View clinical trials related to Congenital Hyperinsulinism.
Filter by:The primary objective of this study is to evaluate the utility of [18F]-DOPA PET to provide improved presurgical planning and distinguish between focal and diffuse forms of HI. The investigators will perform descriptive analysis, relying on visual analysis to diagnose and localize a focal lesion. Our findings will be compared to surgical histopathology to determine sensitivity and specificity or this technique. The investigators will also track patient surgical outcomes, specifically whether the patient is surgically "cured" or still requires medical management to control residual hypoglycemia.
The purpose of this pilot study is to generate data to assess feasibility of study design/procedures and for formal sample size estimation for a larger multicenter study of the efficacy and safety of sirolimus in infants with medically-unresponsive congenital hyperinsulinism (HI) due to inactivating mutations of adenosine triphosphate-sensitive potassium (KATP) channels.
Insulinoma: Insulinoma are rare, small insulin secreting neuroendocrine tumors. The only curative approach is the surgical excision. The preoperative detection remains a challenge. A non-invasive, highly sensitive tool in localizing the insulinomas would be appreciated in the preoperative work-up of these patients. To this aim Glucagon-like peptide-1 receptor (GLP-1R) imaging (Single Photon Emission Computer Tomography co-registered with a CT; SPECT/CT) could be a convenient tool. The possibly more sensitive approach of targeting GLP-1R using Positron emission tomography (PET/CT) methodology has not been investigated in patients so far.
Observational study in patients with non-focal congenital hyperinsulinism showing that restrictive surgery may improve the metabolic situation
Low blood sugars are known to cause brain damage in newborn babies. One of the most common causes of low blood sugars persisting beyond the new born period is a condition called congenital hyperinsulinism (HI). This is a disease whereby the pancreas secretes too much insulin and causes low blood sugars. Twenty to forty percent of these babies will have brain damage. There are two forms of this disease. In one form only a small part of the pancreas makes too much insulin (focal HI) and in the other, the whole pancreas make too much insulin (diffuse HI). Another very similar disease is insulinoma which occurs after birth, but also causes hyperinsulinism. If a surgeon could know which part of the pancreas has the focal lesion he could remove it and cure the patient. The purpose of this study is to investigate whether a new investigational drug called Fluorodopa F 18, when used with a PET scan, can find the focal lesion and guide the surgeon to remove it, thus curing the patient and preventing further brain damage.
Post-gastric bypass hyperinsulinemic hypoglycemia is a recently described disorder occurring in some patients after gastric bypass surgery for obesity. The pathogenesis is incompletely understood but involves a robust insulin response to ingested carbohydrate. The resultant hyperinsulinemia sometimes produces hypoglycemia with neuroglycopenia, confusion and even loss of consciousness. Various treatments have been recommended including low carbohydrate diets, coingestion of the medication acarbose with carbohydrate containing meals, partial pancreatectomy and even total pancreatectomy. None is completely satisfactory. We propose to test two new potential treatments. Using a design with random assignment of three conditions we plan to compare, in 10 patients with post-gastric bypass hyperinsulinemic hypoglycemia, a high carbohydrate test meal (control condition), a high carbohydrate test meal after pre-treatment with rapid acting aspart insulin (insulin condition), and a high fructose, low glucose test meal with carbohydrate and caloric content similar to the control meal (fructose condition).
This purpose of this study is to determine the ability of an 18F-fluoro-L-dihydroxyphenylalanine (18F-DOPA) PET (Positron Emission Tomography) scan to detect a focal lesion of hyperinsulinism and determine the location in patients with congenital hyperinsulinism, Beckwith Wiedemann Syndrome and suspected insulinoma. Safety data will be collected.
The purpose of this study is to continue follow up of conservatively treated CHI patients and to focus on their metabolic outcome, including frequency of hypoglycemia after discontinuing treatment and incidence of diabetes mellitus in the long term.
Children with congenital hyperinsulinism (CHI) have low blood sugar, and some of these children may require surgery to remove part or all of their pancreas. In this study, researchers will test how well a radioactive drug, 18-labeled L-fluorodeoxyphenylalanine (called F-DOPA) can detect a form of hyperinsulinism (focal HI) that may be cured by surgery. Eligible participants in this study will have positron emission tomography/computerized tomography (PET/CT) scans with F-DOPA prior to surgery.
The purpose of our study is to evaluate the efficacy and safety of Lanreotide Autogel in children with congenital hyperinsulinism already treated with Octreotide by pump. Congenital hyperinsulinism is a genetic disorder characterized by inappropriate insulin secretion resulting in persistent hypoglycemia (low blood sugars. Patients exposed to recurrent hypoglycemic episodes are at increased risk of developmental disorders, so identification and prompt management of patients are essential. Many patients are treated with the somatostatin analog Octreotide which is administered by continuous infusion using a pump (we use an insulin pump). This treatment may pose a huge burden and be stressful for patients and families as it demands intensive daily care. In an effort to simplify the daily care of our patients and improve their quality of life we will study the efficacy and safety of Lanreotide Autogel - a long-acting somatostatin analog that can be administered by injection once a month