A 42-year-old woman presents to the emergency department in active labor. She has had no prenatal care and is unsure of the gestational age. Labor progresses rapidly and spontaneous vaginal delivery of a baby boy occurs 3 hours after presentation. On initial exam, the child is 1.9 kg (4.2 lb) with a small head and jaw. A sac-like structure containing intestine, as can be seen in the picture, protrudes from the abdominal wall. What complication is closely associated with this presentation?

Dehydration and necrosis of bowel

Twisting of the bowel around itself

A 15-month-old boy is brought to the pediatrician for immunizations and assessment. His parents report that he is eating well and produces several wet diapers every day. He is occasionally fussy, but overall a happy and curious child. The boy was born at 39 weeks gestation via spontaneous vaginal delivery. On physical examination his vital signs are stable. His weight and height are above the 85th percentile for his age and sex. On chest auscultation, the pediatrician detects a loud harsh holosystolic murmur over the left lower sternal border. The first and second heart sounds are normal. An echocardiogram confirms the diagnosis of the muscular ventricular septal defect without pulmonary hypertension. Which of the following is the best management strategy for this patient?

Reassurance of the parents and regular follow-up

Surgical closure of the defect using cardiopulmonary bypass

Oral digoxin and regular follow-up

Antibiotic prophylaxis against infective endocarditis

Transcatheter occlusion closure of the defect

A 3-year-old girl is brought to the physician for a well-child examination. She was born at term and has been healthy since. She can climb up and down the stairs and can pedal a tricycle. She has difficulty using a spoon to feed herself but can copy a line. She speaks in 2- to 3-word sentences that can be understood by most people. She is selfish while playing with children her age and throws tantrums quite often. She cannot put on her own shoes and socks. She does not tolerate separation from her parents. She is at 60th percentile for height and weight. Physical examination including neurologic examination reveals no abnormalities. Which of the following is the most appropriate assessment of her development?

Fine motor: Delayed | Gross motor: Normal | Language: Normal | Social skills: Delayed

Fine motor: Normal | Gross motor: Normal | Language: Delayed | Social skills: Delayed

Fine motor: Delayed | Gross motor: Delayed | Language: Normal | Social skills: Normal

Fine motor: Normal | Gross motor: Delayed | Language: Normal | Social skills: Delayed

Fine motor: Normal | Gross motor: Delayed | Language: Delayed | Social skills: Normal

A 1-day-old infant in the general care nursery, born at full term by uncomplicated cesarean section delivery, is noted to have a murmur, but otherwise appears well. On examination, respiratory rate is 40/min and pulse oximetry is 96%. Precordium is normoactive. With auscultation, S1 is normal, S2 is single, and a 2/6 systolic ejection murmur is heard at the left upper sternal border. Echocardiography shows infundibular pulmonary stenosis, overriding aorta, ventricular septal defect and concentric right ventricular hypertrophy. Which of the following correlate with the presence or absence of cyanosis in this baby?

The degree of right ventricular outflow tract obstruction

The ratio of reduced hemoglobin to oxyhemoglobin

The concentration of hemoglobin

The size of ventricular septal defect

The concentration of pulmonary surfactant

A 4-year-old girl is brought to the physician by her parents because she is severely underweight. She is easily fatigued and has difficulty keeping up with other children at her daycare. She has a good appetite and eats 3 full meals a day. She has 4 to 5 bowel movements daily with bulky, foul-smelling stools that float. She has had recurrent episodes of sinusitis since infancy. Her parents report that she recently started to snore during her sleep. She is at the 15th percentile for height and 3rd percentile for weight. Her vital signs are within normal limits. Examination shows pale conjunctivae. A few scattered expiratory crackles are heard in the thorax. There is abdominal distention. Which of the following is the most likely underlying cause of this patient's failure to thrive?

Exocrine pancreatic insufficiency

T. whippelii infiltration of intestinal villi

Impaired intestinal amino acid transport

Small intestine bacterial overgrowth

Intestinal inflammatory reaction to gluten

A 2860-g (6-lb 3-oz) male newborn is born at term to a primigravid woman via spontaneous vaginal delivery. The mother has had no routine prenatal care. She reports that there is no family history of serious illness. The initial examination of the newborn shows bowing of the legs and respiratory distress upon palpation of the chest. The skin and joints are hyperextensible. X-rays of the chest and skull show multiple rib fractures and small, irregular bones along the cranial sutures. The patient is at increased risk of which of the following complications?

Costochondral junction enlargement

A 3-month-old African American boy presents to his pediatrician’s office for his routine well visit. He was born full-term from an uncomplicated vaginal delivery. He is exclusively breastfeeding and not receiving any medications or supplements. Today, his parents report no issues or concerns with their child. He is lifting his head for brief periods and smiling. He has received only 2 hepatitis B vaccines. Which of the following is the correct advice for this patient’s parents?

He should start vitamin D supplementation.

He should have his serum lead level checked to screen for lead intoxication.

He needs a 3rd hepatitis B vaccine.

Long-term outcomes and follow-up for USMLE Step 3: High-Yield Pediatrics Lessons

Neuro & Cardiac - Lifelong Watch

Neural Tube Defects (e.g., Myelomeningocele)
- Hydrocephalus: Lifelong shunt monitoring (blockage, infection).
- Neurogenic Bladder: Regular renal function tests (RFTs) & ultrasounds; clean intermittent catheterization (CIC) to prevent hydronephrosis.
- Tethered Cord Syndrome: Monitor for new motor/sensory deficits, back pain.
- Orthopedic surveillance for scoliosis, hip dislocation.
Congenital Heart Disease (CHD)
- Post-Repair (e.g., ToF, VSD): Annual cardiology f/u. Monitor for arrhythmias, ventricular dysfunction, residual shunts.
- Infective Endocarditis (IE) Prophylaxis: Required for high-risk patients (prosthetic valves, previous IE, unrepaired cyanotic CHD).
- Eisenmenger Syndrome: Monitor for polycythemia, heart failure. Avoid dehydration & high altitudes.

⭐ Post-ToF Repair: The most common long-term complication leading to re-operation is severe pulmonary regurgitation. The most life-threatening arrhythmia is ventricular tachycardia (VT), a major risk for sudden cardiac death.

Long-term outcomes of Tetralogy of Fallot repair

GI & GU Issues - Gut Feelings & Flow Problems

Gastrointestinal (GI):
- TEF Repair: ↑ risk of GERD, dysphagia, strictures. Needs EGD surveillance.
- Hirschsprung's: Post-op risks include enterocolitis, chronic constipation, incontinence.
- Anorectal Malformations (ARM): Fecal incontinence & constipation are major concerns requiring a bowel management program.
Genitourinary (GU):
- Posterior Urethral Valves (PUV): High risk of CKD/ESRD. Monitor creatinine, BP. "Valve bladder" causes poor compliance & incontinence.
- Vesicoureteral Reflux (VUR): Risk of recurrent UTIs, renal scarring. DMSA scan assesses scarring.

⭐ Prognostic Pearl: The nadir serum creatinine level at 1 year of age is the best predictor of long-term renal function in boys treated for Posterior Urethral Valves (PUV).

Normal vs. PUV Urinary System

Systemic & Structural - Bones, Syndromes & Beyond

Osteogenesis Imperfecta (OI): Lifelong fracture risk. Follow-up includes audiology (hearing loss), DEXA scans, and cyclical bisphosphonate therapy (Pamidronate).
Marfan Syndrome: Regular cardiology for aortic root dilatation (Z-score monitoring). Annual ophthalmology for ectopia lentis. Beta-blockers or ARBs (Losartan) to slow aortic root growth.
Down Syndrome: Surveillance for hypothyroidism (TSH), atlantoaxial instability (symptom-based screening), and vision/hearing deficits.
Turner Syndrome: Monitor for coarctation of aorta, bicuspid aortic valve, and renal anomalies. Requires estrogen replacement for puberty & bone health.

⭐ In Down Syndrome, the Amyloid Precursor Protein (APP) gene on Chromosome 21 leads to amyloid plaque deposition, causing a high risk of early-onset Alzheimer's disease (often by age 40).

Growth & Mind - Tracking & Thriving

Growth Monitoring: Regular plotting on WHO/IAP growth charts. Crucial for detecting failure to thrive (FTT).
- Red flags: Crossing >2 major centiles, weight-for-height < -2 SD.
- Assess bone age if height is affected.
Developmental Surveillance: Screen at every well-child visit. Use standardized tools.
- Domains: Gross Motor, Fine Motor, Language, Social/Cognitive.
- Tools: DDST-II, Trivandrum Development Screening Chart (TDSC).
Psychosocial Health: Monitor for behavioral issues, learning disabilities, and social integration challenges. Crucial for quality of life.

⭐ Global Developmental Delay (GDD): Significant delay (≥2 SD below mean) in ≥2 developmental domains in children <5 years old.

Infant weight-for-age growth chart with failure to thrive

High‑Yield Points - ⚡ Biggest Takeaways

Neurodevelopmental delay is a key long-term risk in major congenital heart and CNS defects.

Failure to thrive is common in significant cardiac and GI anomalies, needing nutritional support.

Congenital renal anomalies demand lifelong follow-up for CKD and hypertension.

Large uncorrected shunts can lead to irreversible pulmonary hypertension (Eisenmenger syndrome).

SBE prophylaxis is vital for high-risk cardiac lesions, including prosthetic valves and unrepaired cyanotic CHD.

A planned transition to adult care improves long-term outcomes and adherence.

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