FMGE 2024 Practice Questions

Q: A 6-month-old boy presents with recurrent bacterial and fungal infections, chronic diarrhea, and failure to thrive. He is diagnosed with severe combined immunodeficiency due to an autosomal recessive inheritance pattern. Which enzyme deficiency is responsible?

D. Adenosine deaminase. ***Adenosine deaminase*** - The **autosomal recessive** form of **Severe Combined Immunodeficiency (SCID)** is most commonly caused by a deficiency in **Adenosine Deaminase (ADA)**, accounting for about 15% of all SCID cases. - ADA deficiency leads to the accumulation of toxic metabolites (*dATP*), which are highly toxic to rapidly dividing cells, especially **T and B lymphocytes**, resulting in profound lymphopenia and immunodeficiency. *Phosphomannose isomerase* - Deficiency in **Phosphomannose Isomerase (PMI)** causes Congenital Disorder of Glycosylation Type Ib (**CDG-Ib**), which presents with protein-losing enteropathy, hypoglycemia, and failure to thrive, but usually *not* recurrent bacterial and fungal infections severe enough to be classified as SCID. - CDG-Ib is a generalized metabolic disorder affecting **glycosylation**, primarily presenting with liver and gastrointestinal issues. *Ornithine transcarbamylase* - **Ornithine Transcarbamylase (OTC)** deficiency is the most common urea cycle disorder, typically presenting with acute **hyperammonemia** (lethargy, seizures, coma) after an initial period, especially following protein intake, not specifically severe SCID with recurrent infections. - OTC deficiency results in the impaired conversion of **carbamoyl phosphate** and **ornithine** to citrulline, leading to elevated ammonia levels. *Hypoxanthine-guanine phosphoribosyltransferase* - Deficiency in **Hypoxanthine-Guanine Phosphoribosyltransferase (HGPRT)** is responsible for **Lesch-Nyhan Syndrome**, an X-linked recessive disorder characterized by overproduction of **uric acid** (**hyperuricemia**), neurological dysfunction, and self-mutilation. - Although a purine metabolism disorder, it does not cause primary immunodeficiency like SCID; it mainly affects the **nervous system** and purine salvage pathway.

Q: A family died in a closed room that was full of smoke from a wood fire. Which of the following findings is likely to be seen on the body?

A. Cherry red hypostasis. ***A. Cherry red hypostasis*** - Carbon monoxide (CO) from incomplete combustion of wood binds to hemoglobin with ~240 times greater affinity than oxygen, forming **carboxyhemoglobin (COHb)** - COHb has a characteristic **bright cherry-red or pink color**, which manifests as cherry-red hypostasis (post-mortem lividity) and pink coloration of internal organs - This is the **pathognomonic post-mortem finding** in CO poisoning deaths - Levels >50% COHb are typically fatal *B. Cyanosis* - Cyanosis (bluish discoloration) occurs when deoxygenated hemoglobin exceeds 5 g/dL - **Not seen in CO poisoning** because carboxyhemoglobin remains bright red, giving a pink appearance rather than blue - The victim may appear "healthy" or flushed despite being dead *C. Blackish discoloration* - Not a characteristic finding in CO poisoning - May be seen in putrefaction or certain chemical poisonings, but not acute CO exposure *D. Brown colored pigmentation* - Not associated with CO poisoning - Brown discoloration might suggest methemoglobinemia or post-mortem changes, but not CO toxicity

Q: A 27-year-old pregnant female in her first trimester presents to the OPD for a regular antenatal checkup. During blood type screening, potential ABO incompatibility is discussed. The healthcare provider explains that certain antibody types are less concerning than others during pregnancy. ABO incompatibility does not occur due to which antibody in her case?

IgM. ***IgM***- The predominant natural antibodies against **ABO antigens** (anti-A and anti-B) are of the **IgM class**, which are large pentameric molecules. These **IgM antibodies** generally cannot cross the placenta due to their size, meaning they do not reach the fetal circulation and cause significant hemolytic disease of the newborn (HDN). *IgA*- IgA is predominantly found in secretions (mucous membranes, breast milk) and is not generally involved in causing **hemolytic disease of the newborn (HDN)**, as it does not cross the placenta in significant amounts. This antibody class is not the primary mechanism of incompatibility, as the most common non-transmissible antibodies are IgM. *IgG*- **IgG is the only class** of immunoglobulin that efficiently crosses the placenta into the fetal circulation, meaning that any present **IgG anti-A or anti-B antibodies** are the ones responsible for causing **fetal red cell hemolysis** in ABO incompatibility. Though ABO HDN is usually less severe than Rh HDN, the pathology depends entirely on the presence of IgG. *IgD*- IgD antibodies are primarily expressed on the surface of naïve **B lymphocytes** and are involved in B cell activation and signaling. They are not involved in **red blood cell agglutination** or placental transfer relevant to ABO incompatibility.

Q: Identify the option with the least risk of TOLAC (trial of labor after cesarean)?

Low-segment transverse incision. ***Low-segment transverse incision*** - This type of uterine incision is preferred during a Cesarean section as it is made in the least active segment, carrying the **lowest risk** of **uterine rupture** (approximately 0.5% to 0.9%) during a subsequent trial of labor. - It is generally considered the standard requirement for safely proceeding with a **VBAC** (Vaginal Birth After Cesarean) attempt. *Classical C section* - A **classical C-section** involves a vertical incision in the contractile upper segment (**uterine fundus**), which has the highest risk of **uterine rupture** (4% to 9%) during labor. - A history of a classical incision is generally considered an absolute **contraindication** to TOLAC. *Pre-eclampsia* - The presence of **pre-eclampsia** increases the risk of adverse outcomes to both mother and fetus, such as **placental abruption** and **intrauterine growth restriction**. - While not an absolute contraindication, it complicates management and often necessitates induction or delivery, placing it at a higher risk level compared to an uncomplicated TOLAC attempt. *Breech presentation* - **Breech presentation** is itself a risk factor for difficult vaginal delivery in nulliparous women, and combining it with a prior Cesarean scar (TOLAC) elevates the overall obstetric risk. - Many practitioners consider **breech presentation** in the current pregnancy a relative contraindication to TOLAC, favoring a planned repeat Cesarean delivery due to increased risk of complications.

Q: Identify the gynecological instrument shown in the image below:

Cusco vaginal speculum. ***Cusco vaginal speculum*** - In the given image, the instrument shows the characteristic **bivalve design** with two curved blades that can be opened and closed using a **screw mechanism** visible at the handle, which is the defining feature of a Cusco speculum. - The **self-retaining mechanism** and **smooth, curved blades** designed for routine gynecological examinations like **Pap smears** are clearly visible, distinguishing it from other speculums. *Graves vaginal speculum* - The Graves speculum has **wider, more angled blades** that contour to the vaginal fornices, which is not seen in the image. - It typically has a **different handle configuration** and blade curvature compared to what's shown in the instrument. *Auvard speculum* - This is a **weighted speculum** with a distinctly different design featuring a **heavy posterior blade** for retraction during procedures like **D&C**. - The instrument in the image lacks the characteristic **weighted design** and **single posterior blade** typical of an Auvard speculum. *Sims speculum* - The Sims speculum is **non-self-retaining** and has a **double-ended, curved design** that requires manual holding or assistance. - Unlike the instrument shown, it lacks a **screw mechanism** and has a completely different **curved, hook-like shape**.

Q: Which among the following hormones acts on post ovulatory endometrium?

Progesterone. ***Progesterone*** - It is predominantly secreted by the **corpus luteum** during the post-ovulatory phase, inducing the crucial changes of the **secretory endometrium** to facilitate implantation.- Progesterone causes the endometrial glands to become highly **coiled** and secretory, leading to the development of **spiral arteries** and preparing the uterine lining for a fertilized ovum.*Luteinizing hormone* - LH's main role is triggering **ovulation** via the mid-cycle surge and maintaining the function of the **corpus luteum** post-ovulation.- Its primary targets are ovarian cells (theca and corpus luteum), not the direct transformation of the post-ovulatory endometrial structure.*Follicular stimulating hormone* - FSH functions primarily during the preceding **follicular phase**, stimulating the growth of ovarian follicles and inducing **estrogen** synthesis.- Its levels decrease significantly after ovulation, and it has no direct, major trophic effect on the secretory endometrium.*Oestrogen* - **Oestrogen** is the primary hormone responsible for the **proliferative phase** (pre-ovulatory), causing endometrial thickening and repair.- While necessary for endometrial primedness, Oestrogen is superseded by **Progesterone** in dictating the specific glandular and vascular characteristics of the post-ovulatory secretory phase.

Q: Identify the condition that is least likely to cause postmenopausal bleeding?

Ovarian follicular cyst. ***Ovarian follicular cyst*** * **Follicular cysts** result from failed ovulation and require active high **FSH** stimulation, making them generally rare or transient findings in **postmenopausal** women due to ovarian senescence. * Unlike other estrogen-producing tumors, simple follicular cysts usually do not produce sufficient sustained **estrogen** levels to pathologically stimulate the endometrium and cause bleeding in the postmenopausal period. *Endometrial CA* * **Endometrial carcinoma** is the most common cause of postmenopausal bleeding, accounting for 10-15% of cases, and must be ruled out in every patient presenting with this symptom. * Bleeding results from the erosion, ulceration, and breakdown of the friable, neoplastic tissue lining the **endometrium**. *Granulosa cell tumor* * This is a classic example of an **estrogen-producing ovarian tumor** (a sex cord-stromal tumor). * The chronic, unopposed **estrogen** stimulation causes proliferation of the endometrium, leading to subsequent **endometrial hyperplasia** or cancer, resulting in bleeding. *Genital tract trauma* * Trauma, including minor injuries, is a significant cause of postmenopausal bleeding due to underlying **vaginal and cervical atrophy**. * Postmenopausal tissue is thin, lacks pliancy, and is fragile, making it susceptible to bleeding even from minor trauma during examination, intercourse, or other physical contact.

Q: An 8-year-old child with a history of GTCS came with an episode of convulsions for more than 45 minutes. What will be the appropriate management for this patient?

Lorazepam followed by levetiracetam. ***Lorazepam followed by levetiracetam***- **Status epilepticus (SE)** is defined as a seizure lasting more than five minutes or recurrent seizures without regaining consciousness between them. The initial management involves the administration of a **benzodiazepine** (like lorazepam, midazolam, or diazepam) to rapidly terminate the seizure.- If the seizure persists after the benzodiazepine, the next step is to initiate a non-benzodiazepine antiseizure medication (ASM) like **levetiracetam**, **fosphenytoin**, or **valproate** to prevent seizure recurrence.*Valproate followed by gabapentin*- **Valproate** is a suitable second-line agent for SE, but it should not be the first drug; rapid control requires a **benzodiazepine**.- **Gabapentin** is typically not used in the management of acute SE because its onset of action is slow and it lacks efficacy for immediate seizure termination.*Carbamazepine followed by lorazepam*- **Carbamazepine** is a first-line agent for focal seizures but is generally avoided in generalized-onset seizures (like the **GTCS** history suggests) and is not used as a first-line drug for acute SE.- **Lorazepam** is the preferred first-line agent, and delaying it until after a non-benzodiazepine drug is inappropriate for acute SE management.*Levetiracetam followed by valproate*- **Levetiracetam** is an excellent second-line agent for SE but must be preceded by a **benzodiazepine** to rapidly terminate the ongoing seizure.- **Valproate** is also a suitable second-line agent, but the protocol requires immediate cessation of the seizure with a **benzodiazepine** before initiating an ASM like levetiracetam or valproate.

Q: A patient having hypertension is on thiazides and is complaining of fatigue and hypokalemia. Which of the following drugs can prevent potassium loss?

Amiloride. ***Amiloride***- Amiloride is a **potassium-sparing diuretic** that acts by blocking the **Epithelial Sodium Channel (ENaC)** in the collecting duct. [5]- By inhibiting Na+ reabsorption here, it decreases the electrical gradient that drives **potassium secretion**, effectively counteracting the hypokalemic effect of thiazides. [2]*Furosemide*- Furosemide is a **loop diuretic** that inhibits the Na-K-2Cl cotransporter in the thick ascending limb.- It is notorious for causing significant urinary **potassium loss** and would worsen the patient's **hypokalemia** and fatigue. [5]*Acetazolamide*- Acetazolamide is a **carbonic anhydrase inhibitor** that increases the delivery of Na+ and bicarbonate (HCO3-) to the collecting duct. [4]- The increased delivery of these ions promotes the secretion and loss of **potassium** in the urine, thus failing to prevent hypokalemia. [4]*Indapamide*- Indapamide is a **thiazide-like diuretic** that, similar to thiazides, inhibits the NaCl cotransporter in the distal convoluted tubule. [1]- Like other thiazides, Indapamide is a **potassium-wasting diuretic** and would exacerbate the existing **hypokalemia**. [3]

Question 1

A 6-month-old boy presents with recurrent bacterial and fungal infections, chronic diarrhea, and failure to thrive. He is diagnosed with severe combined immunodeficiency due to an autosomal recessive inheritance pattern. Which enzyme deficiency is responsible?

Accepted Answer

D. Adenosine deaminase

Answer

B. Ornithine transcarbamylase

Answer

C. Hypoxanthine-guanine phosphoribosyltransferase

Answer

A. Phosphomannose isomerase

Question 2

A family died in a closed room that was full of smoke from a wood fire. Which of the following findings is likely to be seen on the body?

Accepted Answer

A. Cherry red hypostasis

Answer

D. Brown colored pigmentation

Answer

B. Cyanosis

Answer

C. Blackish discoloration

Question 3

A 27-year-old pregnant female in her first trimester presents to the OPD for a regular antenatal checkup. During blood type screening, potential ABO incompatibility is discussed. The healthcare provider explains that certain antibody types are less concerning than others during pregnancy. ABO incompatibility does not occur due to which antibody in her case?

Accepted Answer

IgM

Answer

IgG

Answer

IgD

Answer

IgA

Question 4

Identify the option with the least risk of TOLAC (trial of labor after cesarean)?

Accepted Answer

Low-segment transverse incision

Answer

Breech presentation

Answer

Pre-eclampsia

Answer

Classical C section

Question 5

Identify the gynecological instrument shown in the image below:

Accepted Answer

Cusco vaginal speculum

Answer

Graves vaginal speculum

Answer

Auvard speculum

Answer

Sims speculum

Question 6

Which among the following hormones acts on post ovulatory endometrium?

Accepted Answer

Progesterone

Answer

Follicle stimulating hormone

Answer

Luteinizing hormone

Answer

Oestrogen

Question 7

Identify the condition that is least likely to cause postmenopausal bleeding?

Accepted Answer

Ovarian follicular cyst

Answer

Genital tract trauma

Answer

Endometrial CA

Answer

Granulosa cell tumor

Question 8

A 10-year-old boy is brought to the ED after being stung by a bee while playing outside. Within minutes of the sting, he developed shock, respiratory failure, and vascular collapse. What type of hypersensitivity reaction is most likely responsible?

Accepted Answer

A. IgE-mediated reaction

Answer

D. T cell-mediated response

Answer

B. IgG-mediated reaction

Answer

C. IgA-mediated hypersensitivity

Question 9

An 8-year-old child with a history of GTCS came with an episode of convulsions for more than 45 minutes. What will be the appropriate management for this patient?

Accepted Answer

Lorazepam followed by levetiracetam

Answer

Levetiracetam followed by valproate

Answer

Valproate followed by gabapentin

Answer

Carbamazepine followed by lorazepam

Question 10

A patient having hypertension is on thiazides and is complaining of fatigue and hypokalemia. Which of the following drugs can prevent potassium loss?

Accepted Answer

Amiloride

Answer

Acetazolamide

Answer

Indapamide

Answer

Furosemide

FMGE 2024

Biochemistry

Forensic Medicine

Obstetrics and Gynecology

Pathology

Pediatrics

Pharmacology