Mutations to the three genes result in decreased or no activity of the enzymes. These amino acids are present in protein-rich foods such as milk, meat, and eggs. These genes provide instruction for the human body to make enzymes (BCKDH complex enzymes) which are essential for breaking down amino acids including leucine, isoleucine, and valine. Maple syrup urine disease is caused by mutations in one of three genes – BCKDHA, BCKDHB or DBT. MSUD is believed to affect 1 in 380 people in the Old Order Mennonite population and 1 in 26,000 in the Ashkenazi Jewish population. MSUD is more common in the Mennonite population in comparison to the general population. MSUD is believed to affect 1 in 185,000 births worldwide. Babies who receive abnormal results from early metabolic screening tests may be referred to the Newborn Metabolic Screening Program at Children’s Hospital of Philadelphia. A heel-prick is used to obtain a sample of the baby’s blood and tested for abnormally high levels of amino acids. Pennsylvania, New Jersey and Delaware all require newborn screening for maple syrup urine disease. In most cases, MSUD is diagnosed at birth as part of routine newborn screening tests, which are required in many states. However, even with treatment, some children with this disease can suffer severe disability and paralysis. In most cases, MSUD can be successfully managed with a specialized diet that breaks down the three specific amino acids – leucine, isoleucine and valine – preventing them and their toxic by-products from accumulating abnormally in the blood. Therefore, early detection and treatment essential.

If not diagnosed and treated soon after birth, maple syrup urine disease (MSUD) can be life threatening – as early as the first two weeks of life. Industry revenue of “flavouring syrup and concentrate manufacturing“ in Washington 2012-20.Maple syrup urine disease is a rare inherited disorder caused by the body’s inability to properly process amino acids, leading to a characteristic odor of maple syrup in the baby's urine.Industry revenue of “flavouring syrup and concentrate manufacturing“ in Illinois 2012-2024.Industry revenue of “flavouring syrup and concentrate manufacturing“ in Washington 2012-2024.

Value of maple product exports in Canada by province 2008-2017.Farm cash receipts of maple products in Canada 2008-2021.Top global importers of maple sugar and maple syrup 2008-2012.Export value of maple sugar and maple syrup from Denmark 2009-2013.Most used types of pancake / table syrup in the U.S.Amount of pancake / table syrup used within 30 days in the U.S.Leading European export destinations for maple sugar and maple syrup from Canada 2013.Leading European countries for export of maple sugar and syrup in 2013.Maple syrup farm value in Ontario 2000-2021.Ontario maple syrup production 2000-2021.U.S.: maple syrup production value 2011-2020.Maple syrup taps in Canada by province 2011-2016.Maple syrup farms in Canada 2016, by province.maple syrup production share 2014, by state consumers, the most popular brand of pancake syrup was Aunt Jemima, followed by store brand. As the top producer of maple syrup in the United States, Vermont also had the lowest price for maple syrup. Connecticut, and Minnesota have the highest average price of maple syrup. The average price per gallon of maple syrup in the United States varies greatly from state to state. There were about 13.34 million maple taps in the United States in 2019.

This sap is then processed into syrup and bottled. Maple syrup is harvested by inserting a tap into a maple tree and collecting the tree sap. The United States is a major producer of maple syrup, with a total production volume of around 4.24 million gallons of maple syrup in 2019. The second leading producer, New York, had a production volume of about 647 thousand gallons of maple syrup in that year. In 2021, the state of Vermont produced over 1.5 million gallons of maple syrup, making it the top producer of maple syrup in the United States.