Surgical Recall - Ch. 57 Endocrine

Question 1

Conn’s Syndrome

1. What is it?
2. What is the normal physiology for aldosterone secretion?
3. What is the normal physiologic effect of aldosterone?
4. What are the two classic clues of Conn’s syndrome?
5. Classically, what kind of HTN?
6. What are the renin levels with Conn’s syndrome?
7. What dx tests should be ordered?
8. What is the saline infusion test?

Answer 1

Conn’s Syndrome

1. What is it? primary hyperaldosteronism
2. What is the normal physiology for aldosterone secretion?
   
   1. BP in renal afferent arteriole is low
   2. Low sodium and hyperkalemia –> renin secretion from JGA
   3. Renin then converts angiotensinogen to angiotensin I
   4. ACE in the lung then converts AI to AII
   5. AII causes adrenal glomerulosa cells to secrete aldosterone
3. What is the normal physiologic effect of aldosterone?
   
   1. Aldosterone causes Na retention for exchange of K+ in the kidney, resulting in fluid retension and inc. BP
4. What are the two classic clues of Conn’s syndrome?
   
   1. HTN
   2. Hypokalemia
5. Classically, what kind of HTN? Diastolic
6. What are the renin levels with Conn’s syndrome? Normal or decreased!
7. What dx tests should be ordered? Plasma aldosterone conc. and PRA
8. What is the saline infusion test? Saline infusion will dec. aldosterone levels in normal pts but not in Conn’s syndrome

Question 2

Addison’s Disease

1. What is it?
2. What are the electrolyte findings?

A pt with mild skin pigmentation is admitted emergently to your service because of sudden abdominal pain, fever, and a rigid abdomen. Her blood work indicates a marked leukocytosis, a blood sugar of 55 mg/dL, a sodium value of 119 mEq/dL, and a potassium value of 6.2 mEq/dL. Her BP is 88/58 mm Hg. She undergoes an ex lap. What is the definitive treatment for her primary condition?

Answer 2

Addison’s Disease

1. What is it? Acute adrenal insufficiency
2. What are the electrolyte findings? Hyperkalemia, hyponatremia

CORTICOSTEROIDS (adrenal insufficiency)

Question 3

Insulinoma

1. What is it?
2. What are the associated risks?
3. What are the signs/sx?
4. What are the neurologic sx?
5. Whipple’s triad?
6. What lab tests should be performed?
7. What diagnostic tests should be performed?
8. What localizing tests should be performed?
9. What is the medical tx? Surgical tx?

Answer 3

Insulinoma

1. What is it? Insulin-producing tumor arising from beta cells
2. What are the associated risks? MEN-1 syndrome (Pituitary, Pancreas, Parathyroid tumors)
3. What are the signs/sx? Sympathetic nervous system symptoms resulting from hypoglycemia: palpitations, diaphoresis, tremulousness, irritability, weakness
4. What are the neurologic sx? Personality changes, confusion, obtundation, seizures, comas
5. Whipple’s triad?
   
   1. Hypoglycemic sx produced by fasting
   2. Blood glucose <50 mg/dL during symptomatic attack
   3. Relief of sx by administration of glucose
6. Lab tests?
   
   1. Glucose and insulin levels during fast;
   2. C-peptide and proinsulin levels (if self-injection of insulin is a concern as insulin injections have no proinsulin or C-peptides)
7. Diagnostic tests?
   
   1. Fasting hypoglycemia inappropriately high levels of insulin
   2. 72 hr fast, then check glucose and insulin levels every 6 hrs
      
      1. ​Dx fasting insulin:glucose ratio >0.4
8. ​​​​Localizing tests?
   
   1. CT scan
   2. A-gram
   3. Endoscopic U/S
   4. Venous catheterization (to sample blood along portal and splenic veins to measure insulin and localize tumor)
9. ​Medical tx? Diazoxide, to suppress insulin release; Surgical tx? Resect!

Question 4

Glucagonoma

1. What is it?
2. Where is it located?
3. Symptoms?
4. Associated lab findings?
5. Classic finding of CBC?
6. Classic nutritional finding?
7. Stimulation test used for glucagonoma?
8. Test used for localization?
9. Medical treatment of necrotizing migratory erythema?
10. What is the treatment?

Answer 4

Glucagonoma

1. What is it? Glucagon-producing tumor
2. Where is it located? Pancreas (usually the tail)
3. Symptoms? Necrotizing migratory erythema (usually below the waist), glossitis, stomatitis, diabetes
4. Associated lab findings? Hyperglycemia, low AA levels, high glucagon levels
5. Classic finding of CBC? Anemia
6. Classic nutritional finding? Low AA levels
7. Stimulation test used for glucagonoma? Tolbutamide stimulation test: IV tolbutamide results in elevated glucagon levels
8. Test used for localization? CT scan
9. Medical treatment of necrotizing migratory erythema? Somatostatin, IV amino acids
10. What is the treatment? Surgical resection

Question 5

Somatostatinoma

1. What is it?
2. What is the dx triad?
3. What is used to make the dx?
4. What is the tx?
5. What is the medical tx if the tumor is unresectable?

Answer 5

Somatostatinoma

1. What is it? Pancreatic tumor that secretes somatostatin
2. What is the dx triad?
   
   1. Diabetes
   2. Diarrhea (steatorrhea)
   3. Dilation of the gallbladder with gallstones
3. What is used to make the dx? CT scan and somatostatin level
4. What is the tx? Resection
5. What is the medical tx if the tumor is unresectable? Streptozocin, dacarbazine, doxorubicin

Question 6

Zollinger-Ellison Syndrome (ZES)

1. What is it?
2. What is the associated syndrome?
3. What % of pts with ZES have MEN-1 syndrome?
4. What % of pts with MEN-1 syndrome have ZES?
5. W/ gastrinoma, what lab tests should be ordered to screen for MEN-1?
6. What are the signs/sx?
7. What causes the diarrhea?
8. What lab tests should be performed?
9. Where are most gastrimonas found?
10. What is the medical tx?
11. What is the surgical option if gastrinoma is in duodenum/head of pancreas and is too large for local resection?

Answer 6

Zollinger-Ellison Syndrome (ZES)

1. What is it? Gastrinoma: non-beta islet cell tumor of the pancreas (usually in the tail)
2. What is the associated syndrome? MEN-1
3. What % of pts with ZES have MEN-1 syndrome? 25% (~75% cases = sporadic)
4. What % of pts with MEN-1 syndrome have ZES? ~50%
5. W/ gastrinoma, what lab tests should be ordered to screen for MEN-1? Calcium level / PTH level
6. Signs/sx? Peptic ulcers, diarrhea, weight loss, abdominal pain
7. Diarrhea? Massive acid hypersecretion and destruction of digestive enzymes
8. What lab tests should be performed?
   
   1. Fasting gastrin level (normal = 100 pg/ml / ZES fasting = 200-1000 pg/ml)
   2. Postsecretin challenge gastrin level
   3. Calcium
9. ​Passoro’s triangle (“gastrinoma triangle”)
   
   1. ​Cystic duct/CBD jxn
   2. Jxn of 2nd and 3rd portions of the duodenum
   3. Neck of the pancreas
10. ​Medical tx: H2 blockers, omeprazole, somatostatin
11. What is the surgical option if gastrinoma is in duodenum/head of pancreas and is too large for local resection? Whipple procedure

Question 7

MEN

1. How is it inherited?

MEN Type 1

1. What is the gene defect in MEN 1?
2. What are the most common tumors?

MEN Type IIa

1. What is the gene defect in MEN IIa?
2. WHat are the most common tumors?

MEN Type IIb

1. What are the most common abnormalities?
2. What is the most common compliant of patients with MEN-IIb?

Answer 7

MEN

1. How is it inherited? AD

MEN Type 1

1. What is the gene defect in MEN type 1? xsome 11
2. What are the most common tumors?
   
   1. Parathyroid hyperplasia
   2. Pancreatic islet cell tumors: gastrinoma, insulinoma
   3. Pituitary tumors

MEN Type IIa

1. What is the gene defect in MEN IIa? RET
2. What are the most common tumors?
   
   1. Medullary thyroid carcinoma (100%) - calcitonin secreted
   2. Pheochromocytoma (>33%) - catecholamine excess
   3. Hyperparathyroidism (~50%) - hypercalcemia

MEN Type IIb

1. What are the most common abnormalities?
   
   1. Mucosal neuromas (100%) - in nasopharynx, oropharynx, larynx, conjunctiva
   2. Medullary thyroid carcinoma (~85%) - more aggressive than in MEN IIa
   3. Marfanoid body habitus
   4. Pheochromocytoma (~50%) - found bilaterally
2. Constipation resulting from ganglioneuromatosis of GI tract

Question 8

Answer 8

E. VIPoma

WDHA (watery diarrhea, hypokalemia, achlorydria)

diarrhea is odorless, tea-colored

Question 9

Answer 9

Insulinoma