Maple Syrup Urine Disease (MSUD) and Recurrent Infection

Patients with Maple Syrup Urine Disease (MSUD) may have recurrent infections. In turn, infection may trigger metabolic decompensation.

Patient selection: maple syrup urine disease (MSUD)

Recurrent infections may include:

(1) esophageal Candidiasis

(2) Molluscum contagiosum

(3) other viral infections

(4) recurrent bacterial infection

(5) infection of central venous catheters (CLABSI, insertion site)

The mechanisms are uncertain:

(1) acidosis

(2) inhibition of T-cell function from elevated plasma leucine

(3) impact of amino acid deficiencies on immunoglobulin production

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