Pediatrics

A 15-year-old adolescent with cystic fibrosis and pancreatic insufficiency is evaluated in the outpatient pulmonary clinic for a 4-week history of worsening productive cough, malaise, and subjective low-grade fevers. He reports expectorating thick, brownish, plug-like sputum. His daily maintenance regimen of dornase alfa, hypertonic saline, and chest physiotherapy has not alleviated the symptoms. Pulmonary function testing performed today shows a forced expiratory volume in 1 second (FEV1) of 60% of predicted, a significant decline from his baseline of 84% recorded two months ago. Vital signs show an oxygen saturation of 95% on ambient air. Lung auscultation reveals prominent bilateral expiratory wheezes and coarse upper lobe crackles. A complete blood count is notable for an absolute eosinophil count of 1,250/mm³. Total serum IgE is markedly elevated at 1,650 IU/mL. High-resolution computed tomography of the chest demonstrates new upper lobe central bronchiectasis with scattered mucous plugging and fleeting infiltrates. Which of the following are indicated in the initial pharmacological management of this patient's current condition? (Select all that apply)

A 7-month-old boy is brought to the outpatient clinic by his mother due to a two-week history of unusual movements. The mother states that shortly after waking from naps, the infant suddenly flexes his head and pulls his arms forward synchronously. These episodes last for a few seconds and occur in clusters of 10 to 15. Prior to this, he had been babbling and rolling over, but he has stopped performing these skills over the past week. Vitals are: temperature 37.0°C (98.6°F), heart rate 110/min, respiratory rate 28/min, and blood pressure 90/55 mm Hg. Neurological examination reveals axial hypotonia and poor head control. Skin examination is notable for three 1.5-cm elliptical, hypopigmented macules on the trunk and right thigh. An electroencephalogram demonstrates a disorganized pattern of chaotic, high-amplitude slow waves and multifocal spikes. Which of the following is the most appropriate initial pharmacotherapy for this patient's condition?

A 36-month-old girl is evaluated during a routine pediatric visit. Her father shares concerns raised by her new daycare provider regarding the child's developmental progress. According to the provider, the child is 'falling behind' because she cannot draw a square during arts and crafts and places both feet on each step when walking down the playground stairs. The father also notes that while he easily understands his daughter, extended family members occasionally ask her to repeat herself. The child currently speaks in three-word sentences, easily pedals a tricycle, and engages in imaginative play with her toys. She accurately states her age and gender when asked. Vital signs are normal, and physical examination shows no abnormalities. Based on her current developmental status, which of the following is the most appropriate next step in management?

A mother brings her 12-month-old son to the pediatric clinic for a routine well-child visit. She expresses concern that her son appears smaller than other children his age at daycare. Upon review of the child's growth chart, the pediatrician notes that the boy's current weight is $9.6 \text{ kg}$ and his length is $75 \text{ cm}$. He has been consistently tracking along the 25th percentile for both parameters since birth, and his developmental milestones are entirely age-appropriate. The physician reassures the mother that her son's growth pattern is completely normal.

Assuming the child has followed classic anthropometric rules of thumb for normal infant growth, which of the following most likely represents this patient's approximate weight and length at birth?

A 39-week gestation infant is delivered via spontaneous vaginal delivery. Immediately after birth, the infant is noted to be apneic, limp, and cyanotic. Arrange the following steps of neonatal resuscitation in the correct chronological order of escalation according to standard guidelines.

An otherwise healthy 36-month-old girl is evaluated via a telehealth consultation for a routine health maintenance visit. Her parents express anxiety regarding her developmental progress compared to other children in her playgroup. Specifically, they note that she cannot hop on one foot or draw a cross on paper. During the video observation, the physician notes the child pedaling a small tricycle across the room and walking up a set of stairs using alternating feet. When given crayons, she successfully copies a circle but simply scribbles when attempting to copy a square. She answers the physician's questions using three-word sentences, and the physician easily understands approximately 75% of what she says. She correctly states her name and age when asked. Based on this presentation, which of the following is the most appropriate next step in management?

A 12-month-old boy is brought to the pediatrician for a routine well-child visit. His medical history is significant for perinatal HIV infection. He is currently prescribed antiretroviral therapy, but his mother admits to frequently missing doses over the past few months. Recent laboratory studies show a CD4 T-lymphocyte count of 450 cells/mm³ and a CD4 percentage of 12%. The parents express concern about his physical development, noting that he is not yet able to run or kick a ball. On examination, the boy is alert and interactive. He can stand independently for a few seconds and uses a fine pincer grasp to pick up a raisin. He says "mama" and "dada" specifically and waves "bye-bye" to the clinician. He has received all previously recommended immunizations. Which of the following is the most appropriate next step in the management of this patient?

During routine well-child care, clinicians follow standardized periodicity schedules to ensure appropriate preventive screening for asymptomatic children and adolescents. Arrange the following universal preventive screening assessments in chronological order, starting with the earliest recommended age of initial administration to the latest.

Match each classic pediatric respiratory clinical presentation with its most likely causative pathogen.

During routine evaluations in the newborn nursery and outpatient pediatric clinic, various abdominal and genitourinary structural anomalies are identified. Accurate diagnosis relies on distinct physical characteristics, which directly dictate the need for further workup and specific prognostic counseling. Match each neonatal clinical presentation to its corresponding underlying pathophysiologic mechanism and classic clinical associations.

During routine rounding in the neonatal intensive care unit, a physician evaluates a male newborn delivered $60$ minutes ago via elective cesarean section at $38$ weeks of gestation. The mother's pregnancy was complicated by diet-controlled gestational diabetes. The infant, weighing $3,400\text{ g}$, had Apgar scores of $7$ and $9$ at $1$ and $5$ minutes, respectively. He was admitted to the unit for close monitoring due to increased work of breathing. Current vital signs show a temperature of $36.9^\circ\text{C}$ ($98.4^\circ\text{F}$), pulse of $158/\text{min}$, and respirations of $78/\text{min}$. He is maintaining an oxygen saturation of $94\%$ on continuous positive airway pressure (CPAP). Physical examination reveals mild intercostal retractions. Detailed skin inspection shows scattered, non-erythematous, superficial vesicles and small pustules over the chin, neck, and lower back. Several of these lesions have ruptured, leaving a collarette of scale surrounding a flat, hyperpigmented macule. Laboratory evaluation reveals a leukocyte count of $18,000/\text{mm}^3$ with a normal differential. A Wright-stained smear of fluid from an intact pustule demonstrates numerous neutrophils and no bacteria. Chest radiography displays increased lung volumes, prominent perihilar vascular markings, and fluid in the minor fissure. Which of the following is the most appropriate next step in management?

A 36-hour-old term male neonate is evaluated in the neonatal intensive care unit for progressive abdominal distension and two episodes of bilious emesis. He was born at 39 weeks gestation via uncomplicated vaginal delivery. Vital signs are within normal limits for age. The infant has not yet passed meconium. Physical examination reveals a markedly distended, firm abdomen without overlying erythema. Bowel sounds are hypoactive. An imperforate anus is excluded by physical examination. The clinical team proceeds with evaluating and managing the patient for the most likely underlying condition. Assume that initial non-operative therapeutic interventions prove unsuccessful in completely relieving the obstruction, necessitating surgical escalation.

Arrange the following clinical steps in the most appropriate chronological sequence for the management and subsequent care of this patient.

A $2$-day-old female neonate is evaluated in the neonatal intensive care unit for persistent cyanosis. She was born at $39$ weeks gestation via normal vaginal delivery to a $28$-year-old primigravida. The mother has a history of bipolar disorder and was maintained on her prescribed psychiatric medication throughout the pregnancy. On physical examination, the neonate appears comfortably tachypneic but cyanotic. Vital signs show a heart rate of $160$/min, respiratory rate of $65$/min, and oxygen saturation of $82\%$ on room air. Cardiac auscultation reveals a widely split $S_1$, a normal $S_2$, and a harsh holosystolic murmur heard best at the left lower sternal border. Chest radiography demonstrates massive cardiomegaly spanning nearly the entire thoracic cavity. Electrocardiography reveals right atrial enlargement and a complete right bundle branch block. Which of the following best describes the underlying cardiovascular anomaly in this patient?

A $10$-week-old male infant is brought to the pediatric emergency department by his parents due to a $3$-day history of rapid breathing, poor feeding, and increased fussiness. The parents report that the infant takes twice as long to finish a bottle and frequently stops to catch his breath while sweating profusely over his forehead. He has had a mild cough but no fever. Medical history is notable for an uncomplicated full-term birth. A cardiac murmur was mentioned at his $1$-month well-child visit, but the family missed the follow-up appointment. Temperature is $37.0^\circ\text{C}$ ($98.6^\circ\text{F}$), pulse is $168/\text{min}$, respiratory rate is $68/\text{min}$, and blood pressure is $85/50\text{ mmHg}$. Pulse oximetry shows $94\%$ on room air. Physical examination reveals an infant in moderate respiratory distress with intercostal retractions. Lung auscultation reveals diffuse bilateral wheezes and fine crackles at the bases. Cardiovascular examination shows a hyperactive precordium, a loud S2, and a grade $3/6$ harsh holosystolic murmur at the left lower sternal border. The liver edge is palpated $3.5\text{ cm}$ below the right costal margin. Which of the following is the most appropriate next step in the management of this patient?

An $8$-month-old girl is brought to a family medicine clinic in mid-December due to a persistent cough and noisy breathing. Her mother reports that the child developed a runny nose, low-grade fever, and mild nasal congestion four days ago. Yesterday, the nasal discharge became thick, and the child developed a "tight" cough. She has no prior episodes of wheezing and was born at term without complications. She has been feeding well and producing a normal number of wet diapers. Vital signs are temperature $37.8^\circ\text{C}$ ($100.0^\circ\text{F}$), heart rate $125\text{/min}$, respiratory rate $44\text{/min}$, and oxygen saturation $96\%$ on room air. Physical examination reveals a happy, interactive infant. Lung auscultation demonstrates diffuse bilateral expiratory wheezes and mild subcostal retractions. Which of the following interventions are appropriate components of the immediate management plan for this patient? (Select all that apply)

A 6-month-old boy is brought to the pediatric clinic by his parents for a routine well-child visit. His parents express significant concern because the infant is not yet crawling and cannot pick up small objects with his thumb and index finger. He is currently able to roll in both directions, sit momentarily without support, and babble. Over the past three weeks, the parents have noted that he is increasingly irritable and has had multiple episodes of forceful vomiting, primarily occurring in the morning. He has no history of fever, trauma, or diarrhea.

Vital signs are temperature 37.1°C (98.8°F), heart rate 110/min, and respiratory rate 24/min. Weight is at the 50th percentile, and length is at the 45th percentile. Head circumference has increased from the 60th percentile at age 4 months to the 98th percentile currently. On physical examination, the anterior fontanelle is full and tense. Neurologic examination reveals downward deviation of the eyes with sclera visible above the iris. The remainder of the examination is unremarkable.

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

A first-time mother brings her $4$-week-old boy to the acute care clinic because he has been forcefully vomiting after every meal for the last $48$ hours. The vomit does not contain bile. She notes that he appears extremely hungry and eagerly attempts to feed again immediately after these episodes. Vital signs reveal a heart rate of $162 \text{ beats/min}$ and a respiratory rate of $32 \text{ breaths/min}$. On physical examination, the infant has dry mucous membranes, a sunken anterior fontanelle, and delayed capillary refill. Careful abdominal palpation reveals a firm, olive-shaped mass in the right upper quadrant.

Laboratory analysis demonstrates:

Which of the following represents the most appropriate initial step in the care of this patient?

A 4-month-old boy is brought to the emergency department due to progressive feeding difficulties, a weak cry, and rapid breathing. Over the past month, his parents noticed he becomes exhausted and sweaty during feeds. He smiles socially and coos interactively, but he has not yet been able to roll over or hold his head up. His temperature is 37.1°C (98.8°F), blood pressure is 85/50 mm Hg, pulse is 165/min, and respiratory rate is 65/min. Physical examination reveals a lethargic infant with a protruding tongue and generalized hypotonia. When pulled to a sitting position, he exhibits severe head lag. Deep tendon reflexes are absent. Lung auscultation reveals bilateral basilar crackles and scattered wheezes. The liver edge is palpated 4 cm below the right costal margin. A chest radiograph demonstrates massive cardiomegaly. Electrocardiography shows short PR intervals and large QRS complexes.

Laboratory studies show:
Arterial blood gas:
pH: 7.30
pCO2: 54 mm Hg
Bicarbonate: 26 mEq/L

Serum creatine kinase: 1,850 U/L (normal: 22-198 U/L)

Which of the following is the most likely underlying etiology of this patient's clinical presentation?

A pediatrician is evaluating a 4-year-old boy who has developed a progressively unsteady gait over the past twelve months. According to his medical records, he achieved independent ambulation at 15 months of age but has recently begun to stumble frequently. On neurological examination, he demonstrates truncal instability and a wide-based stance. When asked to follow a finger with his eyes, horizontal nystagmus and delayed saccades are observed. The physician also notes the presence of dilated, tortuous superficial vessels on the bulbar conjunctivae of both eyes. A subsequent blood panel shows a markedly elevated alpha-fetoprotein level alongside a quantitative IgA deficiency. Which of the following statements regarding the patient's underlying syndrome are correct? (Select 2 that apply)

A $9$-year-old female patient is evaluated in the pediatric acute care unit due to progressive dyspnea that began yesterday. She has a documented history of moderate persistent asthma, currently managed with a daily inhaled corticosteroid and a short-acting beta-agonist as needed. Over the previous $24$ hours, she has required her rescue inhaler every two hours with minimal relief. Vital signs on presentation show a temperature of $37.0^\circ\text{C}$ ($98.6^\circ\text{F}$), blood pressure of $110/70\text{ mm Hg}$, heart rate of $142/\text{min}$, and respiratory rate of $44/\text{min}$. Oxygen saturation is $91\%$ on ambient air. The patient appears highly anxious, sits leaning forward, and speaks only in two-word phrases. Examination highlights significant intercostal and suprasternal retractions. Auscultation of the chest reveals widespread tight wheezing and notably reduced air entry in all lung fields. Supplemental oxygen is applied, and the patient receives a systemic dose of intravenous methylprednisolone alongside three back-to-back treatments of nebulized albuterol combined with ipratropium bromide. One hour later, reassessment shows a heart rate of $150/\text{min}$ and a respiratory rate of $40/\text{min}$. She remains awake and responsive but continues to exhibit severe accessory muscle use and diminished breath sounds. Which of the following is the most appropriate next step in management?