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The current standard of care for MIBC is radical cystectomy (RC) combined with neoadjuvant platinum-based chemotherapy ( Figure 1 )

OROT: Orotic acid, random, urine OAU: Urine organic acids screen AAPD: Urine amino acids management of hyperammonemia management of hyperammonemia getting started: risk stratification and aggressiveness of treatment Notes : Status epilepticus may cause a very abrupt, transient rise in NH3

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Surgery should not be categorically contraindicated in MC4R -deficient patients, but careful patient selection and informed consent are paramount