3-methylglutaconic aciduria in pregnancy

776 Vol 349 • March 15, 1997 identified. Type IV probably represents a heterogeneous group of conditions which will be further sub-classified in the future. There is one previous report in which raised urinary concentrations of 3-MGA were found from two previously healthy women who presented with toxaemia of pregnancy. Both pregnancies resulted in the delivery of healthy infants with normal development. Although the source of 3-MGA in 3MGA aciduria types II to IV has not been identified, it is possible that during pregnancy the flux through the de novo steroid biosynthetic pathway could result in the accumulation of dimethylallylpyrophosphate, which in turn could be converted to 3-MGA through the mevalonic acid shunt pathway. Some of the 3-MGA could be dehydrogenated to yield 3-methylglutarate. Based on our observations and those reported by Kuhara et al we suggest that raised urinary concentrations of 3-MGA may be a normal finding during pregnancy.