Fructose-1,6-bisphosphatase plays an important role in the liver where it facilitates the production of sugars. This is an important process for maintaining blood sugar levels when someone is fasting. Therefore, a defect in fructose-1,6-bisphosphatase will lead to low blood sugar levels and acidification of the blood as soon as there is no intake of food. These problems occur soon after birth.
This condition is extremely rare. It is estimated that 1 in 350,000 people have this condition. Treatment consists of avoiding fasting. Frequent feeding is important. In childhood, nocturnal feeding (with a tube) may be necessary, or uncooked cornstarch is taken at bedtime. Furthermore, foods containing fructose (and sorbitol) should be limited, as they can trigger low blood sugar and acidosis. If frequent feeding is not possible - due to a gastroenteritis, for example - a person should be acutely admitted to administer sugar via IV.