Abstract
Pyruvate dehydrogenase complex (PDHC) is a critically important enzyme for energy production. Hence, disorders related to this enzyme can lead to lactic acidosis. Here, we report a case wherein the patient had a disorder of the PDHC leading to intractable lactic acidosis, and acute blood purification proved to be a useful treatment option. The patient was a 3-year-old girl who was admitted to the intensive care unit because she had developed severe lactic acidosis with disturbed consciousness. Although she immediately received continuous purification therapy, she did not show improvement in the lactic acidosis. Subsequently, we increased the dialysis flow rate, leading to successful reduction of the blood lactate level. Hemodiafiltration was discontinued, but the patient did not gain consciousness because she had already developed a cerebral disorder, which was considered to be due to the lactic acidosis. The clinical course of this case suggests that continuous blood purification with a high dialysis flow rate would be useful for the treatment of intractable lactic acidosis. A review of cases, such as the one presented here, would provide some useful information for determining the best setting for acute blood purification therapy in pediatric cases.
