Congenital hyperinsulinism (HI) is a disorder that causes low blood sugar (hypoglycemia) in infants and children. Other names that have been used in the past include nesidioblastosis or PHHI (persistent hyperinsulinemic hypoglycemia of infancy).
Congenital hyperinsulinism is rare; most children's hospitals encounter only one or two cases a year. Since October 1998 the Congenital Hyperinsulinism Center at The Children's Hospital of Philadelphia has evaluated and treated more than 400 patients — making it the largest program of its kind in North America.
Providing full services for congenital hyperinsulinism requires sophisticated multidisciplinary team capabilities. Our Center includes a team of specialists in endocrinology, pediatric surgery, pathology and interventional radiology who have long experience in treating infants with this condition.
In addition, CHOP has a nationally top-ranked neonatal intensive care unit, allowing us to provide seamless care for children with complex conditions.
Our Center performs 18F-DOPA PET scanning under an FDA-approved research protocol (for appropriate cases). This innovative imaging technique helps surgeons pinpoint abnormal tissue and focal lesions, sparing healthy cells in the pancreas, significantly reducing the risks of diabetes in patients with focal HI, and potentially leading to a cure. This technology can reduce unnecessary near-total pancreatectomies.
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