About the diseases
CHI is a complex disease characterized by congenital low blood sugar levels, requiring prompt treatment and potentially associated with various genetic syndromes. Surgery may be necessary to remove pancreatic tissue. Insulinoma presents with episodes of low blood sugar and can be surgically removed. Pathologic ketotic hypoglycemia occurs without fasting or fever and can be treated with nutrition therapy or surgical intervention, depending on the cause.
Congenital hyperinsulinism (CHI):
CHI will often show shortly after birth with severely low blood sugar and a high risk of brain damage, if not promptly diagnosed and efficiently treated. A genetic analysis is informative for the type of CHI.
The focal type of hyperinsulinism is cured by surgery after a DOPA PET/CT scan of the pancreas. The diffuse type is widest possible treated by medicine and nutritional therapy support. Pancreatic surgery may, however, be necessary in severe cases.
Moreover, CHI can be seen in a number of syndromes.
Insulinoma:
Insulinoma presents with episodes of low blood sugar in school-age or later. Insulinomas are resected by surgery after imaging.
Sometimes, patients with insulinomas also have other hormone-producing tumors.
Pathological ketotic hypoglycemia:
Pathological ketotic hypoglycemia are conditions, in which the child without fever or prolonged fasting has increased fat metabolism and low blood sugar.
In some, a lack of a hormone or an enzyme can be detected as the cause and treated with hormone or enzyme and dietary therapy.
In others, the disease remains unexplained and must primarily be dietary treated, eventually with tube feeding.