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The Congenital Hyperinsulinism Center at Children’s Hospital of Philadelphia, with a high volume of approximately 80 congenital hyperinsulinism (HI) cases a year, sees a large share of unusual cases.
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Unrecognized or inadequately treated hyperinsulinism (HI) poses a high risk of adverse neurodevelopmental outcomes due to the combined insult of hypoglycemia and lack of alternate brain fuels.
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Inactivating mutations in the beta cell KATP channels are the most common cause of congenital hyperinsulinism (HI), accounting for approximately 60% of all cases with known genotype. The beta cell KATP channels play a very important role in the regulation of insulin secretion by coupling the metabolic state of the cell to membrane potential.
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For the children with focal hyperinsulinism, the radiologist’s interpretation of a 18F-DOPA PET/CT scan guides the surgeon, leading to a cure in 97% of cases.
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Nurse coordinators at the Congenital Hyperinsulinism Center assist patient families before the first appointment and through admission and follow-up.
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Sophie Foss, PhD, has joined the CHOP Congenital Hyperinsulinism Center as its neuropsychologist. See how she can help your child.
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For more than 50 years, Children’s Hospital of Philadelphia has been on the forefront of astounding advances in HI that have saved lives and improved lifetimes.
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Much progress has been made over the last 25 years when it comes to identifying the genetic mutation that is causing a child’s congenital hyperinsulinism (HI).
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Many children with hyperinsulinism (HI) have feeding aversion — as many as 60%. The use of tube feedings, and possibly continuous dextrose, to control their blood sugar, among other things (effect of medications, the appetite suppressing effects of insulin, etc.), may exacerbate their aversion to eating by mouth.
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If Leela Morrow, PsyD, the psychologist for the Congenital Hyperinsulinism Center at Children’s Hospital of Philadelphia, gives your child a referral for a neuropsychology evaluation it’s nothing to be afraid of. In fact, it may give your child a roadmap to success.