About Careers Internship MedBlog Contact us
Medindia LOGIN REGISTER
Advertisement

Surgery Benefits for Congenital Hyperinsulinism

by Colleen Fleiss on May 9, 2018 at 1:10 AM
Surgery Benefits for Congenital Hyperinsulinism

Surgeons can treat virtually all patients with the focal, or localized, form of congenital hyperinsulinism, a rare genetic disease, revealed a review of nearly 500 cases of infants with severe congenital hyperinsulinism who underwent partial or near-total removal of their pancreas for persistent hypoglycemia at Children's Hospital of Philadelphia (CHOP).

The review, first presented during the 2018 American Pediatric Surgical Association Annual Meeting, represents the world's largest single-center case series of patients with congenital HI. "Over the course of the past two decades, The Congenital Hyperinsulinism Center at CHOP has taken a multidisciplinary approach that combines pediatric endocrinology, genetics, radiology, pathology, and surgery to diagnose and treat appropriately selected HI patients with pancreatectomy," said N. Scott Adzick, MD, Surgeon-in-Chief at Children's Hospital of Philadelphia and lead author of this paper. "Since most children's hospitals only encounter one or two cases per year, it is important that children with this rare disease receive medical care from an experienced team."

Advertisement


In congenital HI, inherited mutations disrupt the insulin-secreting beta cells, allowing insulin levels to become excessive, essentially making it the opposite of diabetes. This oversecretion causes persistent hypoglycemia (low blood sugar) and can lead to irreversible brain damage or death if not treated properly. "Many families experience weeks of stress before they are transferred to CHOP for definite treatment," said Diva De León-Crutchlow, MD, a pediatric endocrinologist and Director of CHOP's Congenital Hyperinsulinism Center and co-author of the paper. Patients with focal disease can be cured with a selective partial pancreatectomy, which removes only the affected portion of the pancreas and preserves the normal pancreas. Alternatively, patients with diffuse disease often require a near-total pancreatectomy in which surgeons remove at least 95 percent of the pancreas. This surgery helps control the hypoglycemia but increases the patient's long-term risk of developing diabetes. There are also a variety of atypical cases of HI that require special considerations.

According to this review, 47 percent of patients with surgically treated HI between 10 and 20 years old have diabetes, and all patients who developed diabetes had near-total pancreatectomies to treat diffuse HI. The team is studying a variety of aspects of the disease to better treat patients with diffuse HI. In addition to studying the underlying genetic causes of HI, they are also studying how the disease functions at a molecular level with the goal of developing targeted therapies that would reduce the need for surgery. "We are actively pursuing clinical trials for new therapies that may improve the quality of life for patients with diffuse HI, including an investigational drug called exendin-(9-39) that can increase fasting blood glucose in these patients," De Leon-Crutchlow said. "The therapy has been shown to be effective in older patients and we are currently studying its effects in younger patients."
Advertisement

The study team is also examining the use of modern technology for monitoring and treating hypo- and hyperglycemia in children and adults with hyperinsulinism. "Our team's approach is able to distinguish focal from diffuse HI, localize focal lesions, and cure almost all focal patients with a partial pancreatectomy, and while we cannot cure diffuse HI, we can help prevent brain damage and severe hypoglycemia commonly associated with the disease," said Adzick. "When all is said and done, the children treated with our approach to focal HI have been able to lead normal lives and not worry about the effects of this rare condition."

Source: Eurekalert
Font : A-A+

Advertisement

Advertisement
Advertisement

Recommended Readings

Latest Child Health News

Do Adverse Drug Reactions Cause Autism Spectrum Disorder?
Side-effects of valproate drug taken during pregnancy, enhance the expression of Rnf146 gene, causing autism spectrum disorder in fetus.
Amblyopia or Poor Vision in Kids - Does Digital Vision Training Help?
Children with amblyopia or lazy eye who underwent digital vision training, showed no appreciable improvement in their eyesight.
Childhood Malnutrition Linked to Stunted Growth and Mortality Risk
In 2022, over 20% of children worldwide lacked adequate calories for growth, with 45+ million showing wasting (underweight for height).
Pneumococcal Vaccines Reduce Severe Infections in Kids With Sickle Cell Disease
After PCV7 licensure, pneumococcal infection rates in children aged 5+ with sickle cell disease significantly decreased.
Breast Milk Proteins to Boost Baby's Gut Health
Breast milk concentration of certain key proteins indicates abundance of healthy bacteria in babies' guts.
View All
This site uses cookies to deliver our services.By using our site, you acknowledge that you have read and understand our Cookie Policy, Privacy Policy, and our Terms of Use  Ok, Got it. Close
MediBotMediBot
Greetings! How can I assist you?MediBot
×

Surgery Benefits for Congenital Hyperinsulinism Personalised Printable Document (PDF)

Please complete this form and we'll send you a personalised information that is requested

You may use this for your own reference or forward it to your friends.

Please use the information prudently. If you are not a medical doctor please remember to consult your healthcare provider as this information is not a substitute for professional advice.

Name *

Email Address *

Country *

Areas of Interests