Review Question - QID 100882

Children with cystic fibrosis (CF) have a sodium-rich sweat due to failure of chloride resorption in eccrine sweat glands, putting them at risk of hypovolemia due to excessive sodium loss.

Complications of CF patients include recurrent, drug-resistant bacterial or fungal pneumonias (classically Psuedomonas), cor pulmonale (right heart failure), idiopathic hyponatremia, glucose intolerance, Type II diabetes, fat-soluble vitamin deficiency (low vitamin A, D, K, and E), esophageal varices, biliary cirrhosis, gallstones, infertility (95%), and heat exhaustion.

Weiss discusses the diagnosis of wheezing in children, which can include cystic fibrosis. The most common causes of wheezing in children is asthma and allergies. Less common but pertinent causing include foreign body aspiration and cystic fibrosis.

Sojo et al. performed a retrospective chart review of patients with CF presenting with electrolyte abnormalities. Five patients had an acute isolated picture of heat exhaustion that were not caused by extremely high environmental temperatures, though all cases occurred in the summer and fall. They conclude that serum electrolytes should be assessed regularly in children with CF.

Illustration A is a schematic of the defective chloride channel present in CF.

Incorrect Answers:
Answer 1, 3-5: Children with these developmental disorders are not at increased risk for heat-associated morbidity.