Because we have evidence of diabetes mellitus with insulin resistance, variable LD features, and kidney disease, we recommend the following investigations in all individuals with biallelic POC5 variants: yearly cardiovascular risk management screening, including blood pressure, lipids, glucose, insulin, liver and kidney function, ultrasound and fibroscan of the liver, urine albumin-to-creatinine ratio, and CK. Here, INS is linked to diabetes mellitus.