A Sticky (and Thick) Situation
What are the "Gold-Standard" for diagnosing CF?
Sweat Chloride Test
Persistent coughing and this breathing sound are common in children with CF.
Wheezing
Approximately this fraction of Caucasians are carriers of CF.
What is 1 in 25?
The symptoms of CF (Name at LEAST 4)
Wheezing, coughing, chloride in sweat, frequent lung infections, digestive complications
These frequent lung issues often require repeated antibiotic treatment.
What are recurrent respiratory infections?
Name two organ systems affected by cystic fibrosis.
What are....
Describe the difference between a normal and mutated CFTR protein.
What is normal salt/water regulation vs. irregular production of thick mucus?
This neonatal intestinal obstruction is often the first sign of CF.
What is meconium ileus?
Explain why CF is considered a multisystem disease.
What is CFTR is expressed in multiple organs?
Compare acute versus chronic treatment strategies for cystic fibrosis in pediatric patients.
What is acute treatment using IV antibiotics and oxygen to manage exacerbations, while chronic treatment involves lifelong airway clearance, inhaled medications, nutritional support, and CFTR modulators?
Match mutation classes (no protein), (defective processing), (defective gating), (defective conduction), (accelerated turnover), (reduced quantity).
Good Luck
What are: Class I (no protein), II (defective processing), III (defective gating), IV (defective conduction), V (reduced quantity), VI (accelerated turnover)?
Explain how CF leads to failure to thrive in pediatric patients.
What is pancreatic insufficiency causing malabsorption of nutrients?
What has CF, being an autosomal recessive disease with a high carrier frequency resulted in?
What is newborn screening for early detection?
These resources help improve adherence to therapy, mental health, and overall outcomes in patients with cystic fibrosis.
What are support resources?