Pituitary Gland 3

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University of Baghdad

College of Medicine
2023-2024

Title: The pituitary gland/ 3rd lecture

Grade: 4th year


Module: Endocrinology
Speaker: Dr. Omar Mohammed Al-Juboori
Date: 27/3/2024
Objectives University of Baghdad/ College of Medicine 2022-2023

 Define Craniopharyngioma & clarify it's clinical features & treatment


 Define Diabetes insipidus & clarify its types & Management.
 Define short stature & clarify its Management.
 Define Sheehan's syndrome.
University of Baghdad/ College of Medicine 2022-2023

Some pituitary pathologies


University of Baghdad/ College of Medicine 2022-2023

Craniopharyngioma
Craniopharyngiomas are benign tumours that develop in cell rests of
Rathke’s pouch, and may be located within the sella turcica, or
commonly in the suprasellar space. They are often cystic, with a solid
component that may or may not be calcified.
In young people, they are diagnosed more commonly than pituitary
adenomas.
Note: Rathke's pouch is a diverticulum arising from the embryonic buccal
cavity, from which the anterior pituitary gland develops.
University of Baghdad/ College of Medicine 2022-2023
University of Baghdad/ College of Medicine 2022-2023

Clinical presentation
They may present with:
 pressure effects on adjacent structures.
 hypopituitarism.
cranial diabetes insipidus.
Other clinical features directly related to hypothalamic damage may also
occur. These include :
ohyperphagia and obesity,
oloss of the sensation of thirst and disturbance of temperature regulation, and
these features can be significant clinical challenges to manage.
University of Baghdad/ College of Medicine 2022-2023

Treatment
Craniopharyngiomas can be treated by the trans-sphenoidal route but
surgery may also involve a craniotomy, with a relatively high risk of
hypothalamic damage and other complications.
If the tumour has a large cystic component, it may be safer to place
in the cyst cavity a drain that is attached to a subcutaneous access
device, rather than attempt a resection.
University of Baghdad/ College of Medicine 2022-2023

Whatever form it takes, surgery is unlikely to be curative and


radiotherapy may often be given to reduce the risk of relapse.
Unfortunately, craniopharyngiomas often recur, requiring repeated
surgery.
They often cause considerable morbidity, usually from hypothalamic
obesity, water balance problems and/or visual failure.
University of Baghdad/ College of Medicine 2022-2023

Diabetes insipidus
This uncommon disorder is characterized by the persistent excretion
of excessive quantities of dilute urine and by thirst (polyuria and
polydipsia).
 It is classified into two types:
• cranial diabetes insipidus, in which there is deficient production of
vasopressin by the hypothalamus
• nephrogenic diabetes insipidus, in which the renal tubules are
unresponsive to vasopressin.
University of Baghdad/ College of Medicine 2022-2023

Causes of diabetes insipidus / Cranial

Structural hypothalamic or high stalk lesion

Idiopathic

Genetic defect
• Dominant (AVP gene mutation)
• Recessive (DIDMOAD syndrome – association of diabetes insipidus
with diabetes mellitus, optic atrophy, deafness)
University of Baghdad/ College of Medicine 2022-2023

Nephrogenic

Genetic defect
• V2 receptor mutation
• Aquaporin-2 mutation
• Cystinosis
Metabolic abnormality
• Hypokalemia
• Hypercalcemia
Drug therapy
• Lithium
• Demeclocycline
Poisoning
• Heavy metals
Chronic kidney disease
• Polycystic kidney disease
• Sickle-cell anemia
• Infiltrative disease
University of Baghdad/ College of Medicine 2022-2023

Clinical features
The most marked symptoms are polyuria and polydipsia. The patient
may pass 5–20 L or more of urine in 24 hours. This is of low specific
gravity and osmolality.
 If the patient has an intact thirst mechanism, is conscious and has
access to oral fluids, then he or she can maintain adequate fluid intake.
However, in an unconscious patient or a patient with damage to the
hypothalamic thirst center, diabetes insipidus is potentially lethal.
University of Baghdad/ College of Medicine 2022-2023

 If there is associated cortisol deficiency, then diabetes insipidus may


not be manifest until glucocorticoid replacement therapy is given.
The most common differential diagnosis is primary polydipsia,
caused by drinking excessive amounts of fluid in the absence of a
defect in vasopressin or thirst control.
University of Baghdad/ College of Medicine 2022-2023

Investigations
Polyuria should be confirmed by asking the patient to measure a 24h
urine output (normal <3L).
Exclusion of other causes of polyuria and polydipsia like DM.
A corrected calcium and potassium should be measured.
University of Baghdad/ College of Medicine 2022-2023

Diabetes insipidus can be confirmed if serum vasopressin is


undetectable or the urine is not maximally concentrated (i.e. < 600
mmol/kg) in the presence of increased plasma osmolality (i.e. > 300
mOsmol/kg).
Sometimes, the diagnosis can be confirmed or refuted by random
simultaneous samples of blood and urine, but more often a dynamic
test is required.
University of Baghdad/ College of Medicine 2022-2023

Diagnosis
water deprivation test is widely used.
Use
• To establish a diagnosis of diabetes insipidus and to differentiate
cranial from nephrogenic causes
Protocol
• No coffee, tea or smoking on the test day
• Free fluids until 0730 hrs on the morning of the test, but discourage patients from
‘stocking up’ with extra fluid in anticipation of fluid deprivation
• No fluids from 0730 hrs
• Attend at 0830 hrs for measurement of body weight and plasma and urine
osmolality
• Record body weight, urine volume, urine and plasma osmolality and thirst score
on a visual analogue scale every 2 hrs for up to 8 hr
• Stop the test if the patient loses 3% of body weight
• If plasma osmolality reaches > 300 mOsmol/kg and urine osmolality < 600
mOsmol/kg, then administer DDAVP 2 μg IM.
University of Baghdad/ College of Medicine 2022-2023

Interpretation

• Diabetes insipidus is confirmed by a plasma osmolality > 300 mOsmol/kg with a


urine osmolality < 600 mOsmol/kg
• Cranial diabetes insipidus is confirmed if urine osmolality rises by at least 50%
after DDAVP
• Nephrogenic diabetes insipidus is confirmed if DDAVP does not concentrate the
urine
• Primary polydipsia is suggested by low plasma osmolality at the start of the test
University of Baghdad/ College of Medicine 2022-2023

an alternative to water deprivation test is to infuse hypertonic (5%)


saline and measure vasopressin secretion in response to increasing
plasma osmolality. Thirst can also be assessed during these tests on a
visual analogue scale.
Anterior pituitary function and suprasellar anatomy should be
assessed in patients with cranial diabetes insipidus.
University of Baghdad/ College of Medicine 2022-2023
Management

Treatment of cranial diabetes insipidus is with des-amino-


desaspartate-arginine vasopressin (desmopressin, DDAVP), an
analogue of vasopressin that has a longer half-life.
For chronic replacement therapy DDAVP may be administered
intranasally and orally, although the latter formulation has variable
bioavailability.
University of Baghdad/ College of Medicine 2022-2023

The dose of DDAVP should be adjusted on the basis of serum


sodium concentrations and/or osmolality. The principal hazard is
excessive treatment, resulting in water intoxication and
hyponatraemia.
University of Baghdad/ College of Medicine 2022-2023

Short stature
Short stature is defined as height more than 2.5 standard deviations
below the mean for age-matched normal children, growth velocity less
than the 25th percentile, delayed bone age.
Childhood-onset GH deficiency is most commonly idiopathic, but it
may be genetic or associated with congenital anatomic malformations
in the brain or sellar region.
Children with GH deficiency exhibit growth retardation, short
stature, and fasting hypoglycemia.
University of Baghdad/ College of Medicine 2022-2023

Diagnosis
Combined clinical evaluations, along with an inadequate pituitary GH
response to provocative testing, are used in the assessment of childhood GH
deficiency.
In children, there is greater variation in IGF-I levels that do not correspond
to the true GH status, so GH stimulating testing is required.
The historical “gold standard” stimulatory test is insulin-induced
hypoglycemia, others include glucagon & clonidine GH stimulation test.
the normal GH response is greater than 10 ng/mL in children
University of Baghdad/ College of Medicine 2022-2023

Treatment
recombinant human growth hormone (hGH), It is administered as a
daily subcutaneous injection with dose increases at 6-week intervals
based on clinical response, side effects, and IGF-I levels.
University of Baghdad/ College of Medicine 2022-2023

Sheehan’s syndrome
This is a form of post-partum hypopituitarism caused by infarction of
the pituitary, usually associated with hypotension from major post-
partum hemorrhage.
It can present with failure to establish lactation after birth,
amenorrhoea or other features of hypopituitarism. The diagnosis can
be confirmed by tests of pituitary function and treated with hormone
replacement.
University of Baghdad/ College of Medicine 2022-2023

thanks

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