Congenital Hyperinsulinism Center News and Updates

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Two Focal Lesions: Extra Rare Cases Not so Rare at CHOP

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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.

Neurodevelopmental Outcomes in HI

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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.

When Hyperinsulinism is a Family Affair

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Three Generations Under CHOP’s Care for HI - Rian

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.

Radiology: Key Member of the HI Team

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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.

Meet the HI Center’s Nurse Coordinators

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Nurse coordinators at the Congenital Hyperinsulinism Center assist patient families before the first appointment and through admission and follow-up.

Navigating Continuous Dextrose and Nutrition for Children with HI

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kid with dextrose packsack

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. 

Understanding the Benefits of a Neuropsychology Evaluation

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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.


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