tan
• No features of mineralocorticoid deficiency
– Aldosterone secretion unaffected
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TSH Deficiency
• Hypothyroidism
• Atrophic thyroid gland
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Prolactin Deficiency
• Inability to lactate postpartum
• Often 1st manifestation of Sheehan syndrome
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Growth Hormone Deficiency
• Adults– Often asymptomatic
– May complain of
– Fatigue
– Degrees exercise tolerance
– Abdominal obesity
– Loss of muscle mass
• Children– GH Deficiency
– Constitutional growth delay
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hypopituitary
• Two thirds of pituitary macroadenoma ,
craniopharyngioma , and others parasellar lesion �
pituitary reserve function.
• Intrasellar aneurysm , pituitary metastasis ,
parasellar meningioma , optic glioma , hypothalamic
astrocytoma � pituitary failure
Williams Textbook of endocrinology 10 edition
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hypopituitary
• Prolactin should be measure because many
pt. with hypopituitarism also present with
secondary hyperprolactinemia.
• 25% of pt. loss of pituitary function after
surgery
Williams Textbook of endocrinology 10 edition
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Screening for pituitary failure
• Pt with hypothalamic or pituitary mass lesion.
• Developmental craniofacial abnormality.
• Inflammatory disorder
• Brain granulomatous disease
• Prior head or neck radiation
Williams Textbook of endocrinology 10 edition
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Screening for pituitary failure
• Prior skull base surgery
• Head trauma
• Newly discover empty sella
• Experienced pregnancy- associated
hemorrhage or blood pressure change
To prevent long term morbidity Williams Textbook of endocrinology 10 edition
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investigaton
www.Pituitarysociety.org
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investigation
www.pituitarysociety.org
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Pituitary test
• ล
Harrison’s 18th edition
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Pituitary test
Harrison’s 18th edition
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Pituitary test
Harrison’s 18th edition
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treatment
Harrison’s 18th edition
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treatment
Harrison’s 18th edition
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Acquire hypopituitary
• Pituitary apoplexy
• Cranial irridation
• Lymphocytic hypophysitis
• Empty sellar
• Sheehan syndrome (pituitary necrosis)
• ETC.
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Pituitary apoplexy
• Acute intrapituitary hemorrhage
• Occur spontaneous in preexitting adenoma ,
postpartum (sheehan syndrome)
• Associated DM , HT , sickle cell anemia , shock
, warfarin use
• Endocrine emergency
• hypopituitary is very common after apoplexy
Harrison’s 18th edition
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Pituitary apoplexy
• pituitary apoplexy occurs within pituitary
adenomas
• it may also occur in non-adenomatous or
even the normal pituitary gland
• especially during pregnancy � sheehan’s
syndrome � pituitary necrosis
Harrison’s 18th edition
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Pituitary apoplexy
• Acute symptom : severe headache , nausea vomitting , bilateral visual change (bitemporal hemianopsia) , opthalmoplegia
• Others symptoms : severe hypoglycemia hypotension , loss of consciousness , death
• Diagnosis : Pituitary CT , MRI
intratumural or sellar hemorrhage , deviated pituitary stalk and compress pituitary tissue
Harrison’s 18th edition
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Pituitary apoplexy
Acute phase MRI PITUITARY
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Pituitary apoplexy
• Management
no evidence impair consciousness or visual loss : conservative corticosteroid if adrenal insufficiency
significant visual loss , severe opthalmoplegia or loss of consciousness : urgent surgical compression
• Visual recovery � the length of time after acute event
Harrison’s 18th edition
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Cranial irridation
• Whole brain or head and neck therapeutic
irridation
• Two third of pt develop hormone deficiency
after a median dose 50 Gy (5000 rad) direct
base skull
• GH is most common. Follow by gonadotropin
and ACTH
Harrison’s 18th edition
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Lymphocytic hypophysitis
• Most common in postpartum woman.
• Suggests a selective autoimmune process target to
specific cell types.
• Hyperprolactinemia with MRI :prominence pituitary
mass like adenoma.
• Most manifestration of progressive mass effects with
headache and visual disturbance.
• Resolve after several month of glucocorticoid
treatment.
Harrison’s 18th edition
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Empty sella
• Develop insidiously.
• Pituitary mass undergo clinically silent infarct.
• CSF filling the dural herniation
• Usually normal pituitary function.
• Functional pituitary adenoma may arise within the
rim of pituitary tissue.
• Not always visible in MRI
Harrison’s 18th edition
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Sheehan syndrome
• Ischemic pituitary necrosis due to severe
postpartum hemorrhage , Vasospasm,
thrombosis and vascular compression of the
hypophyseal arteries.
• about 3% for women above 20 years of age,
almost two-thirds of whom had delivered
babies at home.
Indian J Endocrinol Metab. 2011 September;
15(Suppl3): S203–S207.
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Pathophysiology of sheehan’s
Indian J Endocrinol Metab. 2011 September;
15(Suppl3): S203–S207.
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Sheehan syndrome
• the average time between the previous
obstetric event and diagnosis of SS was 13
years.
• Lactation failure is a very common clinical
feature
• The absence of amenorrhea or the presence
of postpartum lactation, however, does not
rule out the diagnosis.
Indian J Endocrinol Metab. 2011 September;
15(Suppl3): S203–S207.
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Sheehan syndrome
• The main involvement was the secretion of
growth hormone (GH) and prolactin (90–
100%).
• deficiencies in cortisol secretion,
gonadotropin and thyroid stimulating
hormone (TSH) ranged from 50 to 100%
• Treatment : hormone therapy �
glucocorticoid , GH etc.
Indian J Endocrinol Metab. 2011 September;
15(Suppl3): S203–S207.
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Thank youThank you