Which enzyme deficiency causes hemolytic anemia-
**Core Concept**
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic disorder that affects the enzyme glucose-6-phosphate dehydrogenase, which is a crucial component of the pentose phosphate pathway. This pathway is responsible for generating NADPH, a reducing agent that protects red blood cells from oxidative damage.
**Why the Correct Answer is Right**
The correct answer is due to the role of G6PD in maintaining the reducing environment of the cell. In the absence of this enzyme, red blood cells become more susceptible to oxidative stress, leading to hemolysis. The pentose phosphate pathway generates NADPH through the reduction of NADP+ to NADPH, which is essential for maintaining the reduced state of glutathione. Glutathione, in turn, protects hemoglobin from oxidative damage by reducing methemoglobin to its reduced form.
**Why Each Wrong Option is Incorrect**
* **Option A:** Pyruvate kinase deficiency is a cause of hemolytic anemia, but it is not related to the pentose phosphate pathway or the generation of NADPH.
* **Option B:** Glucose-6-phosphatase deficiency is associated with glycogen storage disease type I, which is a metabolic disorder affecting glycogenolysis, but it is not related to hemolytic anemia.
* **Option C:** Aldolase B deficiency is associated with hereditary fructose intolerance, a disorder affecting fructose metabolism, but it is not related to hemolytic anemia.
**Clinical Pearl / High-Yield Fact**
G6PD deficiency is a common cause of hemolytic anemia, particularly in individuals of African, Mediterranean, or Southeast Asian descent. It is essential to recognize this condition in patients presenting with acute hemolytic anemia, as treatment involves avoiding triggers that exacerbate oxidative stress.
**Correct Answer: D. G6PD deficiency.**