### What is Congenital Hyperinsulinism? Congenital hyperinsulinism (HI) is a rare genetic disorder that causes very low blood sugar levels in babies and children. It occurs when the beta cells in the pancreas produce too much insulin, the hormone responsible for converting blood sugar (glucose) into energy. ### Symptoms Identifying HI can be challenging, especially in newborns, as symptoms can be mistaken for normal baby behavior. Common signs of low blood sugar (hypoglycemia) include: - Jitteriness - Poor feeding - Lethargy - Seizures In older children, symptoms may also include: - Confusion - Irritability - Weakness Severe low blood sugar can lead to permanent brain damage if not treated promptly. ### Causes HI can be inherited due to mutations in any of the 10 genes that control insulin release. Other causes include perinatal stress and various syndromes. Types of HI include: - Diazoxide-responsive and diffuse KATP HI - Focal KATP HI - GDH-HI - Glucokinase HI - HNF1a and 4a defects - MCT-1 HI - SCHAD-HI - UCP-2 HI ### Diagnosis Diagnosing HI involves taking a detailed medical history, performing lab tests to check insulin and blood sugar levels, and possibly genetic testing. ### Treatment The goal is to maintain normal blood sugar levels. Treatments include medications like diazoxide, octreotide, and glucagon. In some cases, surgery to remove part or all of the pancreas may be necessary. Quick diagnosis and proper treatment can help prevent long-term complications like neurological delays.