Endocrinology Practice Questions

Q: Multiple submucosal neuromas are most likely associated with:

Multiple endocrine neoplasia type 2B. ***Multiple endocrine neoplasia type 2B*** - Multiple submucosal neuromas, particularly in the lips, tongue, and gastrointestinal tract, are a **hallmark feature** of MEN2B. - This syndrome is also characterized by **medullary thyroid carcinoma** and **pheochromocytoma**, often with a marfanoid habitus. *Ovarian germ cell tumor* - These tumors arise from **germ cells** in the ovary and include types such as dysgerminomas, teratomas, and yolk sac tumors. - They are not associated with the development of **submucosal neuromas**. *Testicular seminoma* - A type of **germ cell tumor** originating in the testes, which can produce HCG or LDH. - This condition has no known association with the development of **multiple submucosal neuromas**. *Pancreatic neuroendocrine tumor* - These tumors arise from the **endocrine cells** of the pancreas and can be functional (e.g., insulinoma, gastrinoma) or non-functional. - While they are endocrine tumors, they do not present with **submucosal neuromas**.

Q: The triad of diabetes, gallstones, and steatorrhea is associated with which of the following?

Somatostatinomas. ***Somatostatinomas*** - This **triad** is characteristic of a somatostatinoma, as somatostatin inhibits insulin release, gallbladder contraction, and pancreatic enzyme secretion. - The inhibition of **insulin release** leads to diabetes [1], blocked **cholecystokinin (CCK)** release causes gallstones, and reduced **pancreatic enzyme** secretion results in steatorrhea. *Gastrinomas* - Gastrinomas typically cause **Zollinger-Ellison syndrome**, characterized by severe peptic ulcers and diarrhea due to excessive acid production. - They are not directly associated with the specific triad of diabetes, gallstones, and steatorrhea. *VIPomas* - VIPomas are known for causing **Verner-Morrison syndrome** or pancreatic cholera, leading to severe watery diarrhea, hypokalemia, and achlorhydria. - Diabetes and gallstones are not prominent features of VIPomas. *Glucagonomas* - Glucagonomas primarily manifest with **diabetes** (due to elevated glucagon), a characteristic skin rash called **necrolytic migratory erythema**, and weight loss [1]. - While diabetes is present, gallstones and steatorrhea are not typical associations.

Q: A middle-aged male patient who was involved in a road traffic accident and brought to the emergency department in an unconscious state. A computed tomography (CT) scan of the abdomen revealed a splenic laceration, and emergency splenectomy was performed. The patient was then transferred to the intensive care unit (ICU). Despite receiving a bolus of normal saline, his blood pressure remained low in the postoperative period. On examination, he was afebrile, had a moon-like face with central obesity, and exhibited violet striae on his abdomen. A repeat CT scan of the chest, abdomen, and pelvis showed no evidence of hemorrhage. What is the next best step in managing this patient?

Administer hydrocortisone 100 mg IV. ***Administer hydrocortisone 100 mg IV*** - The patient's presentation with **hypotension unresponsive to fluid resuscitation**, combined with a **moon-like face**, **central obesity**, and **violet striae**, strongly suggests **adrenal insufficiency** as a complication of **Cushing's syndrome**. [1] - **Adrenal crisis** requires immediate administration of **stress-dose corticosteroids** to prevent cardiovascular collapse. [1] *Return to the operating room for exploratory laparotomy* - A **repeat CT scan** confirmed no evidence of hemorrhage, ruling out **active bleeding** as the cause of refractory hypotension. - Therefore, subjecting the patient to another surgery without evidence would be unnecessary and introduce additional risks. *Administer vancomycin and piperacillin/tazobactam* - The patient is **afebrile**, and there are no signs or symptoms suggestive of a **postoperative infection**. - Initiating broad-spectrum antibiotics without an indication in an already compromised patient is not appropriate and could contribute to **antibiotic resistance**. *Perform MRI of the spine.* - There is nothing in the patient's presentation that suggests **spinal cord injury** as the cause of his current unstable condition. - While the patient was in a **road traffic accident**, the primary issues are related to **hemodynamic instability** and suspected **endocrine dysfunction**, not neurological deficit.

Q: Nelson syndrome is associated with:

Bilateral adrenalectomy. ***Bilateral adrenalectomy*** - **Nelson syndrome** develops in a subset of patients with Cushing's disease after **bilateral adrenalectomy**. [1] - It is caused by the loss of negative feedback from adrenal cortisol on the pituitary, leading to continued growth of an existing **ACTH-secreting pituitary adenoma**. *Prophylactic craniospinal irradiation* - **Craniospinal irradiation** is a radiotherapy technique used to treat disseminated cancers like medulloblastoma but is not directly associated with the etiology of Nelson syndrome. - While pituitary tumors can be treated with radiotherapy, this is typically given once a tumor has caused symptoms, not prophylactically in this context. *Subarachnoid hemorrhage* - **Subarachnoid hemorrhage** is bleeding into the space between the brain and the surrounding membrane, often caused by ruptured **aneurysms** or **trauma**. - It is a neurological emergency unrelated to the development of Nelson syndrome. *BRCA2 mutation* - **BRCA2 mutations** are strongly associated with an increased risk of developing various cancers, most notably **breast** and **ovarian cancer**. - This genetic mutation is not linked to the pathogenesis of Nelson syndrome, which is an endocrine complication following surgery.

Q: Hyperaldosteronism is associated with all, except:

Metabolic acidosis. ***Metabolic acidosis*** - **Hyperaldosteronism** leads to increased **potassium and hydrogen ion excretion** in the kidneys [1], resulting in **metabolic alkalosis**, not acidosis [2]. - The increased loss of hydrogen ions causes a rise in blood pH and bicarbonate levels [2]. *Hypernatremia* - Aldosterone promotes **sodium reabsorption** in the renal tubules, leading to increased plasma sodium concentration [1], [3]. - This increased sodium reabsorption contributes to the expansion of extracellular fluid volume and **hypertension** [3]. *Hypokalemia* - Aldosterone stimulates the **secretion of potassium ions** into the renal tubules, leading to excessive potassium loss in the urine [1]. - This sustained potassium excretion often results in **low serum potassium levels**. *Hypertension* - The increased reabsorption of **sodium and water** due to aldosterone action expands the extracellular fluid volume [3]. - This volume expansion directly contributes to elevated blood pressure, making hypertension a hallmark feature of **hyperaldosteronism** [2].

Q: A 37-year-old man in diabetic ketoacidosis has yellowish papules scattered over his trunk and extremities. A tube of plasma collected from the patient develops a turbid supernate. This is consistent with which type of hyperlipoproteinemia?

Type IV. Type IV Hyperlipoproteinemia - This patient presents with eruptive xanthomas (yellowish papules) and a turbid plasma supernate, which suggests significantly elevated very-low-density lipoprotein (VLDL) and chylomicrons. - In Type IV hyperlipoproteinemia, there is an overproduction or decreased clearance of VLDL, often exacerbated by underlying conditions like uncontrolled diabetes mellitus (such as in DKA) [1]. Type I Hyperlipoproteinemia - Characterized by a deficiency of lipoprotein lipase or its coenzyme apoC-II, leading to extremely high chylomicron levels. - While it presents with eruptive xanthomas and milky plasma, diabetes mellitus is not a primary driver of this specific type, and the VLDL component is less pronounced than in Type IV. Lipoprotein lipase expression is notably enhanced by insulin [2]. Type II Hyperlipoproteinemia - This type primarily involves elevated low-density lipoprotein (LDL), often due to defects in LDL receptors, leading to conditions like familial hypercholesterolemia. - It's mainly associated with tendinous xanthomas and corneal arcus, and the plasma, though sometimes turbid due to LDL, would not typically show the degree of chylomicron or VLDL elevation expected with eruptive xanthomas and DKA. Type III Hyperlipoproteinemia - This condition involves elevated chylomicron remnants and VLDL remnants due to a defective apoE, specifically apoE2/E2 homozygosity. - While it can manifest with eruptive xanthomas, it more classically presents with palmar xanthomas and tuberoeruptive xanthomas, and the turbid supernate would typically have a distinct 'creamy layer' after refrigeration, which is not emphasized here.

Q: Which clinical features are specifically associated with congenital adrenal hyperplasia due to 21-hydroxylase deficiency?

Female pseudohermaphroditism. ***All of the above*** - 21-hydroxylase deficiency leads to a lack of cortisol and aldosterone, causing adrenal crisis. Thus, **hyponatremia**, **hyperkalemia**, and **ambiguous genitalia** in females are clinical features associated with this condition [1]. - It also results in **female pseudohermaphroditism** due to excess androgen exposure in utero, showcasing the spectrum of symptoms seen [1]. *Hyponatremia and hyperkalemia* - While these are common electrolyte abnormalities seen in adrenal crisis due to **aldosterone deficiency**, they don't capture the entire clinical picture of 21-hydroxylase deficiency [1]. - Focusing solely on these abnormalities neglects significant features like **ambiguous genitalia** and **pseudohermaphroditism**. *Ambiguous genitalia in females* - This is indeed a critical feature of 21-hydroxylase deficiency but does not represent the full array of clinical manifestations involved [1]. - The deficiency also affects electrolyte balance which is a crucial aspect, thereby making it insufficient as a standalone answer. *Female pseudohermaphroditism* - While **female pseudohermaphroditism** due to androgen excess is a key characteristic, it is just one of multiple clinical features of this condition [1]. - This option overlooks **hyponatremia**, **hyperkalemia**, and **ambiguous genitalia**, which are all critical for a comprehensive understanding of the disorder [1].

Q: All of the following may be associated with primary aldosteronism, except:

Von-Hippel-Lindau syndrome. ***Von-Hippel-Lindau syndrome*** - **Von Hippel-Lindau (VHL) syndrome** is a genetic disorder commonly associated with tumors such as **pheochromocytomas**, **renal cell carcinoma**, hemangioblastomas, and pancreatic neuroendocrine tumors, but not primary aldosteronism. - While VHL can involve adrenal glands (pheochromocytomas being a common manifestation), it does not directly cause **aldosterone overproduction** leading to primary aldosteronism. *Adrenal adenoma* - A **solitary adrenal adenoma** is the most common cause of primary aldosteronism, often referred to as **Conn's syndrome** [1]. - These benign tumors autonomously secrete **aldosterone**, leading to hypertension and hypokalemia [1]. *Adrenal hyperplasia* - **Bilateral adrenal hyperplasia** is the second most common cause of primary aldosteronism, where both adrenal glands produce excessive aldosterone. - This condition is often managed medically rather than surgically, in contrast to unilateral adenomas [1]. *Adrenal carcinoma* - Although rare, a **functional adrenal carcinoma** can cause primary aldosteronism by overproducing aldosterone [1]. - These malignant tumors are typically larger and more aggressive than adenomas [1].

Q: What is the preferred insulin regimen during pregnancy?

Insulin NPH + Lispro. ***Insulin NPH + Lispro*** - **NPH insulin** is a preferred basal insulin during pregnancy due to its long-standing safety record and intermediate duration of action [1]. - **Insulin Lispro** is a rapid-acting insulin analog that is safe and effective for postprandial glucose control in pregnant women [1]. *Insulin Regular + NPH* - While NPH is safe, **regular insulin** has a slower onset and longer duration compared to rapid-acting analogs, making it less ideal for precise postprandial control [2]. - Achieving tight glycemic control with regular insulin can be challenging due to its peak action occurring later, potentially leading to **postprandial hyperglycemia** or **unpredictable hypoglycemia** [2]. *Insulin Degludec* - **Insulin Degludec** is a newer ultra-long-acting insulin analog, and while effective, there is currently limited data on its long-term safety during pregnancy. - Due to insufficient robust safety data, it is generally **not the preferred first-line basal insulin** for pregnant women. *Insulin Lispro + Insulin regular* - This combination lacks a dedicated **basal insulin** component, which is crucial for maintaining fasting and between-meal glucose levels [2]. - Relying solely on rapid-acting and short-acting insulins without basal coverage would likely result in **poor glycemic control** throughout the day and night [1].

Q: Which of the following is a specific cause of metabolic acidosis?

Diabetic ketoacidosis. ***Diabetic ketoacidosis*** - **Diabetic ketoacidosis (DKA)** is a specific condition where an absolute or relative insulin deficiency leads to unchecked **ketone body production** (acetoacetate, beta-hydroxybutyrate), which are organic acids [2]. - The accumulation of these **ketoacids** overwhelms the body's buffer systems, resulting in a **high anion gap metabolic acidosis** [1], [3]. *Diarrhea* - **Diarrhea** causes **metabolic acidosis** due to the loss of **bicarbonate** from the gastrointestinal tract. - This is typically a **normal anion gap metabolic acidosis**, also known as hyperchloremic metabolic acidosis, which is less specific than DKA. *Renal failure* - **Renal failure** can cause **metabolic acidosis** primarily due to the kidneys' inability to excrete acid and reabsorb bicarbonate. - While it often leads to a **high anion gap metabolic acidosis**, it is a broader category and not as specific a cause as DKA, as various types of renal dysfunction can be involved. *None of the options* - This option is incorrect because **diabetic ketoacidosis** is indeed a specific and well-defined cause of metabolic acidosis, characterized by the accumulation of ketoacids due to insulin deficiency [2].

Question 1

Multiple submucosal neuromas are most likely associated with:

Accepted Answer

Multiple endocrine neoplasia type 2B

Answer

Ovarian germ cell tumor

Answer

Testicular seminoma

Answer

Pancreatic neuroendocrine tumor

Question 2

The triad of diabetes, gallstones, and steatorrhea is associated with which of the following?

Accepted Answer

Somatostatinomas

Answer

VIPomas

Answer

Gastrinomas

Answer

Glucagonomas

Question 3

A middle-aged male patient who was involved in a road traffic accident and brought to the emergency department in an unconscious state. A computed tomography (CT) scan of the abdomen revealed a splenic laceration, and emergency splenectomy was performed. The patient was then transferred to the intensive care unit (ICU). Despite receiving a bolus of normal saline, his blood pressure remained low in the postoperative period. On examination, he was afebrile, had a moon-like face with central obesity, and exhibited violet striae on his abdomen. A repeat CT scan of the chest, abdomen, and pelvis showed no evidence of hemorrhage. What is the next best step in managing this patient?

Accepted Answer

Administer hydrocortisone 100 mg IV

Answer

Return to the operating room for exploratory laparotomy

Answer

Administer vancomycin and piperacillin/tazobactam

Answer

Perform MRI of the spine.

Question 4

Nelson syndrome is associated with:

Accepted Answer

Bilateral adrenalectomy

Answer

Subarachnoid hemorrhage

Answer

Prophylactic craniospinal irradiation

Answer

BRCA2 mutation

Question 5

Hyperaldosteronism is associated with all, except:

Accepted Answer

Metabolic acidosis

Answer

Hypernatremia

Answer

Hypertension

Answer

Hypokalemia

Question 6

A 37-year-old man in diabetic ketoacidosis has yellowish papules scattered over his trunk and extremities. A tube of plasma collected from the patient develops a turbid supernate. This is consistent with which type of hyperlipoproteinemia?

Accepted Answer

Type IV

Answer

Type I

Answer

Type II

Answer

Type III

Question 7

Which clinical features are specifically associated with congenital adrenal hyperplasia due to 21-hydroxylase deficiency?

Accepted Answer

Female pseudohermaphroditism

Answer

All of the above features are present in 21-hydroxylase deficiency.

Answer

Hypertension due to adrenal crisis

Answer

Hypokalemia and hypernatremia

Question 8

All of the following may be associated with primary aldosteronism, except:

Accepted Answer

Von-Hippel-Lindau syndrome

Answer

Adrenal adenoma

Answer

Adrenal hyperplasia

Answer

Adrenal carcinoma

Question 9

What is the preferred insulin regimen during pregnancy?

Accepted Answer

Insulin NPH + Lispro

Answer

Insulin Regular + NPH

Answer

Insulin Degludec

Answer

Insulin Lispro + Insulin regular

Question 10

Which of the following is a specific cause of metabolic acidosis?

Accepted Answer

Diabetic ketoacidosis

Answer

Diarrhea

Answer

Renal failure

Answer

None of the options

Endocrinology — MCQs

Endocrinology — MCQs

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