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Adrenal and Pitutary Disoreders Adigrat Lec
Adrenal and Pitutary Disoreders Adigrat Lec
Adrenal and Pitutary Disoreders Adigrat Lec
Introduction
Anatomy
Regulation
Metabolism
Cushing syndrome
Mineralocorticoid excess
Adrenal insufficiency
Pheochromocytoma
The adrenal cortex produces three classes of corticosteroid
hormones:
These include fragility of the skin, with easy bruising and broad
(>1 cm), purplish striae, and signs of proximal myopathy
In ectopic ACTH syndrome, hyperpigmentation of the knuckles,
scars, or skin areas exposed to increased friction can be
observed
ADRENAL ADENOMA
Unilateral laparascopic adrenalectomy
ADRENAL CARCINOMA
Poor PX (most die in 2 yrs); tumor removal, Mitotane
CUSHING’S DISEASE
Previously: bilat adrenalectomy(MR 4% & Nelson
syndrome), pitutary irradiation (Now used for failures)
Currently: transphenoidal hypophysectomy (cure 80-90%
micro.)
D, Similar changes in
a 60-year-old man
with tuberculous
Addison's disease
before (left) and after
(right) corticosteroid
therapy.
E, Buccal pigmentation
The C/Fs relate to the rate of onset and the severity of adrenal
deficiency.
Minealocorticoid replacement
Androgen replacement
Fatigue Hyperkinesis
Tremors Tremor
Hyperdefication Tachycardia/arrhythmia
Infection Trauma
DKA MI
CVA PE
Symptoms Signs
Fatigue Hoarseness
Weight Gain Hypothermia
Cold intolerance Periobital puffiness
Depression Delayed relaxation of ankle
jerks
Menstrual irregularities Loss of outer third of eyebrow
Constipation Cool, rough, dry skin
Joint Pain Nonpitting edema
Muscle cramps Bracycardia
Infertility Peripheral Neuropathy
Treatment
Most patient with uncomplicated symptomatic Hypothyroidism may
be referred to the primary physician for further evaluation and
initiation of treatment
If hypothyroidism is due to a secondary etiology initiation of thyroid
hormone therapy may exacerbate preexisting adrenal insufficiency
Myxedema is a rare life threatening decompensation of
hypothyroidism
Usually in individuals with long-standing hypothyroidism
Most often seen in the winter months
More common in elderly women with underdiagnosed or
undertreated hypothyroidism
Precipitating events include
Infection
CHF
Trauma
CVA
Exposure to cold
Drugs
Sedatives
Lithium
Amiodarone
In addition to the clinical features of hypothyroidism
patients may present with
Hypothermia
Altered mental status
Coma, delusions, and psychosis (myxedema maddness)
Hyponatremia
Dilutional secondary to decreased free-water clearance
Hypoglycemia
Secondary to impaired gluconeogenesis
Hypotension
Bradycardia
Respiratory Failure
Secondary to decreased strength of respiratory muscle
Hypercapnia and hypoxia is common
Diagnosis
Must have high clinical suspicion
Commonly has Hx. Of hypothyroidism
Delcine in function is usually insidious in onset
Diagnosis cont
Laboratory evaluation may reveal
Anemia
Hyponatremia
Hypoglycemia
↑ Transaminases
↑ CPK
↑ LDH
↓Po2 and ↑PCo2 on ABG’s
Diagnosis cont.
EKG may reveal
Sinus Bradycardia
Prolonged QT interval
Low voltage
Flattened or inverted T waves
Treatment
Airway stabilization with adequate oxygenation and
ventilation or vital
Cardiovascular status must be monitored closely
Hypothermic patients should be gradually
rewarmed with gentle passive external rewarming
Hypotension from reversal of hypothermic
vasoconstriction should be avoided
Treatment cont.
Hyponatremia typically responds to fluid restrictions. Severe cases
may require hypertonic saline with lasixs
Vasopressors are usually ineffective and should only be used in
severe hypotension
Lovothyroxine 300-500 mcg slow IVP followed by 50-100 mcg daily
Treatment cont.
L-triiodothyronine 25 mcg IV or orally q 8 h is a
alternative
This dose should be halved in patients with cardiovascular
disease
Hydrocortisone 100 mg IV q 8 hours should be given
Send baseline cortisol level to lab if possible
Precipitating causes should be sought and treated
Recognition
Supportive therapy including ventilatory support
Thyroid replacement
Lovothyroxine 300-500 mcg slow IVP followed by 50-100 mcg daily or
T3 25 mcg IV or PO q 8 hrs
Glucocorticoid
Hydrocortisone: 100 mg IV q8h
Hypothermia
Prevent additional loss
Passive external rewarming
Electrolyte correction
Gentle fluid restriction for dilutional hyponatremia
Hypertonic saline for severe hyponatremia
Hypoglycemia
Dextrose-containing IV fluids
Monitoring
2.Neuromuscular – weakness
- myalgia
3.Neurologic and psychiatric
- Memory loss - Confusion
- Depression - Lethargy
- Psychosis - Fatigue
- Neurosis - paresthesias
4.Skeletal
- Bone pain
- Osteoporosis
- Subperiosteal skeletal resorption
5.GI
- Abdominal pain
- Nausea
- Peptic ulcer
- Constipation
- Pancreatitis
6.Hypertension
7.Arthralgia, synovitis, arthritis
8.Band keratopathy
9.Anemia
Classic phrase of these features is “stones, bones, abdominal
groans and psychic moans”
Hypercalcemia is universal
Serum phosphorus is low normal (<3.5
mg/dl) or low (<2.5 mg/dl)
Mild hyperchloremic metabolic acidosis
PTH is elevated or high normal
Modern assays of intact PTH use
immunoradiometric assay (IRMA) or
immunochemiluminescent assay (ICMA)
Alkaline phosphatase may be increased
Definitive treatment is surgical parathyroidism
Cure rate for adenomais >95% (less for
hyperplasia)
No value in pre-op studies to localize
parathyroid tumors
The only localization study needed is to locate
an experienced parathyroid surgeon
No definitive medical therapy for
hyperparathyroidism
In the asymptomatic patient, who should undergo surgery?
Serum calcium of 1 mg/dl or more above upper limit of normal
Hypercalciuria
Creatine clearance 30% or less than age matched normal
subjects
Osteoporosis
Age < 50 years
Difficult follow up
Excessive secretion of PTH in response to hypocalcemia,
hyperphosphatemia, ↓ calcitriol eg. Renal failure,
malabsorption, vitamin D deficiency
Hypercalcemia and parathyroid overactivity in the presence of
a condition that would be expected to cause secondary
hyperparathyroidism
PTH Calcium
Primary N↑ ↑
Secondary ↑ ↓N
Tertiary ↑↑ ↑
Protein with 141 amino acid
Produced by malignant cells
Interacts with the PTH receptor and mimics action of PTH
b.Idiopathic hypoparathyroidism
Age of onset is 2-10 years
Female preponderance
Circulating parathyroid antibodies common
c.Autoimmune hypoparathyroidism
Hypoparathyroidism is a prominent
component of autoimmune polyglandular
syndrome
Associated with primary adrenal
insufficiency, mucocutaneous candidiasis
Age of onset 5-9 years
d.Familial hypoparathyroidism
Autosomal dominant
Mutation in PTH gene leads to defective PTH
e.DiGeorge’s syndrome
f.Congenital aplasia of the parathyroids
g.Iron deposition in the glands
h.Copper deposition
i.Aluminium deposition
j.Infiltration with metastatic carcinoma
k.Magnesium depletion
Ca PO4 PTH 25-Vit D Calcitriol
Hypoparathyr ↓ ↑ ↓ N ↓
oidism
Pseudohypopr ↓ ↑ ↑ N ↓N
arthyroidism
Liver disease ↓ ↑ ↓ ↓N
Renal disease ↓ ↑ ↑ N ↓N
Main treatments available are calcium salts, vitamin D
Acute hypocalcemia
- IV Calcium
- Oral or IV Calcitriol
Chronic hypocalcemia
- Oral calcium
- Calcitriol or vitamin D
Increased GH
Hyper secretion causes Gigantism in children, Acromegaly in
adults
Causes
Pituitary adenomas most common
Rarely Carcinoid tumors and pancreatic islet tumors make GHRH
CLINICAL FEATURE OF ACROMEGALY
11/25/2018 132
Screening- serum IGF-I levels
Due to the pulsatility of GH secretion, measurement of a single
random GH level is not useful for the diagnosis or exclusion
PRL elevated in ~25%
Thyroid function, gonadotropins, and sex steroids may be
attenuated because of tumor mass effects.
Definitive diagnosis: increase in GH with oral glucose
tolerance test
(failure of GH suppression to <1 g/L within 1–2 h of an oral
glucose load 75 g)
Treatment: surgery, radiation, Bromocriptine, Octreotide
PROLACTIN (PRL)
Pituitary Adenoma
Related to size and location
Visual field defects, Oculomotor palsies, Increased ICP
Skull radiograph: “double floor” (large Sella or erosion), calcification
CT and MRI far more sensitive for diagnosis
Related to destruction of gland
• Hypopituitarism
Related to increased hormone secretion
Prolactinoma is most common pituitary tumor
- Galactorrhea
Acromegaly in adults, Gigantism in children
Cushing’s disease = Cushing’s syndrome caused by a pituitary tumor
Tumors secreting LH, FSH and TSH are rare
•
Craniopharyngioma
From Rathke’s pouch
Usually mass lesion with compressive effect
Pituitary Apoplexy
Acute hemorrhage/infarction of pituitary
Note: Ophthalmoplegia with pituitary tumor likely
indicates apoplexy since tumor rarely gets big enough to
encroach on cranial nerves
Etiology
❏ Mnemonic: eight “I”s
Invasive: generally primary tumors
Infarction: e.g. Sheehan’s syndrome
Infiltrative disease e.g. Sarcoidosis, Hemochromatosis,
Histiocytosis
Iatrogenic: following surgery or radiation
Infectious: e.g. syphilis, TB
Injury: severe head trauma
Immunologic: autoimmune destruction
Idiopathic: familial forms, congenital midline defects
Typical clinical progression in panhypopituitarism
laboratory
low levels of trophic hormones in the setting of low target hormone
levels.
Diagnosis by Triple Bolus Test
❏ Rapid sequence IV infusion of Insulin, LHRH and TRH
Insulin ––> hypoglycemia ––> increased GH and ACTH
LHRH ––> increased LH and FSH
TRH ––> increased TSH and PRL
HYPOPITUITARISM: TREATMENT