Pediatrics

A 6-month-old girl is brought to the pediatric emergency department by her parents due to worsening weakness and poor feeding over the past three days. Her parents report that she previously breastfed vigorously and was able to roll from front to back, but she has progressively lost these abilities. Additionally, she has not passed a stool in five days. The family lives on an older rural property and recently began excavating soil to build a large garden. On physical examination, the infant appears lethargic. Her temperature is 37.1°C (98.8°F), heart rate is 110/min, and respiratory rate is 28/min. Neurological evaluation reveals bilateral ptosis, sluggish pupillary light reflexes, and a weak gag reflex. There is profound generalized hypotonia with marked head lag, and deep tendon reflexes are absent bilaterally. Which of the following is the most appropriate initial treatment for this patient?

An $18$-month-old boy is brought to the emergency department in late January by his parents due to severe difficulty breathing. His parents report a $4$-day history of clear nasal discharge, a mild cough, and a low-grade fever. Over the past $12$ hours, his breathing has become increasingly rapid and labored, and he has been feeding poorly. He was born at full term and has no prior history of wheezing or atopic dermatitis. His $4$-year-old sister recently recovered from a mild 'cold'.

Vital signs are:
- Temperature: $38.2^\circ\text{C}$ ($100.8^\circ\text{F}$)
- Heart rate: $158\text{/min}$
- Respiratory rate: $64\text{/min}$
- Oxygen saturation: $87\%$ on room air

Physical examination reveals an irritable infant with significant nasal flaring and marked subcostal and intercostal retractions. Lung auscultation demonstrates a prolonged expiratory phase with diffuse bilateral wheezing and scattered coarse crackles. A rapid viral panel is positive for respiratory syncytial virus (RSV).

Which of the following interventions are appropriate components of the immediate management bundle for this patient? (Select all that apply)

A 9-year-old boy is admitted to the pediatric oncology unit with newly diagnosed T-cell acute lymphoblastic leukemia. He has a 2-week history of worsening fatigue, bone pain, and shortness of breath. Chest radiograph shows a large anterior mediastinal mass. Before the initiation of induction chemotherapy, laboratory studies are obtained:

An electrocardiogram shows normal sinus rhythm with no ST-segment or T-wave abnormalities. The patient does not exhibit tetany or seizures. Vigorous intravenous hydration with normal saline is initiated.

Which of the following is the most appropriate additional pharmacologic intervention for this patient's metabolic derangements?

Match each classic pediatric gastrointestinal clinical presentation with its most appropriate definitive procedural or surgical management.

A 10-day-old male neonate is brought to the emergency department by his parents due to extreme lethargy, poor feeding, and rapid breathing over the past 12 hours. Physical examination reveals a mottled, pale infant with marked intercostal retractions and grunting. Vital signs are temperature 36.5°C (97.7°F), heart rate 185/min, respiratory rate 75/min, blood pressure 55/35 mm Hg in the right arm, and unobtainable in the lower extremities. Brachial pulses are 2+, but femoral pulses are absent. Capillary refill is 6 seconds. The infant is unresponsive to peripheral venous cannulation. Arrange the following interventions in the most appropriate sequential order for managing this patient's condition.

An infant boy born at $27$ weeks of gestation is currently $14$ days old and recovering in the neonatal intensive care unit. Over the past $8$ hours, the nursing staff has reported increased episodes of apnea, bradycardia, and a drop in body temperature. The patient had previously been tolerating slowly advancing orogastric feeds, but a large bilious residual was aspirated prior to his last scheduled feed. On evaluation, his temperature is $36.0^\circ\text{C}$ ($96.8^\circ\text{F}$), blood pressure is $45/28\text{ mm Hg}$, pulse is $182/\text{min}$, and respirations are $62/\text{min}$. Physical examination reveals a lethargic and mottled neonate. His abdomen is markedly distended, firm, and diffusely tender, with prominent erythema spreading across the abdominal wall. Laboratory analysis demonstrates a leukocyte count of $3,200/\text{mm}^3$ with $25\%$ band forms, a platelet count of $55,000/\text{mm}^3$, and a serum lactate level of $4.8\text{ mmol/L}$. A supine abdominal radiograph shows diffusely dilated loops of bowel, branching linear lucencies overlying the right upper quadrant, and a prominent radiolucent outline of the falciform ligament. Intravenous fluid resuscitation, broad-spectrum antimicrobial therapy, and bowel rest (NPO) are promptly initiated. Which of the following is the most appropriate next step in the management of this patient?

A $4$-year-old girl is brought to the pediatric clinic by her father due to a $2$-day history of high fever, productive cough, and decreased appetite. The father notes that she occasionally holds her right side when she coughs. Her medical history is unremarkable, and she has received all scheduled childhood vaccinations. Her temperature is $39.2^\circ\text{C}$ ($102.6^\circ\text{F}$), pulse is $125/\text{min}$, and respirations are $42/\text{min}$. Oxygen saturation is $95\%$ on room air. The patient appears fatigued but is interactive and does not have intercostal retractions or nasal flaring. Lung auscultation reveals focal fine crackles and decreased breath sounds in the right lower lung field. No wheezing or stridor is present. Which of the following is the most appropriate next step in management?

A 14-year-old boy is brought to the emergency department by his parents due to a 1-week history of progressively worsening shortness of breath, a barking cough, and facial swelling. On physical examination, the patient is anxious, diaphoretic, and leaning forward in a seated position. He is using accessory muscles of respiration. Vital signs show a temperature of 37.8°C (100.0°F), heart rate of 135/min, respiratory rate of 32/min, and oxygen saturation of 91% on room air. Inspection reveals distended neck veins and facial plethora. A portable chest radiograph demonstrates a massive anterior mediastinal mass causing significant tracheal deviation and compression. You are concerned for superior mediastinal syndrome with severe airway compromise.

Arrange the following management steps in the most appropriate sequential order to safely stabilize and diagnose this patient.

A 12-year-old boy is brought to the pediatric emergency department after a high-speed motor vehicle collision. On arrival, he is unresponsive, withdraws from painful stimuli, makes no verbal sounds, and does not open his eyes (Glasgow Coma Scale score of 6). Physical examination reveals that his right pupil is 7 mm and nonreactive to light, while the left is 3 mm and reactive. Vital signs demonstrate a heart rate of 48/min, blood pressure of 165/95 mmHg, and irregular, shallow respirations.

Assuming each step represents the standard progression of care from initial emergency resuscitation to the intensive care management of refractory intracranial hypertension, arrange the following interventions in the most appropriate chronological order.

A $7$-month-old male infant is brought to the emergency department due to intermittent episodes of inconsolable crying over the past $6$ hours. During these episodes, the infant intensely draws his knees to his chest and appears visibly distressed. Between episodes, he becomes uncharacteristically lethargic. His parents report one instance of non-bilious emesis and note that his last diaper contained dark red, mucoid stool. Vital signs reveal a heart rate of $165$/min, respiratory rate of $40$/min, temperature of $37.8$°C ($100$°F), and blood pressure of $85/50$ mm Hg. Physical examination is notable for dry mucous membranes and a palpable, slightly tender, sausage-shaped mass in the right upper quadrant. The abdomen is otherwise soft without involuntary guarding.

Arrange the following steps in the most appropriate chronological sequence for the management of this patient.

A 7-month-old boy is brought to the emergency department by his parents because of a sudden episode of deep blue discoloration of his lips and face. The parents report that he was crying vigorously when he suddenly became intensely cyanotic, tachypneic, and irritable. He has a known history of a congenital cardiac defect for which he is awaiting surgical repair. On arrival, the infant is agitated and visibly cyanotic. Vital signs show a heart rate of $165 \text{ /min}$, respiratory rate of $55 \text{ /min}$, and oxygen saturation of $68\%$ on room air. Auscultation reveals a harsh, crescendo-decrescendo systolic murmur over the left mid-to-upper sternal border. The physician immediately places the infant in a knee-chest position and administers $100\%$ oxygen via a non-rebreather mask, but the infant's agitation and cyanosis persist. Which of the following is the most appropriate next step in pharmacologic management?

A 4-year-old boy is brought to the pediatric clinic by his parents due to concerns about his frequent falls and difficulty climbing stairs. The parents report that he tires more easily than his peers when playing at the local park. When getting up from a sitting position on the floor, he uses his hands to push himself up his thighs to stand upright. His developmental history is significant for walking independently at 16 months, and he currently speaks in short sentences. His temperature is 37.0°C (98.6°F), pulse is 95/min, respirations are 20/min, and blood pressure is 95/60 mm Hg. Physical examination reveals bilaterally enlarged, firm calf muscles and a waddling gait. Which of the following is the most appropriate next step in evaluation?

An $11$-month-old male infant is brought to the pediatric hematology clinic for a follow-up evaluation of thrombocytopenia. He has a history of recurrent acute otitis media and a recent hospitalization for pneumococcal pneumonia. His mother reports that he has been pulling himself to a stand and cruising along furniture for the past month. Physical examination reveals scattered petechiae over the lower extremities and intensely pruritic, erythematous, scaly plaques in the flexural creases of his arms and legs. Laboratory evaluation shows:

- Hemoglobin: $10.5 \text{ g/dL}$
- White blood cell count: $6,800/\text{mm}^3$ (Normal differential)
- Platelet count: $28,000/\text{mm}^3$
- Mean platelet volume (MPV): $5.5 \text{ fL}$ (Reference range: $7.5 - 11.5 \text{ fL}$)

A genetic test confirms a mutation in a gene responsible for actin cytoskeleton reorganization. Which of the following is the most appropriate management for this patient?

A medical student is reviewing pediatric chest radiographs in the neonatal intensive care unit and outpatient cardiology clinic. Pair the following congenital cardiac anomalies with their classic, historically described radiographic appearances.

A pediatric oncology clinic evaluates four children and adolescents with newly diagnosed malignancies. Based on their clinical presentation and initial diagnostic findings, match each patient to the most characteristic underlying genetic or molecular abnormality.

Match each pediatric gastrointestinal clinical presentation (left) with its most characteristic imaging or histopathologic diagnostic finding (right).

A 4-year-old boy is admitted to the hospital with a 7-day history of persistent fever measuring up to 39.5°C (103.1°F). Physical examination reveals bilateral bulbar conjunctival injection without exudate, brightly erythematous and cracked lips, a prominent 'strawberry' tongue, erythema and edema of the palms and soles, and a 2.5-cm right-sided cervical lymph node. A diagnosis of Kawasaki disease is made. Arrange the following therapeutic and follow-up steps in the most appropriate chronological order for the optimal management of this patient, from initial hospitalization to long-term preventive care.

A $2$-hour-old male neonate, born at $39$ weeks gestation to a mother with poorly controlled pregestational diabetes mellitus, is evaluated in the nursery for persistent cyanosis. The pregnancy was otherwise uncomplicated. Vital signs show a heart rate of $160$/min, respiratory rate of $65$/min, and blood pressure of $65/40$ mm Hg in the right arm and $68/42$ mm Hg in the left leg. Pulse oximetry reveals an oxygen saturation of $72\%$ on room air. Administration of $100\%$ oxygen via headbox for $15$ minutes fails to increase the oxygen saturation above $75\%$. Physical examination shows a deeply cyanotic infant with mild intercostal retractions. Cardiac auscultation reveals a normal first heart sound, a loud and single $S_2$, and no murmurs. The lungs are clear to auscultation bilaterally. Which of the following is the most appropriate next step in management?

An on-call physician evaluates a 22-month-old girl in the pediatric emergency triage area due to a sudden onset of respiratory distress. Her parents state that she was previously healthy and had been eating mixed nuts with her older brother in the living room when she suddenly began having a severe coughing fit. She has no history of fever, rhinorrhea, or recent ill contacts. Her temperature is 37.1°C (98.8°F), heart rate is 142/min, respiratory rate is 44/min, and oxygen saturation is 92% on room air. Physical examination reveals a visibly anxious toddler with suprasternal retractions. Auscultation demonstrates significantly diminished breath sounds and high-pitched wheezing isolated to the right hemithorax. An upright expiratory chest radiograph shows right-sided hyperinflation and a leftward shift of the mediastinum. Which of the following is the most appropriate next step in the management of this patient?

A 6-year-old girl is evaluated in the emergency department due to a 2-day history of progressively worsening back pain and new-onset difficulty walking. She has not voided urine for the past 14 hours. The patient was diagnosed with stage IV neuroblastoma 8 months ago and is currently receiving systemic chemotherapy. Temperature is 37.0°C (98.6°F), heart rate is 112/min, and blood pressure is 108/68 mm Hg. Physical examination reveals 3/5 muscle strength in bilateral lower extremities, patellar hyperreflexia, and decreased sensation to pinprick below the umbilicus. A palpable, distended bladder is noted. Suspecting an acute oncologic emergency, arrange the following management steps in the most appropriate chronological order from first to last.