Which of the following statements about Gilbert syndrome is false?
Which one of the following is the most common CNS tumor associated with type I neurofibromatosis?
What is the most appropriate initial management for paralysis resulting from organophosphorus poisoning?
Type 3 respiratory failure occurs due to ?
Cerebellar damage causes all of the following except?
Thalassemia gives protection against ?
Primary hypercholesterolemia is:
Which of the following statements about hypercalcemia in sarcoidosis is false?
Most common cause of death in SLE in children
Response to iron therapy in iron deficiency anemia is denoted by?
NEET-PG 2013 - Internal Medicine NEET-PG Practice Questions and MCQs
Question 21: 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 22: 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 23: What is the most appropriate initial management for paralysis resulting from organophosphorus poisoning?
- A. Supportive care, including respiratory support (Correct Answer)
- B. Atropine to counteract muscarinic symptoms
- C. Oximes to reactivate acetylcholinesterase
- D. No specific antidote
Explanation: **Supportive care, including respiratory support** * **Paralysis** in organophosphorus poisoning (OPP) is often due to **nicotinic effects** at the neuromuscular junction, leading to respiratory muscle weakness and failure [2]. * **Respiratory support** through mechanical ventilation is crucial to maintain oxygenation and prevent complications while awaiting the effects of antidotal therapy [1], [2]. * *Atropine to counteract muscarinic symptoms* * **Atropine** primarily blocks **muscarinic receptors**, effectively treating symptoms like bradycardia, bronchorrhea, and miosis [2]. * It does **not reverse the nicotinic effects** responsible for muscle paralysis and respiratory failure. * *Oximes to reactivate acetylcholinesterase* * **Oximes (e.g., pralidoxime)** reactivate **acetylcholinesterase**, thereby addressing the underlying cause of acetylcholine accumulation [2]. * They are most effective if given **early** before irreversible aging of the enzyme occurs, but their effect on established paralysis can be limited without concurrent respiratory support [2]. * *No specific antidote* * This statement is incorrect; **atropine** and **oximes** are specific antidotes for organophosphorus poisoning [2]. * While these antidotes are vital, initial management prioritizing **airway and breathing support** is paramount due to the life-threatening respiratory paralysis [1].
Question 24: Type 3 respiratory failure occurs due to ?
- A. Post-operative atelectasis (Correct Answer)
- B. Kyphoscoliosis
- C. Flail chest
- D. Pulmonary fibrosis
Explanation: ***Post-operative atelectasis*** - **Type 3 respiratory failure**, also known as **perioperative respiratory failure**, is characterized by hypoxemia occurring typically after surgery. - **Atelectasis**, the collapse of lung tissue, is a common cause of hypoxemia in the post-operative period due to shallow breathing, pain, and anesthesia affecting lung volumes. *Kyphoscoliosis* - This condition leads to a **restrictive lung disease** due to chest wall deformity, causing chronic respiratory failure. [1] - It more typically results in **Type 2 respiratory failure** (hypercapnic) due to impaired ventilation over time. [1] *Flail chest* - Flail chest is a severe chest wall injury causing paradoxical movement, leading to **acute respiratory failure**. - It is often associated with **Type 1 (hypoxemic)** or **Type 2 (hypercapnic)** respiratory failure due to trauma-induced lung injury and impaired mechanics. *Pulmonary fibrosis* - This is a progressive interstitial lung disease causing **restrictive ventilatory defect** and impaired gas exchange. - It leads to chronic **Type 1 respiratory failure** (hypoxemic) as the lung tissue becomes stiff and scarred.
Question 25: Cerebellar damage causes all of the following except?
- A. Ataxia
- B. Past-pointing
- C. Dysmetria
- D. Hypertonia (Correct Answer)
Explanation: ***Hypertonia*** - Cerebellar lesions typically lead to **hypotonia**, a decrease in muscle tone, rather than hypertonia [1]. - Hypertonia, or increased muscle tone, is more commonly associated with lesions of the **upper motor neurons** or **basal ganglia** [2]. *Dysmetria* - **Dysmetria** is a common sign of cerebellar damage, characterized by an inability to accurately control the **range, direction, and force** of muscle movements [1]. - This leads to overshooting or undershooting a target during voluntary movements. *Ataxia* - **Ataxia**, particularly truncal or appendicular ataxia, is a cardinal symptom of cerebellar dysfunction [3]. - It refers to a lack of **voluntary coordination** of muscle movements, leading to an unsteady gait and impaired balance [3]. *Past-pointing* - **Past-pointing** is a form of dysmetria where a patient consistently points or reaches **beyond their target** [1]. - It is a specific sign that indicates a deficit in the cerebellum's ability to modulate and refine motor commands.
Question 26: Thalassemia gives protection against ?
- A. Protection against filaria
- B. Protection against kala-azar
- C. Protection against leptospirosis
- D. Protection against malaria (Correct Answer)
Explanation: Protection against malaria - Individuals with thalassemia, particularly thalassemia trait, have some degree of protection against severe forms of malaria, specifically Plasmodium falciparum [1]. - The altered red blood cell structure and reduced hemoglobin content in thalassemia make the red blood cells less hospitable for the parasites, hindering their replication and survival [1]. Protection against filaria - Filaria is caused by parasitic worms (nematodes) transmitted by mosquitoes, leading to lymphatic filariasis (elephantiasis) or onchocerciasis (river blindness). - Thalassemia's primary impact is on red blood cell health and oxygen transport, offering no known protective effect against nematode infections or their associated pathology. Protection against kala-azar - Kala-azar (visceral leishmaniasis) is caused by Leishmania parasites transmitted by sandflies, primarily affecting the reticuloendothelial system (spleen, liver, bone marrow). - There is no established scientific evidence indicating that thalassemia provides protection against Leishmania infections or their clinical manifestations. Protection against leptospirosis - Leptospirosis is a bacterial infection caused by Leptospira bacteria, typically acquired through contact with contaminated water or animal urine. - Thalassemia is a genetic blood disorder; its physiological effects are unrelated to the mechanisms of infection or immunity against bacterial pathogens like Leptospira.
Question 27: Primary hypercholesterolemia is:
- A. Type I
- B. Type IIb
- C. Type IIa (Correct Answer)
- D. Type III
Explanation: ***Type Ha*** - **Primary hypercholesterolemia** specifically refers to **Familial Hypercholesterolemia**, which is classified as Type Ha due to a genetic defect affecting LDL receptor activity [1]. - It typically presents with **high cholesterol levels** and an increased risk of premature cardiovascular disease [1]. *Type I* - Type I hyperlipoproteinemia is associated with **chylomicronemia**, leading to elevated triglycerides rather than cholesterol. - Symptoms include **pancreatitis** and eruptive xanthomas, not primarily high cholesterol levels. *Type III* - Type III hyperlipoproteinemia is known as **Dysbetalipoproteinemia**, associated with **increased IDL** and can cause elevated cholesterol, but is not classified as primary hypercholesterolemia. - It typically presents with **tuberous xanthomas** and is linked to **apolipoprotein E deficiency**. *Type IIb* - Type IIb hyperlipoproteinemia involves **elevation of LDL and VLDL**, but it is not classified as primary hypercholesterolemia; it is a mixed dyslipidemia. - This type usually features **increased cholesterol** and **triglycerides**, distinguishing it from the familial form classified as Type Ha.
Question 28: 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 29: Most common cause of death in SLE in children
- A. Libman sacks endocarditis
- B. Lupus cerebritis
- C. Lupus nephritis
- D. Anemia and infections (Correct Answer)
Explanation: ***Anemia and infections*** - **Infections** are a leading cause of death in pediatric SLE patients, often due to immunosuppression from the disease itself or its treatment. Although pediatric Systemic Lupus Erythematosus (SLE) is not a primary immune deficiency, the susceptibility to encapsulated bacteria and recurrent infections seen in primary B- and T-lymphocyte deficiencies mirrors the infection risks managed in these patients [1]. - **Anemia** can contribute to overall morbidity and mortality, although it is less directly a cause of death than severe infections or organ failure. *Lupus nephritis* - While **lupus nephritis** is a common and severe manifestation of SLE in children and a major cause of morbidity, particularly long-term kidney failure, it is not the most frequent immediate cause of death. - Advancements in treatment for nephritis have improved prognosis, shifting the leading cause of mortality to other factors. *Lupus cerebritis* - **Lupus cerebritis** (neuropsychiatric SLE) can be life-threatening, causing seizures, stroke, or psychosis, but it is less common as the primary cause of death compared to infections. - Its presence usually indicates severe disease requiring intensive treatment, but not the most common direct cause of death. *Libman sacks endocarditis* - **Libman-Sacks endocarditis** involves sterile vegetations on heart valves and is a known complication of SLE, but it rarely causes acute mortality in children. - It is more often associated with chronic complications like valvular dysfunction or a source of emboli rather than being the most common cause of death.
Question 30: Response to iron therapy in iron deficiency anemia is denoted by?
- A. Increase in hemoglobin
- B. Reticulocytosis (Correct Answer)
- C. Restoration of enzymes
- D. Increase in iron binding capacity
Explanation: Reticulocytosis - Reticulocytosis is one of the earliest signs of a positive response to iron therapy in iron deficiency anemia, occurring within 5-10 days. - It signifies that the bone marrow is effectively producing new red blood cells after iron supplementation. Restoration of enzymes - While iron is a crucial component of many enzymes (e.g., catalase, cytochrome oxidase), its restoration takes time and is not the primary immediate indicator of therapeutic response. - Clinical improvement and other hematological parameters precede the full restoration of enzyme function. Increase in hemoglobin - An increase in hemoglobin is a definitive sign of successful treatment, but it occurs later than reticulocytosis, typically visible after several weeks to months of therapy. - Hemoglobin levels rise as the new, iron-sufficient red blood cells fully mature and replace the older, iron-deficient ones. Increase in iron binding capacity - In iron deficiency anemia, total iron-binding capacity (TIBC) is typically increased due to more transferrin being available to bind iron [1]. - Successful iron therapy would lead to a decrease in TIBC as transferrin sites become saturated with iron, not an increase.