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Facharzt FMH Kinder- und Jugendmedizin - independent subject foundations study materials
Facharzt FMH Kinder- und Jugendmedizin - independent subject foundations study resources

Facharzt FMH Kinder- und Jugendmedizin - independent subject foundations practice questions

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Question 1Pediatric emergency recognition and resuscitation

A child has septic shock with poor perfusion in a fully monitored pediatric resuscitation setting. The responsible clinician has assessed fluid responsiveness and no current contraindication to a cautious initial crystalloid bolus. Which listed strategy includes an appropriate initial bolus and repeated cardiopulmonary reassessment?

  1. Immediate rapid IV infusion of 50 mL/kg of 0.45% sodium chloride with 5% dextrose over 10 minutes
  2. Immediate initiation of an intravenous sodium bicarbonate infusion to correct metabolic acidosis
  3. Rapid administration of a 10 to 20 mL/kg bolus of balanced isotonic crystalloid over 10 to 20 minutes with frequent cardiopulmonary reassessment Correct answer.
  4. High-dose intravenous loop diuretic therapy to prevent non-cardiogenic pulmonary edema

Explanation

Initial fluid resuscitation in pediatric septic shock requires rapid administration of 10 to 20 mL/kg boluses of isotonic balanced crystalloid (or 0.9% normal saline) titrated to clinical markers of cardiac output. Continuous reassessment for signs of fluid responsiveness or fluid overload (hepatomegaly, rales, worsening work of breathing) is essential after each aliquot. In cold shock with persistent hypoperfusion despite fluid boluses, early inotropic support with epinephrine should be initiated.

Question 2Pediatric emergency recognition and resuscitation

A 4-year-old boy develops sudden generalized urticaria, facial angioedema, stridor, wheezing, repetitive vomiting, and lethargy within 10 minutes of consuming cashew nuts. What is the immediate first-line pharmacotherapy of choice?

  1. Intramuscular epinephrine (0.01 mg/kg, 1:1,000 solution) administered into the anterolateral mid-thigh Correct answer.
  2. Intravenous clemastine or diphenhydramine administered over 15 minutes
  3. Oral dexamethasone liquid (0.6 mg/kg) administered with oral hydration
  4. Inhaled nebulized budesonide suspension (1 mg) combined with nebulized saline

Explanation

Intramuscular epinephrine into the anterolateral mid-thigh is the undisputed first-line treatment for anaphylaxis. Its alpha-1 adrenergic vasoconstriction reverses hypotension and mucosal edema, while beta-2 adrenergic effects reverse bronchoconstriction and suppress mast cell mediator release. Delay in epinephrine administration is the primary risk factor for fatal pediatric anaphylaxis.

Question 3Pediatric emergency recognition and resuscitation

A 2-year-old girl is brought to the pediatric resuscitation bay in generalized convulsive status epilepticus. The seizure has persisted for 12 minutes despite two weight-appropriate doses of buccal midazolam administered prior to arrival. Intravenous access is established. According to standard pediatric status epilepticus algorithms, which intravenous second-line antiepileptic therapy should be initiated immediately?

  1. Continuous intravenous infusion of propofol with immediate neuromuscular paralysis
  2. Oral ethosuximide via nasogastric tube
  3. Intravenous calcium gluconate bolus combined with magnesium sulfate
  4. Intravenous levetiracetam (60 mg/kg, max 4500 mg) infused over 5 to 10 minutes Correct answer.

Explanation

When status epilepticus persists beyond first-line benzodiazepine therapy (established status epilepticus >10-15 minutes), second-line non-sedating IV antiepileptic drugs must be administered promptly. High-dose IV levetiracetam (60 mg/kg), fosphenytoin/phenytoin (20 mg PE/kg), or IV valproate (40 mg/kg) represent validated, highly effective second-line choices per randomized trials (e.g., ConSEPT, EcLiPSE, ESETT).

Question 4Pediatric emergency recognition and resuscitation

A 15-year-old adolescent presents 5 hours after an acute intentional ingestion of 16 grams of paracetamol (acetaminophen). The patient is currently asymptomatic with normal physical examination findings. A 4-hour post-ingestion serum paracetamol level returns at 220 mg/L (1450 µmol/L), which plots above the treatment threshold on the Rumack-Matthew nomogram. What is the most appropriate management?

  1. Discharge home with psychiatric follow-up after an observation period of 6 hours
  2. Initiate intravenous N-acetylcysteine (NAC) therapy immediately according to standardized dosing protocols Correct answer.
  3. Administer oral activated charcoal alone and repeat liver function tests in 24 hours
  4. Perform emergency hemodialysis to clear unconjugated paracetamol metabolites

Explanation

Serum paracetamol levels falling above the action line on the Rumack-Matthew nomogram indicate significant risk of toxic N-acetyl-p-benzoquinone imine (NAPQI) accumulation and fulminant hepatic necrosis. Intravenous N-acetylcysteine (NAC) replenishes hepatic glutathione stores and acts as a direct glutathione substitute; starting NAC within 8 hours of ingestion affords virtually complete hepatoprotection.

Question 5Pediatric emergency recognition and resuscitation

A 14-month-old infant is suspected of swallowing a foreign body. An urgent anteroposterior chest radiograph demonstrates a 20 mm circular radiopaque object in the mid-esophagus with a characteristic 'double-ring' (halo) sign. The child is irritable and refuses fluids. What is the standard management for this condition?

  1. Outpatient observation with serial stool screening, expecting spontaneous rectal passage within 5 days
  2. Emergent endoscopic removal within 2 hours under general anesthesia Correct answer.
  3. High-dose oral polyethylene glycol administration to accelerate transit into the colon
  4. Repeat chest radiography in 24 hours to confirm whether the object enters the gastric lumen

Explanation

An esophageal button battery needs emergency specialist removal. Do not wait for symptoms or for a two-hour deadline to elapse; serious electrical tissue injury can occur rapidly. The appropriate airway and procedural team should act promptly, with any adjunctive measure governed by actual eligibility and never delaying removal.

Question 6Pediatric emergency recognition and resuscitation

A 2-year-old child presents with mild-to-moderate dehydration secondary to acute viral gastroenteritis. The child is alert, has slightly sunken eyes, moist oral mucosa, and capillary refill time of 1.5 seconds, with no signs of shock. According to WHO and ESPGHAN guidelines, what is the preferred first-line rehydration strategy?

  1. Oral rehydration therapy using a low-osmolarity oral rehydration solution (ORS, ~245 mOsm/L) administered in small, frequent aliquots Correct answer.
  2. Intravenous infusion of 5% dextrose in water at twice the maintenance rate
  3. Exclusive administration of boiled cow's milk and apple juice
  4. Strict fasting with intravenous Ringer's lactate for 48 hours

Explanation

Low-osmolarity oral rehydration solution (ORS, osmolarity ~245 mOsm/L, sodium 75 mmol/L, glucose 75 mmol/L) is the undisputed first-line therapy for mild-to-moderate pediatric dehydration. It utilizes intestinal sodium-glucose cotransporters (SGLT1) to maximize water and electrolyte absorption with lower rates of vomiting, shorter hospital stays, and fewer adverse events than IV hydration.

Question 7Pediatric emergency recognition and resuscitation

A 2-year-old toddler presents with a sudden onset of choking and paroxysmal coughing while snacking on peanuts 4 hours ago. On physical examination, the child is comfortable at rest but has unilateral right-sided expiratory wheezing and decreased breath sounds. An inspiratory-expiratory chest radiograph demonstrates right lung hyperlucency with mediastinal shift to the left on expiration. What is the definitive management of choice?

  1. Chest physiotherapy with postural percussion and drainage in the Trendelenburg position
  2. Initiation of oral broad-spectrum antibiotics and high-dose nebulized salbutamol for 7 days
  3. Immediate blind sweeping of the posterior hypopharynx with Magill forceps
  4. Rigid bronchoscopy under general anesthesia for diagnostic confirmation and foreign body extraction Correct answer.

Explanation

The sudden choking vignette followed by unilateral wheezing and air trapping (hyperlucency with contralateral mediastinal shift on expiration due to a ball-valve mechanism) is classic for bronchial foreign body aspiration (most commonly organic matter like peanuts). Rigid bronchoscopy under general anesthesia is the gold standard diagnostic and therapeutic intervention, securing airway control and enabling safe optical retrieval.

Question 8Pediatric emergency recognition and resuscitation

A 2-year-old boy presents with a 2-day history of low-grade fever, rhinorrhea, and hoarseness. Over the past 6 hours, he has developed a harsh barking 'seal-like' cough and inspiratory stridor at rest with moderate jugular and subcostal retractions (moderate croup / laryngotracheobronchitis). What is the evidence-based first-line pharmacological treatment combination?

  1. A single dose of oral dexamethasone (0.15 to 0.60 mg/kg) combined with nebulized L-epinephrine (or racemic epinephrine) Correct answer.
  2. Intravenous amoxicillin-clavulanic acid with inhaled salbutamol
  3. Nebulized N-acetylcysteine with oral diphenhydramine
  4. Oral azithromycin for 5 days with warm steam humidification alone

Explanation

Croup (viral laryngotracheobronchitis, most commonly parainfluenza virus) causes subglottic mucosal edema. A single dose of oral dexamethasone (0.15 to 0.60 mg/kg) reduces subglottic inflammation and hospital admission rates, while nebulized epinephrine provides rapid vasoconstriction via alpha-1 adrenergic receptors, significantly relieving resting stridor within 10 to 30 minutes.

Question 9Pediatric emergency recognition and resuscitation

A 4-year-old unimmunized child presents to the emergency department with abrupt onset of high fever (39.9°C), toxic appearance, severe sore throat, inability to swallow oral secretions resulting in drooling, and a muffled 'hot potato' voice. The child is sitting upright, leaning forward with the neck hyperextended (tripod position), breathing with inspiratory stridor. Acute epiglottitis is suspected. What is the most critical immediate management principle?

  1. Lay the child supine immediately and perform a thorough oral examination using a tongue depressor to inspect the epiglottis
  2. Perform immediate venous blood sampling and arterial blood gas puncture to measure lactic acidosis
  3. Keep the child calm in the parent's lap, avoid invasive or agitating procedures, and immediately assemble an airway team (anesthesiologist and ENT surgeon) for controlled intubation in the operating room Correct answer.
  4. Administer oral fluids immediately to prevent dehydration while awaiting lateral neck radiography

Explanation

Acute epiglottitis (supraglottitis) is a medical emergency with extreme risk of sudden, fatal laryngospasm. The golden rule of management is to minimize patient agitation: keep the child in the comforting presence of the parent, avoid any intraoral instrumentation or painful phlebotomy, and immediately transport the patient under senior supervision to the operating room for definitive endotracheal intubation by experienced specialists.

Question 10Pediatric emergency recognition and resuscitation

A 6-year-old child in cardiac arrest following submersion is undergoing cardiopulmonary resuscitation (CPR). The cardiac monitor displays pulseless electrical activity (PEA) with a rate of 35 bpm. High-quality chest compressions and bag-valve-mask ventilation with 100% oxygen are in progress, and intraosseous (IO) access is secured. According to Pediatric Advanced Life Support (PALS) algorithms, what is the next critical medication to administer?

  1. Immediate unsynchronized defibrillation at 2 J/kg
  2. Intravenous amiodarone bolus (5 mg/kg)
  3. Intravenous atropine (0.02 mg/kg)
  4. Intravenous/intraosseous epinephrine (0.01 mg/kg, equivalent to 0.1 mL/kg of 1:10,000 concentration) every 3 to 5 minutes Correct answer.

Explanation

Pulseless electrical activity (PEA) and asystole are non-shockable cardiac arrest rhythms in pediatric patients. The primary pharmacotherapy is intravenous/intraosseous epinephrine (0.01 mg/kg = 0.1 mL/kg of 1:10,000) administered as soon as possible and repeated every 3 to 5 minutes, accompanied by high-quality continuous CPR and aggressive evaluation for reversible causes (H's and T's: hypoxia, hypovolemia, hypothermia, etc.).

Question 11Pediatric emergency recognition and resuscitation

An 8-year-old boy is brought to the trauma center after being struck by a car. He is in severe respiratory distress, cyanotic, and hypotensive (BP 68/40 mmHg, HR 165 bpm). Physical examination reveals tracheal deviation to the right, hyperresonance and absent breath sounds over the left hemithorax, and distended neck veins. What is the immediate life-saving intervention?

  1. Immediate needle decompression of the left hemithorax followed promptly by tube thoracostomy Correct answer.
  2. Urgent chest computed tomography (CT) scan to quantify intrathoracic parenchymal injury
  3. Endotracheal intubation with high positive end-expiratory pressure (PEEP >15 cmH2O)
  4. Intravenous administration of 500 mL of 20% albumin over 10 minutes

Explanation

This patient presents with classic signs of left tension pneumothorax: respiratory distress, hypotension, unilateral absent breath sounds, hyperresonance, and contralateral tracheal shift. Tension pneumothorax is a clinical diagnosis requiring immediate emergency needle thoracostomy decompression (in the 2nd intercostal space midclavicular line or 4th/5th intercostal space anterior axillary line) followed by chest tube placement before performing any radiologic imaging.

Question 12Pediatric emergency recognition and resuscitation

After an assessed resuscitation, a child has persistent hypotensive vasodilatory septic shock with low systemic vascular resistance, adequate filling and no indication for further blind fluid boluses. Which listed vasoactive strategy most directly supports vascular tone in this supplied physiology?

  1. Milrinone infusion (0.5 to 0.75 µg/kg/min)
  2. High-dose dopamine infusion (15 to 20 µg/kg/min)
  3. Intravenous phenylephrine boluses exclusively
  4. Norepinephrine infusion (0.05 to 0.50 µg/kg/min) Correct answer.

Explanation

In pediatric septic shock presenting with vasodilatory / distributive ('warm') shock physiology (low systemic vascular resistance, wide pulse pressure, flash capillary refill), norepinephrine is the first-line vasoactive agent of choice. Its potent alpha-1 adrenergic vasoconstriction restores systemic vascular resistance and diastolic perfusion pressure while providing modest beta-1 inotropic support.

Question 13Pediatric emergency recognition and resuscitation

A 7-year-old child is brought to the emergency department after being rescued from a house fire. The child has headache, nausea, dizziness, and mild confusion. Pulse oximetry displays an oxygen saturation of 99% on room air. Blood gas analysis reveals severe metabolic acidosis and a carboxyhemoglobin (COHb) level of 26%. What is the immediate first-line management?

  1. Administration of 100% high-flow normobaric oxygen via a tight-fitting non-rebreather reservoir mask Correct answer.
  2. Immediate oral administration of methylene blue solution
  3. Reassurance and observation on room air because pulse oximetry shows 99% saturation
  4. Intravenous infusion of sodium thiosulfate alone without supplemental oxygen

Explanation

Carbon monoxide (CO) poisoning shifts the oxyhemoglobin dissociation curve to the left and binds hemoglobin with ~200-fold greater affinity than oxygen. Standard pulse oximeters cannot distinguish carboxyhemoglobin from oxyhemoglobin, falsely displaying normal saturation (99%). Immediate administration of 100% high-flow oxygen reduces the elimination half-life of COHb from ~300 minutes on room air to ~60-90 minutes, reversing cellular hypoxia.

Question 14Pediatric emergency recognition and resuscitation

A 4-year-old boy is brought to the emergency department with acute onset of severe right hemiscrotal pain and vomiting that began 2 hours ago. On physical examination, the right hemiscrotum is erythematous and tender, the right testis is high-riding with a transverse lie, and the ipsilateral cremasteric reflex is absent. Elevation of the testis does not relieve the pain (negative Prehn sign). What is the definitive emergency management?

  1. Prescribe oral amoxicillin-clavulanic acid and schedule outpatient scrotal ultrasound in 1 week
  2. Perform manual external detorsion and discharge home if pain improves without surgical consultation
  3. Apply cold compresses and administer oral ibuprofen with 48-hour bed rest
  4. Immediate emergency surgical exploration of the scrotum with detorsion and bilateral orchidopexy within 6 hours of onset Correct answer.

Explanation

The acute painful high-riding testis with the supplied examination findings raises strong concern for torsion and needs immediate skilled surgical assessment and treatment. Salvage probability generally declines with time, but an elapsed six-hour interval does not make later exploration unnecessary or justify waiting for a deadline. A single bedside sign cannot independently exclude torsion.

Question 15Neonatal adaptation, prematurity and early illness

A full-term newborn is delivered limp and apneic. Initial drying and warming steps are completed. Despite 30 seconds of effective positive pressure ventilation (PPV) via a T-piece resuscitator with confirmed bilateral chest expansion, the infant's heart rate remains 48 beats/min. According to European and Swiss Resuscitation Council neonatal guidelines, what is the mandatory next step?

  1. Administer rapid intravenous sodium bicarbonate bolus via peripheral vein
  2. Discontinue ventilation and perform immediate direct laryngoscopy to suction the stomach
  3. Initiate synchronized chest compressions at a 3:1 compression-to-ventilation ratio with 100% supplemental oxygen and prepare for vascular access and emergency medications Correct answer.
  4. Administer high-dose intramuscular naloxone

Explanation

In neonatal resuscitation, if the heart rate remains below 60 beats/min despite 30 seconds of adequate positive pressure ventilation with chest movement, chest compressions must be initiated immediately at a 3:1 ratio (90 compressions and 30 inflations per minute). The fraction of inspired oxygen (FiO2) should be increased to 100% (1.0), and umbilical venous catheterization should be prepared for emergency epinephrine administration if bradycardia persists.

Question 16Neonatal adaptation, prematurity and early illness

A premature infant has a surfactant-deficient respiratory distress pattern. Despite appropriate CPAP, the infant meets the responsible neonatal service criteria for rescue surfactant. Which listed combination addresses noninvasive support and appropriately selected surfactant treatment?

  1. Early continuous positive airway pressure (CPAP) and less-invasive surfactant administration (LISA / INSURE) with natural porcine or bovine surfactant Correct answer.
  2. High-frequency oscillatory ventilation (HFOV) combined with immediate systemic dexamethasone
  3. Broad-spectrum intravenous fluoroquinolones and fluid restriction to 30 mL/kg/day
  4. Routine immediate endotracheal intubation, deep sedation, muscle paralysis, and aggressive suctioning

Explanation

Neonatal respiratory distress syndrome (RDS) is caused by developmental deficiency of pulmonary surfactant in preterm infants. The standard modern approach emphasizes non-invasive respiratory support with early continuous positive airway pressure (CPAP, 6 to 8 cmH2O) combined with less-invasive surfactant administration (LISA / MIST) or INSURE (intubate-surfactant-extubate) to restore alveolar surface tension while minimizing mechanical barotrauma and bronchopulmonary dysplasia.

Question 17Neonatal adaptation, prematurity and early illness

A term infant delivered by elective cesarean section without prior labor develops tachypnea (respiratory rate 82 breaths/min), mild intercostal retractions, and nasal flaring at 2 hours of life. Chest radiography demonstrates prominent perihilar streaking, fluid within the horizontal interlobar fissure, and mild lung hyperinflation. Blood gas shows mild respiratory acidosis with normal lactate. What is the underlying pathophysiology and expected clinical course of this condition?

  1. Congenital diaphragmatic defect causing herniation of bowel; requires emergency laparotomy within 6 hours
  2. Delayed clearance of fetal alveolar fluid due to lack of catecholamine-mediated sodium channel (ENaC) activation; self-limiting condition (Transient Tachypnea of the Newborn) resolving within 24 to 72 hours with supportive care Correct answer.
  3. Severe surfactant inactivation secondary to intrauterine Listeria monocytogenes pneumonia; requires 21 days of IV ampicillin
  4. Primary structural pulmonary hypoplasia resulting from prolonged oligohydramnios; requires permanent home mechanical ventilation

Explanation

Transient Tachypnea of the Newborn (TTN) results from delayed resorption and clearance of fetal lung liquid by pulmonary epithelial sodium channels (ENaC) and lymphatic channels, commonly seen in infants delivered by elective cesarean delivery without labor. It is a benign, self-limiting condition characterized by perihilar vascular congestion and fluid in the fissures on chest radiograph, typically resolving spontaneously within 24 to 72 hours under supportive care (supplemental oxygen or low-level CPAP).

Question 18Neonatal adaptation, prematurity and early illness

A term infant with a positive direct antiglobulin test has unconjugated hyperbilirubinemia above the supplied appropriate age-, gestation- and risk-adjusted phototherapy line, without an exchange indication or acute encephalopathy. Which listed treatment addresses this supplied threshold assessment?

  1. Initiate intensive phototherapy using high-intensity blue-green light (wavelength 460 to 490 nm) and ensure adequate enteral hydration Correct answer.
  2. Perform immediate double-volume exchange transfusion via the umbilical vein
  3. Discontinue breastfeeding permanently and prescribe oral phenobarbital
  4. Reassure the parents that this is physiologic breast milk jaundice and discharge without therapy

Explanation

In ABO incompatibility with immune hemolytic hyperbilirubinemia exceeding the hour-specific phototherapy threshold, intensive phototherapy (delivering irradiance >=30 µW/cm²/nm at 460–490 nm) is the primary first-line intervention. Phototherapy converts unconjugated bilirubin via photoisomerization and structural isomerization into water-soluble lumirubin, which is excreted in bile and urine without requiring hepatic glucuronidation.

Question 19Neonatal adaptation, prematurity and early illness

A 3-day-old term infant with severe Rh(D) isoimmunization hemolytic disease develops lethargy, a high-pitched cry, abnormal posturing with retrocollis and opisthotonus, and a total serum bilirubin of 440 µmol/L (25.7 mg/dL) despite 8 hours of intensive phototherapy. What is the immediate life-saving procedure indicated to prevent irreversible kernicterus (chronic bilirubin encephalopathy)?

  1. Intravenous push of 20% mannitol bolus
  2. Emergency decompressive craniectomy
  3. Double-volume exchange transfusion (160 to 180 mL/kg) using compatible crossmatched reconstituted blood via umbilical vascular access Correct answer.
  4. Total parenteral nutrition with complete enteric rest

Explanation

This neonate demonstrates intermediate-to-advanced signs of acute bilirubin encephalopathy (ABE: high-pitched cry, retrocollis, opisthotonus, extreme hyperbilirubinemia). Emergent double-volume exchange transfusion (160 to 180 mL/kg) is mandatory to rapidly remove circulating unconjugated bilirubin, clear maternal Rh antibodies, and replace sensitized erythrocytes with antigen-negative red cells to prevent permanent basal ganglia and auditory brainstem kernicterus.

Question 20Neonatal adaptation, prematurity and early illness

A 5-week-old term infant presents with persistent jaundice, pale acholic (clay-colored) stools, and dark urine. Laboratory testing reveals total serum bilirubin 118 µmol/L, direct (conjugated) bilirubin 74 µmol/L (elevated), and elevated serum gamma-glutamyl transferase (GGT). Abdominal ultrasonography reveals an absent gallbladder and a fibrous triangular cord sign at the porta hepatis. What is the critical timeline and intervention for this condition?

  1. Lifelong oral ursodeoxycholic acid therapy with postponement of any surgical intervention until adolescence
  2. Urgent referral for intraoperative cholangiography and Kasai portoenterostomy (hepatoportoenterostomy), ideally performed before 45 to 60 days of life Correct answer.
  3. Administration of high-dose intravenous immunoglobulin and observation for spontaneous recanalization
  4. Liver biopsy at 6 months of age followed by elective cholecystectomy

Explanation

Biliary atresia is a progressive fibro-obliterative cholangiopathy of the extrahepatic biliary tree presenting with conjugated (cholestatic) hyperbilirubinemia and acholic stools. Timely surgical restoration of bile flow via Kasai hepatoportoenterostomy before 45 to 60 days of life is critical to achieve bile drainage and prevent progression to irreversible biliary cirrhosis, portal hypertension, and premature liver failure.

Question 21Neonatal adaptation, prematurity and early illness

A preterm infant undergoing medical treatment for necrotizing enterocolitis shows acute clinical deterioration with refractory septic shock, abdominal wall erythema, and blueish discoloration. A cross-table lateral abdominal radiograph demonstrates free intraperitoneal air beneath the anterior abdominal wall (pneumoperitoneum / Bell Stage IIIB). What is the mandatory next step in management?

  1. Initiation of oral activated charcoal to decontaminate the bowel lumen
  2. Immediate emergency pediatric surgical intervention (exploratory laparotomy with bowel resection and enterostomy, or primary peritoneal drainage) Correct answer.
  3. Increase positive end-expiratory pressure (PEEP) on the mechanical ventilator to 18 cmH2O
  4. Perform ultrasound-guided paracentesis and inject intraperitoneal gentamicin

Explanation

Pneumoperitoneum (free intraperitoneal air indicating gastrointestinal perforation) represents Bell Stage IIIB necrotizing enterocolitis and is the absolute, definitive indication for emergency surgical intervention. Surgical options include exploratory laparotomy with resection of necrotic bowel segments and exteriorization (enterostomy) or primary peritoneal bedside drainage in extremely low birth weight infants who are too unstable for operating room transfer.

Question 22Neonatal adaptation, prematurity and early illness

A premature male infant born at 25 weeks of gestation has required supplemental oxygen and non-invasive respiratory support since birth. At 36 weeks post-menstrual age (PMA), he continues to require an FiO2 of 0.30 via low-flow nasal cannula to maintain target oxygen saturation. Chest radiography demonstrates diffuse hyperinflation with scattered coarse reticular densities and areas of atelectasis. What is the formal diagnosis?

  1. Severe cystic fibrosis exacerbation
  2. Acute viral laryngotracheobronchitis
  3. Bronchopulmonary dysplasia (BPD / chronic lung disease of prematurity) Correct answer.
  4. Congenital lobar emphysema

Explanation

Bronchopulmonary dysplasia (BPD) is defined as persistent respiratory support or supplemental oxygen requirement for at least 28 days and at 36 weeks post-menstrual age (PMA) in infants born at <32 weeks gestation. Pathologically, 'new BPD' is characterized by alveolar simplification, impaired vascular development, and variable fibrosis resulting from premature lung injury, hyperoxia, barotrauma/volutrauma, and inflammation.

Question 23Neonatal adaptation, prematurity and early illness

A routine screening cranial ultrasound performed on day 4 of life in a 26-week preterm infant reveals an echogenic hemorrhage originating in the germinal matrix with extensive intraventricular extension filling more than 50% of the lateral ventricle, accompanied by acute ventricular dilation. According to the Papile classification of intraventricular hemorrhage (IVH), which grade is this?

  1. Grade I Intraventricular Hemorrhage
  2. Grade II Intraventricular Hemorrhage
  3. Grade IV Intraventricular Hemorrhage
  4. Grade III Intraventricular Hemorrhage Correct answer.

Explanation

According to the Papile classification: Grade I is isolated germinal matrix hemorrhage (subependymal); Grade II is intraventricular hemorrhage without ventricular dilation (filling <50% of ventricle); Grade III is intraventricular hemorrhage with acute ventricular dilation (filling >50% of ventricle); and Grade IV is intraventricular hemorrhage with parenchymal venous hemorrhagic infarction.

Question 24Neonatal adaptation, prematurity and early illness

A preterm infant born at 30 weeks gestation experiences frequent episodes of central apnea lasting >20 seconds accompanied by bradycardia (HR <80 bpm) and desaturations (SpO2 <80%). Sepsis, intracranial hemorrhage, anemia, and electrolyte disturbances have been excluded. What is the evidence-based first-line pharmacological treatment for apnea of prematurity (AOP)?

  1. Caffeine citrate (oral or intravenous loading dose 20 mg/kg, followed by maintenance 5 to 10 mg/kg/day) Correct answer.
  2. Intravenous diazepam infusion
  3. Oral clonidine solution
  4. Subcutaneous octreotide continuous infusion

Explanation

Caffeine citrate is the undisputed first-line methylxanthine for apnea of prematurity. As a non-selective antagonist of adenosine A1 and A2A receptors in the brainstem respiratory center, caffeine increases central respiratory drive, enhances diaphragmatic contractility, improves hypercapnic sensitivity, reduces the need for mechanical ventilation, and improves long-term neurodevelopmental and pulmonary outcomes (CAP trial).

Question 25Neonatal adaptation, prematurity and early illness

A 10-hour-old term infant of a mother with gestational diabetes is jittery, irritable, hypotonic, and feeding poorly. Bedside point-of-care capillary glucose is 1.4 mmol/L (25 mg/dL), confirmed by venous laboratory measurement at 1.5 mmol/L (27 mg/dL). What is the immediate treatment of choice for symptomatic neonatal hypoglycemia?

  1. Oral administration of 50 mL of undiluted honey
  2. Intramuscular glucagon injection without intravenous glucose
  3. An immediate intravenous bolus of 2 mL/kg of 10% dextrose in water (D10W = 200 mg/kg) over 5 to 10 minutes, followed by a continuous glucose infusion rate (GIR) of 6 to 8 mg/kg/min Correct answer.
  4. Intravenous infusion of 50% dextrose (D50W) at 10 mL/kg

Explanation

Symptomatic neonatal hypoglycemia (<2.0–2.2 mmol/L / <36–40 mg/dL) mandates an immediate IV bolus of 2 mL/kg of 10% dextrose (D10W) followed by a continuous glucose infusion at 6 to 8 mg/kg/min to restore and maintain normoglycemia (>2.5–3.0 mmol/L), protecting the developing brain from hypoglycemic neuronal injury.

Question 26Neonatal adaptation, prematurity and early illness

A newborn delivered at 38 weeks gestation presents immediately after birth with excessive frothy oral secretions, choking, and cyanotic coughing episodes during the initial attempt to feed. A radiopaque 8 Fr feeding tube cannot be advanced beyond 10 cm from the gums. A chest and abdominal plain radiograph reveals the tube curled in a blind upper esophageal pouch, with abundant air visible throughout the stomach and intestines. What is the specific anatomical anomaly?

  1. Isolated esophageal atresia without tracheoesophageal fistula (Type A)
  2. Esophageal atresia with distal tracheoesophageal fistula (Gross Type C / Vogt Type IIIb) Correct answer.
  3. Isolated H-type tracheoesophageal fistula without atresia (Type E)
  4. Esophageal atresia with proximal tracheoesophageal fistula (Type B)

Explanation

Esophageal atresia with distal tracheoesophageal fistula (Gross Type C / Vogt Type IIIb) is by far the most common congenital esophageal anomaly (accounting for ~85% of cases). The blind upper esophageal pouch prevents passage of the catheter and causes saliva accumulation, while the distal fistula connecting the trachea to the lower esophagus allows air to enter the stomach and intestines on plain radiography.

Question 27Childhood respiratory and cardiovascular disease

A 10-year-old girl with a known history of severe persistent asthma presents with an acute severe exacerbation. Despite 3 consecutive nebulized doses of salbutamol (5 mg) plus ipratropium bromide (0.5 mg) and intravenous methylprednisolone (2 mg/kg), she remains in severe distress with a silent chest on auscultation, peak expiratory flow <40% predicted, oxygen saturation 89% on room air, and exhaustion. Which second-line intravenous bronchodilator should be administered next?

  1. Intravenous theophylline bolus over 5 minutes
  2. Subcutaneous terbutaline continuous infusion without telemetry
  3. Intravenous magnesium sulfate (40 to 50 mg/kg, max 2 g) infused over 20 to 30 minutes Correct answer.
  4. Intravenous furosemide bolus (1 mg/kg)

Explanation

Intravenous magnesium sulfate (40 to 50 mg/kg, maximum 2 g) is the standard second-line agent for acute severe or life-threatening asthma refractory to initial inhaled SABA, ipratropium, and systemic corticosteroids. It acts by inhibiting calcium influx into airway smooth muscle cells, promoting rapid bronchial relaxation with a proven record of improving pulmonary function and preventing intubation.

Question 28Childhood respiratory and cardiovascular disease

A 3-year-old child presents with high remittent fever for 6 consecutive days, bilateral non-exudative bulbar conjunctival injection, cracked erythematous lips with strawberry tongue, indurative erythema of the hands and feet, a polymorphous macular trunk rash, and a unilateral cervical lymph node measuring 2.2 cm. Complete Kawasaki disease is diagnosed. What is the evidence-based first-line therapy to prevent coronary artery aneurysms?

  1. Single high-dose intravenous immunoglobulin (IVIG, 2 g/kg infused over 10 to 12 hours) combined with oral acetylsalicylic acid (aspirin) Correct answer.
  2. Intravenous methylprednisolone pulse monotherapy (30 mg/kg/day) for 3 days without IVIG
  3. Intravenous broad-spectrum penicillin combined with oral ibuprofen
  4. Oral acyclovir suspension with supportive antipyretics

Explanation

Kawasaki disease is an acute systemic medium-vessel vasculitis. Administration of a single high-dose infusion of IVIG (2 g/kg) within the first 10 days of fever onset, combined with anti-inflammatory/antiplatelet aspirin, reduces the risk of coronary artery aneurysms from 20–25% down to less than 3–5%. Baseline echocardiography is mandatory to evaluate coronary artery dimensions (z-scores).

Question 29Childhood respiratory and cardiovascular disease

A 7-year-old child with persistent asthma experiences daytime symptoms (cough and wheezing) 3 to 4 days per week, awakens at night due to coughing twice per month, and uses a short-acting beta-2 agonist (SABA) 3 times weekly. Spirometry reveals an FEV1 of 84% predicted with a 14% improvement post-bronchodilator. According to the Swiss Paediatric Pulmonology Society and GINA guidelines (Step 2), what is the preferred daily maintenance controller therapy?

  1. Daily low-dose inhaled corticosteroid (ICS, e.g., fluticasone propionate or budesonide) via a metered-dose inhaler with spacer, plus as-needed inhaled SABA Correct answer.
  2. High-dose oral prednisone daily for 6 months
  3. As-needed inhaled short-acting beta-2 agonist (SABA) monotherapy without any anti-inflammatory controller
  4. Daily long-acting beta-2 agonist (LABA) monotherapy without inhaled corticosteroids

Explanation

In children aged 6 to 11 years with Step 2 persistent asthma, the standard first-line maintenance therapy is a daily low-dose inhaled corticosteroid (ICS) combined with an as-needed rapid-acting bronchodilator (SABA) for breakthrough symptoms. Regular low-dose ICS suppresses chronic eosinophilic airway inflammation, significantly reducing exacerbation risk, emergency visits, and bronchial hyperresponsiveness.

Question 30Childhood respiratory and cardiovascular disease

A 5-month-old infant presents during mid-winter with a 2-day history of clear rhinorrhea, low-grade fever (38.1°C), tachypnea (respiratory rate 64 breaths/min), bilateral expiratory wheezing, fine inspiratory crackles, and mild intercostal retractions. Oxygen saturation is 94% on room air and the infant is drinking 70% of normal feeds. According to Swiss Paediatric Society and international consensus guidelines for acute viral bronchiolitis, what is the primary management approach?

  1. Immediate initiation of intravenous amoxicillin-clavulanic acid and oral prednisolone
  2. Nebulized salbutamol every 2 hours combined with chest physiotherapy percussion
  3. Supportive care consisting of gentle nasal suctioning, fractionated enteral feeds/hydration maintenance, minimal handling, and avoidance of routine bronchodilators, corticosteroids, and antibiotics Correct answer.
  4. Continuous nebulized hypertonic 7% saline every hour with broad-spectrum macrolides

Explanation

Acute viral bronchiolitis (most commonly RSV) is a self-limiting lower respiratory tract infection characterized by bronchiolar epithelial necrosis, edema, and mucus plugging. High-quality evidence demonstrates that routine bronchodilators, systemic/inhaled corticosteroids, antibiotics, and chest physiotherapy do NOT improve outcomes or shorten hospitalization. Management is fundamentally supportive: ensuring adequate hydration, gentle upper airway clearance, and supplemental oxygen only if SpO2 drops consistently below 90–92%.

Question 31Childhood respiratory and cardiovascular disease

A 6-month-old infant with unrepaired Tetralogy of Fallot develops sudden severe distress during blood drawing: vigorous crying, hyperpnea, rapidly deepening central cyanosis (SpO2 drops from 85% to 58%), and the previously loud systolic ejection murmur becomes almost completely inaudible. What is the immediate bedside emergency management for this hypercyanotic ('tet') spell?

  1. Place the infant in the knee-to-chest position, administer high-flow 100% oxygen, and provide subcutaneous or intravenous morphine sulfate Correct answer.
  2. Place the infant immediately in a Trendelenburg position and infuse 50 mL/kg of 0.45% saline rapidly
  3. Administer high-dose inhaled salbutamol and intravenous furosemide
  4. Perform immediate emergency electrical cardioversion at 2 J/kg

Explanation

A hypercyanotic 'tet' spell is caused by infundibular subpulmonary muscular spasm and/or sudden decrease in systemic vascular resistance (SVR), causing massive right-to-left shunting across the VSD and bypassing the pulmonary circulation (softening the murmur). The knee-to-chest position immediately increases SVR (reducing right-to-left shunt), oxygen promotes pulmonary vasodilation, and morphine calms the infant and suppresses the hyperpneic respiratory drive.

Question 32Childhood respiratory and cardiovascular disease

A term newborn infant presents at 4 hours of life with profound central cyanosis (SpO2 62%) that fails to improve after 10 minutes of 100% oxygen administration via hood (hyperoxia test negative). Echocardiography confirms D-transposition of the great arteries (D-TGA) with an intact ventricular septum and a restrictive patent foramen ovale. What is the immediate life-saving medical and interventional strategy?

  1. High-dose intravenous indomethacin infusion to close the ductus arteriosus
  2. Immediate surgical correction with the Fontan procedure within 2 hours
  3. Continuous intravenous infusion of Prostaglandin E1 (alprostadil, 0.01 to 0.05 µg/kg/min) to maintain ductal patency, followed by urgent balloon atrial septostomy (Rashkind procedure) to ensure adequate interatrial mixing Correct answer.
  4. High-frequency oscillatory ventilation with 100% oxygen combined with systemic anticoagulation alone, recruiting the lung to raise the arterial saturation while awaiting spontaneous ductal closure

Explanation

In D-TGA, the systemic and pulmonary circulations run in parallel rather than in series, creating incompatible life physiology unless parallel circuits communicate. Prostaglandin E1 (alprostadil) maintains patency of the ductus arteriosus, and urgent balloon atrial septostomy (Rashkind procedure) creates an unrestrictive interatrial communication to allow oxygenated pulmonary venous blood to mix with the systemic circulation prior to definitive arterial switch surgery (Jatene procedure).

Question 33Childhood respiratory and cardiovascular disease

A 10-day-old infant is brought to the resuscitation room in severe cardiogenic shock with profound metabolic acidosis (lactate 11 mmol/L). Physical examination reveals strong, bounding radial pulses but absent femoral pulses, cool lower extremities, and an upper-to-lower extremity systolic blood pressure gradient of 35 mmHg. Critical coarctation of the aorta is diagnosed following spontaneous closure of the ductus arteriosus. What is the immediate life-saving pharmacotherapy?

  1. Intravenous furosemide bolus (2 mg/kg)
  2. Intravenous calcium gluconate bolus
  3. High-dose oral propranolol
  4. Continuous intravenous infusion of Prostaglandin E1 (alprostadil, 0.02 to 0.05 µg/kg/min) Correct answer.

Explanation

In critical coarctation of the aorta (a duct-dependent systemic circulatory lesion), systemic perfusion to the lower body and kidneys depends entirely on right-to-left flow across the ductus arteriosus. When the ductus closes physiologically in the first 1 to 2 weeks of life, severe lower-body hypoperfusion, renal failure, and cardiogenic shock ensue. Immediate infusion of Prostaglandin E1 (alprostadil) reopens the ductus arteriosus, restoring systemic perfusion and reversing acidosis before definitive surgical coarctation repair.

Question 34Childhood respiratory and cardiovascular disease

A 3-year-old child presents to the emergency room with sudden-onset palpitations and pallor. A 12-lead ECG demonstrates a regular, narrow-complex tachycardia at a rate of 240 beats/min with absent or retrogradely inverted P waves (Supraventricular Tachycardia, SVT). The child is alert, has normal peripheral pulses, capillary refill time of 1.5 seconds, and a blood pressure of 96/60 mmHg (hemodynamically STABLE SVT). What is the appropriate sequential management?

  1. Initiate vagal maneuvers (e.g., application of a bag of crushed ice water to the face for 10 to 15 seconds); if unsuccessful, administer a rapid intravenous push of adenosine (0.1 mg/kg, max 6 mg) followed by an immediate rapid saline flush Correct answer.
  2. Perform immediate unsynchronised defibrillation at 4 J/kg, since any tachycardia above 220 beats per minute in a child requires electrical termination
  3. Administer oral digoxin and discharge home with cardiology follow-up in two weeks, because paroxysmal supraventricular tachycardia in a well child terminates spontaneously
  4. Start a continuous intravenous lidocaine infusion at 50 µg/kg/min to suppress the re-entrant circuit before attempting any vagal manoeuvre

Explanation

In a hemodynamically stable child with supraventricular tachycardia (SVT), non-pharmacological vagal maneuvers (specifically the diving reflex elicited by placing ice water/crushed ice in a glove/plastic bag over the upper face for 10–15 seconds without occluding the airway) should be attempted first. If ineffective, rapid IV push adenosine (0.1 mg/kg, followed by a rapid 5–10 mL saline flush via an IV line as close to the heart as possible) temporarily blocks AV nodal conduction, terminating reentrant tachycardias.

Question 35Childhood respiratory and cardiovascular disease

A 4-year-old child with narrow-complex supraventricular tachycardia (rate 260 bpm) presents with altered mental status (lethargy), mottled cool extremities, delayed capillary refill of 4.5 seconds, and an unrecordable peripheral blood pressure (hemodynamically UNSTABLE SVT). Vascular access is currently being attempted but is not yet established. What is the immediate treatment of choice?

  1. Perform bilateral carotid sinus massage for 60 seconds
  2. Administer high-dose oral sotalol via nasogastric tube
  3. Immediate synchronized electrical cardioversion starting at 0.5 to 1.0 J/kg (increasing to 2.0 J/kg if initial shock fails) Correct answer.
  4. Wait for central venous catheterization to be completed before any intervention

Explanation

In pediatric supraventricular tachycardia with hemodynamic instability (decompensated shock, hypotension, altered mental status, severe hypoperfusion), synchronized electrical cardioversion (0.5 to 1.0 J/kg, increasing to 2.0 J/kg if needed) is the immediate treatment of choice. Delivery of synchronized energy terminating at the R wave must not be delayed for intravenous access attempts.

Question 36Childhood respiratory and cardiovascular disease

A 3-year-old boy is diagnosed with Kawasaki disease on day 8 of fever. Transthoracic echocardiography reveals giant bilateral coronary artery aneurysms with a maximum internal lumen diameter of 8.5 mm and a z-score of +11.2 in the left anterior descending (LAD) coronary artery. In addition to high-dose IVIG and anti-inflammatory aspirin, what long-term antithrombotic management is mandatory?

  1. Systemic therapeutic anticoagulation (low-molecular-weight heparin or warfarin titrated to target INR 2.0 to 3.0) combined with low-dose antiplatelet aspirin Correct answer.
  2. No antithrombotic therapy because aneurysms spontaneously regress within 1 month
  3. High-dose systemic thrombolytic therapy with alteplase daily for 1 year
  4. Lifelong dual calcium channel blocker monotherapy

Explanation

Giant coronary artery aneurysms in Kawasaki disease (defined as internal diameter >=8 mm or z-score >=10) carry an extreme risk of sluggish intracavitary blood flow, mural thrombosis, acute myocardial infarction, and sudden death. Long-term management requires dual antithrombotic therapy combining systemic anticoagulation (therapeutic LMWH or warfarin targeting INR 2.0–3.0) with low-dose antiplatelet aspirin (3–5 mg/kg/day).

Question 37Childhood respiratory and cardiovascular disease

A 12-year-old boy presents with chronic daily productive wet cough since early infancy, recurrent bilateral purulent otitis media, chronic pansinusitis, and bronchiectasis on high-resolution chest CT. Chest and abdominal imaging reveal complete situs inversus (dextrocardia with inverted abdominal viscera / Kartagener triad). What diagnostic screening test has high sensitivity and specificity for Primary Ciliary Dyskinesia (PCD)?

  1. Serum immunoglobulin IgE quantification
  2. Skin prick testing to tree pollen and dust mites
  3. Measurement of nasal nitric oxide (nNO) production (demonstrating extremely low or undetectable levels) Correct answer.
  4. Gastric acid pH monitoring

Explanation

Low nasal nitric oxide can support a primary-ciliary-dyskinesia assessment under the appropriate age, method and eligibility conditions. It is a screening contribution rather than a complete independent diagnosis; repeat assessment and other specialist tests may be needed, and conditions such as cystic fibrosis affect interpretation. Normal or abnormal situs alone does not settle every case.

Question 38Pediatric infection and immune disorders

A symptomatic newborn has suspected early-onset bacterial sepsis. Under a specified empirical pathway targeting GBS, Listeria and susceptible Gram-negative organisms, with local resistance reviewed, which listed initial antibiotic pair is commonly used?

  1. Intravenous vancomycin plus meropenem
  2. Oral ciprofloxacin plus fluconazole
  3. Intravenous cefazolin monotherapy
  4. Intravenous ampicillin (covering Group B Streptococcus and Listeria monocytogenes) plus gentamicin (covering Escherichia coli and other Gram-negative bacilli) Correct answer.

Explanation

Early-onset neonatal sepsis (<72 hours of life) is predominantly caused by pathogens acquired vertically from the maternal genital tract, most frequently Streptococcus agalactiae (Group B Streptococcus, GBS) and Escherichia coli, followed by Listeria monocytogenes. The gold-standard empiric regimen is intravenous ampicillin (providing bactericidal activity against GBS, Listeria, and enterococci) combined with an aminoglycoside such as gentamicin (providing synergistic Gram-negative coverage against E. coli).

Question 39Pediatric infection and immune disorders

An 18-day-old full-term infant is brought to the pediatric emergency department with a documented rectal temperature of 38.6°C. The infant is alert, feeds reasonably well, and physical examination reveals no focal source of infection. According to Swiss and international pediatric guidelines for fever without source in neonates (<28 days of age), what is the mandatory diagnostic and therapeutic approach?

  1. Perform a complete sepsis evaluation (blood culture, catheterized urinalysis and urine culture, and lumbar puncture for CSF analysis and culture), admit to the hospital, and initiate empiric intravenous ampicillin plus an aminoglycoside (or cefotaxime) Correct answer.
  2. Discharge home with oral amoxicillin and scheduled outpatient clinical review in 48 hours
  3. Perform a rapid nasal viral PCR panel; if it is positive for rhinovirus, discharge home without further testing or antibiotics, since a documented viral infection excludes concurrent invasive bacterial infection at this age
  4. Perform urine dipstick only; if negative, reassure parents and advise oral paracetamol as needed

Explanation

All febrile neonates <=28 days of age are at high risk (>10%) for invasive bacterial infections (IBI: bacteremia, meningitis, urinary tract infection) caused by GBS, E. coli, or Listeria monocytogenes due to immature immune defenses. Clinical appearance is unreliable in this age group; therefore, a full sepsis workup including lumbar puncture, mandatory hospital admission, and immediate parenteral broad-spectrum antibiotic therapy is non-negotiable.

Question 40Pediatric infection and immune disorders

A newborn infant fails the universal newborn hearing screening bilaterally. Cranial ultrasonography reveals periventricular calcifications, mild ventriculomegaly, and lenticulostriate vasculopathy. The infant is small for gestational age with microcephaly and mild hepatosplenomegaly. Congenital Cytomegalovirus (cCMV) infection is suspected. What is the gold standard diagnostic method to confirm congenital CMV infection?

  1. Detection of CMV DNA via real-time Polymerase Chain Reaction (PCR) in urine or saliva collected within the first 2 to 3 weeks of life Correct answer.
  2. Measurement of maternal CMV IgG titer at 6 months postpartum
  3. Serum anti-CMV IgE antibody quantification at 1 year of age
  4. Direct visualization of inclusion bodies in peripheral blood eosinophils

Explanation

CMV testing in the first approximately three weeks can help distinguish congenital from later infection, interpreted with the actual specimen and assay. Urine PCR is used for confirmation, and a positive saliva screen is generally confirmed with an appropriate urine specimen because contamination can occur. Test timing, clinical findings and the full diagnostic pathway remain important.

Question 41Pediatric infection and immune disorders

Newborn bloodspot screening (Guthrie card) in Switzerland detects undetectable T-cell receptor excision circles (TRECs), indicating severe T-cell lymphopenia. Confirmatory flow cytometry demonstrates absent circulating CD3+ T cells and profoundly depressed lymphocyte proliferation. Severe Combined Immunodeficiency (SCID) is diagnosed. What are the immediate mandatory protective measures and definitive curative therapy?

  1. Immediate administration of routine live rotavirus and BCG vaccines, followed by outpatient observation
  2. High-dose aspirin therapy and standard non-irradiated blood transfusions, with live vaccines withheld only until the confirmatory genotype becomes available
  3. Oral iron supplementation and avoidance of breast milk, since maternal lymphocytes transferred in milk are the principal cause of graft-versus-host disease in these infants
  4. Strict protective isolation, absolute contraindication to all live-attenuated vaccines, use of irradiated CMV-negative leukodepleted blood products, Pneumocystis jirovecii prophylaxis (cotrimoxazole), and urgent referral for allogeneic hematopoietic stem cell transplantation (HSCT) or gene therapy Correct answer.

Explanation

SCID is a pediatric immunologic emergency characterized by absent adaptive cellular and humoral immunity. Immediate survival depends on strict protective isolation, immediate avoidance of live vaccines (rotavirus, BCG, MMR), prophylaxis against opportunistic infections (cotrimoxazole for Pneumocystis jirovecii and fluconazole), CMV-safe irradiated leukoreduced blood products (to prevent fatal transfusion-associated graft-versus-host disease), and urgent allogeneic HSCT or autologous gene therapy.

Question 42Pediatric infection and immune disorders

A 4-year-old boy presents with recurrent deep-seated abscesses of the liver, lungs, and cervical lymph nodes, and suppurative osteomyelitis. Cultures consistently isolate catalase-positive organisms, including Staphylococcus aureus, Serratia marcescens, and Aspergillus fumigatus. Chronic Granulomatous Disease (CGD) is suspected. What is the gold standard diagnostic assay used to confirm the diagnosis?

  1. Serum total IgE quantification and sweat chloride test
  2. Dihydrorhodamine 123 (DHR) flow cytometry assay (evaluating neutrophil oxidative burst upon phorbol myristate acetate stimulation) Correct answer.
  3. Complement hemolytic 50 (CH50) functional assay
  4. Polymerase chain reaction for Epstein-Barr virus DNA in plasma

Explanation

Chronic Granulomatous Disease (CGD) results from genetic mutations in subunits of the NADPH oxidase complex (gp91phox, p47phox, etc.), disabling the generation of superoxide radicals and reactive oxygen species required for intracellular killing of phagocytosed catalase-positive bacteria and fungi. The gold standard diagnostic test is the DHR 123 flow cytometry assay, which demonstrates absent or severely blunted neutrophil respiratory burst.

Question 43Pediatric infection and immune disorders

In a neonate with confirmed parechovirus central nervous system infection, which listed pattern can occur and explains why a normal CSF white-cell count does not exclude serious illness?

  1. Severe sepsis-like encephalitis and extensive white matter injury (periventricular leukomalacia on MRI) occurring typically in the absence of CSF pleocytosis Correct answer.
  2. Massive neutrophilic pleocytosis (>5,000 WBC/µL) with thick basilar arachnoid exudates
  3. Extensive bilateral temporal lobe hemorrhagic necrosis identical to HSV-1
  4. Multiple ring-enhancing calcified granulomas in the basal ganglia

Explanation

Neonatal parechovirus infection can cause severe sepsis-like illness and central nervous system involvement with little or no CSF pleocytosis. White-matter injury can occur but is not obligatory in every case, and absence of pleocytosis is not unique to this virus. Interpret the molecular results and complete clinical and imaging assessment.

Question 44Nutrition, gastrointestinal, kidney and endocrine disorders

An 11-year-old girl is diagnosed with active luminal pediatric Crohn disease involving the terminal ileum and ascending colon (Paris classification A1b, L1, B1, G0). She presents with chronic abdominal pain, weight loss, and marked growth deceleration. According to ECCO/ESPGHAN pediatric guidelines, what is the recommended first-line therapy for inducing clinical and mucosal remission in pediatric luminal Crohn disease?

  1. Exclusive Enteral Nutrition (EEN) using a complete liquid polymeric formula as 100% of daily caloric intake for 6 to 8 weeks Correct answer.
  2. Immediate surgical right hemicolectomy and ileostomy creation
  3. Lifelong high-dose intravenous hydrocortisone monotherapy
  4. Long-term oral metronidazole monotherapy without nutritional therapy

Explanation

Exclusive Enteral Nutrition (EEN) involving 100% liquid formula feeds for 6 to 8 weeks is the preferred first-line therapy for inducing clinical and mucosal remission in pediatric luminal Crohn disease per ECCO/ESPGHAN guidelines. EEN achieves equal clinical remission rates to systemic corticosteroids (~80%) while delivering superior mucosal healing, reversing nutritional deficits, and avoiding corticosteroid-related growth failure and bone toxicity.

Question 45Nutrition, gastrointestinal, kidney and endocrine disorders

A 4-week-old male infant presents with a 1-week history of forceful, non-bilious projectile vomiting occurring immediately after every feeding. The infant is eagerly hungry after vomiting, has lost 250 g of weight, and physical examination reveals visible gastric peristaltic waves and a firm 2 cm mobile olive-shaped mass in the right epigastrium. What classical venous blood gas and serum electrolyte abnormalities characterize Infantile Hypertrophic Pyloric Stenosis (IHPS)?

  1. Hyperchloremic, hyperkalemic metabolic acidosis with high anion gap
  2. Normal electrolytes and normal arterial blood gas in 100% of cases
  3. Hypochloremic, hypokalemic metabolic alkalosis with elevated serum bicarbonate and compensatory respiratory hypoventilation Correct answer.
  4. Profound hyponatremia with hyperkalemia and severe metabolic acidosis

Explanation

Persistent vomiting of gastric hydrochloric acid (HCl) results in profound loss of hydrogen and chloride ions. To preserve intravascular volume against dehydration, the kidneys reabsorb sodium in exchange for potassium and hydrogen ions in the distal tubules (paradoxical aciduria), generating the classic biochemical profile of hypochloremic, hypokalemic metabolic alkalosis with elevated serum bicarbonate.

Question 46Nutrition, gastrointestinal, kidney and endocrine disorders

A 4-year-old girl with steroid-sensitive nephrotic syndrome currently experiencing an acute relapse (marked ascites, serum albumin 16 g/L) presents to the emergency department with high fever (39.0°C), diffuse abdominal pain, guarding, and rebound tenderness. Diagnostic paracentesis confirms spontaneous bacterial peritonitis (SBP). What is the most common causative pathogen responsible for primary peritonitis in pediatric nephrotic syndrome?

  1. Pseudomonas aeruginosa
  2. Mycobacterium tuberculosis
  3. Streptococcus pneumoniae (Pneumococcus) Correct answer.
  4. Candida albicans

Explanation

Children with nephrotic syndrome are highly susceptible to severe infections with encapsulated bacteria-predominantly Streptococcus pneumoniae (responsible for ~50% of spontaneous bacterial peritonitis cases)-due to urinary loss of immunoglobulins (IgG), loss of complement factor B and properdin (impairing opsonization), and ascitic fluid serving as an ideal culture medium.

Question 47Nutrition, gastrointestinal, kidney and endocrine disorders

A 6-year-old boy presents with symmetrical palpable non-thrombocytopenic purpura over the extensor surfaces of the lower extremities and buttocks, bilateral ankle arthralgia, colicky abdominal pain, and microscopic hematuria with mild proteinuria on dipstick analysis. Henoch-Schönlein Purpura (IgA Vasculitis) is diagnosed. What is the single most important determinant of long-term morbidity and prognosis that requires structured clinical and laboratory surveillance for at least 6 to 12 months?

  1. The duration and intensity of the knee arthralgia
  2. The presence, severity, and histological progression of renal involvement (IgA vasculitis nephritis) Correct answer.
  3. The frequency of recurrent skin purpura crops during the first 2 weeks
  4. The serum antistreptolysin O (ASO) titer

Explanation

While joint and skin symptoms in IgA vasculitis (Henoch-Schönlein purpura) resolve spontaneously without long-term sequelae, renal involvement (IgA vasculitis nephritis) is the primary determinant of long-term prognosis, with up to 1–5% of affected children developing chronic kidney disease or hypertension. Regular monitoring of blood pressure, urinalysis, and spot urine protein-to-creatinine ratio is mandatory for at least 6 to 12 months after diagnosis.

Question 48Nutrition, gastrointestinal, kidney and endocrine disorders

A newborn has laboratory-confirmed primary congenital hypothyroidism with markedly elevated venous TSH and low free T4. Which listed timely treatment approach is indicated to support neurological development?

  1. Wait for clinical symptoms of cretinism (macroglossia, umbilical hernia) to develop before starting therapy
  2. Initiate oral potassium iodide drops at 6 months of age
  3. Initiate oral levothyroxine (L-thyroxine, 10 to 15 µg/kg/day) immediately within the first 2 weeks of life Correct answer.
  4. Perform immediate thyroidectomy followed by external neck radiation

Explanation

Congenital hypothyroidism is one of the most common preventable causes of severe neurodevelopmental disability (intellectual disability / cretinism). Thyroid hormones are essential for early cerebral myelination, synaptogenesis, and neuronal migration. Initiating high-dose oral levothyroxine (10 to 15 µg/kg/day) within the first 2 weeks of life completely prevents cognitive impairment and ensures normal IQ development.

Question 49Nutrition, gastrointestinal, kidney and endocrine disorders

A previously healthy 2-year-old boy presents with sudden, painless, massive rectal bleeding characterized by dark red / maroon-colored stools (hematochezia). He is hemodynamically stable, his abdomen is completely soft, non-distended, and non-tender, and physical examination is otherwise unremarkable. Hemoglobin is 92 g/L. What is the most likely diagnosis and what is the definitive nuclear medicine diagnostic scan?

  1. Bleeding Meckel Diverticulum containing heterotopic gastric mucosa; confirm with Technetium-99m (99mTc) pertechnetate scintigraphy (Meckel scan) Correct answer.
  2. Active ulcerative colitis; confirm with immediate total colonoscopy and full mucosal biopsies
  3. Henoch-Schönlein purpura; confirm with renal biopsy
  4. Intestinal malrotation with midgut volvulus; confirm with upper GI series

Explanation

The supplied painless bleeding pattern suggests a Meckel diverticulum in the differential. Technetium pertechnetate scintigraphy can identify relevant ectopic gastric mucosa, but the test has limitations and a negative result does not exclude every lesion. Stabilization and further investigation depend on the actual bleeding and clinical findings.

Question 50Developmental, neurological and genetic reasoning

At a routine 18-month well-child developmental examination in Switzerland, a toddler cannot walk independently (only pulls to stand), does not speak any meaningful single words, does not respond reliably to his name, and does not point to communicate interest or needs (proto-declarative pointing). How should the pediatrician categorize these findings and proceed?

  1. Reassure the parents that boys naturally develop language and gross motor skills much later, and reassess at age 4 years
  2. Prescribe multivitamin supplements and recommend structured smartphone language applications at home, with a review at the next scheduled well-child visit
  3. Identify these findings as major developmental 'red flags' across gross motor, speech/language, and social-communication domains; initiate a formal audiological hearing evaluation, developmental assessment, and immediate referral to a specialized pediatric neurodevelopmental center (Zentrum für Entwicklungspädiatrie) Correct answer.
  4. Perform an immediate brain biopsy to exclude a leukodystrophy, since simultaneous motor and language delay at 18 months is diagnostic of progressive white-matter disease

Explanation

Key red flags at 18 months of age include: inability to walk independently (normal limit 15–18 months), absence of single words (normal milestone 10–14 words/single words by 12–15 months), failure to respond to name, and lack of pointing/joint attention (red flag for autism spectrum disorder). These findings mandate immediate objective hearing assessment (audiometry) and multidisciplinary referral to a specialized developmental pediatrics center (Entwicklungspädiatrie) for early intervention.