NEET-PG 2012 - Pediatrics Practice Questions

Q: What is the most common cause of conjugated hyperbilirubinemia in infants?

Biliary atresia. ***Rotor syndrome*** - Characterized by **conjugated hyperbilirubinemia** due to a defect in hepatic uptake and storage of bilirubin [1]. - This condition can lead to elevated levels of **direct (conjugated) bilirubin** without significant liver damage. *Crigler Najjar* - This condition primarily causes **unconjugated hyperbilirubinemia** due to a deficiency of the enzyme **uridine diphosphate glucuronyl transferase** [1]. - It typically presents with **kernicterus** in newborns rather than conjugated bilirubin elevation. *Breast milk jaundice* - Mainly leads to **unconjugated hyperbilirubinemia** due to substances in breast milk that inhibit bilirubin conjugation. - Generally occurs in **breastfed infants** after the first week of life, not presenting with increased conjugated bilirubin. *Gilbert syndrome* - This syndrome is associated with **unconjugated hyperbilirubinemia** due to a genetic defect in bilirubin conjugation [1]. - Typically benign, it does not cause **increased conjugated bilirubin** levels as seen in Rotor syndrome. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Liver and Gallbladder, p. 860.

Q: Which of the following cancers has the highest cure rate?

Retinoblastoma. ***Retinoblastoma*** - This **childhood eye cancer** has an excellent prognosis, with a reported cure rate of **over 95%** when diagnosed early and treated promptly. - Treatment options like **chemotherapy**, **radiation**, **laser therapy**, and **enucleation** contribute to its high survival rate. *Wilm's Tumor* - While **Wilm's tumor** (nephroblastoma) also has a high cure rate in children, typically **around 90%**, it is slightly lower than that of retinoblastoma. - It is a **kidney cancer** primarily affecting children and is highly responsive to treatment. *Rhabdomyosarcoma* - The cure rate for **rhabdomyosarcoma**, a rare and aggressive cancer of the soft tissues, varies significantly based on factors like **tumor location**, **stage**, and **histology**. - Overall survival rates are generally lower than for retinoblastoma and Wilm's tumor, often ranging from **60-70%**. *All of the options* - This option is incorrect because while all three cancers listed have good prognoses, **retinoblastoma** specifically stands out with the highest cure rate among them. - The cure rates for Wilm's tumor and rhabdomyosarcoma, while good, are not as high as that for retinoblastoma.

Q: Upper segment to lower segment ratio in a 3-year-old child is:

1.3:1. ***1.3:1*** - At birth, the upper segment to lower segment ratio is around **1.7:1**, indicating proportionally longer trunks. - By the age of **3 years**, this ratio typically decreases to approximately **1.3:1** as the lower limbs grow more rapidly. *1.2:1* - This ratio is typically observed in **older children** or young adults, as lower limb growth catches up further. - A ratio of 1.2:1 would suggest more **proportional body segments** than expected for a 3-year-old child. *1.4:1* - This ratio is closer to that of a **younger infant** or toddler, as the lower segments are still relatively shorter. - A 3-year-old would generally have experienced more **lower limb growth**, reducing this ratio further. *1.6:1* - This ratio is characteristic of a **newborn or very young infant**, where the upper body and head are significantly larger relative to the legs. - It would be **abnormal** for a 3-year-old to still have such a high ratio, indicating a disproportional growth pattern.

Q: What is the recommended rate for blood transfusion in neonates?

1-5 ml/min. ***1-5 ml/min*** - The recommended rate for blood transfusion in neonates is **1-5 ml/min** to prevent **circulatory overload** and other complications. - Slower rates are crucial for neonates due to their limited cardiovascular reserves and smaller blood volume. *5-10 ml/min* - This rate is generally **too fast** for routine neonatal blood transfusions, increasing the risk of **fluid overload** and **cardiac strain**. - Rapid administration can lead to complications such as **necrotizing enterocolitis (NEC)** or **respiratory distress**. *10-15 ml/min* - Administering blood at this rate in neonates is **contraindicated** due to the high risk of **cardiovascular compromise**. - It could quickly overwhelm the neonate's circulatory system, leading to severe adverse events. *15-20 ml/min* - This rate is significantly **too rapid** for any neonatal blood transfusion and would almost certainly result in **severe volume overload** and potential cardiac arrest. - Such high rates are only rarely considered in extreme emergency situations, like massive hemorrhage with specific considerations and monitoring.

Q: Double aortic arch is associated with which syndrome?

DiGeorge syndrome. ***DiGeorge syndrome*** - **DiGeorge syndrome** is caused by a **22q11.2 deletion** affecting the development of the third and fourth pharyngeal pouches, leading to **thymic hypoplasia**, **parathyroid hypoplasia**, and **cardiac anomalies**. - Common cardiac defects include **interrupted aortic arch type B**, **truncus arteriosus**, **tetralogy of Fallot**, and **VSD**. - **Double aortic arch** can occur in 22q11.2 deletion syndrome, though it is less common than other cardiac anomalies; however, among the syndromes listed, this represents the most appropriate association. - The question tests recognition that various cardiac arch anomalies, including double aortic arch, may be seen in this genetic syndrome. *CATCH 22 syndrome* - This is an **acronym** for DiGeorge syndrome: **C**ardiac defects, **A**bnormal facies, **T**hymic hypoplasia, **C**left palate, **H**ypocalcemia, and **22q11 deletion**. - It is **essentially the same condition** as DiGeorge syndrome, just using different nomenclature. - While technically correct, "DiGeorge syndrome" is the more standard medical terminology currently used. *Shprintzen syndrome* - **Shprintzen syndrome** (also called **velocardiofacial syndrome or VCFS**) is caused by the **same 22q11.2 deletion** as DiGeorge syndrome. - It represents a **phenotypic variant within the 22q11.2 deletion syndrome spectrum**, with more emphasis on palatal and facial features. - Since it shares the same genetic basis, it can also present with similar cardiac anomalies, but "DiGeorge syndrome" is the more commonly recognized term for this genetic disorder. *None of the options* - This is incorrect because the three syndromes listed above (DiGeorge, CATCH 22, and Shprintzen) all refer to **22q11.2 deletion syndrome** or its variants, which can be associated with various cardiac anomalies including double aortic arch. - Among the listed options, **DiGeorge syndrome** is the most appropriate and widely recognized answer.

Q: A 3-month-old infant presents with an abdominal palpable mass and non-bilious vomiting. What is the most likely diagnosis?

Hypertrophic pyloric stenosis. ***Hypertrophic pyloric stenosis*** - The classic presentation includes **projectile non-bilious vomiting** and a palpable **olive-shaped mass** in the epigastrium of an infant typically between 3 weeks and 6 months of age. - The vomiting is non-bilious because the obstruction is proximal to the ampulla of Vater. *Intussusception* - While it can present with an **abdominal mass** and vomiting, the vomiting is often **bilious** and the classic stool is **'currant jelly'**, which is not mentioned here. - It usually presents with sudden onset of severe, **colicky abdominal pain** and occurs more commonly in slightly older infants (6-12 months). *Tracheoesophageal fistula* - This condition presents at birth with symptoms such as **choking, coughing**, and **cyanosis** during feeding. - It usually causes respiratory distress and feeding difficulties from the first days of life, not a palpable abdominal mass and non-bilious vomiting at 3 months. *Duodenal atresia* - This is a congenital obstruction that typically presents with **bilious vomiting** (as the obstruction is distal to the ampulla of Vater) within the first 24-48 hours of life. - Imaging usually shows a **“double bubble” sign** on abdominal X-ray, and an abdominal mass is not typically palpable.

Q: Rehydration therapy in a 2 year old severely dehydrated child is -

30 ml/kg in 30 min, 70 ml/kg in 2.5 hours. ***30 ml/kg in 30 min, 70 ml/kg in 2.5 hours*** - This option reflects the recommended rehydration protocol for a severely dehydrated child aged **12 months to 5 years**, where the first 30 ml/kg are given rapidly over 30 minutes, followed by 70 ml/kg over the next 2.5 hours. - This rapid initial infusion helps to quickly restore **circulating volume** and improve perfusion during severe dehydration. *30 ml/kg in 1 hour, 70 ml/kg in 5 hours* - This protocol is typically used for children with **some dehydration**, not severe dehydration, and is usually administered orally when possible. - The slower rate of rehydration would be insufficient for a severely dehydrated child requiring more urgent fluid replacement. *20 ml/kg in 30 min, 80 ml/kg in 2.5 hours* - While reflecting a rapid initial phase, the total volume and distribution of fluids differ from the WHO guidelines for **severe dehydration** in this age group. - The **initial 20 ml/kg over 30 minutes** is generally a slightly lower first bolus than recommended for very severe cases, and the subsequent phase is also adjusted. *75 ml/kg in 4 hours* - This represents a **lower total volume** (75 ml/kg compared to 100 ml/kg) and a different time distribution for severely dehydrated children in the 12 month to 5 year age group. - This protocol is more aligned with the management of **some dehydration** rather than the urgent requirements of severe dehydration.

Q: Most common site of extramedullary relapse of ALL in a 6-year-old boy is

CNS (Central Nervous System). ***CNS (Central Nervous System)*** - The **central nervous system** is the **most common site of extramedullary relapse** in pediatric acute lymphoblastic leukemia (ALL), accounting for the majority of extramedullary relapses. - CNS is an immunologically privileged sanctuary site where chemotherapy penetration is limited due to the blood-brain barrier. - CNS relapse presents with symptoms like headache, vomiting, cranial nerve palsies, or signs of increased intracranial pressure and requires intrathecal chemotherapy and cranial irradiation. - Prophylactic CNS therapy is a standard component of ALL treatment protocols to prevent CNS relapse. *Testes* - **Testes** are the **second most common** site of extramedullary relapse and the most common **solid organ** site in boys with ALL. - Like the CNS, testes are immunologically privileged sites with limited chemotherapy penetration. - Testicular relapse presents as painless testicular enlargement (unilateral or bilateral) and requires testicular radiation plus systemic therapy intensification. *Liver* - While **hepatic infiltration** can occur in ALL, the liver is not a common site for **isolated extramedullary relapse**. - Hepatic involvement typically indicates widespread systemic disease rather than a primary relapse site. *Lung* - **Pulmonary involvement** in ALL is rare as an isolated extramedullary relapse site. - Lung findings in ALL patients are more commonly due to infection, leukostasis in hyperleukocytosis, or disseminated disease.

Q: What is the correct dose of i.v. adrenaline in term infants during neonatal resuscitation?

0.1-0.3 ml/kg in 1:10,000. ***0.1-0.3 ml/kg in 1:10,000*** - The recommended intravenous adrenaline dose for neonatal resuscitation is **0.01-0.03 mg/kg** using a **1:10,000 solution (0.1 mg/mL)**. - Volume calculation: 0.01-0.03 mg/kg ÷ 0.1 mg/mL = **0.1-0.3 mL/kg**. - This is the standard dose as per **NRP (Neonatal Resuscitation Program)** and **AHA guidelines** [2]. - The 1:10,000 concentration is safer for IV/umbilical venous catheter administration in neonates. *0.01-0.03 ml/kg in 1:1,000* - This volume is far too low for a 1:1,000 solution. - Would deliver only 0.01-0.03 mg total (not per kg), resulting in a **sub-therapeutic dose**. - The 1:1,000 concentration contains 1 mg/mL, which is **10 times more concentrated** than the recommended dilution. *0.3-0.5 ml/kg in 1:10,000* - This volume would deliver 0.03-0.05 mg/kg, which **exceeds the recommended maximum** of 0.03 mg/kg. - Higher doses can cause **severe adverse effects** including hypertension, arrhythmias, decreased myocardial function, and compromised coronary perfusion. - Not recommended as the standard initial dose. *0.03-0.05 ml/kg in 1:1,000* - The 1:1,000 concentration (1 mg/mL) is **too concentrated for IV use** in neonates [1]. - This volume would deliver 0.03-0.05 mg/kg from a highly concentrated solution, increasing risk of **severe cardiovascular complications**. - The 1:1,000 solution is reserved for **endotracheal administration** (at higher volumes of 0.5-1 mL/kg), not IV route.

Question 1

What is the most common cause of conjugated hyperbilirubinemia in infants?

Accepted Answer

Biliary atresia

Answer

Rotor syndrome

Answer

Crigler Najjar syndrome

Answer

Dubin-Johnson syndrome

Question 2

Which of the following cancers has the highest cure rate?

Accepted Answer

Retinoblastoma

Answer

Wilm's Tumor

Answer

Rhabdomyosarcoma

Answer

All of the options

Question 3

Upper segment to lower segment ratio in a 3-year-old child is:

Accepted Answer

1.3:1

Answer

1.2:1

Answer

1.4:1

Answer

1.6:1

Question 4

What is the recommended rate for blood transfusion in neonates?

Accepted Answer

1-5 ml/min

Answer

10-15 ml/min

Answer

15-20 ml/min

Answer

5-10 ml/min

Question 5

Double aortic arch is associated with which syndrome?

Accepted Answer

DiGeorge syndrome

Answer

CATCH 22 syndrome

Answer

Shprintzen syndrome

Answer

None of the options

Question 6

A 3-month-old infant presents with an abdominal palpable mass and non-bilious vomiting. What is the most likely diagnosis?

Accepted Answer

Hypertrophic pyloric stenosis

Answer

Tracheoesophageal fistula

Answer

Duodenal atresia

Answer

Intussusception

Question 7

What is the PRIMARY pathophysiological mechanism underlying the most common cause of neonatal hyperbilirubinemia?

Accepted Answer

Immature liver enzyme

Answer

Inefficient erythropoiesis

Answer

RBC hemolysis

Answer

Decreased bilirubin excretion

Question 8

Rehydration therapy in a 2 year old severely dehydrated child is -

Accepted Answer

30 ml/kg in 30 min, 70 ml/kg in 2.5 hours

Answer

75 ml/kg in 4 hours

Answer

30 ml/kg in 1 hour, 70 ml/kg in 5 hours

Answer

20 ml/kg in 30 min, 80 ml/kg in 2.5 hours

Question 9

Most common site of extramedullary relapse of ALL in a 6-year-old boy is

Accepted Answer

CNS (Central Nervous System)

Answer

Testes

Answer

Liver

Answer

Lung

Question 10

What is the correct dose of i.v. adrenaline in term infants during neonatal resuscitation?

Accepted Answer

0.1-0.3 ml/kg in 1:10,000

Answer

0.03-0.05 ml/kg in 1:1,000

Answer

0.01-0.03 ml/kg in 1:1,000

Answer

0.3-0.5 ml/kg in 1:10,000

NEET-PG 2012 — Pediatrics