KETOTICHYPOGLYCEMIA.ORGv Ketotic Hypoglycemia International Who we are, what we do and why Ketotic Hypoglycemia INTERNATIONAL What is ketotic hypoglycemia? “Ketotic hypoglycemia may be unexplained, or idiopathic (IKH). This is a challenge and should urge for more research” For the patients In a normal person, fuel for the brain and the general cell metabolism primarily comes from the burning of sugar deposits (glycogen). When the glycogen stores are depleted, the body will switch to burn fat deposits. The fat burn lead to two fuels for the brain, both glucose (sugar) and ketone bodies. However, ketones in the blood will lead to nausea and eventually vomiting. This will lead to a vicious circle, where you cannot eat or drink sugar-rich items, which again leads to further fat burn and production of ketone bodies. In a KH-patient, the glycogen stores are somehow insufficient. This leads to For the doctors decreased fasting tolerance with earlier Ketotic hypoglycemia can be seen in children Emergency treatment constitutes of oral onset of fat burn and hence ketone bodies. In because of growth hormone deficiency, cortisol or i.v. glucose, eventually i.m. glucagon, to most patients, the hypoglycemia is relatively deficiency, metabolic diseases with intact fatty raise the plasma glucose, which will prevent mild, and the ketone bodies helps to provide acid consumption, including glycogen storage further lipolysis. However, the ketones can fuel to the brain, which prevents loss of diseases (glycogenosis; GSD) type 0, III, VI, and take hours to be eliminated. In more severely consciousness and convulsions. However, in IX, or disturbances in transport or metabolism affected patients, the ketone production relatively few patients, the condition is more of ketone bodies. When these diagnoses can be significant before hypoglycemia severe, but still without an identified cause are excluded, ketotic hypoglycemia can be occurs, why recognition of increased ketones despite intense investigations in hormones categorized as unexplained or idiopathic (IKH), above approximately 1.5 mol/L is important, and cell metabolism. Such patients are said otherwise known as accelerated starvation. sometimes already when the glucose having “idiopathic” KH, or IKH, which simply concentration goes below 3.9 mmol/L (70 mg/ means KH without any known cause. The primary treatment is dietary prevention dL). with long carbohydrates (e.g. uncooked Source: Danielle Drachmann + cornstarch) and frequent meals. In some more Source: Professor Henrik Professor Henrik Christesen, severely affected patients, continuous feeding Odense University Hospital, DK Christesen, Odense University may be needed through a gastrostomy tube. Hospital, DK 2 3 What is this organization about? ”Idiopathic ketotic hypoglycemia is the most frequent state with low blood sugar in preschool kids” This organization has been established to globally unite leading hypoglycemia experts with the patients, to help establish a better understading of ketotic hypoglycemia, and create opportunities for better treatments. The literature provides a very vague description and thesis about the cause of ketotic hypoglycemia, as being in the range of normal. Due to this widespread perception, many patients, often small children, gets dismissed with the note: “eat a snack before bedtime”. We question the understanding of ketotic hypoglycemia being in the range of normal, why we in a collaboration with head of KHI’s Scientific Advisory Board has established a campaign to help spread awareness of ketotic hypoglycemia as more than normal variation. 4 5 Where to find us Patients and KH-families can be found across the web (and we do have a website), but are especially visible on social media pages and groups. This is where we support, guide and help each other navigating the KH-rollercoaster ride. 6 7 v ketotichypoglycemia.org Danielle Drachmann Executive Director Ketotic Hypoglycemia International print sponsored by [email protected] www.ketotichypoglycemia.org +4550691008 v Thank you 8.