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Pituitary Gland 3
Pituitary Gland 3
Pituitary Gland 3
College of Medicine
2023-2024
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
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
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
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
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
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
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