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Hyperinsulinism: Kristen's Story 7 лет назад

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Hyperinsulinism: Kristen's Story
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Hyperinsulinism: Kristen's Story

For decades, Children’s Hospital of Philadelphia has been on the forefront of treating hyperinsulinism. http://www.chop.edu/hyperinsulinism . See how that expertise benefited both Brad and his daughter, Kristen. Back in the 1960s, Brad and his brother were treated for congenital hyperinsulinism (HI) at CHOP’s former location on Bainbridge Street. The brothers even participated in a drug trial that helped identify the front-line medication to treat the disease. The, 27 years later, Brad’s daughter, Kristen, was diagnosed with the same condition, and — of course — the family brought her to CHOP. CHOP’s expertise in this rare disease that began more than 50 years ago had continued to develop, and was in full force to help Kristen. In fact, Kristen was treated with the same medicine her father had helped prove was successful. There is now a multidisciplinary team that makes up CHOP’s Congenital Hyperinsulinism Center, the most active center in the world. Few babies are born with HI, with only one case in 50,000 births, but CHOP’s unparalleled experience treating these children means patients receive the best, most advanced care available. The center is directed by pediatric endocrinologist Diva De León-Crutchlow, MD, MSCE. When a baby has HI, the beta cells in their pancreas release way too much insulin, which lowers their blood sugar to dangerous levels. Severe low blood sugar can cause seizures and, potentially, brain damage or even death. That’s why it’s essential to raise sugar levels to the normal range — and keep them there. Sugar is also necessary for brain growth and development. About half of the approximately 80 patients who come to CHOP each year can have their HI managed with medication, like Kristen does. Some patients, who have a severe form of the disease called diffuse hyperinsulinism, need to have nearly all of their pancreas removed to keep their blood sugars under control. Researchers at Children’s Hospital are actively exploring ways to improve treatment for these children. Other children have a form of the disease called focal hyperinsulinism, where only a part of their pancreas is involved. These babies can often be cured through surgery. Since CHOP’s HI Center opened 20 years ago, more than 240 children have been cured.

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