Patients with urea cycle disorders who are usually well controlled can easily decompensate during surgery, particularly if catabolism is precipitated by fasting and surgery. Elective surgery in these patients is usually best done at the hospital with the regional metabolic unit. It is important to follow an appropriate protocol, minimising catabolism by providing adequate amounts of carbohydrate. This protocol should be used in conjunction with the emergency regimens on the BIMDG website.
The following instructions apply to patients with:
Sections of the guideline include: history taking and preparation, pre-operative management, extra intra-operative instructions, post-operative procedure.