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: 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: 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: A 32-year-old patient presents with blurred vision, photophobia, and mild ocular pain. Examination reveals aqueous flares and keratic precipitates in the anterior chamber. What is the likely diagnosis?

D. Iridocyclitis. ***D. Iridocyclitis***- **Iridocyclitis** is the preferred term for **anterior uveitis**, which involves inflammation of the iris and ciliary body.- The hallmark signs of **aqueous flares** (protein and cells in the anterior chamber) and **keratic precipitates** (inflammatory cell deposits on the corneal endothelium) confirm that the inflammation is localized to the **anterior chamber**.*A. Intermediate uveitis*- Characterized by inflammation primarily in the **vitreous** and **pars plana**, often referred to as *vitritis*.- While it causes blurred vision and floaters, the anterior chamber signs like *aqueous flares* and *keratic precipitates* are not the dominant features; fundoscopy may reveal **snowbanking**.*B. Posterior uveitis*- Involves inflammation of the **retina** and/or **choroid** (e.g., retinitis or choroiditis), often causing significant central vision loss and scotomas.- The primary findings are located in the posterior segment (fundus lesions), and the anterior chamber is often **quiet** or shows only mild reaction.*C. Toxoplasma uveitis*- This is a specific cause of **posterior uveitis**, typically presenting as a focus of **necrotizing retinitis** adjacent to an old, pigmented scar.- Although it can cause a *secondary* anterior chamber reaction, the primary diagnostic feature is the specific retinal lesion, which is not mentioned in this presentation.

Q: Optic nerve glioma is seen in?

A. NF-1. ***NF-1***- **Optic pathway gliomas (OPGs)** are hallmark features of **Neurofibromatosis type 1 (NF-1)**, an autosomal dominant disorder caused by a mutation in the *NF1* gene on chromosome 17.- OPGs are typically low-grade **pilocytic astrocytomas** and commonly present in children under 6 years of age. *Tuberous sclerosis*- The most characteristic CNS tumor in patients with **Tuberous Sclerosis Complex (TSC)** is the **subependymal giant cell astrocytoma (SEGA)**, usually near the foramen of Monro.- TSC is associated with **retinal astrocytic hamartomas** (phakomas), not primary optic nerve gliomas. *NF-2*- **Neurofibromatosis type 2 (NF-2)** is characterized by **bilateral vestibular schwannomas** (acoustic neuromas), which is the diagnostic criterion.- Other common tumors in NF-2 include meningiomas, ependymomas, and other cranial or spinal schwannomas, but rarely **optic nerve gliomas**. *Schwannoma*- A **schwannoma** is a tumor arising from Schwann cells, often seen on peripheral nerves or cranial nerves (like CN VIII in NF-2).- The optic nerve is considered part of the CNS (a tract of the diencephalon) and is myelinated by oligodendrocytes, meaning **schwannomas** are not primary tumors of the optic nerve itself.

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]

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.

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

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 3

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 4

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 5

A 32-year-old patient presents with blurred vision, photophobia, and mild ocular pain. Examination reveals aqueous flares and keratic precipitates in the anterior chamber. What is the likely diagnosis?

Accepted Answer

D. Iridocyclitis

Answer

A. Intermediate uveitis

Answer

B. Posterior uveitis

Answer

C. Toxoplasma uveitis

Question 6

Optic nerve glioma is seen in?

Accepted Answer

A. NF-1

Answer

B. Tuberous sclerosis

Answer

C. NF-2

Answer

D. Schwannoma

Question 7

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 8

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 9

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

Question 10

Which among the following hormones acts on post ovulatory endometrium?

Accepted Answer

Progesterone

Answer

Follicle stimulating hormone

Answer

Luteinizing hormone

Answer

Oestrogen

FMGE 2024

Biochemistry

Obstetrics and Gynecology

Ophthalmology

Pathology

Pediatrics

Pharmacology

Physiology