Philadelphia, Pa. -- A diagnostic test performed in newborns allows physicians to identify a rare, severe imbalance of insulin levels -- a disease that can be cured with organ-sparing surgery. The diagnostic test was developed by physicians at The Children's Hospital of Philadelphia, who reported their findings on June 21 at an international conference of the Endocrine Society in Toronto, Canada.
When insulin levels rage out of control in newborns with a condition called congenital hyperinsulinism (HI), irreversible brain damage may result. Medication can treat some forms of congenital HI, but other forms require surgery on the pancreas, the organ that produces insulin. The Children's Hospital team presented results of an acute insulin response test that allows physicians to accurately identify the subtype of congenital HI before surgery.
Accurate diagnosis is important because one type of HI is diffuse, occurring throughout the pancreas and requiring that 95 percent of the organ be removed, a procedure called near-total pancreatectomy. Focal HI, on the other hand, affects only one localized part of the pancreas and can be cured by surgically removing that portion. The different surgeries have different outcomes: "Children who must receive a near-total pancreatectomy are at risk for diabetes, while those undergoing partial pancreatectomy are cured of HI and not vulnerable to diabetes," said Charles A. Stanley, M.D., Director of the Hyperinsulinism Program at Children's Hospital and senior author of the study.
Clinical symptoms are indistinguishable between diffuse and focal HI, and microscopic cellular differences are not visible by standard imaging techniques or during a surgeon's visual examination of the baby's pancreas, which is about the size of an adult's finger. "Acute insulin response testing allows us to accurately differentiate between diffuse and local lesions, before surgery is performed," said Dr. Stanley. In January,
Contact: Kajsa Haracz
Children's Hospital of Philadelphia