Pediatrics

An 8-week-old girl is evaluated in the pediatric clinic for poor weight gain and respiratory difficulty. Her parents report that over the past three weeks, she has become increasingly irritable during feedings, sweats profusely, and frequently pauses to catch her breath. She has gained only 90 grams since her 1-month well-child visit. Her temperature is 37.0°C (98.6°F), heart rate is 166/min, respiratory rate is 65/min, and blood pressure is 85/50 mm Hg. Pulse oximetry shows 97% on room air. Physical examination reveals intercostal retractions and nasal flaring. Auscultation of the lungs demonstrates scattered bilateral wheezes and mild bibasilar crackles. Cardiac examination reveals a hyperdynamic precordium, a palpable thrill, and a harsh, 4/6 holosystolic murmur heard best at the left lower sternal border. The liver edge is palpated 3.5 cm below the right costal margin. Which of the following is the most appropriate next step in management?

A 13-month-old boy is brought to the emergency department by his parents after a witnessed generalized shaking episode that lasted for approximately 2 minutes. The parents report that he lost consciousness, his arms and legs stiffened, and then jerked rhythmically. Afterwards, he was very drowsy but returned to his normal baseline within 30 minutes. He has had nasal congestion, a mild cough, and a subjective fever for the past 24 hours. The patient was born at term, and his developmental history is notable for sitting independently, pulling to a stand, cruising along furniture, and using a neat pincer grasp. He can say "mama" and "dada" purposefully. He has received all age-appropriate vaccinations. In the emergency department, his temperature is $39.5^\circ\text{C}$ ($103.1^\circ\text{F}$), pulse is 145/min, and respirations are 36/min. Physical examination shows a well-appearing, interactive infant who is playing with a stethoscope. The anterior fontanelle is flat and soft, and the neck is supple without resistance to passive flexion. Tympanic membranes are clear. Lung auscultation reveals mild, diffuse wheezing and upper airway noise with no retractions. Neurologic examination shows normal muscle tone, symmetric reflexes, and no focal deficits. Which of the following is the most appropriate next step in the management of this patient?

Match each pediatric hematologic or oncologic diagnosis with its most characteristic peripheral blood smear finding.

Match each clinical presentation and combination of diagnostic findings to the most likely congenital heart defect.

A 22-month-old boy is evaluated in the emergency department in the late evening for a loud, barking cough and noisy breathing. The symptoms began abruptly a few hours ago. He has had clear rhinorrhea, nasal congestion, and a low-grade fever for the past 48 hours. The patient is fully vaccinated according to the standard schedule. His temperature is $37.8^\circ\text{C}$ ($100.0^\circ\text{F}$), heart rate is $138/\text{min}$, respiratory rate is $42/\text{min}$, and oxygen saturation is $95\%$ on room air. Physical examination reveals an anxious child with a hoarse voice. Inspiratory stridor is clearly audible both when the child is crying and when he is sitting calmly in his father's arms. Mild suprasternal and intercostal retractions are noted. Lung fields are clear to auscultation bilaterally without wheezing. Which of the following is the most appropriate next step in the management of this patient?

A 2-year-old boy is brought to the emergency department after a witnessed choking episode while eating mixed nuts 1 hour ago. He had a severe fit of coughing initially, which has since subsided. On physical examination, he is alert and in mild respiratory distress. Vital signs show a temperature of 37.1°C (98.8°F), heart rate of 122/min, respiratory rate of 28/min, and oxygen saturation of 96% on room air. Lung auscultation reveals diminished breath sounds and localized expiratory wheezing over the right middle lobe. Arrange the following interventions in the most appropriate chronological sequence for the evaluation and management of this patient.

A 20-month-old boy is brought to the pediatric emergency department due to a 7-day history of a brassy cough and a rapid decline in his respiratory status over the past 24 hours. The parents report he has been increasingly restless and absolutely refuses to lie flat, insisting on being held in an upright, leaning-forward position. On presentation, his temperature is $37.5^\circ\text{C}$ ($99.5^\circ\text{F}$), heart rate is $158/\text{min}$, respiratory rate is $56/\text{min}$, and blood pressure is $92/54\text{ mmHg}$. Oxygen saturation is $90\%$ on a non-rebreather mask. Physical examination reveals prominent cervical lymphadenopathy, facial plethora with distended neck veins, severe suprasternal retractions, and a biphasic stridor. A portable bedside chest radiograph demonstrates a markedly widened superior mediastinum with severe extrinsic compression of the distal trachea, narrowing the airway lumen to approximately $2\text{ mm}$. The child is highly agitated and exhibits worsening work of breathing. Which of the following is the most appropriate next step in management?

Parents arrive at the triage desk with their 14-day-old male infant, reporting that he suddenly began vomiting dark green fluid 4 hours ago. Previously, the infant had been breastfeeding well and passing normal transitional stools. On triage assessment, the infant appears lethargic and pale. Vital signs reveal a heart rate of 195/min, respiratory rate of 65/min, and blood pressure of 55/30 mm Hg. Physical examination demonstrates a rigid, distended abdomen with diffuse guarding and absent bowel sounds. Capillary refill time is 5 seconds. An emergency portable abdominal radiograph shows a paucity of gas in the distal bowel. What is the most appropriate next step in the management of this patient?

Match each pediatric patient's clinical presentation with the congenital cardiovascular anomaly most likely to be found on echocardiography.

A 4-year-old boy is brought to the emergency department by his parents due to a sudden onset of high fever, severe sore throat, and difficulty breathing that started 4 hours ago. His parents state that they recently moved to the country and his childhood immunizations are incomplete. On examination, the patient appears toxic, highly anxious, and is sitting upright on his mother's lap, leaning forward with his neck hyperextended and jaw thrust forward. He is actively drooling and speaks in a muffled voice. Pronounced inspiratory stridor is audible without a stethoscope. Vital signs are temperature 39.8°C (103.6°F), heart rate 155/min, respiratory rate 42/min, and oxygen saturation 93% on room air.

Arrange the following management steps in the most appropriate sequential order for this patient.

A 6-month-old boy is brought to the emergency department after a generalized tonic-clonic seizure. His mother reports that he has been increasingly irritable and "clammy" over the last several weeks, particularly in the early mornings before his first feeding. The infant was born at full term. Developmentally, he sits with pelvic support and rolls front-to-back, which his parents worry is delayed compared to his older sister.

Vital signs show a temperature of 36.8°C (98.2°F), heart rate of 155/min, blood pressure of 85/50 mm Hg, and respiratory rate of 52/min. Physical examination reveals a lethargic infant with a round, "doll-like" face and thin extremities. His abdomen is markedly distended with a firm, smooth liver edge palpable 6 cm below the right costal margin. The spleen is not palpable. Several small, yellowish papules are noted on the extensor surfaces of his arms and legs.

Initial laboratory studies show:

Additional testing reveals markedly elevated serum triglycerides and uric acid.

Which of the following is the most appropriate intervention for this patient?

During a health maintenance visit, a $13$-year-old adolescent male reports a $4$-week history of a constant, dull ache in his left anterior thigh and knee. He has no history of recent trauma, fever, or prior medical conditions. His body mass index is $32 \text{ kg/m}^2$. Vital signs are within normal limits, with a temperature of $36.8^\circ\text{C}$ ($98.2^\circ\text{F}$), heart rate of $78/\text{min}$, and blood pressure of $112/72 \text{ mm Hg}$. On physical examination, the left knee has no effusion, erythema, or joint line tenderness, and range of motion is full and painless. However, his gait is noticeably antalgic, and his left foot rests in an externally rotated position while standing. Passive internal rotation of the left hip is restricted and elicits pain. Laboratory evaluation reveals an erythrocyte sedimentation rate of $9 \text{ mm/h}$, a C-reactive protein level of $0.4 \text{ mg/dL}$, and a leukocyte count of $7,100/\text{mm}^3$. Which of the following is the most appropriate next step in management?

A 2-day-old full-term neonate is evaluated in the nursery for failure to pass meconium and progressive abdominal distension. Physical examination reveals a remarkably tight anal sphincter. Upon withdrawal of the examiner's finger during a digital rectal examination, there is an explosive release of gas and foul-smelling stool. Arrange the following steps in the optimal diagnostic and therapeutic sequence for this patient's suspected condition.

A 10-year-old boy is currently admitted to the pediatric intensive care unit following a severe traumatic brain injury. He is intubated, mechanically ventilated, sedated, and has an external ventricular drain in place. Over the last five minutes, his intracranial pressure monitor indicates a sudden and sustained spike to 40 mm Hg (normal is less than 15 mm Hg). Simultaneously, his heart rate drops to 45/min, and his blood pressure rises to 160/90 mm Hg. Arrange the following acute management steps for suspected impending brain herniation in the most appropriate escalating sequence, proceeding from basic initial maneuvers to advanced refractory therapies.

A $15$-month-old boy is brought to the pediatric clinic for a routine well-child visit. His mother is concerned because he is not yet walking independently and his vocabulary consists of only a single word. He was born at $39$ weeks gestation without perinatal complications, and newborn screening was reported as normal. A review of his growth chart demonstrates that his length has decreased from the $50$ th percentile at $6$ months of age to the $5$ th percentile currently, while his weight has remained steady along the $45$ th percentile. Vital signs include a temperature of $36.8^\circ\text{C}$ ($98.2^\circ\text{F}$), heart rate of $88$/min, and respiratory rate of $22$/min. Physical examination reveals an infant with a persistently open and large anterior fontanelle, generalized hypotonia, and delayed relaxation of the deep tendon reflexes. Laboratory evaluation shows:

Thyroid-stimulating hormone (TSH): $2.1\ \mu\text{U/mL}$ (Normal: $0.5-5.0\ \mu\text{U/mL}$)
Free thyroxine (T4): $0.4\ \text{ng/dL}$ (Normal: $0.9-2.4\ \text{ng/dL}$)

Which of the following is the most appropriate next step in the evaluation of this patient?

A 14-month-old girl is rushed to the pediatric emergency center by her parents because she suddenly became severely cyanotic and limp after a prolonged crying episode at home. On arrival, she is highly agitated, inconsolable, and instinctively drawing her legs toward her abdomen. Vital signs show a heart rate of 178/min, respiratory rate of 52/min, and oxygen saturation of 62% on room air. Auscultation reveals a harsh, grade 3/6 systolic ejection murmur at the left mid-to-upper sternal border, which the parents note is much softer than what was described during her last pediatric visit. She is diagnosed with a hypercyanotic spell secondary to an underlying congenital heart defect. Arrange the following interventions in the most appropriate sequential order for the acute escalation of management for this patient, starting with the immediate first step and progressing to treatments for medically refractory symptoms.

A 7-year-old girl is rushed to the pediatric emergency department with continuous rhythmic jerking of her arms and legs that began 12 minutes ago while at school. She has no prior history of seizures. On arrival, she is unresponsive, her pulse oximetry reads 89% on room air, and she has active generalized tonic-clonic movements. A peripheral intravenous line is rapidly established by the nursing staff. Arrange the following management steps in the correct chronological sequence for the optimal acute algorithmic management of this patient's condition, from immediate priority to late-stage refractory care.

A $6$-week-old boy is brought to the emergency department by his parents after a witnessed $15$-second episode of absent breathing associated with perioral cyanosis at home. The parents report he has had progressive nasal congestion, clear rhinorrhea, and decreased feeding over the past three days. He was born at $39$ weeks gestation with no perinatal complications. His rectal temperature is $37.7^\circ\text{C}$ ($99.9^\circ\text{F}$), pulse is $165/\text{min}$, and respirations are $70/\text{min}$. Pulse oximetry shows an oxygen saturation of $90\%$ on room air. Physical examination reveals an appropriately grown infant with copious nasal secretions, marked subcostal and intercostal retractions, and diffuse fine crackles with scattered expiratory wheezes on auscultation. Which of the following are the most appropriate immediate steps in the management of this patient? (Select exactly two)

A 9-year-old boy with a known history of sickle cell anemia is brought to the emergency department by his parents due to fever, cough, and severe chest pain that started yesterday. His temperature is $38.9^\circ\text{C}$ ($102.0^\circ\text{F}$), heart rate is $120/\text{min}$, blood pressure is $105/65\text{ mmHg}$, and respiratory rate is $30/\text{min}$. Oxygen saturation is $89\%$ on room air. Physical examination reveals crackles and decreased breath sounds in the right lower lung field. A chest radiograph demonstrates a new right lower lobe infiltrate. Which of the following is the most appropriate next step in management?

The parents of a $14$-month-old female infant bring her to the emergency department in mid-winter because of a worsening cough and rapid breathing. The infant had a $3$-day history of mild nasal congestion and clear rhinorrhea, but over the past $24$ hours, she developed a high fever and became increasingly lethargic. She was born at full term and is up-to-date on all routine childhood immunizations, including the Haemophilus influenzae type b and pneumococcal conjugate vaccines. Her temperature is $39.4^\circ\text{C}$ ($102.9^\circ\text{F}$), heart rate is $155/\text{min}$, respiratory rate is $58/\text{min}$, and oxygen saturation is $91\%$ on room air. On physical examination, the infant is lethargic but arousable. Nasal flaring and moderate intercostal retractions are present. Auscultation of the lungs reveals decreased breath sounds and fine crackles localized over the right lower lobe. The left lung fields are clear, and no wheezing is appreciated. Chest radiography shows a dense consolidation in the right lower lobe with no pleural effusion. She is admitted to the pediatric ward and placed on supplemental oxygen via nasal cannula. Which of the following is the most appropriate initial pharmacotherapy for this patient?