Here are the key points for Case Scenario 2:- Reasons for short stature: - Growth hormone deficiency - Constitutional delay of growth - Familial short stature- Best test to diagnose GH deficiency: IGF-1 levels- Three further investigations: - Insulin tolerance test - GHRH + arginine stimulation test - MRI pituitary to rule out structural abnormalitiesThe diagnosis is Growth Hormone Deficiency based on the clinical features of short stature, thin skin, decreased muscle mass and social/behavioral issues. IGF-1 levels would help to confirm the diagnosis. Dynamic stimulation tests can further establish the growth hormone deficiency. MRI pituitary is recommended to
Surgical drains, tube, catheters and central lines
Similar to Here are the key points for Case Scenario 2:- Reasons for short stature: - Growth hormone deficiency - Constitutional delay of growth - Familial short stature- Best test to diagnose GH deficiency: IGF-1 levels- Three further investigations: - Insulin tolerance test - GHRH + arginine stimulation test - MRI pituitary to rule out structural abnormalitiesThe diagnosis is Growth Hormone Deficiency based on the clinical features of short stature, thin skin, decreased muscle mass and social/behavioral issues. IGF-1 levels would help to confirm the diagnosis. Dynamic stimulation tests can further establish the growth hormone deficiency. MRI pituitary is recommended to
Similar to Here are the key points for Case Scenario 2:- Reasons for short stature: - Growth hormone deficiency - Constitutional delay of growth - Familial short stature- Best test to diagnose GH deficiency: IGF-1 levels- Three further investigations: - Insulin tolerance test - GHRH + arginine stimulation test - MRI pituitary to rule out structural abnormalitiesThe diagnosis is Growth Hormone Deficiency based on the clinical features of short stature, thin skin, decreased muscle mass and social/behavioral issues. IGF-1 levels would help to confirm the diagnosis. Dynamic stimulation tests can further establish the growth hormone deficiency. MRI pituitary is recommended to (20)
Presentació "Real-Life VR Integration for Mild Cognitive Impairment Rehabilit...
Here are the key points for Case Scenario 2:- Reasons for short stature: - Growth hormone deficiency - Constitutional delay of growth - Familial short stature- Best test to diagnose GH deficiency: IGF-1 levels- Three further investigations: - Insulin tolerance test - GHRH + arginine stimulation test - MRI pituitary to rule out structural abnormalitiesThe diagnosis is Growth Hormone Deficiency based on the clinical features of short stature, thin skin, decreased muscle mass and social/behavioral issues. IGF-1 levels would help to confirm the diagnosis. Dynamic stimulation tests can further establish the growth hormone deficiency. MRI pituitary is recommended to
2. Endocrine Disorders
• Disease states that result from excess
or insufficiency of hormone
• Clinical challenge is determination of the
origin of excess or insufficiency, i.e.,
Hypothalamus (tertiary), Pituitary (secondary)
or target gland (primary)
6. Anterior Pituitary Hormones
• TSH, ACTH, FSH, and LH hormones are
tropic hormones that simulate other
endocrine glands
• TSH-Thyroid
• ACTH- Adrenal Cortex
• FSH, LH- Gonads
7. Posterior Pituitary Hormones
• Vasopressin(ADH)- kidney, baroreceptors
(plasma osmolality, water retention, thirst)
• Oxytocin- breast, uterus (no
known function in males)
• Both are synthesized in specialized neurons
in the hypothalamus (neurohypophyseal
neurons)
8. Too big, Too tall
or
Too short
Prof. Tariq Waseem
9.
10. CASE SCENARIO 1
• A 50 yr old male presented in OPD with C/O
off & on headache, poor concentration and
fatigue. He has gained weight, he sweats a
lot. He also has difficulty in churning the food
bolus in mouth and food particles are stuck in
his unusually widened teeth spaces. His skin
has coarsened and has developed multiple
skin tags. His friends report that he has a
heavy, doughy and sweaty handshake and
often does not notice people sitting on either
sides of his desk.
16. CAUSES OF
GH-DEFICIENCY GH-EXCESS
• CONGENITAL
ABNORMALITIES
• INTRACRANIAL
TUMORS
(CRANIOPHRANGIOMA)
• IRRADIATION,
SURGERY OR TRAUMA
• SHEEHAN SYNDROME
• AUTOIMMUNE
•MICRO OR
MACROADENOMAS
•IN ASSOCIATION WITH
MEN-I
•RARELY BY ECTOPIC GHRH
17. ACROMEGALY
ACROMEGALY is a condition in adults
caused by hyper secretion of GH hormone
after the closure of epiphyseal plates.
GIGANTISM occurs if growth hormone excess
starts in in children or adolescents before
epiphyseal closure.
GIGANTISM
18. Epidemiology
• Acromegaly is more common than gigantism,
with an incidence of 3-4 cases per million
people per year and a prevalence of 40-70
cases per million population.
19. Genetics
• In gigantism inherited and manifested during
childhood or adolescence GPR101, a gene
on the X chromosome, is overexpressed.
• A mutation in the GPR101 gene was found in
about 4% of cases of Acromegaly.
• The GPR101 gene may be a target for the
treatment of growth disorders.
24. LABS
Serum Growth Hormone (Unreliable)
Raised IGF-I, IGF binding protein IGFBP-3
Hypercalcemia
Hyperphosphatemia
T4 & TSH Low (secondary hypothyroidism)
Hyperprolactinemia(mammosomatotrophs are the most
common type of GH-secreting cells involved in childhood gigantism
Hyperglycemia
GLUCOSE TOLERANCE TEST
A 75g glucose syrup is given orally and serum GH
levels are measured @ 60, 90, 120 min.
ACROMEGALY is excluded when levels are < 1ng/ml
28. TREATMENT
• 1ST Line PITUITARY MICROSURGERY
• 2ND Line RADIOSURGERY
• 2nd Line MEDICAL
Somatostatin analogue
Dopamine agonist
GH receptor antagonists
29. MICROSURGERY
• Trans sphenoidal hypophysectomy has the
dual advantage of rapidly improving
symptoms caused by mass effect of the
tumor and significantly reducing or
normalizing GH/IGF-I concentrations.
30. RADIOTHERAPY
• External radiotherapy is given in cases of
remissions after surgery.
• Gamma Knife Surgery
• Associated with risks of panhypopitutirsm.
31. MEDICAL THERAPIES
As 2nd line therapy after surgery to reduce GH
levels < 5mU/l
DOPAMINE AGONISTS
CABERGOLINE effective in tumors secreting
both prolactin & GH.
SOMATOSTATIN ANALOGUE
OCREOTIDE & lanreotide given as s/c
injections.
GH RECEPTOR ANTAGONISTS
PEGVISOMANT is used primarily for
symptomatic relief.
32. Treatment
• Cure, or adequate control, of growth
hormone (GH) excess is defined as a
glucose-suppressed GH concentration of
less than 2 ng/mL, as determined by
radioimmunoassay (1 mcg/L by IRMA), and
normalization of the serum insulinlike growth
factor I (IGF-I) concentration.
33. Summary
Acromegaly is a rare, insidious, and potentially
life-threatening condition for which there is
good, albeit incomplete, treatment that can add
years of high-quality life for the patient.
Increased and unregulated growth hormone
(GH) production, usually caused by a GH-
secreting pituitary tumor (somatotroph tumor),
characterizes acromegaly.
34.
35. • What is the diagnosis?
• What is the best test to diagnose this
condition?
• List three further investigations to confirm the
diagnosis.
37. CASE SCENARIO 2
• A mother brings her 15 yr old son to the
OPD complaining he has not gained height
compared with his siblings and classmates.
He is depressed and feels socially isolated.
He is 3.5 ft tall with thin skin and wrinkles,
decreased body tone. His mother complains
of his unsocial behavior at home and lack of
interest in studies.
• What can be reasons of his short stature?
38. DIFFERENTIALS
• Familial short stature
• Constitutional delay
• GH deficiency
• Achondroplasia
• Vitamin D deficiency
• Sex hormone deficiency
40. SIGNS & SYMPTOMS OF GH-
DEFICIENCY
•Retarded growth
•Central obesity
•Psychogenic symptoms
•Decreased bone and muscle mass
•Thin skin with fine wrinkles
•Poor sweating or temperature regulation
•Decreased energy and endurance
•Low energy levels
•Increased cholesterol and LDL
•Increased systolic blood pressure
•Decreased cardiac output
•Overproduction of insulin
41. Lab tests for GH Deficiency
• LOW IGF-I LEVELS
• INSULIN TOLERANCE TEST
• GHRH + ARGININE STIMULATION TEST
AVOID IN PTS WITH SEIZURES & CAD
AVOID IN PTS WITH KIDNEY &LIVER DISEASE
42. LARON SYNDROME
• Autosomal recessive disorder due to
mutations in the gene for GH receptor.
• Resistance to GH resulting in IGF-I
DEFICIENCY.
• Show resistance to DIABETES.
•Dwarfism
•Depressed nasal bridge
•Underdeveloped mandible
•Central obesity
•Hypoglycemic seizures
BIOSYNTHETIC IGF-I
BEFORE PUBERTY