FMGE 2023 - Pediatrics Practice Questions

Q: A 5-year-old child presented with recurrent respiratory infections with thickened sputum. Chest X-ray showed bronchial wall thickening. He has been suffering from steatorrhea since birth. Which of the following is the first differential diagnosis?

Cystic fibrosis. ***Cystic fibrosis*** - This diagnosis is strongly supported by the triad of chronic **recurrent respiratory infections** (due to thick, retained sputum and subsequent bronchiectasis/bronchial wall thickening), and evidence of **pancreatic exocrine insufficiency** presenting as steatorrhea since birth. - It is an autosomal recessive disorder caused by mutations in the **CFTR gene**, leading to defective chloride transport and thick, sticky mucus formation in multiple organs. *Hyaline membrane disease* - Also known as **Respiratory Distress Syndrome (RDS)**, it is a condition primarily affecting **premature infants** due to deficiency of pulmonary surfactant. - It presents acutely within the first few hours of life and is not a cause of chronic, recurrent respiratory infections or steatorrhea in a 5-year-old child. *Alpha 1 anti-trypsin deficiency* - This hereditary condition typically presents with early-onset **panacinar emphysema** and/or liver disease (cirrhosis). - It is not associated with severe **pancreatic exocrine insufficiency** and steatorrhea in childhood, which is a hallmark feature of this patient's presentation. *Malabsorption syndrome* - While the patient exhibits findings consistent with **malabsorption** (steatorrhea), this is a general descriptive term and not a specific primary diagnosis for the entire clinical picture. - Cystic Fibrosis is the most specific primary diagnosis that explains both the **respiratory and gastrointestinal symptoms** (thick sputum, bronchial wall thickening, and steatorrhea).

Q: A 5-year-old girl is brought to the clinic with a fever, cough, red eyes, running nose, and rash. The appearance of the child is shown below. What is the diagnosis? ![img-36.jpeg](img-36.jpeg)

Measles. ***Measles*** - The clinical presentation of fever with the classic triad of **cough, coryza, and conjunctivitis** (the "3 Cs") is highly suggestive of measles (rubeola). - The image shows **Koplik's spots** (small white spots on the buccal mucosa), which are pathognomonic for measles and appear before the characteristic maculopapular rash. *Mumps* - Mumps is primarily characterized by **parotitis**, a painful swelling of the salivary glands, which is not described in this patient. - The classic prodrome of the "3 Cs" and Koplik's spots are absent in mumps. *Chickenpox* - The rash in chickenpox (varicella) is **vesicular**, often described as a "dewdrop on a rose petal," and appears in crops. This is different from the maculopapular rash of measles. - While a fever and malaise can occur, the prominent upper respiratory symptoms and conjunctivitis of measles are not typical of chickenpox. *Erythema infectiosum* - Caused by **Parvovirus B19**, this condition is characterized by a distinctive "**slapped-cheek**" rash on the face. - It is followed by a **lacy, reticular rash** on the trunk and extremities, which is inconsistent with the patient's presentation.

Q: Which of the following is not a correct match for ABCD according to pediatric advanced life support?

Dehydration. ***Dehydration*** - In the mnemonic **ABCD** used in **Pediatric Advanced Life Support (PALS)** and basic life support (BLS), D stands for **Disability** (neurological status/deficit), not Dehydration. - Assessing **Disability** involves evaluating the infant's or child's neurological status using tools like the **AVPU scale** or **Glasgow Coma Scale (GCS)**. *Airway* - A stands correctly for **Airway** in the **ABCD** approach, focusing on ensuring a patent passage for breathing. - This step involves opening the airway using maneuvers like the **head tilt/chin lift** (for trauma absence) or **jaw thrust** (for suspected trauma). *Breathing* - B stands correctly for **Breathing**, which assesses the presence and adequacy of respiratory effort. - Management includes providing rescue breaths, using devices like a **bag-mask device**, and determining the need for **intubation**. *Circulation* - C stands correctly for **Circulation**, which evaluates heart rate, pulse strength, capillary refill time, and skin color. - Interventions include initiating **chest compressions** if a pulse is absent or too slow/weak, and managing **shock** (e.g., fluid resuscitation/medications).

Q: A neonate was brought with a history of frothiness from the mouth and respiratory distress. An X-ray of the neonate is given below. What is the diagnosis?

Esophageal atresia with tracheo-esophageal fistula. ***Esophageal atresia with tracheo-esophageal fistula*** - The clinical presentation of a neonate with excessive **frothiness from the mouth**, choking, and **respiratory distress** is highly suggestive of this diagnosis. - The X-ray confirms the diagnosis by showing a **coiled orogastric tube** (indicated by the arrow) in the blind-ending upper esophageal pouch. The presence of **gas in the stomach** indicates a communication between the trachea and the distal esophagus (a distal fistula). *Transient tachypnea of newborn* - This is a benign condition caused by delayed clearance of fetal lung fluid, presenting with rapid breathing shortly after birth, usually in term infants delivered by C-section. - The chest X-ray typically shows **perihilar streaking**, **fluid in the fissures**, and mild hyperinflation, not a coiled feeding tube. *Congenital diaphragmatic hernia* - This condition involves the herniation of abdominal contents into the chest, causing severe respiratory distress and a **scaphoid abdomen**. - The chest X-ray would show **bowel loops within the thoracic cavity** and displacement of the mediastinum, which are not seen in this image. *Respiratory distress syndrome* - Primarily seen in premature infants, this is caused by a deficiency of **pulmonary surfactant**. - The characteristic X-ray findings include low lung volumes, a diffuse **ground-glass appearance**, and **air bronchograms**.

Q: Which of the following are true about nephroblastoma except:

Treatment is chemotherapy followed by surgery. ***Correct Answer: Treatment is chemotherapy followed by surgery*** This is the **INCORRECT** statement (which makes it the correct answer for this EXCEPT question): - In **COG (Children's Oncology Group) protocol** used in India and US: **Primary treatment is upfront nephrectomy** (surgery first) followed by chemotherapy - SIOP protocol uses preoperative chemotherapy, but this is NOT the standard approach in India/FMGE context - **Surgery-first approach** allows for accurate staging and immediate tumor removal *Incorrect: Most common intra abdominal malignancy in children* - This is **TRUE** - Nephroblastoma (Wilms tumor) is indeed the most common intra-abdominal malignancy in children (peak age 3-4 years) *Incorrect: Early lung mets* - This is **TRUE** - Wilms tumor has propensity for **early hematogenous spread to lungs** - Lungs are the most common site of metastasis - Chest X-ray/CT is mandatory for staging *Incorrect: Hematogenous spread* - This is **TRUE** - Wilms tumor spreads via **hematogenous route** to lungs (most common), liver, and other sites - Unlike neuroblastoma which can spread lymphatically

Q: What is the most common cause of death in children with Diabetic ketoacidosis?

Cerebral edema. ***Cerebral edema*** - It is the **most common cause of mortality** in pediatric DKA, occurring in 0.5-1% of cases with a mortality rate of 20-25% [1] - Occurs due to rapid changes in **plasma osmolality** during treatment - the rapid decrease of blood glucose and effective osmolarity creates an osmotic gradient, causing fluid shifts into brain cells - Leads to potentially fatal **intracranial pressure** elevation with clinical features of headache, altered mental status, bradycardia, and respiratory depression - Risk factors include: new-onset diabetes, younger age, severe acidosis, and rapid fluid administration *Hypokalemia* - Potassium levels often drop dramatically during DKA treatment as insulin drives potassium into cells, potentially causing life-threatening **cardiac arrhythmias** [1] - This complication is highly anticipated and routinely managed by **potassium replacement therapy** with close monitoring [3] - While potentially fatal if unrecognized, it is a **preventable and treatable** condition with proper electrolyte management *Dehydration* - Severe dehydration causing **hypovolemic shock** is a major factor contributing to morbidity in DKA [2] - Effectively managed by aggressive **IV fluid resuscitation** with careful monitoring [3] - In treated pediatric DKA, the failure leading to death is typically the development of **cerebral edema** rather than persistent volume depletion [1] *Sepsis* - While sepsis can precipitate DKA, it is an underlying **trigger** or precipitating factor, not the direct physiological cause of death during DKA itself - The overwhelming fatality **in** pediatric DKA (as opposed to from the precipitating illness) is generally attributed to **cerebral edema** from rapid fluid shifts leading to central nervous system complications [1]

Question 1

A 5-year-old child presented with recurrent respiratory infections with thickened sputum. Chest X-ray showed bronchial wall thickening. He has been suffering from steatorrhea since birth. Which of the following is the first differential diagnosis?

Accepted Answer

Cystic fibrosis

Answer

Hyaline membrane disease

Answer

Alpha 1 anti-trypsin deficiency

Answer

Malabsorption syndrome

Question 2

A 5-year-old girl is brought to the clinic with a fever, cough, red eyes, running nose, and rash. The appearance of the child is shown below. What is the diagnosis? ![img-36.jpeg](img-36.jpeg)

Accepted Answer

Measles

Answer

Chickenpox

Answer

Mumps

Answer

Erythema infectiosum

Question 3

Which of the following is not a correct match for ABCD according to pediatric advanced life support?

Accepted Answer

Dehydration

Answer

Circulation

Answer

Airway

Answer

Breathing

Question 4

A neonate was brought with a history of frothiness from the mouth and respiratory distress. An X-ray of the neonate is given below. What is the diagnosis?

Accepted Answer

Esophageal atresia with tracheo-esophageal fistula

Answer

Transient tachypnea of newborn

Answer

Respiratory distress syndrome

Answer

Congenital diaphragmatic hernia

Question 5

Which of the following are true about nephroblastoma except:

Accepted Answer

Treatment is chemotherapy followed by surgery

Answer

Most common intra abdominal malignancy in children

Answer

Early lung mets

Answer

Hematogenous spread

Question 6

What is the most common cause of death in children with Diabetic ketoacidosis?

Accepted Answer

Cerebral edema

Answer

Hypokalemia

Answer

Dehydration

Answer

Sepsis

FMGE 2023 — Pediatrics