Pediatrics

A 4-day-old male neonate is brought to the emergency department due to poor feeding, vomiting, and progressive unresponsiveness over the past 12 hours. He was born at 39 weeks gestation via uncomplicated vaginal delivery and was discharged on day 2 of life feeding well. On examination, the infant is comatose, hypothermic (35.4°C), and tachypneic. While being examined, he develops generalized tonic-clonic seizure activity. Initial laboratory evaluation reveals:

Sodium: 140 mEq/L
Potassium: 4.5 mEq/L
Chloride: 105 mEq/L
Bicarbonate: 22 mEq/L
Blood urea nitrogen: 4 mg/dL
Creatinine: 0.3 mg/dL
Glucose: 55 mg/dL
Arterial blood gas: pH 7.52, pCO2 25 mmHg, pO2 95 mmHg
Plasma ammonia: 1,250 µmol/L (normal: 15-50 µmol/L)

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

Match the following pediatric clinical vignettes, characterized by distinctive electrocardiographic (ECG) or chest radiograph (CXR) findings, to their most likely underlying congenital cardiovascular diagnosis.

A 10-day-old male neonate is evaluated in the pediatric orthopedics clinic for bilateral foot abnormalities present since birth. The pregnancy was uncomplicated, and he was born at term via spontaneous vaginal delivery. On physical examination, both feet exhibit deep medial creases, plantar flexion of the ankles, inversion of the heels, and medial deviation of the forefeet. The deformities are rigid and cannot be passively manipulated into a neutral position. There are no dimples over the sacrum, and lower extremity reflexes are normal. The physician discusses the standard evidence-based, nonoperative management protocol with the parents. Arrange the following steps of the standard treatment protocol for this patient's condition in the correct chronological sequence.

An 8-month-old girl with an unrepaired congenital heart defect presents to the acute care clinic with sudden, severe cyanosis and inconsolable crying that began after feeding. Her parents state she has had milder episodes like this before, but they usually resolved quickly. On examination, the infant is highly agitated and has marked circumoral cyanosis. Her peripheral oxygen saturation is 60% on room air. A systolic ejection murmur is auscultated at the left mid-to-upper sternal border, though it is currently faint. The clinical picture is consistent with a hypercyanotic (tet) spell.

Arrange the following interventions in the correct chronological sequence for the acute management of this patient's condition, starting from the immediate initial response to the final definitive treatment step.

Match each pediatric patient's clinical presentation with the most appropriate next step in management.

A medical student is reviewing the pathophysiology of several pediatric genetic and metabolic disorders during a clinical genetics rotation. Evaluate the clinical vignettes below and match each patient presentation with the most accurate description of its underlying biochemical or genetic defect.

A 4-year-old girl is brought to the clinic by her parents due to a two-week history of worsening fatigue, spontaneous bruising on her legs, and intermittent low-grade fevers. Physical examination reveals generalized lymphadenopathy and mild hepatosplenomegaly. The physician suspects acute lymphoblastic leukemia (ALL). Arrange the following diagnostic steps in the most appropriate chronological order, from initial screening to definitive staging.

Match each pediatric patient's clinical presentation with the most appropriate targeted vitamin or cofactor supplementation that serves as specific therapy for their likely underlying metabolic condition.

A crucial component of pediatric hematology and oncology is managing the long-term survivorship of patients exposed to cytotoxic chemotherapy. Match the following chemotherapeutic classes commonly used in pediatric malignancies to their precise mechanism of target-organ toxicity or associated secondary malignancy.

A $7$-month-old boy is evaluated in the urgent care center in early January. His mother reports a $3$-day history of copious nasal discharge, low-grade fever, and a progressively worsening cough. Today, he developed difficulty breathing and has been feeding poorly. His $5$-year-old sister has a history of atopic dermatitis and allergic rhinitis, and she recently recovered from a mild upper respiratory infection. Temperature is $37.8^{\circ}\text{C}$ ($100.0^{\circ}\text{F}$), pulse is $155$ /min, respiratory rate is $62$ /min, and oxygen saturation is $91\%$ on room air. Examination reveals a tachypneic infant with nasal flaring and moderate subcostal retractions. Auscultation of the lungs demonstrates diffuse, bilateral expiratory wheezes and scattered crackles. An empiric nebulized albuterol treatment is administered in the clinic; however, a reassessment $30$ minutes later shows no clinical improvement in his respiratory effort or oxygenation. Which of the following best describes the primary pathophysiologic mechanism responsible for this patient's current respiratory distress and lack of response to the administered therapy?

An 8-month-old female infant is brought to the pediatric clinic by her parents due to a two-week history of abnormal movements. The parents describe sudden, symmetrical, forward-bending jerks of the infant's neck, trunk, and arms. These episodes typically occur in clusters just after the infant wakes up from sleep. The pregnancy and delivery were uncomplicated. The infant had been meeting all early developmental milestones, but the parents note she recently stopped rolling over and babbling. Vital signs are within normal limits. Neurologic examination is notable for generalized hypotonia. Skin examination under Wood lamp illumination reveals three 1.5-cm, elliptical, hypopigmented macules on the infant's trunk. Which of the following are the most appropriate next steps in the evaluation and management of this patient? (Select exactly two answers)

Match each classic pediatric gastrointestinal clinical presentation to its most appropriate definitive management or next best therapeutic step.

A primigravid mother rushes her 12-day-old male newborn to the acute care clinic because he has been persistently vomiting a bright green substance since the morning. The pregnancy and delivery were entirely normal. The mother mentions the infant is usually calm but has been inconsolably fussy and repeatedly drawing his legs up toward his chest. Upon triage assessment, the infant is lethargic with a heart rate of 188/min, respiratory rate of 58/min, and a palpable systolic blood pressure of only 55 mm Hg. Capillary refill time is prolonged at 4 seconds. The abdominal examination is notable for a tense, distended contour with involuntary guarding and absent auscultatory bowel activity. Intravenous access is rapidly secured, and a bolus of normal saline is administered. A bedside abdominal X-ray displays minimal gas in the distal intestine without free intraperitoneal air. Which of the following is the most appropriate next step in the management of this patient?

A seven-year-old girl with a history of retroperitoneal neuroblastoma is brought to the emergency department due to severe, worsening mid-back pain for the past three days and new-onset difficulty walking over the last twelve hours. Her parents report she has not voided urine since yesterday evening. Vital signs are within normal limits for her age. Neurologic examination reveals grade three out of five motor strength in the bilateral lower extremities, hyperreflexia at the patellae, and decreased sensation to light touch below the umbilicus. The bladder is palpably distended. Arrange the following management steps in the most appropriate chronological sequence for this patient's suspected complication.

A $6$-year-old boy with sickle cell disease (HbSS) is brought to the emergency department due to increasing fatigue and generalized weakness over the past $48$ hours. One week ago, he had a low-grade fever accompanied by a bright red "slapped-cheek" facial rash that has since faded. He reports no pain in his chest, abdomen, or extremities. His temperature is $37.1^\circ\text{C}$ ($98.8^\circ\text{F}$), blood pressure is $95/60 \text{ mm Hg}$, pulse is $135/\text{min}$, and respirations are $22/\text{min}$. Physical examination reveals marked conjunctival pallor and a grade $2/6$ systolic flow murmur at the left upper sternal border. The abdomen is soft and non-tender, and the spleen is not palpable. Laboratory studies show:

Which of the following is the most likely underlying mechanism for this patient's acute hematologic presentation?

Paramedics arrive at the emergency department with a 4-week-old infant who is lethargic and feverish. The infant's parents report a 12-hour history of explosive, foul-smelling diarrhea and progressive abdominal swelling. Medical history is notable for delayed meconium passage after birth, which required a glycerin suppository. Vital signs show a temperature of $39.2^\circ\text{C}$ ($102.6^\circ\text{F}$), a heart rate of $185\text{/min}$, a respiratory rate of $60\text{/min}$, and a blood pressure of $58/30\text{ mm Hg}$. The capillary refill time is 4 seconds. On physical examination, the infant is poorly responsive and has a markedly distended, tense abdomen. The clinical picture is highly suspicious for Hirschsprung-associated enterocolitis complicated by septic shock.

Arrange the following management steps in the most appropriate chronological sequence for this patient's immediate care.

A 6-year-old boy is brought to the pediatric emergency department with a 2-day history of high fever, severe headache, and progressively worsening lethargy. His parents recently immigrated, and his immunization status is unknown. Temperature is 39.8°C (103.6°F), blood pressure is 112/72 mmHg, pulse is 135/min, and respiratory rate is 24/min. On physical examination, the child is somnolent but arousable to painful stimuli. Marked nuchal rigidity is present. Neurologic examination reveals a sluggish left pupillary reflex and right-sided pronator drift. Arrange the following steps in the most appropriate sequential order for the acute management of this patient.

An evaluation is requested for a $28$-day-old male infant who presents to the outpatient clinic in late January with worsening nasal congestion, poor sleep, and a mild cough over the past $3$ days. The parents also inquire about a facial rash that erupted two days ago. The infant's $5$-year-old sister has severe asthma and recently missed school due to an upper respiratory infection. The infant was born at $39$ weeks gestation with no perinatal complications. He is breastfeeding slightly less than usual but has adequate urine output. Vital signs include a temperature of $37.3$°C ($99.1$°F), pulse of $152$/min, and respirations of $54$/min. Oxygen saturation is $95$% on room air. Physical examination reveals a well-hydrated, non-toxic appearing infant with copious clear rhinorrhea. Lung auscultation demonstrates prolonged expiration, diffuse high-pitched wheezes, and mild subcostal retractions. Skin examination of the forehead and cheeks shows scattered $1$- to $2$-mm erythematous papules and pustules without comedones. Which of the following is the most appropriate next step in management?

An infant is born at 41 weeks and 3 days of gestation to a 32-year-old G2P1 via urgent cesarean delivery due to repetitive late decelerations. The amniotic fluid is heavily stained with thick meconium. Birth weight is 3,850 g (8.5 lb). Apgar scores are 4 at 1 minute and 7 at 5 minutes. At 2 hours of life, the neonate develops severe tachypnea, marked subcostal retractions, and cyanosis. Vital signs are temperature 36.6°C (97.9°F), heart rate 168/min, and respiratory rate 78/min.

Physical examination reveals a barrel-shaped chest and scattered yellow-white papules on an erythematous base localized to the trunk. A Wright stain of a pustule smear reveals numerous eosinophils.

The infant is placed on continuous positive airway pressure (CPAP) with 100% fraction of inspired oxygen ($FiO_2$). Pulse oximetry demonstrates an oxygen saturation ($SpO_2$) of 95% on the right hand and 82% on the left foot.

An arterial blood gas analysis drawn from an umbilical artery catheter reveals the following:

Chest radiograph displays hyperinflation, patchy bilateral alveolar infiltrates, and flattening of the hemidiaphragms.

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

During a winter afternoon clinic session, a physician evaluates a 4-year-old female for a worsening cough. According to her parents, she initially developed a runny nose and a mild temperature three days ago. Over the past 24 hours, her temperature has spiked to 39.4°C (102.9°F), and she has become more fatigued, though she continues to drink fluids adequately. Her immunizations are current for her age. Vitals show a temperature of 39.4°C (102.9°F), pulse of 130/min, respirations of 44/min, and oxygen saturation of 97% on room air. The child appears tired but non-toxic. Pulmonary auscultation reveals localized crackles and bronchial breath sounds over the right lower lung field, with no wheezing or intercostal retractions. What is the most appropriate empiric pharmacotherapy for this patient?