Which of the following is not true in Turner Syndrome?
Which of the following vaccines is contraindicated in a patient of Severe Combined Immunodeficiency (SCID)?
Which of the following diagnoses can cause stridor in infants? 1. Laryngomalacia 2. Congenital hemangioma 3. Subglottic stenosis 4. Recurrent respiratory papillomatosis (RRP)
A 7-year-old child with a history of ear infection is complaining of lockjaw and limb spasm/fever/neck rigidity. Choose the most appropriate diagnosis:
INI-CET 2025 - Pediatrics INI-CET Practice Questions and MCQs
Question 11: Which of the following is not true in Turner Syndrome?
- A. Short 4th metacarpals
- B. Webbed neck
- C. Widely spaced hypoplastic nipples
- D. Prominent occiput (Correct Answer)
Explanation: ***Prominent occiput*** - This physical feature is **NOT** associated with Turner Syndrome and is therefore the correct answer. - A prominent occiput is a classic distinguishing feature of **Trisomy 18 (Edwards Syndrome)**, not Turner syndrome. - Turner syndrome patients typically have normal occiput configuration, helping differentiate it from other chromosomal aneuploidies. *Incorrect - Short 4th metacarpals* - This is actually a **classic feature** of Turner Syndrome, known as the **metacarpal sign**. - Short 4th and 5th metacarpals are well-documented skeletal abnormalities in Turner syndrome. - On X-ray, extending a line along the 4th and 5th metacarpal heads normally passes distal to the 3rd metacarpal head, but in Turner syndrome it passes through or proximal to it. *Incorrect - Webbed neck* - This is a highly characteristic feature of Turner Syndrome, clinically known as **pterygium colli**. - Results from regression of fetal cystic hygromas (lymphatic malformations). - Strongly associated with the **45,X karyotype** and is a significant phenotypic marker. *Incorrect - Widely spaced hypoplastic nipples* - This describes the **shield chest** appearance, a key physical characteristic of Turner syndrome. - Part of the constellation of congenital developmental anomalies including broad chest with increased internipple distance. - Directly linked to the underlying chromosomal abnormality and developmental changes.
Question 12: Which of the following vaccines is contraindicated in a patient of Severe Combined Immunodeficiency (SCID)?
- A. IPV
- B. DPT
- C. Hepatitis B
- D. MMR (Correct Answer)
Explanation: ***MMR*** - **MMR (Measles, Mumps, Rubella)** is a **live attenuated vaccine**. Live vaccines are absolutely contraindicated in individuals with **Severe Combined Immunodeficiency (SCID)** due to the inability to mount an effective immune response, leading to uncontrolled replication of the vaccinal organism and potentially fatal infection. - SCID causes profound defects in both **T-cell and B-cell immunity**, making the patient susceptible to infections from live vaccines. ***DPT*** - **DPT (Diphtheria, Pertussis, Tetanus)** is an **inactivated (killed) vaccine**; it is safe and typically recommended for SCID patients as it cannot cause the disease. - Only **live vaccines** are contraindicated in SCID, whereas **inactivated or recombinant vaccines** are generally safe. ***Hepatitis B*** - **Hepatitis B vaccine** is a **recombinant vaccine** (inactivated component) and is not contraindicated in patients with SCID. - While the immune response may be suboptimal, the vaccine itself poses no risk of causing the disease. ***IPV*** - **IPV (Inactivated Poliovirus Vaccine)** is a **killed vaccine**. It is safe for SCID patients and must be used instead of the **OPV (Oral Poliovirus Vaccine)**, which is a live vaccine. - Killed vaccines contain non-replicating antigens and thus cannot cause disease, even in highly immunocompromised individuals.
Question 13: Which of the following diagnoses can cause stridor in infants? 1. Laryngomalacia 2. Congenital hemangioma 3. Subglottic stenosis 4. Recurrent respiratory papillomatosis (RRP)
- A. 1, 3 and 4 (Correct Answer)
- B. 1 and 3
- C. 2 and 3
- D. 1 and 4
Explanation: ***1, 3 and 4*** - This option correctly identifies the three most common and clinically significant causes of stridor in infants - **Laryngomalacia (1)** is the most common cause of congenital stridor (60-75% of cases), presenting with inspiratory stridor that worsens with agitation - **Subglottic stenosis (3)** is the second most common cause of congenital stridor, presenting with biphasic stridor and may be congenital or acquired - **Recurrent respiratory papillomatosis/RRP (4)** caused by HPV (types 6 and 11) causes progressive airway obstruction with stridor and hoarseness - While congenital (subglottic) hemangiomas can cause stridor, they are less common than the above three conditions and typically present between 1-3 months of age *Incorrect: 1 and 3* - This option is incomplete as it excludes **RRP (4)**, which is an important cause of pediatric airway obstruction - RRP typically presents in early childhood and requires serial surgical debulking *Incorrect: 2 and 3* - This option incorrectly excludes **Laryngomalacia (1)**, which is the single most common cause of stridor in infants - Missing laryngomalacia in the differential diagnosis would be a significant oversight *Incorrect: 1 and 4* - This option fails to include **Subglottic stenosis (3)**, the second most common cause of congenital stridor - Subglottic stenosis can be life-threatening and requires urgent evaluation and management
Question 14: A 7-year-old child with a history of ear infection is complaining of lockjaw and limb spasm/fever/neck rigidity. Choose the most appropriate diagnosis:
- A. Meningitis
- B. Tetanus (Correct Answer)
- C. Bezold abscess
- D. Sinus thrombosis
Explanation: ***Tetanus*** - The striking combination of **lockjaw (trismus)** and generalized **limb spasms** is the hallmark clinical presentation of **Tetanus**, caused by the neurotoxin from *Clostridium tetani*. - The presence of **fever** and **neck rigidity** alongside an antecedent **ear infection** (potential portal of entry) strongly supports this diagnosis. - Tetanus causes sustained muscle contractions due to the inhibition of inhibitory neurons, leading to characteristic trismus, risus sardonicus, and opisthotonus. - Note: This is distinct from metabolic **tetany** (hypocalcemia), which causes carpopedal spasm without fever or lockjaw. *Incorrect: Meningitis* - While meningitis causes **fever** and **neck rigidity** (nuchal rigidity), it does not typically present with severe **lockjaw (trismus)** or sustained generalized **muscle spasms**. - Meningitis usually presents with headache, photophobia, altered consciousness, and positive Kernig's/Brudzinski's signs. *Incorrect: Bezold abscess* - A Bezold abscess is a deep neck abscess complicating mastoiditis, causing localized symptoms like neck pain, swelling, and **torticollis** (fixed head tilt). - It does not cause neuromuscular hyperexcitability resulting in **lockjaw** and widespread **limb spasms**. *Incorrect: Sinus thrombosis* - Lateral sinus thrombosis is a complication of otitis media presenting with **picket-fence fever**, headache, and signs of raised intracranial pressure. - It does not cause the characteristic muscle spasms and lockjaw seen in this presentation.