Endocrine Flashcards

1
Q

MEN 1

A

Wermer’s Syndrome

Deletion 11q12-13

3 Ps

Parathyroid hyperplasia (90%)
Pancreatic (and duodenal) islet cell tumors (50%)
Pituitary adenomas (25%) - prolactinoma = #1
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2
Q

MEN 2A

A

Sipple’s Syndrome

RET oncogene mutation on chrom 10q11.2

Missense mutations on chrom 1

Medullary thyroid carcinoma (100%) - 20% of all medullary cancers are from MEN

Pheo (33%) - usually b/l

Parathyroid hyperplasia (50%)

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3
Q

MEN 2B

A

Mucosal neuroma may be earliest sign (100%) - hypertrophied lips, thickened eyelids

Medullary thyroid carcinoma (85%)
Pheo (50%)
Marfanoid habitus - skeletal abnormalities of spine (kyphosis), pectus excavatum

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4
Q

Tx for MEN syndromes

A

Perform subtotal or total parathyrodectomy with autotransplantation for parathyroid hyperplasia (MEN 1 and MEN 2A)

Perform total thyroidectomy for medullary thyroid cancer (MEN 2). May require nodal dissection if palpable nodes present

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5
Q

Thyroid development

A

Thyroid develops at base of tongue btw first pair of pharyngeal pouches, in area called foramen cecum

Thyroid gland descends down midline to its final location and develops bilobed organ with isthmus btw lobes

Remains connected to floor of pharynx via thyroglossal duct, which obliterates around month 2. May fail to go away and form a cyst or fistula instead. Usually in kids and should be removed with surg

A pyramidal lobe can be seen in 50-80% of population and represents remnant of distal thyroglossal tract. It extends superiorly from isthmus

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6
Q

Thyroid anatomical relationships

A

Anterior = strap muscles (sternohyoid, sternothyroid, thyrohyoid, omohyoid)

Posterior = trachea

Posterolateral = common carotids, IJ veins, vagus nerves

Parathyroid glands on posterior surface of thyroid and may be IN the capsule

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7
Q

Lymphatic drainage of thyroid

A

To IJ nodes

Intraglandular lymphatics connect both lobes, explaining the high frequency of multifocal tumors in thyroid

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8
Q

Vasculature of thyroid

A

Arteries:

1) Superior thyroid (each side)
- first branch of external carotid at level of carotid bifurc

2) Inferior thyroid (each side)
- from thyrocervical trunk of subclavian artery

3) Ima (sometimes)
- from aortic arch or innominate artery

Venous:

1) Superior thyroid vein (each side)
- drains into IJ

2) Middle thyroid vein (each side)
- Drains into IJ

3) Inferior thyroid vein (each side)
- drains to brachiocephalic vein

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9
Q

Innervation of thyroid

A

***1) R recurrent laryngeal nerve branches from R vagus nerve under R subclavian artery, and ascends (posterior to thyroid) to the larynx btw the trachea and esophagus

L recurrent larygneal nerve branches from L vagus, loops under aortic arch and ascends along tracheoesophageal groove to larynx.

Both innervate muscles of the true vocal cords

2) Sympathetic - superior and middle cervical sympathetic ganglia
3) Parasympathetic - from vagus nerves via branches of laryngeal nerves

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10
Q

Clinical importance of recurrent laryngeal nerve

A

It innervates all the intrinsic muscles of larynx except the cricothyroid (superior laryngeal nerve) and provides sensory innervation to mucous membranes below the vocal cords

It can be damaged during thyroid surgery so surgeon needs to know its course.

Damage produces ipsilateral vocal cord paralysis and results in hoarseness or sometimes SOB due to narrowed airway

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11
Q

TSH function

A

Causes increased formation of TH

Release of TH into circ within 30mins

Increased TH level in blood then feeds back to pit and causes decreased TSH secretion

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12
Q

Assessment and function of thyroid hormones

A

If T4 production goes up, both total T4 and free T4 go up

If T4 production drops, total and free T4 drop

If amount of thyroid-binding globulin changes, only the free T4 changes (not the total)

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13
Q

How much hormone do thyroid follicles store?

A

enough to last 2-3 months

Thus, there is no need to worry about postop hypothyroid patient who is NPO…they can resume taking their synthroid when they begin PO diet

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14
Q

Congenital anomalies of thyroid

A

Persistent sinus tract remnant of developing gland = thyroglossal cyst - may occur anywhere along course as a midline structure with thyroid epithelium, usually between isthmus and hyoid bone

  • # 1 congenital anomaly
  • few symptoms but may be infected
  • Easier to see when tongue is sticking out
  • surgical treatment - excise the duct remnat and central part of hyoid bone (Sistrunk’s operation)

Complete failure to develop

Incomplete descent = lingual thyroid or subhyoid position (if gland enlarges, patient will have earlier respiratory symptoms)
- before surgery to remove it make sure patient has other functioning thyroid tissue (70% of lingual thyroids are the only functioning thyroid)

Excessive descent = substernal thyroid

Malformation of branchial pouch

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15
Q

Causes of hyperthyroidism

A

1) Grave’s
2) Toxic nodular goiter
3) Toxic thyroid adenoma
4) Functional metastatic thyroid cancer
5) Struma ovarii (abnormal thyroid tissue in ovary)

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16
Q

Grave’s disease def

A

1 cause of hyperthyroidism in USA

Autoimmune that causes excess of TH to be produced due to presence of thyroid-stimulating immunoglobulins that stimulate production of TSH

2% of american women; 6x more common in women

Onset 20-40

Families with Graves have higher risk of other AI conditions (diabetes, Addison’s) and other thyroid disorders too

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17
Q

Signs of Graves

A

Nervousness, increased sweating, tachy, goiter, pretibial myxedema, tremor (90%)

Heat intolerance, palpitation, AFib refractory to treatment, weight loss, fatigue, dyspnea, weakness, increased appetite, exophthalmos, thyroid bruit (50-90%)

Amenorrhea, low libidio and fertility

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18
Q

Dx of Graves

A

TFTs - high T3 and/or T4 and low TSH (neg feedback of high hormone levels)

Radioactive iodide uptake test (RAIU) - scan shows diffusely increased uptake

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19
Q

Tx of Graves

A

Antithyroid drugs

Radioiodide ablation with I-131

Subtotal or total thyroidectomy

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20
Q

Choosing a Tx for Graves

A

1) Consider age, severity, size, surg risk, treatment side effects and comorbidities

2) Radioablation is #1 choice
- indicated for small or medium goiters if med therapy has failed, or if other options are contraindicated
- Most patients become euthyroid within 2 months
- Most ultimately require thyroid hormone replacement (Levo)
- Complications include exacerbation of thyroid storm initially
- Contraindicated in pregnancy, women of childbearing age and newborns

3) Surgery is indicated when radioablation is contraindicated or if medical management cannot be used
- Patients should be euthyroid prior to excision
- advantage over radioablation is immediate cure

4) Medical therapy
- B blockers for symptomatic relief
- Antithyroid drugs (PTU, methimazole) inhibit hormone production and peripheral conversion of T4 to T3
- KI reduces hormone production, used to shrink gland prior to surgery
- High recurrence rate with medical tx
- may cause side effects like rash, fever, peripheral neuritis
- Patients relapse if meds are D/C’d
- Check TFTs after any treatment

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21
Q

Risks of thyroid surgery

A

Recurrent laryngeal nerve injury

Hypoparathyroidism

Persistent hyperthyroidism (with subtotal thyroidectomy)

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22
Q

Toxic nodular goiter

A

“Plummer’s Disease”

Causes hyperthyroidism but without the extrathyroidal symptoms

Treatment is surgical since medical therapy and radioablation has a high failure rate

Solitary nodule = lobectomy

Multinodular goiter = subtotal thyroidectomy

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23
Q

Thyroid storm (thyrotoxicosis)

A

Life-threatening extreme exacerbation of hyperthyroidism precipitated by surgery on an inadequately prepared patient (incomplete B blockade and noneuthyroid patient), infections, labor, iodide administration or recent radioablation

Fever, tachy, muscle stiffness, disorientation/AMS

50% with thyroid storm develop CHF

20-40% mortality

Best way to treat is by avoiding it. ppx = achieving euthyroid state preop

Tx = fluids, antithyroid meds, B-block, corticosteroids, sodium iodide or Lugol’s solution (KI) and a cooling blanket

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24
Q

Causes of hypothyroidism

A

Autoimmune thyroiditis

Iatrogenic: s/p thyroidectomy, s/p radioablation, 2/2 antithyroid meds

Iodine deficiency

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25
Signs of hypothyroidism
Infants/peds: down's like facies, failure to thrive, mental retardation ***immediate tx with thyroid hormone will minimize neuro and intellectual effects*** Adolescents/Adults: (particularly when due to AI) - 80% female - brady, low CO, hypotension, SOB 2/2 effusions - fatigue, weight gain, cold intolerance, constipation, menorrhagia, low libido and fertility Less common = yellowish skin, hair loss, tongue enlargement
26
Dx of Tx of hypothyroidism
H&P Low T4, T3 High TSH if primary Low TSH if secondary Confirm with TRH challenge - TSH will not respond in secondary hypo Thyroid autoantibodies in AI Low Hct ECG may show low voltage or flat/inverted T waves Tx = thyroxine PO or IV emergently if patient presents in myxedema coma
27
Thyroiditis (Acute)
Infectious etiology = strep pyogenes, staph aureus, pneumococcus (usually via lymphatics from local infection) Risk = female sex, goiter, thyroglossal duct Signs = unilateral neck pain and fever, euthyroid state, dysphagia Tx = IV ABx and surgical drainage
28
Thyroiditis ( Subacute/de Quervain's)
Post viral- URI Risk = female Signs = fatigue, depression, neck pain, fever, unilateral swelling of thyroid with overlying erythema, firm, and tender thyroid, transient hyperthyroidism usually preceding hypothyroid phase Dx = made by H&P Tx = usually self-limiting (within 6w) = manage pain with NSAIDs 10% become permanently hypothyroid
29
Thyroiditis (Chronic/Hashimoto)
Autoimmune Risk = down's syndrome, Turner, familial Alzheimer's, hx of radiation therapy as child Signs = painless enlargement of thyroid, neck tightness, presence of other AI diseases Dx = H&P + labs - Circulating antibodies against microsomal thyroid cell, thyroid hormone, T3, T4, or TSH receptor Path = firm, symmetrical enlargement; follicular and Hurthle cell hyperplasia; lymphocytic and plasma cell infiltrates Tx = thyroid hormone (usually results in regression of goiter). With failure of medical tx, partial thyroidectomy is indicated 20% present with hypothyroidism at time of dx. A euthyroid state is more common
30
Riedel's Fibrosing thyroiditis
Rare Fibrosis replaces both lobes and isthmus Risk = other fibrosing conditions like retroperitoneal fibrosis or sclerosing cholangitis Signs = usually remain euthyroid; neck pain, possible airway compromise; firm, nontender enlarged thyroid Dx = often bx required to rule out carcinoma or lymphoma Path = dense, invasive fibrosis of both lobes and isthmus. May also involve adjacent structures Tx = with airway compromise: Isthmectomy Without: medical treatment with steroids
31
Workup of thyroid nodule
1) Get TSH/T4 - Low TSH, High T4? Hyperthyroid - Normal? Euthyroid 2) If hyperthyroid: - it's prob a functioning adenoma and NOT cancer - Get RAIU - Hot = functioning adenoma (I2 ablation, surgery, medical management) - Cold = ? (Get US guided FNA) 3) If euthyroid: - Get US guided FNA 4) the FNA - Benign - ? (get RAIU, if functioning then benign. not not then cancer) - Cancer (surgery and I2 ablation) - Get a staging workup if cancer
32
Most common thyroid cancer
Papillary (80-85%) - it's 75% of peds thyroid cancer
33
Risk factors for the dif thyroid cancers
Papillary = radiation Follicular = dyshormonogenesis Medullary = MEN II in 30-40% Anaplastic = Prior diagnosis of well-differentiated thyroid cancer. Iodine deficiency
34
Age groups for each thyroid cancer
Papillary = 30-40 Follicular = 5-20 Medullary = 50-60 Anaplastic = 60-70
35
Sex ratios for each thyroid cancer (F/M)
papillary = 2/1 Follicular = 3/1 Medullary = 1.5/1 Anaplastic = 1.5/1
36
Signs of papillary thyroid cancer
Painless mass Dysphagia Dyspnea Hoarseness Euthyroid
37
Signs of follicular thyroid cancer
Painless mass Rarely hyperfunctional
38
Signs of medullary thyroid cancer
Painful mass Palpable LN (15-20%) Dysphonia Dyspnea Hoarseness
39
Signs of Anaplastic thyroid cancer
Rapidly enlarging neck mass (large mass at presentation) Neck pain Dysphagia Hard, fixed LN (50%)
40
Diagnosis of each thyroid cancer
Papillary = FNA, CT/MRI to assess local invasion Follicular = FNA, CT/MRI to assess local invasion Medullary = FNA, presence of amyloid is diagnostic. Check for calcitonin Anaplastic = FNA
41
Mets for each thyroid cancer
Papillary = lymphatic (5% at time of prez) Follicular = hematogenous Medullary = lymphatic (local neck and mediastinal nodes). Local (into trachea and esophagus) Anaplastic = Aggressive local disease. 30-50% have synchronous pulmonary mets at time of dx
42
Tx of papillary thyroid
1) Minimal cancer ( 1.5cm 3) For + LN, modified radical neck dissection 4) I-131 ablation or thyroid suppression (with thyroid hormone) for patients with residual thyroid tissue or LN mets
43
Tx for follicular thyroid
1) Minimal cancer ( 4cm 3) For +LN, modified radical neck dissection 4) I-131 ablation for patients with residual thyroid tissue or LN mets
44
Tx for medullary thyroid
1) Sporadic (80%): total thyroidectomy 2) Familial (20%): Total thyroidectomy and central neck node dissection 3) No value for I-131 ablation 4) Follow patients with calcitonin levels
45
Tx for anaplastic thyroid
1) Debulking resection of thyroid gland and adjacent structures 2) XRT 3) Doxorubicin-based chemo
46
Prognosis of papillary thyroid
worse for older patients and those with distant mets Presence of +LN not strongly correlated with overall survival 10yr = 74-93%
47
Prognosis of follicular thyroid
Worse for older patients, distant mets, tumor > 4cm, high grade tumors Presence of +LN not strongly linked with survival 10yr = 60-80%
48
Prognosis of medullary thyroid
10yr = 70-80%
49
Prognosis for anaplastic thyroid
Poor Median = 4-5months
50
Position of parathyroids
Superior glands always next to superior lobes of thyroid (from 4th pharyngeal pouch) Inferior glands more variable (posterior/lateral to thyroid and below inf thyroid artery) - from 3rd pouch Not uncommon to have a "missing" inferior gland Most common location for inferiors = thymus. Others could be intravagal, groove in carotid sheath
51
Histology of parathyroids
Chief cells - produce PTH
52
Function of parathyroids
Regulate calcium and P metabolism by PTH, Vit D, and calcitonin Primary organ systems involved = GI, bone, kidney
53
PTH
Serum calcium levels regulate secretion of cleaved PTH by negative feedback 1) Bone - stimulates osteoclasts (more bone resorption) - Inhibits osteoblasts (less bone production) - This causes release of Ca and P 2) Kidney - More reabsorption of Ca - More P excretion 3) GI - Stimulates hydroxylation of 25-OH D to 1,25 OH D - 1,25 OH D increases intestinal absorption of dietary calcium (from duodenum mainly) and P - Promotes mineralization - Enhances PTH's effect on bone
54
Hypercalcemic crisis
Calcium > 13 and symptomatic Treat with saline, furosemide, bisphosphonates, and if needed antiarrhythmic agents
55
Calcitonin
Secreted by thyroid C cells Inhibits bone resorption (inhibits calcium release) Increases urinary excretion of Ca and P Works as counterregulatory hormone to PTH
56
Hyperparathyroidism (Primary) definition
Due to overproduction of PTH, causing increased absorption of calcum from intestines, increased vit d3 production, and decreased renal calcium excretion. This raises overall serum Ca and lowers P
57
Hyperpara (primary) incidence and risk
1/4000 MEN I MEN IIA history of radiation
58
Signs of hyperpara (primary)
Stones, bones, groans, moans Kidney stones Bone pain, pathologic fractures, subperiosteal resorption Groans: nausea, vomit, muscle pain, constipation, pancreatitis Moans: lethargy, confusion, depression, paranoia
59
Causes of primary hyperpara
Solitary adenoma (85-90%) 4-gland hyperplasia (10%) Cancer (
60
Workup and dx for primary hyperpara
Preop imaging = US, sestamibi scan, CT/MRI, operative exploration Dx: 1) Elevation of PTH with inappropriately high serum Ca (normally a high Ca should lower PTH) 2) Check urine for calcium to rule out diagnos of familial hypocalciuric hypercalcemia (will be low if FHH, high if primary hyperpara)
61
Tx for primary hyperpara
1) Solitary adenoma - solitary parathyroidectomy with neck exploration to ID +/- biopsy 3 remaining glands. If preop sestamibi is done to localize area, no need for neck exploration 2) Multiple gland hyperplasia - remove 3 glands, or all 4 with reimplantation of at least 30g of parathyroid tissue in forearm or other site to retain function (makes it easier to resect additional parathyroid gland if hyperparathyroid state persists) Outcomes: First op = 90% success rate Reoperation = 90% success rate if remaining gland is localized preop
62
Secondary hyperpara info
Increased PTH due to hypocalcemia that is the result of chronic renal failure (Phosphate retained leads to low calcium), GI malabsorption, osteomalacia, or rickets
63
Signs of secondary hyperpara
Bone pain from renal osteodystrophy and pruritis Patients often asymptomatic
64
What other conditions should you consider in a patient with hypercalcemia?
R/O malignancy ESP colon, lung, breast, prostate, head, neck, MM Some tumors secrete PTHrP
65
Dx of secondary hyperpara
Made by labs in asymptomatic patients usually due to 4 gland hyperplasia
66
Tx of secondary hyperpara
1) Nonsurgical - In renal failure patients, correct Ca and P. - Restrict P intake, treat with P-binders and Ca/Vit D supplements. Adjust dialysate to maximize Ca and minimize aluminum 2) Surgical - indicated for intractable bone pain or pruritis, or pathologic fractures, with failure of medical therapy - No role for parathyroid surg in secondary hyperpara - Maybe renal transplant
67
Tertiary hyperpara
Due to persistent hyperparathyroidism after treatment for secondary hyperpara. Due to autonomously functioning parathyroid glands that are resistant to negative feedback from high Ca levels. Usually s/p renal transplant Usually a short-lived phenom If persistent, surgery is indicated (3.5 gland resection)
68
Familial hyperpara
MEN 1/2A High recurrence rate Total resection with forearm reimplantation is indicated to facilitate potential reoperation if HPTH persists Patients with sporadic 4 gland hyperplasia may undergo total resection with reimplantation or 3 gland excision
69
Hypopara etiologies
Uncommon Usually from surgery: Following total thyroidectomy, usually transient and treated if symptoms develop Could be congenital absence of all 4 glands Could be DiGeorge - no parathyroid and no thymus Could be functional - chronic hypoMg
70
Hypo para signs
Numbness and tingling of circumoral area, fingers, toes Anxiety, confusion may progress to tetany, hyperventilation, seizures, heart block
71
Tx for hypo para
Supplement with PO Ca and Vit D (to help GI absorption) Pseudohypoparathyroidism = familial disease causing resistance of PTH at target tissue. Patient remain hypoCa and hyperP despite bone resorption from elevated PTH. Tx = Ca and Vit Supplements
72
Signs of parathyroid cancer
40-50% present with firm, fixed mass that is palpable Extremely high Ca and PTH. Usually has high levels of hCG (tumor marker) Neck pain, voice change (due to lesion in recurrent laryngeal nerve)
73
Tx for parathyroid cancer
En bloc surg resection of mass and surrounding structures, along with i/l thyroid lobectomy and i/l LN dissection Postop XRT and chemo not usually helpful Post op complications = recurrent laryngeal nerve damage, severe hypocalcemia (hungry bone syndrome) 5y survival = 70%
74
Chvostek sign
HypoCa Contraction of facial muscles when tapping on facial nerve
75
Trousseau sign
HypoCa Development of carpal spasm by occluding blood flow to forearm
76
Adrenal anatomy
Retroperitoneal T11 Branches of aorta, inferior phrenic and renal arteries Venous = R side drains to IVC. L drains to L renal vein
77
Histo of adrenal glands
Cortex Glomerulosa: aldosterone (salt) Fasiculate: Steroids, cortisol (sugar) Reticularis: Androgens/estrogen (sex)
78
Adrenal adenoma
Most are unilateral If adrenal gland 6cm, surgically resect due to increased risk of adrenocortical carcinoma
79
Adrenal cortical carcinoma signs
Very rare Women more than men, peak
80
Dx of Adrenal Cortical Carcinoma
24h urine for cortisol, aldosterone, catecholamines, metanephrine, VMA, 17-OH corticosteroids, 17-ketosteroids CT (lesions > 7mm) or MRI (esp to assess IVC invasion) CXR to r/o pulm mets
81
Tx for Adrenal Cortical Carcinoma
1) Radical en block but only 1/3 of adrenal carcinomas are operable 2) If resection not done, debulk to reduce amount of cortisol-releasing tissue 3) Bone mets should be palliated with XRT 4) No role for chemo 5) Monitor steroid hormone levels postop 6) Recurrence also warrants resection 7) recurrence: lungs, LN, liver, peritoneum, bone - in 10% of b/l cases, patients develop Nelson's syndome (excess ACTH from pit adenoma. This causes visual issues due to mass effect, hyperpigmentation (more melanocyte-stimulating hormome) and amenorrhea as well)
82
Prognosis for ACC
70% present in stage 3 or 4 5yr = 40% for complete resection If local invasion, median = 2-3y
83
Workup for Cushing's Syndrome
Low THen High 1) 24h urine cortisol elevated then Low dose (single dose) dexa suppression If this fails, we have Cushing's syndrome (too much cortisol being made - we need to find where it's coming from) Is this cortisol driven (ACTH independent) or driven by ACTH? 2) get ACTH level If normal/not high then cortisol itself is driving it. This is a primary adrenal tumor Get CT to find it then resect OR they're eating cortisol If ACTH is high then it's ACTH driven 3) Get High Dose (8mg) Dexa suppression (If ACTH was high) If it suppresses cortisol then it's Cushing's Disease (Ant pit issue. MRI and resect) If it fails then it's ectopic ACTH - CT or PET everything to find it The tests do steroid dose at 11PM and measurement at 8AM (low dose)
84
What is the most common cause of ectopic ACTH production?
#1 = Small cell lung cancer (oat cell carcinoma) #2 = Carcinoid tumors
85
Addison's Disease def
Adrenal insufficiency Primary = Due to destruction of adrenal cortex with sparing of medulla Secondary = Failure due to hypothalamic or pituitary abnormalities
86
Causes of Primary Addison's Disease
1) Post-adrenalectomy 2) AI 3) TB 4) Fungal infx 5) AIDS 6) metastatic cancer 7) Familial glucocorticoid deficiency
87
Causes of secondary Addison's
1) Exogenous steroids (#1 - longterm for medical use or for illicit use) 2) Craniopharyngioma 3) Pituitary surgery or irradiation 4) Empty sella syndrome Abruptly stopping longterm steroid use may cause adrenal insufficiency too. 6 months may be required for intrinsic controls to come back online
88
Addisonian crisis
Acute situation due to some extrinsic stressor like infection or surgery
89
Signs of Addison's
Nause, vomiting Abdominal pain Tachy Weight loss Fatigue Lethargy Hyperpigmentation (low levels of cortisol cause increased pit production of proopiomelanocortin - POMC - which is a precursor to ACTH and MSH) Fever and hypovolemic shock in crisis
90
Dx of Addison's
1) HypoNa, HyperK (due to low aldosterone which is normally produced by adrenals) 2) ACTH stimulation test - give ACTH and measure cortisol level after 30mins. If adrenal failure is present, there will be no increase in cortisol 3) Baseline ACTH level is elevated in patients with primary failure due to absence of negative feedback
91
Tx of Addison's
1) Glucocorticoid therapy for primary and secondary causes 2) Additional mineralocorticoid therapy for primary cause 3) Addisonian crisis - Volume (D5NS) and IV glucocorticoids
92
Stress dose steroids perioperatively?
Patients with Addison's OR anyone who has been on steroids for more than 6 months prior to surgery should get periop stress dose steroids
93
Causes of hyperaldosteronism
Primary (too much aldosterone secretion) = Conn's Syndrome - Aldosterone-secreting tumor (66%) - Idiopathic adrenocortical hyperplasia (30%) Secondary (due to high renin leading to high aldo) - Renal artery stenosis - Cirrhosis - CHF - Normal pregnancy
94
Signs of hyperaldo
HTN Muscle weakness and cramping HA Polyuria Polydipsia HypoK
95
Dx for hyperaldo
Primary/Conn's 1) Diastolic HTN without edema 2) High plasma aldo 3) Normal or low plasma renin 4) HypoK, high urineK (off antihypertensive meds) 5) Post-captopril plasma aldo: - normally results in low aldo - diagnostic of hyperaldo if ratio > 50 6) Imaging - CT picks it up if >1cm. If there is an aldosteronoma, opposite adrenal appears atrophied - Iodocholesterol scan - picks up 90% of aldosteronomas and shows how functional they are. Hyperplasia will present as b/l hyperfunction versus unilateral for tumor - if all imaging inconclusive, maybe sample adrenal vein for aldo and cortisol pre and post ACTH - U/l elevation of aldo or ald/cortisol ratio indicated aldo-secreting adenoma - B/l elevation of aldo is consistent with hyperplasia
96
Caveat to eval for Conn's
Make sure patient isn't just an uncontrolled hypertensive on K wasting diuretics
97
Tx for hyperaldo
Primary 1) Hyperplasia - medical treatment with spironolactone, nifedipine, amiloride and/or other antihypertensive. NO SURG 2) Adenoma - Lap adrenalectomy Outcomes: most patients become normotensive and normokalemic with tx. 20-30% have recurrent HTN in 2-3 years Secondary 1) Treat underlying cause
98
Hypoaldo
Decreased aldo without a change in cortisol production Causes = Congenital error of aldo synthesis, failure of glomerulosa (AI), s/p adrenalectomy, drug inhibition Signs = postural hypotension, persistent and severe hyperK, muscle weakness, arrhythmias Tx = mineralocorticoid replacement
99
Neuroblastoma def
A medullary tumor of adrenals - Embryonal neural crest tumor mainly in children (small round blue cell tumor) - #4 most common peds malignancy - Can occur anywhere along sympathetic chain (50% in adrenal, 25% paraspinal ganglia, 20% thorax, 5% pelvis) - May spontaneously differentiate and regress - Aggressive tumor that commonly presents with distant mets. In 50% of infants and 66% of older children (to LN, bone, liver, subQ)
100
Diseases associated with neuroblastoma
Neurofibromatosis Beckwith-Wiedermann Syndrome Trisomy 18
101
Signs of neuroblastoma
Abdominal or flank mass Respiratory distress SubQ blue tumor nodules (blueberry muffin sign)
102
Dx of neuroblastoma
Imaging CT for staging. MRI Urinary tumor markers - high 24h levels of homovanillic acid, vanillylmandelic acid and metanephrines
103
Tx of neuroblastoma
Localized (stage 1/2) - surg resection Nonlocalized disease (stage 2) - surg resection with chemo +/- radiation Mets (4) - surg resection with chemo +/- radiation LN mets warrants XRT
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prognosis of neuroblastoma
Mortality lower when diagnosed within first year of life 5y survival = 90% for disease confined to primary site, 20-40% for disseminated disease
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10% rule for pheocrhomocytoma
``` 10% malignant 10% familial 10% extra-adrenal (morel likely to be malignant. Often at organ of Zuckerkandl to left of aortic bifurcation at IMA) 10% bilateral (more likely familial) 10% in children ```
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Pheo definition
Chromaffin cell tumor usually in adrenal medulla (90%) but can be anywhere along sympathetic chain. Erroneous production of Epi
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Pheo risk factors
MEN IIA/2B (usually cause bilateral) VHL Neurofibromatosis FHx
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5 Ps of Pheo
Paroxysms (comes and goes - since Epi is released in pulses) Pressure (HTN) Pain (HA) Palpitations (sympathetic overdrive) Perspire
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Dx of Pheo
24h urine VMA and metanephrine (elevated)
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Tx of Pheo
Alpha block first THEN B block THEN resect A before B bc we don't want the B block to cause unopposed alpha activity which would raise BP even more If you resect first you'll release catecholamines everywhere and make patient stroke out and die ( for same reason, ligate veins before resection)
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parts of pit gland
Anterior = Adenohypophysis Posterior = Neurohypophysis
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Sheehans signs
Postpartum infarction and necrosis of pit leading to hormonal failure Pit ischemia due to hemorrhage, hypovolemic shock, pit portal venous thrombosis Failure of lactation Amenorrhea Progressive decreased adrenal function and thyroid function
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Posterior pit disorders
SIADH and DI
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SIADH
15% of hospitalized patients Impaired water secretion Hypersecretion of ADH results in increased urinary sodium with high urine osmolality Causes = CNS injury, cancer, trauma, drugs
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DI
Decreased ADH secretion Impaired water conservation; large volumes of urine, leads to increased plasma osmolality and thirst 1/3 idiopathic 2/3 due to tumor or trauma
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Signs of pit macroadenoma
>1cm Visual loss (b/l hemianopsia), hypopituitarism, HA, hyperprolactinema due to compression of stalk - compression prevents dopamine from going from hypothal to post pit where it would normally inhibit prolactin production This causes hyperprolactinemia and nipple discharge
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Signs of pit microadenoma
Prolactinoma (#1) = secondary amenorrhea, galactorrhea GH - Gigantism or acromegaly depending on age; course facial features, thick finger and heel pads, cardiomegaly, hepatomegaly, enlarged mandible, increased teeth spaces, neuropathy, arthropathy, osteoporosis, HTN, DM, goiter ACTH - Cushings
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Complications of pit surgery
Death (direct hypothal injury
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Treatment for pit adenomas
PreOp: complete endocrine assessment, lytes to look for borderline DI Surg: - Transphenoidal vs transcranial vs perioperative - PostOp XRT for large lesions Primary Radiation: - consider when surg contraindicated for other reasons in nonfunctioning tumor as primary therapy may worsen preexisting hypopituitarism Medical - For prolactin - Bromocriptine - For GH - somatostatin which shrinks tumor size in 20-50%, normalizes GH in 50%, and normalizes IGF-1 in 40-80%