| Popis: |
The pyruvate dehydrogenase complex deficiency (PDCD) is a rare genetic neurometabolic disorder. It belongs to the group of mitochondrial diseases. Clinical manifestations range from often fatal, severe, neonatal lactic acidosis to serious neurological disorders later in life. Most patients do not reach adulthood and are rarely present in intensive care units for adult patients. One of the most important therapeutic procedures in these patients is a ketogenic high-fat diet that produces ketones as an alternative fuel for the body and brain. Sometimes a parenteral ketogenic diet may be required ; however, it has not been yet precisely defined in existing nutrition guidelines for intensive care patients. Here we described the implementation of parenteral ketogenic diet, along with the basics of laboratory and clinical monitoring in the 18-year old patient with mitochondrial disease (PCDC) after abdominal surgery associated with inflammatory complications, while enteral intake was not possible. It required a detailed calculation of the basic ingredients of the diet to ensure an adequate supply of energy, volume and micronutrients, while producing ketosis. Together with other intensive care methods, it helped resolve the complications and treatment outcome of this episode. In conclusion, properly prescribed and implemented parenteral ketogenic diet in patients with PCDC reduces the incidence of lactic acidosis and neurological complications. It should be performed exclusively in intensive care units, mainly due to continuous laboratory and clinical monitoring. Whenever possible, enteral nutrition should be resumed as soon as possible. It would also be desirable to have well-defined guidelines for parenteral ketogenic nutrition. |
