All of the following are seen in a patient with cystic fibrosis except
The options are missing, but common exceptions in CF might include something like normal pancreatic function, absence of meconium ileus, or normal sweat electrolytes. Let me recall the typical findings. CFTR mutations lead to thick mucus, so pancreatic exocrine insufficiency is common. Meconium ileus occurs in newborns. Sweat chloride is elevated.
If an option says "normal sweat chloride test," that would be the exception. Alternatively, if an option states "absence of male infertility," but in reality, most males with CF are infertile due to congenital absence of vas deferens. So an option suggesting normal fertility would be incorrect. Another possibility is normal lung function, but CF usually causes chronic lung issues.
Wait, the question is asking for what is *not* seen. So the correct answer would be an option that contradicts typical CF manifestations. For example, if one of the options is "normal pancreatic function" (Option C), that would be the exception because most CF patients have pancreatic insufficiency. Therefore, the correct answer would be Option C.
Now, structuring the explanation. The core concept is the pathophysiology of CFTR mutations leading to mucus abnormalities. The correct answer explanation would detail why that option is wrong. Each incorrect option should be addressed with why they are typical in CF. The clinical pearl would be to remember the classic triad and common complications.
**Core Concept**
Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the **CFTR gene**, leading to defective chloride and sodium transport. This results in viscous mucus accumulation in exocrine glands, pancreatic insufficiency, obstructive lung disease, and elevated sweat chloride levels.
**Why the Correct Answer is Right**
The exception in CF is **normal pancreatic function**. Over 85% of CF patients have **pancreatic exocrine insufficiency** due to ductal obstruction by thick secretions, impairing enzyme delivery to the intestines. This leads to malabsorption, steatorrhea, and fat-soluble vitamin deficiencies.
**Why Each Wrong Option is Incorrect**
**Option A:** *Meconium ileus in neonates* is a classic CF presentation due to dehydrated meconium.
**Option B:** *Elevated sweat chloride* (>60 mmol/L) is diagnostic of CF.
**Option D:** *Congenital absence of vas deferens* occurs in ~98% of males with CF.
**Clinical Pearl**
Remember the **classic triad**: **chronic obstructive pulmonary disease**, **pancreatic insufficiency**, and **sweat chloride elevation**. Pancreatic insufficiency is nearly universal in CF, making "normal pancreatic function" an impossible finding.
**Correct Answer: C. Normal pancreatic function**