Which statement best describes the classic cystic fibrosis presentation?

Which statement best describes the classic cystic fibrosis presentation?

• A. Classic CF presentation: young patient with bronchiectasis, foul-smelling sputum, chronic sinusitis, pancreatic insufficiency, growth delays, infertility, and meconium ileus at birth
• B. CF presenting only with liver disease
• C. CF presenting as isolated dermatitis
• D. CF presenting in old age with osteoarthritis

Answer: (D)

The key idea is recognizing cystic fibrosis as a multisystem disease that presents early due to thick, sticky secretions affecting both the lungs and the pancreas. Classic CF shows up in childhood with respiratory tract disease and pancreatic insufficiency, leading to growth delays. Meconium ileus at birth is a hallmark early sign, and later manifestations include bronchiectasis with foul-smelling sputum, chronic sinusitis, and infertility in males from congenital absence of the vas deferens. This combination reflects CFTR dysfunction causing obstruction and malabsorption across organs. Descriptions limited to liver disease or dermatitis don’t capture the full, early multisystem pattern, and onset in old age wouldn’t fit the typical CF course.

The key idea is recognizing cystic fibrosis as a multisystem disease that presents early due to thick, sticky secretions affecting both the lungs and the pancreas. Classic CF shows up in childhood with respiratory tract disease and pancreatic insufficiency, leading to growth delays. Meconium ileus at birth is a hallmark early sign, and later manifestations include bronchiectasis with foul-smelling sputum, chronic sinusitis, and infertility in males from congenital absence of the vas deferens. This combination reflects CFTR dysfunction causing obstruction and malabsorption across organs.

Descriptions limited to liver disease or dermatitis don’t capture the full, early multisystem pattern, and onset in old age wouldn’t fit the typical CF course.