All of the following are excreted in urine in Maple syrup urine disease, EXCEPT:

## Core Concept
Maple syrup urine disease (MSUD) is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC). This enzyme complex is necessary for breaking down certain amino acids (leucine, isoleucine, and valine). The disease leads to the accumulation of these amino acids and their toxic byproducts.

## Why the Correct Answer is Right
The correct answer, **Leucine**, is indeed one of the amino acids that accumulate in MSUD but understanding why it's the correct answer requires analyzing the others. In MSUD, the metabolites that are typically excreted in urine include the branched-chain ketoacids (alpha-ketoisocaproic acid, alpha-ketoisovaleric acid, and alpha-ketomethylvaleric acid) and the branched-chain amino acids themselves (leucine, isoleucine, and valine) are elevated but not directly excreted as such; rather, their metabolites are. However, focusing on direct excretion related to the question:

## Why Each Wrong Option is Incorrect
- **Option A:** Not provided, assuming relates to a relevant compound.
- **Option B:** Not provided, assuming relates to a relevant compound.
- **Option C:** Not provided, assuming relates to a relevant compound.
- Given no specifics on A, B, C, but assuming they relate to compounds like **alpha-ketoisocaproic acid, alpha-keto-beta-methylvaleric acid, and alpha-ketoisovaleric acid** (metabolites of leucine, isoleucine, and valine respectively), these are indeed excreted.

## Why the Correct Answer is Right Given Typical Excreted Substances
In MSUD, **Leucine** itself isn't directly "excreted" in the traditional sense but accumulates. The disease is characterized by the excretion of **branched-chain ketoacids** (like alpha-ketoisocaproic acid from leucine) rather than the amino acids directly. However, all options should be evaluated based on typical clinical and biochemical findings.

## Clinical Pearl / High-Yield Fact
A key point to remember is that MSUD leads to severe neurological impairment if not treated early. The urine of patients with MSUD has a characteristic **maple syrup** odor due to the presence of these organic acids.

## Correct Answer: D. Leucine.