10/14- Disorders of Adrenal Glands Flashcards
What are diseases of adrenal gland (list)?
- Adrenal Insufficiency*
- Selective Hypoaldosteronism
- Cushing’s syndrome
- Hyperaldosteronism*
- Pheochromocytoma
- Adrenogenital syndromes
- Adrenocortical carcinoma
Describe anatomy of adrenal glands
- 4g
- 2 cm x 5 cm x 1 cm
- Yellow due to cholesterol content
What are the different layers of the adrenal gland?
Hormones produced there?
(Inner -> outer)
- Zona reticularis: DHEA, androstenedione, cotisol (loss)
- Zona fasciculata: cortisol
- Zona glomerulosa: aldosterone
What controls cortisol secretion/contributing factors?
CRH produced by hypothalamus
- Stressors (IL-1, 6, TNF-a, fever, surgery, burns, exercise)
- Hypoglycemia
- Hypotension
- Circadian regulation
- Negative feedback by cortisol itself
POMC/ACTH by anterior pituitary
- Triggered by CRH
Cortisol by adrenal
- Triggered by ACTH
Describe steroidogenesis
GLOMERULOSA
- Pregnenolone —(3-BHSD)–> Progesterone —(CYP21A2) –> Deoxy-corticosterone —(CYP11B1)–> Corticosterone - In the glomerulus, corticosterone is converted into aldeosterone
FASCICULATA
- Pregnenolone can also be converted into 17-OH pregnenolone
- Progesterone can be converted into 17-OH progesterone
- Then converted into deoxycortisol and eventually cortisol
RETICULARIS
- 17OH pregnenolone can be converted into DHEA
- 17OH progesterone can be converted into androstenedione
Recap: production of cortisol
What are the key hormone influences (which hormones and are levels high/low) for:
- Primary adrenal insufficiency
- Secondary adrenal insufficiency
- Tertiary adrenal insufficiancy
- Primary adrenal insufficiency: elevated ACTH
- Secondary adrenal insufficiency: ACTH deficiency
- Tertiary adrenal insufficiancy: CRH deficiency
Describe the characteristics of primary adrenal insufficiency
- Severity
- Hormone levels
- Elevated ACTH levels
- Symptoms and signs usually are more severe as patients with primary adrenal disease have a deficiency of glucocorticoids and mineralocorticoids as well as androgens
- Suddenness of onset affects severity of symptoms and signs
Describe the characteristics of secondary adrenal insufficiency
- Hormone levels
- ACTH deficiency
- Most cases of secondary and tertiary deficiency are associated with adequate secretion of aldosterone and deficient secretion of cortisol and adrenal androgens
Describe the characteristics of tertiary adrenal insufficiency
- Hormone levels
- CRH deficiency
- We do not differentiate between secondary and tertiary AI in clinical practice
What are the main/broad things causing primary adrenal insufficiency?
- Adrenal dysgenesis/hypoplasia
- Adrenal destruction
- Impaired steroidogenesis
What may cause adrenal destruction?
Autoimmune
- Sporadic PGA I and II
ALD
Infectious
- Sporadic
- Tuberculosis
- Histo, Blasto, Coccidio, Crypto
- Toxo
- Septicemia
- HIV-associated
- CMV
- MAI
- Kaposi’s
Hemorrhagic
- Anticoagulation
- Antiphospholipid syndrome
- Waterhouse-Friedrichsen/sepsis
- Heparin-induced thrombocytopenia
Metastatic
- Lung, breast, gastric, melanoma, lymphoma
Amyloidosis
What may cause adrenal dysgenesis/hypoplasia?
- SF1
- DAX1
- ACTHR
What may cause adrenal impaired sterogenesis?
Cholesterol biosynthesis:
- Smith Lemli Opitz syndrome
- Abetalipoproteinemia
Steroid biosynthesis:
- StAR protein
- Mitochondrial DNA deletion
- CAH
What are etiologies of 2’ adrenal insufficency?
Hormone characteristics?
Low ACTH and cortisol
Etiology:
- Pituitary tumor, craniopharyngioma, metastatic tumor
- Hypophysectomy
- Surgery/radiations in the pituitary hypothalamus area
- Sheehan’s syndrome (pituitary infarction)
- Infection (tuberculosis, syphilis)
What are etiologies of 3’ adrenal insufficency?
- Cessation of treatment with glucocorticoids
- Head trauma
- Sarcoidosis
- Langerhans cell histiocytosis
- Drugs that inhibit CRH secretion (morphine, fentanyl, barbiturates)
- Traumatic Brain injury (up to 30% develop AI)
Describe acute adrenal insufficiency or adrenal crisis.
What causes it?
An adrenal crisis can occur when there is a sudden change in the concentration of cortisol
Examples:
- Patient who had bilateral adrenal infarction or adrenal hemorrhage
- Patient with chronic adrenal insufficiency using hormonal replacement who has an acute infection or another acute stress, requiring higher doses of cortisol
Symptoms and signs:
- Dramatic presentation
- Fever, N/V
- Postural hypotension, shock, fatigue
- Abdominal pain
- Altered mental state
What are signs/symptoms of chronic adrenal insufficiency?
Symptoms:
- Weakness and fatigue (100%)
- N/V (80%)
- Anorexia (50%)
- Depression (20-40%)
- Psychoses (20-40%)
- Diarrhea (15%)
- Muscle, joint, abdominal pain (10%)
Signs:
- Weight loss (100%)
- Hyperpigmentation (9)%)
- Orthostatic hypoTN (90%)
- Adrenal calcification (10%)
- Vitiligo (5%)
What is seen here?
- Hyperpigmentation
- Vitiligo (primary adrenal insufficiency)
What is seen here?
Hyperpigmentation
- Seen here on aereolas
- Can also be seen on inside of lip or gums around teeth