Clinical meaning
In pediatric patients, CF manifests early with meconium ileus in neonates (10-15% of CF infants), failure to thrive due to pancreatic insufficiency, and recurrent respiratory infections. The immature immune system combined with impaired mucociliary clearance makes infants and children particularly vulnerable to Staphylococcus aureus and Haemophilus influenzae initially, with Pseudomonas aeruginosa colonization typically occurring by school age.
Exam relevance
Risk factors: - Newborn with meconium ileus - Failure to thrive despite adequate feeding - Recurrent respiratory infections in infancy - Positive newborn screening (elevated immunoreactive trypsinogen) - Family history of CF - Rectal prolapse in toddlers
Diagnostics: - Monitor growth parameters (weight, length/height, head circumference) at every visit - Monitor respiratory rate and effort - Observe feeding tolerance and caloric intake - Monitor stool pattern (frequency, character, odor) - Observe for rectal prolapse during defecation - Monitor oxygen saturation during respiratory illness