Which metabolic disorder presents with an odor similar to boiled cabbage?

Tyrosinemia is a metabolic disorder characterized by the accumulation of tyrosine and its byproducts due to a deficiency of specific enzymes involved in its metabolism. One of the distinct features of tyrosinemia, particularly in types I and II, is the development of a characteristic odor. The odor described as similar to that of boiled cabbage arises from the presence of certain metabolites that are excreted in the urine, as well as their effect on the skin and breath.

This disorder can lead to several clinical manifestations, including liver dysfunction and neurological symptoms, depending on the type and severity. The association of the abnormal metabolic products with this specific odor helps in the clinical recognition of the disease, providing valuable information for diagnosis and management.

In contrast, other metabolic disorders listed do not typically present with a cabbage-like odor. For instance, maple syrup urine disease produces a sweet odor reminiscent of burnt sugar due to the accumulation of branched-chain amino acids, while urea cycle disorders often lead to a distinct "ammonia" smell due to increased ammonia levels in the blood. Acetoacetyl CoA deficiency is less associated with a specific odor that could point directly to its identification. Thus, the association of tyrosinemia with the odor of boiled