What is the complete classic triad of findings that defines Young's syndrome?
Which of the following statements about hypercalcemia in sarcoidosis is false?
Which of the following does not synthesize von Willebrand factor?
Which one of the following is the most common CNS tumor associated with type I neurofibromatosis?
Which of the following glands is NOT typically involved in Multiple Endocrine Neoplasia type II A (MEN II A)?
Which of the following is NOT a feature of Peutz-Jeghers syndrome?
What is the most common location of gastrinoma?
Which of the following statements about Gilbert syndrome is false?
Which of the following statements about polio is false?
Which of the following is true about Hepatitis A virus?
NEET-PG 2013 - Internal Medicine NEET-PG Practice Questions and MCQs
Question 31: What is the complete classic triad of findings that defines Young's syndrome?
- A. Azoospermia, bronchiectasis, and chronic sinusitis (Correct Answer)
- B. Oligospermia, bronchiectasis, and chronic sinusitis
- C. Azoospermia, asthma, and chronic rhinitis
- D. Azoospermia, chronic bronchitis, and nasal polyps
Explanation: ***Azoospermia, bronchiectasis, and chronic sinusitis*** - Young's syndrome is characterized by the triad of **azoospermia** (due to obstructive epididymal dysfunction), **bronchiectasis**, and **chronic sinusitis** [1]. - This syndrome primarily affects **middle-aged men** and is often mistaken for cystic fibrosis due to similar respiratory symptoms. *Azoospermia, asthma, and chronic rhinitis* - This option incorrectly lists **asthma** and **chronic rhinitis** instead of bronchiectasis and chronic sinusitis. - While respiratory symptoms are part of Young's syndrome, specifically **bronchiectasis** and **sinusitis** are key [1]. *Oligospermia, bronchiectasis, and chronic sinusitis* - This option is incorrect because Young's syndrome is defined by **azoospermia** (complete absence of sperm), not just **oligospermia** (low sperm count). - The obstructive nature of the epididymal dysfunction in Young's syndrome leads to a complete lack of sperm. *Azoospermia, chronic bronchitis, and nasal polyps* - This option incorrectly identifies **chronic bronchitis** and **nasal polyps** as part of the classic triad. - The correct respiratory components are **bronchiectasis** and **chronic sinusitis**, which signify persistent inflammation and structural lung changes rather than simply bronchitis.
Question 32: Which of the following statements about hypercalcemia in sarcoidosis is false?
- A. PTHrP level is increased
- B. Parathormone level is increased (Correct Answer)
- C. Oral steroids are useful
- D. Calcitriol level is increased
Explanation: ***Parathormone level is increased*** - In **sarcoidosis-associated hypercalcemia**, the parathormone (PTH) level is typically **low or suppressed**. [1] - This is because the hypercalcemia is due to **extra-renal 1-$\alpha$ hydroxylation** of 25-hydroxyvitamin D to 1,25-dihydroxyvitamin D (calcitriol) by macrophages in granulomas, not primary hyperparathyroidism. [1] *PTHrP level is increased* - This statement is **false** for sarcoidosis. Elevated **parathyroid hormone-related peptide (PTHrP)** is a common cause of hypercalcemia in **malignancy**, particularly squamous cell carcinomas, but not in sarcoidosis. - Hypercalcemia in sarcoidosis is **PTH-independent** and not mediated by PTHrP. [1] *Oral steroids are useful* - This statement is **true**. **Corticosteroids** (like oral prednisone) are effective in treating hypercalcemia in sarcoidosis. - They work by **inhibiting the activity of 1-$\alpha$ hydroxylase** in alveolar macrophages and reducing intestinal calcium absorption. *Calcitriol level is increased* - This statement is **true**. In sarcoidosis, activated **macrophages within granulomas** aberrantly express **1-$\alpha$ hydroxylase**. [1] - This leads to the **extra-renal synthesis of calcitriol** (1,25-dihydroxyvitamin D), which increases intestinal calcium absorption and bone resorption, causing hypercalcemia. [1]
Question 33: Which of the following does not synthesize von Willebrand factor?
- A. Endothelial cells
- B. Hepatocytes (Correct Answer)
- C. Megakaryocytes
- D. None of the options
Explanation: ***Hepatocytes*** - Von Willebrand factor (vWF) is primarily synthesized by **endothelial cells** and **megakaryocytes** [1], not hepatocytes. - Hepatocytes are responsible for synthesizing other proteins like **clotting factors**, but not vWF. *Megakaryoctyes* - Megakaryocytes play a crucial role in the synthesis of **platelet-derived factors**, including von Willebrand factor (vWF) [1]. - They release vWF into the bloodstream, facilitating platelet adhesion, especially in vascular injury sites. *None* - The option implies all listed cell types synthesize vWF, which is incorrect, as **only endothelial cells and megakaryocytes** produce it [1]. - Suggests a misunderstanding of the synthesis of coagulation-related factors, as hepatocytes do not produce vWF. *Endothelial cells* - Endothelial cells are the primary source of **von Willebrand factor** [1], releasing it to assist in platelet aggregation and clotting. - They are essential for the body's response to vascular injury, facilitating hemostasis. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 669-670.
Question 34: Which one of the following is the most common CNS tumor associated with type I neurofibromatosis?
- A. Optic nerve glioma (Correct Answer)
- B. Meningioma
- C. Acoustic schwannoma
- D. Low grade astrocytoma
Explanation: ***Optic nerve glioma*** - **Optic nerve gliomas** are the most frequently encountered central nervous system tumors in patients with **Type 1 neurofibromatosis (NF1)**, occurring in about 15% of individuals. - They are typically low-grade **astrocytomas** and can cause vision loss and proptosis depending on their size and location. *Meningioma* - While more common in **Type 2 neurofibromatosis (NF2)**, meningiomas can occur in NF1, but are not the most common CNS tumor. - Meningiomas are tumors that arise from the **meninges**, the membranes surrounding the brain and spinal cord. *Acoustic schwannoma* - **Bilateral acoustic schwannomas (vestibular schwannomas)** are the hallmark feature of **Type 2 neurofibromatosis (NF2)**, not NF1 [1]. - These tumors arise from the Schwann cells of the **vestibulocochlear nerve** and can cause hearing loss and balance issues [1]. *Low grade astrocytoma* - While optic nerve gliomas are a type of low-grade astrocytoma, this option is too general; **optic nerve glioma** is the specific and most common presentation in NF1. - Other forms of low-grade astrocytomas can occur in NF1 but are not as universally characteristic as optic nerve gliomas.
Question 35: Which of the following glands is NOT typically involved in Multiple Endocrine Neoplasia type II A (MEN II A)?
- A. Pituitary gland (Correct Answer)
- B. Thyroid gland
- C. Parathyroid gland
- D. Adrenal gland
Explanation: ***Pituitary gland*** - The **pituitary gland** is not a characteristic component of **MEN II A**. It is, however, associated with **Multiple Endocrine Neoplasia type I (MEN I)**, which involves the 3 Ps: **pituitary**, **parathyroid**, and **pancreas** [1]. - **MEN IIA** classically involves **medullary thyroid carcinoma**, **pheochromocytoma**, and **parathyroid hyperplasia** [1]. *Thyroid gland* - The **thyroid gland** is centrally involved in MEN IIA, specifically through the development of **medullary thyroid carcinoma (MTC)**, a hallmark feature. - MTC arises from the parafollicular C cells of the thyroid and secretes **calcitonin**. *Parathyroid gland* - The **parathyroid gland** is often involved in MEN IIA, typically presenting as **parathyroid hyperplasia** or adenoma, leading to **primary hyperparathyroidism**. - This typically results in elevated **parathyroid hormone** levels and **hypercalcemia**. *Adrenal gland* - The **adrenal gland** is a key player in MEN IIA due to the occurrence of **pheochromocytoma**, a tumor of the adrenal medulla. - Pheochromocytomas can be bilateral and secrete **catecholamines**, leading to hypertension and other symptoms.
Question 36: Which of the following is NOT a feature of Peutz-Jeghers syndrome?
- A. Mucocutaneous pigmentation
- B. Autosomal recessive inheritance (Correct Answer)
- C. Autosomal dominant
- D. Hamartomatous polyp
Explanation: ***High risk of malignancy*** - Peutz-Jeghers syndrome is primarily associated with **benign hamartomatous polyps**, not a **high risk of malignancy**, which distinguishes it from other syndromes. - Although patients may develop cancers [1], the syndrome itself does not inherently denote a high malignancy risk like other syndromes such as familial adenomatous polyposis. *Autosomal dominant* - This syndrome is indeed **autosomal dominant**, caused by mutations in the STK11 gene. - Families with this condition typically show **vertical transmission**, characteristic of autosomal dominant inheritance. *Hamartomatous polyp* - Individuals with Peutz-Jeghers syndrome develop **hamartomatous polyps**, which are a hallmark feature of the condition [1]. - These polyps can occur in the gastrointestinal tract and are benign lesions rather than adenomatous type seen in other syndromes [1]. *Mucocutaneous pigmentation* - Mucocutaneous pigmentation, such as **freckling around the lips and buccal mucosa**, is a key clinical feature of Peutz-Jeghers syndrome. - This pigmentation usually appears in childhood and is often a distinguishing sign of the syndrome.
Question 37: What is the most common location of gastrinoma?
- A. Pancreas
- B. Duodenum (Correct Answer)
- C. Jejunum
- D. Gall bladder
Explanation: ***Duodenum*** - The **duodenum** is the most common site for gastrinomas, accounting for over **half of all cases**, particularly in sporadic gastrinoma and Zollinger-Ellison syndrome. - These tumors are often **small** and **multiple** in the duodenum, making them challenging to locate. *Pancreas* - Pancreatic gastrinomas are also common, representing approximately **25-40% of cases**, but are less frequent than duodenal gastrinomas. - Pancreatic gastrinomas tend to be **larger** and more amenable to surgical resection when compared to duodenal gastrinomas. *Jejunum* - Gastrinomas found in the jejunum are **rare**, accounting for only a small percentage of cases. - The small intestine distal to the duodenum is an **uncommon site** for primary gastrinoma formation. *Gall bladder* - The **gallbladder** is not a typical location for gastrinoma development. - Gastrinomas are neuroendocrine tumors that arise from **gastrin-producing cells**, which are not found in the gallbladder.
Question 38: Which of the following statements about Gilbert syndrome is false?
- A. Normal liver histology
- B. Autosomal dominant
- C. Elevated bilirubin levels are present
- D. Causes cirrhosis (Correct Answer)
Explanation: ***Causes cirrhosis*** - **Gilbert syndrome** is a benign condition characterized by intermittent unconjugated hyperbilirubinemia and does **not lead to cirrhosis** [1]. - Cirrhosis is a severe form of **liver scarring** resulting from chronic damage, which is not a feature of Gilbert syndrome. *Normal liver histology* - The liver structure and function in individuals with Gilbert syndrome are typically **normal**, distinguishing it from other liver disorders [2]. - Histological examination of liver biopsies usually reveals no abnormalities, reflecting the **benign nature** of the condition. *Autosomal dominant* - Gilbert syndrome is inherited in an **autosomal recessive** pattern, not autosomal dominant [2]. - It results from a reduction in the activity of the **UGT1A1 enzyme**, which is responsible for bilirubin conjugation [1], [2]. *Elevated bilirubin levels are present* - Individuals with Gilbert syndrome experience **intermittent unconjugated hyperbilirubinemia**, meaning their indirect bilirubin levels are elevated [3]. - This elevation is usually mild and can be exacerbated by stress, fasting, or illness, but it is typically **harmless** [1], [2].
Question 39: Which of the following statements about polio is false?
- A. 99% non paralytic
- B. Aseptic meningitis
- C. Flaccid paralysis
- D. Increased tendon reflexes (Correct Answer)
Explanation: ***Increased tendon reflexes*** - Polio causes **lower motor neuron damage**, specifically to the anterior horn cells of the spinal cord [1]. - This damage leads to **flaccid paralysis** and **decreased or absent deep tendon reflexes**, not increased reflexes [3]. *99% non paralytic* - The vast majority of poliovirus infections (approximately 95-99%) are **asymptomatic** or cause only mild, non-specific symptoms. - Only a small percentage of infected individuals develop the more severe paralytic form of the disease. *Flaccid paralysis* - Poliovirus directly attacks and destroys **motor neurons** in the anterior horn of the spinal cord [1]. - This damage results in **muscle weakness** and loss of muscle tone, leading to **flaccid paralysis** [3]. *Aseptic meningitis* - About 1-5% of poliovirus infections can manifest as **aseptic meningitis**, characterized by symptoms like fever, headache, neck stiffness, and vomiting without bacterial infection [2]. - This form of meningitis is typically **self-limiting** and does not lead to paralysis [2].
Question 40: Which of the following is true about Hepatitis A virus?
- A. Causes chronic hepatitis
- B. Helps HDV replication
- C. Causes cirrhosis
- D. Common cause of hepatitis in children (Correct Answer)
Explanation: ***Common cause of hepatitis in children*** - **Hepatitis A virus (HAV)** infection is often acquired in childhood, particularly in areas with poor sanitation, and many infections are **asymptomatic** or mild in children [1]. - Due to their developing immune systems and often exposure in daycare or school settings, children are a highly susceptible population for HAV transmission [1]. *Causes cirrhosis* - **HAV infection** is an **acute self-limiting illness** and typically does not lead to chronic liver disease or cirrhosis [1]. - **Cirrhosis** is primarily associated with chronic viral hepatitis (e.g., HBV, HCV), alcohol-related liver disease, or certain autoimmune conditions. *Helps HDV replication* - **Hepatitis D virus (HDV)** is a **defective virus** that requires the presence of **Hepatitis B virus (HBV)** surface antigen (HBsAg) for its replication and assembly [1]. - **HAV** has no role in the replication or pathogenesis of **HDV** [1]. *Causes chronic hepatitis* - **HAV infection** results in an **acute inflammatory response** in the liver that resolves spontaneously in most cases [1]. - Unlike **HBV** and **HCV**, **HAV** does not establish a persistent infection and, therefore, does not cause chronic hepatitis [1].