Adrenal Flashcards
1
Q
Where are mineralocorticoids produced?
A
Zona glomerulosa
2
Q
Where are glucocorticoids produced?
A
Zona fasciculata
regulated by ACTH
3
Q
Where are androgens produced?
A
Zona reticularis
4
Q
Wehere are catecholamines produced?
A
Medulla
5
Q
What regulates aldosterone?
A
Renin-angiotensin system and plasma potassium
6
Q
What does angiotensin II do?
A
Causes vasoconstriction and stimulates release of aldosterone from adrenals
7
Q
Name a vitamin which is a steroid?
A
Vitamin D is a steroid
8
Q
Immunological effects of cortisol?
A
Increases capillary permeability
Increases macrophage activity
Increases inflammatory cytokine production
Increases leucocyte migration
9
Q
Metabolic effects of cortisol?
A
Increases lipolysis, central redistribution
Increases break down of carbohydrate (increases blood sugar)
Increases proteolysis
10
Q
Circulatory/renal effects of cortisol
A
Increases cardiac output
Increases blood pressure
Increases renal blood flow and GFR
11
Q
Cortisol effects on bone/connective tissue
A
Accelerates osteoporosis
Decreases serum calcium
Decreases collagen formation
Decreases wound healing
12
Q
Aldosterone and Na+?
A
Aldosterone increases reabsorption of Na+
13
Q
21 year old female ‘unwell’ for few months Weight loss Amenorrhoea Acutely unwell over past 48 hours with vomiting and diarrhoea On examination: Dark skin Dehydrated Hypotensive Decreased Na Increased K
A
Addisons
14
Q
Commonest cause of secondary adrenal insufficiency?
A
Exogenous steroid use
e.g. high dose prednisolone
inhaled corticosteroid
dexamethasone
15
Q
Aldosterone replacement?
A
Fludrocortisone
16
Q
Cortisol replacement
A
Hydrocortisone
17
Q
Might you see acne amenhorrea and hypertension in Cushing’s?
A
Yes
18
Q
Main cause of Cushing’s?
A
-Taking too much steroid (= chronic suppression of pituitary ACTH and adrenal atrophy)
Pituitary adenoma
(then ectopic ACTH e.g. in carcinoid/carcinoma)
19
Q
Types of cancer that produced ACTH?
A
Carcinoid/carcinoma
20
Q
Possible complications of long term steroid and ACTH suppression?
A
Body is unable to respond to stress (illness/surgery)
Need extra doses of steroid when ill/surgery
21
Q
Hypertension and hypokalaemia?
A
Could be primary aldosteronism
22
Q
Difference between primary and secondary hyperaldosteronism
A
Primary hyperaldosteronism = excessive levels of aldosterone independent of renin-angiotensin system
Secondary hyperaldosteronism = excessive levels of aldosteronism because of high renin levels
23
Q
Cardiovascular effects of aldosterone
A
Altered endothelial function Increase in ROS and cytokine synthesis Increased cardiac collagen Increased blood pressure Sodium retention Increased sympathetic outflow
24
Q
Commonest secondary cause of hypertension?
A
Primary aldosteronism (essential hypertension is most common primary cause)
25
Somebosy presents with hypertension, hypokalaemia and cramps (because of alkalosis) what might they have?
Hyperaldosteronism
26
Con's syndrome
Adrenal adenoma (produces aldosterone)
27
Causes of hyperaldosteronism?
```
Conns syndrome (adrenal adenoma)
Bilateral adrenal hyperplasia --> MOST COMMON CAUSE (unilateral is rare)
```
28
KCNJ5 mutations?
May lead to primary aldosteronism
| =allows more sodium into cell and depolarisation etc
29
Management of primary aldosteronism?
```
Surgical
Unilateral laparoscopic adrenalectomy
Only if adrenal adenoma (and excess confirmed in adrenal vein sampling)
Cure of hypokalaemia
Cures hypertension in 30-70% cases
Medical
In bilateral adrenal hyperplasia
Use MR antagonists (spironolactone or eplerenone)
```
30
CAH
Congenital adrenal hyperplasia
21 a hydroxylase
Autosomal recessive
31
Management of hypercalcemia?
Fluids
Frusemide
Bisphosphonates
32
Where do papillary thyroid cancers occur?
In iodine rich areas
33
Do follicular adenoma and carcinoma share the same architecture?
Yes, difficult to distinguish
| Excisional biopsy may be required
34
Which type of thyroid cancer are related with MEN?
Medullary carcinomas are related with MEN
| They originate from parathyroid follicular C cells and secrete calcitonin
35
Which type of thyroid cancers originate from parathyroid follicular C cells and secrete calcitonin?
Medullary
36
How do follicular thyroid cancers spread
Via blood
37
Can phaechromoctyoma lead to diabetes?
Yes
38
Most common cause of death in acromegalics?
Cardiovascular disease
39
Is acne common in acromegaly?
Yes
40
Cardiovascular disease seen in acromegaly?
Fibrosis, hypertrophy, carditis
| VALVE DISEASE IS NOT COMMON
41
Growth in children with Cushing's?
Growth is often arrested due to excess steroid
42
Causes of SIADH?
C, C, C
Chest: pneumonia, TB, carcinoma, abscess
Cranial causes: head injury, intracranial bleed, meningoencephalitis, space occupying lesions
CHLORPROPRAMIDE and other drugs
43
Drugs which could cause gynaecomastia?
Sprionolactone
| Methyldopa
44
Visual field loss, sweaty greasy skin, large tongue, difficulty with dentures (because of jaw or something), spade shaped hands. Who am I
Acromegaly
45
Congenital heart disease can cause growth retardation?
Pastest says yes
46
Diabetic nephropathy and death?
Responsible for 25% of diabetic deaths under the age of 30
47
Is diabetic nephropathy commonly associated with coronary artery disease?
yes
48
Drug which you should give to diabetics, can help slow down proteinuria etc
ACE INHIBITOR OMG
49
Amylase levels in diabetic ketoacidosis
Usually raised
50
Hypothyroidism and Down Syndrome?
Hypothyroidism is common in people with Down Syndrome
51
Salt wasting form of CAH
Weight loss, extreme tiredness, dehydration, vomitting
52
Virilising form of CAH
-Recognised at 2-4 years
Early virilisation
Penis enlargement
Growth of hair
53
Non-classical form of CAH
```
Less severe
Develop sexually at an early age
-Hirsutis
-Acne
-Oligomenorrhea
-Precocious puberty
-Infertility or subfertility
```
54
Diagnosis of CAH?
Basal or stimulated 17-OH-Progeseterone
| Genetic being used more
55
Sources of phaeochromocytoma
Phaechromocytoma in adrenal medulla
| rarely = sympathetic chain --> paraganglioma
56
Chromaffin cell reaction colour
Chromaffin cells reduce chrome salts to metal chromium resulting in a brown colour reaction
57
Phaechromocytoma symptoms?
```
Classic triad: sweating, hypertension, headache
Labile hypertension
Postural hypotension
Paroxsymal sweating, headache, pallor, tachycardia
(chromaffin cells reduce chrome salts to metal chromium resulting in a brown colour reaction)
-Try to diagnose Phaechromocytoma! Risks in pregnancy and other risks for everyone relating to arrythmia and ischemia --> could cause death etc
Palpitations
Breathlessness
Constipation
Anxiety/Fear
Weight loss
Hypertension
Postural hypotension in 50% cases
Pallor
Bradycardia and Tachycardia
Pyrexia
Signs of complications
Left ventricular failure
Myocardial necrosis
Stroke
Shock
Paralytic ileus of bowel
```
58
Differentials for Phaeochromocytoma
```
Angina
Anxiety
Carcinoid
Thyrotoxicosis
Insulinoma
Menopause
Arrhythmia( eg. SVT)
Migraine
Drug toxicity
Alcohol withdrawal
Pregnancy
Hypoglycaemia
Mastocytosis
Autonomic neuropathy
Factitious
```
59
Biochemical Abnormalities in Phaeochromocytoma
Hyperglycaemia – adrenaline secreting tumours
May have low potassium level
High haematocrit – i.e. raised Hb concentration
Mild hypercalcaemia
Lactic acidosis – in absence of shock
60
The 10% tumour
```
Phaeochromocytoma
10% malignant
10% extra-adrenal [probably 20-30%]
10% bilateral
10% associated with hyperglycaemia
10% in children
10% familial (but probably 25%)
```
61
Hypertension and hyperglycemia?
Consider phaeochromocytoma
62
Diagnosis of Phaeochromocytoma/catecholamine excess
-2 x 24 hour urine catecholamine/metanephrine
-Plasma: ideally at time of symptoms
Identify source of catecholamines:
-MRI
-MIBG
-PET
63
Clinical Syndrome Associations (phaechromocytoma)
```
Multiple Endocrine Neoplasia II
Von-Hippel-Lindau syndrome
Succinate dehydrogenase mutations
Neurofibromatosis
Tuberose sclerosis
```
64
MEN II
```
Autosomal dominant
Activating mutation in tyrosine kinase receptor (RET proto-oncogene)
Associations:
Medullary thyroid cancer
Parathyroid hyperplasia
Bilateral phaeochromocytomas
```
65
What is MENII associated with?
Medullary thyroid carcinoma
Bilateral phaeochromocytoma
Parathyroid carcinoma
66
Von Hippel Lindau Syndrome
Mutation in HIF 1-a
Autosomal dominant
Range of vascular tumours
Family screening vital
67
Succinate Dehydrogenase
SDH – B, C & D
Inactivating mutations – stabilise HIF-1a
50% extra-adrenal
SDH-D = head & neck PGL; > 70% penetrance
SDH-B = malignant PGL; < 50% penetrance
68
Pitfalls in Phaeochromocytoma
catecholamines raised in heart failure
episodic catecholamine secretion - levels in plasma + urine may be normal
malignant and extra-adrenal tumours less efficient at catecholamine synthesis Dopamine > Norepinephrine >Adrenaline
remember genetic syndromes
69
Permanent agranulocytosis is a serious side effects of which drugs?
Agranulocytosis - big decrease in WBC, particularly neutrophils
Carbimazole and propylthiouracil (drugs for hyperthyroidism)
70
Someone presents insidiously with diastolic hypertension...
Phaeochromocytoma
71
What is a dermoid cyst?
Contains mature material like skin, hair follicles and sweat glands
(i. e. material usually found on the outside)
- usually present in teenage years
72
Cystic hygroma
```
Posterior triangle swelling
Contains lots of watery fluid
ALMOST ALWAYS FOUND IN CHILDREN
-present from birth
-often large and disfiguring
-just below angle of mandible
-lymph fluid
-transilluminate
```
73
congenital epithelial cysts, which arise on the lateral part of the neck from a failure of obliteration of the second branchial cleft in embryonic development
Branchial cysts
74
Level of carotid bifurcation?
Level of the superior border of the thyroid cartilage
| vertebral body C4
75
What does a branchial cyst contain on fine needle aspiration?
Cholesterol crystals
76
"half filled hot water bottle"
Branchial cyst
77
Branchial cyst and cystic hygroma, anterior or posterior swellings?
Branchial cyst : anterior triangle
| Cystic hygroma: posterior swelling
78
FNA thyroid and when you should re do aspiration?
inadequate Thy 1- repeat FNA
benign Thy 2 – repeat FNA 6/12
suspicious Thy 3 – thyroid lobectomy
malignant Thy 4-5 – total thyroidectomy
79
How is ADH assessed?
Urine osmolarity
80
Low sodium but volume stats unremarkable, what should you think about?
SIADH
| -diagnosis of water excess as the cause is often done as diagnosis of exclusion
81
Talk to me about hypovolemia
Hypovolaemia implies water deficit. If it is present when [Na] is low, then there must be an even bigger sodium deficit. This is important:
hypovolaemia in hyponatraemia → too little sodium.
Hypervolaemia is most often seen as oedema. This too is useful as it gives useful information about sodium and water balanc
82
What does hypovolemia in hyponatremia mean?
Too little sodium
83
What is derived from Rathke's pouch?
Anterior pituitary
84
Acidophils
Somatotrophs
Mammotrophs
(GH and PRL)
anterior pituitary
85
Basophils
Corticotrophs
Thyrotrophys
Gonadotrophs
86
Location of mineralocorticoid receptors?
Kidneys
Salivary glands
Gut
Sweat glands
- sodium/potassium balance
- blood pressure regulation
- control of extracellular volume
87
Loosers zones
Osteomalacia
88
Adrenal crisis
```
Extreme fatigue
V v. low serum sodium, high serum K
Dehydration
Vascular collapse (because of low BP)
Renal shut down
```
89
Which autoimmune diseases are associated with Addisons?
T1Dm
Pernicious anemia
Autoimmune thyroid disorders
90
Normal results of short SynACTHen test?
Normal: cortisol = baseline >250nmol/L, post : >480
91
Hydrocortisone as cortisol replacement
If unwell, give intravenously first
Usually 15-30mg daily in divided doses
Try to mimic diurnal rhythm
92
Which kind of tumour will take up MIBG?
Phaechromocytoma
93
What might someone with a phaeochromocytoma die from?
Arrhythmia
| Ischemia
94
Carcinoid syndrome
Abdominal pain that comes and goes
Bright red flushing of the face, neck, or upper chest
Diarrhea
Heart palpitations
Low blood pressure
Skin lesions on the face called telangiectasias
Wheezing
95
Advice you should give patients if they are taking long term steroids?
- Know how to increase steroid replacement dose for intercurrent illness
- Carry a 'steroid' card
- Wear a Medic-Alert bracelet (gives details of their condition so tat emergency replacement therapy can be given if found unconscious)
96
Hirsutism developing before puberty is suggestive of what?
CAH
97
Biochemistry classical of Addisons?
Classically hyponatremia, hyperkalaemia, high urea (although biochem may be normal)
98
Severe hypotension and dehydration precipitated by intercurrent illness, accident or operation?
ADDISONIAN CRISIS
99
Most common cause of ambiguous genitalia?
CAH
