DGKEnephropathy is a very rare inherited kidney disease. It is caused by changes in a gene called DGKE. This condition usually starts in babies or very young children. It can cause serious kidney problems, either all at once during a flare-up or slowly over time.
Many children with this condition have:
• Protein in the urine
• Blood in the urine
• High blood pressure
• Low red blood cells (anemia)
• Low platelets
• Kidney trouble during flare-ups
Some children have flare-ups that look like a condition called atypical hemolytic uremic syndrome (aHUS). Other children mainly have long-lasting kidney problems with lots of protein in the urine. This condition is usually inherited in an autosomal recessive way. That means a child gets one non-working copy of the gene from each parent. Parents are usually healthy carriers. Because it is a genetic condition, it is not caused by an infection and cannot be spread from one person to another.
DGKE