Data Review Demonstrates That Congenital Hyperinsulinism Patients Benefit From Surgery

According to a story from Newswise, almost 500 infants that were born with congenital hyperinsulinism were treated with surgery that involved the removal of either part of or the entirety of their pancreas at the Children’s Hospital of Philadelphia. A review of these cases indicated that this procedure could cure patients that had the localized variant of this condition. Removal of the pancreas can also help patients that have the diffuse variant as well by preventing complications.
Congenital hyperinsulinism refers to several genetically linked disorders that cause low blood sugar due to excessive insulin production. The severity of symptoms can vary greatly in severity, but the condition typically becomes evident in early infancy. Changes to a total of nine different genes can cause congenital hyperinsulinism. In infancy, the resulting hypoglycemia (low blood sugar) can cause symptoms such as fatigue, jitteriness, seizures, and unresponsiveness. Severe variants result in abnormally high birth weight and abnormalities of the pancreas. In milder cases, infants may need to feed more frequently. Other symptoms include intellectual disability, coma, dizziness, and enlargement of the heart. Treatment usually involves medication to maintain blood sugar levels. To learn more about congenital hyperinsulinism, click here.

In surgery, the determination to remove all or part of the pancreas depends on the severity. Patients with less severe cases can benefit from only removing the affected portion of the pancreas, but the worst cases require total removal of the organ (at least 95 percent). While this prevents patients from hypoglycemia, the surgery also increases the risk for the patients to develop diabetes during their lifetime.

This increased risk is most pronounced in those patients that needed to have the vast majority of the pancreas removed. In the data review, 47 percent of patients who underwent surgery developed diabetes, and all of the patients in this group were from the most severe cases that required the most extensive removal.

More targeted therapies are currently in development that should improve quality of life and reduce the need for surgical intervention. The drug exendin, for example, has proved effective for treating older patients and is currently be evaluated on younger ones as well.


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