Clinical Manifestations. Patients with BCOAs may initially have nonspecific manifestations such as poor feeding, lethargy, dehydration, and vomiting. In the case of isovaleric aciduria, patients may have a distinctive “sweaty feet” odor during the acute illness caused by the accumulation of isovaleric acid. In laboratory analysis, there is evidence of anion gap acidosis, ketosis, hypocalcemia, hyperlactaciduria, and hyperammonemia. Serum glucose level may be normal, reduced, or elevated, and hematologic abnormalities (such as pancytopenia) are common. Other manifestations may include constipation, abdominal distention, pancreatitis, seizures, growth delay, cognitive impairment, and movement disorders (eg, tremors, choreoathetosis, and dystonia). Stroke manifestations are described in the next subsection.